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Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
Part IV Issues Specific to Disability
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Part IV Issues Specific to Disability

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  • Part IV of our recorded program we will explore reproductive health care issues that are disability-specific. We will be discussing reproductive health care as related to physical, developmental and sensory disabilities
  • As we discuss women with physical disabilities we will be focusing on reproductive health care for women with some specific conditions which impair musculo-skelatal movement of arms, legs and or spine requiring assistance or assistive devices to complete activities of daily living. This module is broken into 5 sub-modules. Although there are a variety of events – traumatic, genetic, environmental, infectious – that may result in physical disability, four of the most common conditions encountered by the obstetrician gynecologist are spinal cord injury, spina bifida, multiple sclerosis and cerebral palsy. We will also touch on two other physical disabilities that have specific implications for the obstetrician –gynecologist, osteogenesis imperfecta and post-polio syndrome. Access to comprehensive and safe reproductive health care, a major issue for women with physical disabilities will be addressed in Part 5 of this program..
  • Every year approximately 2,000 women in the US survive an acute traumatic injury to the spinal cord. The majority of these women are under age 25. (DeForge, AHRQ 2004) Women with spinal cord injury (SCI) have unique health care needs related to the physical and psychological changes that occur after injury. Research examining their health issues has been limited. In this module we will cover autonomic dysreflexia, menstruation, sexuality, pregnancy and osteoporosis as they relate to SCI. Other important considerations for women with SCI include bowel and bladder control, diet and physical activity Please see Part 3 for more information on these considerations.
  • Read objectives. To personify these objectives we will be considering composite case studies.
  • Suzanne had a T-4 spinal cord injury as a result of a skiing accident when she was 16. Now 25, she comes to your office requesting contraception. Suzanne works from home as a technical writer. She uses a manual wheel chair, she has good motor control of her upper extremities and is able to accomplish most of daily needs. She performs urinary self-catheterization 3 times daily. She has had frequent urinary tract infections and is currently taking a prophylactic antibiotic daily. Suzanne’s menstrual periods are regular but are quite heavy and her bleeding overflows her pads most every month, resulting in soiled clothing and wheel chair pad and great embarrassment. She has no other current complaints. Suzanne has become sexually active recently with a man she has known for some time. She would like to consider a pregnancy at some point in the future. What do you need to consider when prescribing contraception for Suzanne? What precautions are essential during the pelvic examination you will be performing? What other components of well woman care are crucial for Suzanne during this visit?
  • Spinal cord injury or SCI causes both damage to the myelinated fiber tracts carrying motor signals and damage to the gray matter, causing segmental losses of interneurons and motor neurons. The American Spinal Cord Injury Association has defined an international classification based on neurological levels, sensations tested in each dermatome and strength of ten key muscles on each side of the body. The classification system includes 5 types of injury. A – “Complete” where no motor or sensory function is preserved in the sacral segments S4-S5. B – “Incomplete” where sensory but not motor function is preserved below the neurological level including the sacral segments S4-S5 C and D indicates an ‘incomplete” injury where some degree of motor function is preserved below the neurological level. E – indicates recovery of normal motor and sensory function. (ASIA, 2006)
  • The exact level of the spinal cord injury predicts the probable extent of functional loss. While the prognosis of complete injuries are predictable, incomplete injuries are variable and will differ from this description. Cervical injuries usually result in full or partial tetraplegia. Those with an injury to: C3 vertebrae and above typically lose diaphragm function and require a ventilator C4 and C5 – may have some use of shoulders, but not of the wrists or hands C6 – have wrist control but no hand function C7 is generally the level for functional independence. C7-T1 have manual dexterity problems with fingers Thoracic level and below injuries result in paraplegia. The hands, arms, head and breathing are usually not affected. T1 to T8 lack control of the abdominal muscles so control of the trunk is difficult. Effects are less severe the lower the injury T9-T12 have good trunk and abdominal muscle control. Sitting and balance are not affected. The effect of injuries to the Lumbar and Sacral region are decreased control of the legs and hips, urinary system and anus.
  • Autonomic dysreflexia or ADR is a potentially life-threatening complication of SCI, with a lifetime incidence of 50 to 75% in patients with spinal cord lesions at or above the T6 level. ADR has been reported in patients with injuries below T6, but rarely and in a milder form. This condition results from a loss of hypothalamic control of sympathetic spinal reflexes and occurs in patients with viable spinal cord segments distal to the level of injury. It is important to ask patients with SCI at the time of intake about their history of ADR and treatment that has been used. In ADR, various noxious stimuli such as bladder distension activate pain receptors, sending inputs to the spinal cord below the level of the lesion and the spinothalamic tracts and posterior columns trigger sympathetic activation without supraspinal modulation . This causes sympathetic activation innervated by the efferent impulses below the level of the spinal cord lesion. In addition, arteriolar vasoconstriction results in dangerously high blood pressure and a secondary bradycardia induced by the baroreceptor response and vagal stimulation may also occur. ADR is potentially lethal and considered a medical emergency requiring immediate action.
  • The most common precipitant of ADR is distention or irritation of the bladder or bowel, such as a full bladder, a blocked urinary catheter, cystitis, instrumentation of the urinary tract (e.g., cystoscopy, urodynamic studies), or fecal impaction. Cutaneous lesions, including pressure sores and ingrown toenails are another important cause. Women may experience ADR with menstruation, sexual activity, and during pelvic or rectal examinations. Labor is a potent stimulus. One case report describes ADR in a young woman after treatment with oral misoprostol in conjunction with diclofenac for joint pain; there are no known reports of misoprostol use for obstetric/gynecologic indications in women with SCI and no other data on misoprostol triggering ADR.
  • The predominant finding in ADR is hypertension, which can rise as high as 300 systolic over 220 diastolic. Because resting BP in patients with SCI tends to be lower (~90/60), an otherwise “normal” BP (e.g., 120/80) may in fact be indicative of ADR in susceptible patients. Other common signs and symptoms include pounding headache, piloerection, and sweating and flushing above the level of the injury. Cardiac dysrhythmias may include bradycardia, tachycardia, or premature atrial contractions.
  • The ideal management for ADR is prevention, e.g. with careful bladder and bowel management and gentle examination technique. If the woman has a history of a previous episode of ADR, it is prudent to consider anesthesia for procedures such as colposcopy, endometrial biopsy or IUD placement. When ADR does occur, the key to treatment is to identify and remove the offending stimulus. Patients should be assisted into an upright, seated position, to attempt an orthostatic decrease in BP; any restrictive clothing or devices should be loosened. . If it occurs during an examination or procedure, stop immediately and attend to the patient. If she has experienced ADR in the past, she will likely know what is best for her in regard to treatment. Her blood pressure should be measured every 2-3 minutes to monitor the effectiveness of interventions. If the precipitating stimulus is not immediately apparent (e.g. a speculum examination), the bladder should be checked for overfilling and emptied via clean catheterization or uncoiling or flushing an indwelling catheter. If hypertension persists, a rapid-onset, short-acting antihypertensive such as nifedipine (10 mg sublingual) or nitrate paste may be administered (Campagnolo, 2008) If outside of an acute care setting, emergency assistance should be sought. Additional information on the specific issues of ADR during pregnancy and delivery can be found in the pregnancy section of this module . (link)
  • A multicenter study of self-reported reproductive health in 472 women with SCI found that most women stopped menstruating directly following SCI, resuming menses within an average of 4 months (with a range of 1 week to 24 months) (Jackson 1999) . In this study approximately 8% of women reported a permanent end to their cycles at the time of injury. In this same study, women with SCI reported a significant increase in muscle and bladder spasticity or autonomic symptoms such as sweating, headache or flushing either just before or with the onset of menses (Jackson 1995 and 1999). Although menstrual cramping was significantly decreased following SCI, the incidence or severity of other symptoms associated with premenstrual dysphoric disorder or PMDD such as irritability, emotional instability, and bloating was unchanged (Research Review 2000)
  • Spinal cord injury does not preclude an active and enjoyable sex life for affected women. In a self-reported study, 87% of women reported participating in sexual activity prior to SCI and 67% reported participating after injury. Sexual activity was more likely with increasing time since injury and in women with injuries at the thoracic or lumbar levels. (Jackson 1999) As mentioned in this recorded program, Part 1 Module 2 (link), vaginal lubrication is reflexive in response to genital stimulation, involves nerve roots S2-4, and occurs in women with complete SCI (Sipski 2006). Although changes that a woman experiences following SCI can affect her sexuality, approximately 50% of women with SCI can experience orgasm, which may be mediated via the vagus nerve or other non-spinal nerve pathways (Whipple 1998). No treatment with proven efficacy and widespread availability has been developed to manage female sexual dysfunction when spinal nerve pathways are impaired. However, in a small randomized controlled trial, Sildenafil was shown to increase subjective arousal in women with spinal cord injury (Sipski, 2000). The drug was well-tolerated, and caused only modest (4 mmHg. ) decreases in blood pressure and a concomitant slight increase (5 bpm) in heart rate.
  • It is estimated that as many as 57,000 women in the US are living with a spinal cord injury, most of whom are of reproductive age and capable of becoming pregnant. (Pereira, 2003) However, there are many misconceptions held by women with SCI and their providers concerning pregnancy. Providers may feel that pregnancy places women with SCI at too great a risk for health problems and encourage them not to become pregnant. Although pregnancy in a patient with a spinal cord injury may present clinical challenges to obstetric providers, with careful management, most women with SCI can have successful pregnancies.
  • There are scant data for guiding management of pregnancy in women with SCI. A recent systematic review of reproductive health after SCI revealed no studies investigating fertility after SCI, nor any literature that provided the necessary data to project the number and frequency of complications and other obstetric issues in women with SCI. (DeForge, AHRQ - 2004) Given the limitations in the published evidence, it is difficult to inform practice or policy regarding this important health issue. The guidance in this recorded program on pregnancy management for women with SCI is based largely on case report and other Level III evidence.
  • Some common complications of pregnancy for women with SCI include urinary tract infections, decubitus ulcers, alterations in pulmonary function, deep vein thrombosis, constipation and an increase in spasticity. Potential complications include anemia, pulmonary emboli and unattended delivery. (ACOG CO #275) Asymptomatic bacteruria occurs commonly in pregnant women with SCI. The incidence of lower urinary tract infections and pyelonephritis are increased. Incomplete emptying, neurogenic bladder, urinary diversions, and indwelling catheters are all risk factors for infection. Frequent laboratory monitoring with cultures is required and the use of suppressive antibiotic therapy may be necessary. (ACOG –CO #275 – 2002) Due to increasing uterine pressure on the bladder, pregnant women who use intermittent catheterization will likely require catheterization more frequently or may require an indwelling catheter. During pregnancy women with SCI should have routine skin checks, frequent position changes, and adequate padding to avoid skin breakdown and decubitus ulcers. Adjustments may need to be made to wheelchairs as pregnancy advances. Weight gain, edema, and anemia are contributing factors to the formation of skin ulcers. (See Part 2, Module 2 – Skin care) Women with high thoracic and cervical spine lesions (above T5) may develop impaired pulmonary function. It is suggested that these women have pulmonary function testing as their pregnancy progresses. Some women may require ventilatory support in late pregnancy and during labor and delivery. (Baker 1996) In later pregnancy, pressure from the growing fetus can impede venous blood return. (Jackson 2004) Lower extremity edema should to be monitored closely. Stockings that promote circulation, leg elevation and passive range of motion may help to reduce swelling. If the woman has a high risk of thrombosis, anticoagulant therapy may become necessary. (Jackson 2004) Constipation may become a serious problem for women with spinal cord injuries whose intestinal motility has been affected and/or who may also be using iron supplements to address pregnancy associated anemia. Stool impaction may also be associated with autonomic dysreflexia. (Pope 2001) Diets high in fiber, increased fluid intake and stool softeners are the first line of care for constipation. When indicated, a regime with suppositories or laxatives is necessary. Some pregnant and early postpartum women with SCI experience an increase in spasticity. This is typically only treated if the spasticity interferes with everyday activities or put the woman at risk for developing pressure ulcers. However, a sudden increase in spasticity may be an indication of an underlying medical condition or the onset of labor. Therefore a report of a change in the frequency or intensity of spasms should be quickly investigated. (Jackson 2004)
  • Pregnant women with SCI may have increased difficulty in accomplishing transfers. If they use a manual wheel chair, they may have diminished strength or ability to propel it. Some pregnant women with SCI require the temporary use of a power wheel chair and assistance with transfers. Excessive weight gain and edema may further increase the difficulty of independent movement and transfers. Although it may be difficult to locate accessible scales, accurate weight monitoring is an important clinical measurement for pregnant women with SCI. Passive range of motion exercises and leg elevation may decrease dependent edema and prevent DVT. It is important to frequently ascertain the woman’s need for home and transportation services to assure she is safe and able to accomplish her daily needs.
  • Although ADR usually occurs in patients with spinal cord lesions at or above the T6 level, there is some evidence that it can occur during labor in women with lower injuries. Pregnancy increases the incidence of the occurrence of ADR. Prevention includes avoiding distended bladder, constipation and fecal impaction and asking during intake about past episodes and measures to relieve ADR. Stress and pain are also precipitating stimuli for ADR, and ADR is frequently a sign of the onset of labor. ADR poses a danger to the fetus in that the hypertension associated with ADR may result in uteroplacental vasoconstriction and nonreassuring fetal heart tracings.
  • It is important to distinguish ADR from preeclampsia, as the preferred management for the two conditions differs. The most important difference between ADR and preeclampsia is that in ADR the blood pressure increases during contractions while blood pressure remains consistently elevated in preeclampsia. Also, in ADR proteinuria is absent. Though the incidence of preeclampsia is not increased in SCI, preeclampsia can coexist with ADR, and requires seizure prophylaxis when diagnosed. (Pereira, 2003) Keep this slide for 15 extra seconds
  • Delivery of women with a spinal cord injury should take place in a unit with a multidisciplinary team capable of invasive hemodynamic monitoring. (ACOG Co# 275 – 2002) Patients with a lesion above T10 may have a painless labor. These patients require instruction in uterine palpation for contractions and home uterine monitors may be indicated. Symptoms under the control of the sympathetic nervous system such as abdominal or leg spasms, increased spasticity or shortness of breath may make the patient aware of labor. Autonomic dysreflexia is also a sign of labor. (ACOG CO #275, 2002) Regional anesthesia (spinal, epidural, or combined spinal-epidural) should be administered early in labor to prevent ADR and the patient must be watched closely for ADR symptoms The second phase of labor is often prolonged if the patient is unable to push. There is an increased incidence of operative vaginal delivery. If there is evidence of ADR during the second stage of labor, delivery can be expedited by forceps or vacuum assisted delivery with adequate anesthesia. (ACOG CO#275, 2002) If Autonomic dysreflexia during labor cannot be controlled by any means, cesarean delivery may be necessary. (ACOG CO 275, 2002)
  • Postpartum, the perineum should be inspected daily, to detect early signs of infection that may not be felt by the woman. Women who have no sensation in their perineal area should use ice packs with care to prevent skin injury due to excessive cold exposure. Postural hypotension is more common for women with spinal cord injury. Particularly in the immediate postpartum period when intensive vascular and fluid changes are taking place, women with SCI should be watched closely as they are raised to a sitting position or attempt to transfer. Use of elastic stockings and an abdominal binder may be helpful to ease the problem. Having a spinal cord injury does not necessarily prevent the women from breast feeding. They may need particular assistance in positioning the infant. Because the feeling of suckling increases let down and milk production, women with decreased nipple sensation and lesions above T6 may require additional stimulation. In one small study, tetraplegic women were able to sustain breastfeeding despite absent suckling-induced afferent stimuli with the use of active mental imaging or oxytocin nasal spray to facilitate the let-down reflex. (Cowley 2005) Breastfeeding women with decreased sensation need to be alert for nipple cracks, plugged milk ducts and breast abscesses. (Link to Part 3, Module 3 breastfeeding)
  • Bone loss following SCI occurs throughout the skeletal system with the exception of the skull. The distal femur and proximal tibia are the bones most affected. (Weiss, 2008) The mechanism is in part due to disuse, however neural lesion and hormonal changes also seem to be involved in this process. (Jiang 2006) Spasticity, common with SCI, leads to additional bone loss. Rapid bone loss occurs immediately post injury, and osteoporosis begins early in those with SCI. In one study of 498 patients with SCI at a median age of 42 years, the fracture rate was 2% per year. (Vestergaard, 1998). In another report, over 90% of postmenopausal women with spinal cord injury studied had osteoporosis on screening examinations. (Smeltzer 2005). Weight-bearing exercises with standing frames and bikes and the use of electrical stimulation have been shown to be effective in osteoporosis prevention if started within 6 weeks of injury, but are not effective in preventing or reversing bone loss in chronic SCI (Weiss 2008). Use of bisphosphonate derivatives and parathyroid hormone as a preventive measure is currently being studied for persons with SCI. Bisphosphonate derivatives should not be used in women with reproductive desires. (link to Part III Osteoporosis – Bisphosphonates )
  • Women with spinal cord injury, particularly in those with tetraplegia, have an increased risk for other medical conditions which may present during a gynecology office visit. Skin breakdown and ducubitus ulcer formation are frequent and potentially lethal complications of SCI. Two thirds of decubitus ulcers are found in the pelvic region. Positioning for pelvic examination is a critical opportunity to assess vulnerable skin overlying pelvic bones, particularly the ischial tuberosities and greater trochanters. The section on skin examination –Part 2, module 2 of this program includes more information on the prevention and detection of decubitus ulcers. Other risks include, extreme weight loss and muscular atrophy which occur rapidly post injury. Although the exact incidence of superior mesenteric artery (SMA) syndrome in individuals with SCI has not been reported, SMA syndrome is a well recognized clinical entity characterized by compression of the transverse portion of the duodenum against the aorta by the SMA, resulting in some degree of duodenal obstruction. In those with SCI, the wasting of the trunk muscles, the rapid decrease in mesenteric fat and the change in posture are thought to be the cause of SMA syndrome. Symptoms of SMA syndrome include: epigastric pain, eructation, voluminous vomiting, postprandial discomfort, and sometimes, subacute small-bowel obstruction. Temporary relief is possible by assuming a knee-chest position to relieve the duodenal compression. Individuals with SCI are at increased risk for the development of gallstones and, because they often do not manifest classic symptoms of biliary colic, they are more likely to present with advanced biliary complications. The causation of increased gallstone formation in this population is unknown. In one study, the incidence of gallstones in a subset of spinal cord injured patients was 31% with risk factors being increasing age, female gender and greater severity of injury. (Moonka, 1999)
  • Back to our case study. Remembering that Suzanne has a T4 Spinal Cord injury and is 25 years old. She is seeking contraception and menstrual care is difficult for Suzanne to manage. Her injury puts her at greater risk for deep vein thrombosis and low bone density. What contraception methods would you recommend for Suzanne? (Levo IUD, Progestin only OCP, Short term DMPA) What precautions are essential during the pelvic examination? (Empty bladder, decrease stimuli to prevent ADR, be alert for ADR) What other components of well woman care are important ? (Menstrual history, osteoporosis prevention, skin examination)
  • The most important OB/GYN concern of SCI is ADR which is often precipitated by reproductive events. The menstrual cycle is often temporarily affected following SCI. Normal menses management may present problems for some women due to hygiene concerns. SCI does not interfere with fertility and women with SCI can have healthy and successful pregnancies. Preconception counseling and preparation for pregnancy is ideal. Women with SCI should be delivered by a multidisciplinary team in a unit equipped for hemodynamic monitoring. Regional anesthesia administered early in labor is advised to prevent ADR. Osteoporosis at an early age is common for individuals with SCI and is associated with an increased rate of fractures.
  • Spina bifida is a neural tube defect arising from the failure of fusion of the caudal portion of the neural tube, usually of 3–5 contiguous vertebrae. The spinal cord or meninges or both are exposed to amniotic fluid   It is the most frequently occurring birth defect that results in permanent disability and is more common in girls than boys. (Suzawa,2006) The higher the spinal lesion, the more severe the neurologic dysfunction. Varying degrees of paralysis of the lower limbs as well as bowel and bladder dysfunction are common. Many individuals born with Spina Bifida also have hydrocephalus and or learning disabilities as well as lower limb deformities. (ACOG Practice Bulletin. #44, 2003) It is important to remember that, with treatment, 90% of individuals with spina bifida survive to adulthood with varying degrees of disability.
  • The objectives for this module includes: Recognize the specific interaction of menses, sexuality and menopause for women with spina bifida Describe considerations involving pregnancy, labor, delivery and postpartum. Identify specific resources available for the woman with spina bifida and her provider.
  • Jennifer was born with spina bifida at the level of T-8. She had a VP shunt shortly after birth and underwent an illeal conduit at age 12 to achieve urinary continence. Jennifer works as a clerk for the county courts system. She is now 19 and just married. She and her husband come to you to discuss planning a pregnancy. What more information do you need from Jennifer regarding her history? What considerations are important for Jennifer prior to her pregnancy? What are 3 issues related to her spina bifida that may be of concern during Jennifer’s pregnancy? What considerations are necessary to prepare for Jennifer’s delivery?
  • Women with spina bifida may have a number of medical conditions which require understanding and attention within reproductive health care. The primary condition directly related to spina bifida is neurologic impairment. Secondary conditions result from living with spina bifida and include: GI, orthopedic and dermatologic conditions.
  • Hydrocephalus is a common secondary condition with spina bifida, occurring in 70 to 90% of those with myelomeningocele. About 90% of adults with spina bifida have had a ventriculo-peritoneal or VP shunt as an alternative outlet for cerebral spinal fluid. (Klingbell 2004) Caution is required during abdominal and pelvic surgical procedures and during pregnancy and delivery to not occlude or sever this shunt. About 80% of individuals with spina bifida have normal intelligence (Hochber 2006) however most adults with spina bifida will exhibit some attention and executive function disorders. (Rose 2007) Though they will likely have strong verbal skills they may have difficulties with memory, organization, understanding and attention. (Liptak GS 2003) It is recommended that advice directed toward these women be written in a step- by- step manner and that reminder systems be in place. Women with poor executive functioning are not good candidates for the exclusive use of barrier or oral contraception methods.
  • Most individuals with spina bifida, even those with low lesions, have some degree of impairment of bladder and bowel function. Despite impairment, more than 80% of adults with spina bifida are able to develop social bladder and bowel continence. More information on this issue can be found in Part III- Module 4 – Urinary and bowel Considerations. Individuals with spina bifida are at high risk of overweight and obesity and should be encouraged to consume a healthy diet and participate in regular exercise.
  • The amount of paralysis associated with spina bifida is dependent on the level of the cord lesion. Strength tends to decrease with age so that the ability to ambulate with braces and crutches may be diminished by early adulthood. In one study, children who walked with braces at age 12 stopped walking altogether by age 20. (Suzawa 2006). Mobility issues are life long for individuals with spina bifida. Contractures may develop from joint underuse and osteoarthritis as well as ligament and tendon strain from joint overuse and spasticity. Kyphosis and scoliosis as well as spasticity contribute to problems with positioning, including positioning for examinations. Orthopedic complications increase with aging. A robust and regular passive and active exercise regime overseen by professionals and which is begun as early as possible is critical to diminishing these complications.
  • About three quarters of adults with spina bifida have a severe allergic reaction to latex. The etiology is unknown but it may be due to frequent and early exposure to the latex in medical equipment such as gloves, catheters and adhesive tape. Other medical sources of latex where use should be avoided are tourniquets, ace wraps and some IV tubing. Even the stopper on medication vials are a latex hazard for those with severe allergies. Barrier methods of contraception – condoms and diaphragms are traditionally made with latex. Synthetic condoms like polyethylene are a good alternative. The frequency of the development of pressure ulcers increase as the individual with spina bifida ages. Frequent skin examination and position changes, regular examination of equipment to assure proper fit, and keeping skin dry and protected from sheering are steps in reducing pressure ulcer formation. (Link to Skin examination – Part 2, Module 2)
  • Neural tube defects, such as Spina bifida, occur in about 1 per 1000 pregnancies and are the second most common major congenital anomaly world wide. Fertility is usually not impaired. It is believed that nonsyndromic spina bifida is a result of genetic predisposition and environmental influence. Parents who have spina bifida or those who have had a child with spina bifida are at significantly increased risk to have another child with spina bifida. However, about 90% of neural tube defects occur in families with no prior history. Impaired folate metabolism is believed to be an important mechanism underlying the development of neural tube defects. (ACOG, Neural Tube Defects 2003) It is recommended that women with spina bifida and those identified at increased risk for an infant with spina bifida, including those having a previous child with spina bifida or having a history of spina bifida in a first or second degree relative, take 4 mg of folic acid daily for at least 1-3 months prior to pregnancy and through the first trimester. This is ten times the dose of preconceptional and first trimester folic acid recommended for other women.
  • When pregnant, women with spina bifida who have undergone complex urological reconstruction such as illeal conduit or bladder reconstruction may require special monitoring, particularly during labor and delivery to avoid injury. As mentioned, urinary tract infections or UTIs are quite common in women with spina bifida. The gravid abdomen often increases stasis of urine and the frequency of UTI’s. Frequent monitoring of temperature and urinalysis for bacteria and white cells is necessary for early UTI diagnosis and treatment. Women with spina bifida who have a ventricular-peritoneal shunt to relieve hydrocephalus may experience shunt failure as the pregnancy progresses and it may be necessary for a referral to check shunt patency. Spinal abnormalities may make women with spina bifida more susceptible to disk problems and back pain. Pressure on nerve roots may cause intractable pain in some women. For more pregnancy and delivery considerations, see the previous module on Spinal cord injury.
  • As with women who have a spinal cord injury, the pregnant woman with spina bifida may not be able to feel labor contractions. Other signs of labor include increased spasticity, pain in the back and/or legs, and shortness of breath. A consult with an anesthesiologist is imperative for pregnant women with spina bifida well before her delivery date. Regional anesthesia can be achieved in most patients. When the patient has a VP shunt, A vaginal delivery is preferred and cesarean delivery should only be done for obstetrical indications. Shunt management at the time of delivery requires discussion with a consulting neurosurgeon. Pushing during second stage of labor is not contraindicated as shunt backflow is prevented by one-way valves in the shunt. If a Cesarean delivery is required, the obstetrician needs to consider the following: Providing antibiotic prophylaxis to include coverage for the VP shunt Anticipating adhesions from multiple prior abdominal surgeries and The need for a thorough peritoneal cavity irrigation. For more pregnancy and delivery considerations, see Module 1 Spinal cord injury.
  • Spinal deformities such as kyphosis and scoliosis increase with aging, causing discomfort and compromising respiratory volume. The increasing spinal deformities make positioning for examinations difficult. The kyphosis also complicates the interpretation of bone density scans. (Klingbeil 2004) For those with spina bifida, osteoporosis can occur in childhood and persist through the adult years with an increasing risk of fractures. Treatment with calcium, vitamin D supplements and bisphosphonates may be considered. However, the use of bisphosphates in women still considering childbearing is cautioned. See Aging and Osteoporosis Module in Part III for additional information. ( link )
  • In summary, most individuals with spina bifida have normal intelligence but impaired executive functioning. In order to develop bladder and bowel continence, many children with spina bifida have undergone urinary diversion surgery as well as bowel procedures. The incidence of having a VP shunt is also high. There are many pregnancy and GYN surgery considerations for these women. There is an increased risk for the off-spring of parents with spina bifida to have neural tube defects. Those desiring pregnancy should have genetic counseling and should consume 4 mg of folic acid supplements daily. Osteoporosis at an early age is common for individuals with spina bifida and prevention is an important consideration.
  • Information needed Past issues with VP shunt Support system for pregnancy and parenting Ability to transfer Preconception considerations Genetic counseling Folic acid Pregnancy concerns and considerations VP shunt failure Pressure ulcers Maintaining ileal conduit Labor and delivery considerations Anesthesia consult Teaching labor recognition Urology consult
  • Multiple sclerosis (MS) is an autoimmune disease affecting the central nervous system characterized by multicentric inflammation and destruction of myelin and underlying axons, disseminated in different areas of the brain and spinal cord. It is the leading cause of disability in young adults. (Benedetto-Anzai M. 2006) The majority of persons with MS are white premenopausal women. Individuals with MS have a life expectancy close to normal. Approximately 85% of those with MS have a relapsing-remitting form of the disease, experiencing clearly defined exacerbations of worsening neurologic function followed by partial or complete remissions during which no disease progression occurs. Approximately 15% of individuals with MS experience the progressive form of the disease which may be steadily worsening or may have periods of steady progression and occasional flare-ups, minor remissions or plateaus. (Multiple Sclerosis Society).
  • This module on Multiple Sclerosis has the following objectives: Read objectives
  • Julie is 38. She was diagnosed with the relapsing-remitting form of multiple sclerosis 3 years ago and has had increasing fatigue and leg weakness over the past year. She comes to you for help with decreased libido. She complains of great fatigue and lack of vaginal lubrication. She also has decreased genital sensation. What additional information do you need from Julie? What might you recommend to help her?
  • Because most cases of MS initially present to a primary care physician, it is important to be familiar with presenting signs and symptoms so that appropriate referrals can be made. The onset of the disease is often subtle; patients may present with visual changes, extreme fatigue, paresthesias, spasticity, lower extremity weakness, or loss of coordination, pain, and acute onset of bowel and bladder dysfunction.
  • By self report, many women with MS experience a worsening of neurologic symptoms immediately before the onset of menses which resolve once the period begins (Giesser, 2003). Among other symptoms, patients may note increased spasticity and limb paresis that impairs walking ability. Studies indicate that between 40 and 80 percent of women with MS report a wide variety of sexual concerns. Most common are complaints of decreased genital sensation, fatigue, decreased libido and vaginal lubrication and difficulties with orgasm. The few studies that have been done on the issue generally indicate that the rate of sexual dysfunction correlates with the overall level of disability. (MS Society Clinical Bulletin 7/08) To combat fatigue, sexual encounters can be planned for the times when energy is greatest and choosing positions that conserve energy. MS can cause impairment of attention and concentration that interferes with maintaining sexual desire. To decrease distraction the couple needs to decrease non sexual stimuli and increase sexual stimuli - creating the mood. (Foley 2008) Depression, like sexual dysfunction, may be primary, secondary or tertiary in origin. It may be directly associated with the CNS changes that occur due to damage to central areas of emotional expression and control. It can be a side effect of medications taken to modulate MS or flare-ups, particularly corticosteroid use, and /or the depression can be a response to the many changes and losses in the life of the woman with MS. One population-based study from Sweden, which explored and analyzed the prevalence of depressive symptoms in people with MS found that 19% of those with MS were depressed and that the depression was associated with worse self report of physical functioning, poor memory function and sense of coherence. (Gottberg, 2007). Women with MS should be screened regularly for depression and offered referral for counseling and/or medications to manage it. Studies have shown that smoking may increase the speed of MS disease progression. One recent study suggested that smoking may be a risk factor for transforming a relapsing-remitting clinical course of MS into a progressive course of the disease.(Hernan, 2005)
  • There is no evidence that MS decreases fertility, therefore, it is prudent for women with MS to develop a pregnancy plan. Many women with multiple sclerosis will have a remission or stabilization of symptoms during pregnancy, however relapse rates rise significantly in the first three to six months post partum and then return to baseline. The more active a woman’s disease was during pregnancy and the year prior to pregnancy, the higher her risk of severe post partum relapse. There is, however, no evidence that pregnancy alters the life-course of MS. (Confavreaux 1998) Children born to a parent with MS are at increased risk for developing MS. This risk is of MS in offspring of women with MS is estimated at about 1 in 40 births, and girls are more likely to be affected than boys. (Giesser 2003)
  • Women with MS who are contemplating pregnancy need to discuss all of the medications she is taking with her obstetrician and neurologist. There are limited data to judge the safety of disease-modifying agents (DMAs) during pregnancy. Clinicians must weigh and discuss the benefits of therapy as compared to the risks of fetal exposure.
  • Women with prominent spinal cord involvement or loss of sensation below T 11 may not be aware of the onset of labor. It is important to teach other labor symptoms including increase in spasticity, GI upset, flushing and back pain. Increased spasticity during labor may be controlled using diazepam or an epidural. There has been some controversy over the use of spinal anesthesia for women with MS. Current recommendations do not limit the use of spinal or epidural anesthesia for labor and delivery . Consultation with an anesthesiologist is prudent. (Freedman 2002) During labor, women with MS must be watched closely for early fatigue. Operative vaginal delivery or cesarean section may be indicated for obstetrical reasons or maternal exhaustion.
  • With the exception of a critical need to resume disease modifying agents (DMAs) immediately after delivery, there is no contraindication for breastfeeding for women with MS unless fatigue or disability level make breastfeeding impractical. Due to the high rate of depression in women with MS, it is essential to watch closely for the presence of perinatal and postpartum depression. Treatment with medications and/or counseling are indicated. As mentioned, the rate of relapse of symptoms for MS increases during the first three months postpartum-(Confavreux 1998)
  • The frequent use of steroids and immunosuppressants as well as spasticity and decreased ambulation increases the risk of osteopenia for women with MS. In one study, the low trauma fracture rate was reported as high as 22%. Over half of postmenopausal women with MS are found to have low bone mass density on dexascan.
  • Although most individuals are diagnosed with MS between ages 20 and 50, some are diagnosed at older age. Those diagnosed later in life tend to have the progressive form of MS. (DiLorenzo 2004). The physical effects of aging may mask the symptoms of MS such as decreased muscle strength, fatigue, weakness, poor balance, visual changes and alterations in bowel and bladder function. (Stern 2005) Despite these overlapping symptoms, aging individuals with MS have a greater risk of urinary tract infections, pneumonia, septicemia and cellulitis. (Fleming 1994). The incidence of depression decreases with age in MS. (Kneebone, 2003) About 65% of individuals with MS experience disease-based cognitive loss. This along with natural aging processes put older persons with MS at greater risk for cognitive decline. (DiLorenzo 2006)
  • In summary, the presenting symptoms of multiple sclerosis are often subtle. Neurological symptoms associated with MS may increase in the premenstrual period. Medications used to treat MS are not recommended during pregnancy. These medications are usually stopped or altered when a pregnancy is planned. During pregnancy the disease may stabilize or remit however relapse generally occurs within 3 to 6 months postpartum. Fatigue during labor may necessitate an operative vaginal delivery. Osteoporosis is a common condition in premenopausal women with MS.
  • Back to Julie. What additional information would be helpful for you to help with a management plan for her decreased libido? Presence of pain Current medications which might decrease libido What she and her partner have tried What recommendations can you give? Try sexual encounters after a nap or when energy greatest Use stimulating lubricants Try sexually arousing images/movies ?
  • Next, we’ll discuss reproductive health issues as they relate to women with cerebral palsy. .
  • This module has the following objectives: Read the objectives
  • Heather is a 40 year old with cerebral palsy She lives in community housing for the disabled and has a personal assistant every morning to assist her with bathing, dressing and meal preparation. Heather uses an power wheelchair. She works in a building adjacent to her apartment in the mail room. She has a mild cognitive impairment and her speech is difficult to understand. She comes to you for her annual physical and Pap smear. Heather has spasticity of her legs and arms and her legs are contracted so that it is impossible to position her in a classic lithotomy position. What particular preventive health measures are important to note for Heather?
  • There are three main types of cerebral palsy or CP, each caused by damage to a different area of the brain. Damage to the motor regions of the cerebral cortex results in spastic CP, in which muscles are under a continuous state of tension with increased reflex activity. This is the most common type of CP, with over half of all patients affected exhibiting at least a partial spastic CP. Damage to the basal ganglia produces athetoid type of CP characterized by disorganized spontaneous muscular movements. About 40% of CP patients have the athetoid type. Ataxic CP is caused by damage to the cerebellum. In this form the patient experiences trembling of one or more of the extremities. In this rare form of CP, patients fall frequently and are unable to move without assistance. When several areas of the brain are affected the patient may exhibit multiple forms of CP. All forms of CP carry the symptoms of irregular muscle tone, reflexes, posture, coordination and motor development. People with cerebral palsy may or may not have associated developmental disability and communication impairment. Seizure disorders occur in approximately 30% of individuals with CP. Spasticity and associated contractures are common with CP.. Vision, hearing and speech centers may be involved. There is a growing number of adults with CP, due to excellent health care and because CP is not a progressive disease
  • A cross sectional telephone survey of 63 community residing women receiving CP specific services from a local medical clinic was conducted. This group of women ranging in age from 20 to 74 perceived themselves as healthy, the majority did not smoke and participated in some form of regular physical exercise particularly if they were able to walk or independently use a wheel chair. Half of the women did not require assistance with daily living activities. Of these women in the study, about 1/3 reported cognitive impairment, ¼ had learning disabilities, and 40% had a history of seizures. Most of the women reported having pain. Other common secondary conditions included back and hip deformities, bowel and bladder problems, poor dental health and gastro-esophageal reflux.
  • Women with CP have some specific reproductive health considerations. Many report an increase in spasticity and incontinence during menstruation. For these women, it may be reasonable to weigh the benefits and risks of medications to reduce or eliminate menses. Some anti-seizure medications often used by these women interfere with the effectiveness of combined oral contraceptives. (Please see the section on contraception in Part 3 for more information on this). Many women with CP report having chronic pain, this pain along with frequent hip and spine deformities may lead to concerns with sexuality. It is important for the clinician to help the woman with CP and her partner explore timing and positions that facilitate positive sexual experiences. Women with CP under-use mammography screening. Barriers to mammography include: lack of access to the mammography facility, communication difficulties, and physical, attitudinal and psychological barriers within the mammography facility. (Poulos 2006) Most women can be positioned successfully for mammograms with the use of wedges and pillows. If spasticity is an issue, the use of baclofen or another antispasmodic may be considered. The use of ultrasound along with frequent clinical breast examinations has been reported as an alternative for women who are unable to be positioned or to remain still for mammograms, however this is not as good as mammography in detecting breast cancer. (Smith 2003). Alternative positioning methods, pillows and wedges and good patient physician communication can facilitate the GYN examination. Guidance to positioning can be found in Part 2 of this program. As with all women, those with CP require confidential and developmentally appropriate sex and contraception education.
  • The management of pregnancy in CP is not unlike other physical and cognitive disabilities, depending on the area affected. This would include a discussion of current medications and their effect on the pregnancy. The influence of spasticity and contractures on positioning , labor and delivery needs to be addressed. Social supports need to be identified to provide for anticipated needs during and after pregnancy. Pregnancy outcomes in the few women with CP who have been studied have generally been normal (Winch 1993)
  • Osteoporosis may present an early age due to lifelong diminished weight bearing. Patients may also demonstrate an increase in non traumatic fractures (Klingbiel 2004). Individuals with CP may become non-ambulatory due to fatigue or gait inefficiency, thus increasing bone loss. The use of upright or semi-prone standing periods for non-ambulatory children and young adults has been shown to lead to improved posture and increased vertebral bone mineral density (Caulton, 2004) The prevention of osteoporosis in women with CP begins in adolescence with Calcium and Vitamin D supplementation and early BMD screening. As mentioned in the module on Osteoporosis (LINK) the use of bisphosphonates in premenopausal women who are at risk for pregnancy has not been studied.
  • Although cerebral palsy is a non-progressive disease, life-long abnormal movements, altered postures, immobility, chronic medication consumption and poor nutrition lead to the emergence of a number of secondary conditions. These conditions then lead to diminished functional independence and quality of life . (Klingbiel 2004) The secondary conditions include bladder and bowel dysfunction, fatigue, gastroesophageal reflux, increased spasticity and progressive musculoskeletal deformity and dysfunction. Oral motor and dental dysfunction may require dietary adjustments. As women with CP age, they have a greater risk for developing additional bone, muscle, and joint-related diseases, such as scoliosis, spinal stenosis, radiculopathy, myelopathy, subluxation or dislocation of the hip. These musculo-skeletal conditions can lead to chronic pain as well as a decreased ability to ambulate due to fatigue and gait inefficiency.
  • Back to Heather our 40 year old with CP. What more information do you need? She is difficult to understand so the history may take more time and a nurse may be able to assist with the history taking Current medications – particularly meds for seizures and spasticity If she has had a mammogram Is she sexually active? Need for birth control Factors that facilitated previous examinations Examination See if OB boots are helpful . If painful for her try a diamond or side lying position. If she uses baclofen, make sure she has taken it to reduce spasticity during the exam Proceed slowly and speak softly telling her every touch she will experience. Preventive health Even though she is premenopausal a BMD screening will be helpful . Please note, a Dexascan may require prior approval from her health insurance provider. Make sure she is taking Vit. D and Calcium Consider Mammogram
  • In summary Cerebral palsy is a non-progressive condition present at birth characterized by irregular muscle tone and reflexes. Many individuals with CP have spasticity of the extremities which may lead to contractures and early osteoporosis. Cognitive impairment is found in about 1/3 of those with CP and about 40% have seizure disorders. Most individuals with CP have chronic joint pain which increases with age. Examination of women with CP can best be facilitated by trying alternative positioning and supporting the extremities. The use of antispasmodics has also proven to be helpful in positioning. Women with CP who are planning a pregnancy need to have their medications reviewed to reduce fetal exposure to the teratogenic properties associated with many antispasmodics and antiepieptics. Osteoporosis is a real concern at an early age .and prevention activity should be explored
  • This module discusses the obstetric and gynecological implications of other physical disabilities which may be rare but which have significant implications for the obstetrician/gynecologist. Included in this module are Osteogenesis Imperfecta and Post-polio Syndrome. Over time, other physical disability types may be added to this sub-module.
  • This sub module will guide the women’s health care provider in specific implications and reproductive care management strategies for their patients with osteogenesis imperfecta and post-polio syndrome.
  • Women with OI may exhibit bleeding tendencies due to platelet abnormalities and thus may have heavy menstrual periods. Although reproduction may be hampered due to increased susceptibility to fractures or limited hip movement, OI does not influence fertility or miscarriage rates. Most types of OI have a 50% transmission rate if one parent is affected with OI. Genetic counseling therefore is highly encouraged. (OI Foundation, 2007) During pregnancy, women with mild forms of OI may experience loose joints, reduced mobility, increased bone pain and dental problems. Those with more severe forms of OI who have short stature and spinal curvature may experience respiratory and cardiac insufficiency. As the uterus grows, the shortened distance between the thoracic cage and pubic bone can cause great discomfort and require extended bedrest. At the time of delivery there are several concerns for women with OI. Those who have a contracted pelvis, previous pelvic fractures or have a severe form of OI will likely require a cesarean delivery. (OI Foundation, 2007) There is also a risk of fractures resulting from obstetrical manipulation during vaginal delivery. Excessive bleeding postpartum may occur for women who have a history of recurrent nosebleeds, easy bruising, or excessive bleeding following other procedures. Individuals with OI are at increased risk of hyperpyrexia, particularly with general anesthesia. Although epidural anesthesia is a preferred approach for delivery or pelvic surgery, spinal deformity and previous spinal fractures may complicate epidural and spinal anesthesia. Hernias, likely secondary to immature collagen, are common. It is therefore important to use permanent suture for abdominal aponeurosis closure. (Roberts 1975)
  • Although acute poliomyletis was essentially eradicated from the United States in the 1950’s following the discovery of the Salk and Sabin vaccines, it remains prevalent in pockets of Africa and Asia where the immunization cold-chain has failed. There are an estimated 440,000 polio survivors in the U.S. and post-polio syndrome (PPS) affects about 50% of those individuals about 15 years or more after the original illness. It is a slowly progressive syndrome characterized by increasing muscle weakness, decreased endurance, muscle and joint pain, muscle atrophy, severe fatigue and occasionally respiratory difficulty and dysphagia.
  • The ob-gyn is likely to encounter post-menopausal US born women with post-polio syndrome (PPS) but may also see the syndrome in younger foreign born women. The management of PPS is symptomatic . Most will benefit from energy conservation, a nonfatiguing exercise program and weight loss. (Jubelt, 2000) Decreased mobility due to muscle weakness and joint pain put women with PPS at great risk for osteoporosis. The muscle and joint weakness with resulting poor balance accelerates their risk for fractures due to falls. Aggressive prevention of low bone mineral density and home fall prevention management is critical for women with PPS (link with osteoporosis – Part 3, Module 7). Potential chest muscle weakness and decreased pulmonary function occurring with age may greatly compromise the individual with PPS. It is important to prevent respiratory illness through influenza and pneumonia vaccination. Some individuals will require breathing assistance at night to improve sleep and energy. (Jubelt, 2000, Welner 2002) Those who experience dysphagia may benefit from speech therapy. Atrophic changes in urinary tract leading to incontinence (link Part 3, Mod 4) Joint tenderness and pain as well as fatigue is a cause for sexual dysfunction for those with PPS. Timing sexual encounters following rest periods, taking pain medication prior to sex and experimenting with alternate positions may be beneficial. (See Sexuality Part 1, Module 2)
  • This module will suggest approaches to reproductive health care for women with intellectual and developmental disabilities or IDD. People with IDD exhibit great diversity in their capacity to function independently, therefore it is impossible to generalize statements concerning this population. Most women with IDD, particularly those with mild to moderate disabilities, live in the community. Access to special education programs has resulted in employment and a greater potential for productive lives for those with IDD. (Muram 1994) Individuals with IDD have the same range of medical conditions as the general population, however, they may not be able to communicate prodromal or initial symptoms, and they may respond in unexpected ways to both medical conditions and healthcare encounters. Please see the resources section of this program for some excellent materials for patients, their families and providers of social services and health care.
  • Gwen is a 16 year old with mild Down Syndrome who attends special classes within the city’s public high school. Gwen has seizures well controlled by carbamazepine (Tegretol). Her mother notices Gwen has not had her period for several months. Her periods had not previously been regular. When a 4 month pregnancy was confirmed in her OB/GYN’s office, Gwen confided that her school bus driver had a secret with her. The bus driver would drop her off last and usually stop along the way home to give her some “special attention” for which Gwen was given candy. After recovering from the shock, Gwen and her mother decide to continue this pregnancy. What is your role and response as her obstetrician/gynecologist?
  • Read slide. After completing this module, the participant will be able to: Identify special considerations in taking a history when working with women with IDD Describe appropriate methods of providing sex education Discuss how to gain cooperation of the woman with IDD during a GYN examination Describe the impact of menstruation and surgical procedures for women with IDD Discuss resolution for issues of informed consent for women with IDD
  • Intellectual and developmental disabilities are chronic mental and/or physical conditions that cause limitations in three or more areas of function. Those areas are defined as self care, language, learning, mobility, self direction, independent living and economic self-sufficiency. (Quint 1996) In many cases, the onset of the disabling condition is in childhood (e.g. cerebral palsy, Down syndrome), although onset may occur in adulthood (e.g. traumatic brain injury). Developmental disabilities are specifically defined as having onset before the age of 22 years IDD is usually classified according to intelligence quotient. An IQ of 52-83 is mild, 36-51, moderate and less than 36 severe cognitive impairment.
  • In this module we will be covering these health care issues frequently addressed within reproductive health care. They include: History taking and education Physical examination Menstruation issues Contraception –(link to Part 3, Module 1) Pregnancy Informed consent and Aging and osteoporosis
  • Women with IDD seek gynecologic health care for the same reasons other women do: the onset of menarche, the development of menstrual-related or other bleeding problems, commencement of sexual activity, pregnancy and menopause. (Bradshaw, 1996) However, they may be highly resistant to assessment, many having previously had examinations forced upon them. Many equate medical examinations with pain, provoking anxiety and hostility (Edwards, 1988). As described in Part 1, Module 4 , a large percentage of women with IDD have experienced sexual abuse and have extreme reaction to intimate touch. Despite the fear associated with GYN health care, it is usually best if the medical personnel who are to participate in the GYN examination wear white coats or uniforms so that the patient understands that an examination of her body should only take place in a medical setting. (Bradshaw)
  • Patients with IDD are often very sensitive to the attitudes of those they feel are in authority and tend to withdraw if interviewed by those who are brusque, hurried or indifferent. (Muram D. 1994). If at all possible communication should begin in a quiet setting and face to face with the patient before she disrobes for the examination. If the patient is nonverbal, it is important to establish how she communicates and how she indicates what she wants – this could be through facial expressions, vocalisations or challenging behavior. There may be an imbalance between receptive and expressive language skills. It may take time to realize that although the patient is giving clear answers, she does not understand the question. Even if the patient has limited or absent verbal skills, establish contact with the patient first, and ask if she will allow the person accompanying her to interpret.
  • Obtain the medical history as much as possible from the patient, other wise a person accompanying the patient may be able to complete the history. (Lennox 2005). For individuals who are not able to supply the information needed. the provider can request that the parent or caretaker who knows the patient write out a simple history and description of any current problems. Ask that they keep a calendar of menses that also notes the occurrence and intensity of behavioral changes and other symptoms of PMS. There are simple social/sexual assessment scales available to assist in the evaluation of her sexuality and socialization skills. Photos or drawings may help the provider to assess her past and current sexual activities. Photos or anatomical models are useful to assess her knowledge of her body and also help to familiarize her with the upcoming GYN examination. The Resources page in this module has more information on these assessment tools.
  • No matter the degree of IDD, all women respond to some level of health education. For women with severe disabilities, this could include giving a simple explanation of what you are touching and how that might feel, in a calm and matter of fact tone. Or it could mean responding to resistance with reassurance and never with force. Health education for teens and women with IDD should consist of body part awareness and care by life stage, disease prevention, sexuality and pregnancy prevention, and physician patient interactions including examinations, There are some excellent resources and curricula for teaching reproductive health education to teens and women with IDD. Please see the Resources Section at the end of this module for details in ordering. Two to be highlighted are Women Be Healthy: A curriculum for Women with Mental Retardation and Other Developmental Disabilities. It was developed by the North Carolina Office on Disability and Health and the University of NC at Chapel Hill. This is free and can be ordered through the web site The other resource is from the ARC of New Jersey entitled “Let’s Talk About Health, What Every Woman Should Know. This resource is available in a spiral bound print version as well as video and audio tapes. These resources utilize both written, audio tape and video format for a variety of settings.
  • Health care providers serving women with IDD are faced with balancing the issues of protecting the patient from sexual abuse while maintaining individual rights and freedom of choice. This issue often evolves around requests by the patient, family or care provider for contraception. There is not a standard screening tool to determine readiness or ability to consent to sexual relations, however there are several general components of informed sexual relations decision making. It includes assessing the understanding of the patient on the following: That sex is an activity that both participants want and engage in voluntarily. That no one can force or threaten you to have sex. That you can refuse to engage in sexual activity with someone even if you have agreed to engage in it before with the same person, and that it is enough to just say "no" without having to provide justification for the refusal. That sex is usually engaged in private That it is not proper to have sex for money or gifts That it is not proper to have sex with children, immediate blood relations or animals. (Griffiths, 2002)
  • Individuals with IDD have the same diversity of sexual needs and desires as the rest of the community. They usually require assistance to understand the complexities of human relationships and the rights and responsibilities of sexuality and how it can be incorporated into their lives. Many parents of individuals with IDD are uncomfortable dealing with issues pertaining to sex or sexuality as their child ages. They often take the stand that if the child is not exposed to information they will be less likely to experiment sexually. Parents need direction and guidance in helping their child express their sexuality and to guide sexual expression into appropriate and acceptable behavior. Without guidance, physically maturing girls with IDD are extremely vulnerable to sexual abuse, pregnancy and may act out sexually in an inappropriate or clandestine manner. Sex education should be kept simple and concrete using terms she can understand. Information should be repeated and she should be able to repeat back what was said to her. Sex education should include strategies to avoid situations when sexual abuse may occur, inappropriate touching, saying NO and getting help if a situation is out of control. Sex is more than penile penetration of the female. When developmentally appropriate, women with IDD need information on variety in sexual expression and that women have orgasms and receive pleasure. Sexual relationships need to be discussed in a matter-of -fact manner so that she is not frightened to report experiences. Those who are at risk for sex, should be counseled, supplied with and taught the use of condoms as well as other contraception. Women with mild to moderate IDD at risk for unsupervised sexual activity outside of a monogamous relationship can usually be taught about condom use and taught to keep a condom supply available when they chose to have sexual encounters. There are some excellent tools for teaching sexuality and social relationships in the resources section of this module.
  • The onset of puberty may be an anxious time for the parents of a girl with IDD. This is because her body changes with breast and pubic hair development coupled with undeveloped social skills and lack of sexual education can make her a target for sexual predators. Approximately 20% of teens with IDD will experience menarche prior to age 12. Parents often fear this event and seek medical counsel for assistance in menses management. Menstrual hygiene training requires a step-by-step process that is repeated frequently. Use medical intervention only after behavioral intervention has been tried and failed Most women who manage their own toileting, can be taught how to care for their menses through patience, positive reinforcement and perseverance. Hormonal intervention may be considered after behavioral intervention has failed and the menses interfere with the patient’s quality of life.
  • In a study reported by Quint, 16% of the clinic population of women with IDD presented with physical or behavioral symptoms the week before or the first week following the onset of menses. The symptoms include clusters of temper tantrums, crying spells, autistic or self-abusive behavior and seizures. Over 1/3 of those with symptoms were reported to have aggressive behavior in the perimenstrual period. The first line of therapy for these women was NSAIDs with a 65% response rate. Oral contraceptive pills and DMPA were also helpful in treating symptoms. (Quint 1999). Selective seritonin reuptake inhibitors may be considered, however the health care provider should be alert to polypharmacy.
  • Part 3 of this program has an entire module on abnormal uterine bleeding. The workup, care and treatment of women with IDD is generally not different than for the general population. However, it is important for the woman or someone taking care of her keep an accurate calendar of bleeding, including a pad /tampon count to determine the frequency and general amount of bleeding experienced to gauge her response to management. Menorrhagia has been associated with hypothyroidism. It is estimated that 15% of adolescents with Down syndrome are hypothyroid, and there is evidence for a steady decline in thyroid function as age increases (Pueschel 1991). Those with Down syndrome should be screened annually for hypothryoidism. (Down’s Syndrome Assoc. 2006) Women with IDD, particularly Down syndrome, are often obese. This over-abundance of fatty tissue, with resulting high levels of circulating estrogens, can lead to anovulation and excessive stimulation of endometrial glandular tissue causing irregular, heavy menses and the early development of endometrial hyperplasia (Elkins 1992) In addition, antipsychotic medications used for behavior control in this population have been linked to hyperprolactinemia, with an increase in the prevalence of abnormal menses such as secondary amenorrhea and oligomenorrhea. Part 3, Module 2 discusses management of AUB for women with disabilities. In most instances, non-surgical management is effective.
  • A large percentage of women with developmental disabilities have physical disabilities that need to be accommodated during a GYN examination. Transfer and positioning information can be found in Part 2, Module 1 of this program. Generally, it is advisable not to use stirrups and to assure that the patient’s legs are well supported. If she can assume a lithotomy position, obstetrical boots may offer that support. It is important to maintain her privacy. She should be given the option of who accompanies her for the examination. If she will tolerate a speculum, use a small bladed Pederson or Huffman that is moistened with warm water. Most patients will tolerate the bimanual examination if modified. Start with some deep breathing or diversion tactics to relax the abdomen. Then insert one finger into the vagina and palpate the abdominal organs with the other hand. If the patient is resistant, this can be attempted with one finger in the rectum instead of the vagina. (Bradshaw 1996) For women over age 50, a stool sample from the gloved finger can then be checked for occult blood.
  • In general, cervical cancer screening should follow the existing recommended guidelines. Screening should begin within 3 years of the onset of vaginal intercourse or no later than age 21. Women up to age 30 should have this done annually. After age 30 with 3 consecutive years of negative testing and without risk of HPV infection the interval for screening with cytology alone or with viral testing and cytology can be extended to 3 years (ACOG PB #46). For women with IDD, there may be inadequate information regarding the risk of HPV infection. This may be due to incomplete past medical history, including Pap history and sexual risk factors, such as abuse and STIs and leads to difficulty assessing the risk for cervical cancer and determining an optimal schedule for Pap and STI testing. If a pap smear is difficult to obtain because the patient cannot tolerate the speculum, an alternative is the blind pap smear. With a moistened finger palpate the cervix and slide a brush or “q-tip” over your finger and insert into the cervix. Keep in mind that the rate of endocervical cells is significantly lower using this method. Blind Pap smears in women with IDD using liquid cytology yield endocervical cells about 44% of the time. The Q-Tip technique yields endocervical cells about 18% of the time. This suggests that the blind technique with liquid cytology is a reasonable alternative for obtaining Pap smears in women with difficult pelvic examinations who would otherwise not receive cervical screening. (Kavoussi, 2009, Quint 1997) Some patients come to their gynecologist with a directive for a Pap test from a social service agency or residential institution. There are no laws mandating Pap tests for any group of women. A comprehensive reproductive evaluation is often needed, but it may not need to include a Pap test if that is impossible to obtain. In such instances, the provider can inform the agency following the recommended ACOG and US Preventive Services Task Force screening guidelines. (Special Issues in Women’s Health, Pg. 49)
  • There are no data on the efficacy of ultrasound as a screening option for women who cannot be examined and therefore ultrasound is usually used for screening on indication only. Ultrasounds do not take the place of an attempted physical examination with inspection of the external genitalia and possibly cervix and vagina.
  • There are several instances when it is appropriate to consider the use of sedation or anesthesia to accomplish a pelvic examination. . General anesthesia is usually NOT considered for a screening exam, but reserved for an indicated exam, for instance postmenopausal bleeding. When a patient is scheduled to undergo another procedure or dental work under anesthesia, it would be very prudent to do a screening pelvic exam at the same time. In a study of women with IDD who were examined with oral sedation, the rate of endocervical cells on their Pap tests was similar to that in women with IDD examined without sedation (Quint 1997 ). An ethical concern of any sedation is that there is no information on the emotional effects of a pelvic examination in a sedated state. Mild sedation with oral agents may be appropriate in women who are able to cooperate and consent to that , but who are anxious.
  • Hysterectomy and endometrial ablation may be indicated for women with menstrual issues that are unresolved with other medical treatments or for those who have uterine pathology. The indications are the same as in the general population. Endometrial ablation should not be used in young women to obtain amenorrhea, as there is no long term outcome data on these procedures and complete amenorrhea is unlikely. Since both hysterectomy and endometrial ablation are irreversible, consent is a very important issue. For these procedures federal, state and local laws and regulations must be satisfied, if the patient cannot give her own consent. Often, review from a hospital or institutional ethics committee is needed. Physicians should be aware of the possibility of undue pressure from family members whose interests, no matter how legitimate, may not be the same as the patient’s.(ACOG CO #371, 2007)
  • When women with IDD have surgical procedures, every effort should be made to arrange for continuous bedside attendance by a family member or familiar caretaker. Normalization of her environment including familiar food and comforting objects are helpful to decrease her anxiety and improve cooperation. It is important that she be provided with adequate pain relief, particularly if she is not verbal. Staples for wound closure should be avoided as women with DD may have a heightened sensory issue with the staples, resulting in their pulling and picking at them. As mentioned later in this module, special attention to infection prevention and treatment should be made for women with Down syndrome who are often immuno-compromised.
  • Ascertaining the capacity of the woman with IDD to provide informed consent can be complex. The capacity to consent is not static, altering with the patient’s language and culture as well as the comfort level of the patient, the quality of the information provided the patient, and fluctuations in the patient’s comprehension based on stressors and medications. Multiple interviews over the course of time and involving a person trained in communication with a person with IDD may be required to ascertain the patient’s comprehension of the nature of the procedure and its impact on or to her. Even when it is determined that the woman does not have the capacity to consent, it is important to gain her assent prior to commencing a procedure. The determination of the capacity to consent should be done based on the level of risk to the patient. Increased scrutiny is necessary with more invasive or risky procedures. It is important to determine the reason for a request by the caregiver, family or patient who requests contraception or sterilization. If the request is from the patient, determine if she was put up to making the request by another and if she is a consenting sexual partner. (Dinerstein RD 1999) Knowledge of state or jurisdictional requirements is key. Part 5 contains more information on informed consent issues and resources.
  • Prompted by fear of unwanted pregnancy, parents and guardians may approach the obstetrician-gynecologist with a request for sterilization for the women with IDD. Since 1979, regulations have prohibited the use of Federal funds for sterilization of women with disabilities. This regulation follows a Supreme Court proclamation that reproduction is a basic right of all humans. Individual states have developed laws determining gaining informed consent of the individual and the process needed to assure that sterilization is the treatment or resolution of last resort. In some states, parents are not allowed to consent to sterilization on behalf of their child, regardless of whether or not she is a minor. Only the woman herself can legally agree and then only if she is capable of meeting the criteria for “informed consent”. In most cases, hormonal preparations and patient education are adequate to prevent unwanted pregnancies. It is important to remember that patient autonomy to determine her procreative right is key. The presence of a mental disability, in itself, does not justify either sterilization or its denial. (ACOG CO#371 2007)
  • Very few intellectual and IDD affect fertility. According to US law, every citizen has “the essential right to conceive and raise one’s children” (in R.E. Montgomery 1984) Individuals with mild to moderate developmental disabilities can be excellent parents with education and support.(Hayman, 1990) Current studies indicate that approximately 90% of the offspring of parents with IDD have normal intelligence. (Elkins TE, 1992) Of those intellectual developmental disabilities with genetic links, when one parent has a chromosomal defect there is a 35 to 50% risk of that condition being passed on. Genetic counseling therefore should be offered according to the patient’s ability to understand the information. Women with IDD may delay in seeking timely prenatal medical care due to ignorance of menstrual cycle changes or pregnancy symptoms. Many women with IDD have seizure disorders, requiring the use of anticonvulsants with known teratogenic properties. In these situations, it is important to consult with the woman’s neurologist to weigh the risk of fetal exposure. Within the perinatal care setting there is a need for intellectually and developmentally appropriate education about pregnancy, labor and delivery, and infant care. The organization, Through the Looking Glass, offers excellent perinatal education resources including parenting assessment tools. In addition, the ARC , a national and state level advocacy and service organization for people with IDD offers guidance and information on local programs. Many women with IDD may be afraid the baby will be taken away from them after delivery. A team approach, including patient and partner, care providers, social work, extended family, if appropriate, is best for the pregnancy and postpartum planning for the baby
  • Women with IDD are at high risk for osteoporosis due greater rates of menstrual irregularities, hypothyroidism , use of anticonvulsant and steroid medications, early menopause (especially those with Down syndrome and Fragile X) and generally lower rate and quality of physical activity. Women requiring supervision often have less than optimal exposure to sunlight with resultant Vitamin D deficiency. The US Preventive Services Task Force recommends yearly bone densitometry beginning at age 40 for institutionalized patients and at age 50 for community dwelling patients.(Wilkinson 2007) For those with risk factors, calcium and vitamin D supplements should begin in adolescence in addition to the encouragement of exercise and muscle resistance programs.
  • Down Syndrome (DS) or Trisomy 21 is the most commonly occurring genetic disorder affecting 1 in 733 live births. All people with Down syndrome experience mild to moderate cognitive delays. Common physical traits of DS are low muscle tone, small stature, an upward slant to the eyes, and a single deep crease across the center of the palm. Individuals with DS have an increased risk for a number of health problems as a result of their condition. Approximately half of children with DS will have a congenital heart defect. As they age, those who had septal defect repairs as children have a greater incidence of heart conduction and heart valve disorders (Meijboon 1994).. DS is associated with an increased frequency of infections, hematologic malignancies, and autoimmune diseases.(deHingh 2005) Immunodeficiency is an integral part of DS that contributes significantly to the observed increased morbidity and mortality. The immunodeficiency is due to abnormalities in t-cell and antibody-mediated immunity functions. (NICHD 08). Therefore antibiotic prophylaxis may be necessary should surgery be required. Approximately 20% of individuals with DS have hypothyroidism, Most guidelines suggest yearly thyroid testing for individuals with DS. (Management Guidelines, 2005) Bone and sinus structure abnormalities may be the underlying cause of hearing problems for as many as 70% of those with DS. Thickening of the tongue and low muscle tone have been identified as a cause of obstructive sleep apnea which occurs in about 50% of those with Down syndrome (Smith 2001). Altered vision is present in many women with DS Lastly, individuals with DS have an increased incidence of depression, obsessive compulsive disorders and early dementia. (Schupf 2003)
  • Menarche occurs at about the same time for women with Down syndrome as the general population, however their cycles may be annovulatory. (Ranganath P 2004) The literature reports impaired fertility for women with Down syndrome. Infants born to women with Down syndrome are at increased risk for prematurity and low birth weight.. Women with Down Syndrome would be expected to produce 23, X and 24, X+21 gametes with equal frequency; theoretically, one may expect 50% of offspring, then, to be affected with Down Syndrome. In a summary of reported pregnancies to women with DS, among 27 live births, 10 infants had DS. Of note, 6 of 17 chromosomally normal offspring had congenital malformations and/or mental retardation (Bovicelli 1992). The presence of maternal health conditions frequent for women with Down syndrome also contribute to pregnancy risk. (Van Dyke 1995)
  • Women with Down syndrome experience age related secondary conditions at an earlier age than the general population. Approximately 40% of those 50-59 years old with Down syndrome develop dementia. (NCS 2008) This dementia is often accompanied by depression and the use of SSRI medications has been found to be helpful to assist in patient management. (Smith 2001) Early menopause, before age 42, is common for women with Down syndrome And, Down syndrome is an independent risk factor for osteoporosis and fracture due to lower muscle tone contributing to lower peak bone mineral density. (Schrager 2005)
  • Women with IDD should be afforded autonomy in their health care management as is appropriate for their developmental level. During health care interactions with providers, they should be directly addressed by providers. All women benefit from intellectually and developmentally appropriate health and sex education. There are excellent resources to assist parents and providers in delivering this education. Menstruation often has a great impact on the woman with IDD and their care givers. Most women who are able to use the toilet can be taught menstrual management. For women who are non-verbal or unable to express their feelings, acting out behavior is often a symptom of physical or psychological conditions and the source of the distress needs to be determined. Women with IDD often have early menopause and other signs of early aging. Women with Down Syndrome may have premenopausal osteoporosis.
  • Back to Gwen. Remember that she is 16 years old, and 4 months pregnant due to possible sexual abuse. After recovering from the shock, the mother and Gwen decide to continue the pregnancy. What is your first response? Child protective services needs to be notified about the sexual abuse of Gwen by her school bus driver. Due to her age and disability, it is likely that the bus driver will face criminal charges. Testing for STI’s is also essential. Gwen is taking carbamazepine (Tegretol – Pregnancy category D) for a seizure disorder. Consult with Gwen’s neurologist to discuss the risks of further fetal exposure vs. the risks of Gwen’s having seizures. A consult with her pediatrician to identify other issues that may impact the pregnancy is also very helpful. What are other pregnancy concerns for Gwen? Genetic counseling and testing due to the possibility the infant will have Down syndrome. Additionally she is immunocompromised- with both pregnancy and Down syndrome She also needs clear and developmentally appropriate prenatal health education The local high-risk pregnancy home visiting program sent nurse educators to her home to teach Gwen about her pregnancy and to prepare her for labor and delivery. This same nurse will be available to help the family adjust to the infant. Gwen delivered a healthy baby girl. What are her postpartum needs? Appropriate contraception, sex education, readjustment of medications. The outcome of this case: Child protective services will monitor the situation to determine parental rights.
  • Communication is fundamental to good health care. Medical history taking, instructions for discharge care, proper medication use, counseling regarding test results, and so many other elements of clinical care and continuing self-care are based on the ability to successfully exchange health information and mutually confirm understanding. Health care providers must take steps to appropriately serve the needs of patients with sensory disabilities to ensure adequate communication.
  • Kate is in your office, referred by her primary care clinician for evaluation of pelvic discomfort and vaginal discharge. She has been deaf since birth and mainly uses sign language to communicate, although she is able to read lips when she is directly addressed. Now 29, Kate had her first pelvic examination at age 19. It was a terrible experience for her. Her clinician did not explain what would happen. She was put up in stirrups with a sheet draped over her legs, unable to see what was going on. The bright light in her face, the sudden touching followed by the uncomfortable insertion of the cold speculum without warning was just too much, and she vowed not to repeat the experience. She is now obviously frightened that the poor experience will be repeated. When her appointment was made, you were not aware that Kate is deaf and have no office accommodation prepared for deaf patients and no sign language interpreters. What are some key components of your communication with Kate? How will you work with Kate on this appointment? How will you accommodate future appointments with Kate?
  • The objectives for this module on sensory disabilities are as follows: At the completion of this module the participant will be able to: Describe unique strategies in health care delivery for women who are hard of hearing Describe unique strategies in health care delivery for women who have visual impairment
  • People with hearing loss constitute approximately 9% of the US population and research suggests that this population is increasing in number (Reis, 1994). Accurate communication is the disabilities-specific goal of health care providers with their patients who are deaf or hard of hearing. The timing and amount of hearing loss and the preferred communication modality of the patient generally determines the communication strategies that should be employed by the provider. Those who are of reproductive age, have had a major hearing impairment since early childhood and who were born in the US have likely become facile in the use of American Sign Language. Foreign born patients and those whose first language is not English may have learned to communicate using other sign languages or other modalities. Women who are hard of hearing may use hearing aids or microphone devices to augment sound. Some have had cochlear implants or other surgical procedures to assist hearing. Some women who are deaf rely entirely on oral and written communication. A growing majority of deaf individuals communicate primarily by sign language. The key to successful communication with people with hearing loss is the ability to adapt to the needs of the situation. Those with hearing loss need to be asked how to best communicate with them.
  • Many patients who are deaf are able to gather some essence of a conversation through reading lips, however only 30 to 40% of English sounds are unambiguously visible on lips, (Barnett 2002). Deaf and hard-of-hearing persons report misunderstanding words when lip reading, especially when the health care provider wears a beard, speaks rapidly, does not look directly at the patient or speaks with a foreign accent or an impediment. During procedures, providers and staff wearing masks make lip reading impossible. Both deaf and hard of hearing individuals describe fear when they can not see clinicians or technicians during physical examinations or procedures.(Iezzoni 2004). Note writing as a chief means of patient /provider communication is often inadequate. It is significant to note that the average reading level for deaf persons aged 18 is estimated at the fourth grade.(Kelly 1998) Most clinical situations do not allow sufficient time for the patient to adequately express in writing symptoms, feelings or comprehension of the encounter and management plan. This is especially true for the elderly, who may also have arthritic hands. Using family members as interpreters is inappropriate in most clinical situations in women’s health care. Private and confidential discussion between the provider and patient is an essential component of patient care. Additionally, family members are likely not familiar with the signs for medical terms resulting in miscommunication. Telephone communication is often problematic for the deaf and hard of hearing. Making an appointment or getting results from tests can be frustrating for the provider and patient. Automated telephone systems pose special difficulties for those who are hard of hearing as they often have to listen to the instructions several times to understand what is said. Lisa Iezonni in her book, More than Ramps writes: “ Although note writing and lip reading sometimes provide effective communication with deaf and hard-of-hearing individuals, often they do not . Miscommunication can ensue, with mismatches between clinician’ perceptions and patients’ realities: clinicians erroneously assume that patients accurately “hear” or know what they are saying. (Iezzoni, 2006)
  • A little known and under-utilized service available across the country 24/7 is the free telephone relay service. Telecommunications Relay Service, also known as TRS, Relay Service, or IP-Relay, is an operator service that allows people who are Deaf, Hard–of–Hearing, Speech–Disabled, or Deaf Blind to place calls to standard telephone users via a keyboard or assistive device. Originally, relay services were designed to be connected through a TDD (TTY) or other assistive telephone device. Services have gradually expanded to include almost any generic connected device such as a personal computer, laptop, mobile phone, PDA, and many other devices. (See Part 6 -resources section for more information) Text messaging via cell phone has become an integral form of communication in the Deaf community and should be added to patient /provider communication modalities. Some patients who are deaf use a hand held communication device similar to a PDA which has a key board as well as frequently used phrases which greatly speeds note writing. Some providers may decide to purchase a TTY machine or communication devices for augmented voice. These are often available free or at greatly discounted cost through the state services for the deaf. (See resources, Part 4, for details) All accommodation services for deaf and hard of hearing patients that have a cost are subject to tax deductions for the provider.
  • Suggestions that have been made by patients who are deaf or hard of hearing to promote communication with health care providers include Of greatest importance, ASK the patient how she prefers to communicate. If that method is not possible in this situation, work with her on a temporary accommodation. Prepare low literacy instructions for basic office procedures that can be handed to the patient. For example, this might include: the clothing that needs to be removed for an examination how to tie the examination gown (front or back) how to prepare and be positioned for a pelvic examination how to give a urine specimen and where it should be placed what to do after the examination Background noise is especially distracting and uncomfortable for the hard of hearing and those using sound augmentation devices. The interview and examination area should be kept as quiet as possible, so the patient can focus on the provider. Good lighting facilitates the patient’s use of visual signals and interpretation of lip movements. Individuals who are deaf and hard-of-hearing require the same privacy as every patient. In the open office setting do not use raised voices to call the patient or gather information. Also, it is important to communicate with the patient before touching her or using any equipment.
  • The Americans with Disabilities Act (ADA) requires physicians to provide effective communication to patients who are deaf or hard of hearing. When necessary the provision of a qualified medical interpreter is required. A “qualified interpreter” is defined as “ a n interpreter who can interpret effectively, accurately and impartially both receptively and expressively, using any necessary specialized vocabulary”. Qualified and registered interpreters for the deaf are bound by a code of ethics. Reasonable modifications to office and hospital policies, practice and procedures will assure compliance with ADA regulations as well as a more mutually satisfactory clinical relationship.
  • Women who have been deaf or hard of hearing from birth, may not have been exposed to the family stories a person uses to develop a family medical history. This is especially important to discern the past occurrence of genetically linked syndromes. Although family centered childbirth has made the use of face masks less common during routine deliveries, it is necessary to remember that this component of the typical surgical garb will eliminate communication with a person who uses lip reading. Attention needs to be taken of the desired communication means with deaf and hard of hearing persons for routine and emergency follow-up communications.
  • Low vision and blindness are catch words for a spectrum of visual impairment and response to conditions of the eyes, optic nerve or brain. The person’s accommodation to low vision is dependent on a wide variety of factors and particularly the timing of the impairment – whether from childhood or more recently acquired. Asking the woman with a visual impairment how she can be helped is the first step in providing excellent care. It is important to record information about the specific accommodation needed for the patient rather than to make broad statements. For instance, instead of making a chart note “patient is blind”, note what the patient can and can not see and the assistance needed for routine care. Mobility aids should never be moved unless requested by the patient. If it seems to be in the way of your work with her, let her place it in another location. It is essential to take time prior to touching the patient to explain exactly what you are going to do and what she will need to do. Voice directions need to be precise. For instance, “turn to your left to 3 o’clock” or “move your buttocks to the foot of the table until you feel my hand”. The ADA requires that the contents of written material must be effectively communicated. This includes consent forms, financial responsibility forms and bills. This should happen in a location that assures privacy. Patient education and information often requires reinforcement. In addition to reading this information for patients, another method of reinforcement is advisable. The method depends on the visual acuity of the patient, the type and amount of information she needs to have, and the support system and equipment available. Follow up telephone calls to reinforce instructions are often very helpful. Office staff who take payments from patients, including co-payments, should become familiar with how to handle currency for those who are blind or have low vision. Bills should be identified and counted. Credit cards and insurance cards should be directly handed to the patient, not laid on a table or counter top.
  • Individuals with low vision or blindness have the right to access all of the necessary information related to their treatment or condition even though most of the information is likely to be in print form. Appropriate time must be taken and privacy assured for the completion of registration forms, informed consent documents and other materials necessary to an effective clinical encounter. Pregnancy is one of the circumstances in which patients are most interested to obtain educational material. There is limited availability of large type, Braille or recorded information on pregnancy. A list of resources and adaptive tools is included with this presentation so that clinicians can become familiar with ways to insure information access for their patients.
  • Means of effective communication are key for pregnant women who have low vision or are hard of hearing. The pregnant woman and the provider’s office need to set up plan for communication in emergency situations. Pregnant women who are blind or have low vision will need to be able to determine the presence and nature of vaginal bleeding or discharge. This will require an individual plan depending on risk factors, visual acuity and home support. Some women with low vision may consider wearing white or light colored panties to be able to distinguish darker blood stains. In the second trimester, a plan should be developed to assure good communication through labor and delivery. When possible, women with sight and hearing disabilities may do best to be roomed within sight and hearing distance of the hospital nurses’ station.
  • Persons with disabilities have the right under the ADA to use their service and companion animals. These are working animals and not pets. Never touch, address, or otherwise try to interact with or direct a service animal unless specifically asked or permitted. The animal’s continued availability to the owner should be preserved to the greatest extent possible. The service animal has been trained to respond directly to the commands of its owner. In most situations the service animal should remain with its owner. The provider and patient may need to discuss the nature and impact of a procedure or treatment that may be temporarily uncomfortable in regard to the service animal. In court rulings, the presence of a service animal is permissible unless a qualified medical professional determined with specificity the reason the animal would pose a threat to health or safety in the medical setting that a human would not pose.
  • Back to Kate. Her pelvic pain and vaginal discharge require your evaluation during this visit. In light of the fact that she is very frightened of a pelvic examination and there is no sign language interpreter available, how might you conduct this office visit History taking and discussion with patient Do modified history using note writing and having patient complete history form Patient examination Consider doing an external examination and wet prep. Symptom management If appropriate, start medication treatment. Follow up Schedule follow up appointment as soon as possible with an ASL interpreter to complete patient history and full GYN examination Use phone relay to report findings of culture and changes in treatment.
  • In summary, accurate communication is the key consideration when accommodating the needs of women with sensory disabilities in a clinical setting. In most situations, asking the patient what will be helpful in the situation is the best start. Each woman with a sensory disability is unique with varying degrees and forms of sight and hearing loss. There are a number of technology devices that can assist the clinician with communication. The ADA requires that the clinician provide effective communication to patients who are deaf and hard of hearing. The cost of sign language interpreters is not billable to the patient or their insurance, however the cost of accommodation for people with disabilities is subject to tax credits.
  • Transcript

    • 1. Part IV Reproductive Health Specific to Disability
    • 2. Module 1 - Physical Disabilities Spinal cord injury Spina bifida Multiple sclerosis Cerebral palsy
    • 3. Sub-Module 1 Spinal Cord Injury (SCI)
    • 4. Objectives Spinal Cord Injury - SCI <ul><li>Upon completion of this module, the participant will be able to: </li></ul><ul><li>Recognize the specific interaction of menses, sexuality and menopause in women with spinal cord injury. </li></ul><ul><li>Describe the onset, symptoms and management of autonomic dysreflexia (ADR) </li></ul><ul><li>Describe considerations involving pregnancy, labor, delivery and postpartum for women with SCI </li></ul><ul><li>Identify specific resources available for the patient with SCI and her provider. </li></ul>
    • 5. Suzanne
    • 6. About SCI
    • 7. Source: Wikipedia, 2008 Segmental Spinal Cord Level and Function Level Function C1-C6 Neck flexors C1-T1 Neck extensors C3-C5 Supply diaphragm (mostly C4) C5-C6 Shoulder movement, raise arm, flex elbow, supinates arm C6-C7 Extends elbow and wrist, pronates wrist C7-T1 Flexes wrist, supply small muscles of the hand T1-T6 Intercostals and trunk above waist T7-L1 Abdominal muscles L1-L4 Thigh, hip muscles, L4-S1 Hamstrings and dorsiflexion of foot L4-S2 Plantar flexion of foot and toe movement
    • 8. About Autonomic Dysreflexia <ul><li>Autonomic dysreflexia (ADR) is the most important ob/gyn concern for women with spinal cord lesions </li></ul><ul><li>Spinal cord lesions at or above T6 segment – 50% incidence of ADR </li></ul><ul><li>Causes severe hypertension </li></ul><ul><li>Potentially lethal medical emergency </li></ul>
    • 9. Precipitating Factors of ADR <ul><li>Bladder or bowel distention or irritation </li></ul><ul><li>Cutaneous lesions </li></ul><ul><li>Menstruation </li></ul><ul><li>Sexual activity </li></ul><ul><li>Pelvic and rectal exams </li></ul><ul><li>Labor </li></ul>
    • 10. Signs and Symptoms of ADR <ul><li>Hypertension </li></ul><ul><ul><li>Baseline BP in SCI  90/60 </li></ul></ul><ul><ul><li>120/80 may be abnormally high </li></ul></ul><ul><ul><li>BP can reach 300/220 </li></ul></ul><ul><li>Piloerection </li></ul><ul><li>Flushing </li></ul><ul><li>Pounding headache </li></ul><ul><li>Sweating </li></ul><ul><li>Nasal congestion </li></ul><ul><li>Malaise </li></ul><ul><li>Skin tingling </li></ul><ul><li>Nausea </li></ul><ul><li>Blurred vision </li></ul><ul><li>Cardiac dysrhythmia </li></ul>
    • 11. Management of ADR During GYN Examinations
    • 12. Menstruation after SCI <ul><li>Usually stop menstruation up to 6 months following injury, most return to cycling </li></ul><ul><li>Increased incidence of prolonged amenorrhea </li></ul><ul><li>Increased autonomic symptoms during menses </li></ul><ul><li>Premenstrual dysphoric disorder (PMDD) symptoms continued after SCI </li></ul>
    • 13. Sexual Activity <ul><li>Women with SCI can have active and enjoyable sex lives post injury. </li></ul><ul><li>Lubrication is dependent on level and completeness of injury. </li></ul><ul><li>Orgasm may be independent </li></ul><ul><li>of the level of injury </li></ul><ul><li>LINK Part 1 Module 2 </li></ul>
    • 14. Pregnancy and Spinal Cord Injury
    • 15. Data on Pregnancy and Spinal Cord Injury (SCI) <ul><li>No studies investigating fertility and pregnancy after SCI </li></ul><ul><li>Case-reports and expert opinion inform obstetric management of pregnancy </li></ul>
    • 16. Pregnancy Complications for Women with SCI Urinary tract infections Decubitus ulcers Alterations in pulmonary function Deep vein thrombosis Constipation Increased spasticity
    • 17. Prevention of Pregnancy Complications <ul><li>Monitor ability to transfer and ambulate </li></ul><ul><li>Monitor weight gain closely </li></ul><ul><li>Leg elevation </li></ul><ul><li>Range of motion exercises </li></ul><ul><li>Monitor need for increased services </li></ul>
    • 18. ADR and Pregnancy/Labor <ul><li>Prevention </li></ul><ul><ul><li>Avoid distended bladder </li></ul></ul><ul><ul><li>Avoid constipation/fecal impaction </li></ul></ul><ul><ul><li>Discuss past episodes of ADR and triggers </li></ul></ul><ul><ul><li>Discuss measures to relieve ADR </li></ul></ul><ul><li>Link to ADR section (slides 8-11) </li></ul>
    • 19. ADR v. Preeclampsia Source: Pereira 2003 ADR Preeclampsia Clonus, edema Clonus, edema Acute onset Variable onset BP increased during contractions BP consistently elevated Bradycardia Tachycardia Proteinuria absent Proteinuria Normal labs Abnormal labs Intense flushing, gooseflesh, diaphoresis common Not prominent
    • 20. Labor and Delivery <ul><li>Instruct patient in uterine palpation and unique symptoms of labor </li></ul><ul><li>Judicious use of early labor regional anesthesia for sympathetic blockade </li></ul><ul><li>Monitor closely for ADR symptoms </li></ul><ul><li>Increased incidence of operative vaginal delivery </li></ul>
    • 21. Postpartum Care Spinal Cord Injury <ul><li>Inspect perineum for signs of infection. Use ice packs with care. </li></ul><ul><li>Watch closely for postural hypotension </li></ul><ul><li>Assist with breast feeding, particularly if low nipple sensation (link to Part III- Mod 3 breastfeeding) </li></ul><ul><li>Monitor for breast health </li></ul>
    • 22. Osteoporosis and SCI <ul><li>Rapid bone loss of 25-50% in lower extremities occurs immediately post-injury. </li></ul><ul><li>Spasticity with consequent bone tension leads to additional bone loss. </li></ul><ul><li>Over 90% of postmenopausal women have osteoporosis on screening exams </li></ul><ul><li>Treatment should be used, but the safety of bisphophonates in women with reproductive potential is still unclear. </li></ul><ul><li>Link Part 3 – Module 7 </li></ul>
    • 23. Other Medical Risks with SCI
    • 24. Case Study <ul><li>Considerations when prescribing contraception </li></ul><ul><li>Precautions during pelvic examination </li></ul><ul><li>Other components of well woman care </li></ul>
    • 25. Summary SCI <ul><li>The most important OB/GYN concern of SCI is ADR </li></ul><ul><li>Temporary amenorhea is common after acute SCI </li></ul><ul><li>Intrapartum care with SCI should be managed by a multidiciplinary team. </li></ul><ul><li>Early regional anesthesia is usually advised for labor management </li></ul><ul><li>Low bone density is common </li></ul>
    • 26. References – Spinal Cord Injury <ul><li>DeForge D, Blackmer J, Moher D, et al. Sexuality and Reproductive Health Following Spinal Cord Injury. Summary. Evidence Report/Technology Assessment: Number 109. AHRQ Publication No 05-E003-1, December 2004. Agency for Healthcare Research and Quality. Rockville MD. Downloaded from http://www.ahrq.gov/clinci/epcsums/sexlspsum.htm. on 8/8/08 . </li></ul><ul><li>American Spinal Cord Injury Association. Standard Neurological Classification Of Spinal Cord Injury. 2006. Downloaded from http://www.asia-spinalinjury.org/publications/2006_Classif_worksheet.pdf on 12/5/08 </li></ul><ul><li>Wikipedia – Spinal cord injury,. Downloaded from http://en.wikipedia.org/wiki/Spinal_cord_injury </li></ul><ul><li>on 12/17/08 </li></ul><ul><li>Campagnolo DI. Autonomic dysreflexia in spinal cord injury. 2006. Downloaded from http://emedicine.medscape.com/article/322809 on 12/17/08 </li></ul><ul><li>Jackson AB, Wadley V. A multicenter study of women’s “self-reported reproductive health after spinal cord injury. Arch Phys Med and Rehab 1999;80:1420-8. </li></ul><ul><li>Jackson AB. Medical management of women with spinal cord injury: A review. Topics in Spinal Cord Inj Rehabil 1995;1:11-26. </li></ul><ul><li>Research Review, Fall 2000 Published by UAB-RRTC on Secondary Condition of SCI, Birmingham, AL. Sownloaded from http://www.spinalcord.uab.edu/show.asp?durki=3237 on 8/7/08 </li></ul><ul><li>Whiple B. Sexual response in women with complete spinal cord injury.. Symposium at INABIS ’98. McMaster University. Downloaded from http://www.mcmaster.ca/inabis98/komisaruk/whipple0437/tow.html . On 8/11/08 </li></ul><ul><li>Sipski ML. Sildenafil effects on sexual and cardiovascular responses in women with spinal cord injury. Urology 2000;55(6):812-815 </li></ul><ul><li>Jackson A, Lindsey L, Llebine P, Poczatek R. Reproductive health for women with spinal cord injury. SCI Nursing 2004;21:88-91. </li></ul><ul><li>American College of Obstetrics and Gynecology. Committee Opinion #275 – Obstetric Management of Patients with Spinal Cord Injuries 2002;ACOG, Washingto n DC. </li></ul>
    • 27. References SCI <ul><li>Pope CS, Markenson GR, Bayer-Zwirello LA, Maissel GS. Pregnancy complicated by chronic spinal cord injury and history of autonomic hyperreflexia. Obstet. & Gyne. 2002;97:802-3 </li></ul><ul><li>Pereira L. Obstetric management of the patient with spinal cord injury. Obstetrical and Gynecological Survey 2003;58:678-86 </li></ul><ul><li>Estores IM, Sipski ML. Women’s issues after SCI. Topics in Spinal Cord Injury Rehabilitation 2004;10:107-25 </li></ul><ul><li>Pentland W, Walker J, Minnes P, Tremblay M, Brouwer B, Gould M. Women with spinal cord injury and the impact of aging. Spinal Cord 2002;40:374-387 </li></ul><ul><li>Weiss D. Osteoporosis and spinal cord injury. eMedicine Specialties2008.downloaded from www.emedicine.com/pmr/topic96.htm. on 8/1/08 </li></ul><ul><li>Jiang SD, Jiang LS, Dai LY. Management of osteoporosis in spinal cord injury. Clinical Endocrinology 2006;65:555-65. </li></ul><ul><li>Vestergaard P, Krogh K, Rejnmark L, Mosekilde L. Fracture rates and risk factors for fractures in patients with spinal cord injury. Spinal Cord 1998;36:790-6. </li></ul><ul><li>Smeltzer, S Zimmerman,V, and Capriotti,T. 2005 Arch Phys Med Rehab 86 (3); 582-6. </li></ul><ul><li>Physicians Desk reference (Bisphosphates) </li></ul><ul><li>Ornoy A. Wajnberg R. Diav-Citrin O. The outcome of pregnancy following pre-pregnancy or early pregnancy alendronate treatment. Reproductive Toxicology. 2006;22:578-9 </li></ul><ul><li>Cowley KC. Psychogenic and pharmacologic induction of the let-down reflex can facilitate breastfeeding by tetraplegic women: A report of 3 cases. Archives Of Physical Medicine And Rehabilitation 2005;86:1261-4. </li></ul><ul><li>Massagli TL, Reyes MR. Hypercalcemia and spinal cord injury. 2008. Downloaded from http://emedicine.medscape.com/article/322109 on 12/18/09 </li></ul><ul><li>Moonka R, Stiens SA, Resnick WJ, McDonald JM, Eubank WB, Dominitz, JA, Steizner, MG. The prevalence and natural history of gallstones in spinal cord injured patients. J AM Coll Surg. 1999;189:274-81 </li></ul>
    • 28. Spina Bifida Sub-Module 2 Spina Bifida
    • 29. Objectives Spina Bifida <ul><li>Recognize the specific interaction of menses, sexuality and menopause in women with spina bifida </li></ul><ul><li>Describe considerations involving pregnancy, labor, delivery and postpartum. </li></ul><ul><li>Identify specific resources available for the woman with spina bifida and her provider. </li></ul>
    • 30. Spina Bifida – Case Study <ul><li>Desires pregnancy </li></ul><ul><li>Spina bifida lesion at T8 </li></ul><ul><li>VP Shunt </li></ul><ul><li>Ileal conduit </li></ul>
    • 31. Medical Concerns in Women With Spina Bifida Source: Suzawa, 2006 Neurologic GI Urologic Orthopedic Dermatologic
    • 32. Neurologic Complications <ul><li>Hydrocephalus – VP Shunt </li></ul><ul><li>Most have normal intelligence </li></ul><ul><li>Most individuals with SB have strong verbal skills but have difficulties with attention and executive functioning. </li></ul>
    • 33. Urologic and GI Complications <ul><li>Urologic </li></ul><ul><li>Neurogenic bladder </li></ul><ul><li>GI </li></ul><ul><li>Constipation </li></ul><ul><li>Fecal incontinence </li></ul><ul><li>Obesity </li></ul>
    • 34. Orthopedic Complications
    • 35. Dermatologic <ul><li>Severe latex allergy in 75% </li></ul><ul><ul><li>Unknown etiology </li></ul></ul><ul><ul><li>Latex in many medical, clothing and household items </li></ul></ul><ul><li>Pressure ulcers </li></ul><ul><ul><li>Frequent skin examination ( link to Part 2-Mod 2 skin ) </li></ul></ul><ul><ul><li>Encourage frequent weight shifts </li></ul></ul>
    • 36. Pregnancy Preparation <ul><li>Fertility is not impaired </li></ul><ul><li>Genetic Counseling </li></ul><ul><li>Risk of neural tube defect in offspring </li></ul><ul><ul><li>Depends on frequency of occurrence within the family. </li></ul></ul><ul><ul><li>Small risk of affected pregnancy despite folic acid prophylaxis </li></ul></ul>
    • 37. Pregnancy Considerations <ul><li>Bladder and urinary tract </li></ul><ul><ul><li>Special care after urinary diversion surgery </li></ul></ul><ul><ul><li>Increased frequency of UTI </li></ul></ul><ul><li>VP Shunt failure </li></ul><ul><li>Increased risk of back pain due to spinal abnormalities </li></ul><ul><li>( Link -– Mod 1 ) </li></ul>
    • 38. Labor and Delivery Considerations <ul><li>May be unaware of the onset of labor </li></ul><ul><li>Anesthesia consult </li></ul><ul><li>Delivery of patients with a VP Shunt </li></ul><ul><ul><li>Vaginal delivery preferred </li></ul></ul><ul><ul><li>Consult with neurosurgeon </li></ul></ul><ul><ul><li>Pushing not contraindicated </li></ul></ul><ul><ul><li>Cesarean delivery precautions </li></ul></ul>
    • 39. Aging and Osteoporosis <ul><li>Kyphosis and scoliosis increase with age </li></ul><ul><ul><li>Compromise respiratory status </li></ul></ul><ul><ul><li>Complicate positioning for exams and the interpretation of bone densitometry. </li></ul></ul><ul><li>Osteoporosis can occur in childhood and persist into adult years . </li></ul>
    • 40. Summary <ul><li>Impaired executive functioning </li></ul><ul><li>Bladder and bowel incontinence </li></ul><ul><li>VP shunt may affect pregnancy, delivery and GYN surgery. </li></ul><ul><li>Genetic counseling and folic acid </li></ul><ul><li>Increased risk of osteoporosis due to onset of immobility at birth </li></ul>
    • 41. Case Study – Spina Bifida <ul><li>What more information do you need from Jennifer regarding her history? </li></ul><ul><li>What considerations are important for Jennifer prior to her pregnancy? </li></ul><ul><li>What are 3 issues related to her spina bifida that may be of concern during Jennifer’s pregnancy? </li></ul><ul><li>What considerations are necessary to prepare for Jennifer’s delivery? </li></ul>
    • 42. Resources <ul><li>Spina Bifida Association – Information and publications for providers and public. This includes the books featured below. Access at www.spinabifidaassociation.org </li></ul>
    • 43. References <ul><li>Hochber L and Stone J. Etiology, prenatal diagnosis, and prevention of neural tube defects. Up to Date. 2006 </li></ul><ul><li>American College of Obstetricians and Gynecologists. Neural tube defects. Practice Bulletin #44 ACOG 2003. Washington DC </li></ul><ul><li>Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: a 25-year prospective. Pediatric Neurosurg 2001;34:144-20. </li></ul><ul><li>Velde SV, Biervliet SV, Renterghem KV, Laecke EV, Hoebeke P, Winckel MV. Achieving Fecal Continence in Patients With Spina Bifida: A Descriptive Cohort Study. Journal of Urology. 2007 </li></ul><ul><li>Suzawa, H. Spina Bifida (powerpoint presentation. 2006. Downloaded from http://www.bcm.edu/medpeds/powerpoints/Spina%20Bifida.pps#257,3,Epidemiology on 8/13/08 </li></ul><ul><li>Rose BM, Holmbeck GN. Attention and executive functions in adolescents with spina bifida. J of Ped Psych 2007;32:983-94. </li></ul><ul><li>Liptak GSEvidence –based practice in spina bifida: Developing a research agenda. Presentation at the conference May 2003. Washington DC. Downloaded from http://www.spinabifidaassociation.org on 8/13/2008. </li></ul><ul><li>Klingbeil H, Baer HR, Wilson PE. Aging with a disability. Arch Phys Med Rehabil 2004;85(Suppl 3) S68-73 </li></ul><ul><li>Singhal B, Mathew KM. Factors affecting mortality and morbidity in adults spina bifida. Eur J Pediatric Surg. 1999:9(Suppl 1):31-2. </li></ul><ul><li>Levy E. Addressing sexuality in spina bifida. Pediatric News in Entrepreneur.com. Downloaded from http://www.entrepreneur.com/tradejournals/article/print/168434757.html on 8/14/08 </li></ul><ul><li>Vogel LC, Krajci KA, Anderson CJ. Adults with pediatric-onset spinal cord injury: part 2: musculoskeletal and neurological complications. J Spinal Cord Med 2002;25:117-23 </li></ul><ul><li>Arata M, Grover S, Dunne K, Bryan D. Pregnancy outcome and complications in women with spina bifida. J Reprod Med 2000; 45:743-748. </li></ul>
    • 44. Sub-Module 3 Multiple Sclerosis
    • 45. Objectives - Multiple Sclerosis <ul><li>Recognize the specific interaction of menses, sexuality and menopause for women with MS </li></ul><ul><li>Describe considerations involving pregnancy, labor, delivery and postpartum. </li></ul><ul><li>Identify specific resources available for the woman with MS and their provider. </li></ul>
    • 46. Case Study – Multiple Sclerosis (MS) <ul><li>38 years old </li></ul><ul><li>Decreased libido </li></ul><ul><li>Fatigue </li></ul><ul><li>Lack of lubrication </li></ul><ul><li>Decreased sensation </li></ul>
    • 47. Presenting Symptoms of MS <ul><li>Optic neuritis </li></ul><ul><li>Extreme fatigue </li></ul><ul><li>Paresthesias </li></ul><ul><li>Spasticity </li></ul><ul><li>Lower extremity weakness </li></ul><ul><li>Loss of coordination </li></ul><ul><li>Pain </li></ul><ul><li>Acute onset of bowel and bladder dysfunction </li></ul>
    • 48. GYN Considerations <ul><li>Possible worsening of neurologic symptoms with menses (self-report) </li></ul><ul><li>40-80% of women report sexual dysfunction </li></ul><ul><li>Fatigue commonly contributes to sexual dysfunction </li></ul><ul><li>Depression may be associated with CNS changes. </li></ul><ul><li>Smoking may increase disease progression </li></ul>
    • 49. The Effect of Pregnancy on MS <ul><li>No change in fertility </li></ul><ul><li>Symptoms of MS may stabilize or remit during pregnancy with 20-40% of patients having relapse within 3 months after delivery. </li></ul><ul><li>No evidence suggests that pregnancy affects long-term course of MS </li></ul><ul><li>Increased risk for child having MS (2.5X) </li></ul>
    • 50. Drug Therapy Used for MS and Pregnancy Category Drug Cat Drug Cat Interferon Beta-1a and 1b C Cyclophosphamide D Glatiramer acetate B Azathioprine D Mitoxantrone D Corticosteroids B-C Methotrexate X Baclofen C
    • 51. MS – Labor and Delivery <ul><li>Patient may not recognize labor onset </li></ul><ul><li>Epidural anesthesia does not increase relapse rate and is effective for treating labor-induced spasticity </li></ul><ul><li>Weakened maternal expulsive effort may be indication for operative vaginal delivery </li></ul>
    • 52. MS- Postpartum
    • 53. MS -Osteoporosis <ul><li>Frequent use of steroids and immunosuppressants increases risk </li></ul><ul><li>Low- trauma fracture rate as high as 22% </li></ul><ul><li>More than half (53.7%) of postmenopausal women with MS were found to have low BMD on screening (Smeltzer 2005) </li></ul>
    • 54. MS - Aging <ul><li>Older individuals diagnosed have more progressive MS </li></ul><ul><li>Overlapping symptoms of MS and aging </li></ul><ul><li>More risk of UTI, pneumonia, septicemia and cellulitis </li></ul><ul><li>Decrease in cognition due to disease </li></ul>
    • 55. Summary: Multiple Sclerosis <ul><li>Neurological symptoms may worsen in the premenstrual period </li></ul><ul><li>Fertility and menstruation are not altered </li></ul><ul><li>Pregnancy and MS: </li></ul><ul><ul><li>Symptoms may stabilize or remit during pregnancy, relapse after delivery is common </li></ul></ul><ul><ul><li>Overall no long term effect on disease course </li></ul></ul><ul><li>Increased risk of steroid related osteoporosis </li></ul>
    • 56. Case Study – Multiple Sclerosis <ul><li>Additional Information needed? </li></ul><ul><li>Recommendations </li></ul>
    • 57. Resource <ul><li>National Multiple Sclerosis Society - http://www.nationalmssociety.org/index.aspx </li></ul>
    • 58. References - MS <ul><li>Benedetto-Anzai MT. Obstetric and gynecological management of women with multiple sclerosis. Presentation at ACOG Annual Clinical Meeting 2005, San Francisco </li></ul><ul><li>Multiple Sclerosis Society. What is Multiple Sclerosis. Downloaded from http://www.nationalmssociety.org/about-multiple-sclerosis/what-is-ms/index.aspx on 8/13/08 </li></ul><ul><li>Shabas D, Weinreb H. Preventive healthcare in women with multiple sclerosis. J Women’s Health & Gender-Based Med 2000;9:389-95 </li></ul><ul><li>Foley FW. Clinical Bulletin: Assessment and treatmetn of sexual dysfunction in multiple sclerosis. National Multiple Sclerosis Society 2008. Downloaded from http://www.nationalmssociety.org/about-multiple-sclerosis/symptoms/sexual-dysfunction/index.aspx on 8/14/08 </li></ul><ul><li>Somers EC. Marder W. Christman GM. Ognenovski V. McCune WJ. Use of a gonadotropin-releasing hormone analog for protection against premature ovarian failure during cyclophosphamide therapy in women with severe lupus. Arthritis & Rheumatism. 52(9):2761-7 </li></ul><ul><li>Confavreaux C, Hutchinson M, Hours MM, et al. Rate of pregnancy related relapse in MS. NEJM 1998;339:285-91 </li></ul><ul><li>Damek D, Shuster E. Pregnancy and MS. Mayo Clinic Proc 1997;72:977-1009 </li></ul><ul><li>Giesser B. Reproductive issues in persons with multiple sclerosis. Clinical Bulle tin Information for Health Professionals. National Multiple Sclerosis Society 2003. Downloaded from www.nationalmssociety.org/PRC.asp . on 8/18/08 </li></ul><ul><li>Freedman MS. Is spinal anesthesia contraindicated for patients with MS? Medscape: Ask the experts about Multiple Sclerosis/Neuroimmunology 20002. At http:// www.medscape.com/viewarticle/442083 accessed 1/26/07. </li></ul><ul><li>Dilorenzo TA, Halper J, Picone MA. A comparison of older and younger individuals with multiple sclerosis: A preliminary investigation. Rehabilitation Psychology 2004;49:123-5. </li></ul>
    • 59. MS- References Con’t. <ul><li>DiLorenzo TA. Aging with Multiple Sclerosis Clinical Bulletin.National Multiple Sclerosis Society 2006. Downloaded from http://www.nationalmssociety.org/for-professionals/healthcare-professionals/publications/clinical-bulletins/download.aspx?id=167 on 8/22/08 </li></ul><ul><li>Stern, M. Aging with multiple sclerosis. Physical Medicine and Rehabilitation Clinics of North America 2005:16:219-34 </li></ul><ul><li>Fleming ST, Blake RL. Patterns of comorbidity in elderly patients with multiple sclerosis. Journal of Clinical Epidemiology 1994;47:1127-32 </li></ul><ul><li>Kneebone, II, Dunmore EC, Evans E. Symptoms of depression in older adults with multiple sclerosis: Comparison with a matched sample of yournger adults. Aging and Mental Health 2003;7:182-5. </li></ul><ul><li>Schwid SR, Goodman JE, Puzas, McDermott MP, Mattson DH. Sproadic corticosteroid pulses and osteoproosis in multiple sclerosis. Archives of Neurology 1996;53:753-7. downloaded from http://archneur.ama-assn.org/cgi/content/abstract/53/8/753 on 8/21/08 </li></ul><ul><li>Smeltzer, S Zimmerman,V, and Capriotti,T. Osteoporosis risk and low bone mineral density in women with physical disabilities .Arch Phys Med Rehab 2005; 86 : 582-6. </li></ul>
    • 60. Sub-Module 4 Cerebral Palsy www.ucp.org/uploads/Heather%20Dibblee%2007.jpg
    • 61. Objectives – Cerebral Palsy <ul><li>Recognize the specific interaction of menses, sexuality and menopause in women with cerebral palsy </li></ul><ul><li>Describe considerations involving pregnancy, labor, delivery and postpartum. </li></ul><ul><li>Identify specific resources available for the woman with cerebral palsy and their provider. </li></ul>
    • 62. Case Study – Cerebral Palsy <ul><li>40 years old </li></ul><ul><li>CP with speech and cognitive impairment </li></ul><ul><li>Spasticity and some contractures </li></ul>
    • 63. Characteristics of Cerebral Palsy (CP) <ul><li>Disorder of motor function arising from insult or injury to the developing brain </li></ul><ul><li>Characterized by abnormal muscle tone, deep tendon reflexes, and posture </li></ul><ul><li>Variable degree of cognitive and communicative impairment </li></ul><ul><li>Seizure disorder present in 30% </li></ul><ul><li>Vision and hearing centers may be involved </li></ul><ul><li>Growing number of adults with CP </li></ul>
    • 64. Health Status of Women with CP <ul><li>Health behaviors, high majority </li></ul><ul><li>Non-smoker, Non-drinker </li></ul><ul><li>Participate in some physical activity </li></ul><ul><li>Associated conditions: </li></ul><ul><li>Cognitive impairment (33%), </li></ul><ul><li>Learning disabilities (25%) </li></ul><ul><li>Seizure disorders (40%) </li></ul><ul><li>Pain ( 84%) </li></ul><ul><li>Secondary conditions: </li></ul><ul><li>Pain, hip and back deformities </li></ul><ul><li>Bowel and bladder problems </li></ul><ul><li>Poor dental health, GE reflux </li></ul>Source: Turk 1997
    • 65. CP - Reproductive Health Issues <ul><li>Increased spasticity and incontinence during menstruation reported </li></ul><ul><li>Seizure medications may interfere with contraception methods – ( Link Part 3-Mod 1) </li></ul><ul><li>Pain and contractures may affect sexuality </li></ul><ul><li>Contractures and deformities may require adaptive measures for mammograms (link Part 2-Mod 2) and GYN examinations . (link Part 2-Mod 1 ) </li></ul><ul><li>Developmentally appropriate sex and contraception education </li></ul>
    • 66. CP-Pregnancy <ul><li>Expected pregnancy changes: </li></ul><ul><ul><li>mobility, pain </li></ul></ul><ul><ul><li>bladder and bowel control and function </li></ul></ul><ul><li>Monitoring/adjustment of medications (Link Part 3 Module 3) </li></ul><ul><li>Influence of contractures and spasticity on positioning for exams, labor analgesia, and delivery (Link Part 2-Module 1) </li></ul><ul><li>Mobilization of parenting supports </li></ul><ul><li>Pregnancy outcomes appear to be normal </li></ul><ul><li>Link Part 3- Module 3 </li></ul>
    • 67. CP –Osteoporosis <ul><li>Osteoporosis at early age, due to non-use of bones </li></ul><ul><li>Increased non-traumatic fractures </li></ul><ul><li>Use of upright or semi-prone standing in young adults may improve some bone mineral density. </li></ul><ul><li>Treatment considerations ( Link Part 3-Module 7) </li></ul>
    • 68. CP – Aging (Link to Part 3 – Module 7)
    • 69. Case Study – Cerebral Palsy <ul><li>What more information do you need? </li></ul><ul><li>What will assist your examination? </li></ul><ul><li>What preventive health measures are needed? </li></ul>
    • 70. Summary – Cerebral Palsy <ul><li>Symptoms of irregular muscle tone and reflexes. usually associated with spasticity. </li></ul><ul><li>Increased symptoms during menstruation. </li></ul><ul><li>Chronic pain </li></ul><ul><li>Examinations can be facilitated by alternative positioning and antispasmodics </li></ul><ul><li>Pregnancy planning will likely require medication adjustment </li></ul><ul><li>Osteoporosis at early age </li></ul>
    • 71. References – Cerebral Palsy <ul><li>Turk, MA, Geremski CA, Rosenbaum PF, Weber RJ. The health status of women with cerebral palsy. Arch of Physical Medicine and Rehabilitation 1997;78 (Supp 5) S10-17 </li></ul><ul><li>Poulos AE, Balandin S, Llewellyn G, Dew AH. Women with cerebral palsy and breast cancer screening by mammography. Archives of Physical Medicine and Rehabilitation 2006;87:304-7. </li></ul><ul><li>Smith RA, et al. American Cancer Society guidelines for breast cancer screening: update 2003. CA Cancer J Clin 2003;53:141-69 </li></ul><ul><li>Winch r, Bengtson L, McLaughlin j, et al. Women with Cerebral Palsy: Obstetric experience and neonatal outcome.Developmental Medicine in Child Neurology.1993;35:974-82 </li></ul><ul><li>Klingbeil, H. 2004 Arch Phys Med Rehab 85 (3); 68-73. </li></ul><ul><li>Caulton, J et al. 2004 Arch Dis Child 89;2;131-5. </li></ul><ul><li>Resource: United Cerebral Palsy Association - http://www.ucp.org/ </li></ul>
    • 72. Sub-Module 5 Other Physical Disabilities Osteogenesis Imperfecta Post-Polio Syndrome
    • 73. Objectives – Other Physical Disabilities <ul><li>Following this sub-module the participant will be able to: </li></ul><ul><li>Identify some women’s health care implications of osteogenesis imperfecta and post-polio syndrome </li></ul><ul><li>Discuss women’s health care management strategies for those with osteogenesis imperfecta and post-polio syndrome </li></ul><ul><li>Identify resources and references on these disabilities for the provider and patient. </li></ul>
    • 74. OI – Reproductive Health <ul><li>May experience heavy bleeding </li></ul><ul><li>Fertility rate not influenced </li></ul><ul><li>Genetic counseling </li></ul><ul><li>Cesarean delivery may be recommended to reduce pelvic bone fracture and for diminished pelvic outlet </li></ul><ul><li>Anesthesia consult </li></ul><ul><li>Hernia prevention with permanent suture to close aponeurotic tissues </li></ul>
    • 75. Post-Polio Syndrome (PPS) <ul><li>Affects up to 50% of polio survivors years after recovery from an initial attack. </li></ul><ul><li>Symptoms: </li></ul><ul><ul><li>increasing muscle weakness </li></ul></ul><ul><ul><li>fatigue </li></ul></ul><ul><ul><li>often pain. </li></ul></ul><ul><li>Slowly progressive. </li></ul>
    • 76. Post-Polio Syndrome (PPS) Women’s Health <ul><li>Increased risk of osteoporosis (Link Part 3, Mod 7) </li></ul><ul><li>Accelerates physiological effects of menopause </li></ul><ul><ul><li>Compounded muscle weakness leads to poor balance </li></ul></ul><ul><ul><li>Weakening chest wall leading to decreased pulmonary function </li></ul></ul><ul><ul><li>Atrophic changes in urinary tract leading to incontinence (link Part 3, Mod 4) </li></ul></ul><ul><ul><li>Joint stiffness and fatigue leading to sexual dysfunction (Link Part 1, Mod 2) </li></ul></ul>
    • 77. Resources/References <ul><li>Resources </li></ul><ul><li>Osteogenesis Imperfecta Foundation – http://www.oif.org </li></ul><ul><li>Post-Polio Health International - http://www.post-polio.org </li></ul><ul><li>References </li></ul><ul><li>Osteogenesis Imperfecta Foundation. OI Issues: pregnancy. 2007. Accessed at http://oif/org on 1/13/09. </li></ul><ul><li>Robetts JM, Solomons CC. Management of pregnancy in Osteogenesis Imperfecta: New perspectives. Obstet and Gynecol 1975;45:168-70 </li></ul><ul><li>Vogel TM, Ratner EF, Thomas RC, Chitkara U. Pregnancy complicated by severe osteogenesis imperfecta: A report of two cases. Anesthesia and Analgesia 2002;94:1315-17. </li></ul><ul><li>March of Dimes. Post-Polio Syndrome Quick feference : Fact sheets. Downloaded from http://www.marchofdimes.com/printable Articles/14332_1284.asp. on 2/10/09 </li></ul><ul><li>Jubelt B, Agre JC. Characteristics and management of Postpolio Syndrome. JAMA 2000;284:412-14 </li></ul><ul><li>Welner SL, Simon JA, Welner B. Maximizing health in menopausal women with disabilities. Menopause 2002;9:208-19 </li></ul>
    • 78. Module 2 Intellectual and Developmental Disabilities (IDD)
    • 79. Case Study- IDD Sexual abuse Pregnancy Seizure medications Sex education Contraception
    • 80. Objectives Intellectual and Developmental Disabilities <ul><li>After completing this module, the participant will be able to: </li></ul><ul><li>Identify special considerations in taking a history when working with women with IDD </li></ul><ul><li>Describe appropriate methods of providing sex education </li></ul><ul><li>Discuss how to gain cooperation of the woman with IDD during a GYN examination </li></ul><ul><li>Describe the impact of menstruation and surgical procedures for women with IDD </li></ul><ul><li>Discuss resolution for issues of informed consent for women with IDD </li></ul>
    • 81. Definition - Intellectual and Developmental Disabilities <ul><li>Includes at least 3 of the following limitations: </li></ul>Classified according to intelligence quotient 83-52 Mild 51-36 Moderate Below 36 Severe <ul><li>Self care </li></ul><ul><li>Language </li></ul><ul><li>Learning </li></ul><ul><li>Mobility </li></ul><ul><li>Economic self-sufficiency </li></ul><ul><li>Self direction </li></ul><ul><li>Independent living </li></ul>
    • 82. Reproductive Care Issues
    • 83. Issues Seeking Health Care <ul><li>Resistance to exams due to history of forced examinations causing </li></ul>Pain Anxiety Hostility
    • 84. Establishing Communication <ul><li>Establish face to face contact with non-hurried manner </li></ul><ul><li>Choose an environment with the fewest distractions </li></ul><ul><li>Use basic language and establish contact with the patient </li></ul><ul><li>Assess how the non-verbal patient communicates </li></ul><ul><li>Assess patient understanding </li></ul>
    • 85. Taking A GYN History <ul><li>Gain information first from patient and then from other means available. </li></ul><ul><li>Use menstrual and PMS calendars </li></ul><ul><li>Use drawings/photos/anatomic models to </li></ul><ul><ul><li>assess level of body and sexual knowledge </li></ul></ul><ul><ul><li>assess level of sexual activity and possibly abuse </li></ul></ul><ul><ul><li>to explain GYN examination </li></ul></ul>
    • 86. Health Education <ul><li>All women benefit from reproductive health education </li></ul><ul><li>Excellent resources are available: </li></ul><ul><ul><li>Women Be Healthy </li></ul></ul><ul><ul><li>Let’s Talk About Health: What Every Woman Should Know </li></ul></ul>
    • 87. Consent and Sexual Relations <ul><li>No standard screening but the following understanding should be assessed: </li></ul><ul><ul><li>That sex is an activity that both participants want and engage in voluntarily. </li></ul></ul><ul><ul><li>That no one can force or threaten you to have sex. </li></ul></ul><ul><ul><li>That you can refuse to engage in sexual activity with someone even if you have agreed to engage in it before with the same person, and that it is enough to just say &quot;no&quot; without having to provide justification for the refusal. </li></ul></ul><ul><ul><li>That sex is usually engaged in private </li></ul></ul><ul><ul><li>That it is not proper to have sex for money or gifts </li></ul></ul><ul><ul><li>That it is not proper to have sex with children, immediate blood relations or animals </li></ul></ul><ul><li>Adapted from : Griffiths (Ed) Ethical Dilemmas: Sexuality and Developmental Disability, 2002 </li></ul>
    • 88. Sexuality and Sex Education <ul><li>Have same sexual needs as the general population </li></ul><ul><li>Parental and society uncomfortable with sexual needs and expression </li></ul><ul><li>Keep sex education simple and concrete with repetition and demonstrations. </li></ul><ul><li>Teach avoidance strategies for sexual abuse. </li></ul><ul><li>See Part 1, Module 2 – Sexuality (link) </li></ul>
    • 89. Menstruation and Menstrual Hygiene <ul><li>Menarche is often early </li></ul><ul><li>Teach menstrual hygiene in a repetitious step-by-step manner </li></ul><ul><li>Most women who manage their own toileting can be taught to self-care for menses </li></ul><ul><li>Use hormonal intervention only </li></ul><ul><ul><li>After behavioral intervention has failed </li></ul></ul><ul><ul><li>If menses interfere with patient’s quality of life </li></ul></ul>
    • 90. Cyclical Behavior Changes <ul><li>Occurrence –16% menstruating women with IDD </li></ul><ul><li>Symptoms –temper tantrums, crying, autistic or self abusive behavior, seizures </li></ul><ul><li>Diagnosis - Documentation </li></ul><ul><li>Therapy – </li></ul><ul><ul><li>First NSAIDs (behavior may be due to cramps) </li></ul></ul><ul><ul><li>Then try OCPs, DMPA, SSRIs </li></ul></ul>Source: Quint 1999
    • 91. Abnormal Uterine Bleeding <ul><li>Menorrhagia common with Down syndrome, hypothyroidism and obesity </li></ul><ul><li>Antipsychotic medications linked to hyperprolactinemia </li></ul><ul><li>Menstrual calendars and </li></ul><ul><li>pad counts assist diagnosis </li></ul><ul><li>Non-surgical management </li></ul><ul><li>See Part 3, Module 2 </li></ul>
    • 92. The GYN Examination <ul><li>See Part 2, Module 1 for preparation and positioning tips (link), particularly avoid the use of stirrups </li></ul><ul><li>Give options of who will accompany her. </li></ul><ul><li>If the patient can tolerate a speculum, use a small bladed Huffman or Pedersen </li></ul><ul><li>Use a modified bimanual examination (one finger) through the vagina or rectum </li></ul>
    • 93. Cervical Cancer Screening <ul><li>Guidelines same as general population </li></ul><ul><li>Often inadequate past medical history to identify HPV risk </li></ul><ul><li>Alternative to speculum exam is a blind Pap smear </li></ul><ul><li>Resolving institutional requirements </li></ul>
    • 94. Ultrasound <ul><li>Use for screening vs. medical indication </li></ul><ul><li>Issues for this population: </li></ul><ul><ul><li>Bladder filling </li></ul></ul><ul><ul><li>Patient cooperation </li></ul></ul><ul><li>Does not reveal condition of the cervix or vagina including lesions, discharge or bleeding. </li></ul>
    • 95. Sedation vs. Anesthesia
    • 96. Surgical Procedures <ul><li>Guidelines for hysterectomy and endometrial ablation same as general population </li></ul><ul><li>Endometrial ablation should not be used in the younger population for menstrual hygiene </li></ul><ul><li>Federal, state and local regulations </li></ul>
    • 97. Considerations for Surgical Procedures
    • 98. Informed Consent <ul><li>Assessment of capacity to consent requires multiple criteria </li></ul><ul><li>Despite ability to consent – important to get patient’s assent </li></ul><ul><li>Be alert to coercion and conflict of interest </li></ul><ul><li>Process often involves state and jurisdictional statutes </li></ul>
    • 99. Sterilization <ul><li>Legal /ethical issues </li></ul><ul><li>Exhaust other possibilities: i.e. hormonal preparations and patient education. </li></ul><ul><li>Jurisdictional differences govern guardian and parental requests </li></ul><ul><li>Patient autonomy is key: “The presence of a mental disability does not, in itself, justify either sterilization or its denial” </li></ul><ul><li>Source: ACOG Committee Opinion #371, 2007 </li></ul>
    • 100. Pregnancy and Parenting <ul><li>Fertility </li></ul><ul><li>Genetic Counseling </li></ul><ul><li>Delay in seeking prenatal care </li></ul><ul><li>Anti-seizure medications </li></ul><ul><li>Education on impact of pregnancy on the body </li></ul><ul><li>Concerns for labor and delivery </li></ul><ul><li>Care of the child after delivery </li></ul>
    • 101. IDD-Osteoporosis <ul><li>Greater risk for women with IDD due to: </li></ul><ul><li>Menstrual irregularities </li></ul><ul><li>Hypothyroidism </li></ul><ul><li>Use of anticonvulsants </li></ul><ul><li>Use of steroids </li></ul><ul><li>Earlier menopause </li></ul><ul><li>Greater inactivity </li></ul><ul><li>Vitamin D deficiency </li></ul>
    • 102. Down Syndrome (DS) <ul><li>Most common genetic disorder. Secondary conditions include: </li></ul><ul><li>Congenital heart disease – 50% </li></ul><ul><li>Altered immune response </li></ul><ul><li>Hypothyroidism – 20% </li></ul><ul><li>Auditory disorders – 60 to 70% </li></ul><ul><li>Obstructive sleep apnea – 50% </li></ul><ul><li>Altered vision – 50% </li></ul><ul><li>Mental illness – particularly depression and obsessive compulsive disorder and dementia </li></ul>
    • 103. DS - Reproduction
    • 104. DS - Aging <ul><li>Early aging </li></ul><ul><li>Frequent early dementia </li></ul><ul><li>Early menopause </li></ul><ul><li>Osteopenia and </li></ul><ul><li>osteoporosis </li></ul>
    • 105. Intellectual and Developmental Disabilities Summary <ul><li>Assure confidentiality and autonomy to the maximum the situation allows </li></ul><ul><li>Provide sex and health education that is age and developmentally appropriate </li></ul><ul><li>Menstruation has impact on the patient and caregivers. </li></ul><ul><li>Acting out behavior is often a response to physical or psychological conditions </li></ul><ul><li>Premature aging and osteoporosis </li></ul>
    • 106. IDD Case Study Discussion Sexual Abuse Pregnancy Seizure Medications Sex education Contraception
    • 107. Resources - Curricula <ul><li>Women Be Healthy – Curricula for teaching general and reproductive health education – University of NC at Chapel Hill – one copy free of charge. Order at http://www.fpg.unc.edu/~ncodh/WomensHealth/week2.cfm </li></ul><ul><li>Let’s Talk About Health – What Every Woman Should Know – Illustrated 170 pg. workbook, DVD, audio tapes. Author: Caryl Heaton, DO. The ARC of New Jersey contact Dianne Flynn – [email_address] or call 732-246-2525 x 28. </li></ul><ul><li>Sexuality education videos for persons with developmental disabilities. Choices, inc. Making Connections and Person to Person - www.johncarmody.net/clients/choices/ </li></ul><ul><li>Through the Looking Glass – has a variety of publications and services to assist women with disabilities with pregnancy and parenting issues. http://lookingglass.org/index.php </li></ul><ul><li>Management Guidelines: Developmental Disability, Version2, 2005. Therapeutic Guidelines Ltd. N. Melbourne Aust. www.tg.com.au </li></ul>
    • 108. References <ul><li>Quint EH. Gynecological health care for mentally disabled women. Presentation at NASPaG Meeting, May 1996. </li></ul><ul><li>Muram D, Elkins TE. Reproductive health care needs of the developmentally disabled. In: Sanfilippo JS, ed. Pediatric and Adolescent Gynecology, Philadelphia, PA. WB Saunders Co, 1994:490-498 </li></ul><ul><li>Bradshaw, KD, Elkins TE, Quint EH. The patient with mental retardation: Issues in gynecologic care. University of Texas Southwestern Medical Center, Dallas Texas. 1996. </li></ul><ul><li>Edwards JP, Elkins TE. Just Between Us: A Social Sexual Training guide for Parents and Professionals with Concerns for Person with Developmental Disabilities. Austin, TX: Pro_Ed. 1988. </li></ul><ul><li>Lennox N, Beange H, Davis R, Duvasula S, Edwards N, Graves P, et al. Management guidelines, Developmental Disabilities, 2 nd ed. Melbourne, Australia: Therapeutic Guidelines, 2005. Downloaded from www.tg.com.au/index.php?sectionid=100 on 1/6/08 </li></ul><ul><li>Lee JK, Saw HS. Can human papilloma virus DNA testing substitute for cytology in the detection of high-grade cervical lesions? Arch Pathol Lab Med. 2004;128:298-302. </li></ul><ul><li>American College of Obstetricians and Gynecologists. Practice Bulletin #45: Cervical cancer screening. ACOG 2003 </li></ul><ul><li>American College of Obstetricians and Gynecologists. Access to Reproductive Health Care for women with Disabilities in Special Issues in Women’s Health Care. ACOG, Washington DC, 2004. </li></ul><ul><li>Kavoussi SK, Smith YR, Ernst SD, Quint EH. Cervical cancer screening with liquid cytology in women with developmental disabilities. J of Women’s Health 2009;16:115-8. </li></ul><ul><li>Quint EH, Elkins TE. Cervical cytology in women with mental retardation. Obstet Gynecol 1997;89:123-6 </li></ul><ul><li>Rosen DA, Rosen KR, Elkins TE, Anderson HF, McNeeley SG, Song C. Outpatient sedation: An essential addition to gynecologic care for person with mental retardation. Am J Obstet Gynecol. 1991;164:825-828 </li></ul><ul><li>Pueschel SM, Jackson IMD, Giesswein P, et al. Thyroid function in Down syndrome. Res Dev Disabil Res 1991;12:287-96. </li></ul>
    • 109. References , Con’t. <ul><li>Down’s Syndrome Medical Interest Group. Medical Series No. 2: Thyroid disorder among people with Down’s Syndrome: Notes for doctors. Down’s Syndrome Association 2006. Downloaded from www.dsmig.org.uk . 1 on 0/3/08 </li></ul><ul><li>Elkins T. gynecologic care. In: Purschel SM, Pueschel JK, eds. Biomedical Concerns in Persons with Down Syndrome. Baltimore, MD: Paul H. Brookes Publischin gCol, Linc.: 1992: pgs 139-46. </li></ul><ul><li>American College of Obstetricians and Gynecologists. Committee Opinion No. 371: Sterilization of women, including those with mental disabilities. ACOG, 2007, Washington, DC. </li></ul><ul><li>In re Montgomery, 311 N.C. 101,316, E.E.2d 246 (1984). </li></ul><ul><li>Hayman RL. Presemptions of justice: Law, politics and the mentally retarded parent. Harvard Law Review. 1990;103. </li></ul><ul><li>National Institute on Child Health and Human Development. Facts About Down Syndrome. Downloaded from http://www.nichd.nih.gov/publications/pubs/downsyndrome.cfm?renderforprint=1 on 10-6-08. </li></ul><ul><li>deHingh YC, van der Vossen PW, Gemen EF, Mulder MB, et al. Intrinsic abnormalities of lymphocyte counts in children with Down syndrome. J Peds 2005;147:744-7. </li></ul><ul><li>Smith DS. Health care for adults with Down syndrome. 2001. Downloaded from http://healthlink.mcw.edu/article/1001820316.htm . on 10/6/08 </li></ul><ul><li>National Health Service Direct. Down’s syndrome complications. 2008. Downloaded from : http://www.nhsdirect.nhs.uk/articles/article.aspx?articleId=136&sectionId=6 on 10/7/08 </li></ul><ul><li>Elkins TE, Anderson FH. Sterilization of persons with mental retardation. JASH. 1992;17:19-26 </li></ul>
    • 110. References <ul><li>Meijboom F, Szatmari A, Utens E, Deckers JW, Roelandt JR, Bos E, Hess J. Long-term follow-up after surgical closure of ventricualr septak defect in infancy and childhood. J Am Coll Cardiol. 1994;24:1358-64. </li></ul><ul><li>Tager-Flusberg, H. Neurodevelopmental Disorders , MIT Press, Cambridge MA. 1999.- Pg 163 </li></ul><ul><li>Wilkinson JE, Culpepper L, Cerreto M. Screening tests for adults with intellectual disabilities. J Am Board Fam Med. 2007;20:399-407. </li></ul><ul><li>Management Guidelines: Developmental disability Version 2Therapeutic Guidelines Ltd. N. Melbourne Australia. 2005, Pg. 230 </li></ul><ul><li>Schupf N, Pang D, Patel BN, Silverman W, Schubert R, Lai F, Kline JK, Stern Y, Ferin M, Tycko B, Mayeus R. Onset of dementia is associated with age at menopause in women with Down’s syndrome. Ann Neurol 2003;54:433-8 </li></ul><ul><li>Ranganath R, Rajangam S. Menstrual history in women with Down syndrome – A review.. Indian J of Human </li></ul><ul><li>Genetics 2004;10:18-21 </li></ul><ul><li>Bovicelli L, Orsini LF, Rizzo N, Montacuti V, Bacchetta M. Reproduction in Down syndrome. Obstet Gynecol 1992;59(Supple)13S-17S. </li></ul><ul><li>Van Dyke DC, McBrien DM, Sherbondy A. Issues of sexuality in Down syndrome. Down Syndrome Research and Practice. 1995;3;65-69 </li></ul><ul><li>Schrager S. Epidemiology of osteoporosis in women with cognitive impairment. Mental Retardation 2006;44:203-11. </li></ul><ul><li>Dinerstein RD, Herr SS, O’Sullivan . A guide to Consent. 1999, Washington DC. ;American Association on mental retardation. </li></ul>
    • 111. Module 6 Sensory Disabilities Hard of Hearing Low Vision
    • 112. Case Study – Sensory Disabilities Previous adverse experience with pelvic exam Communicates via sign language but also does some lip reading. Acute symptoms requiring evaluation.
    • 113. Objectives – Sensory Disabilities <ul><li>At the completion of this module the participant will be able to: </li></ul><ul><li>Describe unique strategies in health care delivery for women who are hard of hearing </li></ul><ul><li>Describe unique strategies in health care delivery for women who have visual impairment. </li></ul>
    • 114. Hearing Impairment Overview <ul><li>9% of US Population (Ries, 1994) </li></ul><ul><li>The patient’s age, place of birth, the timing and amount of hearing loss and preferred communication modality determines communication strategies: </li></ul><ul><ul><li>Hard of hearing </li></ul></ul><ul><ul><li>Deaf who communicate orally </li></ul></ul><ul><ul><li>Deaf who communicate using sign language </li></ul></ul>
    • 115. Communication Barriers for the Deaf or Hard-of-Hearing <ul><ul><li>Lip reading </li></ul></ul><ul><ul><li>Masks </li></ul></ul><ul><ul><li>Note writing </li></ul></ul><ul><ul><li>Reading comprehension </li></ul></ul><ul><ul><li>Family as interpreters </li></ul></ul><ul><ul><li>Automated telephone systems </li></ul></ul>
    • 116. Communication Facilitators for the Deaf and Hard-of-Hearing <ul><li>Free telephone relay services </li></ul><ul><li>Text messaging </li></ul><ul><li>Discounted communications equipment </li></ul><ul><li>Tax incentives for providing accommodation </li></ul>
    • 117. Suggestions for Communication <ul><li>ASK patient how she prefers to communicate </li></ul><ul><li>Prepare written easy to read instructions for basic office procedures </li></ul><ul><li>Minimize back ground noise </li></ul><ul><li>Have good lighting </li></ul><ul><li>Be discrete in public setting – No raised voices </li></ul><ul><li>Inform patient before touching or moving </li></ul>
    • 118. Hard of Hearing ADA Requirements <ul><li>Provision of a qualified interpreter </li></ul><ul><li>Flexibility in policy, procedure and practice </li></ul>
    • 119. Hard of Hearing OB/GYN Health Issues <ul><li>May have decreased family history information </li></ul><ul><li>Face masks during delivery and surgical procedures inhibit lip reading </li></ul><ul><li>Assure means for follow up communication </li></ul>
    • 120. Communication Suggestions for the Blind and Low Vision <ul><li>Don’t make assumptions about functional effects of visual acuity </li></ul><ul><li>Do not touch or remove mobility aids </li></ul><ul><li>Describe procedures before performing them </li></ul><ul><li>All written forms and documents should be read aloud in a private setting </li></ul><ul><li>Reading aloud may not provide effective communication for some patients </li></ul><ul><li>Use preferred techniques when handling money or credit cards </li></ul>
    • 121. Health Information Considerations for the Visually Impaired <ul><li>Assure patient information is in a form patient can utilize </li></ul><ul><li>Formats include : braille, large-print texts, audiotape recordings, videotapes or DVDs with oral descriptions and computer diskettes (Iezzoni 2006) </li></ul><ul><li>See resources section to access large type, braille and recorded patient information. </li></ul>
    • 122. Pregnancy, Labor and Delivery <ul><li>Assure that an appropriate means of communication is established in each setting </li></ul><ul><ul><ul><li>Cues for anticipated needs </li></ul></ul></ul><ul><ul><ul><li>Contact procedures for off hour emergencies </li></ul></ul></ul><ul><li>Determining the presence of bleeding or discharge for pregnant women with low vision </li></ul><ul><li>Establish a plan for labor and delivery </li></ul>
    • 123. Working with Service Animals Service animals should remain with their owner whenever possible
    • 124. Case Study – Sensory Disabilities <ul><li>Communicates with ASL, no interpreter available </li></ul><ul><li>Fearful due to past experience </li></ul><ul><li>Current pelvic disorder, requiring evaluation </li></ul>
    • 125. Summary – Sensory Disabilities <ul><li>Assuring accurate communication is key to patient care </li></ul><ul><li>ASK the patient what will help in the situation </li></ul><ul><li>Utilize technology available </li></ul><ul><li>ADA requirement to provide qualified interpreter and other office accommodations. </li></ul>
    • 126. References – Sensory Impairment <ul><li>Ries PW. Prevalence and characteristics of persons with hearing trouble: United States, 1990-91. Vital Health Stat. 10. 1997 </li></ul><ul><li>Barnett S. Communication with deaf and hard-of-hearing people: A guide for medical education. Academic Medicine2002;77:694-700 </li></ul><ul><li>National Institutes of Health. Healthy People 2010: #28, Vision and Hearing. Downloaded from http://www.healthypeople.gov/Document/HTML/Volume2/28Vision.htm on 6/6/08 </li></ul><ul><li>Steinburg AG, Sullivan VJ, Montoya LA. Lipreading the stirrups: An investigation of deaf women’s perspectives of their health, health care, and providers. Paper presented at National Health Service Corps 25 th Anniversary Meeting in Washington, DC 1998. </li></ul><ul><li>Iezzoni LI, O’Day BL. More than Ramps: A Guide to Improving Health Care Quality and Access for People with Disabilities. Oxford University Press, New York, NY 2006. </li></ul><ul><li>Price,S. Legal Briefings: Service animals under the ADA. ADA & IT Technical Assistance Centers 2006. Downloaded from http://www.equipforequality.org/resourcecenter/ada_serviceanimals.pdf on 10/20/08 </li></ul><ul><li>Meador HE, Zazove P. Health care interactions with deaf culture. J American Board of Family Medicine 2005;18:218-22 </li></ul>
    • 127. Resources <ul><li>American Council of the Blind http://www.acb.org/resources/index.html </li></ul><ul><li>American Foundation for the Blind: </li></ul><ul><li>National Association of the Deaf –http//: www.nad.org </li></ul><ul><li>Pregnancy information DVD with Signing for the Hard of Hearing – “ Your pregnancy and what to expect” contact Harris Communications www.harriscomm.com click on consumer education. </li></ul>

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