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  • Give an example of each abnormality: structure, function, and metabolism. How can they cause disabilities and/or death?
  • Describe each classification. Review one or two of each type of defect and discuss nursing assessment/interventions needed for each.
  • What is the main purpose of the March of Dimes? Audience Response Question #1 Active and efficient participation in prenatal care can significantly decrease the risk of congenital anomalies. 1. True 2. False
  • How are neural tube defects treated?
  • Refer to Figures 14-1 and 14-2 on page 323. Discuss hydrocephalus.
  • Discuss the pathophysiology regarding each cause of hydrocephalus.
  • Audience Response Question #2 Complete the analogy. Communicating hydrocephalus : inadequate absorption of CSF as non-communicating hydrocephalus : ____________________. 1. increased ICP 2. brain tumor 3. obstruction of CSF 4. shunting of CSF
  • How are classic signs related?
  • Describe each procedure listed.
  • Discuss the pathophysiology regarding each symptom listed.
  • Discuss Figure 14-3 on page 324.
  • Discuss the proper procedure to measure head circumference. What methods protect the infant from infection? How is pain measured in an infant?
  • Discuss Figure 14-4 on page 325.
  • How are the two types of spina bifida different?
  • Discuss the pathophysiology regarding an increased incidence of hydrocephalus with meningomyelocele spina bifida.
  • In what form is folic acid taken?
  • Discuss the term habilitation and the nurse’s role in the treatment process.
  • What methods assist the child during the habilitation process? Discuss methods to assist the parents and child in developing a healthy personality.
  • Discuss methods to prevent infection and injury to the sac. Which nursing interventions prevent contractures? What is the nurse’s role in providing education for the parents of a child with spina bifida?
  • How would you document assessment of the sac, head circumference, and fontanels?
  • Discuss the pathophysiology regarding each complication in relation to the disorder.
  • Refer to Figure 14-7 on page 327. Discuss cleft lip.
  • Discuss the rationale for preventing sucking and crying. Describe a Logan’s bow.
  • What is the difference between a cleft lip and a cleft palate? Discuss potential life-threatening complications associated with cleft palate.
  • What can each member of the team contribute to the therapy of a child with cleft palate?
  • Discuss the nurse’s role in assisting the family and child with each factor listed.
  • Describe talipes equinovarus.
  • Refer to Figure 14-8 on page 329. Discuss clubfoot.
  • What are passive stretching exercises?
  • Give examples of the nurse providing cast care instructions and emotional support to the parents.
  • Refer to Figure 14-9 on page 331. Discuss the pathophysiology of hip dysplasia.
  • Describe the gait of a child in which hip dysplasia is suspected.
  • Audience Response Question #3 Complete the analogy. Barlow's test : feeling as Ortolani's sign : _____________. 1. seeing 2. hearing 3. auscultating 4. palpating
  • Refer to Figure 14-10 on page 331. Discuss the Pavlik harness.
  • See Figure 14-10 on page 331 and Figure 14-11 on page 332.
  • Describe neurovascular assessment of the affected extremity.
  • Give an example of a toy that might fit inside the cast.
  • Give an example of an assessment of an infant with a suspected metabolic defect.
  • Discuss the pathophysiology regarding the manifestations listed.
  • Does your state require early screening for PKU?
  • What are some dietary restrictions for the infant or child with PKU?
  • What foods need to be restricted in the infant’s or child’s diet? Most meats, dairy products, and diet drinks.
  • What is acidosis?
  • Discuss the pathophysiology of the sweat characteristics and ketoacidosis.
  • If this defect is left untreated, what can occur? Cirrhosis of the liver, cataracts, and mental retardation.
  • In what ways can you provide parental support and education?
  • Discuss the pathophysiology regarding the parent’s age and this disorder. Occurs in 9 of 10,000 live births and may increase to 1 in 365 live births in mothers older than 35. Paternal age is also a factor, especially if he is older than 55.
  • What is nondisjunction? The failure of a chromosome to follow the normal separation process into daughter cells.
  • Discuss AFP, HCG, inhibin-A, and unconjugated estriol levels and confirmation of Down syndrome by amniocentesis.
  • Refer to Figure 14-12 on page 336. Discuss the manifestations of Down syndrome.
  • In what ways can the nurse assist the parents of a child with Down syndrome?
  • Compare a normal child’s development to the development of a child with Down syndrome. Refer to Table 14-1 on page 337.
  • Compare the normal child to the child with Down syndrome regarding feeding, toileting, and dressing. Refer to Table 14-2 on page 337.
  • Review terms related to this condition in Box 14-2 on page 338.
  • Discuss the maternal sensitization process in the newborn. Refer to Figure 14-13 on page 338. What is anasarca?
  • Discuss the maternal health history and erythroblastosis fetalis. Describe the indirect Coombs’ test.
  • Discuss the pathophysiology of early induction of labor and/or intrauterine fetal transfusions.
  • Why is RhoGAM used in treatment?
  • Discuss the life-threatening condition of kernicterus.
  • Describe phototherapy and exchange transfusions.
  • Although phototherapy may prevent an increase in the level of bilirubin, it has no effect on the underlying cause of the jaundice.
  • Discuss the Biliblanket shown in Figure 14-16 on page 343.
  • Discuss the possible signs and symptoms of intracranial hemorrhage: Poor muscle tone Lethargy Poor sucking reflex Respiratory distress Cyanosis Twitching Forceful vomiting High-pitched cry Convulsions
  • Discuss the rationale for each question listed.
  • What nursing and medical care should be provided for a newborn with TTN?
  • How does amnioinfusion prevent meconium aspiration syndrome?
  • Discuss the rationale for the treatments listed.
  • What are some long-term effects an infant could experience who is born to a drug-addicted mother?
  • Discuss the pathophysiology of the symptoms listed. What is the rationale for each treatment listed?
  • Define macrosomia . Refer to Figure 14-17 on page 344.
  • How would you describe the Cushingoid appearance of the newborn? Give an example of a morning assessment of the newborn.

Chapter 014 lo Chapter 014 lo Presentation Transcript

  • Chapter 14The Newborn with a Perinatal Injury or Congenital Malformation
  • Objectives• List and define the more common disorders of the newborn.• Describe the classifications of birth defects.• Outline the nursing care for the infant with hydrocephalus.• Describe the symptoms of increased intracranial pressure.• Discuss the prevention of neural tube anomalies.Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 2
  • Objectives (cont.)• Outline the preoperative and postoperative nursing care of a newborn with spina bifida cystica.• Differentiate between cleft lip and cleft palate.• Discuss the dietary needs of an infant with phenylketonuria.• Discuss the early signs of developmental hip dysplasia.Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 3
  • Objectives (cont.)• Discuss the care of the newborn with Down syndrome.• Outline the causes and treatment of hemolytic disease of the newborn (erythroblastosis fetalis).• Devise a plan of care for an infant receiving phototherapy.• Describe home phototherapy.• Discuss the assessment and nursing care of a newborn with macrosomia.Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 4
  • Birth Defects• Abnormalities that are apparent at birth• The abnormality may be of – Structure – Function – Metabolism• May result in a physical or mental disability, may shorten life, or may be fatalElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 5
  • Classifications of Birth Defects• Malformations present at birth• May also be known as congenital malformations• Inborn errors of metabolism• Disorders of the blood• Chromosomal abnormalities• Perinatal injuriesElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 6
  • March of Dimes• Birth defects cannot be attributed to a single cause.• Combination of environment and heredity – Inherited susceptibility – Stage of pregnancy – Degree of environmental hazardElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 7
  • Nervous System• Neural tube defects – Most often caused by failure of neural tube to close at either the cranial or the caudal end of the spinal cord • Hydrocephalus • Spina bifidaElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 8
  • Hydrocephalus• Characterized by an increase in CSF within the ventricles of the brain – Causes pressure changes in the brain – Increase in head size – Results from an imbalance between production and absorption of CSF or improper formation of ventriclesElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 9
  • Hydrocephalus (cont.)• Most commonly acquired by – An obstruction – A sequelae of infection – Perinatal hemorrhage• Symptoms depend on – Site of obstruction – Age at which it developsElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 10
  • Hydrocephalus (cont.)• Classifications – Noncommunicating • Obstruction of CSF flow from the ventricles of the brain to the subarachnoid space – Communicating • CSF is not obstructed in the ventricles but is inadequately reabsorbed in the subarachnoid spaceElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 11
  • Manifestations of Hydrocephalus• Depends on time of onset and severity of imbalance• Classic signs – Increase in size of head – Cranial sutures separate to accommodate enlarging mass – Scalp is shiny – Veins are dilatedElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 12
  • Diagnosis and Treatment of Hydrocephalus • Diagnosis • Treatment – Transillumination – Medications to reduce – Echoencephalography production of CSF – CT scan – Surgery to place a – MRI shunt – Ventricular tap or punctureElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 13
  • Symptoms of Increasing Intracranial Pressure • Increased blood pressure • Decrease in pulse rate • Decrease in respirations • High-pitched cry • Unequal pupil size or response to light • Bulging fontanels in infants • Headaches in children due to closed cranial sutures • Irritability or lethargy • Vomiting • Poor feedingElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 14
  • Ventriculoperitoneal Shunt • Treatment – Medications to reduce CSF production – Surgery – Shunt acts as a focal spot for infection and may need to be removed if infections persistElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 15
  • Preoperative and Postoperative Nursing Care• Pre-Op • Post-Op – Frequent head position – Assess for signs of changes to prevent increased intracranial skin breakdown, head pressure must be supported – Protect from infection – Head must be – Depress shunt “pump” supported at all times as ordered by surgeon while being fed – Position dependent – Measure head upon multiple factors circumference along – Assess and provide for with other vital signs pain controlElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 16
  • Parent Education• Teach signs that indicate shunt malfunction may be occurring – How to “pump” the shunt• Signs of shunt malfunction in the older child can include – Headache – Lethargy – Changes in LOCElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 17
  • Spina Bifida (Myelodysplasia)Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 18
  • Spina Bifida (Myelodysplasia) (cont.)• Group of CNS disorders characterized by malformation of the spinal cord• A congenital embryonic neural tube defect with an imperfect closure of the spinal vertebrae• Two types – Occulta (hidden) – Cystica (sac or cyst)Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 19
  • Spina Bifida Occulta • Minor variation of the disorder • Opening is small • No associated protrusion of structures • Often undetected – May have a tuft of hair, dimple, lipoma, or discoloration at the siteElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 20
  • Spina Bifida Occulta (cont.)• Treatment generally not necessary unless neuromuscular symptoms appear, such as – Progressive disturbances of gait • Foot drop – Disturbances of bowel and bladder sphincter functionElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 21
  • Spina Bifida Cystica• Development of a cystic mass in the midline of the opening in the spine – Meningocele • Contains portions of the membranes and CSF • Size varies – Meningomyelocele • More serious protrusion of membranes and spinal cord through the opening • May have associated paralysis of lower extremities • May have poor or no control of bladder or bowel • Hydrocephalus is a common complicationElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 22
  • Prevention of Spina Bifida• Mother takes folic acid 0.4 mg per day prior to becoming pregnant and/or continues to take the folic acid supplement until the 12th week of pregnancyElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 23
  • Treatment of Spina Bifida• Surgical closure• Prognosis is dependent upon extent of spinal cord involvementElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 24
  • Meningocele• Contains portions of the membranes and CSF• If no weakness of the legs or sphincter involvement, surgical correction is performed with excellent resultsElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 25
  • Meningomyelocele• Protrusion of the membranes and spinal cord through the opening• Surgical intervention is done for cosmetic reasons and to help prevent infection• Habilitation is usually necessary post-op because the legs remain paralyzed and the patient is incontinent of urine and fecesElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 26
  • Habilitation• Patient is disabled from birth• Aim is to minimize the child’s disability• Constructively use all unaffected parts of the body• Every effort is made to help the child develop a healthy personality so that he or she may experience a happy and productive lifeElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 27
  • Nursing Care of Spina Bifida• Prevent infection of, or injury to, the sac• Correct positioning to prevent pressure on sac• Prevent development of contractures• Good skin care• Adequate nutrition• Accurate observations and charting• Education of the parents• Continued medical supervision and habilitationElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 28
  • Nursing Care of Spina Bifida (cont.)• Upon delivery, the newborn is placed in an incubator• Moist, sterile dressing of saline or an antibiotic solution may be ordered to prevent drying of the sac• Protection from injury and maintenance of a sterile environment for the open lesion are essentialElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 29
  • Nursing Care of Spina Bifida (cont.)• Size and area of sac are checked for any tears or leakage• Extremities are observed for deformities and movement• Head circumference is measured• Fontanels are observed to provide a baseline for future assessmentsElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 30
  • Nursing Care of Spina Bifida (cont.)• Complications that can be life-threatening must be monitored – Meningitis – Pneumonia – UTI• Urological monitoring• Skin care• Feeding• Potential for latex allergyElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 31
  • Gastrointestinal SystemElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 32
  • Cleft Lip • Characterized by a fissure or opening in the upper lip • Failure of maxillary and median nasal processes to unite during embryonic development • Many cases are hereditary, others are environmental • Appears to occur more often in boys than girlsElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 33
  • Treatment• Initial repair of cleft lip is known as cheiloplasty• Repair by 3 months of age• Infant may have to have elbow restraints to prevent the infant from scratching the lip• A special syringe or bottle may be needed to assist in feeding the child until surgery has occurredElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 34
  • Postoperative Nursing Care• Prevent infant from sucking and crying• Careful positioning to avoid injury to operative site• Preventing infection and scarring by gentle cleansing of suture lines to prevent crusts from forming• Providing for the infant’s emotional needs by cuddling and other forms of affection• Providing appropriate pain relief measuresElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 35
  • Feeding• Fed by medicine dropper until wound is completely healed (about 1 to 2 weeks)• Cleanse the mouth by giving the infant small amounts of sterile water at the end of each feeding sessionElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 36
  • Cleft Palate• The failure of the hard palates to fuse at the midline during the 7th to 12th weeks of gestation• Forms a passageway between the nasopharynx and the nose – Increases risk of infections of the respiratory tract and middle earsElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 37
  • Cleft Palate Treatment• Goals of therapy – Union of the cleft – Improved feeding – Improved speech – Improved dental development – The nurturing of a positive self-imageElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 38
  • Multidisciplinary Team Care• Along with the patient and family – Psychologist – Speech therapist – Pediatric dentist – Orthodontist – Social worker – PediatricianElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 39
  • Other Factors• Psychosocial adjustment of the family• Follow-up care• Home care• Surgery between 1 year and 18 months of ageElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 40
  • Postoperative Treatment and Nursing Care• Nutrition – Diet is progressively advanced – No food through straws to prevent sucking• Oral hygiene – Follow each feeding with clear water to cleanse the mouth• Speech – Encourage children to pronounce words correctly• Diversion – Crying should be avoided whenever possible; play should be of the quiet type (e.g., coloring, drawing, reading to the child)• Complications – Ear infections and tooth decayElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 41
  • Musculoskeletal SystemElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 42
  • Clubfoot• Most common deformities• Congenital anomaly – Foot twists inward or outward• Talipes equinovarus is the most common type – Feet turned inward – Child walks on toes and outer borders of feet – Generally involves both feetElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 43
  • Clubfoot (cont.)Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 44
  • Treatment and Nursing Care• Started as soon as possible or bones and muscles will continue to develop in an abnormal manner• Conservative treatment – Splinting or casting to hold foot in correct position – Passive stretching exercises• If not effective after about 3 months, surgical intervention may be indicatedElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 45
  • Parent Education• Stress importance of • The nurse should complying with review with the physician orders to parents prevent skin – Cast care breakdown and – Emotional support possible isolation of the older childElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 46
  • Developmental Hip DysplasiaElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 47
  • Developmental Hip Dysplasia (cont.)• Hip dysplasia applies to various degrees of deformities, subluxation or dislocation (can be partial or complete)• Head of femur is partly or completely displaced• Seven times more common in girls• More apparent as infant/toddler begins walkingElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 48
  • Developmental Hip Dysplasia (cont.)• Usually discovered at routine health checks during the first or second month of life• Most reliable sign is limited abduction of the leg on the affected sideElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 49
  • Diagnostics for Hip Dysplasia• Barlow’s test: upon adduction and extension of the hips (with health care provider providing stabilization to the pelvis), may “feel” the dislocation actually occur• Ortolani’s sign (or click): health care provider can actually feel and hear the femoral head slip back into the acetabulum under gentle pressureElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 50
  • Treatment of Hip Dysplasia• Hips are maintained in constant flexion and abduction for 4 to 8 weeks – Keeps head of femur within the hip socket• Constant pressure enlarges and deepens acetabulum• Can use a Pavlik harness to provide the necessary positioning• Surgical intervention may be necessaryElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 51
  • Pavlik Harness, Body Cast, and TractionElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 52
  • Nursing Care of Infant/Child in a Spica Cast • Neurovascular assessment of affected extremities • Place firm, plastic-covered pillows beneath the curves of the cast for support • In the older child, a “fracture” bedpan should be readily available for toileting • Head of bed slightly elevated to help drain any body fluids away from cast • Frequent changes of position are needed to prevent skin breakdownElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 53
  • Nursing Care of Infant/Child in a Spica Cast (cont.)• Toys that are small enough to “hide” in the cast should not be given to the child• Important to meet everyday needs• A special wagon with pillows inside it for support is one of the safest ways to transport a child in a spica castElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 54
  • Metabolic Defects• Inborn errors of metabolism involve a genetic defect that may not be apparent until after birth• Symptoms to report would include – Lethargy – Poor feeding – Hypotonia – Unique odor to body or urine – Tachypnea – VomitingElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 55
  • Phenylketonuria (PKU)• Faulty metabolism of phenylalanine, an amino acid essential to life and found in all protein foods• Infant unable to digest this essential acid and phenylalanine accumulates in blood and is found in the urine within the first week of life• Results in severe mental retardation if not caught earlyElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 56
  • Phenylketonuria (PKU) (cont.)• Appears normal at birth• By the time urine test is positive, brain damage has already occurred• Delayed development apparent at 4-6 months• May have failure to thrive, eczema, or other skin conditions• Child has a musty odor• Personality disorder• Occurs mainly in blonde, blue-eyed children• Results from a lack of tyrosine (needed for melanin formation)Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 57
  • PKU Diagnostics• Guthrie test• Blood for this test should be obtained 48 to 72 hours after birth• Preferably after the infant has ingested proteins• Many states require this test to be performed prior to discharge from hospitalElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 58
  • PKU Treatment• Close dietary management• Frequent evaluation of blood phenylalanine level• Synthetic food that provides enough protein for growth and tissue repair – Special formulas are available • Infants: Lofenalac or Phenex-1 • Children: Phenyl-free • Adolescents: Phenex-2Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 59
  • PKU Nursing Care• Teach parents importance of reading food labels• Following up as required with health care provider for blood tests• Referral to a dietitian is helpful in providing parental guidance and support• Genetic counseling may also be indicatedElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 60
  • Health Promotion• Children with PKU must avoid the sweetener aspartame (NutraSweet) because it is converted to phenylalanine in the bodyElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 61
  • Maple Syrup Urine Disease• Defect in the metabolism of branched-chain amino acids• Causes marked serum elevations of leucine, isoleucine, and valine• Results in acidosis, cerebral degeneration, and death within 2 weeks if not treatedElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 62
  • Maple Syrup Urine Disease (cont.)• Appears healthy at birth, but problems soon develop• Feeding difficulties• Loss of the Moro reflex• Hypotonia• Irregular respirations• ConvulsionsElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 63
  • Maple Syrup Urine Disease (cont.)• Manifestations – Urine, sweat, and cerumen (earwax) have a characteristic maple syrup odor caused by ketoacidosis – Diagnosis confirmed by blood and urine testsElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 64
  • Maple Syrup Urine Disease Treatment and Nursing Care• Treatment – Removing the amino acids and their metabolites from the body tissues • Hydration and peritoneal dialysis to decrease serum levels – Lifelong diet low in amino acids leucine, isoleucine, and valine – Exacerbations are usually related to degree of abnormality of leucine level• Infection can be life-threateningElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 65
  • Galactosemia• Unable to use galactose and lactose – Enzyme needed to help the liver convert galactose to glucose is defective or missing – Results in an increased serum galactose level (galactosemia) and in the urine (galactosuria)• If untreated can cause – Cirrhosis of the liver – Cataracts – Mental retardation• Galactose is present in milk in the form of sugar; therefore, early diagnosis is essentialElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 66
  • Galactosemia (cont.)• Begins abruptly, worsens gradually• Early signs – Lethargy – Vomiting – Hypotonia – Diarrhea – Failure to thrive• Symptoms begin as the newborn is fed• Jaundice may be presentElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 67
  • Galactosemia Treatment and Nursing Care• Milk and lactose-containing products are eliminated from the diet• Breastfeeding must be stopped• Lactose-free formulas or soy protein–based formulas are often used instead• Parental support and education is essentialElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 68
  • Chromosomal AbnormalitiesElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 69
  • Down Syndrome• Most common chromosomal abnormality• Risk increases with – Mothers 35 years and older – Fathers 55 years and older• Infant has mild to severe mental retardation• Some physical abnormalities are also seenElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 70
  • Down Syndrome (cont.) • Three phenotypes – Trisomy 21 • Most common • There are three number 21 chromosomes instead of the usual two • Results from nondisjunction (failure to separate) – Mosaicism • Occurs when both normal and abnormal cells are present • Tend to be less severely affected in appearance and intelligence – Translocation of a chromosome • A piece of chromosome in pair 21 breaks away and attaches itself to another chromosomeElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 71
  • Down Syndrome (cont.)• Screening for this is offered during prenatal care starting around week 15 of gestation – Allows parents the opportunity to decide on whether to continue or terminate the pregnancy• “Quad Test”: Alpha-fetoprotein (AFP), hCG, unconjugated estriol, inhibin-A levels are used for diagnosis• Amniocentesis is most accurateElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 72
  • Down Syndrome ManifestationsElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 73
  • Down Syndrome Manifestations (cont.)• Limp, flaccid posture caused by hypotonicity of muscles – More difficult to position and hold – Contributes to heat loss• Prone to respiratory illnesses and constipation due to the hypotonicity• Incidence of acute leukemia is higher• Alzheimer’s disease more common to those who reach middle adult life• Encourage parents to express their feelings and concerns• Provide parents with support and community referralsElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 74
  • Developmental Milestones• Sitting• Rolling over• Sitting alone• Crawling• Creeping• Standing• Walking• TalkingElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 75
  • Self-Help Skills• Eating• Toilet training• DressingElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 76
  • Perinatal InjuriesElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 77
  • Hemolytic Disease of the Newborn (Erythroblastosis Fetalis)• Becomes apparent in utero or soon after birth• Rh-negative mother and Rh-positive father produce Rh-positive fetus• Even though maternal and fetal blood do not mix during pregnancy, small leaks may allow fetal blood to enter the maternal circulation causing the mother’s body to start producing antibodies that cross the placenta and destroy the blood cells of the fetus, which can cause anemia and heart failure in the developing/growing fetusElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 78
  • Erythroblastosis Fetalis: Maternal SensitizationElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 79
  • Erythroblastosis Fetalis: Maternal Sensitization (cont.)• Mother accumulates antibodies with each pregnancy• Chance of complications occurs with each subsequent pregnancy• Severe form, hydrops fetalis, progressive hemolysis causes anemia, heart failure, fetal hypoxia, and anasarcaElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 80
  • Erythroblastosis Fetalis Diagnosis and Prevention• Maternal health history that includes – Previous Rh sensitizations – Ectopic pregnancy – Abortion – Blood transfusions – Child who developed jaundice or anemia during a neonatal period• Indirect Coombs’ test will indicate previous exposure to Rh-positive antigensElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 81
  • Erythroblastosis Fetalis Diagnosis and Prevention (cont.)• Confirmed by amniocentesis and monitoring of bilirubin levels in the amniotic fluid• Fetal Rh status can be determined non- invasively via free DNA in maternal plasma• Diagnostic studies will help the physician to determine if early interventions, such as induction of labor or intrauterine fetal transfusions, are neededElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 82
  • Erythroblastosis Fetalis Diagnosis and Prevention (cont.)• Use of Rh(D) immune globulin (RhoGAM)• Administered within 72 hours of delivery with an infant who is Rh-positive, an ectopic pregnancy, or after an abortion• May also be given to the pregnant woman at 28 weeks gestationElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 83
  • Erythroblastosis Fetalis Manifestations• Direct Coombs’ test on umbilical cord blood• Symptoms vary – Anemia caused by hemolysis of large numbers of erythrocytes – Pathological jaundice occurs within 24 hours of delivery; liver cannot handle the amount of hemolysis, bilirubin levels rise rapidly – Enlargement and edema of liver and spleen – Oxygen-carrying capacity of the blood is diminished, including blood volume – Infant at major risk of shock or heart failureElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 84
  • Erythroblastosis Fetalis Manifestations (cont.)• Kernicterus—bilirubin has reached toxic levels• Accumulated bilirubin in the brain tissue can cause serious brain damage and permanent disability• Infant will have jaundice along with – Irritability – Lethargy – Poor feeding – High-pitched, shrill cry – Muscle weakness – Progresses to opisthotonos – SeizuresElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 85
  • Erythroblastosis Fetalis Treatment• Prompt identification• Laboratory tests• Drug therapy• Phototherapy• Exchange transfusions, if indicatedElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 86
  • Erythroblastosis Fetalis Nursing Care• Ensure eyes are protected from phototherapy• Cover gonads• Provide incubator care• Central line care (usually the umbilical vein)• Observe newborn’s color• Apply wet, sterile compresses to the umbilicus, if ordered, until transfusions are completeElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 87
  • Nursing Tip• Assessing jaundice involves – The skin and the whites of the eyes assume a yellow-orange cast – Blanching the skin over bony prominences enhances the evaluation of jaundice• Jaundice that occurs on the first day of life is always pathological and requires prompt interventionElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 88
  • Home Phototherapy• Used for newborns with mild to moderate physiological (normal) jaundice• Less costly• May decrease the need for hospitalizationElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 89
  • Intracranial Hemorrhage• Most common type of birth injury• May result from trauma or anoxia• Occurs more often in preterm infants• May also occur during precipitate delivery or prolonged labor• Signs and symptoms vary depending on severityElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 90
  • Intracranial Hemorrhage (cont.)• Diagnosis • Treatment – History of traumatic – Oxygen delivery – Gentle handling – CT or MRI scan – Elevated head – Evidence of an – Medications may be increase in CSF prescribed pressure – Care with feeding because sucking reflex may be affectedElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 91
  • Intracranial Hemorrhage (cont.)• If convulsion occurs, notify physician immediately• Be ready to answer the following questions – Were the arms, legs, or face involved? – Was the right or left side of the body involved? – Was the convulsion mild or severe? – How long did it last? – What was condition of infant before and after the seizure (i.e., vital signs, skin color)?Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 92
  • Transient Tachypnea of the Newborn (TTN)• Characterized by • Typically resolves – Tachypnea suddenly after 3 days – May also include – May be caused by slow • Chest retractions absorption of fluid in • Grunting lungs after birth • Mild cyanosis • Supportive nursing• Often referred to as and medical care respiratory distress syndrome, type IIElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 93
  • Meconium Aspiration Syndrome• In utero – Fetus expels meconium into amniotic fluid – Cord compression or other condition interrupts fetal circulation – If asphyxia or acidosis occurs, fetus may have gasping movements that cause meconium-stained amniotic fluid to be drawn into the lungs• At delivery – Can occur if newborn inhales before nose and mouth have been suctionedElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 94
  • Meconium Aspiration Syndrome (cont.)• Symptoms • Treatment – Respiratory distress – Warmth – Nasal flaring – Oxygen – Retractions – Supportive care – Cyanosis – NICU – Grunting – Rales and rhonchi – Tachypnea may persist for several weeksElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 95
  • Neonatal Abstinence Syndrome (NAS)• Caused by fetal exposure to drugs in utero• Many illicit drugs cross the placental barrier; therefore, an infant born to a woman who is an addict will suffer drug withdrawal after birth• Infant may also have long-term developmental and neurological deficitsElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 96
  • Neonatal Abstinence Syndrome (NAS) (cont.)• Symptoms • Testing – Body tremors and – Meconium may be hyperirritability (primary more accurate than sign) neonatal urine testing – Wakefulness for presence of drugs – Diarrhea • Treatment – Poor feeding – Swaddling – Sneezing – Quiet environment – Yawning – Observe for seizures – PhenobarbitalElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 97
  • Infant of Diabetic Mother• Large amounts of glucose are transferred to fetus• Causes fetus to become hyperglycemic• Fetal pancreas produces large amount of fetal insulin• Leads to hyperinsulinism, along with excess production of protein and fatty acids, often results in an LGA newborn weighing 9 pounds (4082 g) or more (macrosomia)Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 98
  • Infant of Diabetic Mother (cont.)• After delivery, infant may have low blood glucose levels and Cushingoid appearance or look healthy• May have developmental deficits and suffer complications of RDS• Suffers from – Hypoglycemia – Hypocalcemia – HyperbilirubinemiaElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 99
  • Infant of Diabetic Mother (cont.)• Monitor – Glucose levels – Vital signs – Signs of irritability – Tremors – Respiratory distress• Glucose levels below 40 mg/dL can result in rapid and permanent brain damageElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 100
  • Question for Review• How can prenatal care prevent neural tube defects?Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 101
  • Review• Objectives• Key Terms• Key Points• Online Resources• Review QuestionsElsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 102