SB Adults Multidisciplinary clinic, United Kingdom
Adult CareA Multi-disciplinary clinic experience Dr. D. J. Richard Morgan Imperial College School of Medicine Chelsea & Westminster Hospital Mrs. Ann Wing Chelsea & Westminster HospitalSpina Bifida & Continence Nurse Specialist
Multi-disciplinary Assessment clinic for AdultSpina Bifida and/or Hydrocephalus patients• Specialist interest clinic advising a specific disability group• 19 year experience of complex needs in people born with a neural tube defect which results in life long issues• Referral from paediatrics, community agencies, General Practitioners and patient support agencies
Spina Bifida +/- Hydrocephalus• Outlook transformed in last 40 years• 1963 – 60% survival neonatal period• 1974 – 90% survival neonatal period• 2000 – 50-70% survive to adulthood• Adult services in UK & USA are uneven and fragmented, many patients „lost‟ after paeds• 66% have no regular review leading to serious complications – nephrectomy, decubitus ulcers.
Spina Bifida Adult Morbidity• Mobility – 33% Independent, 22% with assistance, 44% wheel chair dependent• 75% have IV shunts. 40% have epilepsy• 25% have mild to severe learning difficulties• 40% have scoliosis, 66% have joint deformities and contractures• 90% have urinary continence problems• 30-40% have faecal continence problems
Conceptual model of care• Medical needs are complex and challenging• Goal is to optimize physical, psychological & social health• Cross specialty multi-disciplinary care is required but not readily available in the adult setting• Adults with congenital complex disabilities need to be considered as adults
Main conceptual dimensions• Comprehensiveness • Standards of care • Integrate services• Coordination • Improve efficiency • Adult attitudes• Continuity • Maximize potential
The origins of our service• 1990 – Paediatric surgeons no longer allowed to admit patients over 16 to their „adolescent‟ unit• Surgeon concern for cohort of patient‟s future• Anxiety from families about follow-up, and rapid access availability when in difficulty• Approach made to „take on the challenge‟
First steps• Transition of care from paediatrics to adult clinic• Monthly combined clinic to meet patient & family with surgeon for hand-over• Also present – Continence advisor and Daily Living advisor from ASBAH
Adult clinic at Westminster• Routine out-patient suite• All age, many elderly patients• Small single consultation room• Small examination cubicles for fit adults• Lack of ability to meet patient alone• Life-line service for emergency care only
Chelsea & Westminster 1993• Use of Medical Day Unit for multi-disciplinary clinic development• Aim to maximise the visit by planning in advance• Annual MOT concept• Education potential• Research opportunity
Club 18-30• 1993 86 patients, 37 m, 49 f• Mean age 21.3 years• 48 SpB + HC, 32 HC, 6 SpB• 23 had continence problems (26%)• Less than half were independently coping• 10% had significant faecal control difficulty
Clinic Population 2006• 520 patients age 16-64 55% male• 55% SpB & HC 34% HC 10% SpB only• 1% other – (CP or other neurodegenerative diseases)
What would you wish to improve to increase your independence, or enhance your quality of life?
Advantages of Urostomy• Tried and tested, in use since 1950• Surgery not as big as newer options• Stoma care is relatively easy to learn• Lower incidence of post-operative complications
Disadvantages of Urostomy1. Continual urine leakage requiring need for appliance2. Skin excoriation3. Altered body image4. Inhibition of maintaining or creating new relationships5. Stoma site problem in chair bound/obese pt.
Advantages of a Continence Urinary Diversion1. No need to wear appliance2. Small stoma, 0.5-1.0 cm. diameter3. No urine leakage4. Improves or maintains body image5. No skin excoriation
Disadvantages/Drawbacks1. Patient must be enthusiastic and motivated to self catheterisation2. No guarantee of absolute stoma continence3. Major laparotomy scars may affect image4. Physical and psychological ability to sustain long term CIC5. Long operation, more post op complications6. Limited expertise to perform surgery
Bowel Continence Problems• Soiling• Manual Evacuations by carers• Social effects
Case studies: 1 -JO’D. 30 f. SpB & HC• Works P/T clerical, lives independently• Wheelchair dependent, transfers with boards• IDC for 19 years, recurrent blockage with scale• DN „upset at having to change it more than 6/52ly• Loosing time at work, job threatened• Fed up with overflow blockage & leakage
Case 1• Recurrent stones and intermittent UTIs• Does not want „bag‟• Fed up with IDC, cannot wear skirts in summer• Bowels spontaneous evacuation, soiling. Uses pads regularly• Consideration for mitrofanoff
Case 2: IC, 25 m, HC, SLD, Epilepsy, L 1/2p, W/C dep• Doubly incontinent, spontaneous voiding• Large volumes. Attempts to toilet train ineffective. Requires maxi size pads• Bowels regular laxatives and enemas• Attends adult training centre, lives in residential project Mon-Fri, W/E at home• Local continence supplier has restricted daily allowance to 3 pads per day.
Case 2 continued• Patient often returns from DC soaking.• Parents spending £20+ p.w. for high st pads• Clinic letter from Medical to request review.• CA to contact local CA• ASBAH field worker to contact local HA
Case 3: MZ, 18 f, SpB, ambulant, doing A levels• Neurogenic bladder – never dry• Wears nappies• Urodynamics show hyper-reflexic bladder• Trial of anti-muscarinics some help• CIC x 3hrly – still wet• 1996 Clam Cystoplasty – mucus++. Still wet• Refer for artificial sphincter
Case 4: SW 18m, SpB & HC,W/C dependent, attends college• Ileal conduit age 6• ACE aged 12 – „Brilliant‟ uses x3 pw.• Occasional UTIs. Bladder in situ. Recurrent discharge per urethra.• Urology – re-connect bladder +/- cystoplasty
Case 5: GN 24, SpB, W/C.• Mitrofanoff bladder.• Bowel problem. Soiling++• Nothing works, suppositories, enemas, shandling catheter.• “I want a bag”• Colostomy – Delighted. Revolutionised his life.
Case 6: ES, 24m, SpB. City worker, ambulant• Doubly incontinent, referred for this reason• Enjoys life, likes a few beers• Uses convene sheath leg bag. Gets embarrassed at work by this• Bowels – no awareness. Spontaneous daily evacuations. Some disasters. Pads not possible in city suit.• „Normal‟ sexual function
Case 6: Investigations• Bladder U/S- pre-mict vol 110 ml• post-mict vol 10ml• moderate hydronephrosis• U&Es normal• DPTA minor delay on left• Urodynamics unstable at high pressure. Delay sphincter release, on opening detrusor relaxes• Plan Trial of CIC and oxybutinin
Case 6 continued• CIC & oxybutinin - no different• Offered clam cystoplasty – declined• Bowels – own regime of codeine in week and picolax at weekends• Now married. Referred to ACU
Continence Conclusions1. Continence is a major concern for young disabled adults.2. Many factors contribute to incontinence3. Constant review by multi-disciplinary teams provide the best results.4. Newer surgical techniques are promising but not a panacea.5. Control of continence is the mark of independence which disabled adults prize most highly.
Sexuality• First steps – broaching the subject and dealing with parent/carers attitudes• ♀ - discussing menstrual concerns, contraception issues, sexual health issues, and possible future fertility desires. Links with ANC and ACU• ♂ - ED and Fertility discussions• Being aware of possible abuse in vulnerable
Shunt and Related problems• Acute disconnection/blockages – lack of local expertise• Insidious blockages – gradual obtunding of cognition• Hydrocephalus cognitive dysfunction – need to explain and support patients in employment.• Epilepsy – 40% shunt patients affected.• Emotional & Behavioural effects
‘Orthopaedic’ issues• Scoliosis – progressive early spinal degeneration causing LBP• Pressure ulcers – links with TVN and Plastics – essential input from OT /Physio/Orthotics• Progressive deformity from being chair bound• Shoulder wear and tear increasing• Obesity – 90%. Electric chairs make this worse
Other Medical problems• OSA – Headache, drowsy/lethargic – 25 patients in our clinic successfully treated with NIV• GORD is common.• Cervical Spinal cord atrophy• Late onset ACM
Life needs – the SLA role• Discovering the real concerns and needs of the patient• Helping with the possible and pointing out the impossible• Making contacts with agencies to support the vulnerable• Feed back to other professionals – and advise on options for local support.
The aim of the clinic• To provide expertise and support for Adults with the complex multi system disorder of Neural Tube Defects by regular annual review.• To provide an immediate contact point when in difficulty where possible and where appropriate.• To maximise every patient‟s potential by considering them as whole individuals not system conditions independent of the rest of their body