This document describes an adult multi-disciplinary clinic in the UK for patients with spina bifida and/or hydrocephalus. The clinic has 19 years of experience providing specialized care for this patient population to address their complex medical needs. It utilizes a team of specialists from various disciplines to provide comprehensive, coordinated care. Through case studies, it illustrates some of the common issues patients face with mobility, continence, sexuality, shunt complications and how the clinic works to optimize patients' physical, psychological and social health through specialized treatment and management plans.

1. Adult Care
A Multi-disciplinary clinic
experience
Dr. D. J. Richard Morgan
Imperial College School of Medicine
Chelsea & Westminster Hospital
Mrs. Ann Wing
Chelsea & Westminster Hospital
Spina Bifida & Continence Nurse Specialist

2. Multi-disciplinary Assessment clinic for Adult
Spina 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

3. 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.

4. 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

5. 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

6. Main conceptual dimensions
• Comprehensiveness • Standards of care
• Integrate services
• Coordination • Improve efficiency
• Adult attitudes
• Continuity • Maximize potential

8. 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‟

9. 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

10. 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

12. 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

14. Multi-professional staffing
• Continence advisor • Physician
• Occupational Therapist • Urologist
• Physiotherapist • Proctologist
• Specialist Living Advisor • Orthopaedic surgeon
• Sexuality Advisor • Neurosurgeon
• Neuro-psychologist • Obstetrician/ACU
• Othotist • General clinic nursing
• Orthoptist • Neurologist
• TVN • Gynaecologist

17. Clinic resources
• Imaging cooperation – reserved slots for
ultrasound scans, nuclear medicine scans, plain
x-rays, shunt sydtem x-rays
• Physiology studies – Urodynamics, Pelvic floor
• EEG, CT/MRI scanning
• Endoscopy facilities- cystoscopy/GI
• Flexible colleagues
• Transport, Catering, Stamina

18. 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

19. 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)

20. What would you wish to improve to
increase your independence, or
enhance your quality of life?

21. Become continent
78%

22. Continence Problems 1997
• 82 Neurogenic Bladders
• 22 Diversions/stomas
• 43 CIC
• 3 Artificial sphincters
• 84 Faecal continence problems

23. Urinary Continence
1. Assessment & evaluation- CIC?
2. Infection Control
3. Improve bowel function
4. Drug therapy
5. Surgical options

24. Urology options
1. IDC
2. SPC
3. Urostomy/Ileal Conduit
4. Clam cystoplasty/augmentation
5. Mitrofanoff
6. Artificial Sphincter

26. 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

27. Disadvantages of Urostomy
1. Continual urine leakage requiring need for
appliance
2. Skin excoriation
3. Altered body image
4. Inhibition of maintaining or creating new
relationships
5. Stoma site problem in chair bound/obese pt.

28. Advantages of a Continence Urinary
Diversion
1. No need to wear appliance
2. Small stoma, 0.5-1.0 cm. diameter
3. No urine leakage
4. Improves or maintains body image
5. No skin excoriation

30. Disadvantages/Drawbacks
1. Patient must be enthusiastic and motivated to
self catheterisation
2. No guarantee of absolute stoma continence
3. Major laparotomy scars may affect image
4. Physical and psychological ability to sustain
long term CIC
5. Long operation, more post op complications
6. Limited expertise to perform surgery

31. Bowel Continence Problems
• Soiling
• Manual Evacuations by carers
• Social effects

32. •Bowel options
• Diet and routine
• Enemas/suppositories/laxatives
• Shandling catheter
• Anal Plugs
• ACE procedures
• Peristeen

33. 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

34. 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

35. 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.

36. 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

37. 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

38. 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

39. 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.

40. 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

41. 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

42. 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

43. Continence Conclusions
1. Continence is a major concern for young disabled
adults.
2. Many factors contribute to incontinence
3. 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.

44. 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

45. 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

46. ‘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

47. 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

48. 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.

49. 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

50. Recent Problems Identified
Diabetes
Anaemia
Vitamin D Deficiency
Osteoporosis
Shunt problems

51. ‘Care more for the individual
patient than for the special features
of the disease’
William Osler