Living with Spina Bifida Transitional Care to adulthood & beyond 30 years experience

1. Living with Spina BifidaTransitional Care to adulthood & beyond30 years experience Dr. D. J. Richard Morgan Imperial College School of Medicine Chelsea & Westminster Hospital

2. Multi-disciplinary Assessment clinic for Adult Spina Bifida and/or Hydrocephalus patients • Specialist interest clinic advising a specific disability group • 30 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. Comprehensiveness Coordination Continuity Standards of care Integrate services Improve efficiency Adult attitudes Maximize potential Main conceptual dimensions

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

17. Clinic resources • Imaging cooperation – reserved slots for ultrasound scans, nuclear medicine scans, plain 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. What would you wish to improve to increase your independence, or enhance your quality of life?

20. Become continent78%

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

22. Urinary Continence • Assessment & evaluation- CIC? • Infection Control • Improve bowel function • Drug therapy • Surgical options

23. Urology options • IDC • SPC • Urostomy/Ileal Conduit • Clam cystoplasty/augmentation • Mitrofanoff • Artificial Sphincter

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

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

26. Spina Bifida/Hydrocephalus Medical Problems Neurological – LD, Epilepsy, Emotional problems, STM and Executive function impairment Mobility difficulties – Multiple issues relating paraplegia. Neuropathic ulcers. Cellulitis. Leg fractures Fertility issues – male infertility. Female obstetric problems, and foetal health concerns

27. Clinic Population 2017 • 650 patients age 18-84 55% male • 55% SpB & HC 34% HC 10% SpB only • 1% other – (CP or other neurodegenerative diseases)

28. When I’m Sixty-four When I get older – losing my hair, many years from now Will you still be sending me an clinic appointment? Kidney ultrasound, MRI scan? If I’d been absent and missed an appointment Would you close the file? Will you still heed me? Will you still see me - When I’m Sixty-four?

29. Older Cohort Statistics Male – 33 Female - 51

30. Age and Gender

31. Aging Spina Bifida

32. Associated Neurological Issues Arnold-Chiari M – 2 – one decompression Cervical cord Syrinx – One Cord Tethering – 6 Spinal Stenosis Epilepsy – 10 Learning Difficulties – 4 Visual problems - 4

33. Continence • 48 Continent • 9 Neurogenic Bladders - ISC • 12 Diversions/stomas –and 1 sheath leg bag • 3 IDC – 1 SPC • 1 Artificial sphincters • 2 Renal impaired, 2 Renal Calculi • 2 Colostomy

34. Mobility 38 Independent – No aids 6 Use sticks/crutches 26 Wheel Chair 5 Decubitus Ulcers

35. Orthopaedic issues Amputations – BKA 6, Bilateral 1 Arthritis & LBP -10 THR – 2 Osteomyelitis – 3 Osteoporosis – 5 Spontaneous/Low impact # - 3 Lymphoedema - 3

36. General Medical conditions Hypertension – 4; IHD – 2 Asthma/COPD/RLD – 4 Diabetes – 6 OSA – 4 CVA – 1 Depression – 1 Malignancy - 5

37. When I’m Sixty-four Doing the garden, digging the weeds Who could ask for more Will you still heed me? Will you still see me when I’m 64? Every summer we can rent a cottage On the Isle of Wight, if it’s not to dear We shall scrimp and save Grandchildren on your knee – Vera, Chuck and Dave.

38. Social circumstances 24 live independently alone 33 are married/live with partner 3 live in Residential care 6 live with parents 3 live with assistant support

39. Social Circumstances 2 7 are in employment 2 are retired 3 do voluntary work 1 attends day centre 23 Drive – and 3 more have stopped driving

40. Late problems – Middle life Obesity – 90% patients with SpB Obese T2DM – New finding in patients each year. OSA – often missed in SpB patients Cord Tethering problems in ambulant patients Chronic Vitamin D deficiency Psycho-social issues when parents/carers age Autonomy versus Care safety concerns.

41. Conclusions • Spina Bifida is a complex condition with multiple health and social issues for young disabled adults. • Many different ‘system’ factors contribute to potential problems in health for these people • Constant review by multi-disciplinary teams provide the best results and opportunities for their well-being. • Continuity of care provides expertise and support which disabled adults value most highly.

42. Conclusions 2 • Aging Spina B/HC patients pose continuing health and social concerns beyond the scope of single specialty services. • Many factors contribute to the complexity of their care. Long term follow-up can prevent complications developing before serious consequences occur. • Constant review by multi-disciplinary teams provide the best results. • Many are enjoying successful independent living.

43. When I’m Sixty-four Send me a postcard, drop me a line stating point of view Indicate precisely what you mean to say Yours sincerely – wasting away Give me your answer, fill in a form Mine for evermore Will you still heed me? Will you still see me When I’m sixty-four? Lennon & McCartney 1967

44. Multidisciplinary team 2012