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

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

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

• Coordination

• Continuity

• Standards of care

• Integrate services

• Improve efficiency

• Adult attitudes

• 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

Multi-professional staffing

• Continence advisor

• Occupational Therapist

• Physiotherapist

• Specialist Living Advisor

• Sexuality Advisor

• Neuro-psychologist

• Othotist

• Orthoptist

• TVN

• Physician

• Urologist

• Proctologist

• Orthopaedic surgeon

• Neurosurgeon

• Obstetrician/ACU

• General clinic nursing

• Neurologist

• Gynaecologist

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

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?

Become continent

78%

Continence Problems 1997

• 82 Neurogenic Bladders

• 22 Diversions/stomas

• 43 CIC

• 3 Artificial sphincters

• 84 Faecal continence problems

Urinary Continence

1. Assessment & evaluation- CIC?

2. Infection Control

3. Improve bowel function

4. Drug therapy

5. Surgical options

Urology options

1. IDC

2. SPC

3. Urostomy/Ileal Conduit

4. Clam cystoplasty/augmentation

5. Mitrofanoff

6. Artificial Sphincter

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 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.

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

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

Bowel Continence Problems

• Soiling

• Manual Evacuations by carers

• Social effects

•Bowel options

• Diet and routine

• Enemas/suppositories/laxatives

• Shandling catheter

• Anal Plugs

• ACE procedures

• Peristeen

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 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.

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

Recent Problems Identified

Diabetes

Anaemia

Vitamin D Deficiency

Osteoporosis

Shunt problems

‘Care more for the individual

patient than for the special features

of the disease’

William Osler