aaddm newsletter · 2016. 7. 11. · aaddm newsletter issue 24 november 2015 page 2 presentation:...

AADDM Newsletter Australian

Association of

Developmental

Disability

Medicine Inc.

T: 0401122273

E: [email protected]

By Christel Burton Funding cuts and changes in

structures, the ‘knock it down and

build it again’ approach; how do we

deal with the dissolution of our

Centres for Disability Health (CDH)?

Thank you, AADDM executive

committee, especially Dr Jacqueline

Small for inputs into submissions

about the flow on effects of these

changes.

There is such an enormous level of

unfairness in shutting down

Australia’s CDH. Apart from the fact

that they are an established system that

is functional and valued, specialist

clinicians refer to these clinics,

patients need the specialist adult

disability health expertise, carers need

them, health professionals need them

in order to upskill and train in

community disability medicine, and

families rely on them. The list

continues …

CDH patients will not ‘blend into

mainstream general practice’ for the

whole list of reasons that led to the

establishment of these clinics in the

first place! Leverage at the national

policy level is needed; in the absence

of that (we are clinicians not

politicians), the people who make the

funding decisions need to visit the

clinics. The clinics are not an abstract

concept, but are a combination of

years of experience, intelligence and

resourcing. I think I’ll have a

breakdown if I continue to talk about

this!

Thankfully, we have AADDM, which

is small in numbers, but is an

innovative organisation of

professionals using their attitudes and

knowledge to pull in the direction of

the best health for those with complex

disabilities. We welcome our president

elect, Dr Jaqueline Small. We thank

Dr Nick Lennox, who retired from the

presidency after many years. Nick is

thanked for his research, leadership,

meaningful collaborations, and

countless hours of volunteer work.

Nick continues on the executive

committee; in that context, he will

continue to provide invaluable input.

As a token thank you, he was given a

very large jar of nougat by the

executive committee. Thanks Nick, for

everything.

The highlight of AADDM was

definitely the ‘It’s Alimentary

Conference’ on Thursday, 13th August,

in Sydney. The rest of this newsletter

will summarise some of the

presentations from the conference

(albeit without the passion and humour

of a live presenter).

The next newsletter will review the

Looking after the Waistline Positive

Cardiometabolic Health for People

with Intellectual Disability workshop,

Committee Members Nick Lennox Helen Leonard Margaret Kyrkou John Entwistle Bob Davis Seeta Durvasula Aline Smith Christel Burton Cathy Franklin Jenny Torr

President

Dr Jacki Small

Vice President

Prof Julian Trollor

Secretary:

Dr Jane Law

Treasurer:

Dr Jean Graham

Experiences of a consumer Joanne Argent, Ian and Brodie

2

A smattering of conference photos All :)

Obesity Epidemiology Dr Shirley Alexander

3

Surgical approaches to obesity Prof Reg Lord

4

Gluten Intolerance/Malabsorption Dr Samantha Tilakaratne

5

Lower gastrointestinal disorders Professor Martin Weltman

6

Epidemiology of gastrointestinal disorders in people with intellec-tual disability Professor Nick Lennox

8

Inside this issue:

Editorial

AADDM Newsletter Issue 24 November 2015

which occurred after the AADDM

conference.

Thank you for reading this

newsletter and being part of

AADDM.


Presentation: Experiences of a mother of a child with intellectual disability and gas-trointestinal problems Presenter: Joanne Argent, Ian and Brodie Joanne Argent has given us food for thought. Her daughter, Brodie, has Cornelia de Lange syndrome with severe small stature, severe ID, cannot walk, has gastroesophageal reflux disease, a range of feeding issues and a histo-ry of caecal volvulus. However, given all of this, Brodie is very rarely ill. Joanne spoke about what she has learned from her experi-ences of the hospital system; her observa-tions may be applicable to any small adult with ID: 1 Patients need a coordinator of medical care, who can often be a ‘Godsend.’ An overseeing paediatrician is essential, and the lack of an equivalent in the adult world is a huge gap. 2 If there is an adult patient who presents as a child, carefully choose the hospital into which he or she will be admitted. First prefer-ence is a hospital with co-located adult and paediatric services.

3 No one wants to be in hospital with a child who is unwell, one who groans and makes strange noises day and night. Such a child is generally not accepted in an adult ward. Without being glib or elitist, Joanne explained that what makes hospital bearable is a pri-vate room because, ‘no matter what happens, it is better in a private room. ‘ 4 For day surgery procedures, these children need to be given priority, and should be placed at the top of the surgery list to prevent a long period of fasting during waking hours. Fasting for a person who does not under-stand is much easier if they are asleep. Joanne spoke of ‘parental shortcomings’, and explained that parents are imperfect. When they deal with the same issues for a very long time they start to think that these health is-sues are normal. Good medical advice can be ignored. Joanne implored us to, ‘please perse-vere with parents.’


Presentation: Obesity Epidemiology Presenter: Dr Shirley Alexander, The Children’s Hospital at Westmead The underlying principal of management of obesity, particularly in children with disabilities, is to optimise health. If health is being affected by weight, then weight must be addressed. Globally, one in ten chil-dren is overweight or obese. There is a higher preva-lence in those from socially disadvantaged back-grounds and those with developmental disabilities. In Australia, one in five pre-schoolers are overweight (one in three if indigenous or Asian). Paper on London Special Olympics International Health Promotion database: Healthy athletes were screened at events in the past 10 years and in over 100 countries, with data accumu-lated on almost 10,000 children (8-19 years), includ-ing weight and height. In this cohort:

· On average, 30% are overweight/obese · In North America, the prevalence of over-

weight/obesity in children is almost 50% · Africa and the middle east are going through a

‘nutritional transition’ · Obesity in children with disabilities is a global

phenomenon · One limit to this study is that it did not take

note of the various disabilities that the chil-dren had

Pitfalls in measuring obesity in those with disabili-ties:

· Problems with measurement: inability to

stand, scoliosis, contractures

· Alternatives include arm span and skin fold

thickness

· Total body fat % underestimated, higher % fat

and low lean tissue

· Reduced linear growth but same weight gain

Predisposing factors of obesity include, low levels of physical activity (multiple barriers: no adapted equip-ment in mainstream schools, staff issues, etc.), and increased sedentary behaviour (videogames, TV watching, etc.). On the nutrition side: atypical feeding behaviour, texture, colour, taste challenges, hyperpha-gia, food used to reinforce good behaviour, financial burdens , reduced ability to participate in the work-force. Health complications of obesity:

· Affects every organ of the body

· Psychosocial: Teasing

· Pulmonary: OSA, asthma, exercise intolerance

· GIT: Gallstones, GORD, NSALD

· Renal: Glomerulosclerosis

· MSS: Ankle sprains, flat feet, tibia vara, SCFE,

forearm fracture

· Neurological: pseudotumour cerebri

· CVS: Hypertension, dyslipidaemia, coagulopa-

thy , chronic inflammation, endothelial cell

dysfunction

Management ‘Super 7’ (principles that help reduce weight):

1) Drink water as the main drink

2) Eat breakfast every day

3) Eat as a family once a day with the TV off

4) Be physically active for one hour per day

5) Limit screen time to less than two hours per

day

6) Get plenty of sleep and develop a good sleep

regimen

7) Weigh once a week and record in a notebook

Notes:

· Start early, and get the parents involved.

· It is essential that patients are measured and

charted. There are different charts for differ-

ent disabilities, e.g., for Down syndrome ,

achondroplasia, Prader-Willi syndrome, etc.

· Waist to height ratio should be less than 0 .5

to reduce metabolic health risks.

· Parents have many priorities, such as dealing

with medical complications, behaviour con-

trol, finances, time constraints, and other fam-

ily stresses, so weight management may be

low on their priority list. When dealing with

parents, it is crucial to be solution focused and

supportive.

COMING SOON: E Learning: ‘paediatric obesity’ to Health Education and Training Institute (HETI) website.


Presentation: Surgical approaches to obesity Presenter: Prof Reg Lord, upper gastrointestinal surgeon Common Eating Disorders and Issues

· Fussy/ Picky Eaters

· Overeaters

· Addictions- sugary foods/ cola

· Pica

Surgical Options

· Sleeve Gastrectomy

· Band

· Roux-en-Y Gastric bypass

· Sleave (complications related to the bas-

ket apparatus in the duodenum) which is

subsequently removed

Interventions

· Treatment is multimodal

· CBT for fear and anxiety

· Reinforcement programs

· Task analysing where breakdowns are

occurring

· Gradual exposure program and skill de-

velopment

Overeating/food addiction, contributed by:

· Medical factors

· Social and environmental factors: bore-

dom, opportunity, and availability of

healthy foods

· Poor education on nutrition

· Psychological factors as a sign of depres-

sion

· As part of routine

Interventions

· Education

· Opportunity to eat healthy and engage in

a healthy lifestyle

· Routine changes

· Role modelling and healthy choices

· Behaviour therapy

· Possible restrictions: Restricting access to

food or monies


Presentation: Gluten Intolerance/Malabsorption Presenter: Dr Samantha Tilakaratne, Paediatric Gastroenterologist Epidemiology Initially thought to mainly affect people of Euro-pean origin, but now seen in countries all over the world. Similar prevalence is seen in healthy populations in Europe and countries where Euro-peans migrate: UK 1%; US 0.9%; NZ 1.1%. Clinical features

· Diarrhoea/steatorrhoea

· Nausea/vomiting

· Constipation

· Abdominal pain

· Weight loss

· Short stature/delayed puberty

· Recurrent mouth ulcers

· Alopecia

· Rash

· Iron deficiency anaemia

· Folate deficiency

· Vitamin D deficiency

· Abnormal liver function

· Fractures fatigue

· Dermatitis herpetiforms (pruritic vesicu-

lar rash)

Family history Strong evidence of a genetic component: First degree relatives are at increased risk (approx. 10%). MZ twins 80%-86% vs DZ 10-11%. Associated features TYDM, thyroid disease, autoimmune liver disease, trisomy 21, Turner syndrome, William’s Syn-drome. Genetics

· Human Leukocyte Antigen (HLA) is

strongest genetic determinant of CD risk

· HLA genes are involved in regulation of

immune processes

· HLA DQ2 or HLA DQ8 positive in CD pa-

tients

Screening tests · Anti gliadin IgA and G (AGA)

· Endomysial IgA Ab ( EMA)

· Tissue –transglutaminase IgA ( tTG)

· Total IgA (Deficient in 1:50 CD)

· HLA DQ2 and DQ8 30% general popula-

tion positive

Changes in diagnostic criteria British/European guidelines have suggested a change in screening tests and diagnostic criteria (BSPGHAN): Use HLA testing in addition to serol-ogy to guide the need for biopsy. Treatment Most children on GF diet have a resolution of symptoms, and achieve full growth potential. They can develop constipation, and it can take time for serology to normalise. Why treat? Untreated CD can lead to poor growth, nutrient deficiencies, chronic inflammation, impaired fer-tility, osteopenia and osteoporpisis, and SB malig-nancy (long term). Gluten-free diet There are many anecdotal reports of improve-ment of ASD children on GF diets. Systematic re-view failed to find increased incidence of CD and ASD. A Danish study conducted over 24 months found reduced ADHD and autistic behaviours sus-tained beyond 12 months. Longer reviews did not replicate the findings.


Presentation: Lower gastrointestinal disor-ders Presenter: Professor Martin Weltman, Gastroenter-ologist and Hepatologist Constipation is defined by the Rome 3 Criteria (2006). For constipation to be defined as ‘chronic’, a patient must be symptomatic for 6 months. ‘They are the people who are wishing for diarrhoea’. Several studies show that, in those children with chronic constipation, there are diffi-culties in behaviour. The trio of insufficient dietary fibre, fluids and exercise has been widely ascribed in magazines and newspapers, but, in fact, the evidence behind these factors is inconsistent and of low to medi-um quality. Sometimes we can be too obsessive about all of this. With chronic constipation, there are intrinsic fac-tors; sometimes they are difficult to understand and can be a bit boring. There are 2 categories: pelvic floor dysfunction (PFD) and slow colon transit time. A retrospective study, based on physiologic tests, reported the prevalence of PFD as 37% and slow colon transit time as 23%. How-ever, a clear distinction between the two is often impossible, with an overlap of up to 55%. PFD involves laxity of the pelvic floor muscles, im-paired rectal sensation and decreased luminal pressure in the anal canal. Slow transit time is a poorly understood condition thought to be a cause of intractable constipation in children and young women. It is characterised by reduced high- amplitude propagated contractions in the colon, a very uncoordinated bowel leading to slow transit of faeces, bloating abdominal dis-comfort and infrequent defecation. This is self-perpetuating. Because of the increased transit time, the faeces are small and hard and, because of poor rectal stretch, fail to trigger the rectal re-flex. Other than feeling uncomfortable and generally terrible, there are other complications of chronic constipation. For children, it is overflow faecal incontinence, caused by fresh faecal matter by-passing the obstruction So to what extent do we investigate? This is aged-determined, and there is no absolute fixed struc-

ture; currently, it is geared to those children who are getting faecal incontinence at school and not toilet trained by 5. The management of chronic constipation: We live in a day of evidence-based medicine. While there is no substantial evidence to sup-port lifestyle modification, such as adding fibre, water and exercise, we recommend them never-theless. Thereafter, it is important to ‘ensure that the patient optimises the opportunities for adequate defecation’, which is a generalised statement. This begins with ensuring that any spontaneous urge to defecate is not being ig-nored due to environmental stressors. For the management of the PFD type of consti-pation, biofeedback provides retraining of the anorectum, which involves a nurse, a technician and a psychologist. This technique aims to stop the paradoxical contractions of the anus/rectum during defection. Notes should be tak-en, and the patient needs to be very compliant. If we look at pharmacological management we have many medications but none that are very specific: Bulk forming agents are insoluble fibre; there are many of these available—methylcellulose bran, psyllium. It is very important when in-gesting bulking agents to ensure an adequate water intake; otherwise, the constipation and bloating can become worse. There have been many randomised controlled trials about psylli-um and it has been shown to have efficacy in at least 3 RCT in treating constipation. Bulk form-ing agents are still used in first-line therapy with many children. Mineral oils and emollients are used for lubri-cating effect on stools. There are no randomised controlled trials about this and you have to be very careful in children because of the docu-mented risk of aspiration pneumonia. Similarly, ducosate sodium is prescribed for a detergent effect but there is no confirming evidence as to its efficacy. Osmotic agents, e.g., lactulose/sorbitol, absorb and retain moisture, causing an increase in stool softness. An osmotic can be used when bulk forming agents fail.


Stimulants act on the enteric plexus of the bowel to increase contraction. There is no evidence base for the use of senna, although it has been around for 100 years. Probiotics seem like they are good things and have been found in low levels in patients with chronic constipation, but there is no RCT for this. Enemas and suppositories are only going to get rid of the stool that is in the rectum. If the patient is impacted all the way through the colon, some-thing must be given that is going to loosen the upper obstruction. Surgery is used in desperate situations. There are children who get a colectomy to bypass the colon. Often these children have diffuse motility disorders throughout the GIT. What often hap-pens is that, over time, the small bowel also be-comes slow, and, 10 years after surgery, consti-pation returns.


Presentation: Gastrointestinal Disorders : Epidemi-ology of gastrointestinal disorders in people with intellectual disability Presenter: Prof Nick Lennox, AADDM President 2014, Director Queensland Centre for Intellectual and Developmental Disability Acknowledgment to Lyn McPherson, research head UQ, for putting together the presentation. Diseases of the digestive system as a primary cause of death are 2.5 times more common than in the general population. ORAL HEALTH

· Increased prevalence of periodontal disease

· Increased rates of untreated caries

· Decreased prevalence of filled teeth and

increased caries and missing teeth

· 2 groups most at risk of not being able to

access dental services are those with Down

syndrome and those unable to cooperate

Anders PL and EL Davis (2010) oral health of pa-tients with intellectual disabilities: a systemic re-view. Special care in dentistry.40% required some for of behavioural assistance to receive dental treatment (Moran 2012) DYSPHAGIA Nick is involved in the mortality review committee. One of the outstanding features is the high level of dysphagia. Over 50% of those 50+ have dysphagia. Many cases are seen in the mortality review com-mittee, and people are dying unnecessarily. GORD Prevalence in the Netherlands approx. 50% of insti-tutionalised people had GORD. Prevalence figures all have limitations in this popu-lation and generally we underestimate the preva-lence as it misses out those with mild disease.

Is associated with Cerebral palsy and intellectual disabilities Hermans and Even huis (2014) 20% diagnosed used medical files of 1047 of 1047 adults 50+ Charot et al (2011) Second most common problem in non psychiatric health problems in psychiatric patients with ID H PYLORI Twice the rate of the general populations, Transmission is via faeces, vomit, saliva: Recurrence after triple drug treatment at a rate of nearly 7 times that of the general population Breath test is difficult in this population. GIT CARCINOMA 3 X increased risk of GIT neoplasms among people with ID Patja in Finland (2001) Does not confirm this excess risk but do suggest a moderate excess in some can-cer types such as gall bladder, oesophagus and un-defined GI cancers- Dr Lennox trusts this study – it is probably the largest sample as it followed 3000 people over 35 years. COELIAC DISASE One thing that we can do something about! We should be screening people who have Down syndrome. It can explain irritability and restless-ness. In autism the there an interest in gut disorders, in large studies there is no conclusive evidence be-tween coeliac disease and autism. There is a marked increase in serology positive and biopsy negative in patients with autism ALudvigsson JF (2013) A nationwide study of the association between celiac disease and the risk of autistic spectrum disorders. JAMA psychiatry CONSTIPATION Constipation demonstrated in almost 70% of insti-tutionalised population with ID Correlates with non ambulancy CP, medications (major tranquilisers) ,food refusal and IQ <35 Similar prevalence in adults and children suggesting it does not develop over the lifespan Bohmer (2001)


Next Newsletter : Feb 2016

Speech pathology approach to dysphagia:

Ms Tanya Govey

Gastroesophageal reflux:

Dr Reuben Jackson

Cardiometabolic health

Rett Syndrome

ENCOPARESIS 20% of patients still have it at age 20 80% of children attained bladder control OBESITY Adults with ID have a prevalence rate for obesity of 38% cf the general population of 28% Maiano (2011) THE LINK BETWEEN GIT PROBLEMS AND BEHAV-IOURS OF CONCERNS: GIT and behaviour charts show that there are parallels between gut issues and behaviour. Its alimentary and we have a problem!! HAVE A WONDERFUL CHRISTMAS.

aaddm newsletter · 2016. 7. 11. · aaddm newsletter issue 24 november 2015 page 2 presentation:...

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