Diet in Neuromuscular
Disorders
Rachel Mochrie
Neuromuscular Paediatric
Dietitian RHSC
Common Symptoms in NM
Diseases
Reduced or increased muscle tone
Progressive muscle weakness worsening
with activity
Spasms
Myalgia
And in conditions such as DMD eventual
loss of ambulation
Swallowing and respiratory problems
Main Aims of Management
Improvement of symptoms
Prevention and treatment of complications
such as Spinal deformity, respiratory
complications and cardiomyopathy
Minimise the impact of complications to
maximise quality of life
Nutritional Assessment
Weight
Problems may occur if
there is no appropriate
equipment available e.g. sit
on scales, wheelchair
beams e.t.c.
Weight should be
measured at each clinic
visit or more frequently if
any concerns
Assessment - Height/Length
Wherever possible a standing height or
supine length should be taken.
It is often difficult to obtain a height or
length for children
This can be due to the child’s leg
contractions and / or inability to stand or
lie straight.
Alternative Height Measurements
In Neuromuscular disorders -Ulna length –
Specialist Anthropometer
Arm span
Assessment – Dietary Intake
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24hr recall
Diet History
Recorded intake
Food diary
Observation
Barriers to an adequate
intake/nutritional status
Increased work of breathing, respiratory
failure, gastric dysmotility, constipation/
diarrhoea
Post surgery e.g. Spinal
Psychological aspects- aversion/ inability
to self feed/ reluctance for assistance
Chewing /swallowing difficulties – joint
review with Speech Therapist
Advice
Establishing regular meals + appropriate
eating pattern
Encouraging small, frequent meals and
nutritious snacks
Advice on appropriate food textures/
thickening agents
Practicalities of eating –manage
seating/wheelchairs/arm rests
Food Fortification
 Optimise intake
 Behavioral modification
 Dairy products
 Fats + Oils
Supplements
 0-1yr
High Energy infant formula
 1-3yrs (8kg)
Paediatric sip feeds +/- Fibre
 >3yrs
Paediatric sip feeds +/- Fibre
Adult sip feeds milk/juice based +/- Fibre
Supplements
 Useful to improve/maintain calorie intake
in some children
 Important not to displace food
 Monitor use to ensureSupplements are being taken
Oral intake is not compromised
 Discontinue as soon as possible
Tube feeding- Indications
Inability to suck or swallow
Anorexia associated with chronic illness
Increased requirements
Congenital anomalies
Primary disease management
Clinical Paediatric Dietetics 3rd Edition
Tube Feeding – Gastrostomy
Support nutritional intake
Early decisions regarding placement may prevent further deterioration, reverse
faltering growth and reduce stress and
anxiety around eating with the family
Weight Management
Excessive weight gain is also common
Early intervention important
Appropriate portion control
Reduction of high fat/ high sugar foods and
drinks
Increasing fruit and vegetable intake
Use of Steroids e.g. In DMD can lead to rapid
weight gain and an increased appetite
Bone Health
Increased risk of bone disease
Due to lower activity levels
Use of long term corticosteroids (DMD)
Low vitamin D levels- limited sun exposure
Dietary sources of vitamin D – fortified
margarines/fortified cereals/oily fish
Vitamin D status monitored closely
Vitamin D Status
Serum 25-OHD
concentration
Vitamin D Status
<15nmol/l
15-30nmol/l
31-50nmol/l
>50nmol/l
Severe Deficiency
Deficiency
Insufficient
Adequate
Bone Health
Insufficiency and deficiency treated with
oral Calciferol
Early Intervention and prevention is key
In RHSC we advise optimal dose of
Vitamin D 800 iu
Routine use of Bisphosphonates in
children with NM conditions?
A Team Approach to optimising
Nutrition
The child should be assessed
by a multidisciplinary team,
e.g. Dr, Physiotherapist,
SALT, Psychologist and a
Dietitian.
References
Miller F, Koreska J(1992). Height measurement of
patients with neuromuscular disease and
contractures.Developmantal Medicine and Child
Neurology;34:55-60
Ramelli,et al (2007).Gastrostomy placement in paediatric
patients with neuromuscular disorders: indications and
outcomes. Developmental Medicine and Child
Neurology;49:367-371
Davidson ZE, Truby H (2009).A review of nutrition in
Duchenne muscular dystrophy. Journal of Human
Nutrition and Dietetics;22 :389-393
Bushby K (2005).The Multidisciplinary management of
Duchenne muscular dystrophy. Current
Paediatrics;15:292-300
Pearce SHS, Cheetham TD(2010).Diagnosis and
management of vitamin D deficiency. British Medical
Journal;340:142-147
Questions?