Diet in Neuromuscular Diet in Neuromuscular DisordersDisorders

Rachel MochrieRachel Mochrie

Neuromuscular Paediatric Neuromuscular Paediatric Dietitian RHSCDietitian RHSC

Common Symptoms in NM Common Symptoms in NM DiseasesDiseases

Reduced or increased muscle toneReduced or increased muscle tone

Progressive muscle weakness worsening Progressive muscle weakness worsening with activitywith activity

SpasmsSpasms

MyalgiaMyalgia

And in conditions such as DMD eventual And in conditions such as DMD eventual loss of ambulationloss of ambulation

Swallowing and respiratory problemsSwallowing and respiratory problems

Main Aims of ManagementMain Aims of Management

Improvement of symptomsImprovement of symptoms

Prevention and treatment of complications Prevention and treatment of complications such as Spinal deformity, respiratory such as Spinal deformity, respiratory complications and cardiomyopathycomplications and cardiomyopathy

Minimise the impact of complications to Minimise the impact of complications to maximise quality of lifemaximise quality of life

Nutritional Assessment Nutritional Assessment WeightWeight

Problems may occur if Problems may occur if there is no appropriate there is no appropriate equipment available e.g. sit equipment available e.g. sit on scales, wheelchair on scales, wheelchair beams e.t.c.beams e.t.c.Weight should be Weight should be measured at each clinic measured at each clinic visit or more frequently if visit or more frequently if any concernsany concerns

Assessment - Height/LengthAssessment - Height/Length

Wherever possible a standing height or Wherever possible a standing height or supine length should be taken.supine length should be taken.

It is often difficult to obtain a height or It is often difficult to obtain a height or length for children length for children

This can be due to the child’s leg This can be due to the child’s leg contractions and / or inability to stand or lie contractions and / or inability to stand or lie straight. straight.

Alternative Height MeasurementsAlternative Height Measurements

In Neuromuscular disorders -Ulna length – In Neuromuscular disorders -Ulna length – Specialist AnthropometerSpecialist Anthropometer

Arm span Arm span

Assessment – Dietary IntakeAssessment – Dietary Intake

24hr recall24hr recall Diet HistoryDiet History Recorded intakeRecorded intake Food diaryFood diary ObservationObservation

Barriers to an adequate Barriers to an adequate intake/nutritional statusintake/nutritional status

Increased work of breathing, respiratory Increased work of breathing, respiratory failure, gastric dysmotility, constipation/ failure, gastric dysmotility, constipation/ diarrhoea diarrhoea

Post surgery e.g. SpinalPost surgery e.g. Spinal

Psychological aspects- aversion/ inability Psychological aspects- aversion/ inability to self feed/ reluctance for assistanceto self feed/ reluctance for assistance

Chewing /swallowing difficulties – joint Chewing /swallowing difficulties – joint review with Speech Therapistreview with Speech Therapist

Advice Advice

Establishing regular meals + appropriate Establishing regular meals + appropriate eating patterneating pattern

Encouraging small, frequent meals and Encouraging small, frequent meals and nutritious snacksnutritious snacks

Advice on appropriate food textures/ Advice on appropriate food textures/ thickening agents thickening agents

Practicalities of eating –manage Practicalities of eating –manage seating/wheelchairs/arm restsseating/wheelchairs/arm rests

Food FortificationFood Fortification

Optimise intake Optimise intake

Behavioral modificationBehavioral modification

Dairy productsDairy products

Fats + OilsFats + Oils

SupplementsSupplements

0-1yr 0-1yr High Energy infant formula High Energy infant formula

1-3yrs (8kg)1-3yrs (8kg) Paediatric sip feeds +/- FibrePaediatric sip feeds +/- Fibre

>3yrs>3yrs Paediatric sip feeds +/- Fibre Paediatric sip feeds +/- Fibre Adult sip feeds milk/juice based +/- FibreAdult sip feeds milk/juice based +/- Fibre

SupplementsSupplements

Useful to improve/maintain calorie intake Useful to improve/maintain calorie intake in some childrenin some children

Important not to displace foodImportant not to displace food

Monitor use to ensure-Monitor use to ensure-

Supplements are being takenSupplements are being taken

Oral intake is not compromisedOral intake is not compromised

Discontinue as soon as possibleDiscontinue as soon as possible

Tube feeding- IndicationsTube feeding- Indications

Inability to suck or swallowInability to suck or swallow

Anorexia associated with chronic illnessAnorexia associated with chronic illness

Increased requirementsIncreased requirements

Congenital anomaliesCongenital anomalies

Primary disease managementPrimary disease management

Clinical Paediatric Dietetics 3rd EditionClinical Paediatric Dietetics 3rd Edition

Tube Feeding – GastrostomyTube Feeding – Gastrostomy

Support nutritional intakeSupport nutritional intake

Early decisions regarding placement may -Early decisions regarding placement may -prevent further deterioration, reverse prevent further deterioration, reverse faltering growth and reduce stress and faltering growth and reduce stress and anxiety around eating with the familyanxiety around eating with the family

Weight ManagementWeight Management

Excessive weight gain is also commonExcessive weight gain is also commonEarly intervention importantEarly intervention important

Appropriate portion controlAppropriate portion controlReduction of high fat/ high sugar foods Reduction of high fat/ high sugar foods

and and drinksdrinksIncreasing fruit and vegetable intakeIncreasing fruit and vegetable intake

Use of Steroids e.g. In DMD can lead to rapid Use of Steroids e.g. In DMD can lead to rapid weight gain and an increased appetiteweight gain and an increased appetite

Bone HealthBone Health

Increased risk of bone diseaseIncreased risk of bone disease

Due to lower activity levels Due to lower activity levels

Use of long term corticosteroids (DMD) Use of long term corticosteroids (DMD)

Low vitamin D levels- limited sun exposureLow vitamin D levels- limited sun exposure

Dietary sources of vitamin D – fortified Dietary sources of vitamin D – fortified margarines/fortified cereals/oily fishmargarines/fortified cereals/oily fish

Vitamin D status monitored closely Vitamin D status monitored closely

Vitamin D StatusVitamin D Status

Serum 25-OHD Serum 25-OHD concentrationconcentration

<15nmol/l<15nmol/l

15-30nmol/l15-30nmol/l

31-50nmol/l31-50nmol/l

>50nmol/l>50nmol/l

Vitamin D StatusVitamin D Status

Severe DeficiencySevere Deficiency

DeficiencyDeficiency

InsufficientInsufficient

AdequateAdequate

Bone Health Bone Health

Insufficiency and deficiency treated with Insufficiency and deficiency treated with oral Calciferoloral Calciferol

Early Intervention and prevention is keyEarly Intervention and prevention is key

In RHSC we advise optimal dose of In RHSC we advise optimal dose of Vitamin D 800 iuVitamin D 800 iu

Routine use of Bisphosphonates in Routine use of Bisphosphonates in children with NM conditions?children with NM conditions?

A Team Approach to optimising A Team Approach to optimising NutritionNutrition

The child should be assessed The child should be assessed by a multidisciplinary team, by a multidisciplinary team, e.g. Dr, Physiotherapist, e.g. Dr, Physiotherapist, SALT, Psychologist and a SALT, Psychologist and a Dietitian.Dietitian.

ReferencesReferencesMiller F, Koreska J(1992). Height measurement of patients Miller F, Koreska J(1992). Height measurement of patients with neuromuscular disease and contractures.Developmantal with neuromuscular disease and contractures.Developmantal Medicine and Child Neurology;34:55-60Medicine and Child Neurology;34:55-60Ramelli,et al (2007).Gastrostomy placement in paediatric Ramelli,et al (2007).Gastrostomy placement in paediatric patients with neuromuscular disorders: indications and patients with neuromuscular disorders: indications and outcomes. Developmental Medicine and Child outcomes. Developmental Medicine and Child Neurology;49:367-371Neurology;49:367-371Davidson ZE, Truby H (2009).A review of nutrition in Davidson ZE, Truby H (2009).A review of nutrition in Duchenne muscular dystrophy. Journal of Human Nutrition Duchenne muscular dystrophy. Journal of Human Nutrition and Dietetics;22 :389-393and Dietetics;22 :389-393Bushby K (2005).The Multidisciplinary management of Bushby K (2005).The Multidisciplinary management of Duchenne muscular dystrophy. Current Paediatrics;15:292-Duchenne muscular dystrophy. Current Paediatrics;15:292-300300Pearce SHS, Cheetham TD(2010).Diagnosis and Pearce SHS, Cheetham TD(2010).Diagnosis and management of vitamin D deficiency. British Medical management of vitamin D deficiency. British Medical Journal;340:142-147Journal;340:142-147

Questions?Questions?