Angela ReddySenior Specialist DietitianLane Fox Respiratory Unit

St Thomas’ HospitalGuys and St Thomas’ Foundation Trust

Action Duchenne Conference 2015

Nutrition and Dietary Advice

Lane Fox Respiratory Unit Specialists in Chronic Respiratory Care Est. 1989

Varied: both sides of the nutritional spectrum

> 14/5 yrs

>5 yrsloss ambulation and steroids

20 yrs onwards

1 yr

Weight increasing dietary advice

Healthy-eating Dietary advice

Tracey Davis, Specialist Dietitian, GOSH

• Nutrition is a critical part of long term DMD management

• Both malnutrition and obesity are harmful to respiratory function

• Overweight/Overweight/obesity (obesity (BMI of more than 25kg/m2)

Impedes breathing and increases the work of the respiratory muscles/Spinal jackets become too tight/Decreased mobility/Increased difficulty for carers to lift

Introduction

• MalnutritionMalnutrition is acknowledged as a predictor of adverse outcomes in patients with neuromuscular diseases (BTS, 2002)

• Malnutrition is a key feature of chronic respiratory disease

• It adversely affects respiratory muscles, reducing muscle mass and strength

• It additionally affects immune function, wound healing, sensitivity to oxygen prolonging ventilator weaning and psychosocial function

• Poor nutritional statusPoor nutritional status is associated with non invasive ventilation (NIV) failure.

Introduction

Child with DMD

>5 yrsloss ambulation and steroids

1 yr

Healthy-eating Dietary advice

Nutrition advice

• Weight increase tends to coincide with • Loss of ambulation• Steroid therapy (effects on appetite)• Around 8 - 12 years of age

• What causes one to become overweight?• Energy balance: Energy in = energy out• Increased appetite (steroids)• Reduced mobility

• Always difficult to lose weight ESPECIALLY if mobility affected

• Therefore, prevention better than cure!

Overweight in DMD

Tracey Davis, Specialist Dietitian, GOSH

• Parents/carers/family can helpParents/carers/family can help• Can become pro-active in preventing excessive weight Can become pro-active in preventing excessive weight

gaingain

• How? How? • By following a By following a ““healthy eatinghealthy eating”” diet diet

Overweight in DMD

Tracey Davis, Specialist Dietitian, GOSH

Traditional Meal

Healthy ‘Balanced’ Meal

Weight Reducing Meal

Unhealthy meal proportions

Changing the proportions of food in this way leads to a healthier meal

Changing the proportions in this way will reduce energy intake, help you lose weight, but allow you to eat the same volume of food

Meat &Alternatives StarchyFoods

Vegetables& Fruit

Meat &Alternatives StarchyFoods

Vegetables& Fruit

Meat &Alternatives StarchyFoods

Vegetables& Fruit

Healthy Eating

Tracey Davis, Specialist Dietitian, GOSH

Hidden fats

2 sausages, pork (fried) 4 fish fingers (grilled)

60g chocolate bar Crunchie bar

60g nuts (large handful) 60g raisins

High Fat Products Low Fat Alternatives

1 pat = 5g

Tracey Davis, Specialist Dietitian, GOSH

Hidden sugars 1 cube = 5g of sugar

Small bowl of Frosties Small bowl of All-Bran

1 slice Victoria Sponge 1 currant bun

8 oz tinned fruit in syrup 8 oz tinned fruit in juice

Tracey Davis, Specialist Dietitian, GOSH

Healthy Eating

• Low glycaemic index (GI) food

• Ranks how carbohydrate rich food affects blood levels

• Slowly absorbed food have a low GI rating.• Keeps you fuller for longer

Beans & LentilsFruit

Pasta & Noodles

Porridge, oats based cereals,All Bran and Sultana Bran

Yam/Sweet Potato

Wholegrain Bread(Bread with ‘bits’ i.e. Pumpernickel

or granary)

Basmati RiceBarley

Slowly absorbedstarchy food

Barley

Tracey Davis, Specialist Dietitian, GOSH

Adolescent/Adult with DMD

> 14/15 years

20 yrs onwards

1 yr

Weight increasing dietary advice

• Malnutrition or protein-energy malnutrition A condition of decreased body stores of protein and

energy (calorie) furl stores – i.e. lean body mass and fat mass

In developed countries disease is the principal cause

• Nutritional intake may be compromised due to the inflammation imposed by the chronic disease

• This loss of LBM associated with inflammation and functional impairment is considered disease related malnutrition – a significant problem in respiratory and neurological disease

Underweight or malnutrition

• Malnutrition common in DMD adults

• Protein energy malnutrition is classified: As a body mass index (BMI) of less than 18.5 kg/m2

Unintentional weight loss of greater than 10% A BMI of less than 20 kg/m2 and unintentional weight

loss of greater than 5% within the last 3-6 months (NICE, 2006)

• BMI of less than 20kg/m2-commonly used in clinical practice

• Malnutrition is common in hospitalised patients and associated with poor outcomes

Underweight or malnutrition

Effects of malnutrition

• Chewing fatigue

• Prolonged meal times accompanied by drooling and

spilling

• Dysphagia and aspiration

• Inadequate nutritional intake

• Weight loss as disease progresses

• Constipation

• Gastric and intestinal dilatation

• Gastro-oesophageal reflux

• Timely consideration of gastric tube feeding is necessary

Feeding and nutritional problems are common

• Nutritional needs or requirements (protein and energy) are increased

During illness and infection Surgery Increased work of breathing

• In clinics and on the ward, we screen patients (weight, weight loss, eating less than normal) to detect those with or at risk of malnutrition

• Early identification is essential to provide help and correct nutritional issues

Malnutrition risk

• Assessment of nutritional status provides information on severity and causes of malnutrition

Low body weight/reduced total fat/decreased

muscle mass

Anthropometry

% Weight loss

Low energy or protein intakes

Biochemical

Clinical conditions

Assessment of nutritional status

What can we do?

• Prevent weight loss/aid weight gain via via manipulation of manipulation of the dietthe diet

Increase Increase frequency frequency of consumption of nutrient-dense of consumption of nutrient-dense snacks snacks Plus encourage milky drinks/condensed soups Plus encourage milky drinks/condensed soups Try 3 meals plus 2-3 snacksTry 3 meals plus 2-3 snacks

Increase Increase nutrient density nutrient density of foodsof foods• Fortifying foodsFortifying foods• Adding nutrients e.g., fats/add protein to food Adding nutrients e.g., fats/add protein to food • Add multivitamins such as Forceval solubleAdd multivitamins such as Forceval soluble• Avoid low calorie drinks e.g., tea/coffeeAvoid low calorie drinks e.g., tea/coffee

• Prevent weight loss/aid weight gain via via nutritional nutritional supplementssupplements Not as a meal replacement, but in addition to normal Not as a meal replacement, but in addition to normal

intakeintake

• High calorie high protein supplementsHigh calorie high protein supplements Fortisip Compact Protein has 300kcal and 18g protein in Fortisip Compact Protein has 300kcal and 18g protein in

125ml (60ml x 2 shots)125ml (60ml x 2 shots) Fresubin 2kcal has 400kcal, 20 g protein in 200mlFresubin 2kcal has 400kcal, 20 g protein in 200ml Scandishake has 600kcal, 12 g protein (250ml) – can Scandishake has 600kcal, 12 g protein (250ml) – can

add ice cream, strawberries and liquidiseadd ice cream, strawberries and liquidise High calorie drinks – juice based in 200mlHigh calorie drinks – juice based in 200ml High calorie fat supplements such as Calogen, Procal in High calorie fat supplements such as Calogen, Procal in

30ml shots x 3 per day30ml shots x 3 per day Use supplements in recipes Use supplements in recipes

• Regular preventative nutritional therapy recommended

• Prevent weight loss/aid weight gain via via enteral nutritionenteral nutrition

Short or long term Short or long term

Via Nasogastric tube or gastrostomy Via Nasogastric tube or gastrostomy

Might aim to meet 100% of requirements in initial stage Might aim to meet 100% of requirements in initial stage of feeding of feeding Transition to oral feeding in short term enteral Transition to oral feeding in short term enteral

feedingfeeding

LaxativesLaxatives

Prokinetic agentsProkinetic agents

• Admission and weekly nutritional screening

• Weights: weekly on the ward or in clinic

• Intake - if adequate nutritional intake on food charts

• Bowels!!

• Fluids

• Vomiting

• Nutritional requirements

• Fatigue

• Meal timing

• NG and gastrostomy care

• Daily MDT

Monitor! Monitor! Monitor!

Gastrostomy or PEG feeding

• It is a feeding tube inserted through the stomach wall into the abdomen for the purpose of nutrition support

• Can be done using an endoscope, and is referred to as a percutaneous endoscopic gastrostomy (PEG)

• A PEG might be required due to the common nutritional problems mentioned above

• It is there to support normal eating and not to replace it• People report feeling very excited

– that they can eat what they enjoy at mealtimes and at their own pace– Eating and gaining weight

• It is frequently used to take medications and additional water• Feed regimens are varied and is designed to suit the individual• Some people prefer overnight feeding whilst some prefer

daytime or bolus feeding or combination of both• Different types of gastrostomy tubes such as, low profile devices

sit flush with the stomach and is not noticeable under the clothes• All discreet

When do we insert gastrostomy feeding tubes?

Retrospective Study of Nutritional Status and Outcomes in DMD with

Chronic Respiratory Failure

To assess the nutritional risk status of adult patients with DMD and chronic respiratory failure and to investigate its association with hospital outcomes over a 12-month period

• Retrospective case series study using hospital records

• Nutritional parameters: weight body mass index (BMI) feeding route (enteral or oral)

• Hospital outcome measures: cumulative length of hospital stay (LOS) ventilator adherence (hours) frequency of admission forced vital capacity (FVC) mortality

Methods

RESULTS

•30/79 (38%) were oral fed and 24/79 (30%) were PEG fed

Enteral tube fed patients (n=30) had a greater admission risk compared to oral (n=49) fed patients

• Weight in patients admitted to hospital was less than those non-admitted patients

• Admission risk was greater in the underweight (BMI<20kg/m2) or overweight (BMI>25kg/m2) patient groups compared to normal BMI patients

• There was an association between weight and feeding method:

- patients with higher weights were associated with decreased risk of enteral feeding

- Enteral feeding was associated with an increased non-invasive ventilator use per day and LOS

Conclusion• Malnutrition is a potentially neglected clinical area in this cohort of

patients with incomplete recording of nutritional data• Weight was associated with adverse outcomes in DMD

– Overweight and underweight patients were more likely to be admitted than those with a normal body habitus

– Patients who are enterally fed had a greater admission risk, infective episodes and LOS than orally fed patients, indicative of disease severity

• Future prospective research to assess nutritional status and hospital outcomes is recommended

• Regular nutritional monitoring and intervention may improve patient outcome

Where do we insert gastrostomy feeding tubes?

Lane Fox Approach

• Currently– Assessed in outpatients by doctor, transitional NMD specialist,

nurse and dietitian– Weighed at each visit (in wheelchair)– Focus on feeding issues and malnutrition – Assessed as inpatient only by SLT– PEG tubes inserted on the unit by Lane Fox Gastrostomy

Insertion Team (Lane Fox Consultant, Gastroenterology Consultant, Anesthetic Consultant)

– 4 insertions per month

• New– Dietitian part of outpatient assessment team– To screen and identify those requiring aggressive nutritional

support– Develop care pathway for nutritional support in DMD

• Enteral feeding is initiated when adequate oral nutritional cannot be safely accomplished

• There is a need for early assessment for gastrostomy insertion and guidelines for insertion in adult DMD

• Early insertion of feeding tubes should be considered as this may potentially reduce the risks associated with enteral feeding

• Further studies are required to establish the optimum time to initiate enteral feeding

Gastrostomy Feeding - When and Where?

Are there guidelines to help?

• Action Duchenne• Treat NMD• Muscular Dystrophy Campaign• Some guidelines for DMD, however

– Adequate nutritional status described as weight to age ratio or BMI for age from the 10th to the 85th on national percentile charts

– Gastrostomy is recommended when weight and hydration can no longer be met by oral means

– Managing complications in adults is acknowledged but recommendations are centred on children (Bushby et al, 2010)

– Nutrition is highlighted as a critical aspect of long-term DMD care– Emphasises lack of reliable evidence exploring malnutrition and nutritional

assessment in adults– Lack of nutritional consensus and practice guidelines is highlighted– Limited evidence examining gastrostomy feeding (American Thoracic Society,

2004)

Constipation

• Increase sources of fibre, which– Alleviates constipation– Has a bulking action– Holds water – increasing stool weight– Facilitates bowel regularity

• Sources - wholegrain foods such as oats, seeds (linseeds/flaxseeds), potato skins, lentils, pulses, fruit with edible seeds, vegetables (beans, cauliflower, courgette, celery, peas)

• Oats and linseeds do not produce gas/do not bloat

• Increase fluid to at least 6 – 8 glasses per day

• Co-Enzyme Q10• Vitamin-like substance in the mitochondria or the

“powerhouse” of the cell

• No proven use for strengthening muscles in DMD: Cooperative International Neuromuscular Research Group

pilot trial only muscle strength, but numbers very small (12/13

completed) Not controlled study!

Novel foods

Tracey Davis, Specialist Dietitian, GOSH

• Creatine

• Role - supplies energy to body via increasing ATP (energy) formation• Weak evidence in MD - no evidence it increases muscle

strength• The Cochrane review (Kley, Vorgerd and Tarnopolskyonly, 2007)

included 1 study in patients with MD.• We do not know the dose, how long to give it for and after

effects. • Very little research into its effectiveness and safety – not

recommended

Novel foods

• Malnutrition common

• Referral

• Food –> nutritional supplement drinks –> enteral feeding

• Monitor! Monitor! Monitor!

• Regular preventative nutritional therapy is recommended

Overall conclusion

Any Questions?