Issue 127 enteral feeding duchennes muscular dystrophy

CLINICAL

Kate Roberts RD Freelance Dietitan Kate is a Freelance Dietitian with a wide range of clinical experience of working with adults and children from previously working in the NHS, her specialities are Diabetes and Allergies.

CASE STUDY: ENTERAL FEEDING AND NUTRITION SUPPORT IN A PATIENT WITH DUCHENNE’S MUSCULAR DYSTROPHY This is a case study of a 17-year-old boy with Duchenne’s muscular dystrophy (DMD). His case was complicated due to ensuring his wishes, family dynamics and problems with establishing his weight.

I first met Master M when he was referred to the Nutrition and Dietetic Service when he needed nasogastric (NG) feeding on the ward. There was no weight completed on admission. He had been admitted due to shortness of breath and a productive cough and was diagnosed with a lower

respiratory tract infection. His previous medical history included DMD and one previous admission for pneumonia.

Master M was wheelchair bound and lives with mother and siblings. His mother is his main carer. The only medicine he had been taking was Movicol.

1 ASSESSMENT Weight history: 54kg 15 months ago, unable to weigh on the ward.

Nutritional diagnosis: Inadequate energy intake related to dysphagia evidenced by deteriorating nutritional status and chest infection.

Overall aims of treatment as an inpatient To meet the patient’s estimated nutritional requirements via nasogastric (NG) feeding by Day 6 of his regimen.

To prevent any further weight loss by meeting his estimated nutritional requirements by Day 6 of his feeding regimen.

To re-establish oral intake hopefully within one month as directed by Speech and Language following a videofluoroscopy (VFS).

First assessment on Day 6 of inpatient stay Anthropometrics Weight: 47kg, height 1.572m, BMI: 19kg/m2. Approximation based on discussion with mother. However, he appeared undernourished.

He was already being seen by the Physiotherapists and Speech and Language Therapists (SLT). An SLT had advised stage 1 fluids via teaspoon only and nil by mouth would be safest for food, NG tube if for active treatment. They also arranged a VFS. Master M reported that he will be drinking up to 800mls as per SLT recommendations. He was nil by mouth. The NG feed was on the out-of-hours regimen and ran at 20ml/hr the day before until 0100hrs.

Master M and his mother consented to the NG tube. He was keen to only have NG feed during the day and wanted to be able to switch it off for bathroom breaks and physiotherapy.

2 IDENTIFICATION OF NUTRITION AND DIETETIC DIAGNOSIS Objectives: At initial assessment, estimate nutritional requirements based on estimated weight using Schofield.

Meet estimated nutritional requirements via NG tube by Day 6 of feeding regimen.

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CLINICAL Medical team to monitor biochemistry (potassium, phosphate, magnesium and corrected calcium) daily until feeding regimen is established by Day 6 to reduce risk of refeeding syndrome.

Nursing staff to monitor feeding position, tolerance, fluid balance and bowels closely to reduce complications and ensure patient does not aspirate.

Accurate and timely records should be kept to ensure effective monitoring of patient.

Estimated nutritional requirements: Schofield, as Henry does not cover <18 years old1 BMR: 17.7 x 47 + 657 = 1,489 X 15% DIT = 1,712 Protein: 50-59g (0.17-0.20) Fluid 1,645ml Na (mmol) 1 x 47 = 47 K (mmol) 1 x 47 = 47 3 PLAN NUTRITION AND DIETETIC INTERVENTION Proposed treatment plan Nursing staff to weigh patient before next review.2 Nursing staff to feed via NG feed as per regimen outlined below.1,3 Medical team to monitor potassium, phosphate, magnesium and corrected calcium daily until stable, correct if needed.1, 3 Nursing staff to monitor feeding position, tolerance, fluid balance and bowels.1,3 800mls of stage 1 fluids via teaspoon as per SLT recommendations.

Table 1: Feeding regimen: Day

Feed

Rate

Fluid

Provides

1

300ml Nutrison Standard

30mls/hr over 10 hrs

800ml fluid orally and 550ml extra flushes

300kcals, 12g protein, 1,650ml fluid

2

500ml Nutrison Standard

50ml/hr over 10 hrs

800ml fluid orally and 350ml extra flushes

500kcals, 20g protein, 1,650ml fluid

3

750ml Nutrison Standard

75ml/hr over 10 hrs

800ml fluid orally and 100ml extra flushes

750kcals, 30g protein, 1,650ml fluid

4

500ml Nutrison Concentrated

50ml/hr over 10 hrs

800ml fluid orally and 350ml extra flushes

1,000kcals, 37.5g protein, 1,650ml fluid

5

750ml Nutrison Concentrated

75ml/hr over 10 hrs

800ml fluid orally and 100ml extra flushes

1,500kcals, 56.25g protein, 1,650ml fluid

6

850ml Nutrison Concentrated

85ml/hr over 10 hrs

800ml fluid orally and 100ml extra flushes

1,700kcals, 63.75g protein, 1,750ml fluid

4 IMPLEMENT NUTRITION AND DIETETIC INTERVENTION Day 6 Discussion with SLT: M has started having food at mealtimes.

Still due to have a VFS and continue with NG. Oral trials: normal liquids, mash/puree options, small amounts for meals, e.g. ice-cream/scrambled egg/Weetabix. I requested food record charts.

Day 8 review There was no new weight. There were ongoing discussions whether he should have a PEG fitted. Medically he was dehydrated and they queried whether he had worsening pneumonia. Master M had decided he wanted to eat and his SLT had explained the risks associated. He was still happy to have a VFS. Potassium and phosphate both had to be replaced due to being low on Day 4 of the admission, this is on Day 2 of the out-of-hours NG feeding regimen and indicated to me refeeding syndrome. Phosphate levels are a

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Table 2: Biochemistry Day in hospital Na 135-145mmol/l K 3.5-5.5mmol/l Ur 2.5-6.5mmol/l Cr 55-100umol/l Bilirubin ALK Phos 35-104U/I ALT 0-45U/I T Pro60-80g/l Alb 35-50g/l CCa 2.2-2.65mmol/l PO4 0.7-1.4mmol/l Mg 0.7-0.95mmol/l

Day 4 139 3.2* 0.6* <20* 15 74 54* 66 40 2.49 0.47* 0.77

Day 6 138 4.3 2.2* 6* 15 85 47 76 44 2.54 1.29 0.83

good marker for RFS5 and although the potassium is slightly below normal levels, it confirms it.

Staff nurse reported that Master and mum did not want the NG feed, just Weetabix. When I spoke to them they reported that they did not realise he could have both at the same time.

Day 12 Discharged from ward. NGT had come out. Medics report eating and drinking (however, SLT reported that there is only one packet of crisps and a glass of milk documented all weekend).

Safest texture would be mashable diet and stage 1 fluids, however, the patient has decided to accept risk of aspiration and have normal food and fluids.

For follow up in community.

5 MONITOR AND REVIEW It is difficult to assess if there had been any progress or positive outcomes from his inpatient stay. The patient was not weighed at any point. Daily requirements were therefore estimated, which gave something to aim towards. However, we did not meet the objective of meeting these requirements as the patient was discharged before he was weighed. The medical team did not check the patientâ&#x20AC;&#x2122;s biochemistry after Day 6 despite being asked which was potentially dangerous as outlined below. There was also confusion over his NG feed. It was unfortunate that it was not flagged up that the patientâ&#x20AC;&#x2122;s mother had learning difficulties. The nursing staff reported that the patient and mother were refusing the NG feed, however, they did not realise that they could have the feed and oral intake at the same time. If the nursing staff realised that his mother did not understand, they would have perhaps taken more time to explain rather than assuming that they were refusing treatment. The objectives were, therefore, not met.

The patient had been eating before he was put nil by mouth. On Day 2 of the out of hours feeding protocol, his phosphate and potassium were low and then were corrected. At the time, I felt that he was still at risk of refeeding syndrome which was reflected by his abnormalities in biochemistry and malnourished appearance.

In the care plan, it was outlined for the medical staff to monitor and correct K, PO4, Mg, CCa daily until stable.

His feed was started at less than 50% of his total energy requirements. Pabrinex should have been given on Days 1 and 2 of the out-of-hours feeding regimen. He should also have been prescribed thiamine, vitamin B Co Strong and multivitamins as outlined in NICE guidance3 Was he discharged from hospital too early? He was not established on a feeding regimen or on oral feeding.

On reflection, I would now try to keep him in hospital for longer, if we had had a weight, the decision could have been based on his low BMI. He was never fed past Day 2 of the feeding regimen as the tube came out. He would probably have refused to have another NG passed. However, if his weight had been known, PEG insertion may have been requested at the time.

If it had reached Day 6, his feeding regimen would have provided above his requirements by: 100kcals, 10g protein and 300mls fluid. There would not have been a problem with the extra kcals and protein but it could have led to him being fluid overloaded.

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CLINICAL This highlights the importance of getting a timely weight on admission. This should now have been addressed due to the new local guidelines on malnutrition screening on arrival. The CQC has also recently been in the hospital and have reiterated the importance of correctly completing MUST training. The dietetic department has offered training to all nursing staff subsequently.

6 EVALUATION After his discharge home, I telephoned promptly to speak to his mum and then liaised with his community nurse.

We arranged a joint visit. I was then able to estimate his nutritional requirements which at the time were less than his reported estimated oral intake. He was taking the vitamins A, D, E and C. Master M’s BMI was on the 0.4th centile line, his height was between the 0.4th and 2nd centile. His weight was well below the 0.4th centile for his age.

I should have perhaps discussed gastrostomy feeding with the patient earlier. He was just very keen to eat normally when he was discharged. And discharged without my knowledge. Also, we did not have an accurate weight as the ward staff had not weighed him.

Home gastrostomy feeding does not always improve quality of life (QoL) in adult patients,4 despite preventing weight loss and improving some aspects of quality of life (QoL). However, Sullivan et al found a significant improvement in the QoL of carers of children with cerebral palsy, which is partly due to a decreased concern regarding their nutritional status. It has been found that if parents of children with severe developmental disabilities are well informed of the benefits, then they would agree to earlier PEG placement.6 PEG placement is safe and can improve the QoL of the family and the child’s global status.6 Often parents are reluctant due to worries regarding potential complications. The majority of DMD patients and their families are pleased about the PEG placement and benefits include amelioration of malnutrition as well as improvements in swallowing and respiration.7 It is recommended that gastrostomy placement should be encouraged when swallowing problems have just started to decrease risks of respiratory failure and peritonitis.7 Gastrostomy placement can improve nutritional outcomes and is generally well tolerated with few complications.8 Another thing that could have been considered was supplementing Master M’s diet with Creatine Monohydrate.9 A Cochrane review10 reported that this amino acid helped increase muscle strength, lean muscle mass and bone density in patients with neuromuscular disease (NMD). It was also found that in patients with DMD glutamine, as well as other amino acids, also inhibited protein degradation.11 Although Master M’s direct nutrition, clinical and health status did not improve, I believe that his treatment was always patient centred. His wishes were always taken into consideration and respected, despite the risks of eating and drinking normal foods and fluids. Patients can refuse suggested treatments if they are mentally competent, even if it does not seem to be in their best interests.12 I believe that Master M’s quality of life has been maintained even at risk of aspiration.

Master M was not known to the dietetic service before his hospital admission. It is strange that he was not referred to our Paediatric dietitians earlier. Parents with learning disabilities often find it difficult to access services for when their children are in need and require extra help with getting support.13 He had not been weighed for a year.

Hopefully with the compulsive MUST screening, future patients will be seen in a more timely fashion.

References 1 Todorovic VE and Micklewright A (2011). Pocket Guide to Clinical Nutrition, 4th Edition. The Parenteral and Enteral Nutrition Group of the British Dietetic Association 2 Bushby K et.al (2009). Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care. The Lancet [online].

DOI:10.1016/S1474-4422(09)70272-8 3 Nutrition support in adults. Clinical guideline CG32, 2006, www.nice.org.uk/page.aspx?o=cg032 4 Loeser C, von Herz U, Küchler T, Rzehak P and Müller MJ (2003). Quality of life and nutritional state in patients on home enteral tube feeding. Nutrition. Vol 19, Issues 7-8, July-August, Pages 605-611 5 Khan LUR, Ahmed J, Khan S and MacFie J (2011). ‘Refeeding syndrome: a Literature Review’. Gastroenterology Research and Practice, Vol 2011, Article ID 410971, 6 pages, 2011. doi:10.1155/2011/410971 6 Martínez-Costa C, Borraz S, Benlloch C, López-Sáiz A, Sanchiz V, Brines J (2011). Early decision of gastrostomy tube insertion in children with severe developmental disability: a current dilemma. Journal of Human Nutrition and Dietetics, Vol 24, Issue 2, pages 115-121, DOI: 10.1111/j.1365-277X.2010.01146.x 7 Mizuno T et.al (2012). Efficacy and tolerance of gastrostomy feeding in Japanese muscular dystrophy patients. Brain & Development, Vol 34, pp 756-762, doi:10.1016/j.braindev.2011.11.012 8 Martigne L, Seguy D, Pellegrini N, Orlikowski D, Cuisset JM, Carpentier A, Tiffreau V, Guimber D, Gottrand F (2010). Efficacy and tolerance of gastrostomy feeding in Duchenne muscular dystrophy. Clinical Nutrition, Vol 29, pp 60-64. doi:10.1016/j.clnu.2009.06.009 9 Davidson ZE and Truby H (2009). A review of nutrition in Duchenne muscular dystrophy. J Hum Nutr Diet, 22, pp 383-393. doi:10.1111/j.1365-277X.2009.00979.x 10 Kley RA, Vorgerd M and Tarnopolsky MA (2007). Creatine for treating muscle disorders (Review). Cochrane Database Syst. Rev. 1, CD004760 11 Mok E et.al (2006). Oral glutamine and amino acid supplementation inhibit whole-body protein degradation in children with Duchenne muscular dystrophy’.

Am J Clin Nutr, Vol 83, pp 823-8. [Online] Accessed from ajcn.nutrition.org on May 28, 2013 12 Mental Capacity Act (2005). c9, London: HMSO. Available at www.legislation.gov.uk/ukpga/2005/9/contents 13 MENCAP (2011). Inclusive support for parents with a learning disability www.mencap.org.uk/sites/default/files/documents/2011-03/making the difference.pdf

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