Issue 140 An adult cystic fibrosis dietitian

A DAY IN THE LIFE OF . . .

AN ADULT CYSTIC FIBROSIS DIETITIAN Vanessa Bara Specialist Dietitian in Adult Cystic Fibrosis, Royal Brompton and Harefield NHS Foundation Trust Vanessa has worked in a variety of areas, including general paediatrics, head and neck cancer surgery, gastroenterology, respiratory and lung cancer. She currently works with adults with cystic fibrosis.

Cystic fibrosis (CF) used to be considered a disease of childhood, with many sufferers dying of malnutrition. In 1965, The Royal Brompton established the first adult CF service in Europe and with more than 600 patients from age 16 years upwards, it remains one of the largest CF centres in Europe.

CF is a recessive genetic disorder affecting the transport of salt and water into and out of cells. CF disease ranges from mild to severe and different organs may be more or less affected in different people. Table 1 shows some of the complications of CF. Good nutritional status is key to the wellbeing of people with CF and more effective nutritional care has resulted in gains in survival.1 Better understanding of CF and associated therapeutic advances have continued to improve outcomes: the median age of survival from birth in the UK has increased from 35 years in 20072 to 47 years now.3 As part of a 2.5 whole-timeequivalent CF dietetic team, I work within a MDT alongside specialist

nurses, physiotherapists, psychologists, pharmacists and doctors. The role of the dietitian includes nutrition support (oral and enteral), CF-related diabetes (CFRD), fat soluble vitamins, Pancreatic Enzyme Replacement Therapy (PERT), gastrointestinal symptoms, bone health and, increasingly, weight management.

We provide a clinical service to approximately 20 inpatients and participate in the weekly MDT ward round discussion. Each week we attend three clinics (seeing 15-20 patients per clinic), conduct 12 Annual Reviews and respond to 15-20 phone or email enquiries. We also support additional clinics including transition, CFRD and gastrointestinal.

Table 1: Examples of complications in cystic fibrosis Infective lung exacerbations Nasal polyps Sinusitis Pancreatic insufficiency Low levels of fat soluble vitamins Constipation Distal Intestinal Obstruction Syndrome CF-related diabetes Reflux CF liver disease Arthropathy Osteopenia/osteoporosis Infertility issues

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CASE EXAMPLES My day will vary depending on the clinical schedule, but here are some recent cases I have been involved with: CF-related diabetes An inpatient was diagnosed with CFRD when routine monitoring of blood sugar levels revealed consistently elevated results. She had several non-CF comorbidities and a limited range of foods that she could tolerate, many of which were high glycaemic index choices. I provided education sessions for her and her supportive family. She was fearful that she would be unable to manage the significant changes needed, so I focused on reassurance and helping her to develop her confidence. She has since made remarkable progress with following the dietary advice, resulting in good blood glucose control.

Nasogastric (NG) feeding Our service looks after approximately 30 patients who use gastrostomy or NG feeding tubes at home. We provide their ongoing dietetic care, but liaise with local dietetic services regarding the funding and provision of feed and equipment. A patient was discharged recently after trialling NG feeding for the final week of his admission. His body mass index (BMI) had been under 20 for about two years. (We aim for a body mass index (BMI) of at least 20kg/m2, with optimal BMI of 22-25kg/m2 in women and 23-25kg/m2 in men.4) We had been discussing the benefits of tube feeding with him for around 18 months, but he had been resistant to the idea. Unfortunately, his weight was on a downward trend, despite drinking ONS and receiving a lot of dietary education. This is a common situation that we encounter every week. During this admission his weight dropped to its lowest level, exacerbated by a loss of appetite during hot weather. To my surprise, his proposed plan to avoid further weight loss was to start overnight NG feeding and so he started that night, gaining nearly 2kg by discharge without any improvement in his oral intake. He is not yet psychologically ready to feed at home, but has said that he will consider it during his next admission.

CF and pregnancy At any one time, at least one of our 600 patients is pregnant. A current inpatient in her final trimester has not managed to sustain weight gain for reasons that include nausea, fatigue and missing meals because of her hectic job. Her lung function has dropped significantly too. In hospital and without her busy schedule, she is now eating well and drinking ONS, but has gained only 0.5kg in a week. At our last consultation, I raised the topic of NG feeding as an additional tool to promote weight and functional gain. She is considering this option and we will be working closely with the MDT, including monitoring her weight, exercise tolerance and lung function, before making a joint decision about whether to start feeding or not.

Pancreatic enzyme dosing Some patients find it difficult to appropriately dose their PERT and complain of the typical symptoms of malabsorption, including abdominal discomfort and loose, greasy, orange stools. If a verbal consultation doesn’t pinpoint the offending food (the most common cause is a takeaway!), we suggest they send us a diary of food and drink intake, PERT dose and bowel symptoms. On reviewing a recently returned diary, it was obvious that the patient was not adding extra PERT for desserts and that this was most likely responsible for his symptoms. I phoned him and provided more education regarding when and how to change PERT doses, keeping the changes as simple as possible to help him remember them. At follow-up in clinic, the patient’s symptoms had resolved after making the changes and he felt more confident with PERT dosing.

Of course, my days are not all clinical.

For example, I am the Secretary of the UK's CF Dietitian's Group. At the Group’s study day in May, I presented the results of a survey I had conducted. I have been

involved in a multidisciplinary Quality Improvement project regarding inpatient care of gastrostomy tubes. Working in the area of adult CF offers up its challenges, but each day is fulfilling.

References 1 Corey M et al. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol 1988; 41(6): 583-91 2 Cystic Fibrosis Trust. UK CF Registry Annual Data Report 2007 3 Cystic Fibrosis Trust. UK Cystic Fibrosis Registry 2017 Annual Data Report 4 Cystic Fibrosis Trust. Nutritional Management of Cystic Fibrosis, 2nd edition, September 2016

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