Case studies

Data on the participants who frequently missed enzymes were examined in more detail and it provided a representation of patients enzyme practices that are best described as case studies. Three examples are detailed below with suggestions of where things are going wrong and what improvements could be made.

Case study 1 18-year old female

Lung function: FEV1 78%. No inpatient admissions and 5 outpatient appointments in the

past 12 months.

Nutritional status: BMI 22 kg/m², CFDM

PERT: Creon 25,000 - 10 capsules per day (4310 LU/kg/d). Theses are counted out, swallowed whole and taken before, during and after food. Misses PERT with 6-9 meals per week and >10 snacks per week. She reports symptoms of abdominal pain. Bowels are open twice a day. Enzyme therapy works ‘ok’ for her. She is prescribed a proton pump inhibitor and takes lactulose. She receives ‘occasional’ advice from the doctors, dietitian and family regarding her enzyme therapy.

It is ‘always’ embarrassing taking enzymes. She takes enzymes in front of people ‘most of the time’ but gave the example response ‘it’s embarrassing and people ask questions’. She also reports that she ‘often’ forgets them. She ‘usually’ takes enzymes when eating out and carries enzymes around ‘most of the time’. She has no problems finding the time to take enzymes at

Comment: This case study describes a young lady with good nutritional status and lung function. However she does not take PERT with a considerable number of meals and snacks. In view of her good lung function and nutritional status it could be presumed that she is generally managing well and dietetic input may be minimal. However she reports abdominal symptoms which have probably resulted in the prescription of a proton pump inhibitor and lactulose. Determining the reasons for non-adherence to enzymes and improving the situation may have led to an improvement in gastrointestinal symptoms. Instead, it has resulted in her being prescribed two potentially unnecessary medications. It can be estimated that one meal and 1-2 snacks per day are taken without PERT and the reasons behind this appear to be forgetting to take and embarrassment. It is simply not enough to recommend routine use of PERT, clinicians need to be able to identify circumstances that make taking PERT difficult and practical strategies to help.

Case study 2

19-year-old single male

Lung function: FEV1 55%, 1 inpatient stay and 10 outpatient visits in the past 12 months. Nutritional status: BMI 16.5 kg/m². CFRD. No feeds or supplements.

PERT: Creon 25,000 - 30 capsules per day (14,124 LU/kg/d). These are counted out,

swallowed whole and taken before and during meals. PERT is taken with every meal but missed with 6-9 snacks per week. He reports symptoms of abdominal pain. Bowels are open twice a day. No adjunct therapy is prescribed. Enzyme therapy works “very well”. He receives ‘frequent’ advice from the dietitian and his family regarding his enzyme therapy.

It is ‘never’ embarrassing for him to take enzymes and he still manages to take them when eating out. He takes PERT in front of people ‘all of the time’. He reports that he ‘sometimes’ forgets to take them“. Enzymes are carried on him ‘most of the time’. There is ‘sometimes’ no time to take PERT at college and when there is a special occasion, but never misses them at home or when he has problems with his health.

Comment: This case describes a young man with moderate lung function. We have come to expect good lung function from patients on transition to the adult service, and his poor nutritional status may be partly to blame for this. He is not taking any nutritional supplements or enteral feeds. With such a low weight it is very likely that this will have been previously recommended, which suggests that the patient has declined to take them. He is managing to take his enzymes well with meals but misses frequently with snacks. If the energy from snacks were fully utilized with optimal PERT, then weight gain is likely to be achieved. From his responses in the questionnaire, he does not appear to present any specific issue as to

why he doesn’t manage enzymes with all of his snacks, other than he sometimes forgets. Issues’ regarding his poor weight and inadequate PERT use need to be tackled in a fresh way as previous advice has obviously had no benefit.

Case study 3

18-year-old single male.

Lung function: FEV1 120%, No inpatient stays and 5 outpatient visits in the past 12 months. Nutritional status: BMI 17.4 kg/m². CFDM. No feeds or supplements

PERT: Creon 10,000 - 8 capsules per day (538 LU/kg/d). No adjunct therapy. These are

counted out, swallowed whole and taken during food. PERT is taken with every meal but no snacks. He reports no abdominal pain. Bowels are open 2-3 times per day. No adjunct therapy is prescribed. Enzyme therapy works “very well”. He receives occasional advice from the doctor on his enzyme therapy.

Taking enzymes is embarrassing ‘some of the time’. He still manages to take them when eating out. He has no problems taking PERT in front of other people. He finds the time to take PERT at college, home, special occasions and when health is a problem.

Comment: This young man has exceptional lung function despite his low BMI. This is a

classic example of where if this patient had presented in clinic we would have gone down the route of discussing the need for nutritional supplements. However, as a first line it is probably more appropriate and cost effective to ensure optimal digestion of the food he is currently managing and this may be enough to achieve his target weight. He is on a relatively low dose of PERT. However this is only being taken with meals and not snacks. This is a common assumption that PERT is only required with meals yet snacks can often be high in fat. He reports no abdominal pain which may indicate that he is asymptomatic if maldigestion occurs. A trial of PERT with all snacks containing fat is recommended to determine whether an improvement can be seen. Not taking PERT with snacks could have arisen due to the embarrassment of having to take in front of other people therefore requires investigation.

To conclude, the case presentations were chosen due to frequently missing PERT with food. Coincidently, all three cases had recently been transferred from paediatrics and were therefore relatively new to the service. It is unclear whether their inappropriate PERT use would have been identified as patients became established with the service or whether these were issues that we would never have been detected. Two out of the three individuals had CFDM and the data did identify that diabetic patients were more likely to be non-adherent