- Read the case study below and answer the questions.
- Explain the pathophysiology of cystic fibrosis and the related clinical manifestations.
- Discuss the comorbidities of diabetes and malnourishment and the impact of these on the health of the person and his/ her family.
- Analyse relevant literature and discuss the associated functional impairment related to this case study.
- Reflect on the complexity of caring for this person in the community and discuss the proactive care intended to keep the client well as long as possible.
- Use evidence based practice to recommend resources/ services that are needed for caring for the client, including pharmacological management.
Stephanie Mills is a 15 year old, who was diagnosed with cystic fibrosis at birth. Stephanie lives with her parents and two older siblings, Tim 19 and Emily 17. She has regular meetings with the cystic fibrosis team every three months and is required to blood tests and throat swabs at every visit. Stephanie also has diabetes and needs to check her blood sugar levels three times a day before meals. If the blood sugar level are high then she needs to have insulin to maintain her blood sugar levels. She is also malnourished and weighs only 50 Kgs. Stephanie is on multiple vitamins and a high energy, high protein diet. She has around 30 minutes of inhalers and physiotherapy every day. With every meal, beginning with breakfast, she has to take a number of “Creons”, mixed in with her food, which helps her to digest her food. In the evening, Stephanie has another 40 minutes of inhalers, physiotherapy and nebulisers. Stephanie has to do her own breathing exercises, take her own peak-flow test readings and own medications as well. She also has to take a variety of antibiotics, which get changed from time-to-time. She is encouraged to be active but finds it very hard to find the energy and time.
Every time the family go on a holiday all her medications and equipment needs to be taken with her. If they go out for the day, or when they are on holiday, Stephanie’s daily routine of physio each morning and evening has to be fitted into the schedule. This can be really hard at times as this impacts the whole family and can be very frustrating at times. Due to the difficulties involved, the family often take turns caring for Stephanie, while the others holiday.
Stephanie’s dad works full time as a retail manager and is often not home until very late in the evenings. Her mum, Alice works part time in catering as she has to work around Stephanie’s care needs. Tim attends University during the day and works at the local milk bar in the evenings. Emily attends the local high school, is in year 11 and is busy with her netball. Lately with the multiple admissions and the inability for mum to attend work, the family have been struggling financially to pay university fees for Tim.