Background: Cystic fibrosis(CF) is a genetic disease with pulmonary complications which leads to malabsorption and nutritional derangement. Nutritional care is the essential part of multidisciplinary care for CF patients.
Client History: Ms. L, a 26-years-old single lady who worked in a private school was diagnosed with CF since 2006. Patient was referred for dietary advise in preparation for lung transplantation.
Assessment: Patient’s weight and height were 39.2kg and 1.56m; with BMI of 16 (underweight). Patient’s had normal BP and heart rate with SPO2 of 93.5% during visit. Claimed easily get tired and had SOB even when climbing one floor of stairs. She had symptom of vomiting (food particles) everyday with frequency 6x/day, usually happening during secretion of mucus while coughing. Dietary assessment showed that patient had exceeded energy (130%) and protein (103%) requirement.
Nutrition Diagnosis: Increased nutrient needs related to physiological cause increasing demand for nutrients (CP) as evidenced by anthropometric data (BMI=underweight) despite exceeded energy and protein need.
Objective & Nutrition Prescription: Nutritional intervention aimed to achieve 120-150% of the normal requirement. Patient was prescribed with ONS with ratio of 20% and 80% of ONS and solid foods respectively. Nutritional counseling focusing on tips to reduce vomiting symptoms/frequency were given to minimize energy and nutrients loss.
Outcome and Follow-up: After the third visit, patient had significant weight gain to 45.2kg (15%) within 4 months. All readings of patient’s biochemical data were normalized.
Discussion: Pancreatic insufficiency with chronic malabsorption, recurrent sinopulmonary infections, chronic inflammation and increased energy expenditure are major determinants of malnutrition in patients with CF. More than 90% of patients with CF have pancreatic exocrine insufficiency resulting in chronic nutrient malabsorption with increased energy expenditure due to work of breathing and frequent infections which increased caloric and protein requirement.
Lesson Learnt:
- Poor nutrition in CF resulted from unfavorable energy balance due to multitude of interlinked factors including increased resting energy expenditure (infection and inflammation) and energy losses (vomiting, fat malabsorption)
- Continuous nutritional monitoring will help detect early deterioration in nutritional status
- Aggressive nutritional intervention resulted in better outcomes which can lead to normal growth and lung function preservation
