Question

A six-month-old with cystic fibrosis has been started on pancrelipase (Viokase) powder. Which finding indicates that the medication is having the desired effect? A. The infant has fewer respiratory infections. B. The infant's skin no longer has a salty taste. C. The infant shows a favorable gain in weight. D. The infant sleeps throughout the night.

Short answer

Answer: C. The infant shows a favorable gain in weight.

Step-by-step solution

Understanding Pancrelipase

Pancrelipase is a medication used to treat people with pancreatic enzyme insufficiency, often seen in cystic fibrosis patients. It contains a mixture of digestive enzymes, including lipases, proteases, and amylases, which help digest and absorb nutrients from food. The primary goal of this medication is to improve digestion and promote healthy weight gain in people with pancreatic enzyme insufficiency.

Option A: Fewer respiratory infections

Pancrelipase's primary function is to improve digestion, which does not have a direct impact on the respiratory system. While good nutrition may contribute to better overall health, it is not the primary effect of the medication. Therefore, this option is not the correct answer.

Option B: No salty skin taste

Cystic fibrosis patients have a salty taste on their skin due to an imbalance in the body's chloride and sodium levels. Although this is a symptom of cystic fibrosis, pancrelipase specifically targets aiding the digestion process and does not address the salty skin taste. Therefore, this option is also incorrect.

Option C: Favorable weight gain

Pancrelipase's main function is to help the patient digest food, allowing better absorption of nutrients and ultimately promoting healthy weight gain. Given that pancreatic enzyme insufficiency often results in malabsorption and malnutrition, this medication will assist in delivering the necessary nutrients that the body needs. As a result, observed favorable weight gain indicates that the medication is having the desired effect. This option is the correct choice.

Option D: Sleeps throughout the night

While good digestion can contribute to overall wellbeing and comfort, sleeping throughout the night is not a primary or direct effect of pancrelipase. Therefore, this option is not accurate, making option C the correct answer. In conclusion, the correct answer is: C. The infant shows a favorable gain in weight.

Key concepts

Pancreatic Enzyme Insufficiency

When an individual has pancreatic enzyme insufficiency, their pancreas is unable to produce enough of the enzymes needed for proper digestion. This condition is common in cystic fibrosis patients because the thick mucus associated with the disease blocks the release of these enzymes. As a result, patients struggle to break down and absorb nutrients from their food, leading to malnutrition and weight loss.

Symptoms of pancreatic enzyme insufficiency can include bulky stools, weight loss, and nutrient deficiencies. In cystic fibrosis, the correct identification of this insufficiency is crucial as it directly affects the patient's growth and overall health. Early intervention with enzyme replacement therapy is essential to manage symptoms and improve quality of life.

Digestive Enzymes Supplementation

Digestive enzymes supplementation, as seen with pancrelipase, is a critical treatment to counteract pancreatic enzyme insufficiency. Pancrelipase contains lipases for fat digestion, proteases for protein breakdown, and amylases for carbohydrate digestion. By supplementing these enzymes, the medication aids the digestive process, helping patients absorb the nutrients from their meals more efficiently.

Treatment with pancrelipase typically involves taking the medication with every meal and snack to ensure proper digestion. Correct dosage and adherence to the medication schedule are essential for optimal results. By enabling better nutrient absorption, enzyme supplementation directly supports growth and development in children with cystic fibrosis.

Malabsorption and Malnutrition in Cystic Fibrosis

Malabsorption in cystic fibrosis patients occurs when the mucus blocks the pancreatic ducts, preventing enzymes from reaching the intestines to break down food. This leads to malnutrition, as the body cannot absorb the necessary nutrients from the diet.

Malnutrition in cystic fibrosis is a significant concern because it can lead to poor growth, weakened immune system, and reduced lung function. It's important to monitor the nutritional status of patients closely and adjust treatment as needed to prevent these serious complications. The effectiveness of pancrelipase can be judged by improvements in symptoms of malabsorption, such as better weight gain and growth patterns in children with cystic fibrosis.

Improving Patient Nutrition

Improving patient nutrition in cystic fibrosis involves a multifaceted approach beyond just enzyme supplementation. A high-calorie, high-fat diet is often recommended to counteract the malabsorption of nutrients and the increased energy expenditure from lung infections and physiotherapy.

Alongside pancrelipase, vitamin and mineral supplements are commonly prescribed due to difficulties in absorbing these essential nutrients. Regular assessments by healthcare professionals guide dietary modifications and ensure that patients are receiving the nutrients needed for their growth and lung health. By paying close attention to diet and digestive help from enzymes, patients with cystic fibrosis can maintain better nutritional status and overall wellbeing.