287285-67526-30-Nutrition-_1

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Nursing

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Nov 24, 2024

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1 Nutrition in Healthcare: Cystic Fibrosis Name (Institutional Affiliation) Course Professor Date

2 Nutrition in Healthcare: Cystic Fibrosis Cystic fibrosis (CF) is a complicated genetic disorder that, impacts several organs; including the lungs; pancreas; liver; kidneys; and gut. Fundamentally; CF is brought on by mutations in the CFTR gene. This mutation causes a serious breakdown in the body's mucus production, and management system. People with CF create a thick; sticky mucus instead of the thin; slick fluid that, normally lines, and protects numerous channels in the body. There are serious repercussions from this abnormal mucus; especially in the lungs where it may obstruct airways; and serve as a haven for germs ( Kimball et al.; 2021). This leads to a vicious cycle of severe; recurring lung infections; which over time may do a great deal of harm. Although CF is a relatively uncommon illness in the US; it nonetheless affects 30;000 individuals; making it a major presence. This prevalence emphasises how crucial it is to continue research, and find efficient therapies to control, and lessen the symptoms of this difficult illness. Common Medications Leading the way in treating the symptoms, and course of CF treatment are a few prominent drugs. One such medication is ivacaftor; often known as Kalydeco; it is essential in treating certain genetic abnormalities linked to cystic fibrosis. Another important medicine is Lumacaftor/Ivacaftor; also marketed as Orkambi; which combines two medications to increase their efficacy. Furthermore; many CF patients have hope for a better quality of life because to the combination of Elexacaftor; Tezacaftor; and Ivacaftor; which is marketed under the brand name Trikafta (Zhu et al.; 2021). By focusing on the disease's fundamental causes; these drugs represent a significant development in the treatment of cystic fibrosis. Subjective and Objective Findings The complicated genetic disorder known as CF affects several different systems in the body, and presents with a wide variety of symptoms and findings. Constitutionally; overall weakness, and exhaustion are common in patients; they are direct results of the body's defences against ongoing infections, and other consequences associated with cystic fibrosis.

3 One prominent sign is weight loss, which is often ascribed to malabsorption, a prevalent problem with CF caused by pancreatic insufficiency impairing digestion. With the noteworthy exception of nasal polyps, the majority of discoveries made while evaluating the head, eyes, ears, throat, and mouth (HEENT) of CF patients are usually non-specific to the illness. Patients with cystic fibrosis often develop these growths in their nasal passages, most likely as a result of ongoing infection and inflammation. The effects of CF are mostly felt by the respiratory system. Shortness of breath, wheezing, and a chronic cough are common symptoms in patients. When the lungs are auscultated, crackles or wheezes may be heard, which are signs of mucus build-up and infection that are common in CF lungs. Regarding examinations of the heart, genitourinary system, nervous system, and throat/mouth, CF usually does not show any particular results. On the other hand, the gastrointestinal tract and abdomen are severely impacted. Patients who have malabsorption of nutrients may have poor weight growth even with appropriate ingestion. In addition to being prevalent, oily, bulky stools and intestinal obstruction are indicators of the digestive issues related to CF (Zhu et al., 2021) . Clubbing of the fingers and toes is a musculoskeletal symptom of persistent hypoxia. The abnormal transport of chloride over the epidermis causes an abnormal taste in the integumentary system, and patients often experience this. Patients with cystic fibrosis may have respiratory distress on their vital signs, which is a typical consequence of lung involvement. Monitoring and diagnostic technologies are essential for managing cystic fibrosis. Because it captures the faulty chloride transport across the epithelium, the sweat chloride test is still considered the gold standard for diagnosis. Given the extensive pulmonary involvement in CF, regular screenings for lung health include lung function tests and chest X-rays. Bronchiectasis, and sinusitis are two more physical abnormalities that, are often linked to CF. While bronchiectasis reflects chronic lung; damage brought on by recurrent infections; and inflammation; sinusitis is often the outcome of the same irregularities in mucus that, affect the lungs. Nutritional Needs

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4 Drug-food interactions may greatly affect how well drugs work for people with CF. The impact of high-fat meals on the absorption of some CF medicines is one noteworthy interaction. High-fat diets may cause CF patients' drugs to be absorbed more readily; which might have an impact on how well they work as a treatment. The interaction between grapefruit, and grapefruit juice, and some CF drugs is another crucial factor to consider. Components in grapefruit may affect how some drugs are metabolised; changing the strength, and duration of their effects. Medication side effects that, affect food habits, and metabolism are also possible with CF medications. Certain drugs may result in gastrointestinal problems; such nausea, and diarrhoea; which may have a big effect on digestion, and appetite. For CF patients; these side effects could make it difficult to keep up a healthy diet. Allergies, and food intolerances are prevalent among CF patients (Zhu et al.; 2021). A lot of people with CF could have trouble breaking down lactose, and gluten. As such; it is imperative that, they exercise caution while making food decisions, and choose options that, are less taxing on their digestive systems. It could be required to use different feeding techniques in severe instances of CF. It could be necessary to use enteral feeding; which is often done with a feeding tube; to make sure patients get enough food, and maintain their general health. For individuals with CF, therapeutic diets are often advised, especially for those who have dysphagia. Diets heavy in calories and protein may support people in meeting their nutritional requirements and overcoming the obstacles their disease presents. According to Kim et al. (2019), p atients with cystic fibrosis may be offered supplements to treat particular dietary deficits. Supplementing with pancreatic enzymes is a frequent way to help with digestion; on the other hand, vitamins A, D, E, and K could be required to be healthy overall. Patients with CF should be careful while choosing diets since foods heavy in fat and salt might make their

5 condition worse. To promote their health and wellbeing, kids are urged to choose nutrient- dense, high-calorie foods like fruits, vegetables, and whole grains. Use of Nursing The respiratory and digestive systems are severely impacted by the complicated genetic disorder known as CF. It is mostly distinguished by the formation of viscous, thick mucus in the lungs and digestive system, among other organs. To enhance the quality of life for those who have CF, managing the illness requires a multimodal strategy that combines dietary modifications and medicines ( Thorat et al., 2023) . Taking medications; like Ivacaftor; is essential to controlling cystic fibrosis. Ivacaftor is a new drug designed to treat the underlying genetic defects that, cause cystic fibrosis. It works by increasing the activity of a specific protein called CFTR (cystic fibrosis transmembrane conductance regulator); which is necessary to maintain the right proportion of salt to water in many organs. Ivacaftor is a useful supplement to the therapy regimen since it enhances CFTR activity; which lessens some of the symptoms, and effects related to that, gene. Interventions with Nutrition Nutritional interventions have an equally significant role in the treatment of cystic fibrosis. These medicines target the unique dietary needs, and challenges faced by individuals with cystic fibrosis. According to Hjelm et al. (2023); people who have CF need more energy since it takes more effort for their bodies to fight infections, and breathe. As such; they often need a diet that, is high in calories, and rich in protein to maintain growth, and maintain muscle mass. It is crucial to educate patients, and their caretakers the importance of eating meals that, include adequate calories, and protein to meet these nutritional demands. Because CF may make it more difficult for the body to absorb essential nutrients; malnutrition may ensue. It's critical to routinely look for signs of malnutrition; such as children's delayed growth; vitamin deficiencies; and weight loss. Healthcare professionals may then provide

6 supplements or change the patient's diet to quickly treat these problems. Moreover; pancreatic insufficiency affects a large number of CF patients; which complicates their ability to absorb, and digest lipids, and fat-soluble vitamins. Supplemental enzymes may speed up the breakdown of fat, and enhance nutritional absorption. To do this; pancreatic enzyme replacement therapy is often used. To maximise their nutritional status; patients should be informed that, taking these enzymes with meals; especially those high in fat; is necessary. Conclusion Therefore; a comprehensive strategy is needed to treat the inherited illness known as cystic fibrosis. Drugs such as Ivacaftor provide specific treatment for the underlying genetic problems; but nutritional therapy is essential for maintaining general health. Patients with CF should learn how to consume meals high in calories; and protein; and part of their treatment regimen should include taking enzyme supplements; as directed, and keeping an eye out for any indications of malnutrition. Healthcare professionals may improve the quality of life, and long-term results for people with CF by treating both the genetic, and dietary aspects of the disease. References

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7 Hjelm, M., Hente, E., Schuler, C. L., Duan, Q., Strong, S., Boat, T., & Filigno, S. (2023). Education‐related needs for children with cystic fibrosis: Perspectives of US pediatric care teams. Pediatric Pulmonology . Kim, E., & Lo, A. W. (2019). Venture philanthropy: a case study of the cystic fibrosis foundation. Available at SSRN 3376673 . Kimball, H., Douglas, T., Sanders, M., & Cobham, V. E. (2021). Anxiety in children with cystic fibrosis and their parents: a systematic review. Clinical Child and Family Psychology Review , 24 , 370-390. Thorat, T., McGarry, L. J., Jariwala-Parikh, K., Limone, B., Bonafede, M., Chandarana, K., & Konstan, M. W. (2021). Long-term impact of ivacaftor on healthcare resource utilization among people with cystic fibrosis in the United States. Pulmonary Therapy , 7 (1), 281-293. Zhu, Q., Sundstrom, E., & Xu, Y. (2021, December). Better understand rare disease Patients’ needs by analyzing social media data–a case study of cystic fibrosis. In 2021 IEEE international conference on bioinformatics and biomedicine (BIBM) (pp. 2618-2621). IEEE.

287285-67526-30-Nutrition-_1

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