clinical applications WEEK S CLINICAL APPLICATIONS CASE STUDY: PAUL\'S ADAPTATIO
ID: 128153 • Letter: C
Question
clinical applications
WEEK S CLINICAL APPLICATIONS CASE STUDY: PAUL'S ADAPTATION TO CYSTIC FIBROSIS Paul is a 12-year-old boy with cystic fibrosis. He is hospitalized with pneumonia, and he has difficulty breathing. Paul is a thin child with little muscle development who tires easily, although he has a large appetite. His stools are large and frequent, and they contain undigested food material. Questions for Analysis: 1. What is cystic fibrosis? Account for the clinical effects of the disease as evidenced by Paul's appearance and symptoms. 2. What are the basic goals of the treatment of cystic fibrosis? Why is vigorous nutrition therapy a primary part of treatment? 3. Describe the role of enzyme replacement therapy in this aggressive nutrition support. 4. Why does Paul require therapeutic doses of multivitamins, including fat-soluble vitamins?Explanation / Answer
1. CYSTIC FIBROSIS
Cystic fibrosis is an inherited disorder that affects the exocrine glands of the body.This disorder affects the sweat glands,respirstory system,digestive tract,reproductive tract.
According to the given case study, CYSTIC FIBROSIS is defined as the congenital restrictive lung disorder in which the secretions of the exocrine i.e, mucus producing glands are abnormal.The exocrine gland dysfunction leads to abnormal levels of sodium and chloride,an increased viscosity of mucus secretions, and susceptibility to pulmonary colonization of bacteria.
The epithelium of body tissues exhibits marked impermeability to chloride and an excessive reabsorption of sodium which alter the water movement across the membrane,leading to thick secretions of mucoproteins that plug the airway.Due to the airway obstruction,stasis of thick , sticky pulmonary secretion leads to the risk of pathogen invasion.
Chronic respiratory infections are a hallmark of cystic fibrosis.The manifestations associated with this are cough, sputum production, hyperinflation of alveoli,bronchiectatasis, and pulmonary insuffiency, failure to thrive,foulsmelling bulky stools,deficiency of vitamins A,D,E,K.
2. TREATMENT GOALS OF CYSTIC FIBROSIS
The goals of therapy for cystic fibrosis are to ensure a reasonable quality of life for as long as possible and prevent or slow the decline in pulmonary functioning.
The treatment of cystic fibrosis is aimed at
Aggressive managemaent of the lung disease includes - promoting the removal of secretions from lungs, preventing and treating infections,and managing related pulmonary complications.
Nutritional management
Maintenence of adequate nutrition is very important in a child with cystic fibrosis.The majority of children with cystic fibrosis have some exocrine pancreatic insufficiency as a result of obstruction of the pancreatic ducts.Lack of pancreatic enzymes cause malabsorption of nutrients and vitamins A,D,E,K defficiencies. Failure to thrive is even evident in a child who has good appetite. Hence, Nutritional management is very important in a child with cystic fibrosis.
The management associated with malabsorption includes
3. Most of the clients with cystic fibrosis benefits from pancreatic enzymes replacement. Capsules generally contains between 4000 and 29000 units of lipase. The doze of enzymes (typically no more than 20000 units/kg per meal)
4. Paul requires nutrional support as he has symptoms of malabsorption and failure to thrive
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