Cystic Fibrosis Case Study PDF

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Cystic Fibrosis Case Study Clinical Week Five: Median life Expectancy 36yrs Disease Explained: Respiratory disorder that results from inheriting a mutated gene. Both parents must have the gene to pass the disease to their child. The disease is characterized by thick tenacious secretions that cause a mechanical obstruction of organs. Lungs, pancreas, liver, small intestine, reproductive system. CF affects more than 30,000 kids and young adults in the United States. It disrupts the normal function of epithelial cells — cells that make up the sweat glands in the skin and that also line passageways. The inherited gene directs the epithelial cells to produce a defective form of the protein CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) Molly 10y/0 brought to the emergency department suffering with chronic cough and wheezing. Upon questioning the parents, they commented on the fact that when they kiss Molly, her skin tastes salty.

1) What is the significance of the salty taste to the skin of a patient with CF? In people with CF there is a problem in the transport of chloride across cell membranes. This causes thicker, stickier mucus in the lungs and digestive system, but also results in higher levels of chloride (as salt) in sweat compared with those who do not have cystic. 2)What symptoms is Molly having that are suspicious for CF? Very salty-tasting skin, Chronic Cough, and Wheezing

A diagnosis of CF is confirmed after a workup is completed. 3)Discuss the diagnostic testing that is done to confirm the diagnosis of CF?

Diagnosing cystic fibrosis is a multistep process. A complete diagnostic evaluation should include a newborn screening, a sweat chloride test, a genetic or carrier test, and a clinical evaluation at a CF Foundation-accredited care center. 4)What are some of the findings that would lead the provider to this diagnosis? The findings that would lead the provider to this diagnosis may include Studies shows that, if a sweat test is done correctly, then results that are positive will show a high chloride level. To understand what the sweat test results mean, a chloride level of: Less than or equal to 29 mmol/L = CF is unlikely regardless of age.

The nurse is reviewing the provider prescriptions for medications and treatments with the patient and her family. Molly is going to receive her first nebulizer treatment and chest physiotherapy. Xopenex, Pulmozyme (dornase alfa) have been prescribed to be given via nebulizer. 5) What education would the nurse provide to Molly and her family about these treatments? The patients with CF should always take their medications, and use airway clearance techniques, in a specific order. This is important because each medication achieves a different result. Inhaled or Nebulized Medications e.g. Pulmozyme (dornase alfa) - A preventive medication that helps break down mucous in the lungs making it easier to cough out. Bronchodilators (albuterol, Xopenex )- These help open the airways to improve clearance of mucous. Chest physical therapy (CPT), which involves lying in various positions and clapping on the chest. Breathing out through a flutter device (also known as oscillating positive).

6) Which medication should be given first and why? Xenopex should be given first. This is because; it helps open the airways that move air to or from the lungs. However, not all people with CF will respond to xenopex, so they may not be ordered. Any type of nebulizer may be used with this medication.

One of the medications the provider prescribed for Molly is called Creon (pancrelipase) 7) Describe this medication and why this would be prescribed for Molly? Creon (pancrelipase)- This is a prescription medicine used to treat people who cannot digest food normally because their pancreas does not make enough enzymes due to cystic fibrosis, chronic pancreatitis (which is the swelling of the pancreas that lasts a long time), pancreatectomy (which is the removal of some or all of the pancreas).This medication will be prescribed to Courtney because; She is young and the CF might inhibit the ability of the child's pancreas to digest fats, proteins, and carbohydrates (sugars) in food.

8) What education is important for this medication? The most important education for this treatment is that one will need to take Creon capsules whenever they eat a meal. It is best to take them just as you start to eat your food as this means that they will be mixed with the food in the stomach. Flume, P. & All (2009). Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. American journal of respiratory and critical care medicine, 180(9), 802-808

Testing for cf. CF Foundation. (n.d.). https://www.cff.org/What-is-CF/Testing/. CREON® (pancrelipase) delayed-release capsule. (2017, July). CREON® for Exocrine Pancreatic Insufficiency (EPI) | Official Website. https://www.creon.com/children-and-adults...