Most commonly the CF genetic mistake is the DF508 protein that results in hypochloremia that progresses to lung issues related to thick mucus which inhibits proper air exchange that leads to infections, lung damage, and ultimately respiratory failure (CFF, n.d.). The CFF (n.d.) state CF is attributable to 90% of all childhood pancreatic diseases and can affect other organs, but the most common cause of death is related to lung disease. CFF (n.d.) state the malnutrition and poor growth issues with CF patients are due to the mucus build up in the pancreas that interferes with food and nutrient absorption; therefore, treatment is aimed at therapy to clear airways, and pancreatic enzyme supplements to improve absorption of nutrients. There is also a high risk of chronic endobronchial infection with children. Bacteria such as Staphylococcus aureus, Pseudomonas aeruginosa and Burkholderia cepacia are prone to colonize in the lung which makes antibiotics difficult to reach them. Chronic problems related to CF include; bacterial infections in the lungs, obstructive pulmonary disease due to decreased mucocillary clearance, malnutrition from pancreatic insufficiency and failure to thrive.
What teaching related to her diagnosis would you provide the parents?
Cystic fibrosis treatment and teaching depends on the stage of the disease. I will advise the parents the importance of breathing exercises, percussion and postural drainage techniques. I will also review the need to avoid known respiratory irritants such as smoke and air pollutants and persons with upper respiratory infections. The child may have trouble breathing when she lies flat, so I will instruct the mother to elevate the child’s head when she sleeps and use a cool mist humidifier to increase air moisture in the home. This may make it easier for the child to breathe and to cough up mucus. An enriched diet with vitamin and enzyme replacement to help maintain body weight will be added to her plan of care. And as far as medication, I will encourage the parents to continue giving the Antibiotics to treat and prevent lung infections and medicines to thin the mucus.
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