Nursing Care for Cystic Fibrosis

As a nurse caring for a patient with cystic fibrosis, there are several different interventions that you should have in place. As a patient with cystic fibrosis, it is important that you recognize your needs and communicate them to your nurse and other members of your patient care team to ensure that you receive the care that you deserve.

One of the primary goals of treatment is to prevent and treat pulmonary infections. This can be accomplished by improving aeration/oxygenation, removing the thick secretions, and providing the patient with antibiotic medications. A primary concern for the nurse is the patient's airway. It is important that the patient's respiratory status is monitored and the airway is clear from secretions so that the patient can breathe as normally as possible. Lung sounds should be monitored and various ways of expelling mucus should be employed (including chest physiotherapy, flutter mucus clearance devices, and hand-held percussors/special percussor vests. It will be important to have oxygen available in the patient's room in case of a bad episode of decreased breathing and oxygenation.

The second area of focus for the nurse will be on the gastrointestinal system. The nurse should ensure that the child's nutritional needs are being met with a high-calorie, high-protein diet that is rich in many vitamins. The nurse should be monitoring daily weights and heights, since many children with CF tend to not grow as quickly. The nurse should also pay attention to any bowel movements that the patient has and listen to the bowel tones in the intestines to make sure there is no intestinal obstruction. The stool should be observed to determine how sticky and difficult to pass it may be. The nurse should teach the child to sit upright after eating to prevent the development of gastroesophageal acid reflux.

Some other areas the nurse should focus on include: checking blood glucose levels, providing emotional support to the parents/child, and educating the child/parents about the care involved. It is important that the nurse supports the patient as they undergo the different stages of childhood development. The nurse plays a very important role in preparing the parents and child for home care with cystic fibrosis. This includes education on different pieces of equipment used to manage the disease and education on signs and symptoms to watch for that may indicate a serious complication and the need for hospital care.

The table below summarizes what areas of focus the nurse should maintain during their care of the patient with cystic fibrosis.

System of Focus	Nursing Interventions
Respiratory System	-prevent and treat pulmonary infections -improve oxygenation -remove thick mucus secretions (chest physiotherapy, flutter device, percussion vests/hand percussion) -administer antibiotics -listen to lung sounds -have oxygen available in room
Gastrointestinal System	-ensure adequate nutrition of the patient -diet should be high-calorie, high-protein, and high in vitamins -monitor daily weight/height -watch for signs of constipation/intestinal obstructions -listen to bowel tones -observe stool for stickiness -prevent GERD by placing child upright after eating
Other	-monitor blood glucose levels -monitor bone growth -provide emotional support to parents and child -provide support to child as they undergo stages of childhood development -provide patient and parent education -prepare patient and parents for home-care

There are many helpful websites with information on the nursing care of the patient with cystic fibrosis. These websites are valuable resources for both nurse and the patient. It is important that the patient understand their diagnosis and can verbalize to the nurse what their needs are. Look at the websites below for more information. They all provide information on what areas of focus should be in nursing care, the importance of interdisciplinary collaboration to care for the patient with CF, and some information on CF centers that specialize in the care of cystic fibrosis patients. 

http://www.cysticfibrosis.org.uk/media/82046/nursing-management-standards-may01.pdf

http://nursing.advanceweb.com/Continuing-Education/CE-Articles/Cystic-Fibrosis.aspx

http://www.elsevier.com/__data/promis_misc/2005.pdf

References: 

Silvestri, L. (2014). Pediatric Respiratory Disorders. In Saunders comprehensive review for the NCLEX-RN examination (6th ed., pp. 482-483). St. Louis, Mo.: Elsevier/Saunders