Cystic fibrosis (CF) is an autosomal recessive genetic disorder that causes abnormalities in the secretory glands that produce mucus and sweat and mostly affects the lungs, pancreas, liver, intestines, and sex organs. The mucus that is produced in the body becomes thick and sticky. Instead of lubricating the lungs and other organs, it clogs the airways in the lungs and the ducts, most frequently in the pancreas and liver. Almost all males with CF are sterile and most females have a hard time getting pregnant. Symptoms can range from mild to severe. CF can lead to other diseases such as diabetes and osteoporosis. Screening for CF is routinely done on newborns and diagnosis is generally made before symptoms are evident, often within the first month of life.
Cystic Fibrosis is caused by a recessive defect in the CFTR gene. The CFTR gene makes a protein that regulates the movement of salt and water through the cells. The mutation causes the protein to either not work very well, or not be produced at all. When this happens, there is no regulation of salt and water across the cell membranes and mucus that the body produces becomes dehydrated and salty. The hallmark sign for this disease is a salty taste to the skin.
The patient will maintain adequate ventilation and respiratory status; the patient will have optimal nutritional status; the patient will be free from infection.
Cystic Fibrosis Nursing Care Plan
- Exercise intolerance
- Nasal congestion
- Abdominal pain
- Coughing, non-productive or with thick sputum
- Meconium ileus
- Failure to thrive
- Salty taste to skin
- Oily stools
- Abdominal distention
Nursing Interventions and Rationales
- Assess respiratory status; note rate, rhythm, and quality of breathing; auscultate lungs
CF patients get frequent respiratory infections because the thick mucus in the lungs traps bacteria and becomes infected.
- Review growth charts, assess changes or decrease in height or weight
Children may fail to thrive and lose weight or not grow properly because of malabsorption. The body does not absorb nutrients well enough to feed the cells adequately.
- Assess abdomen
- Look for distention
- Listen for active bowel sounds and gas pattern
- Feel – palpate for mass or signs of constipation
Lack of water regulation in the cells prevents adequate lubrication in the colon and often results in constipation. Newborns may have a meconium ileus that prevents the infant from passing meconium stools.
- Insert and monitor nasogastric feeding tubes
Feeding tubes may be placed to help with nutrition and absorption.
- Monitor nutritional status
- Caloric intake
- Excessive thirst or hunger
- Fiber intake
- Use of feeding tube
Children may have an adequate diet, but the body cannot absorb the nutrients. A blockage of the pancreas prevents digestive juices from being released into the intestines which aid in the absorption of nutrients.
CF-related diabetes is common due to the impairment of the pancreas
- Assess for signs of infection
- Monitor temperature
- Note cough with or without sputum
- Obtain sputum culture and sensitivity
Lung infections are common in patients with CF. Culturing the sputum can help determine bacteria involved and the course of treatment. Educate patients and caregivers that they should avoid sick contacts and stay away from other CF patients who may be ill, due to susceptibility to infections.
- Monitor for signs of dehydration and encourage fluid and salt intake
Patients with CF lose excess amounts of fluid and salt and can become dehydrated or develop hyponatremia quickly.
- Assess for bone malformation, deformity or fractures
Bones may become thin, the patient develops osteoporosis or osteopenia in the later stages. Fractures are common. Note clubbing, or widening, around the tips of the fingers and toes due to poor oxygenation of tissues
- Administer medications and supplements appropriately
- IV antibiotics may be given to treat infections.
- Antipyretics may be used to manage fever.
- Anti-inflammatories help to reduce swelling in the airways
- Inhaled steroids and bronchodilators may be used to decrease inflammation and open airways.
- Suction secretions as necessary per facility protocol
Thick mucus in the lungs and weakened muscles make it difficult for patients to expel secretions. Suctioning may be required.
- Perform chest physical therapy or vest therapy 2 – 4 times per day per facility protocol
This technique helps loosen mucus within the lungs making it easier to expel or suction
- Provide education for home treatment and resource information for patients and parents
- Educate parents on how to spot signs of infection and when to seek medical help.
- Encourage nutrition with high fiber foods and adequate fluid intake to prevent constipation.
- Teach the importance of supplements to ensure adequate absorption of nutrients.
- Provide information for support groups and resources as available.
Cornell Note-Taking System Instructions:
- Record: During the lecture, use the note-taking column to record the lecture using telegraphic sentences.
- Questions: As soon after class as possible, formulate questions based onthe notes in the right-hand column. Writing questions helps to clarifymeanings, reveal relationships, establish continuity, and strengthenmemory. Also, the writing of questions sets up a perfect stage for exam-studying later.
- Recite: Cover the note-taking column with a sheet of paper. Then, looking at the questions or cue-words in the question and cue column only, say aloud, in your own words, the answers to the questions, facts, or ideas indicated by the cue-words.
- Reflect: Reflect on the material by asking yourself questions, for example: “What’s the significance of these facts? What principle are they based on? How can I apply them? How do they fit in with what I already know? What’s beyond them?
- Review: Spend at least ten minutes every week reviewing all your previous notes. If you do, you’ll retain a great deal for current use, as well as, for the exam.
For more information, visit www.nursing.com/cornell