Nursing Care Plan for Cystic Fibrosis

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Pathophysiology

Cystic fibrosis (CF) is an autosomal recessive genetic disorder which 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.

Etiology

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.

Desired Outcome

Patient will maintain adequate ventilation and respiratory status; patient will have optimal nutritional status; patient will be free from infection.

Cystic Fibrosis Nursing Care Plan

Subjective Data:

  • Exercise intolerance
  • Nasal congestion
  • Constipation
  • Abdominal pain

Objective Data:

  • 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 adequate diet, but the body cannot absorb the nutrients. A blockage of the pancreas prevents digestive juices from being released into the intestines which aids in absorption of nutrients.

 

  • Monitor blood glucose

 

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 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, 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.

 


References

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