cystic fibrosis

Cystic Fibrosis Nursing Diagnosis Care Plan

Cystic Fibrosis Nursing Diagnosis Care Plan NCLEX Review

Nursing Study Guide: Cystic Fibrosis

Cystic fibrosis (CF) is a genetic disorder that primary affects the lungs and the digestive system. CF involves a defective gene that triggers the body to secrete mucus via exocrine glands. This causes digestive juices and sweat in a thick and sticky consistency.

Normally, these secreted liquids are used by the body as lubricants, but if they become thick, they block the ducts, passageways, and tubes.

These result to lung and pancreatic problems. CF has no cure and is progressive in nature, so the treatment is mostly focused on the control of symptoms and the prevention of infections and other complications.

Signs and Symptoms of Cystic Fibrosis

  • chronic productive cough
  • wheezing
  • nasal congestion and/or inflammation
  • activity intolerance
  • recurrent lung infections
  • Recurrent sinusitis
  • greasy and foul stools
  • growth retardation in children
  • poor weight gain
  • chronic or severe constipation which may cause rectal prolapse
  • intestinal blockage (meconium ileus in newborn babies)

Causes and Risk Factors of Cystic Fibrosis

CF is caused by the mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This gene is associated with the change in the protein that is responsible for controlling the movement of salt to and from the cells.

Cystic Fibrosis is an autosomal recessive disorder meaning that the patient received one mutated gene from each parent. Usually, parents who are carriers do not have signs and symptoms for CF.

With the increased level of salt in sweat, sticky and thick mucus in the respiratory, digestive, and reproductive organs can occur.

Some Risk factors for CF also include family history of CF and North European people.

Complications of Cystic Fibrosis

Respiratory problems: CF can cause bronchiectasis, a long-term lung condition wherein there is scarring and widening of the bronchial tubes, causing difficulty of breathing and productive cough with clear mucus.

CF can also lead to chronic respiratory infections, as thick mucus can be a breeding ground for pathogens such as bacteria and fungi.

Pneumonia, bronchitis, and sinusitis can be recurrent in patients with CF. Because of the chronic and recurrent nature of the infections, the bacteria may eventually become antibiotic resistant.

Other respiratory issues include nasal polyps, acute exacerbation, pneumothorax, and hemoptysis or coughing up of blood. The most common cause of death of CF patients is respiratory failure.

Digestive problems: Nutritional deficiency is common with CF patients due to the blockage of the tubes that connect the pancreas to the intestines.

The pancreatic enzymes could not reach the intestines, causing the proteins, fats, and vitamins to be unabsorbed.

This results in undernourishment and delayed growth in children, as well as weight loss and pancreatic inflammation.

Patients with CF may also develop diabetes due to the inability of the insulin to travel from the pancreas due to block tubes.

Liver and gallbladder problems may also occur due to blockage and inflammation of the bile’s passageway from these organs to the small intestine.

Intestinal obstruction can also happen in CF patients.

Infertility: Most men with CF are infertile because of the blockage of the vas deferens, but surgical procedure may help to fix this reproductive problem.

Women with CF may have reduced fertility, but some can still conceive and experience successful pregnancy.

Other complications can include:

Dehydration and electrolyte imbalance

Osteoporosis and arthritis

Mental health challenges

Nursing Tip:

Remember that Cystic Fibrosis is an exocrine gland dysfunction which affects:

  1. Bronchi of the lungs: leading to bronchial obstruction causing chronic bronchial pneumonia, obstructive emphysema, and pneumothorax.
  2. Pancreatic Ducts: leading to malabsorption syndromes.
  3. Small Intestine: leading to intestinal obstructions in newborns.
  4. Bile Ducts: leading to portal hypertension.

Diagnosis of Cystic Fibrosis

Newborn screening – a blood test that shows high levels of the chemical immunoreactive trypsinogen (IRT) indicate the need for a confirmatory diagnosis of CF; sweat test for infants aged 2 weeks old and above to check for salt levels in the sweat; genetic testing may also be done.

Physical examination – checking for presence of nasal polyps or signs of lung problems; imaging tests such as Xray, MRI, and CT scan may be done to help with the diagnosis

Treatment for Cystic Fibrosis

Medications: There is no cure for CF, so the treatment is usually focused on symptom control and infection prevention. Antibiotics may be given to treat recurrent lung infections.

Mucolytics may be prescribed to thin out the mucus and help improve lung function. Bronchodilators can help with breathing problems by keeping the airway open.

Anti-inflammatory drugs can be used to reduce swelling of the airways.

Other symptom-control medications include stool softeners, antacids, and diabetic medications as indicated.

The doctor may also prescribe targeted medication called Tikafta, a combination of three drugs (elexacaftor, ivacaftor, and tezacaftor), to target gene mutations in CF patients age 12 years and older.

Chest physical therapy (CPT): Also known as airway clearance techniques, CPT can help loosen the thick mucus in the lungs, reduce inflammation in the airways, and lower the risk of pulmonary infections.

Pulmonary rehabilitation: This is a long-term program that focuses on the improvement of the CF patient’s lung function, nutrition, and overall health.

Surgical procedures: A CF patient who has developed severe pulmonary disorders (such as bronchiectasis) or respiratory failure may require a lung transplant.

Nasal polyps can be removed surgically.

Diet: CF patients are given a high calorie high protein diet to meet energy and growth needs. Vitamins A,D,E, and, K are needed to replace the deficiency of fat soluble vitamins. Deficiency of these vitamins can cause anemia, bleeding, and bruising.

Nursing Care Plans for Cystic Fibrosis

Nursing Care Plan 1

Nursing Diagnosis: Impaired Gas Exchange related to airway and alveolar inflammation secondary to cystic fibrosis, as evidenced by shortness of breath, oxygen saturation of 82%, restlessness, and reduced activity tolerance

Desired Outcome: The patient will demonstrate adequate oxygenation as evidenced by an oxygen saturation within the target range as set by the physician/ respiratory team.

InterventionsRationales
Assess the patient’s vital signs, especially the respiratory rate and depth. Auscultate the lungs and monitor for wheezing or other abnormal breath sounds.To create a baseline set of observations for the CF patient, and to monitor any changes in the vital signs as the patient receives medical treatment.
Monitor the color of skin and mucous membranes.Peripheral cyanosis (bluish discoloration of the skin, ear lobes, or nail beds) may be evident with hypoxemia. Central cyanosis involving the mucosa may indicate further reduction of oxygen levels.
Encourage the patient to cough to expectorate thick sputum. Suction as needed.Impaired small airways experience impaired gas exchange primarily due to thick, tenacious mucoid secretions. The patient may be unable to cough the phlegm, therefore deep suctioning may be required.
Provide humidified oxygen as prescribed.To reduce the risk of drying out the lungs.
Reposition the patient by elevating the head of the bed and encouraging him/her to sit on an upright position. Encourage pursed lip breathing and deep breathing exercises.To improve the delivery of oxygen in the airways and to reduce shortness of breath and risk for airway collapse.
Refer the patient to a chest physiotherapist/ respiratory therapist.To enable to patient to receive more information and specialized care in the removal of thick lung secretions and enabling of improved gas exchange.

Nursing Care Plan 2

Ineffective Airway Clearance related to increased mucopurulent production secondary to cystic fibrosis, as evidenced by shortness of breath, wheeze, SpO2 level of 85%, chronic productive cough, respiratory rate of 24, difficulty to expectorate phlegm

Desired Outcome: The patient will be able to maintain airway patency and improved airway clearance as evidenced by being able to expectorate phlegm effectively, respiratory rates between 12 to 20 breaths per minutes, oxygen saturation within the target range (as set by the physician), and verbalize ease of breathing.

InterventionsRationales
Assess the patient’s vital signs and characteristics of respirations at least every 4 hours. Assess breath sounds via auscultation.To assist in creating an accurate diagnosis and monitor effectiveness of medical treatment. Wheeze is one of the signs of ineffective airway clearance related to cystic fibrosis.
Suction secretions.To help clear thick phlegm that the patient is unable to expectorate.
Administer supplemental oxygen, as prescribed. Discontinue if SpO2 level is above the target range, or as ordered by the physician.To increase the oxygen level and achieve an SpO2 value within the target range.
Administer the prescribed medications (e.g. bronchodilators, steroids, or combination inhalers / nebulizers) and antibiotic medications.Mucolytics: To thin out the mucus and help improve lung function. Bronchodilators: To dilate or relax the muscles on the airways. Steroids: To reduce the inflammation in the lungs. Antibiotics: If there is any bacterial infection that is causing the inflammation
Elevate the head of the bed and assist the patient to assume semi-Fowler’s position.Head elevation and proper positioning help improve the expansion of the lungs, enabling the patient to breathe more effectively.
Refer to a respiratory therapist/ chest physiotherapist as needed.To provide specialized respiratory care to the patient.

Other Nursing Diagnoses:

  • Imbalanced Nutrition: Less Than Body Requirements
  • Deficient Knowledge
  • Infection

References:

Ackley, B. J., Ladwig, G. B., Makic, M. B., Martinez-Kratz, M. R., & Zanotti, M. (2020). Nursing diagnoses handbook: An evidence-based guide to planning care. St. Louis, MO: Elsevier.

Gulanick, M., & Myers, J. L. (2017). Nursing care plans: Diagnoses, interventions, & outcomes. St. Louis, MO: Elsevier.     

Ignatavicius, D. D., Workman, M. L., Rebar, C. R., & Heimgartner, N. M. (2018). Medical-surgical nursing: Concepts for interprofessional collaborative care. St. Louis, MO: Elsevier.

Silvestri, L. A. (2020). Saunders comprehensive review for the NCLEX-RN examination. St. Louis, MO: Elsevier.

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