Cystic Fibrosis Nursing Care Plans Diagnosis and Interventions
Cystic Fibrosis NCLEX Review and Nursing Care Plans
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
- 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.
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
Mental health challenges
Remember that Cystic Fibrosis is an exocrine gland dysfunction which affects:
- Bronchi of the lungs: leading to bronchial obstruction causing chronic bronchial pneumonia, obstructive emphysema, and pneumothorax.
- Pancreatic Ducts: leading to malabsorption syndromes.
- Small Intestine: leading to intestinal obstructions in newborns.
- 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 Diagnosis for Cystic Fibrosis
Nursing Care Plan for Cystic Fibrosis 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.
|Cystic Fibrosis Nursing Interventions||Rationales|
|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 for Cystic Fibrosis 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.
|Cystic Fibrosis Nursing Interventions||Rationales|
|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.|
Nursing Care Plan for Cystic Fibrosis 3
Imbalanced Nutrition: Less than Body Requirements
Nursing Diagnosis: Imbalanced Nutrition Less than Body Requirements related to chronic malabsorption secondary to cystic fibrosis as evidenced by abnormal stool patterns, hypoalbuminemia and deficit of 10 to 20% of ideal body weight.
- The patient will be able to demonstrate healthy weight gain and maintenance of adequate nutritional status.
- The patient will be free from clinical manifestations of malabsorption.
|Cystic Fibrosis Nursing Interventions||Rationale|
|Evaluate the patient’s gastrointestinal status. Assess the abdomen for bowel sounds, presence of bloating and palpable mass. Take note of the stool characteristics such as frequency, odor, consistency, presence of grease, etc.||Malabsorption issues are common in patients with cystic fibrosis. It is important that medication adjustments for pancreatic enzymes are done accordingly so as to prevent under treatment and normalization by patients and family of the manifestations.|
|Evaluate the patient’s skin. Take note of the color, integrity and turgor.||Deviations in the patient’s physiology is apparent, especially in the skin, for patients suffering from cystic fibrosis related malnutrition. Due to the nature of the condition, patient’s are also prone to develop jaundice when the liver is compromised that will exhibit the appearance of yellowish discolorations.|
|Observe any increase in the patient’s weight and appetite.||Upward trends in the patient’s weight and appetite towards food would signify a better health outlook for patients with cystic fibrosis due to the lifting of previously experienced respiratory burden brought about by excessive secretions.|
|Observe the presence of excessive thirst (polydipsia), urination (polyuria) and hunger (polyphagia). Anticipate monitoring of capillary blood glucose (CBG) readings as indicated.||Polydipsia, polyuria and polyphagia are manifestations associated with diabetes. Statistically, diabetes can occur up to 30% of patients with cystic fibrosis (cystic fibrosis). Likewise, cystic fibrosis associated pulmonary exacerbations may occur interchangeably with patients having blood glucose issues.|
|Keep watch of the patient’s serum chemistry results as ordered.||Alterations in the patient’s laboratory values are apparent with cystic fibrosis. Hypoalbuminemia, increased liver enzyme levels, hyperglycemia and electrolyte imbalances can all affect the patient and present as complications for patients with cystic fibrosis.|
|Motivate the patient to increase fluid and fiber intake in the diet.||Patients with cystic fibrosis are prone to develop chronic constipation due to the resulting dehydration in the intestines and the associated excessive mucus secretion. Adequately managing the patient’s malabsorption issues and ensuring that stool consistency is normalized would decrease the risk for cystic fibrosis associated constipation.|
|Encourage the patient to increase salt intake in the diet.||Patients with cystic fibrosis are prone to develop hyponatremic dehydration wherein the body is losing salt easily due to the associated defects. Liberal salt usage will help address these issues and prevent complications of hyponatremia such as seizures, etc.|
|Advise the patient to have a high protein, high-calorie diet as tolerated.||Patients with cystic fibrosis can have caloric needs of 1.2 to 1.5 of their recommended daily intakes. A high protein, high-caloric diet ensures that physiologic demands are met despite the burden of the condition.|
|Ensure that fat-soluble vitamins are administered with meals and enzymes.||In cystic fibrosis, fat malabsorption is common thus making fat-soluble vitamins improbable to be properly absorbed by the body. Because of this, fat-soluble vitamin deficiencies are prominent and would need assistance by using supplemental pancreatic enzymes for better absorption.|
|Ensure that pancreatic enzymes are administered before meals and snacks, especially those with fat and protein.||Patients with cystic fibrosis are unable to fully digest food rich in protein or fat as a complication of the disease. Ensuring that pancreatic enzyme supplementation are utilized will ensure that the patient will be able to digest and absorb these macronutrients.|
|Seek the professional assistance of a registered dietician in acquiring a complete nutritional work-up.||cystic fibrosis patients are commonly diagnosed with chronic nutrition issues. Careful and multidisciplinary collaboration would ensure that patient needs are met and interventions are timely given to prevent further complications and worsening of the disease.|
Nursing Care Plan for Cystic Fibrosis 4
Nursing Diagnosis: Deficient Knowledge related to ineffective education in the past secondary to cystic fibrosis as evidenced by anxiety related to hospitalization and verbalization of misconceptions
Desired Outcome: The patient and his significant others will be able to verbalize understanding of the disease process, necessity of the treatment regimen and importance of adherence to goals of therapy.
|Cystic Fibrosis Nursing Interventions||Rationale|
|Evaluate the patient’s core knowledge of the disease as it impacts his life and well being.||Cystic fibrosis (CF) is a condition affecting the secretory glands wherein it compromises the control and production of the necessary enzymes for proper physiology of the various organ systems, such as the lungs and pancreas. Recognizing the learner’s core knowledge is crucial for the formulation of an applicable therapeutic regimen.|
|Evaluate the patient’s core knowledge regarding his nutritional needs||Patients with cystic fibrosis oftentimes have chronic nutritional issues. Having the patient evaluated on his knowledge regarding this will assist in formulating applicable interventions and the promotion of self-management by the patient.|
|Evaluate the patient’s core knowledge regarding his infection control needs for his condition.||Patients with cystic fibrosis are highly susceptible in developing opportunistic infections. Assessing the knowledge base of the patient will help in developing instructions that are geared towards preventing infections and promoting self care.|
|Evaluate the patient’s core knowledge on the genetics of cystic fibrosis.||Cystic fibrosis happens when there is a defect on the person’s transmembrane conductance regulator (CFTR) gene. This gene is responsible for the production of proteins that controls the movement of water and salt in and out of the cells. Having one parent with the condition will predispose their children in developing cystic fibrosis.|
|Evaluate the patient’s core knowledge on the medications, therapies and regimen, especially respiratory interventions.||Treatment regimens for cystic fibrosis vary and are oftentimes difficult to maintain. Ensuring that routine assessment of the patient’s knowledge is crucial in identifying gaps and addressing these timely through appropriate interventions.|
|Evaluate the patient’s core knowledge of the diagnostic methods (e.g., laboratory, pulmonary function tests, cultures and sensitivity for pathogens.)||It is crucial that the patient comprehends the importance of his baseline pulmonary capacities against opportunistic respiratory pathogens in order to fully understand the treatment goals and interventions for his condition.|
|Educate the patient with basic information regarding the pathology and treatment goals of cystic fibrosis.||Cystic fibrosis is a complex disease that still has no direct cure. In relation to this, goals of treatment include prevention of lung infections, removal of thick mucus in lungs, prevention of blockage in the intestines, nutrition enhancement and lowering of dehydration risk. The patient made aware of these treatment goals ensures careful self management and tenacious medical regimen that will enable cystic fibrosis clients to live longer.|
|Educate the patient of support groups for cystic fibrosis patients.||It is challenging and emotionally draining for patients with chronic diseases, such as cystic fibrosis, to live with the condition. The patient made aware of support groups will enable better care management due to the opportunities to share common experiences to people with the same condition.|
|Ensure to review respiratory function tests and baseline data trends of the patient.||Since cystic fibrosis patients are more likely to experience pulmonary issues, having a grasp of the baseline data will act as a guide on the progression or improvement of the condition.|
|Ensure to review breathing methods to remove mucus in airway passages.||Cystic fibrosis patients are prone to develop pulmonary issues. Due to this, chest physiotherapy is one method for ensuring that tenacious secretions are removed from the airways. However, most clients oftentimes find difficulties in performing such strategies therefore would need constant readjustments of techniques learned for better care management.|
|Ensure to review infection control techniques with the patient.||The healthcare team is responsible for providing and ensuring that the patient and his significant others are made aware of the infection control methods in relation to cystic fibrosis due to the high susceptibility to opportunistic infections.|
|Ensure to review with the patient his pulmonary and nutritional medications with regards to his condition.||Complex medication protocols accompany patients with cystic fibrosis and would necessitate frequent review in order to stress the importance of compliance. Respiratory medications utilized may consist of antibiotics, bronchodilators, steroids and mucus thinners. Nutritional therapies may include pancreatic enzymes, vitamin supplementation and salt additives.|
Nursing Care Plan for Cystic Fibrosis 5
Risk for Infection
Nursing Diagnosis: Risk for Infection related to the potential of acquisition of new pathogens commonly associated with cystic fibrosis.
Desired Outcome: The patient will be able to show improvement in infection as evidenced by decrease in cough episodes, improvement in respirations, lowering of body temperature and normalization of white cell blood counts.
|Cystic Fibrosis Nursing Interventions||Rationale|
|Evaluate for clinical manifestations of infection such as fever, cough, tachypnea, tachycardia, increased sputum production, etc.||Respiratory infections often exhibit manifestations associated with general infection symptoms. Determining these early on will help in planning for timely interventions for the condition.|
|Make sure to obtain sputum for culture and sensitivity testing as ordered.||Acquiring new pathogens is often associated with cystic fibrosis related respiratory exacerbations. Recognizing common pathogens related to cystic fibrosis infections such as staphylococcus aureus, pseudomonas aeruginosa, methicillin-resistant staphylococcus aureus (MRSA), and other pathogens will enable for guided antibiotic therapy to address the infection effectively.|
|Observe the patient’s oxygen saturation levels.||Oxygen requirements increase as respiratory infections arise for patients with cystic fibrosis. Ensuring that saturation levels are monitored continuously through pulse oximetry can alert the healthcare team in recognizing early oxygen desaturations that can impact survival rates.|
|Take note of the patient’s immunoglobulin E (IgE) as ordered.||Cystic fibrosis associated respiratory exacerbations can develop to allergic bronchopulmonary aspergillosis (ABPA). ABPA is an allergic response by the airways in relation to aspergillus exposure. Deviations in the patient’s IgE levels are significant in determining allergic components in the condition.|
|Take note of pending culture and sensitivity results and presence of associated antibiotic resistance.||Monitoring for antibiotic resistance would indicate changes in the therapeutic regimen. The presence of a new organism will affect the infection control principles in the patient’s care.|
|Observe and take note of viral studies as ordered.||Viral infections can further worsen respiratory issues as a response of the body suffering from cystic fibrosis.|
|Take note of the patient’s white blood cell levels as ordered.||Elevated white blood cell counts occur on worsening respiratory issues or the presence of infection from a new causative pathogen.|
|Observe the patient’s antibiotic trough levels as ordered.||Inadequate drug dosages may result in ineffective therapeutic regimen and the development of antibiotic resistance. Excesses can result in drug toxicity that can lead to adverse events and compromised health outcomes.|
|Anticipate administration of antipyretics as ordered. Take note to avoid giving non steroidal anti-inflammatory drugs (NSAIDS) in clients given intravenous aminoglycosides.||Antipyretics are types of medications utilized to control fever and reduce metabolic needs. Combining aminoglycosides with NSAIDS can result in kidney toxicity.|
|Render antibiotics as ordered and ensure to administer them within the prescribed schedules.||Timing in antibiotic therapy is crucial. The patient receiving antibiotics within the first 4 hours of admission has been considered the standard to effectively address infections.|
|Make sure that patients with cystic fibrosis receive care as per their individualized care plans.||Cohorting a treatment regimen for cystic fibrosis patients is not ideal for each individual and presents with unique health challenges.|
|Anticipate consultation with an audiology expert, especially for patients with antibiotic history of repeated use of intravenous aminoglycosides.||One of the common adverse effects of aminoglycosides is ototoxicity. Recognizing hearing changes early on will prevent development of complications. It will also aid the healthcare team to consider other antibiotics to avoid debilitating health effects.|
|Comply with the prescribed infection control protocols for patients with cystic fibrosis.||Patients with cystic fibrosis associated infections would usually require special isolation precautions prescribed by the Centers for Disease Control and Prevention (CDC) and Cystic Fibrosis Foundation (CFF) Consensus Guidelines. This is due to the fact that cystic fibrosis patients are prone in developing complicated infections that would require special precautions in their care management.|
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