Cystic fibrosis (CF) is a life-threatening genetic disorder that affects the lungs and digestive system in more than 30,000 Americans. It is characterized by permanent lung damage, pancreas, liver, and intestinal tract damage. While there is no cure for CF, effective interventions are available to improve quality of life and reduce lifespan risk factors associated with this condition.
To ensure maximal patient outcomes and improved quality of life, doctors and nurses must master appropriate nursing interventions and develop an individualized care plan based on each patient’s unique needs.
This post will explore different acute/long-term treatment strategies best suited for patients struggling with CF, along with beneficial nursing approaches to prevent major medical crises.
Table of Contents
Signs and Symptoms of a Patient
Patients with CF may have a range of signs and symptoms. Common symptoms include:
- Difficulty in breathing
- Abnormal growth patterns
- Persistent coughing
- Frequent lung infections
- Poor nutrition due to malabsorption of nutrients in the intestines can lead to slow weight gain or even weigh loss
- Salty-tasting skin.
Additionally, individuals may experience greasy stools, abdominal pain/bloating, male infertility due to clogged sperm ducts, and increased sweat production.
Pathology Of Cystic Fibrosis
CF is caused by a genetic mutation in the CFTR gene, which produces an abnormal protein that interferes with chloride transport throughout the body. As a result, organs such as the lungs and pancreas become thickened with mucus deposits, leading to symptoms of respiratory distress, digestive problems, and other health complications.
The most basic classification system divides the disorder into acute and chronic forms.
- Acute pancreatitis. Acute pancreatitis does not usually lead to chronic pancreatitis unless complications develop.
- Chronic pancreatitis. Chronic pancreatitis is an inflammatory disorder characterized by progressive destruction of the pancreas.
What Happens in Cystic Fibrosis?
In cystic fibrosis, the body produces an abnormal form of the CFTR protein or none. As a result, the cells that line the passages (tubes) inside some organs produce thick, sticky mucus instead of thin, watery mucus. This can cause bacteria to become trapped in the lungs, leading to infection, inflammation, and difficulty breathing.
Additionally, it blocks digestive enzymes from flowing between the pancreas and intestines, which makes it harder for children to digest food and absorb vital vitamins and nutrients. Thick mucus also affects other organs, including the liver, sweat glands, and reproductive organs.
Treatment for cystic fibrosis is often divided into two categories: acute treatments and long-term treatments. Acute treatments for CF include antibiotic therapy, chest physiotherapy, mucus thinning medications, and other therapies used to treat symptoms as needed.
Long-term treatments for CF seek to address the disorder’s underlying cause, aiming to slow down or stop further damage to the body. These treatments include enzyme replacement therapy, gene therapy, and nutritional supplementation.
The cause of cystic fibrosis is a genetic mutation in the CFTR gene, which produces an abnormal protein that interferes with chloride transport throughout the body. This results in organs such as the lungs and pancreas becoming thickened with mucus deposits, leading to symptoms of respiratory distress, digestive problems, and other health complications.
The patient admitted to the hospital with a diagnosis of pancreatitis is acutely ill and needs expert nursing care.
Nursing assessment of a patient with pancreatitis involves:
- Evaluation of current nutritional status and increased metabolic requirements.
- Assessment of respiratory condition.
- Assessment of fluid and electrolyte status.
- Assessment of sources of fluid and electrolyte loss.
- Assessment of abdomen for ascites.
Cystic Fibrosis Nursing Diagnosis
An evaluation of the patient’s condition reveals several nursing diagnoses for cystic fibrosis, such as:
Acute pain: caused by swelling in the pancreas, enlargement, and irritation in the peritoneum;
Inadequate nutrition: Because of diminished dietary intake, disruption of pancreatic secretions, amplified nutritional needs; and
Ineffective breathing patterns: Due to extreme pain, lung infiltrates, fluid buildup around the lungs
Nursing care plan for cystic fibrosis & Goals
The nursing plan of care should be individualized to the patient based on the type and severity of pancreatitis.
Goals for a patient with acute pancreatitis may include relief from pain, improved nutritional status, decreased risk factors for infection, and improved respiratory rate.
For a patient with chronic pancreatitis: goals may include symptom management, lifestyle modification, and diet to reduce pain and improve nutrition and coping strategies.
Cystic Fibrosis Nursing Interventions
Performing nursing interventions for a patient with pancreatitis needs expertise and efficiency.
Relieve pain and discomfort: Current guidelines for pain control in this group of people advise administering parenteral opioids, such as morphine, hydromorphone, or fentanyl, either through patient-controlled analgesia (PCA) or bolus.
Promote proper breathing pattern: It is essential to keep the patient in a semi-Fowler’s position and frequently change their position.
Enhance nutritional status: They should be given a diet high in carbohydrates and low in fat and protein between episodes of acute illness.
Protect skin integrity: Per medical orders, the nurse performs wound care and takes steps to guard intact skin against contact with drainage.
The nurse must incorporate various interventions to meet the patient’s needs. Specific nursing interventions for pancreatitis may include:
- Monitoring vital signs as indicated
- Administering medications as prescribed by a physician
- Maintaining adequate hydration with IV fluids
- Administering enteral or parenteral nutrition
- Utilizing airway clearance techniques to maintain adequate oxygenation and ventilation
- Assisting with nerve blocks or epidural anesthesia to provide pain relief
- Educating the patient on disease process, treatments, and management options
- Encouraging relaxation to reduce stress levels
Evaluating & Reassessing
Nurses must evaluate the patient’s response to treatment, reassess symptoms regularly, and modify care plans as necessary. Patient education should also be included in the evaluation of care. The nurse should assess whether the patient understands their condition, changes made in lifestyle due to their diagnosis, medications prescribed, side effects, and any other factors that may contribute to their care.
Overall, nursing management of cystic fibrosis requires competent care delivered by well-trained nurses to ensure maximal patient outcomes and improved quality of life. With appropriate acute/long-term treatment strategies and beneficial nursing approaches to prevent major medical crises, CF patients can enjoy a better quality of life for extended periods.
Mrs. Marie Brown has been a registered nurse for over 25 years. She began her nursing career at a Level I Trauma Center in downtown Chicago, Illinois. There she worked in the Emergency Department and on the Surgical Intensive Care Unit. After several years, she moved to the Midwest and continued her nursing career in a critical care setting. For the last 10 years of her nursing career, Mrs. Brown worked as a flight nurse with an air ambulance service. During this time, she cared for patients throughout the United States.