Cystic fibrosis is an autosomal recessive genetic disorder that is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR) present in the mucus-secreting cells of the body, primarily affecting the respiratory, reproductive, and gastrointestinal tracts.
The hallmark manifestation of CF is chronic, progressive lung disease, resulting from secretion of dehydrated mucus with airway obstruction, and malnutrition from pancreatic insufficiency. However, there is a wide range of disease variety. Pulmonary symptoms can range from nearly asymptomatic disease with undetectable changes in the lung function to severe obstructive disease early in childhood, whereas gastrointestinal symptoms may range from mild constipation, normal liver function, and intermittent pancreatitis to severe loss of pancreatic function, malabsorption with subsequent malnutrition, CF-related diabetes, and end-stage liver diseases.
The sweat glands and reproductive glands are also affected; almost all males with CF are sterile because of absence or impairment of vas deferens, whereas females have reduced fertility. The impact on the sweat gland give rise to abnormally high chloride secretion; a simple analysis of sweat chloride content continues to be the gold standard for CF diagnosis.
Nursing Care Plans
Nursing care plan for clients with cystic fibrosis includes maintaining adequate oxygenation, promoting measures to remove pulmonary secretions, emphasizing the importance of adequate fluid and dietary intake, ensuring adequate nutrition, and preventing complications.
- Impaired Gas Exchange
- Ineffective Airway Clearance
- Imbalanced Nutrition: Less Than Body Requirements
- Deficient Knowledge
Ineffective Airway Clearance
May be related to
- Increased mucopurulent production and mucous plugging in response to new or increased airway bacterial growth.
Possibly evidenced by
- Abnormal chest x-ray film
- A decline in pulmonary function testing (spirometry)
- An ineffective cough
- Chest pain
- Crackles, rhonchi
- Labored breathing
- Reduced breath sounds
- Client will be proficient in using effective airway clearance therapies to clear secretions on a daily basis, as evidenced by decreased work of breathing and improved pulmonary function.
- Client will maintain clear, open airway as evidenced by normal breath sounds, normal rate and depth of respirations, and an airway free of secretions, with an effective cough.
|Assess cough for effectiveness.||Habitual cough suppression is common in clients with cystic fibrosis; an effective mucus-clearing is important for adequate airway clearance.|
|Assess respiratory rate, work of breathing, use of accessory muscles, and the presence of retractions.||Infection, inflammation, and mucous plugging will cause an increase in the respiratory effort to compensate for airway obstruction. As moving air into and out of the lungs becomes more difficult, the breathing pattern alters to include the use of accessory muscles and retractions.|
|Assess the chest wall for even chest expansion.||Unequal chest expansion can indicate a pneumothorax– a complication of cystic fibrosis.|
|Auscultate lung for adventitious sounds, and the degree of aeration.||Crackles, rhonchi, and decreased aeration signify an ongoing infection and inflammation.|
|Assess sputum for color, amount, and consistency.||A decreasing amount and frequency of sputum production, lighter color, and thinner consistency of sputum indicate improvement in exacerbation. Scant amounts or streaking of blood in the sputum can occur from airway inflammation; however, major hemoptysis can occur and is a life-threatening emergency.|
|Monitor oxygen saturation.||Hypoxemia may result from impaired gas exchange and from the buildup of secretions and bronchial constriction.|
|Monitor pulmonary function testing.||Improvement in spirometry signifies improved airflow and aeration, effective airway clearance, and treatment of infection.|
|Assess the client’s comfort level with and adherence to recommended airway clearance therapy (ACT).||Client satisfaction and proficiency with the selected ACT regimen is essential for adherence.|
|Assess for barriers to ongoing airway clearance.||The identification of barriers to ACT can guide interventions to improve adherence.|
|Provide the opportunity for exercise and physical therapy.||Exercise supports the goal of airway clearance by loosening the mucus and facilitating effective coughing. It also improves overall physical conditioning and recovery.|
|Encourage a frequent and effective cough- particularly around airway clearance therapy (ACT).||Habital cough suppression results in the retention of mucus; clients should be encouraged to actively and intentionally use effective coughing to clear airway mucus.|
|Administer bronchodilators before ACT as ordered.||Bronchodilators are administered to treat hyperactivity and to reverse bronchospasm.|
|Administer mucolytics as ordered in concert with ACT.||Mucolytics decrease the viscosity of the sputum and promote expectoration of secretions.|
|Administer pain medication as needed.||Attention to pain relief facilitates the client being able to cooperate comfortably with ACT and exercise/physical therapy.|
|Collaborate with the client and staff to determine the preferred ACT. Options include:
||The Cystic Fibrosis Transmembrane Conductance Regulator defect causes mucus to become dehydrated. Secretions in cystic fibrosis are generally thick, sticky, and more difficult to clear. Frequent airway clearance is a mainstay in the treatment of acute exacerbations, as well as an integral part of health maintenance in cystic fibrosis. Individualized care and client choice in therapy will affect adherence and the effectiveness of treatment. A variety of supportive devices is available. A respiratory therapist can be helpful in these selections.|
|Collaborate with the client and staff to ensure that the schedule for therapy is amenable to all and does not interfere with meals, rest times, or medications.||ACT is time-consuming and must be part of a balanced treatment plan. ACT should not be done immediately after meals, nor should it interfere with rest or other required therapies such as medication and physical therapy; it therefore requires careful scheduling.|
Recommended nursing diagnosis and nursing care plan books and resources.
- Nursing Care Plans: Nursing Diagnosis and Intervention (10th Edition)
An awesome book to help you create and customize effective nursing care plans. We highly recommend this book for its completeness and ease of use.
- Nurse’s Pocket Guide: Diagnoses, Prioritized Interventions and Rationales
A quick-reference tool to easily select the appropriate nursing diagnosis to plan your patient’s care effectively.
- NANDA International Nursing Diagnoses: Definitions & Classification, 2021-2023 (12th Edition)
The official and definitive guide to nursing diagnoses as reviewed and approved by the NANDA-I. This book focuses on the nursing diagnostic labels, their defining characteristics, and risk factors – this does not include nursing interventions and rationales.
- Nursing Diagnosis Handbook, 12th Edition Revised Reprint with 2021-2023 NANDA-I® Updates
Another great nursing care plan resource that is updated to include the recent NANDA-I updates.
- Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5(TM))
Useful for creating nursing care plans related to mental health and psychiatric nursing.
- Ulrich & Canale’s Nursing Care Planning Guides, 8th Edition
Claims to have the most in-depth care plans of any nursing care planning book. Includes 31 detailed nursing diagnosis care plans and 63 disease/disorder care plans.
- Maternal Newborn Nursing Care Plans (3rd Edition)
If you’re looking for specific care plans related to maternal and newborn nursing care, this book is for you.
- Nursing Diagnosis Manual: Planning, Individualizing, and Documenting Client Care (7th Edition)
An easy-to-use nursing care plan book that is updated with the latest diagnosis from NANDA-I 2021-2023.
- All-in-One Nursing Care Planning Resource: Medical-Surgical, Pediatric, Maternity, and Psychiatric-Mental Health (5th Edition)
Definitely an all-in-one resources for nursing care planning. It has over 100 care plans for different nursing topics.
Other recommended site resources for this nursing care plan:
- Nursing Care Plans (NCP): Ultimate Guide and Database
Over 150+ nursing care plans for different diseases and conditions. Includes our easy-to-follow guide on how to create nursing care plans from scratch.
- Nursing Diagnosis Guide and List: All You Need to Know to Master Diagnosing
Our comprehensive guide on how to create and write diagnostic labels. Includes detailed nursing care plan guides for common nursing diagnostic labels.
Other nursing care plans related to respiratory system disorders:
- Asthma | 8 Care Plans
- Bronchiolitis | 5 Care Plans
- Bronchopulmonary Dysplasia (BPD) | 5 Care Plans
- Chronic Obstructive Pulmonary Disease (COPD) | 7 Care Plans
- Cystic Fibrosis | 5 Care Plans
- Hemothorax and Pneumothorax | 3 Care Plans
- Influenza (Flu) | 5 Care Plans
- Lung Cancer | 5 Care Plans
- Mechanical Ventilation | 6 Care Plans
- Near-Drowning | 5 Care Plans
- Pleural Effusion | 6 Care Plans
- Pneumonia | 11 Care Plans
- Pulmonary Embolism | 4 Care Plans
- Pulmonary Tuberculosis | 5 Care Plans
- Tracheostomy | 5 Care Plans
Other nursing care plans for pediatric conditions and diseases:
- Acute Glomerulonephritis | 4 Care Plans
- Acute Rheumatic Fever | 4 Care Plans
- Apnea | 4 Care Plans
- Brain Tumor | 3 Care Plans
- Bronchiolitis | 5 Care Plans
- Cardiac Catheterization | 4 Care Plans
- Cerebral Palsy | 7 Care Plans
- Child Abuse | 4 Care Plans
- Cleft Lip and Cleft Palate | 6 Care Plans
- Congenital Heart Disease | 5 Care Plans
- Congenital Hip Dysplasia | 4 Care Plans
- Croup Syndrome | 5 Care Plans
- Cryptorchidism (Undescended Testes) | 3 Care Plans
- Cystic Fibrosis | 5 Care Plans
- Diabetes Mellitus Type 1 (Juvenile Diabetes) | 4 Care Plans
- Dying Child | 4 Care Plans
- Epiglottitis | 5 Care Plans
- Febrile Seizure | 4 Care Plans
- Guillain-Barre Syndrome | 6 Care Plans
- Hospitalized Child | 5 Care Plans
- Hydrocephalus | 5 Care Plans
- Hypospadias and Epispadias | 4 Care Plans
- Intussusception | 3 Care Plans
- Juvenile Rheumatoid Arthritis | 4 Care Plans
- Kawasaki Disease | 6 Care Plans
- Meningitis | 7 Care Plans
- Nephrotic Syndrome | 5 Care Plans
- Osteogenic Sarcoma (Osteosarcoma) | 4 Care Plans
- Otitis Media | 4 Care Plans
- Scoliosis | 4 Care Plans
- Spina Bifida | 7 Care Plans
- Tonsillitis and Adenoiditis | 4 Care Plans
- Umbilical and Inguinal Hernia | 4 Care Plans
- Vesicoureteral Reflux (VUR) | 5 Care Plans
- Wilms Tumor (Nephroblastoma) | 4 Care Plans