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
Impaired Gas Exchange
- Impaired Gas Exchange
May be related to
- Airway obstruction by nasal obstruction.
- Airway and Alveoli inflammation.
- Bronchiectasis with decreased surface area for gas exchange and loss of lung function.
- Infection with lung consolidation, alveolar collapse.
Possibly evidenced by
- Activity intolerance
- Pale, cyanotic skin color
- Client will maintain optimal gas exchange as evidenced by oxygen saturation of 90% or greater, arterial blood gasses (ABGs) within the client’s usual range, relaxed breathing, baseline heart rate, alert response mentation and no further deterioration in the level of consciousness.
|Monitor respiratory and heart rate for any changes.||There will be an increase in the respiratory and heart rate as a way of compensation for early hypoxia.|
|Assess for changes in respiratory status such as cyanosis, pallor, changes in the level of consciousness, labored breathing and tachypnea.||Client will adapt their breathing pattern over time to facilitate gas exchange. Abnormalities may indicate respiratory compromise, hypercarbia, or hypoxia.|
|Monitor transcutaneous carbon dioxide as ordered.||Chronic hypercarbia can be present in clients with moderate to severe cystic fibrosis lung disease; increasing levels can indicate the progression of acute infection and pending respiratory failure.|
|Monitor arterial blood gasses and oxygen saturation as indicated||Increasing Paco2 and decreasing Pao2 are signs of respiratory failure. Severe hypoxemia and hypercarbia can lead to hypotension, dysrhythmias, and failing respiratory effort.|
|Provide for adequate rest between activities during the day, with a minimal nighttime interruption in sleep.||Activity increases oxygen needs and should be paced appropriately to avoid fatigue.|
|Place the client in a semi-Fowler’s position.||Semi-Fowler position promotes lung expansion and decreases airway collapse, dyspnea, and breath work through gravity.|
|Administer oxygen therapy as indicated. (Avoid giving high oxygen concentration in clients with chronic carbon dioxide retention).||Supplemental oxygen maintains adequate oxygenation, decreases the work of breathing and calorie expenditure, and relieves dyspnea, increasing the level of comfort. Maintaining an oxygen saturation greater than 90% is the goal.|
|Collaborate with respiratory care personnel in the monitoring and administration of noninvasive ventilation as indicated.||Bilevel positive airway pressure is a noninvasive ventilation indicated for clients with severe lung disease and a superimposed acute illness or for assistance with nighttime ventilatory needs.|
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