Nursing Care Plan (NCP) for Cystic Fibrosis
Included In This Lesson
Study Tools For Nursing Care Plan (NCP) for Cystic Fibrosis
Outline
Lesson Objectives for Cystic Fibrosis
- Understanding Cystic Fibrosis (CF) Basics:
- Gain knowledge about the etiology, pathophysiology, and clinical manifestations of cystic fibrosis, including the impact on the respiratory and digestive systems.
- Caring for Patients with CF:
- Learn the principles of providing holistic care for individuals with cystic fibrosis, including the management of respiratory and nutritional challenges, infection prevention, and psychosocial support.
- Medication Management:
- Understand the various medications used in the treatment of cystic fibrosis, including bronchodilators, mucolytics, antibiotics, and pancreatic enzymes, and learn the appropriate administration and monitoring procedures.
- Nutritional Support and Monitoring:
- Explore strategies for promoting optimal nutrition in individuals with CF, emphasizing the importance of pancreatic enzyme replacement therapy, nutritional supplements, and dietary modifications.
- Psychosocial Considerations:
- Recognize the psychosocial aspects of living with cystic fibrosis, including the impact on mental health, coping mechanisms, and strategies for providing emotional support to individuals and their families.
Pathophysiology of Cystic Fibrosis (CF)
- Defective CFTR Gene:
- Cystic fibrosis results from mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene located on chromosome 7, leading to a dysfunctional CFTR protein.
- Impaired Chloride Transport:
- The CFTR protein is responsible for regulating chloride ion transport across cell membranes. Mutations cause reduced chloride secretion and increased sodium absorption, leading to thick, sticky mucus production.
- Viscous Mucus Production:
- Abnormal CFTR function results in the production of thick, sticky mucus in various organs, particularly affecting the respiratory and digestive systems. This mucus obstructs airways and ducts, causing complications.
- Respiratory Complications:
- In the respiratory system, the thick mucus obstructs airways, promoting bacterial colonization and recurrent infections. Chronic inflammation and damage to lung tissue contribute to progressive respiratory decline.
- Digestive System Involvement:
- In the digestive system, the thick mucus obstructs pancreatic ducts, leading to insufficient release of digestive enzymes. This impairs nutrient absorption and causes malnutrition. Liver and gallbladder complications may also occur.
Etiology of Cystic Fibrosis (CF)
- Genetic Inheritance:
- Cystic fibrosis is an autosomal recessive genetic disorder, meaning that an individual must inherit two defective CFTR genes (one from each parent) to develop the condition.
- CFTR Gene Mutations:
- The etiology is linked to mutations in the CFTR gene, specifically in the code for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Various mutations can occur, affecting the functionality of this protein.
- Carrier Parents:
- Parents who carry a single mutated CFTR gene are carriers and do not exhibit symptoms. When both parents are carriers, there is a 25% chance with each pregnancy that the child will inherit two mutated genes and develop cystic fibrosis.
- Ethnic and Geographic Variations:
- The prevalence of specific CFTR gene mutations can vary among different ethnic groups and geographic regions. Certain mutations are more common in specific populations.
- New Mutations:
- In rare cases, individuals may develop cystic fibrosis due to new mutations in the CFTR gene that occur spontaneously. This can happen even if there is no family history of the condition.
Desired Outcome for Cystic Fibrosis (CF) Management
- Optimal Respiratory Function:
- Improve and maintain optimal lung function to minimize respiratory symptoms, reduce the frequency of respiratory infections, and enhance overall respiratory well-being.
- Nutritional Adequacy:
- Achieve and maintain proper nutritional status by addressing malabsorption issues and ensuring adequate caloric intake. This includes promoting healthy growth and development in pediatric patients.
- Effective Mucus Clearance:
- Enhance and facilitate mucus clearance from the airways to reduce the risk of airway obstruction, infections, and respiratory complications.
- Quality of Life Improvement:
- Enhance the overall quality of life by managing symptoms effectively, promoting independence, and addressing psychosocial aspects of living with cystic fibrosis.
- Prevention of Complications:
- Minimize and manage complications associated with cystic fibrosis, such as diabetes, liver disease, and osteoporosis, through comprehensive care and proactive interventions.
Cystic Fibrosis Nursing Care Plan
Subjective Data:
- Exercise intolerance
- Nasal congestion
- Constipation
- Abdominal pain
Objective Data:
- Coughing, non-productive or with thick sputum
- Meconium ileus
- Failure to thrive
- Salty taste to skin
- Oily stools
- Abdominal distention
Nursing Assessment for Cystic Fibrosis (CF)
- Respiratory Status:
- Monitor respiratory rate, pattern, and effort.
- Assess for cough characteristics, sputum production, and any signs of respiratory distress.
- Evaluate oxygen saturation levels using pulse oximetry.
- Nutritional Assessment:
- Monitor growth and development in pediatric patients.
- Assess dietary habits, nutritional intake, and weight trends.
- Evaluate signs of malabsorption, including steatorrhea and nutritional deficiencies.
- Mucus Clearance:
- Assess the effectiveness of airway clearance techniques.
- Evaluate the consistency and color of respiratory secretions.
- Monitor for signs of respiratory infections.
- Psychosocial Well-being:
- Evaluate the impact of cystic fibrosis on mental health and psychosocial well-being.
- Assess coping mechanisms and support systems in place.
- Gastrointestinal Function:
- Monitor for symptoms of gastrointestinal involvement, such as abdominal pain, distension, and changes in bowel habits.
- Assess pancreatic enzyme replacement therapy effectiveness.
- Skin Integrity:
- Inspect skin for signs of excessive salt loss, such as sweat-induced skin changes.
- Monitor for skin breakdown, especially in areas prone to friction and moisture.
- Bone Health:
- Assess bone density and risk factors for osteoporosis.
- Monitor for signs of bone pain or fractures.
- Psychosocial Factors:
- Evaluate the impact of the disease on the patient’s daily life, school/work, and social activities.
- Assess for signs of depression or anxiety and provide appropriate support.
Implementation for Cystic Fibrosis (CF)
- Respiratory Care:
- Administer respiratory treatments, including bronchodilators, mucolytics, and airway clearance therapies as prescribed.
- Monitor and assist with chest physiotherapy and postural drainage techniques.
- Administer oxygen therapy as needed, ensuring proper saturation levels.
- Nutritional Support:
- Administer pancreatic enzyme replacement therapy with meals to support nutrient absorption.
- Collaborate with a dietitian to create a well-balanced, high-calorie diet.
- Encourage adequate fluid intake to prevent dehydration.
- Medication Management:
- Administer prescribed medications, including antibiotics for respiratory infections and medications to manage symptoms.
- Educate the patient and family on the proper administration of medications at home.
- Psychosocial Support:
- Provide emotional support to the patient and family, addressing the challenges and impact of CF on daily life.
- Facilitate access to support groups and mental health resources.
- Collaborate with the healthcare team to address psychosocial needs.
- Education and Self-Management:
- Educate the patient and family about the importance of adherence to the treatment plan.
- Teach airway clearance techniques and ensure the patient can perform them independently.
- Provide information on recognizing early signs of respiratory infections and when to seek medical attention.
Nursing Interventions and Rationales
- Assess respiratory status; note rate, rhythm, and quality of breathing; auscultate lungs
- Review growth charts, assess changes or decrease in height or weight
- Assess abdomen
- Look for distention
- Listen for active bowel sounds and gas pattern
- Feel – palpate for mass or signs of constipation
- Insert and monitor nasogastric feeding tubes
- Monitor nutritional status
- Caloric intake
- Excessive thirst or hunger
- Fiber intake
- Use of feeding tube
- Monitor blood glucose
- Assess for signs of infection
- Monitor temperature
- Note cough with or without sputum
- Obtain sputum culture and sensitivity
- Monitor for signs of dehydration and encourage fluid and salt intake
- Assess for bone malformation, deformity or fractures
- Administer medications and supplements appropriately
- IV antibiotics may be given to treat infections.
- Antipyretics may be used to manage fever.
- Anti-inflammatories help to reduce swelling in the airways
- Inhaled steroids and bronchodilators may be used to decrease inflammation and open airways.
- Suction secretions as necessary per facility protocol
- Perform chest physical therapy or vest therapy 2 – 4 times per day per facility protocol
- Provide education for home treatment and resource information for patients and parents
- Educate parents on how to spot signs of infection and when to seek medical help.
- Encourage nutrition with high fiber foods and adequate fluid intake to prevent constipation.
- Teach the importance of supplements to ensure adequate absorption of nutrients.
- Provide information for support groups and resources as available.
Evaluation for Cystic Fibrosis (CF)
- Respiratory Function:
- Monitor lung function through regular pulmonary function tests to assess the effectiveness of respiratory treatments.
- Evaluate the frequency and severity of respiratory infections and the need for antibiotic interventions.
- Nutritional Status:
- Assess weight gain and growth patterns, monitoring for improvements in nutritional status.
- Review enzyme replacement therapy effectiveness in supporting nutrient absorption.
- Medication Adherence:
- Evaluate the patient’s adherence to the prescribed medication regimen.
- Assess the impact of medications on symptom management and overall well-being.
- Psychosocial Well-being:
- Assess the emotional well-being of the patient and family, considering the impact of CF on their daily lives.
- Monitor participation in support groups and utilization of mental health resources.
- Self-Management Skills:
- Evaluate the patient’s ability to independently perform airway clearance techniques.
- Assess the understanding and application of information provided regarding early signs of respiratory infections and appropriate actions.
References
- https://kidshealth.org/en/parents/cf.html
- https://childrensnational.org/choose-childrens/conditions-and-treatments/airway-lungs/cystic-fibrosis
- https://www.cincinnatichildrens.org/health/c/cystic-fibrosis
- https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/diagnosis-treatment/drc-20353706
Transcript
Today we are going to be tackling cystic fibrosis. Cystic fibrosis is a genetic disorder of the secretary of glands. So the glands that secrete different mucus and sweat. It causes it to overproduce mucus, sweat, and it causes it to affect the lungs, pancreas, liver, intestines, and sex organs. The mucus is very thick and it impedes the normal processes of the body. So instead of lubricating the lungs and other organs, it actually clogs the airways and the ducts; the most, frequently affected organs are the pancreas and the liver. Some things we want to look for, we want to look for signs of infection. These patients tend to have some respiratory infections. We also want to just think in the back of our mind vest therapy, that’s some therapy that the cystic fibrosis patients undertake. We’re going to monitor the vital signs.
We’re going to draw some labs. We’re going to administer medications as ordered. We’re going to do a good abdominal assessment, and we’re going to do a glucose check because the blocked pancreas is also affected and that can cause some diabetes. And also we’re going to monitor their fluid intake. This patient is going to maintain adequate ventilation and respiratory status as well as have optimal, nutritional status. And the cystic fibrosis patient is going to be free of infection.
So cystic fibrosis: subjective. What is the patient telling us? Well, the patient is going to have some exercise intolerance. They’re going to get tired and that’s because a lot of their ducts are blocked. So their lungs are going to be tired. They’re going to have some nasal congestion. Remember the thick mucus. They’re also going to have some constipation because their GI tract is going to also be blocked and they are going to have some abdominal pain.
Okay. Um, some objective things that we’re going to observe as nurses, we’re going to see coughing, but it’s going to be non productive. Or if it’s productive, it’s going to be thick sputum. They’re going to have what we call meconium ileus. If you remember, meconium is that first stool that is passed as a child. So they’re going to have ileus, which is, it’s not going to come. They’re going to have a failure to thrive. These patients also have what we call a salty taste to the skin, and they’re going to have oily stools and some abdominal distention. So what are some things that we can do as nurses and intervene? Well, the first thing I think we should do is assess the respiratory status. Very important that we note a few things. We want to note the rate, the rhythm and the quality of breathing, because those things can give us some good information.
So cystic fibrosis patients tend to get frequent respiratory infections because of the thick mucus that traps bacteria. We want to perform chest physical therapy or vest therapy, two to four to four times a day. And that’s all based on the facility’s protocol. We’re going to look at the abdomen. We’re going to do a good abdominal assessment. We’re going to feel or palpate for signs of constipation. In these patients, the mucus that usually lubricates the GI tract becomes an area for distension and constipation. Okay. We’re going to monitor their blood sugar. These patients tend to have increased blood glucose levels. CF related diabetes is very common.
We’re going to, again, assess for signs of infection. So because lung infections are common, we’re going to culture the sputum. We’re going to have determined which bacteria is involved. If an infection is present, because we want to be able to pick the correct antibiotics in order to develop a course of treatment. Last, but certainly not least, we are going to monitor for signs of dehydration, and we’re going to encourage fluid and salt intake. So patients with cystic fibrosis tend to lose an excess amount of fluids and salt. So salt leaves and fluid goes right behind it. They can become dehydrated or develop hyponatremia, which is another word for low sodium. They can develop low sodium and that develops quickly. We want to avoid all of those complications. We talked about this, but we want to make sure that we perform the vest therapy two to four times a day. Like I said, per the protocol, this technique is just shaking up the body and loosening up that thick mucus and making it easy to either suction out or expel with a cough.
Here are some key points that we want to focus on when we are taking care of the cystic fibrosis patient. Okay. Remember, cystic fibrosis is an autosomal, recessive genetic disorder. Okay. It just makes your secretary glands produce thick mucus. Okay. The subjective and objective details that we want to focus on are subjective. These patients are going to have some exercise and tolerance, constipation, abdominal pain, and congestion. Objective: we’re going to notice a few things. Few of them, a meconium ileus, and abdominal distension, as well as increased blood sugar. So let’s add that here. Increase B G blood glucose. We’re going to monitor glucose for CF related diabetes and best therapies should be done two to four times per day. That’s going to help break up that thick mucus. We love you guys; go out and be your best self today. And, as always, happy nursing.
NCLEX
Concepts Covered:
- Circulatory System
- Emergency Care of the Cardiac Patient
- Cardiac Disorders
- Cardiovascular
- Shock
- Shock
- Disorders of the Posterior Pituitary Gland
- Endocrine
- Disorders of Pancreas
- Disorders of the Thyroid & Parathyroid Glands
- Hematology
- Gastrointestinal
- Upper GI Disorders
- Liver & Gallbladder Disorders
- Newborn Complications
- Lower GI Disorders
- Multisystem
- Neurological
- Nervous System
- Central Nervous System Disorders – Brain
- Renal
- Respiratory
- Urinary System
- Respiratory System
- Noninfectious Respiratory Disorder
- Test Taking Strategies
- Note Taking
- Basics of NCLEX
- Prefixes
- Suffixes
- Medication Administration
- Gastrointestinal Disorders
- Respiratory Disorders
- Pregnancy Risks
- Labor Complications
- Hematologic Disorders
- Fundamentals of Emergency Nursing
- Factors Influencing Community Health
- Delegation
- Perioperative Nursing Roles
- EENT Disorders
- Basics of Chemistry
- Adult
- Emergency Care of the Neurological Patient
- Acute & Chronic Renal Disorders
- Emergency Care of the Respiratory Patient
- Respiratory Emergencies
- Studying
- Substance Abuse Disorders
- Disorders of the Adrenal Gland
- Behavior
- Documentation and Communication
- Preoperative Nursing
- Endocrine System
- Legal and Ethical Issues
- Communication
- Understanding Society
- Immunological Disorders
- Infectious Disease Disorders
- Oncology Disorders
- Female Reproductive Disorders
- Fetal Development
- Terminology
- Anxiety Disorders
- Cognitive Disorders
- Musculoskeletal Trauma
- Intraoperative Nursing
- Tissues and Glands
- Vascular Disorders
- Renal Disorders
- Eating Disorders
- Prenatal Concepts
- Microbiology
- Male Reproductive Disorders
- Sexually Transmitted Infections
- Infectious Respiratory Disorder
- Depressive Disorders
- Personality Disorders
- Psychotic Disorders
- Trauma-Stress Disorders
- Peripheral Nervous System Disorders
- Integumentary Disorders
- Neurologic and Cognitive Disorders
- Integumentary Disorders
- Newborn Care
- Basics of Mathematics
- Statistics
- Labor and Delivery
- Proteins
- Emergency Care of the Trauma Patient
- Hematologic System
- Hematologic Disorders
- Developmental Considerations
- Skeletal System
- Digestive System
- Urinary Disorders
- Postpartum Care
- Basic
- Musculoskeletal Disorders
- Bipolar Disorders
- Metabolism
- Cardiovascular Disorders
- Concepts of Population Health
- Musculoskeletal Disorders
- EENT Disorders
- Postpartum Complications
- Basics of Human Biology
- Postoperative Nursing
- Neurological Emergencies
- Prioritization
- Disorders of Thermoregulation
- Writing
- Community Health Overview
- Dosage Calculations
- Neurological Trauma
- Concepts of Mental Health
- Health & Stress
- Endocrine and Metabolic Disorders
- Childhood Growth and Development
- Prenatal and Neonatal Growth and Development
- Concepts of Pharmacology
- Integumentary Important Points
- Emotions and Motivation
- Renal and Urinary Disorders
- Developmental Theories
- Reproductive System
- Adulthood Growth and Development
- Psychological Emergencies
- Growth & Development
- Basics of Sociology
- Somatoform Disorders
- Reading
- Intelligence and Language
- Oncologic Disorders
- Med Term Basic
- Med Term Whole
- Central Nervous System Disorders – Spinal Cord
- Muscular System
- Neonatal
- Learning Pharmacology
- Pediatric
- Psychological Disorders
- State of Consciousness
- Sensory System