Whats-cystic-fibriosis

Nursing Interventions and Care Plan for Cystic Fibrosis-A Student’s Guide

Introduction

Nurses are an essential part of a patient’s care team. Cystic fibrosis is an inherited chronic lung disease characterized by thick mucus accumulation in the lungs and other organs. The goal of cystic fibrosis nursing interventions should be to promote coughing, clear secretions from airways, maintain adequate nutrition, prevent infection, and manage pain.

This post will explore different nursing interventions that a nurse can provide to patients with cystic fibrosis. As you read, keep in mind that our nursing writers are ready to help in case you get stuck with your nursing assignment. All you need to do is place an order with us.

Disclaimer: The information presented in this article is not medical advice; it is meant to act as a quick guide to nursing students, for learning purposes only, and should not be applied without an approved physician’s consent. Please consult a registered doctor in case you’re looking for medical advice.

What is Cystic Fibrosis?

Cystic Fibrosis is a genetic disease that affects the lungs and digestive system. The disease causes thick and sticky mucus to build up in the lungs, making it difficult for people with CF to breathe. This can lead to life-threatening pneumonia or bronchiectasis with repeated episodes of bacterial infection leading to chronic inflammation and progressive scarring of the airway walls restricting airflow. A person’s lung function typically declines over time; however, people with CF live longer than expected due to advances in treatment and improved therapies.

What Are the Causes of Cystic Fibrosis?

Some of the causes for CF include:

1) A person born with two copies of the mutated gene, inheriting one copy from each parent, or receiving an additional copy of the gene due to a mutation in the egg or sperm cells during conception. One interesting fact about this disease is that people who have it are often diagnosed when they’re toddlers because they suffered from chronic cough and repeated lung infections. In some cases, death may occur as soon as three years after diagnosis if left untreated.

2) Environmental factors like smoking or exposure to secondhand smoke can increase a person’s risk of getting the disease, but these factors do not cause CF. The disease is caused by a mutation in a gene called the cystic fibrosis transmembrane conductance regulator gene (CFTR). A person need not have any symptoms or problems related to CF before being identified as having the gene.

3) Exposure to certain bacteria, environmental toxins, or other damaging substances during development in the womb. This exposure may result from a mother infected with pathogens that cause lung disease or has experienced toxic chemical ingestion before and during pregnancy.

Signs and Symptoms of Cystic Fibrosis

Signs of CF include;

-Persistent or chronic cough;

-Breath sounds like wheezing;

-Frequent lung infections;

-Shortness of breath or trouble breathing out after resting or laughing (called post-exertional respiratory distress);

-Poor growth and weight gain despite good food intake;

-Delayed puberty for girls and boys–girls have periods later than age 13 years.

Nurses Diagnosis of Cystic Fibrosis

Health practitioners can diagnose CF by testing for abnormalities in sweat electrolytes or DNA mutations in genes that regulate salt transport. It has been shown that those with this condition have difficulty absorbing vitamins from food, leading to malnutrition over time if not treated properly.

You may check our detailed guide on the Nursing Diagnosis for Cystic Fibrosis

Preparation for Cystic Fibrosis

Before performing any care procedures with a patient, nurses should assess the patient first by checking their vital signs like heart rate and blood pressure and assessing their overall health and mental status. After these assessments are made, the nurse should make sure to have all the necessary equipment and supplies they will need for their patient to provide them with quality care.

What Are the Complications of CF?

There are many complications of this disease, including malnutrition, lung infections, and pancreatic insufficiency.

Cystic Fibrosis may also lead to diabetes mellitus type 1, infertility, liver cirrhosis, and increased risk for certain cancers, including lymphoma.

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What is the Impact of Cystic Fibrosis on Patients? 

Affected by repeated infections, people with cystic fibrosis often have difficulty maintaining their nutritional status.  

The following topics highlight some of these specific impacts, as well as the resulting nursing care implications.  

Impact of Cystic Fibrosis on Patients’ Nutrition:

People with cystic fibrosis have difficulty absorbing most nutrients, including fat-soluble vitamins and electrolytes. Oral intake of pancreatic enzymes or a feeding pump can help patients break down solid foods and absorb the vitamins, protein, and other nutrients they contain.   Even with these options, nutritionists must make sure clients still meet their unique nutritional needs. 

Cystic fibrosis also impacts patients’ growth and development, immunity, and body systems.

Male Infertility in Teens and Young Adulthood:

Fertility is low in males with cystic fibrosis due to scarring of the ducts that carry sperm from testicles to the urethra.   When available, assisted reproductive technology can help men with cystic fibrosis father children and increase future pregnancy rates for women with CF. They can use their eggs, which have been fertilized outside the body, in a lab. The eggs may then be inserted into their uterus during IVF (in vitro fertilization). 

Pelvic Pain:

With endometriosis, the tissue that usually lines the uterus is found outside the uterus. It can cause pelvic pain before or during periods (dysmenorrhea) due to inflamed and compromised tissue with this disease.  For women with cystic fibrosis, scarring from repeated lung infections causes similar problems. Women with CF are more likely to have endometriosis, and it can lead to infertility. Hence, they need regular gynecological care throughout life to treat symptoms as well as prevent reproductive complications.

What is the Goal of CF Interventions?

The goal of cystic fibrosis nursing intervention is to promote patient comfort and promote optimal physical, psychosocial, and emotional well-being.

Cystic Fibrosis Interventions in the Ambulatory Care Setting 

Patients with cystic fibrosis can perform most activities of daily living, including getting dressed, brushing their teeth, and eating independently. They may need assistance with activities that require more energy than usual or are particularly physically demanding, such as walking long distances.

Cases of severe infections should avoid strenuous physical activity like contact sports because of the risk of damaging the lung tissue. They also should wear masks and germicidal sprays to help prevent infection and avoid sick people or have infections to keep their lungs free of germs. 

For patients with mild symptoms, physical activity can help manage symptoms such as fatigue and pain. Aerobic exercise like jogging or swimming helps reduce pain, improves thinking abilities, and shortens the amount of time it takes for lungs to clear themselves of mucus.

Cystic Fibrosis Nurses Interventions in the Community Setting 

People with CF also have a greater risk for developing depression because they are often chronically ill and face ongoing illness-related challenges throughout life. Additional challenges of managing a daily regimen of medication, monitoring weight and diet performance, dealing with an infection, and carrying out physical therapy are all stressors that can contribute to feelings of sadness or depression.

Cystic fibrosis is also linked to higher rates of anxiety disorders due to increased risk for rejection from peers. Special considerations may be needed for children at school, mainly if the CF student is younger than their peers. Inconspicuous placement of an inhaler may reduce self-consciousness about its presence and volume usage.

Cystic fibrosis affects the body’s ability to regulate temperature through sweating by increasing water loss from cells and lungs. This leads to increased body temperature or hyperthermia. Hyperthermia can be life-threatening if the body cannot regulate itself or respond appropriately to heat stress properly.

For people with cystic fibrosis who are at increased risk for heat-related illness due to physical limitations (e.g., difficulty moving from a bed into an upright position), alcohol consumption, or dehydration, extra preventive measures may be needed. Observations may include ensuring the patient is rested and fully hydrated before exertion, limiting activity during times of extreme heat or humidity, arranging transportation to/from appointments in advance, or providing shopping excursions with assistance from friends and family members as appropriate.

Personal Safety Care for CF Patients 

Because cystic fibrosis patients may be more susceptible to infection and frequently require hospitalization, they have a higher risk for physical abuse from caregivers or family members.

-Friends and classmates of children with cystic fibrosis may also experience difficulty adjusting to personal differences associated with the disease (e.g., frequent absences from school, medical crises) and may be inclined to pick on the child.

-Some of the symptoms associated with cystic fibrosis are large body size, frequent coughing or phlegm production, chest-related breathing problems (e.g., wheezing), and food aversion due to smell. Because of these differences, children and adults with cystic fibrosis may be ridiculed and ostracized; therefore, they need assistance from adults to teach them how to respond appropriately if this occurs.

-Children with CF who are bullied or teased should immediately report the incident to their teachers, coaches, and parents so that actions can be taken to resolve the issue.  

-Self-Esteem: Children and adolescents with cystic fibrosis may experience low self-esteem if they cannot have the same experiences as their peers (e.g., going on field trips, being involved in extracurricular activities). Teachers should be alerted when children do not attend school or participate in extracurricular activities regularly due to illness.

-Other aspects of self-esteem that may be affected include physical appearance, body image, personal hygiene, and sexual/reproductive health (e.g., difficulty conceiving children). These issues should be discussed with parents before starting puberty.

-Children with CF may also experience feelings of shame if they are singled out as different or receive extra attention than their peers. Teachers and other school staff should be educated on interacting with CF students to treat all students consistently.

Parental Support:

-Parents can help instill self-esteem by encouraging their children to participate in sports or extracurricular activities, regardless of limitations associated with the disease. Provide opportunities for your child to be involved and develop friendships with other children who have CF so that they are less likely to feel isolated.   

School support: 

Because children with cystic fibrosis may have frequent absences from school, it may be necessary for teachers to develop a plan for making up missed assignments. Teachers and other staff should also know how physical illnesses manifest in children who have CF to recognize when the student is experiencing difficulty (e.g., frequent interruptions of the class by coughing or abdominal pain).

What Are Three Nursing Interventions for a Postoperative Patient for Liver Transplant?

A postoperative patient is someone who has undergone surgery.

The first intervention is monitoring vital signs such as heart rate, blood pressure, respiration rate, and oxygen saturation levels. This helps ensure that the patient is stable enough to move from their bed to another location in the hospital.

The second intervention is administering medications following doctor’s orders or protocol guidelines, and this includes pain relievers like morphine or ketorolac (Toradol) which may help with the discomfort caused by surgery or other treatments such as chemotherapy drugs.

What is the Importance of a Nursing Care Plan for Cystic Fibrosis?

A cystic fibrosis nursing care plan is designed to help you manage your CF patient’s symptoms and prevent complications. It will include a care schedule and specific instructions for each activity, such as the bathing routine. This plan is vital because it can educate other family members or caregivers about how to help you with your CF, so they know what to expect and how best to assist you. It can also provide detailed information if you cannot communicate due to pain, discomfort, or sedation.

What is Included in a Nursing Care Plan for Cystic Fibrosis?

In the medical field, patients with Cystic Fibrosis (CF) require care and treatment plans from a specialized nurse in CF.

A nursing care plan for CF will include what to do if there is an emergency or complication. It will also include how often to clean out the lungs by using chest physiotherapy, giving medications such as antibiotics and steroids, and managing nutrition. The goal of these treatments is two-fold: firstly, it aims to prevent infections. Secondly, it seeks to maintain lung function by preventing lung damage due to mucus accumulation.

A nursing care plan for cystic fibrosis should also include:

-How often the patient needs to come into the clinic or hospital for treatments such as bronchoscopies and nebulizer treatments;

-What type of breathing treatments they need at home (such as chest physical therapy);

-When they need to drink lots of liquids, so they don’t get dehydrated;

-What foods are safe to eat, and which ones might trigger an allergic reaction.

Ps: You can enhance your knowledge on care plans by checking examples of care plans for GERD

What Are the Interventions for a Patient with Cystic Fibrosis?

Interventions for people with cystic fibrosis include promoting comfort, maintaining nutrition, eliminating respiratory secretions, and addressing complications of the disease. These are explained in greater detail below:

Promoting Comfort:

Children who have CF may experience a wide range of symptoms that can make them uncomfortable. Your nurse will help you develop an individualized plan of action that may include breathing treatments, medications, and relaxation techniques to help you feel better. Your health care team needs to learn about all aspects of the disease so they can develop a comprehensive approach to managing your symptoms. This plan should also include various support services available in the community, such as physical therapy, nutrition programs, or social services.

Maintaining Nutrition:

One of the first things you should do is take a dietary history to determine whether any specific foods cause you or have nutritional deficiencies. Your care plan may need to be modified, and your nurse will guide how to manage your diet in a gentle on your digestive tract. 

Eliminating Respiratory Secretions:

You may need to wear a humidifier in your room or use an oxygen therapy device when you are at home to help keep the mucus in your lungs thin so it can be quickly expelled.  It is also helpful if you can cough as much as possible to clear the airways. If you have difficulty coughing, your nurse may suggest that they do it for you or prescribe medication to thin the mucus. 

Addressing Complications:

Individuals with CF are at increased risk of developing certain conditions such as pneumothorax (collapsed lung) and diabetes. Your nurse will be able to give you more information about these conditions and your risk factors.

What Are the Interventions to Prevent Weight Loss for CF Patients?

Protein-calorie malnutrition:  occurs when food and fluid cannot be absorbed in the gastrointestinal tract (stomach).

-Ensure the patient receives adequate nutrition and calories; refer to a specialized dietitian if the problem persists.        

-Maintain hydration, monitor urinary output (patient should be voiding every hour)

What Are Interventions for Intravenous Therapy with Cystic Fibrosis?

-Assess IV site, rotate sites every 24 hr to prevent phlebitis.      

-Determine flushing solution type (normal saline or D5W) and amount of infusion (30ml/hr.).      

-Observe for any allergic reaction to medications; discontinue if so directed.                     

-Focus on infusion safety by drawing up into a syringe to ensure the solution is clear and free of particles.

You may also check nursing interventions for Hypothyroidism

Treatment of Cystic Fibrosis

Treatment options exist, such as

-Medication-antibiotics, bronchodilators,

-Chest physical therapy

-Exercise routines explicitly designed for people with cystic fibrosis

-Nutritional counseling.

-Physiotherapy and supplemental oxygen therapy.

Besides, check the treatment of metabolic alkalosis

Conclusion

Cystic Fibrosis is a life-threatening genetic disorder that causes thick and sticky mucus to build up in the lungs, making it difficult for people with CF to breathe. The symptoms of this disease are identified by abnormalities in sweat electrolytes or DNA mutations in genes that regulate salt transport. There are three significant interventions used when dealing with CF:

-Monitoring vital signs such as heart rate, blood pressure, respiration rate, and oxygen saturation levels,

-Administering medications

Providing emotional support through social means like family members and friends.

Lastly, we recommend that you check nursing metaparadigms to enhance your knowledge on the nursing philosophy, or the 7 nursing leadership styles to better position yourself as a nursing leader.

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