Nursing Diagnoses

1. Ineffective airway clearance: Patients with Cystic Fibrosis suffer from excessive thick, sticky mucus production in several body organs including the lungs. The mucus is difficult to loosen and extract without the use of specialized machines (see "Treatment")

2. Risk for infection: CF patients have frequent respiratory infections.  The mucus trapped in the lungs creates an environment that promotes bacterial growth.

3. Activity intolerance: CF patients struggle with aerobic activity and exercise because they cannot inhale as much oxygen as a person with healthy lungs.  They experience shortness of breath and fatigue with exercise. 

4. Imbalanced nutrition- less than body requirements: CF patients are not able to extract nutrients from food the way healthy people can.  As a result, patients who eat a normal balanced diet may be malnourished and require supplementation.  

5. Risk for ineffective coping:  CF patients are at high risk for ineffective coping because of the short life expectancy and impact on daily life.  Most people are diagnosed at a young age and it can be very challenging to accept a diagnosis that will eventually be fatal.  

Nursing Diagnosis	Actual or Potential?	Related to	Plan or Outcome	Nursing Intervention
Ineffective airway clearance	Actual	Excessive mucus secretion in the lungs	Patient will expel predetermined volume of mucus (example: 50 mL) and verbalize feeling of improved breathing after treatment	Perform chest physiotherapy every 4 hours

http://www.atitesting.com/ati_next_gen/FocusedReview/data/datacontext/RM%20NCC%20PN%207.1%20Chp%2019.pdf

http://faculty.mu.edu.sa/public/uploads/1380604673.6151NANDA%202012.pdf

Nursing care of patients with Cystic Fibrosis

In nursing care of CF patients, it is important adjust your approach based on the individual patient.  Caregivers and other family may or may not be involved in the care.  It is important to establish trust with the patient, and family members if applicable, to determine who will be involved in the patient care team.  CF is often diagnosed in infancy, however we know that many people with CF live well into adulthood.  Some of these adult patients may be treated in a pediatric setting for continuity of care.  It is important to address and interact with these patients based on their developmental age, not the care setting. Many CF patients endure frequent medical appointments and hospitalizations throughout their lives.  These patients may be understandably fed up with the routine and their feelings can be manifested in their interactions with nursing staff.  It is important to remember, however, that some CF patients do not experience hospitalization until late in childhood or even into adulthood.  This experience can be very frightening for the patient and family members.  It is important for nursing staff to assess and treat the physical and emotional manifestations of the disease.  A few areas of system-specific assessment of CF patients are listed below.

These assessments can require a wide variety of nursing interventions.  Some of which include:

·       Respiratory Interventions

·       Promptly treat respiratory infections with antibiotic therapy.

·       Provide pulmonary hygiene with chest physiotherapy (CPT) (e.g., breathing exercises to strengthen thoracic muscles) a minimum of twice a day (in the morning and at bedtime).

·       Have the child use the Flutter mucus clearance device to assist with mucus removal.

·       Administer bronchodilators through metered dose inhalers (MDIs) or hand-held nebulizer to promote expectoration of excretions.

·       Administer dornase alfa (Pulmozyme) through a nebulizer to decrease viscosity of mucus.

·       Promote physical activity that the child enjoys to improve mental well-being, self-esteem, and mucus secretion.

·       Gastrointestinal Interventions

·       Administer pancreatic enzymes with meals and snacks.

§  The amount of enzyme replacement will vary between children based on each child’s deficiency and response to the replacement.

§  Instruct the child/family that the capsules can be swallowed whole or opened to sprinkle the contents on a small amount of food.

§  Encourage the child to select meals and snacks if appropriate.

§  Facilitate high-caloric, high-protein intake through meals and snacks.

§  Multiple vitamins and water-soluble forms of vitamins A, D, E, and K are often prescribed.

·       Hospitalization

·       The child with cystic fibrosis is at an increased risk for hospitalization related to pulmonary complications (e.g., respiratory infection, acute respiratory distress).

·       The child will receive respiratory treatments to include aerosol therapy, CPT, breathing exercises, and assistance with coughing/expectoration of secretions.

·       Perform CPT 1 hr before meals or 2 hr after meals if possible.

·       Use oxygen with caution to prevent oxygen narcosis.

·       Promote adequate nutritional intake, and provide pancreatic enzymes at meals and with snacks.

·       Encourage adequate fluid and salt intake.

·       Provide meticulous skin care and oral hygiene.

·       Provide encouragement and support to the child/family by using family-centered nursing care.

·       Care in the Home

·       Ensure parents/caregivers have information regarding access to medical equipment.

·       Provide teaching about equipment prior to discharge.

·       Instruct parents/caregivers in ways to provide CPT and breathing exercises. For example, a child can “stand on her head” by using a large, cushioned chair placed against a wall.

·       Administer antibiotics through a venous access port. Parents/caregivers need instruction in administration techniques, side effects to observe for, and how to manage difficulties with the venous access port.

·       Promote regular primary care provider visits.

·       Ensure up-to-date immunizations with the addition of initial influenza vaccine at 6 months of age and then a yearly booster.

·       Encourage regular physical activity.

·       Encourage participation in a support group(s) and involvement in community resources.

Overall, it is important to treat each patient as an individual, advocate for your patient, and equip the patient (and family if applicable) with the knowledge and support to maintain the best possible disease management at home.  

Hockenberry, M., Wilson, D., Winkelstein, M. (2005). Wong’s essentials of pediatric nursing care. (7th ed.). St. Louis, MO: Mosby. 

http://www.atitesting.com/ati_next_gen/FocusedReview/data/datacontext/RM%20NCC%20PN%207.1%20Chp%2019.pdf

Treatment

Though there is no cure for Cystic Fibrosis, there are many therapeutic measures that have been shown to relieve symptoms.

Antibiotics can be used to fight respiratory infections, which are common in CF patients. Many CF patients take one or more antibiotics even when they are not suffering from a current infection, to prevent new infections from developing.  This use of antibiotics is termed prophylaxis, because it is a preventative measure.  Prophylactic use of antibiotics can be effective in preventing respiratory infections, but with overuse, some bacteria may develop a resistance to antibiotics and can be difficult to treat.  It is important to monitor antibiotic levels in the blood and find a balance between effectiveness and antibiotic sensitivity.

As discussed earlier, a primary symptom of Cystic Fibrosis is excess mucus production in the respiratory tract.  There are several mechanisms to loosen this mucus accumulation so that it may be expelled.  The simplest of these mechanism is called chest physiotherapy (CPT), in which caregivers use their hands to percuss the patient's chest and back, loosening secretions for expulsion several times each day.  Several portable machines have been developed to recreate this mechanism, including vests and ventilators, so that patients can undergo treatments at home, school, or work.  Inhaled medications and steroids are also useful in loosening secretions.  A BiPAP machine can be used to promote respiratory function during sleep.  The BiPAP uses positive pressure to push air into the lungs.  If respiratory function decreases to severe condition, a double lung transplant may be indicated.  This intervention involves many risks including bleeding, infection, anesthesia complications, rejection of the transplanted organ, and even death.

Other surgical interventions may be indicated to alleviate intestinal obstruction or nasal/sinus congestion.  Digestive enzyme supplements can be administered to improve pancreatic function and Vitamin D and calcium supplements can be taken to prevent osteoporosis.  The development and improvement of these treatments over the past decades have played an integral role in extending the average lifespan and improving quality of life for people with Cystic Fibrosis.

Source: Cystic Fibrosis Foundation and Uptodate.com

Signs and Symptoms

Cystic fibrosis signs and symptoms vary, depending on the severity of the disease. A specific patient's symptoms may change, for better or worse, throughout life.  Because of newborn screening throughout the United States, most Cystic Fibrosis patients are diagnosed at a very young age.  However, symptoms may begin anytime from infancy to adolescence or even adulthood.  A sign that is commonly noticed by parents of infants with CF is salty-tasting skin.  When the parents kiss their child, they taste an unusual saltiness. Unfortunately, Cystic Fibrosis affects multiple body systems and can cause a multitude of problems.  

Other symptoms include:
-persistent cough
-excessive mucus and sputum
-wheezing
-shortness of breath
-difficulty exercising
-frequent respiratory infections
-nasal inflammation
-foul-smelling or greasy stools
-poor weight gain/growth
-intestinal blockage
-constipation
-rectal prolapse
-infertility
-pancreatic insufficiency
-diabetes
-sinus disease
-liver disease
-venous thrombosis
-liver disease
-frequent fractures
-kyphoscoliosis 
A more complete list of possible manifestations is included below. 

The variety of symptoms and presentations of Cystic Fibrosis presents providers with a difficult task is treatment.  Luckily there are a several options for reducing symptom severity. 

Source: Uptodate.com

Diagnosis

The are a variety of measures in place to test for Cystic Fibrosis.  All newborns in the United States are screened for CF by either a blood test or a genetic test.  The blood test assesses pancreas function, while the genetic test indicates whether the CFTR gene is faulty.  If one of these newborns screenings indicates an abnormality, the infant will undergo a sweat test between two and four weeks of life.  During the sweat test, a technician will apply a chemical solution to a small area of the arm or leg.  This chemical does not pose a risk to the child.  An electrode is placed on the area and a mild electric current is applied to induce sweating.  This part of the procedure takes about 5 minutes and the child may feel some tingling or warmth, but it is not strong enough to cause pain or damage.  The sweat is collected on a piece of filter paper or gauze and then sent to the lab for evaluation. The lab is checking for chloride levels in the infant's sweat.  Some physicians may order the test to be performed twice for verification.  This test is safe and reliable.  It has been the "Gold Standard" in CF diagnosis for over fifty years.  The entire test takes about an hour, but may take longer.  The child should not have lotion or ointment applied to the skin for 24 hours before the test.  Otherwise no changes in diet or medications need to be made.  Once a positive or negative result has been confirmed, the results will not change in later sweat tests.  Other diagnostic tools include chest x-Ray, sinus x-Ray, lung function tests and sputum culture.  These tests are more helpful is analyzing the progress of the disorder once a diagnosis has been confirmed.


Sources: National Institutes of Health and Cystic Fibrosis Foundation

Etiology and Pathophysiology

To fully understand the causation and progression of Cystic Fibrosis, it is helpful to have a visual representation of the disease.  Khan Academy presents a clear and concise visual summary of CF.

https://www.khanacademy.org/science/health-and-medicine/respiratory-system-diseases/cystic-fibrosis/v/what-is-cystic-fibrosis

Who has it?

Throughout the world, approximately 70,000 children and adults suffer from Cystic Fibrosis.  Almost half of these cases (30,000) are within the United States.  Both males and females of all races and ethnicities are at risk, but CF is most prevalent in Caucasians of Northern European descent.   About 1,000 new cases are diagnosed in the US each year.  Historically, about half of the CF population is diagnosed by five months of age, and over 75% are diagnosed before their second birthday.  However, some people with less severe cases have been diagnosed well into adulthood.  Diagnosis has been evolving over the past few years, and now all states screen newborns for cystic fibrosis within a few days of birth.  The screening is not 100% accurate, and produces many false positive.  False positives occur when a healthy baby is tested for a disease and the results indicate that the baby has the disease.  To find out if the results were accurate or not, all newborns who test positive for CF go through a follow-up test that is more accurate.  This secondary screening rules out CF in over half of those who tested positive in the initial newborn screening.  Because of the recent implementation of newborn screening, more cases are being confirmed at an early age, improving treatment outcomes and documentation of CF epidemiology. 

It is believed that CF susceptibility is entirely genetic.  However, the severity of the illness is affected by many environmental factors including air quality, exposure to bacteria, access to medical care and age of diagnosis and treatment.  These factors, along with genetics and others health conditions determine the lifespan of each CF patient.  There is no cure for the disease, but people with CF who maintain successful treatment regimens can live into their fifties with reasonably high quality of life.  This possibility is a thrilling advancement in comparison with CF outcomes in 20 years ago.  CF was considered a childhood disease in recent years because so few patients lived into adulthood.  Today almost half of those with CF are 18 or older.  According to a recent article published by BMC Pulmonary Medicine, this improvement is due to earlier diagnosis and follow-up treatment over the past two decades.  The results point toward newborn screening as a major indicator in disease outcome.







Citations

Cystic Fibrosis. http://www.nhlbi.nih.gov/health/health-topics/topics/cf.  Published December 26, 2013.  Accessed January 16, 2015.

Marson FA, Hortencio TD, Aguiar KC, Ribeiro JD. Demographic, clinical, and laboratory parameters of cystic fibrosis during the last two decades: a comparative analysis. BMC Pulmonary Medicine. 2015; 15(3). doi:10.1186/1471-2466-15-3 

O’Sullivan BP, Freedman SD. Cystic fibrosis. Lancet 2009;373:1891–1904.

What is Cystic Fibrosis?

Cystic Fibrosis (CF) is a genetic disorder that affects the lungs, gastrointestinal tract, pancreas, liver, kidney, and reproductive organs through overproduction of secretions. It is most commonly associated with increased sputum production and frequent lung infections. Other common symptoms include shortness of breath, sinus infections, stunted growth, gastrointestinal ileus, and infertility. There is no cure for CF, but some patients are eligible for lung transplant for improved respiratory function and other treatments are available for symptom management. The average life expectancy for a CF patient is just under 40 years, and most CF deaths are related to the lungs.

CF is classified as an autosomal recessive disorder. It is determined by the functionality of the gene CTFR. CTFR is responsible for bodily secretions including sweat, saliva, mucus, and digestive juices. When this gene is mutated, the thin, slippery secretions of the body become thick and sticky, creating a barrier to normal bodily functions. Most healthy people have two normal copies of the gene CTFR. Some people have one normal copy and one abnormal copy. These people are termed “carriers” and they do not have any CF symptoms. When two carriers procreate, there is a 25% chance that the offspring will inherit both abnormal genes and have cystic fibrosis. Currently about 30,000 Americans suffer from CF. In the United States, it is most common in Caucasians, followed by Hispanics, Native Americans, African Americans, and least common in Asian Americans.

In the past, CF has been considered a childhood disease, primarily because most CF patients did not live into adulthood, and those who did weren’t able to maintain a job and live independently. In 1959, the average survival of a CF patient was just 6 months. Thanks to recent advancements in treatment and management, CF is no longer a childhood death sentence. Many people are able to live into adulthood with well-managed symptoms that allow for a relatively normal lifestyle. For unknown reasons, men with CF live an average of 40 years in the United States, while women average about 37 years. Some parts of the world screen infants for CF at birth, but the test is not completely accurate, so testing has become controversial. CF is currently undergoing extensive research due to recent increased funding and is a very exciting topic in the field of medicine.

Sources
http://www.nhlbi.nih.gov/health/health-topics/topics/cf.  Published December 26, 2013.  Accessed January 10, 2015.

http://www-uptodate-com.offcampus.lib.washington.edu/contents/cystic-fibrosis-clinical-manifestations-and-diagnosis?source=search_result&search=cystic+fibrosis&selectedTitle=1%7E150 Published June 25, 2014.  Accessed January 10, 2015.