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