CASE #3
Max, a 12-year-old with cystic fibrosis is being seen in the outpatient clinic for
a biannual evaluation. Max lives at home with his parents and 7-year-old
sister, Lea, who also has cystic fibrosis. Max reports that he “doesn't feel
good,” explaining that he has missed the last week of school, doesn't have any
energy, is coughing more, and is having “a hard time breathing
1. Describe the pathophysiology of cystic fibrosis (CF). Be sure to address the
multisystem component of this disorder
Pathophysiology:
Cystic Fibrosis is caused by mutations or dysfunction in the protein cystic
fibrosis transmembrane conductance regulator (CFTR), which normally transports
chloride ions across epithelial cell membranes. Gene mutations affect transport of
these ions, leading to Cystic fibrosis, which is characterized by thick, viscous
secretions in the lungs, pancreas, liver, intestine, and reproductive tract as well as
increased salt content in sweat gland secretions. Mutations in the CFTR gene result
in abnormalities of cAMP-regulated chloride transport across epithelial cells on
mucosal surfaces. Defective CFTR results in decreased secretion of chloride and
increased reabsorption of sodium and water across epithelial cells. The resultant
reduced height of epithelial lining fluid and decreased hydration of mucus results
in mucus that is stickier to bacteria, which promotes infection and inflammation.
Secretions in the respiratory tract, pancreas, GI tract, sweat glands, and other
exocrine tissues have increased viscosity, which makes them difficult to clear.
In individuals with cystic fibrosis, the lungs are normal in utero, at birth, and
after birth, before the onset of infection and inflammation except possibly for the
presence of dilated submucosal gland ducts in the airways. Shortly after birth, many
persons with cystic fibrosis acquire a lung infection, which incites an inflammatory
response. Infection becomes established with a distinctive bacterial flora. A
repeating cycle of infection and neutrophilic inflammation develops.
The hallmark pathology of cystic fibrosis is bronchial mucus plugging,
inflammation, and eventual bronchiectasis. Commonly, the bronchiectasis begins
in the upper lobes and progresses to involve all lobes.
Cystic fibrosis (CF) is an inherited life-threatening disease that affects many
organs. It is an inherited, multiorgan, multifactorial protein misfolding disease with
its major pathologic impact being on respiratory function.
Cystic fibrosis affects various organ systems in children and young adults,
including the following:
 Respiratory system
 Digestive system
 Reproductive system
1. Cystic Fibrosis and the Respiratory System
The abnormal electrolyte transport system in cystic fibrosis causes the cells in
the respiratory system, especially the lungs, to absorb too much sodium and water.
This causes the normal thin secretions in the lungs to become very thick and hard to
move. These thick secretions increase the risk for frequent respiratory infections.
Recurrent respiratory infections lead to progressive damage in the lungs, and
eventually death of the cells in the lungs. Because of the high rate of infection in the
lower respiratory tract, people with CF may develop a chronic cough, blood in the
sputum, and often even have a collapsed lung. The cough is usually worse in the
morning or after activity.
People with CF also have upper respiratory tract symptoms. Some have nasal
polyps that need surgical removal. Nasal polyps are small protrusions of tissue from
the lining of the nose that can block and irritate the nasal cavity. People with CF also
have higher rates of sinus infections.
2. Cystic Fibrosis and the Gastrointestinal (GI) System
Cystic fibrosis mainly affects the pancreas. The pancreas secretes substances
that aid digestion and help control blood sugar levels. The secretions from the
pancreas also become thick and can clog the ducts of the pancreas. This may cause
a decrease in the secretion of enzymes from the pancreas that normally help digest
food. A person with CF has trouble absorbing proteins, fats, and vitamins A, D, E,
and K. The problems with the pancreas can become so severe that some of the cells
in the pancreas die. Over time, this may lead to glucose intolerance and Cystic
Fibrosis-Related Diabetes (CFRD), a unique type of insulin-dependent diabetes.
The symptoms of CF that may be due to involvement with the GI tract include:
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Bulky, greasy stools
Rectal prolapse (a condition in which the lower end of the bowel comes out
of the anus)
Delayed puberty
Fat in the stools
Stomach pain
Bloody diarrhea
The liver may also be affected. A small number of people may develop liver disease.
Symptoms of liver disease include:
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Enlarged liver
Swollen belly
Yellow color to the skin (jaundice)
Vomiting of blood
3. Cystic Fibrosis and the reproductive system
Most males with CF have blockage of the sperm canal. This is called
congenital bilateral absence of the vas deferens (CBAVD). This results from the
thick secretions clogging the vas deferens and keeping them from developing
properly. It causes infertility because sperm can't travel out of the body. There are
some newer techniques that allow men with cystic fibrosis to have children. Women
also have an increase in thick cervical mucus that may lead to a decrease in fertility,
although many women with CF are able to have children.
2. What will be your priority nursing management?
Nursing Diagnosis: Impaired Gas Exchange d/t airway obstruction as
evidenced by activity intolerance, coughing, pale cyanotic skin color,
tachypnea, abnormal breath sounds, and a barrel chest appearance.
Priority Nursing Management:
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Relieving immediate respiratory distress.
Maintaining adequate oxygenation.
Remaining free from infection.
Improving nutritional status.
Relieving anxiety.
Nursing Interventions:
IMPROVE AIRWAY CLEARANCE.
Monitor the child for signs of respiratory distress; teach the child to cough
effectively; examine and document the mucus produced; increase fluid intake;
and encourage the child to drink extra fluids.
IMPROVE BREATHING.
Maintain the child in a semi-Fowler’s position; use pulse oximetry; maintain
oxygen saturation higher than 90%; administer oxygen as ordered; administer
mouth care every 2 to 4 hours; perform chest physiotherapy every 2 to 4 hours as
ordered; plan nursing and therapeutic activities and diversional activities; and
teach them to exercise to help loosen the thick mucus.
PREVENT INFECTION.
Good handwashing techniques should be practiced by all; practice and teach
other good hygiene habits; monitor vital signs every 4 hours; restrict people with
an infection from contact with the child; and administer antibiotics as prescribed.
MAINTAIN ADEQUATE NUTRITION.
Greatly increase the child’s caloric intake; provide the child with highcalorie, high protein snacks, such as peanut butter and cheese; administer
pancreatic enzymes with all meals and snacks; encourage the child to eat salty
snacks; report any changes in bowel movements; and weigh and measure the
child.
REDUCING THE CHILD’S ANXIETY
Provide age-appropriate activities to help alleviate anxiety and boredom;
encourage the family caregiver to stay with the child; allow the child to have
familiar toys or mementos from home.
PROVIDE FAMILY SUPPORT
Give the family and the child opportunities to voice fears and anxiety;
respond with active listening techniques; and provide emotional support
throughout the entire hospital stay.
3. Why is Max at risk for developing pulmonary infections?
People with cystic fibrosis are at greater risk of getting lung infections because
thick, sticky mucus builds up in their lungs, allowing germs to thrive and multiply.
Mark is at risk for developing respiratory infections because his lungs are not able
to clear the mucous, due to its very dense, tenacious nature. Due to the mucous being
so thick and sticky, it is hard to break it up and expel it from the lungs, especially
within the smaller lung airways. With time it can eventually affect all airways. This
becomes a perfect breeding ground for bacteria to grow due to the obstructions and
stasis of atypical materials, and many of the respiratory infections end up being
chronic due to the difficulty eliminating them.
6. You are reviewing the physician orders for respiratory care. Which of these
nursing interventions would you expect to perform, and why?
1. Administer aerosolized albuterol (a bronchodilator).
Albuterol is a bronchodilator approved by the U.S. Food and Drug
Administration (FDA) to treat bronchospasm (a narrowing of the airways) in
children and adults. Albuterol is available by prescription and may be prescribed
to treat coughing and shortness of breath in patients with cystic fibrosis (CF).
Bronchodilators are also administered to treat hyperactivity and to reverse
bronchospasm.
2. Monitor continuous pulse oximetry.
Pulse oximetry measures the arterial oxygen saturation of circulating
hemoglobin and is considered the standard of care for noninvasively monitoring
oxygen levels. In cystic fibrosis, pulse oximetry is a reliable and simple method for
assessing oxygen saturation. Hypoxemia may result from impaired gas exchange and
from the buildup of secretions and bronchial constriction. Using pulse oximetry also
enables the health care providers to determine if a patient is in need of supplemental
oxygen.
3. Administer nebulized NS (normal saline).
Nebulized normal saline is used to help the patient cough up mucus (sputum)
in their lungs. It works by increasing the amount of sodium (salt) in the airways.
Salt attracts water into the airways, which thins the mucus, making it easier to
cough out. Research has shown that inhaling hypertonic saline twice a day helps
people with cystic fibrosis experience fewer lung infections.