Cystic Fibrosis: Causes, Complications and Treatment

Author:
Alexa Ferrante
Cystic Fibrosis chart

3-minute read

What is Cystic Fibrosis?

According to the American Lung Association, 30,000 people in the United States alone have been diagnosed with Cystic Fibrosis (CF). It is a genetic disease that is generally found in people of Northern European descent, occurring in one in every 2,500 to 3,500 Caucasian newborns. Cystic fibrosis is less common in other ethnic groups, affecting about 1 in 17,000 African Americans and 1 in 31,000 Asian Americans.

As a life-threatening and incurable illness, Cystic Fibrosis affects thousands of families every year. A defective gene causes secretions in the lungs and pancreas to be abnormally thick, blocking the airway and causing the body's natural digestive enzymes to damage the pancreas and liver. A multi-organ disease, CF affects the lungs, digestive tract, sinuses, and increases the risk for diabetes, osteoporosis, and infertility.

Signs, Symptoms and Complications

Cystic Fibrosis can now be diagnosed just a few days after birth through genetic testing for newborns, before any signs or symptoms develop. More than 1,700 different mutations have been identified that can cause cystic fibrosis, with varying degrees of illness. Some patients may manifest symptoms as babies, while others may not have any symptoms until later in life, even well into adulthood. Newborn screening allows healthcare providers to take immediate steps to keep children with CF as healthy as possible.

CF patients have a higher than normal salt level in their sweat and are affected by various symptoms including:

  • Persistent cough
  • Sputum production
  • Frequent lung infections
  • Wheezing and shortness of breath
  • Poor growth or weight gain
  • Male infertility
  • Sinus inflammation and infection

In addition to the above symptoms, CF patients experience various complications of their condition. Some of these complications include:

  • Blockage of the intestinal tract
  • Pancreatitis
  • Bronchiectasis (abnormal widening of the airways)
  • Coughing up blood
  • Diabetes
  • Osteoporosis
  • Pneumothorax
  • Respiratory failure

Living with Cystic Fibrosis

Though there is no existing cure for Cystic Fibrosis, individuals with this condition can utilize treatments to ease their symptoms and reduce the complications of the disease. Newborn screenings are now fully available in the United States and can determine whether or not a child has CF before symptoms even develop. Some of the treatment options for CF patients include anti-inflammatory drugs to improve lung function, inhaled medications to promote airway clearance, antibiotics to fight bacteria, pancreatic enzymes replacement to help absorb nutrients. More recently, a newer class of medication called CFTR modulators have been developed to correct the underlying genetic defect for certain CF mutations.

Treatment and Evaluation

Due to the specialized nature of CF, it is recommended for all CF patients to receive care at an accredited CF center where they can receive multi specialty and personalized care. The adult CF program at MemorialCare Long Beach Medical Center and the pediatric CF program at MemorialCare Miller Children's & Women Hospital are part of a nationwide network of approximately 130 centers in the US accredited by the CF Foundation. These centers partner with patients with CF to help them live longer, healthier, more productive lives. Our centers are also part of the CF research network, which provides our patients with opportunities to participate in the newest CF research studies.

Our CF team includes pulmonologists, gastroenterologists, respiratory therapists, dietitians, social workers, pharmacists, diabetes nurse practitioners, and nurses who work with each patient to develop and implement a personalized treatment plan that best fits his or her needs.

Resources

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