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What is Cystic Fibrosis?

Cystic fibrosis (CF) is a condition passed down through families that causes a buildup of thick and sticky mucus in the lungs, digestive tract, and other areas of the body. It is one of the most common chronic lung diseases in children and young adults. It affects about 30,000 people in the United States and approximately 70,000 worldwide.

What causes Cystic Fibrosis?

Cystic Fibrosis is caused by a defective gene that causes the body to produce abnormally thick and sticky fluid, called mucus. This mucus builds up in the breathing passages of the lungs and in the pancreas, the organ that helps to break down and absorb food. This collection of sticky mucus results in lung infections and serious digestion problems. The disease may also affect the sweat glands and the male reproductive system.

Millions of Americans carry the defective Cystic Fibrosis gene, but do not have any symptoms. That’s because a person with Cystic Fibrosis must inherit two defective Cystic Fibrosis genes—one from each parent. Most children with CF are diagnosed by age 2. A small number, however, are not diagnosed until age 18 or older. These patients usually have a milder form of the disease.

How is Cystic Fibrosis diagnosed?

A blood test is available to help detect Cystic Fibrosis. The test looks for variations in a gene known to cause the disease.

Other tests used to diagnose Cystic Fibrosis include:
  • Immunoreactive trypsinogen (IRT) test is a standard newborn screening test for CF.
  • A high level of IRT suggests possible Cystic Fibrosis and requires further testing.
  • Sweat chloride test is the standard diagnostic test for CF.
  • A high salt level in the patient’s sweat is a potential indicator of the disease.

Other tests that identify problems that can be related to Cystic Fibrosis include:
  • Chest X-Ray or CT Scan
  • Fecal Fat Test
  • Lung Function Tests
  • Measurement of Pancreatic Function
  • Secretin Stimulation Test
  • Trypsin and Chymotrypsin in Stool
  • Upper GI and Small Bowel Series

What to expect?

Thanks to advances in therapies and patient care, more people with cystic fibrosis are surviving into their adult years than ever before. To help enhance the quality of life when treating Cystic Fibrosis, patients, families and caregivers can expect to maintain a close and collaborative care relationship with their healthcare team to determine and understand treatment options at every stage of the condition; learn and practice ways to stay healthy and limit exposure to germs; create and abide by a consistent treatment routine; and manage diet and exercise, among other activities.

How is Cystic Fibrosis treated?

An early diagnosis of Cystic Fibrosis and a comprehensive treatment plan can improve both survival rates and quality of life. Follow-up and monitoring are very important. If possible, patients should be cared for at Cystic Fibrosis specialty clinics that focus specifically on the diagnosis, treatment and support of the condition. When children reach adulthood, they should transfer to a Cystic Fibrosis specialty center for adults.

Treatment for lung problems includes:
  • Antibiotics to prevent and treat lung and sinus infections. They may be taken by mouth, or given in the veins or by breathing treatments. People with Cystic Fibrosis may take antibiotics only when needed, or all the time. Doses are usually higher than normal.
  • Inhaled medicines to help open the airways.
  • DNAse enzyme therapy to thin mucus and make it easier to cough up.
  • High concentrations of salt solutions (hypertonic saline).
  • Flu vaccine and pneumococcal polysaccharide vaccine (PPV) yearly (ask your health care provider).
  • Lung transplant is an option in some cases.
  • Oxygen therapy may be needed as lung disease progresses.

Lung problems are also treated with aerobic exercise or other therapies to thin the mucus and make it easier to cough up out of the lungs.

Treatment for bowel and nutritional problems may include:
  • A special diet high in protein and calories for older children and adults (see: Cystic Fibrosis nutritional considerations).
  • Pancreatic enzymes to help absorb fats and protein.
  • Vitamin supplements, especially vitamins A, D, E, and K.
  • Your doctor can suggest other treatments if you have very hard stools.

Care and monitoring at home should include:
  • Avoiding smoke, dust, dirt, fumes, household chemicals, fireplace smoke, and mold or mildew.
  • Clearing or bringing up mucus or secretions from the airways.
  • Drinking plenty of fluids.
  • Exercising two or three times each week.

How a specialty pharmacy can help?

Kroger Specialty Pharmacy's total life care programs set a clear path to caring, compassionate Cystic Fibrosis therapy management and support.

We are here to provide patients, providers and partners with the necessary coordination of care vital to achieving successful treatment outcomes. By utilizing our expert patient care team comprised of Doctors of Pharmacy, registered pharmacists and nurses, reimbursement specialists and dedicated Patient Care Coordinators (PCCs), we are able to offer each and every patient and partner with high-quality, personalized care, ongoing patient evaluation and clinical support including frequent patient follow-up and continual education about their Cystic Fibrosis treatment.