Cystic Fibrosis Symptoms - Ask The Pharmacist

May 28, 2014
The specially trained pharmacists in the Express Scripts Specialty Therapeutic Resource Centers® have disease-specific expertise in helping cystic fibrosis patients manage their symptoms and ensure proper medication adherence so they can lead a normal life.

Cystic fibrosis is the most common lethal genetic disease in the Caucasian population. This hereditary disease caused by a gene mutation affects about 30,000 people in the U.S.

Only those who inherit one defective gene from each parent develop the disease, but one in every 31 people is a carrier.

The predicted median age of survival for someone with cystic fibrosis is in the lower 40s, and about 85% of deaths are a result of pulmonary dysfunction. Given the statistics, cystic fibrosis can be a difficult diagnosis to receive and, with the severe symptoms, an even harder condition to manage.

The specially trained pharmacists in the Express Scripts Specialty Therapeutic Resource Centers® have disease-specific expertise in helping cystic fibrosis patients manage their symptoms and ensure proper medication adherence so they can lead a normal life.

Cystic fibrosis symptoms include:

  • Persistent coughing, at times with phlegm
  • Thick mucus clogging the lungs
  • Frequent lung infections
  • Wheezing or shortness of breath
  • Poor growth and weight gain in spite of a good appetite 

The condition also affects the pancreas and can lead to diabetes. This is called cystic fibrosis-related diabetes. 

Treatment

Treatment for cystic fibrosis focuses on reducing and delaying complications, maintaining lung function and helping patients lead a normal life. Along with treatment, ensuring proper nutrition is also critical. Treatment options for cystic fibrosis are diverse and can include pharmacologic and non-pharmacologic options.

Non-pharmacological ways to manage cystic fibrosis include:

  • A high-calorie diet
  • Vitamins and supplements
  • Chest physiotherapy
  • Supplemental oxygen

Medications may include specialty drugs to help break up mucus. However, some of these can cause voice alteration, pharyngitis, laryngitis, rash, chest pain, sore throat, cough, conjunctivitis and fever.

Common antibiotics such as azithromycin and specialty antibiotics such as inhaled powders and nebulized solutions also are used in the treatment of cystic fibrosis. These can cause dizziness, ringing in ears, voice changes and cough.

While side effects and potential risks can vary among medications, there are a few things for patients to always keep in mind:

  • Make sure you stay adherent to your medication regimen. If needed, set an alert or reminder to remember to take medications as prescribed.
  • Use a pill case to divide, store and remember which medications to take and when.
  • Inhaled antibiotic therapies are generally administered after all other inhaled therapies and airway clearance techniques are complete to ensure the antibiotic stays in the lungs for the longest amount of time.
  • Never mix different medications in a nebulizer at the same time.
  • Stay up to date on vaccinations, including flu and pneumonia.
  • Stay hydrated and drink plenty of water.

Author Bio

Lab Staff
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