Cystic Fibrosis Specialty Area Cont.

cf-diseaseCystic Fibrosis (CF) is an inherited, life-threatening disease that occurs most frequently in Caucasians, but is found in all races. CF is the #1 genetic life-shortening condition of children and adults worldwide.

However, survival in CF patients has been steadily increasing each year because treatment options are improving. The median life expectancy is the late 30s to early 40s. However, some patients may now live into their 50s, 60s and 70s.

Approximately 30,000 children and adults in the U.S. (over 70,000 worldwide) have Cystic Fibrosis, a life-long disorder. About one in 3,500 babies is born with CF, as determined by newborn screening tests. These tests aid in early treatment and care.

Each year about 1,000 new cases are diagnosed with nearly half of those aged 18 or older. Three quarters of the diagnoses occur by age two.

Additionally, about one in every 31 Americans (10 million) do not have the disease, but are symptomless carriers of the defective CF gene. If they parent a child with another carrier, there’s a 25 percent chance of having a child with CF.

Currently, there is no cure, but research continues. In addition to the various treatments and medications available, proper CF nutrition and exercise is important.

Due to advancements in medicine and healthcare over the past three decades, individuals with C.F. are living both longer and healthier lives. This is due, in part, to improved antibiotic therapies, nutritional support and innovative therapies. (Generally, these basic medications and treatment programs are covered under Medicare if patients have been classified as disabled).

CF, also known as Mucoviscidosis, is an autosomal recessive genetic disorder that affects many organ systems in the body. It affects mostly the respiratory and digestive systems, plus the liver, sweat glands and the reproductive organs, etc.

In CF, the body produces abnormally thick, sticky mucus where bacteria can grow and cause infections. The mucus and other fluids build up, block the airways and cause frequent coughing up of spit or mucus. Repeated incidences of sinusitis, bronchitis and pneumonia often lead to long-term lung damage.

Not only can CF clog up the lungs and cause life-threatening lung infections, but the thick mucus also affects the pancreas and liver. It can block tubes or ducts in the pancreas; stopping natural enzymes from assisting the body in breaking down food and absorbing related fats and protein nutrients.

This can cause a host of digestive symptoms including diarrhea and stomach pain and can lead to Pancreatitis. Plus, without proper nutrition, it causes abnormal growth in children.

It is important for patients to develop a therapeutic alliance with healthcare team members, including their pharmacist and pharmaceutical care team. Not only will this aid in their ongoing quality of life, but also is helpful in the transition from childhood to adult life with CF.

The Cystic Fibrosis Pharmacy, a pioneer in the treatment of Cystic Fibrosis, is here to assist the CF patient and their family in their daily journey with the most innovative drug treatments and therapies. We continue to be an advocate for patients and raise community awareness about CF!


*NOTE: All information provided on this website is NOT medical advice and is not intended to be a substitute for professional medical advice. It is simply general information for educational purposes only; not meant to be inclusive of all situations.    

It should not be considered the most current information available on the subject as medical information is continually updated and medical sources may differ in exact descriptions and symptoms, care, etc. Please consult your physician for the most up-to-date, accurate information on your specific condition/s or those of your loved ones. HHCS Health Group of Companies, Freedom and Cystic Fibrosis Pharmacies do not guarantee the accuracy of the information provided on this website.