cystic fibrosis

Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:
  • clogs the lungs and leads to life-threatening lung infections; and
  • obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.
People with CF can have a variety of symptoms, including:
  • very salty-tasting skin;
  • persistent coughing, at times with phlegm;
  • frequent lung infections;
  • wheezing or shortness of breath;
  • poor growth/weight gain in spite of a good appetite; and
  • frequent greasy, bulky stools or difficulty in bowel movements. 

Sam's Story... and treatments...

Sam was diagnosed with CF at 6 mos. after a chronic cough for 6 weeks and failure to thrive (while breastfeeding PLUS consuming 8, 8-10 oz bottles a day!). He was tested for CF at our local hospital, with inconclusive results, and then sent to our regional Children's Hospital for further testing. I was already pretty sure that he had CF, so the positive test results didn't come as much of a surprise. Still, I was devastated. We met with the CF Care Team (Pulmonologist, Respiratory Therapist, Nurse, Social Worker and CF Coordinator) that day and were bombarded with all kinds of information. Feeding routines, Chest Physical Therapy, would never be the same. 

As soon as we began treatment (pancreatic enzymes, nebulized medicines, VEST therapy) he turned around and has had remarkable health ever since. He cultured positive for pseudomonas at about 2 1/2 and has been on TOBI-an inhaled anti-biotic- ever since  (although he did go almost a year with negative cultures). His first hospitalization was Aug. 2009 for I.V. antibiotics. At that time he also started Pulmozyme. In July 2010 he started Hypertonic-Saline. He is pretty much cough and wheeze-free (by the grace of God). His weak point has always been his stomach/pancreas. He takes Prevacid 1x daily (to reduce acid and enable his enzymes to work properly). And is always at the maxed out dose of enzymes for his weight. He has a mediocre appetite (like most CFers) and prefers fruits, vegetables and fish to sweets and fatty foods (except french fries and chicken nuggets!). Because of the high content of salt in his body, he is easliy dehydrated in the heat of summer. To counteract this, we keep him drinking lots of water and Gatorade.
Inhaled Albuterol 2xdaily, Pulmozyme 1x daily, TOBI 2x daily (28 days on/off), Prevacid 15mg 1x daily, fat soluable vitamins 1x daily, Creon 12 enzymes- 4 w/meals, 2/3 w/snacks, Inhaled Hypertonic Saline 2xdaily.
Vest 2x daily (20 mins each)