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, medicine…life 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 and off of 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). 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.
 

 ProAir Inhaler 2xdaily, Pulmozyme 1x daily, Prevacid 15mg 1x daily, fat soluable vitamins 1x daily, Creon 12 enzymes- 3 w/meals, 2 w/snacks, Inhaled Hypertonic Saline 2xdaily.
Vest 2x daily (20 mins each)
Advertisements

your turn to scribble something

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out / Change )

Twitter picture

You are commenting using your Twitter account. Log Out / Change )

Facebook photo

You are commenting using your Facebook account. Log Out / Change )

Google+ photo

You are commenting using your Google+ account. Log Out / Change )

Connecting to %s

%d bloggers like this: