Cystic Fibrosis

I've taken this from the Cystic Fibrosis Foundation website.  This is an overview.  To read more about CF and what is being done to help find a cure, you can visit  http://www.cff.org

What Is Cystic Fibrosis? 

Cystic fibrosis (CF) is a life-threatening genetic disease that primarily affects the lungs and digestive system. An estimated 30,000 children and adults in the United States (70,000 worldwide) have CF.

In people with CF, 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.

• Obstructs the pancreas and stops natural enzymes from helping the body break down food and absorb vital nutrients.

In the 1950s, few children with CF lived to attend elementary school. Since then, tremendous progress in understanding and treating CF has led to dramatic improvements in the length and quality of life for those with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.

Symptoms of CF

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 and slow weight gain, in spite of a good appetite
• Frequent greasy, bulky stools or difficulty in bowel movements

CF is a progressive disease and I'm so grateful for the progress being made everyday to help extend the lives of those living with CF.  So far Max's lungs have always been healthy and clear.  He rarely coughs and even when we do his daily treatments, he coughs very little, if at all.  We seem to deal more with pancreas/digestive issues.   

Completing his daily treatments

Max is a typical 3yr old.  Just by looking at him, you wouldn't think there was anything different about him.  We do normal activites and are around people all the time. But, we do have to be careful about the germs he is exposed to.  So, this means that sometimes we miss out on activities, school, church, etc.  Whenever he isn't feeling well or catches even a simple cold it tends to linger longer than with most people - especially a cough.  He has a pretty weak stomach and a bad gag reflex. I've cleaned up more vomit over the past 3 years than I've seen in my 36yrs. of life!

When he is sick we also have to up the amount of times he gets his daily treatments. It can be tricky to juggle additional breathing/airway clearance treatments and enough feedings to make sure he gets what he needs each day. Sometimes these sick days can be really rough and require a lot of extra work on our part as we need to be more creative on how to help him release all his energy. Especially since he feels like he's just sitting around all day being hooked up to one machine or another.

Due to the fact that it's always been difficult for him to eat enough food or take in enough calories each day to gain weight, we had to make the decision to have a G-tube (feeding tube) placed. And because of his weak stomach and gag reflex, at times, we have to run his feeding tube with smaller amounts more slowly. Right now he needs to take in about 1800 calories a day to gain weight. 

There are many side effects to CF that many are not aware of.  One side effect we've seen is pruney skin. This boy loves the water, but not even 5 minutes in it, and this is what happens to his skin. Which can make for some very quick baths. 

Another side effect that he has are sinus infections. He tends to get a sinus infection every couple of months.

CF is intense and takes a lot of time and energy. Every 3 months we meet with a team that consists of a Pulmonologist, Dietician, GI Doctor, social worker, and a Respitory Therapist when needed.  Between daily feedings, breathing/airway clearance treatments, doctor visits and getting his energy out, we are constantly on the go. 

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