Cystic Fibrosis

Linz asked me to write a post about my daughter and Cystic Fibrosis (CF). I don't really know exactly what to write, so I guess I will just give you the basics. This is an excerpt from the Cystic Fibrosis Foundation website, "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." So basically her lungs get clogged with mucus a lot easier and she doesn't absorb food very well. The defective gene has to come from both parents in order to have CF, so apparently my husband and I are both carriers.

When Reagan was born she spit up a lot of stomach bile and didn't have a bowel movement. After a lot of tests, x-rays, ultrasounds, barium enemas, etc… she underwent surgery. The surgeon was not sure what he would find, but luckily was able to fix her up. She had almost half of her small intestine removed because it had twisted back on itself. Apparently this twisted bowel is sometimes a sign of CF, so they drew some blood and sent it over to genetics to test. There is absolutely no history of CF in either my husband's or my family, so we didn't expect the test to come back positive. Basically that means generations have been carrying the gene, but no one ever married another carrier. Reagan spent 4 weeks in the NICU before coming home.

Day to day life with her is a little different than for a child without CF. Weight gain is a constant struggle with her. As if losing half of her small intestine wasn't enough, CF causes her to not absorb foods adequately. I have to give her pancreatic enzymes mixed in a spoon of applesauce before every meal and snack. That helps to make up for the fact that her pancreas cannot transport her own enzymes into her intestines for absorption.

To help prevent lung infections I give her pulmozyme through a nebulizer once a day (about 15 minutes) and then I percuss her twice a day (about 20 minutes each time). A percussor is a rubber cup-like thing that causes vibrations in her lungs to help break up any mucus build-up. She is also quarantined during the first two winters to prevent her from getting the flu, RSV, or colds.

There was a great article in the October Ensign about a woman with CF. It made me super emotional, but also made me realize what a support the church is to us. Our Bishop has one foyer closed off for us to sit during sacrament meeting, and has asked the congregation to refrain from using it so we can come to church and still remain isolated.

Having a daughter with CF is sometimes really hard for me, because it makes me ultra paranoid about germs. Luckily my daughter is wonderful and makes it all worthwhile! I think one of the hardest things about having a daughter with CF, is explaining to people that she is "normal." CF is not a disability, it is a disease, and it doesn't affect her mental or physical abilities. The other hard thing is trying to explain to people why we are going to have more children. A lot of people (specifically outside the church) think that it is cruel to bring more children into the world when there is a 25% chance that they will have CF. We have prayed about having more children, and we know that we have at least one more child waiting. If the Lord wishes for us to take care of more than one child with CF then we will. Anyways, I think I have babbled long enough. If you have any questions, I will try my best to answer them.

A few interesting facts about CF:

• 1 in every 30 Caucasian-Americans is a symptom-less carrier of CF
• There are more than 10 million carriers in the United States
• 1 in every 400 marriages involves 2 carriers
• More than 30,000 people in the U.S. have CF
• CF is one of the most common genetic disorders of Caucasians
• There is evidence that CF existed as early as 1595

(left) Reagan using her nebulizer (right) Me percussing her

Hope that helps, Linz!