Cystic Fibrosis and a little girl

I have an on-line friend, Julie, who’s daughter, Jenna, has cystic fibrosis and I asked her if she would tell us a little about CF and what it means to her family. Here is Julie’s touching and personal account of CF and her little girl, Jenna.

I am 28 years old and work as a PCA on the ortho unit of my local hospital. Jenna is 4 years old and goes to pre-school. She loves books, playing outside, and can not WAIT to be a big sister! (Julie is pregnant with her second child – maryann)
Can you give us an over view of what CF is? “Cystic fibrosis is a life-threatening genetic disease that causes mucus to build up and clog some of the organs in the body, particularly the lungs and pancreas. When mucus clogs the lungs, it can make breathing very difficult. The thick mucus also causes bacteria (or germs) to get stuck in the airways, which causes inflammation (or swelling) and infections that lead to lung damage.” (taken from the Cystic Fibrosis Foundation’s website. www.cff.org)

When did you find out Jenna had CF? It was kind of a fluke that we found out how we did. On my 19 week ultrasound, they saw what is called an echogenic bowel. I was told that it “could be absolutely nothing or cystic fibrosis”. Her father had carrier testing done to see if he was a carrier for the disease…his results came back positive. I was a positive carrier too… (FYI- both parents need to be carriers in order for the child to be born with the disease)

We were then set up with genetic counselors, to go over what CF was, the genetic details, things like that. We were given the option of doing an amniocentesis and finding out then if she had it or not. I didn’t want to risk having one done, so I opted for passing on that and waiting until after she was born. In the meantime, I tried to educate myself on what CF really was, and what it might mean for us.

The most common way of diagnosing CF initially, is a sweat test. Jenna actually passed the sweat test, and it was when we did genetic blood work at 3.5 months old that we found out. It took a couple weeks so the actual official diagnosis came when my daughter was 4 months old.

What treatments/protocols do you follow to help treat CF? Jenna has a “mild” case of CF, compared to most with it. The average CF patient takes between 4-5 pills before every meal or snack. Most CF patients need these enzymes to help digest and absorb their food.

Jenna goes to her cystic fibrosis clinic every 1-3 months.

Daily, she does chest therapy with a special vest. She puts on the vest that has tubes attached. The tubes go from the vest to a machine that forces air through to pound on her chest. This process helps break up the excess mucous in her lungs. She does it for 20 minutes in the morning and 20 minutes at night.

In addition to the chest therapy, Jenna does one nebulizer treatment daily. This medicine is called pulmozyme (a mucoyitic) and she will need to take it for the rest of her life.

Right now, those are her only daily treatments. If she gets sick, or develops a bad cough, we will often have to do additional vest therapy and additional medicines.

This past December she spent two weeks in the hospital. While there she received a PICC line (http://en.wikipedia.org/wiki/Peripherally_inserted_central_catheter) through which she received some heavy duty antibiotics. This was her first hospitalization, so we consider ourselves very blessed. The average CF patient spends more time in the hospital in a year than most people will in their entire lifetimes.

Is there a cure? And what is Jenna’s prognosis? Currently, there is no cure for cystic fibrosis. Jenna will always have this disease, unless a cure is found. The current median age of survival for someone with CF is 37.4 years.

What are your biggest concerns/fears for Jenna with CF? The obvious, that I will lose her at an early age. I also fear that people will treat her differently once they learn she has CF. While the disease is in no way contagious, people still hear the word and get afraid.

Every time she gets a cold or a cough, it could be much more severe for her than the average person. SO I am always concerned.
How has this effected your family? It was a huge shock. To actually hear the words CYSTIC FIBROSIS…it’s very unnerving. It has had quite an impact. Tho, Jenna was my first child, so her lifestyle being the way it is, has been all I know.

For my extended family, I think it has meant more tolerance and understanding. Also, it’s brought attention to a illness that lots of people know nothing about.

Now that we know I am a carrier and my daughter has the disease, when members of my family decide to have children, they have a heads up to see if they are carriers for the illness.

How do you stay positive? At first, I was very negative. It’s a very scary thing!

Then I realized we were blessed because of her mild case. While we have medicine and treatments to do daily, there are plenty of people out there who deal wit a lot more than we do.

ALSO, we have come leaps and bounds in the field of CF. Studies on new medications are extending the life of patients all the time! Back in 1955, children who were diagnosed with CF were not expected to live long enough to attend grade school. So, to come as far as we have, is huge.

I feel that as long as we continue to raise funds and awareness, we WILL cure this disease. One day CF will stand not for cystic fibrosis…but for CURE FOUND.

If anyone would like more information, they can check out the CF foundation’s website at www.cff.org or email me at mi_cfmom@hotmail.com

I urge you to go to the CF Foundation’s website and learn more about CF. I pray that sometime in the very near future doctors will find a cure for CF. And I thank Julie and Jenna for sharing their story with us.