Wednesday, February 1, 2012

Cystic Fibrosis

What You Need to Know

What Is 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.
In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.

Symptoms of Cystic Fibrosis

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.

About 1,000 new cases of cystic fibrosis are diagnosed each year.
More than 70% of patients are diagnosed by age two.
More than 45% of the CF patient population is age 18 or older.
The predicted median age of survival for a person with CF is in the late 30s.
*information from

My 19 year old daughter, Rachel has been sick since she was just weeks old. First diagnosed with gastronomic reflux at 2 weeks and then asthma at 6 months of age. That was only the beginning of the long journey to her recent diagnosis. For four (4) years she was in and out of doctor offices, emergency rooms, etc with everything from constipation, fevers, colds, respiratory infections, bronchitis, pneumonia, dehydration etc.. Then finally we were referred to Children's National Medical Center in Washington, DC. It was there, where the extensive tests were done, numerous procedures combined with numerous hospital stays that she was diagnosed borderline cf (cystic fibrosis) , gastronomic reflux was confirmed and a diagnosis of bronchiectasis.  

So what is borderline cf? A sweat test was performed several times and each time the numbers came back high. Too low to be called normal but not high enough to say they were 100% sure it was cf. The only thing this did was cause problems with the insurance company because she needed to be treated with the same medications as a person with cf but the insurance didnt want to pay for it because she wasn't actually diagnosed with it.

She was treated as if she had cf and was on medication for years and then one day she no longer needed the medicines she had been taking for her breathing. So then they said ok she is fine. Her only diagnosis then was the gastronomic reflux and asthma despite the fact that she continued to have bowel problems which was never treated. We were always told to increase her fiber intake, give her laxatives etc...

A few years later her health started to decline again and no one could figure out the problem.  She was having problems with breathing, her chest hurt and she wasn't having bowel movements. 

To make a long story short, a few months ago we finally got the diagnosis of cystic fibrosis and she is being treated. However, the medications, the special diet and the light exercise doesn't cure and sometimes doesn't even help.

They have come along way with extending the life and quality of life of someone with cf but they still have a long way to go before they have a cure.  So why not give so they can further their studies and experiments and hopefully one day come up with a cure.

Try this. Go outside take a deep breath of fresh clean air and exhale slowly. Doesn't it feel good to feel that fresh crisp air fill your lungs? Then come inside and thank God that you are able to do that.

Rachel (picture below) has formed a team, Reaching out for Rachel, to walk on June 2, 2012 with Great Strides to help raise money for a cure. Please find it in your heart to walk with us, make a donation or if you want to, do both.  If you cannot do either we will gladly accept prayers. Please pray for each person afflicted with cf, their families, their friends, doctors, researchers and any person who is adversely affected by this disease.Thank you from the bottom of my heart and May God Bless each and everyone of you.


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