I’m pregnant with my first baby and I got some tests done and the doctors informed me that my daughter will have Cystic Fibrosis. My mother had a brother and sister who died from the disease when they were quite young. Anyway, I’m wondering what is the current life expectancy for children/people with Cystic Fibrosis and how can you best take care of them to maximize their life?

I want to get my daughter involved in some good sports/physical activities. She’s only 3 years old. She has Cystic Fibrosis. We live in New York and walk a lot, she gets out of breath often when we go for walks and walk up hills. But the doctors say its good for her to have to work her lungs. What are the best sports and other physical activities I should get her involved in?

My son’s fiancee has Cystic Fibrosis and she’s pregnant with their baby, she’s 21. I know she’s generally very weak/frail and takes a bunch of different medications, and nebulizers and digestive enzymes. I was just wondering if anyone is familar and knows if such medications could cause harm to an unborn baby? She’s also having a hard time keeping enough weight on.

From Kirby Dick’s “Sick,” Bob Flanagan sings his own version of the Mary Poppins song.

It was back in 2000 when I rode my first ever “century” ride. I had prepared for about 6 months for the ride and had chosen the Cystic Fibrosis Foundation as the recipient of any money I was able to raise for the ride. I had studied the disease and spoke with representatives in Atlanta even met with some of the reps in Albany Georgia. I had also got support from the radio station B-100 in Albany and they would be following the ride. I remember the day well and the ride too. Maybe I will write an article about that soon.

During my time at Ohio State after leaving the Navy back in the Viet Nam Era days I had known a few people with CF. In 2000 I lived in Moultrie Georgia and do to this day, but I now knew quite a few more victims of CF. Many of the students at the Green Oaks Center where two of my sons attend were CF patients.

I had actually wanted to do the ride for the Autism Society, two of my sons are autistic, but when I contacted them they were not interested. They told me that they had a walk for a fundraiser and I was welcome to do that but not ride.

When I contacted CFF in Atlanta they said they would set it up as a wraparound event and would even let people know about it through their newsletters and press releases. The date was set May 19 2000, National Ride Your bike to Work Day and the day before Flag Day. I trained a lot for the ride. I had never ridden more than 50 miles in one day, so a 100 mile bicycle ride was new to me. To top that I was to celebrate my 48th birthday on May 21, just 2 days after the ride.

I also learned a lot more about CF and about people with CF from the ride and the Great Strides walk where I gave the money I had raised to the representatives on May 20. So the following is some of the stuff I learned about CF.

What is Cystic Fibrosis

Cystic Fibrosis is a genetic disease that interferes with operation of the exocrine glands. The exocrine glands are the ones that make sweat. mucus, tears, and saliva as well as digestive juices. CF affects primarily the respiratory and digestive systems.

Basically what happens is that when a patient has Cystic Fibrosis (also known as 65 Roses) the secretions made by the exocrine glands are too thick and can plug up the ducts in the lungs, pancreas and intestines. The disease also may cause higher than normal amounts of salt in the sweat. When I did my ride there were approximately 40,000 known cases of Cystic Fibrosis in the US.

The good news is that even though people are dying every day from CF, the life expectancy of the CF patient is improving. 1 in 29 Americans are have the CF gene and are carriers of the disease.

What are the signs and symptoms?

Many times it is found in babies because they have digestive problems such as a blockage of the intestine. Remember earlier we found that the secretions were too thick and that they often interfered with the digestive and respiratory systems. People with CF often have problems with breathing, long term coughs, pneumonia and wheezing.

Some of the symptoms that may take some time to develop would be things like clubbing of the toes and fingers. Clubbing is when the ends of the fingers and or toes become rounded and larger. The thick mucus in your intestines can cause abdominal discomfort, stools may be large greasy and smell very bad. Another symptom is sinusitis or inflammation of the sinus, nasal polyps may also grow in the nasal passages.

What can be done for CF?

At this time there is no known cure for CF and the treatment depends on the stage of the disease. Antibiotics are used to treat lung infections, digestive enzymes would be used to help improve digestion. A medicine to thin the secretions may help with breathing problems. Research has been ongoing and the life expectancy and the quality of life for a patient with CF has improved considerably since the 1970′s.

The CF patient can do a lot to help improve their life by eating a healthy diet, increasing water and juices, and possibly taking a fiber medicine. Note that food allergies are more common in CF patients so it is a good idea to test any new foods with very small amounts first.

Foods containing EFA’s such as fish oil and flax oil should be made a regular part of the CF patient’s diet.

Exercise should be used as tolerated by the CF patient.

By: Jerry Goodwin

About the Author:

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Cystic fibrosis, which affects 30,000 American children and adults, is a multisystem disease caused by a defective gene. Presently only symptomatic management is possible, but there are very promising gene-therapy trials under way.

In the respiratory system the thin mucus lining becomes thick and sticky. In cystic fibrosis management, the primary treatment of the system is to thin or clear this mucus. Bronco dilators like albuterol are used to clear the clogged airways. Mucus thinning drugs delivered by aerosol, like pulmozyme, are helpful. The most effective way of clearing this mucus is by mechanically dislodging it. Clapping on the chest and back, with the head tilted on the edge of a table, is quite effective. There is an electrical clapper that does the job safely. There is an electrical inflatable vest that vibrates and dislodges the mucus. Infection is an ever-present risk with cystic fibrosis patients. Regular shots for pneumonia and influenza are very important. Bacterial infection is fought with newer antibiotics like TOBY, which delivers the medicine directly into airways with aerosols.

Because of the blocked ducts of the pancreas and liver, the enzymes and bile do not reach the intestine. Though the patient eats normally or even in excess, the fats and proteins are not digested. So the cystic fibrosis patient needs to have the enzymes supplemented with oral pancreatic enzymes. There is also the need to take vitamins, especially the fat-soluble ones. If lung function is completely damaged, the only alternative would be lung transplantation. This would involve many factors, like the availability of a donor and the patient’s present health, prior to undergoing major surgery.

The Cystic Fibrosis Foundation has 115 centers all over America, and provides guidelines and advice. They support many studies to find gene therapy to cure cystic fibrosis. The target is to add normal genes to the cells of the airways to prevent further damage. Biotech laboratories produce healthy genes, which need to be delivered to the appropriate cells. These vectors, or delivery systems, are the key areas of research to combat cystic fibrosis. One method that is actively pursued is to modify the common cold virus to carry the healthy genes into the correct cells. On another track, the DNA molecules are compacted to their minimum size and delivered directly to the relevant cells without the need for a carrier such as a virus. This technology is called PLAS min.

As this gene therapy experiments are at an advanced stage, the cystic fibrosis sufferers can hope for a permanent cure in the near future.

By: Kent Pinkerton

About the Author:

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I walk you through my treatment routine.

I’m 15 and I joined a health club that has a hot tub, steam rooms and saunas and I have Cystic Fibrosis. Are these things good to use or not with my condition and for how long at a time?

I am pregnant and was tested for cystic fibrosis along with a million other things on the 30th of August. I was back at the doctor a week later on the 6th of August, and the doctor said my results all came back normal but the down syndrom and cystic fibrosis tests had not come back yet. On the 6th I had my pap smear and pelvic exam and yesterday on the 15th got a letter in the mail saying that my pap smear, urine, and blood tests, and quad screen all came back normal, but the box for cystic fibrosis was not checked, I’m wondering if that means I am a carrier of the disease or if it means they haven’t gotten the results in yet. I’m going to call my doctor in the morning, but I’m really worried. Does anyone know how long it takes to get the results back from that test?

I found out that my twin daughters have cystic fibrosis, what is the average current life-expectancy for people with this disease and how can the you maximize their life expectancy?