Cystic Fibrosis Foundation

The Cystic Fibrosis Foundation is working constantly to raise money to support new studies that will benefits people who suffer from Cystic Fibrosis. New technology and medication has lead to great advances. Patients are living longer, and healthier lives than ever before. Over 40 % of the CF population is over the age of 18. Many of the people who have Cystic Fibrosis can look forward to a life that usually last into their 40's. Researcher's are constantly coming up with new technological advances that will improve that life span even more. Kids with Cystic Fibrosis are just like any other normal kids, they just have to watch their diet and medicine intake. Most kids would take the following:

• A pancreatic enzyme supplement
• A multi-vitamin
• Aerosolized medication
• Take part in breathing clearance exercises

Other than that, kids are able to take part in everyday activities that kids without CF are able to, such as play sports and other things.

Current and Past treatments for Cystic Fibrosis

As of right now, there isn't a set cure for Cystic Fibrosis but doctors along with the Cystic Fibrosis Foundation are working tirelessly to find new ways of limiting harmful symptoms. The goals of the treatments are to controls infections of your lungs, loosten and remove the thick mucus from your lungs, prevent intestinal blockages and provide nutrition suitable for patients with CF. Treatments for the lungs include:

• Antibiotics for airway infections
• Physical therapy to increase lung performance
• Exercise programs
• Other medications

The antibiotics that patients are given for their infection depends on the strains of the bacteria involved, how serious the infection is and tolerance to antibiotics. The chest physical therapy involves pounding on your chest and back to loosen the mucus and become able to cough up to mucus to remove it from your system. Chest physical therapy (CPT as it is more commonly referred to) should be done 3 to 4 times a day. CPT also involves letting gravity work its magic. Patients can lay on their stomachs with their heads down while you perform CPT, allowing gravity to help dislodge the mucus from your lungs. In some cases, CPT is too physical for some people so there have been devices created that create vibrations that help dislodge the mucus. Breathing techniques are also used frequently among patients. Exercise is encourage because it can:

• Loosen the mucus
• Encourage coughing that helps remove mucus
• Improve endurance and overall wellness

Anti-inflammatory medicines are also used to help reduce inflammation of the lungs. Oral steroids are most commonly used. In serious cases, lung transplants can be performed but patients must find a donor similar to them in size and are qualified to donate organs.

Symptoms of Cystic Fibrosis

Most of the symptoms are directly related to the thickening of the mucus inside your body. People with CF often suffer from frequent coughing, decreased immunity to bronchitis and pneumonia which can lead to permanent scarring and inflammation of the lungs, salty tasting skin, dehydration, infertility (primarily in men), ongoing diarrhea or other digestive problems, extreme appetite but lack of weight gain due to malnutrition and finally stomach and intestinal pain. CF can also lead to chronic diseases such as sinusitis, bronchiectasis, pancreatitus, nasal polyps and the collapsing of lungs. While everyday life with CF is managable, if not treated correctly it can lead to many harmful diseases later in life.

How is Cystic Fibrosis Diagnosed?

Your family doctor will have to conduct a through physical exam of yourself as well as look at medical records from family members. The doctor will also have to conduct many tests to ensure an accurate diagnosis. One test is the sweat test, this is the most effective in detecting CF. This tests for the amount of sodium within your perspiration. This test is conducted by having a doctor rub a small amount of the chemical pilocarpine onto your arm or leg. An electrode is then attached to that spot, this electrode produces a mild current that produces sweat. After this, they attach a gauze pad or filter paper to the spot and wrap it in plastic. After about 30 or 40 minutes, the doctors remove the plastic so the sweat produced in on the paper or gauze and then can be further analyzed. This test is usually done multiple times and sweat with high contents of sodium is usually CF. Chest X-rays can also be done to show any scarring or inflammation of the lungs. Sinus X-rays can also be conducted to show signs of sinusitis. Basic lung function tests can be completed such as testingyour lung capacity, how quickly you breathe air out of your lungs and how well your lungs add oxygen and remove carbon dioxide from your blood. These tests can all be completed to give doctors accurate information to base their diagnosis on.

What causes Cystic Fibrosis?

Cystic Fibrosis is caused by a defect in the gene called the cystic fibrosis transmembrane conductance regulator gene. This gene is responsible for producing a protein that controls the movement of salt and water in and out of the cells in your body, and people with cystic fibrosis, that gene doesn't function properly. We recieve one CFTR gene from each of our parents, offspring who inherit one abnormal CFTR gene from each parent will have Cystic Fibrosis. People who inheirit one abnormal gene and one normal gene will not have CF, but will be CF carriers. CF carriers have no symptoms of Cystic Fibrosis and lead normal lives but are able to pass it to their offspring. When two CF carriers have a child, that child has a 1/4 chance of inheireting two abnormal CFTR genes and having Cystic Fibrosis. A 1/4 chance that they will inherit two normal CFTR genes and not have CF or become a carrier. 2/4 chance of inheriting one normal and one abnormal CFTR gene and becoming a carrier of CF without getting it, just like their parents.

What is Cystic Fibrosis?

Cystic Fibrosis is a genetic disorder that affects your mucus and sweat glands. In most cases, mucus in the body is thin and watery. It prevents the lining of certain organs from drying out and becoming ceceptable to harmful infections. People with Cystic Fibrosis have mucus that is thick and sticky, which builds up over time and blocks airways to the lungs. That blockage makes it very easy for bacteria to build up in that area and can lead to serious lung infections. That thick, sticky mucus also affects other vital organs such as the pancreas. Clogging of your pancreas ducts results in a restriction on digestive enzymes from reaching your small intestine, where they are most effective. Without those enzymes, your intestines become unable to absorb proteins and fats completely. As a result of these restrictions, nutrients leave your body unused which can lead to you being malnourished. Intestines become unable to absorb vitamins A, D, E and K as well and you might experience intestnal gas, bloating and pain. This abnormal gene also causes your perspiration to become extremely salty. This results in an extreme loss of sodium when your perspirate. That loss of sodium can lead to an unbalence in minerals in your blood and makes you more ceceptable to a heat emergency. Cystic Fibrosis can also lead to infertility, mostly in men.