This website is dedicated to Philip Hopkins. He was officially diagnosed at 3 weeks old via sweat test. I am just a mom trying to save my baby's life.
(The first half of this website is written to educate about CF and keep people updated on Philip. The second half is dedicated to our fundraising events and sales).
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. Because the mucus is so thick, normal bacteria that do not usually harm you and I, can be deadly to a CF patient. This is because the cilia in the airways cannot move the mucus containing bacteria out. It becomes trapped, and often leads to pneumonia.
Mucus also can block the digestive tract and pancreas. The mucus stops digestive enzymes from getting to the intestines. The body needs these enzymes to break down food, which provides important nutrients to help people grow and stay healthy. People with cystic fibrosis often need to replace these enzymes with medicine they take with their meals and snacks, which helps them digest food and get proper nutrition. Eating food or drinking without taking enzymes can cause poor absorption and very painful cramping.
Approximately 30,000 children and adults in the United States have cystic fibrosis. An additional 12 million more—or about one in every 3o Americans—are carriers of the defective CF gene, but do not have the disease.
Cystic fibrosis is a genetic disease. That means people inherit it from their parents through genes (or DNA), which also determine a lot of other characteristics including height, hair color and eye color. Genes, found in the nucleus of all the body's cells, control cell function by serving as the blueprint for the production of proteins.
To have cystic fibrosis, a person must inherit two copies of the defective CF gene—one copy from each parent. If both parents are carriers of the CF gene (i.e., they each have one copy of the defective gene, but do not have the disease themselves), their child will have a 25% chance of inheriting both defective copies and having cystic fibrosis, a 50% chance of inheriting one defective copy and being a carrier, and a 25% chance of not having CF or carrying the gene. As in Philip's case, many families have no known CF family members, and the disease has never manifested in their family before now.
There is no known cure for Cystic Fibrosis. There are currently some promising drugs in clinical trials that we are hopeful for. There are, however, many excellent treatments and preventative therapies available now that were not available even a few years ago. In 2008, the median predicted age of survival rose to 37.4 years, up from 32 in 2000. The median predicted age of survival is the age by which half of the current CF Patient Registry population would be expected to survive, given the ages of the patients in the registry and the distribution of deaths in 2008.
A typical day in the life of a CFer can vary greatly, however, most require enzymes before every meal, airway clearance techniques at least once a day, special vitamins (CF patients do not absorb fat soluble vitamins A,D,E, and K), salt replacement, nebulizer treatments, and a host of medications to prevent or treat illness. CF patients can do most things that a normal person can do, including school, college, sports, etc. They just have to take care of themselves.
CF patients must avoid all forms of smoke, especially any type of cigarette smoke, whether it is in the air or on clothing. Candles and fireplaces can also be a danger. Basically anything that can introduce foreign particles into the lungs should be avoided.
This website explains our journey from start until present in hopes of enlightening friends and family and maybe helping new parents into the club they never thought they would have to join, but will definitely meet some of the greatest people ever!