Forum:Matthew Ferguson's disease

Forums: Index > Personal Experiences >

Recessive Disorder (Cystic Fibrosis)
Every day when I wake up, I begin to deal with a condition I have known all my life, Cystic Fibrosis. I have this machine that shakes my chest for at least 20 minutes to help clear my lungs of the thick mucus that sometimes makes it difficult for me to breathe. At school, I cough a lot. In gym class, I participate in sports but often get tired easily. At lunchtime, I take pills to help me digest my food and get all the vitamins I need, which helps me keep up my strength to deal with this disease. There was this story I heard and it was about this woman and her son and her son had cystic fibrosis and she was on the phone talking about it and the boy over heard her and now when ever she talks about cystic fibrosis he thinks that she is talking about sixty-five roses.

What Is CF (Cystic fibrosis)?
Cystic fibrosis is a disease that causes the body to make thick, sticky mucus. This causes problems in two areas, the lungs and the digestive system. Healthy lungs produce mucus, which protects the airways and makes it easier to breathe. But for me, the mucus is thick and sticky and can clog up the lungs. This creates a place where bacteria can easily grow and cause infections. And it's not only the airways and lungs that are affected. Mucus-producing cells line the digestive tract, including the stomach, intestines, liver, pancreas, and reproductive organs. The pancreas produces enzymes that help digest food and hormones that help absorb sugar. When thick mucus in the pancreas clogs up the narrow passageways, it can make it difficult for me to digest food and get all the vitamins and nutrients I need. Cystic fibrosis affects more than 30,000 children and young adults in the United States. It can be mild or severe, depending on the person. To make normal mucus, the body needs a special protein. This protein is defective in cystic fibrosis, producing the thick, sticky mucus that causes problems for people with CF.

What Causes CF?
CF is an inherited disease, which means that it's passed down from parent to child. Someone who has CF was born with it. Maybe you've heard someone say, "It's in your genes." They don't mean your blue jean pants. Genes make up the blueprint each of your cells follows to make you a unique individual. Genes determine your eye and hair color and also are responsible for certain health problems. People with CF got the disease because their moms and dads each had a gene for CF. You need two CF genes (one from mom and one from dad) to have cystic fibrosis. Most people don't know that they carry the gene for CF until they have a child who has the disease because carriers of the CF gene do not have the disease themselves.

How Is CF Diagnosed?
Although someone with cystic fibrosis is born with it, it isn't always obvious at birth. It may take a while for symptoms to develop. Doctors may suspect that a baby has CF if he or she coughs a lot and gets a lot of lung infections. The baby also might have unusual large, bulky bowel movements or may not gain weight as expected. To know for sure, the doctors can do a simple test that doesn't hurt. Kids with CF have more salt in their sweat than other kids, so by simply collecting a sample of sweat and testing to see how much salt is in it the doctors can determine if a person has CF. Other tests may be done, including one that looks for the gene that causes CF.

How Is CF Treated?
The aim of CF treatment is to keep the lungs clear of mucus and free of infection. It's also important for me to eat well. Let's start with the lungs. I seem to always cough. I’m coughing to clear mucus out of my lungs. When I hook myself up to the vest machine that shakes me up in the morning, it is helping to break up the mucus that collected in my lungs overnight, which makes it easier for me to clear it out of my lungs. If the mucus stayed in there, it would make it harder to breathe and would increase the chance of getting a serious lung infection. A kid with CF will work with a medical team, including doctors, nurses, nutritionists, physical therapists, social workers, and respiratory therapists. A respiratory therapist knows a lot about breathing and how the lungs work. He or she can teach a kid with CF to do special breathing exercises that help get rid of extra mucus. Breathing treatments also help by adding moisture and delivering medicine into the lungs. Even if everyone does everything right, kids with CF will still get respiratory infections and need to take antibiotics, which kill bacteria. Kids with CF also may need to stay at the hospital for a while. The good news is that newer medicines are more effective and help kids with CF recover more quickly. Kids with cystic fibrosis have to work a little harder to breathe, and this burns more calories so it's important that they get enough to eat. Even a kid with CF who eats healthy foods and gets plenty of calories may still have trouble growing and gaining weight. The problem is that the pancreas is not working and can't deliver the enzymes needed to break down protein and fat in food. A dietitian can help kids figure out which foods to eat and provide special recipes packed with nutrients and calories. Along with eating right, it's important for kids with CF to get regular exercise to make their lungs as strong as they can be.

Living with CF
I have to take care of myself, but thanks to a better understanding of what causes CF and improved treatments, I can go to school and do regular stuff, just like other kids. Meanwhile, researchers are working on even better treatments and hoping that one day there will be a cure.