I chose Cystic Fibrosis as my topic because I know what it's like to live with it day after day. I know what it's like to wonder about the future, or to wonder if I even have one. Not many people know about CF (Cystic Fibrosis) or what comes with the territory of the disease. So I'm going to take you into my world. Please keep in mind that this is a world I live in everyday and there's no escaping it.
Cystic Fibrosis is a genetic disorder that affects the respiratory, digestive, and reproductive systems. It's an incurable and sometimes deadly disease. CF causes chronic coughing and difficulty breathing. Also, it prevents the body from getting nutrients and energy from food, causing the patients to be hungry more often because of the lack of nourishment they are taking in. Often, CF patients feel very tired do to a lack of energy, which is why an occasional nap can work wonders.
CF is caused by a mutation (my defective genotype is Delta F 508) in the gene (called cystic fibrosis transmembrane conductance regulator - CFTR), that produces a protein that helps regulate the flow of chloride, sodium, and bicarbonate ions in the epithelial cells. Epithelial cells are found in the sinuses, and bronchial mucosa, and in the pancreas, salivary glands, biliary tract, intestines and reproductive tract. When this process is slowed or blocked it results in the classic symptoms of CF: dehydration and thick mucus that clogs up the lungs and other organs, which in turn can cause a lot of infections and colds. A normal person's lungs have a thin slippery layer of mucus along the lining. A CF patients' lungs have a thick, sticky layer of mucus that clogs up the lungs and traps dirt, dust, and pollen which makes it very difficult to breathe and often makes them sick most of the year.
The mucus of a CF patient also blocks enzymes from getting to the stomach. So CF patients have to take special pills full of enzymes to help them digest their food. If these pills are not taken the food they eat won't be digested properly and can give them bad stomachaches, gas, and other discomforts. Cystic Fibrosis affects the body in other ways as well, such as slowing down puberty. Kids with CF generally develop a lot slower than their friends, which can be embarrassing for anyone.
According to the Cystic Fibrosis Foundation the life expectancy as of 2000 is about 32.5 years. Now, almost 40% of people with CF in the United States are 18 or older.
Clinic visits are another faucet of CF. The visits can take up to three hours because there are several people to see: a nutritionist, a counselor, a physical therapist, lab technicians, and of course the doctor himself. They poke you, measure you, weigh you, and the take blood and mucus samples. They also have to test to see how much air your lungs take in by having you breathe into a machine that measures the intake by how much is breathed in and how long it takes to release it. Then an inhaler must be used to do the test all over to see if the results change.
Doctors do all they can to keep us from getting sick, because our immune system is weak, as well as our lungs because over time, CF destroys lung tissue. Pneumonia and other illnesses are very dangerous to CF patients because they tend take a very heavy toll on the lungs and makes it extremely difficult to breathe.
However, not all is lost for patients do to the brilliant minds of the doctors who are fighting this battle with us. They have come up with ways to help clear their patient's lungs of the thick mucus. The flutter, which is similar to a child's bubble pipe has a metal ball in it that vibrates when breathed into hard and fast, which then sends waves of vibration through the passages of the lungs, knocking the phlegm loose so that it can be coughed up.
The ABI vest is another device used to loosen the mucus. It is a vest that is hooked up to an air pump. The vest covers mostly the chest and upper abdomen. The air pump blows up the vest to a snug fit. Then air is sent into it rapidly, causing a very strong vibration, which knocks the phlegm loose to be coughed up. Another method of clearing the lungs is to take decongestants in through a nebulizer, which is a pump hooked to a long hose, and mask that goes over the mouth.
Having CF is no picnic if you haven't gotten the idea by now. Here is a quote from a 17 year old young man with CF no doctor or even I could have said this better about CF:
"When you're sick with CF, you feel as if you're drowning in your own body fluids. For everyday you're lying in that bed, the nights stretch twice as long. You're scared because there's no guarantees that you're doctor's bag of tricks will work this time?it seems that the nasty little germs get more resistant every time their faced with a new antibiotic. I know what it feels like to think that everybody in the world's having a good time but you because the only faces you see are nurses, doctors, and lab technicians. Who poke you full of needles and suck blood out of you until it hurts so bad, you think you're going to scream. I know what it's like to cough up your guts and to vomit blood. And I know how it feels when you wake up one morning and realize you're still alive and that you've made it through another episode, only to know that you'll have to do it all over again. Until one day you don't wake up. "
I don't think it could have been stated better. Life with CF is hard at times but it has its ups and downs. I'm teased a lot no matter where I am because of my CF, my weight, and how crazy I can sometimes be when I'm having fun, but a friend of mine once told me that, " Life is to short to live it by other people's rules. Who cares what someone else thinks of you? What matters is what the people you love think of you. What other kids think of you isn't important. Because the friends who appreciate you for who you are, are the ones that will be there for you when you need a friend."
One-day scientists hope that the letters CF will stand for Cure Found! (CF Foundation 15) and that when people hear about CF if will be ancient history, that no one will have to live with it ever again. That is just a glimpse of my world. If I went into all the emotional details, I would be writing for the rest of my life. Life is too short for that especially when you have a potentially fatal, incurable disease like Cystic Fibrosis.