Info about cystic fibrosis?!

Info about cystic fibrosis?

No, I don't have it. one of my best friends does, she's a year older than me. she's 14, turning 15 in november. i know she does treatments and i know it has to do with the lungs and it basically cuts your life in half, or around that. i also know that she's had multiple surgeries and also has an inhaler. she's a great friend and it scares me, just the thought of her having to go through so much and i dont like talking to her about it, so i was just wondering:

the treatments- what exactly does she have to do?
surgeries- for what?! something with the lungs, i know.
CF in general- just more information. like, what effects does it have?
and, why does it take years of her life?

My son has Cystic Fibrosis- he's now almost 6 months old. Although I have not gone through 14 years of treatment I can help answer some of your questions. First of all... it is so wonderful that you are showing enough love and concern for your friend to want to learn more about what she's dealing with. She's lucky to have a friend like you.

About 2500 babies are born with CF each year... that's about 1 in 3000. And about 30,000 are living with it in the US now. It's most common in caucasians and especially those of a Northern European decent (some mutations are specifically tied to Swedish and Dutch decent). A baby with CF who has not been diagnosed will likely not gain weight like they should... and sometimes will be admitted to the hospital with "failure to thrive" meaning they aren't getting enough nutrients and aren't healthy. They might even be born with something called meconium illeus which means that the first bowel movement is stuck in the intestines and they have to go through surgery to fix it. Babies with CF also have really icky, stinky diapers. That's because before they get the medications, the food they eat isn't being digested and it's just passing right through them... ewwww!!! As if dirty diapers aren't bad enough!! They also might get colds often or infections that don't go away often because their body doesn't fight it off.

Here's a funny thing about CF... it makes you taste salty! Since that salt isn't being transferred correctly, it comes out in the sweat! I remember when my son was very little, I would kiss him and he tasted like a salt lick! That can be very important when it's hot outside because CF patients can get dehydrated VERY quickly! It's important that they drink LOTS of water on these hot summer days!

Cystic Fibrosis is a genetic disease (that means you get it from mutated genes passed on from BOTH of your parents and you're BORN with it- you don't develop it). That also means that it is not in ANY way contagious. Both of your parents need to be "carriers" of a defective CF gene in order to pass it to their child. A carrier means that you have only ONE copy of the defect, someone with CF has TWO copies of the defect- one from each parent. There are thousands of different gene mutations that cause CF but the most common is called Delta F508. My son is a homozygous Delta F508 which means he has two copies of the same mutated gene- DF508. He got one copy from each of us- myself and his dad. As carriers of CF, we have no symptoms. In fact there are MILLIONS of people out there who are carriers and don't even know it becuase they aren't affected by the disease.

Cystic Fibrosis is a terminal illness meaning that it results in death if we don't find a cure. CF affects the salt transfer between cells in your body through "ducts" in the organs. Any organ that has ducts is affected by CF but the most affected organs are the pancreas and the lungs. Most CF patients who carry DF508 are pancreatic insufficient meaning that the pancreas does not work to break down the food they eat- this causes them to be malnourished and many many times very thin- even if they eat a LOT of food! Your friend could probably eat FOUR TIMES what you do and still be skinny!! And it's because the pancreas is not able to excrete the enzymes that are needed to break that food down and digest it so she can get the nutrients from it. The medication/treatment for that is pretty easy- she takes pills before every meal or snack that are full of the pancreatic enzymes she isn't making anymore. That breaks down her food for her inside her stomach and intestines so she can get the nutrients and vitamins she needs to stay healthy. My son, at only 6 months old, takes two capsules every time he eats so I imagine your friend takes 5-10. And that's with EVERY meal!! That's a lot of pills! The pancreas insufficiencies can also cause Diabetes later in life but she's probably not dealing with that yet...

The other main problem CF causes is lung disease. CF patients are VERY susceptible to viruses and bacterias because they are not able to just cough up the icky stuff that makes us sick. You know when you have a cold and you cough all that gross stuff up and then a few days later, you feel better and all cleared out? Well, someone with CF has a VERY hard time getting rid of the junk in their lungs because the mucous becomes very thick and very sticky and it sticks itself to the lining of the lungs and stays there. So if she gets a cold, it can easily develop into something serious like pneumonia and require a trip to the hospital! The thick, sticky mucous inside the lungs of a CFer make it a very happy place for bacteria to grow and live.... they don't die off like they would in a healthy person with clear lungs. CF patients are also very sensitive to a bacteria called pseudomonas that can be very very dangerous and cause permanent lung damage. She probably has regular throat cultures (where they stick that long Q-tip thingy in the back of your throat and make you gag... ick!) to check for bacteria. If they find bacteria, she will take antibiotics to try to get rid of it. Your friend probably takes a lot more antibiotics than you ever will in your entire life. Now, the way she can help her lungs is to do what's called Chest Physiotherapy. My son uses a little cup device that I call the "Thumper" and I just hit his chest and his back to help loosen up the mucous that is stuck in there. When they are older (like your friend) they get to use a vest that vibrates to shake it loose instead of having to beat it out of them! :) She also probably takes a nebulizer (inhaled) medication called Pulmozyne that is specific to CF patients and helps make that mucous thinner so she can cough it up and get rid of it. Most people with CF cough a lot more than anyone else and that's a GOOD thing! It's good when she coughs cause it means her lungs are trying to get rid of the mucous that can make her sick. The inhaler she carries around might be a little device called a "Flutter" which also helps loosen the mucous when she breathes into it... it vibrates when she exhales and helps loosen anything that might be stuck.

There are a couple other meds they can take like Zantac (for acid reflux) and Vitamins A, D, E, and K because they aren't absorbed well when you have CF so you need extras. She might also take a certain amount of Ibuprofen every day just to help with inflammation from coughing and build-up.

All in all, she probably takes 3-5 medications a day, multiple times a day and does the vibrating vest treatment once or twice a day. But, it's just her routine. Part of her life. She probably grew up doing it and didn't know any different until she starting hanging out with friends who don't have CF. The surgeries she's talking about probably have to do with something called a G-Tube. When your body can't digest food and you start losing too much weight and become sick, the doctors will put a tube straight into your stomach to get food and nutrients into your system to keep you healthy. Actually, many CF patients have had a G-Tube at least once in their life by 14 or so. Her body just needed a little extra help getting food for awhile. She might even have a little scar on her tummy from it. The other surgery she MAY have had is if she got a bowel obstruction at some point. If her intestines became blocked with food and junk, they would have had to go in and remove it by surgery.

They are currently working on a cure for CF. Just ten years ago, many CF children were not even making it through their school years and the average life span was about how old your friend is now... Today, with the hard work and advances in medications that we have to help them live longer, the average life expectancy is between 31-35 years. There may come a point in your friend's life when she will go on a waiting list for a lung transplant. That will help her- although it will not cure her CF- it will just give her cleaner lungs to kind of start over with. The main reason people with CF die is from respiratory failure. The lungs just get too clogged and eventually they can't get oxygen to the blood and the body can't breathe. As you know, you need oxygen to breathe and to live and so when your lungs aren't strong enough to get the oxygen in your body, it's like suffocating. I know that sounds so horrible and awful and I'm really not trying to scare you! I'm just trying to be honest with you so maybe you can understand her a little bit better. And just so you know, she won't just wake up one day with her lungs not working at all- it would take time and she would become very sick and probably go to the hospital. So please don't think that it's just going to happen one day without warning!

Something really really cool that they have been working on recently in the search for a cure is called gene therapy. Since CF is a genetic disorder and is caused by a defective or mutated gene in the DNA, gene therapy is designed to go in and "replace" the bad gene with a normal one. Sounds pretty easy, huh? If you know there's a broken one, go in and fix it! Well, you'll probably learn more about gene therapy in school here soon- High School most likely.

Just be there for her. She might not be afraid to talk about it and answer questions you have. Some people with CF have trouble with their disease because they feel alone and they feel sickly compared to others. I don't know how she feels about it but you might try. Just tell her you're curious about what CF is all about and what her day is like! Go over to her house after school one day... maybe she'll let you try the vibrating vest!!

Good luck, hun! Hope I was able to help you understand a little bit... and don't be scared for her. Just be THERE for her. And try not to treat her any differently- don't treat her like she super fragile and can't do anything... she knows her limitations and is just like you and anyone else- a kid! Enjoy life!!