I was born with a genetic disorder called Cystic Fibrosis. This is a disease that is caused by a genetic mutation in a salt channel called the CFTR. This affects multiple systems in the body, but mostly it affects the lungs and digestive system. In the lungs the salt is not able to get into the airways, preventing water from entering as well. This causes the mucus in the lungs to get very dry and sticky. It remains there and then gets infected with bacteria causing constant infections that result in scar tissue. This is normally the cause of death for people with cystic fibrosis.
I was diagnosed with CF at one day old. A nurse noticed that my stomach was extended so I was Mercy Flighted to Children's Hospital in Buffalo, NY. There I had to have a surgery to remove a blockage in my intestines. I was then given a sweat test to determine if I had CF. With CF there is an increased amount of salt in the sweat so that is how it is diagnosed. I remained at Children's hospital for three weeks and then finally allowed to go home. At nine months old my electrolytes were messed up causing me to become dehydrated. I ended up in the hospital for ten days and spent my first Christmas in the hospital.
I was relatively healthy growing up. My CF was mild and I barely got sick. I didn't have to take too many medications compared to some others with CF. I had to take enzymes before I ate food and had some other medications that I had to take on a daily basis. I had to take some extra vitamins (because my body does not absorb them well), albuterol through a nebulizer, and a constant antibiotic to keep bacteria away. This was discontinued when doctors started finding out that bacteria grew resistances to antibiotics with extended use.
I didn't do any airway clearance until I was a little older. I believe I was around the age of nine when I started with a handheld device called a flutter. I blew into it and a large metal ball inside would flutter up and down causing a vibration in the lungs when blown into. It was suppose to be done twice a day for about 15 minutes. I hated this thing and my parents fought with me all the time to do it. I used this for quite a few years until the vest airway clearance system came out. 
The vest is placed around the chest and the machine fills it with air and then generates a pulse of air that compresses and releases the chest wall. I do the vest twice a day for thirty minutes each session. The picture on the left was the first model that came out. That thing was huge and very heavy. It was really hard to take any where if going on vacation. The one on the left is the one that I have now. It is much smaller and lighter and easier to take places. It is still hard to lug around with all the other luggage, but after having the original one, this new one is so much nicer.
I was always small for my age, both height and weight. At the age of twelve I only weighed fifty pounds. I was told by my doctors that I had to gain five pounds by my next appointment or I was going to need a feeding tube placed. We scheduled a follow up appointment for two months later and went home. I was determined not to have to need a tube so I was constantly eating. Before I went to bed I was making milkshakes and eating spoonfuls of peanut butter just for some added calories. After the two months we went back to the doctor's and found out that I lost three pounds. I was very upset and begged for a little more time, but they told me it couldn't wait anymore.
In May 1999 I had the feeding tube placed in. It is a small plastic device that goes into the stomach. The picture to the left shows how it is placed. From the start I had problems with the g-tube. After the surgery I was suppose to be in the hospital for three days, but I got pneumonia and then the incision got infected so I ended up staying there for about eight days. I came home was pretty sore for a couple of weeks. I was able to start the extra feeds about a month after the surgery. I used a large syringe and would push two 8-oz cans of a formula called Nutren 2.0 into my stomach two times a day. I did this for a few months without much problem. Then one day I did my feed in the morning before school. I got into a fight with my mom about something and stormed off to school mad. I went to my friend Katie's house because I would meet her there and we would walk to school together. When I was waiting for her to get ready I all of sudden didn't feel good. I ran to the bathroom and threw up. Her dad took me home and told me mom what happened. My mom figured it was just because of the fight and I was upset so she waited for my stomach to settle down and sent me to school. I was fine all day, but when I did my feed again I threw up again. Come to find out my stomach couldn't handle all the formula at once anymore. This is when I switched to night feeds where a machine pushed in all four cans over seven hours while I slept. This worked much better and was actually easier then having to fit in two feeds in the day and still eat food. 
About a year later I had to go in and have the g-tube replaced. Initially they gave me a tube called a PEG tube. This type of tube has a hard plastic end and it placed in first to allow the stomach to heal. However it only lasts for about two years. It is not a great fit so the stomach acid tends to leaks out. I went in to have it replaced with a MIC-KEY tube. This
one has a balloon on the end that is filled with water making it a lot easier to remove. To remove the PEG they place a metal stick into the hole which stretches the round peg allowing it to come out. When they did this they found out that it was stuck to the inside of my stomach. They then cut the tube in half hoping the other half would fall into my stomach and I would just pass it. When they cut it the other half was still stuck so they had to take the metal stick and actually push it into my stomach. It was extremely uncomfortable and I definitely felt it when the thing finally gave and fell in. After it was in we all considered it a success and they placed the MIC-KEY. Well when I went home I could not stop throwing up. I had to come back that night and they did an X-ray and found out that the peg was just swimming around in my stomach. I was admitted into the hospital. Long story short...I threw it up the day just 30 minutes before they were planning on putting a scope down my throat to remove it. Thankfully I was able to go home and never have to worry about something like that again. With the MIC-KEY the balloon can just be deflated and taken out. The downfall of the MIC-KEY is that the acid breaks down the balloon and after three months it breaks and has to be replaced. When that happened I would just have to tape it to my stomach and go in for a new one. The MIC-KEY did not help with the leaking though. I kept a gauze pad on it at all times, but that didn't do much. The acid still ruined a lot of my clothes and burned my skin. The constant acid on my skin caused the area around the g-tube to be red and raw. It also caused scar tissue to form underneath it, which looked like a large bubble. I had to constantly burn it with silver nitrate sticks to keep the growth under control.
There was some good news with the g-tube though. In less than a year I was starting to look better. My face wasn't so drawn and my arms and legs had some meat on them. By the time I was fourteen, I no longer looked like a bean. Overall I gained over forty pounds with the feeding tube. At the age of seventeen, I was finally able to keep my weight on without the extra feeds. I had it removed right before Thanksgiving. One last problem though...the hole in my stomach was suppose to close within three hours after being removed and it never did. I had to go in for surgery and have it closed by a surgeon. The good thing that came out of that was while I was under the surgeon removed the large bubble of scar tissue.
Throughout all this time my lungs remained healthy. The doctors keep track of my lung with a pulmonary function test (PFT's). They are based on a percent predicted for a given age and height. So 100% means the lungs are functioning at a level that is predicted. I get one done every time I go and see my CF doctors. In 2003 my PFT's were 93. This is when I was seventeen and at my best health wise. I no longer needed the feeding tube, I didn't need enzymes and I felt great.
In 2007 they went from 76 to 55 in just four months. This was the first time I was hospitalized for a CF exacerbation. This is the term that is used when the bacteria in the lungs get out of control and affect breathing. The hospital stay helped me get my PFT's back up to 76 in June 2007, but in July they fell back to 55 and they remained in the fifties. Two days before Easter 2008, I got this stabbing pain in my side. I wasn't sure what had happened to me. All I knew was that my side hurt real bad and I was having trouble breathing. On Easter I couldn't handle the pain anymore and went to the ER where they admitted me. The doctor told me that part of my lung had collapsed. This resulted in bringing my PFT's down to 39. I was able to get it back up the fifties after my admittance, but they quickly fell to the 40's and by 2009 they were down in the thirties. When my PFT's remained in the low 30's that is when my doctor recommended me to go meet with a lung transplant team. She said it is still a little early for an actual transplant to occur, but it is a good idea to have the information with them because just one flu can drop my numbers dramatically.
At first I was going into the hospital once or twice a year. After the lung collapse I was in the hospital every four months. After two years of doing that I was going in every 2 1/2 months. That is another reason why they referred me for transplant.
Thing continued to get worse while I was on the list. Before my transplant I was going in every six weeks and my PFT's dropped as low as 18%. My doctor was getting worried and suggested that I try and list at other centers to possibly increase my changes. I knew (as well as everyone else around me) that I wasn't going to last too much longer. I was working on getting listed at Pittsburgh, but I got the call before it was complete. On July 6, 2013 at 4:22 AM I got the call from New York that a pair of lungs were available. Jason and I flew to NY and twelve hours later I was on the operating table.
My life has changed dramatically since I received that precious gift. Even when I was recovering I felt better than I had in years. Once I gained my strength back I was able to drive and go places on my own. The feeling of getting my independence back is a greater feeling than I can describe. Before my transplant I hated that I couldn't do anything. I was always stuck in the house on my computer and watching TV. I was missing everything. This even included cooking and cleaning. I promised that if I got lungs I would never complain about doing those things again. I have kept to that promise. There are days that I have a lot going on and feel a little overwhelmed, but I wouldn't want it any other way. I have the energy to keep up and that is all I need. I love cooking now. I don't mind grocery shopping and cleaning either. Even when I am tired. I love that I CAN be the one doing it now. You will not hear me complain about doing any of these things. I refuse to take anything for granted. You know that feeling when you have a long day and you are exhausted? You lay down in bed and your body relaxes into the mattress and pillow all the tenseness melts from your body? That is an awesome feeling. I didn't get that feeling when I was sick. I was exhausted all the time and lying down in bed didn't make it feel any better. Now I get that feeling where my body can relax after a long productive day. I love it!
