Hello. My name is Jennifer and I am 15 years old and in 10th grade. Before I begin to tell you about Neurofibromatosis (NF for short), let me tell you a little about myself. I love drama and journalism. In both 6th and 8th grades, I landed the main parts in school plays. In 9th and just last November in 10th grade, I participated in my school's musicals, though I didn't land a "part" since I cannot sing to save my life! I still had a lot of fun, though, and made many great friends.
Now, about my "disease." When I was about 13 months old, I was diagnosed with Neurofibromatosis (NF). It is a rare disease that can basically affect any part of the body. When most people think of NF, they think of tumors that grow on the outer part of your skin, making you look like you have a lot of bumps and warts. But that is only one type of NF, and not the type that I have.
My NF affects my major arteries, which is not too fun. My parents tell me that I was a pretty happy, but not a very healthy baby. I was born almost 7 weeks premature and weighed only 5lbs. My blood pressure was always extremely high, and the doctors just couldn't figure out what the culprit was. In fact, one time, when I was about 6 months old, my blood pressure was 244/146 (normal should be around 110/60), so you can imagine how scared my parents were. The doctors gave me some pain killers because I was crying (I guess I was in pain), and sent me home.
The thing about high blood pressure is that it is EXTREMELY hard on the heart. If the diastolic (the number on the bottom of the fraction) is higher than about 80, and the systolic (the number on top) is above about 125, then your heart can be permanently damaged. Needless to say, I was in the hospital a lot...about 7 times before I turned one. Not long after my 1st birthday, the tests really started and I was diagnosed. My parents were devastated and I spent most of the next 3 years of my life "living" in hospitals, undergoing more than 6 operations because most of my arteries were closing. One of my major arteries to my right kidney completly closed up. Though the doctors tried to do bypass surgery to open up the artery four times, it failed each time. Finally my kidney "died" and had to be removed. Then about 1/2 of my left kidney shut down, so I am now living on the other half. Cross your fingers...so far it hasn't really affected me.
This clogging of my arteries is really a problem. When I was 10, the doctors found that 20% of my aorta (the main artery carrying blood from the heart to the body) was 80% closed. Once the aorta reaches 90% closed, you have a good chance of having a heart attack. I was absolutely terrified --wouldn't you be? Because of the blockage, I was not getting enough blood to my body. Just walking around the house made me tired. Many doctors were afraid to take my "case" and help me. The condition was so rare, and they were afraid they wouldn't be able to correct the problem. Finally, when I was 13 years old, we flew to Georgia where one of my earlier surgeons agreed to try. I was terrified.
When we arrived in Georgia, my doctor said that he didn't think I really needed to be operated on. He didn't think it was serious enough yet. However, he did say that I was going to have to be in a wheelchair. A WHEELCHAIR A WHEELCHAIR A WHEELCHAIR!!! That was all I could think about. You see, I was going to a private school where everyone strived their hardest to be "perfect"; where everyone looked down on you if you didn't make straight A's, which I didn't. I got a few B's and an occasional C and they didn't like me for that. I was terrified about what they were going to say if I suddenly appeared in a wheelchair. I worried that in 7th grade, hormones race and everyone acts a little weird anyway. My mom understood my concerns and agreed to homeschool me. My friends would come to visit, and that was great, except when they wanted to do EVERYTHING for me, thinking that I was helpless.
I got stares from people in public places. You should have seen the looks when we came upon a curb or something that we could not get my wheelchair on. I would just say, "oh I can walk up that" or "I can climb that." People were dumb- founded. The thing that made it even worse was that I participated in so many activities in my community that I could no longer do anymore: softball, volleyball, swimming, hanging out at the mall.
When I was 14, I was put BACK in the hospital (this being my more than 30th time) to have stents put into my aorta (little metal rods that are supposed to open up my aorta and allow blood to flow through). The surgery was successful and I was able to go to school the next month, right on time. (I had the procedure before freshman year). Freshman year went smoothly...well, as smoothly as it could being a FRESHMAN (no offense to any freshman, its just that you get teased a lot) and I had to see the doctor a LOT. I see 8 different doctors at my clinic. I am deaf in my right ear, probably due to one of the 7 strokes I had as a baby. I see a doctor to make sure that I do not go deaf in my left ear. I see an opthamoligist (eye doc) because I was born with a SMALL, SMALL, SMALL tumor on my right optic nerve (a part in the back of your eye). It has not grown since the day I was born, but you can never be too careful. I see a cardiologist becaue of the damage done to my heart as a result of my aorta "crisis." I have to see a doctor once a year to make sure my 1/2 of a left kidney is functioning correctly. I also see a doctor that x-rays my brain to make sure my eye tumor does not grow. I have to miss a LOT of school. Other than that, I am fine!
Then on August 22 of last year (sophmore year, 8 days before school started), I had to have 2 more stents put into my aorta because it was narrowing again. I was not nervous because I had already had the procedure done before. No biggie. That's where I was wrong. During the operation, I was awake, but "out of it". Not for long, though. I woke up right in the middle feeling like I had been kicked in the stomach. It turned out that my aorta had been nicked, causing internal bleeding, and TREMENDOUS pain. Thank goodness I had scar tissue from previous surgeries, or the internal bleeding would have been much worse.
For the next few days I had to live not only with the pain, but with the doctors mumbling around my bedside and talking in "their language" that I couldn't understand. They didn't discuss things with me. They would pull my mother aside and tell her, and tell her not to tell me "just yet." They were afraid that more stress would make things worse.
All of this happened recently, and I am still recovering from the ordeal. I cannot carry heavy loads, and I keep all of my school books at home. The school provided me with an extra set to keep in the classroom. A lot of times while I was in hospitals, people would say things like, "Oh, I'd hug you, but I'm afraid I'd break you." and things like that. When I tell people about NF, they think they can catch it and they step back. I do not resent them for this: they are just uninformed.
I am also a "teacher's pet" in some of my classes. I have a hard time keeping up with the "pace" because I am tired often. Many of my teachers give me extensions on assignments and this makes many class mates jealous. Luckily, I have had MANY great friends who look at me for who I am and not "what I am." There have been teachers who didn't believe there was anything wrong with me because there isn't anything "external" that you can see.
I just want to say that if the person next to you gets special privileges or other things, there's probably a good explanation. And the next time you see people in wheelchairs or with another type of disability, don't look at them as "disfigured" or "crippled." Think of them as people. Everyone deserves that!
Thanks for reading this. Sorry it's so long. It would be longer, but I summed it up for you... Jen
Here I am with my brother Zachary and my sister Carrie
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Joan Fleitas, Ed.D., R.N.
Associate Professor of Nursing, Lehman College, CUNY
Bronx, New York 10468
Last updated: November 14, 2004