My battle with Diamond-Blackfan Syndrome began at birth. I was born with a
Hgb. of less than 2 gms. That means I had no red cells. After getting a blood
transfusion, my parents were told that I was fine. Then at the age of ten weeks,
my mother noticed a problem and took me to a doctor. Again my blood count was
around 2 gms. They rushed me to the hospital again for transfusions. I nearly died
several times before they got the blood into me, but the doctor brought me back. Eventually,
at four months of age, the diagnosis was finally made after a bone marrow biopsy.
Diamond-Blackfan Syndrome/Anemia, or DBA, is a rare disease which affects the bone marrow.
In simple form, the bone marrow does not produce or make red cells. I was started on prednisone,
a steroid which the patients usually respond to and begin making their own red cells. However,
I did not respond to the treatment, and I have had to be transfused all my life as the blood cells
die off and I become anemic.
I have tried several experimental treatments through the years, which I have not respnded to.
The problem with getting too many blood transfusions is that I am also getting too much iron and it is
storing in my body. To get rid of the iron, I take a daily 12 hour i.v. of a drug called Desferal. It is
used to chelate the iron and get rid of it through the urine. Even though I have taken this drug since I
was 2, the iron is still building and is around 8000. It has caused Hemochromotosis in my liver and is
affecting other organs in my body. I developed Hepatitis C and am now fighting the effects of liver disease.
I also have been diaganosed with pancreatic insufficiency, and have to take enzymes every time I eat to help
digest my fats and absorb them. This condition usually is not found in DBA, and some doctors have told me that
I may have my own disease. We are still calling it DBA because there is nothing in the medical books that has
all the symptoms that I have with this. My doctor I am using now feels like it is a rare variation of DBA.
I have been having a lot of problems with low platelets and my white cells are lower than normal also. I have
been taking IVIG every three weeks or so for a year and a half and it has helped me fight infections. As of yet,
we haven't needed to get platelets. I have trouble with my thyroid because of the iron, so I take thyroid medicine
through my central line- the enzymes I take were breaking down the pill form and I wasn't absorbing the medication
like I should. A central line or a Hickman Catheter is an I.V. that is put in a main artery or vein and all I have
to do is hook up to my I.V. and get my blood drawn without being stuck all the time with needles. They also have a port
which is under the skin, but since I get medicine everyday, I need the line.
We did all the testing on my brother for a bone marrow transplant, but he didn't match well enough. We discussed with
my doctor about an unrelated bone marrow transplant a few years ago. After another biopsy on my liver, the risks were
too high. They said I would need a liver and a bone marrow transplants, which is too risky. I decided with my doctor
that I didn't want to go through the transplants.
I have had to use a wheelchair for several years because my legs and bones are weak. I don't like to use it, but my bone
doctor says I need to use it. I also wear braces on both my knees to help control them since they are weak. My occupational
therapist is working now on trying to get me a motor on my chair to give me more freedom when I am out so my Mom won't have
to push me around. My arms are weak and I get too tired when I try to push myself. My specialist agreed on the motor since I am
still weak from the recent infection I had.
Now this page will be sort of a diary with updates on my condition. I became really sick on April 20, 1997, with a bacteria
in my blood stream. I was visiting friends in New Orleans for the weekend, including seeing my friend Tracy Lawrence in
concert, when I got sick. The morning after the concert, my mom took me to the emergency room at Tulane Hospital. My blood
pressure was real low and I had a high fever. They admitted me to get I.V. fluids and cultures. I was started on antibiotics.
They were hoping I would get to go home and go on to Little Rock to my doctor the next day. The tests showed the bacteria was
a dangerous kind so until they got me stable, I stayed there. I developed fluid in my lungs and around my heart and started going into what they call congestive heart failure a few days after being admitted. They gave me I.V. Lasix to pull off the fluid, and they finally got me on the right antibiotics and I started to get better. The
next week, my doctor in L.R. put the transport team of Arkansas Children's Hospital to get me up there. The came down to get me
and my mom on a big plane with two nice medics. I was happy when they finally got me there to my doctors that I knew.
After getting to Children's, they decided to take me to surgery and remove my central line, which had become infected when the
blood cultures were drawn back into the tube. It is hard to sterilize a line. Six days later, they took me back to surgery for
a new line. I got to go to the Ronald McDonald House the next day. My blood sugars were high so they were watching them and I
finally started taking a pill to help control them. I am still needing to use oxygen when I sleep and some during the day. I don't
know why, but my blood count is not holding up since I got sick and I am needing blood about every week it seems. I got to come home
for a week before going back. It is good to be home after five weeks. Thanks to Children's and my doctors for all they have done for me.
Thanks to my friends at the Ronald McDonald House in Little Rock for spoiling me as Mom says. Thanks Mom for helping me on my page.
July 1, 1997
There's not much going on lately. I got to go spend a few days in Nashville last month. I went to the City of Hope Celebrity Softball Game.
It was fun, but it was really hot. I went to Tracy Lawrence's Fan Club party, and it was great. His special guests were Charlie Pride and Troy Aikman.
The highlight of my day there was when Tracy dedicated my favorite song, "Texas Tornado" to me. He is so nice to me, and I can't wait to see him again. I would have liked to do more, but there is only so much
to see in the few days we were there. I hope to go back next summer and stay the week for Fan Fair.
I went back to Little Rock last week. I saw the Hemotologist, who sent me to my Liver Specialist. He ordered some tests on my liver and stomach, which
weren't too bad. We are waiting to hear from the results of the tests. I got two units of blood and my I.V.I.G. treatment over three days. We go back in
two more weeks. The best thing I did in Little Rock, was see a friend of mine from California. She came to see me while traveling through the states with her
Mom on their way back up to the N.I.H.. We haven't seen each other for two years. Melissa has H.I.V. and we met up in Maryland at the Children's Inn. She is Cool, and
we have a lot of fun when we are together. We went swimming and to the water park for about three and a half hours one day. We had fun, but I got really tired. It was fun to see her again.
I went up to see one of my friends, Jonathon, while at the hospital last week. He is going to another hospital this week to prepare for his bone marrow transplant, so I
hope you will remember him in your prayers. They have found a donor for him. I hope to get to go up and see him as soon as he is feeling better. Well, that is all that has
been going on lately. I am feeling stronger and am not using my oxygen as much during the day. Take care and come back to visit me soon.
July 29, 1997---Well, I just got home from Little Rock again. I went for my regular treatments of IVIG and got sick that night. We first thought it was a reaction to the I.V.I.G.,
but since I had been having some dizzy spells at home, the doctor on call wanted me to come over to the emergency room. Thank goodness we were still at the Ronald McDonald House across the
street. They ran some blood work and my glucose level was nearly 900, so they admitted me. After trying to stablize the level with pills, they finally had to put me on insulin. I spent a week
in the hospital to be sure the insulin was adjusted to my needs. I am having to take three different types of insulin mixed twice a day. I also had to be started on my nightly tube feedings again.
All of this has been a little depressing, but I know I have to do it. After staying two weeks in Little Rock, I got to come home for a week.
Also, my platelets are not doing so well. They have been falling and were down to 31 last week. That may not seem too bad for some of the cancer patients, but I have a problem with my clotting so it is
not good for me. The places I am having to give my shots have been bleeding in the tissues, causing some horrible looking bruises. My doctor still wants to hold off on giving me platelets until the last
minute. I hope they start coming up again. The nurse told me that what platelets I had were going straight to my finger sticks and shot places. I also got two units of blood before I came home.
I got to visit my friend Jonathon at the hospital. He was feeling better and we hope he can go for his transplant soon. He is a good friend. I pray for him and all my friends every day.
I haven't been to any concerts in nearly a month. I had planned on going to see my buddy, Ty England, next week, but I am going to have to cancel my plans. It is an outside show and the weather is just too
hot to go. I will just have to wait till next time.
October 2, 1997--Well, there isn't much been going on lately. I got home this weekend from a week in Little Rock. I saw four different doctors and Mom talked to another one. Since my blood sugars are running high again lately, they increased my insulin doses and I have been getting some smaller shots in between. They also changed up my thyroid medicine some to get it under control. My blood counts were okay, but I did get two units of blood on separate days, plus, the I.V.I.G. treatment.
I saw my orthopedic specialist, and he told me I could take off one of my braces since it was bothering me. I got my motor on my chair, which I didn't like at first. After seeing how easy it was for me to get around on my own, I think it will be all right. It is hard on my Mom to have to take it apart to put it in the car trunk, but we are hoping to get something put on the back of the car to transport it.
While I was in Little Rock, I was invited to a fish fry on Friday night. As a special treat, we were picked up from the Ronald McDonald House in a Limosine. That was my first time in a Limo and it was so cool! They limo driver was so nice to show me all about the different things like putting up the privacy window, the t.v. and the music. We had so much fun. They are so nice to me.
October 18, 1997- Hi! We just got back home from Little Rock last night. Heather received two units of blood and her I.V.I.G. treatment. They also did some xrays of her spine and hip area, where she has been having so much pain lately. The xrays didn't show any fractures or holes, but did show some osteopenia, which confirms the Osteoporosis that the doctor suspected. We understand this happens to the patients that are in Heather's stage of the disease. We will continue with the current pain management at this time.
While we were there, we took her and her friend, Jonathon, to the State Fair one night. They didn't stay but a few hours, but they both had some fun. Heather rode the ferris wheel and two more rides that didn't jerk her around. She isn't able to ride the rough rides because of the pain and the blood counts. The two enjoyed teasing the nurses at the clinic the next morning by telling them they went Bungy-Jumping. A few actually believed them for a while.
We are home now for a few weeks before going back for more treatments. Thanks to all of you that have e-mailed Heather with your compliments about her page. She loves to get cards and e-mail from everywhere to cheer her up.
Well that's all for now. Don't forget to go back to my home page and sign my guestbook before you leave! It was a pleasure having you stop by. Until next time, Take care and God bless
all that enter here.
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