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My daughter, Diana, is now 21 and was diagnosed with DBA when she was one. I don't usually post, and
just tend to "lurk", but some of the Posts have prompted me to share her story. It took about 8months to have her
diagnosed, as all of you I'm sure are familiar, this was a long and frustrating experience, and we went
through several Dr, before arriving at the Children's Hospital in Winnipeg, Mb, and finding someone with experience with
DBA who could make a proper diagnosis. She was steroid responsive, and at her lowest dose she was at 2.5Md every other
day. She continued on this regimen until she was about 9, with no problems, or side effects. Her counts and growth were
normal, and she was normal in every way. At about 9, during the summer, we became busy, and she (and I) forgot her
meds.
All did continue as normal, and we pretty much forgot about the DBA,other than a blood test every year. By this time we
had moved to Southern Alberta, lost touch with her original Doctors, no one was familiar with DBA here at that time, and
all was well. We were lulled into a false sense of security, and we all pretty much forgot about her being "sick". Anyway,
when she was 14 she became very tired and pale. As she'd had a blood test about 2 months prior to this, I wasn't too
concerned, so we went on holidays, and didn't think too much about it. We were busy, took her white water rafting, hiking,
and I nagged her a bit about being a typical "lazy" teen. (oops...sharing mother guilt right now). On return home, I took
her in for tests, and her counts had dropped to 30 (I feel very blessed she survived that summer), and the floor dropped
out of our lives. We were sent to Calgary, where we went through 2 hematologists. She was no longer steroid dependant,
and we also had to convince the new Docs that she did have DBA, and that I had returned (we were warned that if she
went off steroids, that she might not respond again when she was younger). We then started transfusions, and this was
very difficult on her. The first Dr. wanted her to go to about 70 before transfusing to try to challenge her system to
produce hemoglobin, but she had zero energy. He was also very unsympathetic to travel, and issues for a teenager with
DBA. We switched, and she was able to transfuse at 100. We made this decision so she could continue life, and studies, as
normally as possible.
She followed this site very closely, and was terrified about needing chelation, and she started investigating BMT, and
bringing it up with her Dr. We went ahead with testing of the family, and agreed if no matches, no BMT. I have 4
children, and 2 were perfect matches. She made the decision, and I ensured with psychologists, school counselors, and 2 of
her DR. that she was old enough to make this decision. (I was terrified, and would have been glad to allow transfusions,
and chelation forever). We used her older brother as the donor, and in Feb 2001, she had the stem cell transplant. All
went extremely well with the process, until about 39 day post transplant, then her liver and kidney shut down. She was put
on EXTREMELY high doses of IV steroids, and taken off all other meds. It was a terrible time, and we didn't know if she
would pull through...she did. Even medical staff told me she was considered a miracle. She recovered well, with some GVHD
of the skin (bad rash, that did go away) and gut (bad diarrhea, that went away). During this time she didn't miss a beat,
and graduated high school with her class. Have I said she is an amazing person? About a year later she began complaining
about pain in her hip joint. Again it took a while, but was diagnosed with vascular necrosis (due to steriods...most likely the
extremely high doses used during the liver/kidney problems). We decided on a new procedure, rather than a hip replacement
in which a piece of the fibula (or tibia-get confused) is put into the hip, to prolong need for replacement...than was a
mistake, as exactly a year later she had to have a total hip replacement (for that year she could barely walk, and had to
use a crutch). After the replacement, she immediately moved, and looked normal again. Her recovery after both the
transplant, and the replacement have gone smoothly.
Earlier this year she had to have her gall bladder removed. I'm telling you about the hip, and gall bladder, because these
are two things that are can happen after a BMT. When speaking with the transplant Dr., and staff, it was like, oh yes,
that does happen. We weren't told about any of it, but should have been made more aware, I think. The transplant was
done at Alberta Children's Hospital in Calgary, Alberta, and her follow up now is at Tom Baker Cancer Clinic, in Calgary.
Both are great places, and she has received excellent care. Diana is now in Halifax for a year and a half, living a
completely healthy and happy life. We have talked about if she could go back in time, and remake her decisions, she says
she would do it again. She is living a normal active life, and even the hip replacement hasn't slowed her down.
Someone asked if a child was steroid responsive, would the do the BMT...my answer, not on your life. I'm not even sure I
could have made the decision about a BMT, if she had been younger getting transfusions. That first year after the BMT is
probably one of the hardest I ever endured as a parent. There are also the "after effects" to deal with, that I feel
weren't fully explained to us. Diana was the first DBA stem cell transplant in Alberta, and I can't help but wonder if they
had done chelation, prior to the transplant if she would have had the liver and kidney complication. (was never given and
answer, and it probably never could be answered).
Pat, mother to Diana, 21, BMT, Feb, 2001...doing great
My son Mark was the first one to try the mini for DBA. He is now making his own blood keeping at 14 or more for a
hemoglobin. His mini was done on 10/03/03 at Brighams and Womens hospital in Boston. His hematlogist is at Dana Farber
in Boston. Dr. Lipton is also Mark's doctor since Mark was around 5 years old. Beleive they have a very good relationship.
I'll tell you what Mark had to go thru. To Start with a mini just means that he had chemo for 4 days to bring his bone
marrow to almost none as compared to aregular bmt which would not have any left. This is the mini part because it is
mostly all the same. As his Boston doctor explained, doing the mini means that the patient has the his own marrow left to
get him going if the mini does not take. Also if he wanted to he still had the option of doing the regular bmt if he wanted
to do that in the future. Mark said he does not think he would have done it but you never know. He had to stay in the
hospital around 10days in isolation instaed of I believe longer for a regular bmt. We were home but with many trips to
Boston for a while. He had to stay away from people and especially crowds for the better part of a year. He made it to
the Camp Sunshine to see Dr lipton and Dr Alter and of course every one else even if he was still feeling unsure if he
would continue to be blessed with blood we might say Ha! It definitely was an experience but I am so glad he tried the
mini instead of the regular. Mark's donor was from out of the country and believe me if I met that gentleman I think I
would break down in front of him. God bless him. That was the other thing his sister was not a match so we had to get in
the system for a match. They found two. Dr Antin wanted two if possible and the second was the best choice.I would
speak to Dr Lipton he knows Mark very well and I am sure Dr Vlachos is also involved in his case as far as working with
Dr Lipton. Maybe we will meet at the Camp Sunshine this year. We eill be there at least for 1 day hopefully. This year
people will be able to speak to Mark more about the mini. He was kind of shy last time and really was only starting to
find out what the future would hold for him. He did get a little host vs Graf but was lucky with that too. After he went
off all his meds over 1 year after his mini he came down with a cold and then a severe case of shingles which he is still
feeling to this day but doing much better. As for how we found out about the mini it was Mark's hematlogist here in town
who researched it and felt Mark should try it. May God bless him. It is a very hard decision and I am glad he made it
with the help of people who love him. If I can help you please let me know but I absolutely recommend speaking to Dr.
Lipton.
Take Care
Maddy-mother of Mark born with dba 10/18/70 and had mini bmt 10/03/03
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