Update on Grant B

[Wendy Beltrami]

Hello All,

Thought I'd check in and let everyone know what's going on with Grant.
Well, it's been 11 months since Grant had his second round of ATG (rabbit this time) and he is STILL transfusion dependent. Keep in mind, he was never transfusion dependent for the first 7 years of his AA so this has been quite a year for us.
We are still fighting a losing battle with Blue Cross trying to obtain Exjade for a cost that normal people can afford. His ferritin is about 1900 at last check and he (thanks to a special angel) has so far had a month's worth of Exjade.
The newest news is that a donor was found that is a 9/10 female with one pregnancy and a mismatch at A. Not a great choice.
We have always said that if we did ever go to transplant, we wanted to go to where they have done the most for children with AA.That is Children's Hospital of Wisconsin- as of June 2006 they have done 40 according to the NMDP statistical chart. We have spoken with Dr. Margolis by phone and after our initial conversation, he speculated that accepting this donor and moving to transplant may not be the best choice with Grant's current state of health.
(Transfusion dependent on prbcs every 3-4 weeks and platelets every 2-3 weeks with ANC usually over 1000). He may be a little too healthy to move to a 9/10 donor transplant at this time.
We have decided to take a trip to Cleveland to consult with Dr. Maciejewski and then hop over to Milwaukee to consult with Dr. Margolis.
Several things have been mentioned as possible next steps and after talking with these guys, we hope to have a clearer picture of which direction to take.
*another ATG, this time back to horse
*male hormones
*daclizumab
*rituximab
*campath
*hope for new trial to start with AMG 531 (this is exciting- it's for platelets!)
*do nothing
So, I ask that everyone keep us in your thoughts as we make this quick trip.
Grant's dx date was 12/4/98 so we just passed the 8 year mark. It seems like forever, and yet it seems like only yesterday that this bomb was dropped on us. It's hard to believe he has been dealing with this for over half his life.
It's also hard to believe that he is still so "healthy". He is a high school sophomore and singing in the choir and just being a teenager who happens to spend a couple of days a month in the hospital getting blood transfusions.

Wendy B.
mom to Grant/ age 15
dx 12/4/98 AA
ATG x 2

[Marlene]

Hi Wendy,

Have you considered adding Johns Hopkins on your list of who to check out? The High Dose Cytoxan has had good results when used as the first line of treatment. When used after AGT failures, it's not as successful. You are exploring so many avenues for Grant, this is the only one you did have on your list.

Hang in there....

Marlene

[Wendy Beltrami]

HI Marlene,
As you said, when used after 2 rounds of ATG, success rate is not good. We are more interested in looking at options that are likely to have higher success rates or at least ones that would not have detrimental effects. Since cytoxan is pretty harsh, that is not one that we would likely try at this stage.

Wendy

[julia69]

Hi my name is Julia.I to have a son with A.A. he has had it for 4 years and has had 3 treatments.I to feel the frustration with being sick but not sick enough for certain treatments.We did go and do a protocol at N.I.H. FOR THE DACLIZAUMB.Yippie it did'nt do anything.I say that we don't have moderate or severe A.A. WE HAVE STUBBORN!
God bless and good luck,
Julia

[Wendy Beltrami]

Hi Julia-
Amen to that! Grant is now trying Danazol, 200mg bid, along with 125 cyclosporine. It's been about 4 1/2 weeks now and I don't think there is much improvement. I think daclizumab is on the list as one thing we will try next.

I was looking at some of the labs that were done when we went for the consult at Cleveland Clinic prior to starting the Danazol. One was for erythropoietin level and another was haptoglobin. I don't recall him being tested for these two things in the past.

Can anyone give me any interpretive info on understanding this lab result?

Epo value was 855

Haptoglobin level was <20

Hematocrit on this day was 32.4

Wendy B. mom to Grant

[Marlene]

Hi Wendy,

I can comment on the EPO level. The normal range is 4 - 19 for an adult male. It's not uncommon for it to be high (way out of range) for those with AA. We were told to expect it to be in hundreds, or even in the thousands.

They say if it's under 500, then procrit may effective in boosting red cells. John's was under 500, and procrit did work for him for a couple of years. He's no longer on procrit and his reds are holding around 10.8 without it so I assume is EPO levels are good enough.

Hope this helps....

marlene

[Wendy Beltrami]

Thanks Marlene, that is helpful and explains why Grant hasn't been given Procrit.


Wendy

[julia69]

Originally Posted by Wendy Beltrami

Hi Julia-
Amen to that! Grant is now trying Danazol, 200mg bid, along with 125 cyclosporine. It's been about 4 1/2 weeks now and I don't think there is much improvement. I think daclizumab is on the list as one thing we will try next.

I was looking at some of the labs that were done when we went for the consult at Cleveland Clinic prior to starting the Danazol. One was for erythropoietin level and another was haptoglobin. I don't recall him being tested for these two things in the past.

Can anyone give me any interpretive info on understanding this lab result?

Epo value was 855

Haptoglobin level was <20

Hematocrit on this day was 32.4

Wendy B. mom to Grant

I know you said it's on your list, I really loved the idea of a kindler gentler way but I swear it was a placibo we got, nothing changed, I mean I could how one cbc next to a before and nothing. We went severe a few month after trial.
Best of luck,
Julia and Gabe