BMT Failure

[chic88]

Hi! I am new here and we are desperately looking for a community during this difficult times.

My boyfriend was diagnosed with SAA last April 2015 he then underwent BMT the following month May 2015. The donor was his sister. After his BMT he was receiving BT continuously during those months because his blood cells are not proliferating. 2 PRBC/day and went on for several months. Until he reached his 100th day in the hospital that the doctor said that its graft failure. He has frequent ICU admissions because of this. In addition, he developed atherosclerosis just 3 months ago and developed somed arrythmias. Last one he had was ventricular tachycardia thank God he was treated immediately.

Anyway, since hid BMT he never went out of the hospital, thats 9 months hospital stay and its very tormenting and depressing especially for him he is just 28yrs old and had a very active life.

It has been a month and a half that he was not receiving BT because his blood cells were okay but recently just a couple of days ago his blood cells drastically went down and we really dont know what to do he is receiving BT again as of now.

What is his chance of going back to his normal life or even at least go back home out of the hospital? Can this mere BT work for him since 2nd BMT would be too risky as what his doctor told him.

Any insights or comments are really appreciated. We are really scared of this situation I hope that he would cope up with this.

[Marlene]

I'm really not certain if I can lend any insights. I probably have more questions than answer at this point.I don't know the exact terminology but others have received an additional boost of stem cells from their original donor. I don't think it happens often but have they considered this as an option?

Are all three blood lines affected or just red cells?

I don't know where he is being treated but if it's not at a hospital with lots of experience with SAA and BMTs, then I would consider getting another opinion.

Others experienced with BMTs will chime in and may have more suggestions for you.

[chic88]

Thanks for your reply. The doctor just told us that getting 2nd transplant would be too risky for him and said that he will keep on receiving blood transfusions until his blood cells will normalize. Im afraid of the iron overload and some other complications of long term blood transfusions. He has this nasty pneumonia right now as a result of frequent BT.

With regards to his blood cells, most of the time it's his WBC and platelets are low but just recently his RBC went down as well.

I think the doctor that he has is quite experienced but now we are thinking of looking for some other opinions as well but really dont know where to start...

[Marlene]

An additional infusion of the donor cell does not necessarily require any additional pre-conditioning. Here's one study reflecting that.

http://www.ncbi.nlm.nih.gov/pubmed/11279309

I'm not certain I understand how BT is causing pneumonia? Of all the cell lines, red cells tend to be the easiest one to tolerate. Why are they continuing to treat him as a an inpatient? Even if he's getting BT daily, that can be managed as an outpatient. Patients seem to do better out of the hospital than in especially after such a long stay. I'm sure there's more to his story though.

I would seek another opinion. Maybe post in the transplant section of this site and ask people for recommendations. Where is he being treated at now? You may need to travel to a different center.

[carrieridge]

If u have graft failure it means the donor cells are gone and your cells are back. If they do a dli then yes chemo would be required because you would have to kill of your immune system before infusing the donors. We are in this position now

[Barbara K]

Hi, Chic88. About 15 years ago now my nephew (then age 13/14) underwent a mismatched unrelated BMT for Chronic Myelogenous Leukemia. Similar sort of story--weeks turned into months that went by with him living on transfusions and having no immunity at all, and then finally his counts started to rise but it turned out to be his own cells. So the transplant was a failure, except for the fact that it did temporarily knock back his leukemia. After about seven months of combined hospitalization or living in a very sheltered situation very close to the hospital, he was allowed to go back home, quite a bit the worse for wear and seriously demoralized. After about a year or two the doctors started to talk about trying a second transplant (and no, they would certainly not have considered trying one immediately), but he had been switched to a then-new med called Gleevec which was keeping the cancer (which had returned along with his own bone marrow) in check, and he made the decision not to subject himself to a second attempt.

The upshot? Today he is happily married to a lovely young woman, gainfully employed, and hoping to start a family. He does have a few side effects from the radiation and Gleevec, but nothing that prevents him from living a rich and full life.

Will this kind of story be your boyfriend's? I can't say but very much hope so. I just wanted to share it as a way of encouraging you to believe that he can indeed overcome this setback. It's a different illness he is facing than the CML my nephew had, and different challenges are involved, but it's also 15 years down the road, and the doctors have a whole new arsenal of treatment techniques that weren't available in 2000/2001.

Best wishes,
Barbara

[Hopeful]

Hi chic88,

Do you know if your boyfriend was tested for inherited forms of AA (like Fanconi's) before transplant?

I hope he can quickly fight his current infection!

[chic88]

Hi Marlene!

Thanks for the link.
He has been in the hospital until up to this time because of his unstable blood results, fever, arrythmias and all and the the doctor wont allow him to go home.

Yes I agree with you that recovering at home would help, I guess but the doctor wont allow him to be out of the hosp in that matter.

This is his 2nd hosp actually in NY. Is it okay to say the hosp here? Anyway, we will try to ask for some other opinions and look for another hosp with BMT specialty.

[chic88]

Hi carrieridge! You also have same experience with this BMT graft failure? Did he/she undergo chemotherapy? Is his/her case is also SAA?

[chic88]

Hi Barbara!

This story really inspired us a lot! Thank you so much for sharing this. It lifted his spirit up! We always encouraged him in all the way that we can and your story made him smile!

We will keep our hopes up with this battle and I know that he will keep fighting and win over this! We hope to find a way for his treatment as well..

[chic88]

Hi Hopeful!

No, he wasnt tested for that. But what i know is that he doesn't have any relatives who have AA.. Yes We really hope that this infection would at least lessen and his blood components would recover too..

[Hopeful]

Hi chic88,

The reason that I asked is that the pre-conditioning regimen for inherited forms of the disease is different from that of immune-mediated forms. When I met with my local transplant team, they said that they would have to refer me to another facility if I tested positive for the inherited forms.

So, it is very important to rule out the inherited forms of the disease before transplant - especially with a sibling donor.

It is possible for someone to have an inherited form of AA, even if their parents showed no signs of the disease, if both parents were carriers. It is usually auto-recessive.

Hopefully, this is not the issue with your boyfriend, but it is worth talking to his doctor's about the possibility.

You may want to check out Dana-Farber, if you are in the NYC area and are looking for a second/third opinion. You should look for an AA specialist, not just a BMT specialist. Another option to consider is NIH in Maryland.

Stay strong and keep searching for answers!

[Marlene]

I too echo Hopeful's comment on ruling out inherited forms. They tested John even though there was no history in his family. He was 51 yrs old at the time.

When John was undergoing treatment, they discharged him with zero counts. He went in every day for blood products and IV meds. He would get re-admitted if he got a fever though. That being said, Johns Hopkins was set up to handle the outpatient care and when he would have to be re-admitted, he would go directly to the room. All the re-admit paper work was done in his room bypassing all admit processes they make you go through in normal circumstances.

And yes, you can say what hospital he is being treated.

[chic88]

Hi Hopeful!

Thank you so much for the information. My knowledge bout AA is so limited since its so new to us. I would ask his doctor about this.

We will also try to inquire regarding that institution here in NY as well. Do they have some good AA specialist there? We will give everything a shot.

Thank you for the encouragement! I appreciate it so much..

[chic88]

Hi Marlene,

It's nice to know that in Johns Hopkins they allow that. I think it's too much for a patient to be admitted for almost a year already. I will raise this query to his doctor too.

He's admitted in Montefiore Hospital in NY.. Do you think Johns Hopkins is a good hospital of choice based on your experience? We are trying to gather some infos about hospitals and specialists for 2nd/3rd opinion pertaining to this matter.

[Marlene]

John's doctor has treated many SAA patients. He was also at NIH prior to joining Hopkins. We felt Hopkins was a good match for us as was his DR.

The one thing I feel strongly about is that it is important to have a doctor experienced with these rare diseases. If that's not possible, then the doctor should be in contact with them.

In the Northeast/Mid Atlantic area, Dr. Brodsky at Hopkins, Dr. Neil Young at NIH and Dr. Maciejewski at the Cleveland Clinic all have good reputations in the SAA world. There may be a few more but I can't recall at this point. Other may be able to suggest some.

[Hopeful]

Hi chic88,

Since you are new to AA, I would suggest checking out the Aplastic Anemia and Myelodysplasia International Foundation website (if you haven't already) at:
http://www.aamds.org

They are a fantastic resource for information and support.

They have a bunch of on-line video presentations by leading experts across the country that can help you come up to speed on the basics [and beyond] of this disease. Here is the link to the ones on AA:
https://www.pathlms.com/aamdsif/categories/300/courses

They also have a support line that can help you find specialists in your area.

As Marlene said, it is critical that you get a second opinion by an expert in this rare disease! You can call the research nurses (Olga R. or Barbara W.) at the National Institute of Health (NIH) to explain the situation and see if your boyfriend could get a consult at NIH. Treatment there is free. Here is a link to NIH that lists the phone numbers/email for Olga and Barbara:
http://www.nhlbi.nih.gov/research/in.../research-team

Good luck!

[chic88]

Hi Hopeful!

Thank you so much for the link you provided!