Re: Bleeding Disorders in Mito?

[Guest guest]

my son is now on amicar as he has a similar bleeding issue. we spoke to a surgeon last night and he feels that most of the mito kids he has dealt with have similar issues with being very "oozy" in surgery..more so than normal healthy kids. My son builds a clot but his body washes it away. another child has platelet morphology issues. the platelets leave the bone marrow small and mis-shaped. then they do not work right. He does DDAVP and amicar.

There has to be a connection between the two, just no one can explain why or how....but that's what keeps us searching...the unknown answers of mito.

Donna

mito mom of 5 mito kids....

Bleeding Disorders in Mito?

I received a call from Hematology on Friday. has been bleeding on and off for a few years, worse lately with drops in her red cell count. They finally identified the "cause" of the problem. Something they are calling a Plasminogen Activator Inhibitor defect. This defect means it is very difficult for her body to make and maintain a blood clot when necessary. They are tryign to determine how best to treat. Some of what they suggested, like birth control pills, was vetoed by our mito docs becuase of the clot/stsroke potential. They are considering another drug, Amicar, which as a similar safety profile. That makes me quite anxious as lies has had 5 strokes (assuming and lies have the same base mitochondrial defect). From some of the comments during the initial consultation appointment I feel strongly that the hematologist really doesn't know or understand mito.Have any of you ever heard of this defect before? If so, how was it treated? Was treatment successful? If we don't get a handle on this soon, transfusions will be necessary. Her RBC is dropping dramatically each month with no rebound between cycles.Please contact mito-owner with any problems or questions.

[Guest guest]

.. He does DDAVP and amicar.

>

Our children also have bleeding issues. Our son has low von Willebrand

factor. Our daughter has borderline levels. Our son uses DDAVP for bleeds.

Do you use the nasal DDAVP? We have mostly used the IV form but the other

day they used the nasal in the ER. I brought it home because they would have

thrown it away after we used it. Just wondering how well the nasal form does

with stopping bleeds. I would like to use the nasal form to stop bleeds earlier

if

possible. Our son is having GI bleeds and our daughter looks like she's

beaten regularly because of all the bruises. She's had swollen knees from

falls at school.

[Guest guest]

> we actually have pill form DDAVP, but at the hospital we get the IV form.

Pill form? Wow, I didn't know that there was a pill form. Just looked it up in

my

drug book and there is one listed. We were told by hematology that they only

use the nasal form of DDAVP for nose bleeds lasting more than 1/2. They

didn't mention the pill form at all. When we were in the ER they gave us the

nasal form of DDAVP but they only used one spray of the strenght meant for

enuresis--our hematologist said that that was worthless.

My drug book lists only the IV form as being appropriate for von Willebrand's

disease.

I often wonder if there is some other bleeding disorder going on with me and

my kids. My daughter (who has low side of normal factor 8 and von

Willebrand's factor) has had a joint bleed and my son keeps having GI bleeds

with unknown cause. Depending upon the pathologist, he may or may not

have eosinophilia. But, he keeps bleeding. He had a lower GI bleed over the

weekend and had tarry black stools yesterday, indicating a stomach bleed. I

called the pedi to get a CBC today.

My son has fluid retention issues even without the DDAVP so that concerns

me too.