Re: Bone Marrow Suppresion Question

[Guest guest]

,

Thanks for emailing me this off group. I did get to read it and

can relate. does have bonemarrow failure of unknown origin.

She is transfused approx. every three to four weeks. since birth.

When she gets a virus or sepsis her counts will quickly decline. At

one point she was so sick that she recieved two transfusions in

week. It affect her hemoglobin and platelets as well. Some viruses

just do this. She had a staph infection and then came home and had a

virus. Hr hgb dropped and her plateletes dropped about 400,000 in

just a couple of days. Within a few days though they went back up

(not the hbg. becuase she does not produce any red cells at all).

They have been unable after 4 yrs. to find the cause of the bone

marrow issues. We also see Cohen and he is at a loss. Have they

decided what the cut off number is for her to have to be transfused?

They will tell you that some children are fine around six and not to

worry. These are also children without mito issues as well. Mito

affects your energy level. So this is like a double whammy. I would

tell them that you need to transfuse her based on symptoms not

numbers. Rapid heart rate, exhaustion, bruising, increased

resperations, decreased appetitie and fluid intake. I have battled

this with our docs for a long time. there are some months that

is fine with a hgb of 7.5 and other months when she is

sympotmatic at 8.5 especially if there is an infection. I hope this

helps a little and I hope Miranda feels better soon. More than

anything I hope you can find some answers.

Dawn

[Guest guest]

,

We have these issues to and they were more exacerbated when was on TPN. His bone marrow biopsy was normal, however, they found that he had an underlying immunodeficiency. He then got septic and when they removed the port, they found underlying necrotic tissue. They think that because of his immunodeficiency his body was rejecting the port. He no longer has a central line, however, if the need arises in the future, it will have to be a broviac instead of a port. This way they would have better visualization of his bodies reaction to the line.

Right now he has been pretty stable with his blood counts. He is always slightly anemic or in the borderline normal range. His red cells are very big and he sometimes has oddly shaped red cells. His white count usually ranges between 2 - 3 and his platelets have been running just below normal. He also has a platelet aggregation defect and Von Willebrands Type 1 so his platelets don't clot properly.

son has Type 1 as his primary diagnosis with Pyruvate Dehydragenase as a secondary.

Good luck with this. I know how frustrating this can be.

Laurel