What are we waiting for? Totally Confused! Please help!

[Jennifer M]

Totally Confused!!! My Wonderful Husband has been receiving red blood trasfusions since July 5, 2011. (About every 4-6 weeks). He has had 4 red blood transfusions (2 units each time) to date. We went to NIH to see Dr. Young: Loved him! We were told he was Moderate AA & also has PNH. He sugessted to watch & wait. He also sugessted that he could be "On the cusp" of a SAA. He & his staff explained that @ this time they really do not suggest any treatments until you become severe. He was told to live a normal life as possible. Well: What is "Normal"?.....Because he is living far from "Normal". He is used to working out every day & was working full time with many goals he wanted to acheive. He is sooo Fatigued that he can do none of these! We are confused as to what we are watching & waiting for. My Husband has severe fatigue & sees spots & short is of breath & has heart flutterings even after this last transfusion.

Any advise would be appreciatied!

Health, Recovery & Peace to All!!!!

[Chirley]

Hi Jennifer, I don't know what level they are trying to keep your husbands Hb at but it is obviously too low for him to function properly. Different people function at different Hb levels. When I first became transfusion dependent I would function quite well until I dropped into the low 70s but as time passed I needed my Hb to be higher to feel okay.

Your husband needs to tell the doctor that he needs to be transfused at a higher Hb.

Good luck

Regards

[Jennifer M]

Thank you so much for your advice. We will definately ask the Dr. @ his next appt.

I just wonder why they won't go for the immunosuppressive therepy treatments. When/if will they?

Anyone with any thoughts about that subject?

Thanks & Love & Health to All!

[Ellen McDonough]

Hi Jennifer,

I have severe AA and not PNH but have done some reading about PNH.Red blood cell transfusions can cause iron overload and with PNH can overload the kidneys with iron.Maybe that is why the doctor was being judicious with transfusions of red blood cells. I think PNH affects the kidneys quite alot. If he has moderate AA he may want to hold off on transfusions for now.
As for Cyclosporin I don't know what the status is for or not using. It is rough on the kidneys as well.I am weaning off Cyclosporin[just started] after 2 years of Cyclosporin therapy.It has some nasty side effects.I have noted my kidney function labs are moderately affected and have paresthesias.It has caused high blood pressure and dizziness and when I was at a higher dose had some"foggy thinking" until the dose was lowered.I was diagnosed at age 49 years and always was very fit and athletic and "healthy". It is an adjustment to have limitations placed because of this illness. I still "workout-swim,hike, bike ,spinning- but at my own pace now.I am short of breath ,at times dizzy and have heart racing episodes with just climbing the stairs in my home but with a short rest I am ok.
I too have had the privilege of seeing Dr Young at the NIH.He is such an expert with this disease.
When I was being transfused I unfortunately built antigens to the platelets[which can happen with RBC transfusions also.I then had to have the blood bank find platelet donors who matched me to donate .There are risks involved with transfusions--iron overload ,antigen formation etc.
I wish you and your husband hope and health.He is lucky to have you as an advocate!
I would love to help if I can.
Take care ,Ellen McDonough

[Marlene]

Hi Jennifer,

Welcome to the world of "watch and wait". These diseases, even when treating, require us to watch and wait.

At what point does your husband get red cell transfusions? John threshold was a hgb of 8. Some need it sooner and you husband may need to set his higher, maybe at 9.

I would also look at his some of his nutritional status. B12 should be at least 500. I'm sure they checked it but, many doctors feel a low normal B12 is OK. But your husband, depending on how much the PNH is destroying, will have a higher demand for blood building nutrients. So make sure they checked his B12, folate, B6, copper, zinc and iron. And again, if they haven't already done so, get his testosterone checked. Finally, get copies of these results and all future results.

Since he's not at point needing treatment, this would be a good time to find out about all the options. I would suggest getting another opinion from Dr. Brodsky at Johns Hopkins.

[Lisa V]

Jennifer, have you had any follow-up conversations with Dr. Young? If it were me I'd contact him and ask him specifically what it is they are waiting to see and why he feels it is better to hold off treatment.

With a diagnosis of MAA they usually like to wait because treatment doesn't necessarily bring about a complete return to normal blood counts, it may just return someone with SAA to an MAA level. That would be fine if he wasn't requiring transfusions. In my mind if you are needing to be transfused it's time to treat, but there may be other factors at work here that we don't know about. What are his current counts? If it is just his Hgb that is low and not the other lines, that may help explain why he doesn't fit the criteria for SAA.

[Jennifer M]

Thanks to Everyone for your kind thoughts & much needed advise! We are seeing the Hemotologist this Friday & we will ask about some of the things you have all mentioned to me/us. Again: Thanks so much!

Peace, Health & Well Wishes To ALL!!!!