PNH just dignosed
 

hi,

i posted a few months ago in AA, as i had AA 17-18 years ago and suddenly my blood counts were bad this year (along with many other aspects of my life). After being diagnosed first as having an hemolytic autoimmune anemia and treated with lots of cortison and Mabthera for 7 months, nothing was really working. They then said the next step was splenectomy, I went to see another doctor who diagnosed PNH. i still don't know if this is good or bad news...

i'm now on fraxiparin, he lowered the cortison, i had a bone marrow biopsy and aspiration. I don't know what's next... i'll get all the results next week. i only know for the moment that my kidney is not doing well. i have severe migraines.

For those with PNH, how are you coping? i need positive feedback today please :(

i live in Switzerland, alone with my 4 year old daughter. Not easy to be a super mum when weak and ill and today I'm feeeeed up!

thanks for reading...
anja

Anja

You are in my thoughts and prayers. Hopefully the Doctors will sort everything out soon and you can begin to heal again. I was diagnosed with AA in March and understand PNH and AA are closely related. I do not know much about PNH. Have you been to the AAMDS.org website?

Best wishes for you and your daughter.

Scott AA March 2012 levels 10/24/2012 Hgb 9.4, platelets 57, ANC 2000

Anja,

In addition to the PNH information at the AA&MDSIF website and on this website, you'll find PNH patients and information at the PNH Support Group website.

Hello Anja,

PNH can be a scary diagnosis since it is such a rare disease. I was first diagnosed with MDS in June 2010, and PNH in Feb 2011. These two diseases plus Aplastic Anemia can all be intertwined. In fact, my doctor now thinks that my AA and MDS symptoms are all related to the PNH. PNH diagnosis is confirmed by flow cytometry, which can be performed on blood cells and doesn't require a bone marrow biopsy, although I've had my share of them as well.

My treatment has been an infusion of Soliris (eculizumab) once every two weeks. It is an incredibly expensive drug, but it "cures" PNH. That's to say, if you take the infusions every two weeks for the rest of your life, the PNH won't attack your blood cells. Of course, then you are still left with the AA to deal with. Patients with only PNH report feeling better almost immediately.

I second Neil's recommendation of arming yourself with information about PNH. And find a hematologist/oncologist who is familiar with these bone marrow disorders. Read as much as you can and ask lots of questions.

I wish you well in your journey to feeling better and becoming informed. As I said, PNH is rare, so that means you are in rare company!

Sue

Swiss Educated PNH Specialist Contact Info
 

Hi Anja,

I'm sorry about your diagnosis and that you're not feeling so well. Soliris protects the kidneys and I know patients who's severe migraines went away once they began it. Most have far more energy on it. Best to consult with PNH specialist to decide if needed.It is most likely covered by national insurance in Switzerland.

My PNH specialist can help you locate the closest specialist to you if you'd like her suggestion. There aren't very many of them. The PNH support group neal mnetioned has much info and you can 'talk' to many other patients there.
Dr Bessler Contact/bio: http://www.research.chop.edu/researc....php?ID=941940

Her PNH presentation: http://www.rarediseases.org/docs/pnh...012PNHTalk.pdf

Suz

How well is the Soliris working for you?
 

Hi Sue-

My Dad is only 66 and a Cancer Survivor (4th stage non-hodgkins lymphoma). He was also diagnosed with PNH over 8 year ago. He has been suggested Soliris by all of his Doctors in the past 8 years, and is hospitalized most recently for thrombosis. This disease is such a horrifying thing to watch take over my Dad's body, and we all want nothing more than to see him get on Soliris, as it seems that at this point, it is his only hope for improved quality of life. Can you tell me the pros and cons of being on Soliris? I would love to hear from someone who's experienced it first-hand.

Thanks in advance for your input.
In the meantime I'm sending good vibes and well- wishes your way.

Desiree

Hello Desiree

My experience with Soliris and PNH is clouded by the fact that I have these other diseases as well. But from talking to a lady whose only diagnosis is PNH, she said Soliris was a miracle drug in terms of her quality of life. So that is your pro right there. It is the only treatment that will cure PNH so long as you continue to take the medication.

The cons are first, its expense. It may very well be the most expensive drug in the world. But there are so few PNH patients that we're all worth the cost, right? As it is admininstered by IV every two weeks, it made sense to me to have a port implanted. The surgery for this is not awful and it saves veins from frequent punctures. Another con is that it needs to be given every two weeks. But what the heck, for an hour every two weeks to get quality of life and relief from the symptoms, that is hardly an inconvenience.

I'm sorry that your dad is feeling worse as time goes by. That's what happens with PNH. But the Soliris will reverse that and you will all be amazed at how much better he can feel.

Soliris is manufactured by Alexion Pharmaceuticals. They have some very knowledgeable and helpful case managers at OneSource Treatment Support. I'm sure that if you contact them at 1-888-765-4747 you'll be put in touch with someone who can give you more information that I can.

Don't despair - there is help for your dad!