First post

[Jools]

Hi, I am the wife of a recently diagnosed MDS RAEB2 sufferer.
We have entered a world where 'normal' has a whole new meaning.
We are in watch and wait mode - my husband is reasonably well in himself, only red cells bombing all the time and he receives 2 units of blood approx fortnightly now.
The last 4 months have been hugely stressful,bewildering, anxious and many more emotions. Each visit to the consultant has him saying "when you need treatment it may kill you,so go and enjoy yourselves". Any information we have learned apart from a very basic pamphlet given to us at diagnosis has been found on the internet. Obviously a lot we read frightens us to death!
We are trying to stay upbeat and positive that the disease will not progress to AML anytime soon, but some days are harder than others.

[Debbie W]

The UK hospital I have seen mentioned on this site is Kings, you can read the messages on the transplant thread here...

http://forums.marrowforums.org/showthread.php?t=1032

Maybe either Vidaza or Dacogen will work for him???

Best regards,

Debbie

[Birgitta-A]

Hi Jools,
You know in the UK the authorities think Vidaza is too expensive and the MDS patients in your country are not allowed to get Vidaza though the drug is approved in the EU. The correct treatment of RAEB2 is Vidaza or stem cell transplantation if the patient is in good condition and not too old.

Here is a link to clinical trials for MDS patients in the UK:
http://clinicaltrials.gov/ct2/results?term=mds+and+uk

As you can see there is one study (number 7) with Azacytidine (Vidaza) for the treatment of MDS/AML with high risk - chromosome 7 and or complex cytogenetic abnormalities. That means that if your husband has a chromosome 7 aberration or 3 or more chromosome aberrations he can participate in that study. Have you asked if your husband has any chromosome aberrations and if he has aberrations which aberrations he has?

Then patients with RAEB2 can participate in a study where they get a stem cell transplantation (number 5). They don't write anything about age in the exclusion criteria but he has to have a HLA compatible sibling donor - that is a close relative that is a good match with similar stem cells.

Both these studies are carried out in King's College Hospital, London, the hospital Debbie told you about. You could contact the hospital and ask for treatment options for your husband - perhaps they could give him Vidaza off record.

Don't give up!
Kind regards
Birgitta-A

[rose mcmillin]

Hi Jules, Just letting you know that my husband was diagnosed two years ago with raeb-2. He has been on Vidaza since then. I like you was in panic mode but a lot of the info on line is old and out of date. They let me and my husband think that he had just a few months. Also, you might want to to check out Kirby Stones entries. He inspired me to have a whole new attitude.
This disease is scarry business and sometimes it takes being demanding to get what you need. I realize that its hard to get vidaza in the UK but perhaps you can get into some clinical trials. Stay positive and strong. If you need any more info please feel free to ask. I will answer any questions that you have regardless of what they are. Sincerely, Rose

[Jools]

Thanks for your replies.
My husband is a fit active 64 next month - no siblings or other possible donors.
We have asked about Vidaza,but been told that as he has "no particular chromosome abnormality",watch and wait is the way to go whilst he is still reasonably well.
Our consultant at Blackpool is excellent and appears to be aware of and involved with trials etc, I guess my problem is coming to terms with everything this illness has the power to do.
He has taken the diagnosis amazingly well, says its no use worrying about it,accepts it and wants to carry on as before as far as possible,obviously with a few adjustments to our lifestyle. Very positive about his future.
I am heartened to see a RAEB-2 patient still here 2 yr after diagnosis - it gives me hope that we may also have longer than consultations seem to imply.

[Birgitta-A]

Hi Jools,
Good that your doctor in Blackpool is exellent. Very positive that your husband hasn't got any chromosome aberrations! Still a patient with RAEB-2 should get Vidaza or Dacogen in most Western countries except the UK.

If you read this article you will see that the author thinks that: "All patients with MDS should be considered for a clinical trial with the possible exception of those with 5q–. However, because of the recently demonstrated efficacy of the DNA-hypomethylating agents, some think it is unethical to withhold such a therapy from higher-risk patients. At the very least, one should consider a clinical trial in which a DNA-hypomethylating agent is used as part of a novel combination regimen."
http://bloodjournal.hematologylibrar...ll/113/25/6296

Watch and wait is not for patients with high risk disease like RAEB-2 - instead you should try to get treatment while your husband is well enough to manage with the adverse effects of treatment.

Ask your doctor if he is engaged in any clinical trials for MDS. If not ask about stem cell transplantation - many patients without siblings get stem cell transplantations with stem cells from special centers or cord blood.
Kind regards
Birgitta-A
71 yo, dx MDS Interm-1 May 2006, transfusion dependent, Desferal and Exjade for iron overload, Neupogen for low WBCs, Thalidomide and Prednisone for bone marrow fibrosis

[squirrellypoo]

Hi Jools.

I had my BMT from an unrelated donor at Kings last summer and I cannot recommend Kings or Professor Mufti (the MDS specialist there) highly enough. You should check with your consultant to make sure that he is referring with Prof Mufti on your case and sending your BMB results down for a second opinion.

As Birgitta has mentioned, Vidaza is a hot topic right now, with NICE initially rejecting it, and now it's being reconsidered thanks to the fight of a bunch of cancer groups and the UK MDS Patients Support Forum.

There was a BBC News article about the fight to overturn the ruling back in March.

cheers, melissa

[Carole in Sudbury]

Hello,
I was affected with a low red cell only also. Could you give me more detail? The first time I went to hospital, my Hmg was 61. I received 4 pints of red cell only. There doesn't seem to be very many people with red cell only. I'm wondering if this is a different disease. Confused

[evansmom]

Hi Jools,

I'm from the aplastic anemia family so I can't offer you much insight, however your husband's positive attitude will serve him very well - good for him. You've found a great resource in this forum. Many folks here will help carry you through this - trust me!

Carole-

You might want to read up on "pure red cell aplasia". Good luck.

[Zoe's Life]

Carol,

Actually, it is very common for only the red cells to be affected with MDS. All my other counts are fine. Only my reds are impacted right now.

zoe

[Carole in Sudbury]

Hi Zoe,
Thanks for responding. Were you told that you had MDS? Did they find this out through a BM extraction.

So far I've had every test done and nothing is showing as abnormal, but how can your bone marrow just stop functioning out of the blue. It took 2 transfusions and very high dosage of prednisone to start it up again. I have been very lucky. The Dr just tell me now to wait and see. I go for blod every 3 months.

Can you tell me more about what's going on with you?

Carole

[Carole in Sudbury]

Zoe,

When you say only the red red is affected right now, do you mean that others were affected before?

carole

[starz]

I second Squirellypoo. My husband requested a second opinion at Kings with Prof Mufti. He agreed to fund Vidaza (after his PCT refused to pay) to reduce the blast count, prior to the stem cell transplant on 30/6/10. They are so great there, and fill you with confidence.

[Zoe's Life]

Carole,

Sorry I was confusing. My reds are the only ones that have ever been low. I guess I think someday my whites may go whaky too. They have trended down over the last several months, but they are still normal, they just tend to stay lower than before. With my 5q- whites tend to stay normal to slightly low. Platelets normal to slightly high. My platelets are slightly high most of the time. From what I understand, the whites and platelets in me look funny but work well. I get sick less than the rest of my family. I am just more tired than they are.

[Jools]

Watch and wait is now over. My husband is now transfusion dependant (fortnightly),neutropenic and platelets dropping fast. Another bone marrow being arranged to see if blasts over 20% yet. If they are not, the "good news" is that in the last 2 weeks a local charity has agreed to pay for him to have Vidaza - it is not available on NHS in UK - as he is now High Risk MDS, IPSS 2.5 and therefore eligible for the funding.
I cannot put into words the gratitude we feel that this is now a real possibility.
We continue to try and remain optimistic and upbeat for his future, alongside the underlying fear,dread and anxiety I know you will all recognise as being part of living with this horrible disease.

[Birgitta-A]

Hi Jools,
Wonderful that your husband can get Vidaza through a local charity ! Too bad that you have to have high risk MDS to be eligible for the funding.

As you know the adverse effects of Vidaza often come first and the positive effects after several cycles. Be careful and try to avoid infections if his WBCs are low http://www.neutropenia.ca/about/living.html

Avoid everything that can decrease platelets. Especially many drugs like pain killers can decrease platelets. http://www.pdsa.org/about-itp/warnings.html
Kind regards
Birgitta-A
71 yo, dx MDS Interm-1 May 2006, transfusion dependent, Desferal and Exjade for iron overload, Neupogen for low WBCs, Thalidomide and Prednisone for bone marrow fibrosis

[Neil Cuadra]

Jules,

Having "no particular chromosome abnormality" no longer means watch and wait for your husband. Let's hope for a low blast count.

If I may ask, what type of local group made such a wonderful act of charity in offering to pay for his Vidaza?

[Jools]

We understand it is a local cancer charity who are funding isolated cases. Aswe have not had the next bone marrow yet we're still not sure if he will receive Vidaza or high induction chemo.

Birgitta - thank you so much for the links.

[Jools]

Our situation has rapidly moved on to the point where my husband is now scheduled in for a Bone Marrow transplant end Feb/early March.
We went to see Prof. Yin at Manchester on Monday of this week and he has decided John is a suitable candidate for BMT. Amazingly, the register has also shown 3 potential matches in the UK.
He has to have one course of High Induction Chemo in January (may be next week depending on latest marrow findings) then all systems go.
It has taken until today to get a grip on the huge range of emotions this development has caused.
I will keep you all posted!

[Birgitta-A]

Hi Jools,
Hope everything will function well with the conditioning and SCT!
Kind regards
Birgitta-A

[Jools]

Update.

Having managed to remain infection free up to now, John is due to go into Blackpool for a course of chemo tomorrow, prior to going to Manchester for conditioning and transplant in March - and on Dec 26th he has started on a course of antibiotics for a chest infection. Totally gutted that we have got so near to treatment starting and this has happened. Know it is hopefully only a slight set back and sure there will be lots more to come as we begin our transplant journey. Will be telephoning hospital in the morning to see if he should still go in to have his lines fitted tomorrow.

May I take this opportunity to wish everyone a Happy New Year, and hopefully a healthier one too!