One year of MDS

[freedom99]

My wife has now been diagnosed with MDS for one year now. June 2010
It started with an emergency visit for swelling to her ankles and then the CBC results showed Hg at 90 and PLT at 40. The doctor did some more blood work and waited to see where the counts would go. After another emergency visit for major pain in the legs the blood counts went down more and after a week hospital stay was referred to oncologist in Windsor who after a bone marrow biopsy diagnosed this as MDS in a type 2 severe category with 2 cytogenic abnormalities. One of those abnormalities was 5q deletion syndrome which means that the long arm of number 5 chromosome is missing.
None of this is good news.
After two more biopsies and a round of Vidazza there is no treatment left except transfusions.
The PLT count now June 17 2011 is down to about 11 while getting two transfusions of PLTS per week to maintain a 20 level or more. The Hg count goes down sometimes to 75 and is maintained at 80 with RBC transfusions of one every two weeks.
There are very few symptoms at this point but still the oncologist gives my wife less than a year.
With MDS, reading on the net, it may be possible to maintain platelets at a count as low as 5 and still be in a maintenance mode
Lowering the iron was considered but not carried out because of the risk of exjade causing abdominal bleeding and with low plateletes this would not be good. Besides that treatment with exjade for high risk patients with a short life prognosis is usually not beneficial

[Greg H]

Hey Freedom!

I'm very sorry to hear about the severity of your wife's MDS. Given her 5q deletion, was there any thought of trying revlimid or thalidomide?

By "a round of Vidaza" do you mean she tried it for six months or so? I understand it can generally take that long to work.

I fortunately have not had very, very low platelet counts, but I gather from other folks on the list that there's a good deal of variation in how people respond to low platelets.

It's an odd disease with many variations; I'm always skeptical of predictions about life expectancy.

Take care!

Greg

[freedom99]

Revlimid wasn't going to touch this. Revlimid a Thalidomide type of treatment was ordered and ready to go but the oncologist changed her mind. Vidazza was tried for three months from December to March and then a bone marrow biopsy was done. This biopsy showed that the Vidazza was not making a difference. No other treatments available. A bone marrow transplant would involve a one month in hospital stay to destroy everything in the bone marrow before implanting the donor marrow. Because in MDS the bone marrow would still be dysfunctional it would return to it's old habits and fail. Also generally bone marrow transplants are not given to older people over 60 because it doesn't last very long. In Leukemia the bone marrow would be able to replenish the blood cells without this permanent dysfunctionality of MDS so a bone marrow transplant would work better in this case.
You are right in saying there are so many sub groups of MDS with so many variations.
Life expextancy prediction is definitely a stab in the dark. I told my wife to relax and live each day to the max.
I think the low platelets are the big concern because a small bleed anywhere in the body could become a major problem without really detecting it right away. The two transfusions of platelets per week are keeping her stable at an average of 11. As long as it stays that way we are still in the ball game. I think that platelet transfusions can go on for a long time. She also has a port which makes it so much easier and also the hospital is only 6 blocks up the road and they are just fantastic in accomodating us.
The following web site explains MDS in a very good way:
http://www.cancer.gov/cancertopics/p...lastic/Patient

[gipper]

Hi Freedom,

Sorry to learn of your wife's condition; however, I do agree with Gregg that Revlimid might be worth trying except that it will likely suppress her platelet count (it did mine when I took it). So she might not be able to go that route. Also note that while patients can receive hundreds of red blood cell transfusions, that is not necessarily the case with platelets. I became refractory to platelet transfusions after getting only 25 units. Some at the end were cross matched as well. About half of patients needing continued platelet transfusions will become refractory to them. See the following:

http://enotes.tripod.com/transfusion.htm

I trust you are aware that age is not a limiting factor in BMTs - rather it is comorbidities. Many of older folks have other conditions besides the MDS, like COPD, diabetes, and/or hypertension. A man aged 78 received a mini-transplant at the Fred Hutchinson Institute in Seattle. I will be turning 76 this month and my secondary hemo at the Emory University Hospital in Atlanta is considering such a procedure for me. It is however associated with a 30% mortality so I'm not sure if I'll go that route if offered.

Wishing your wife the best,

Gipper

[freedom99]

Hi Gipper;
Initially there was no treatment offered until the first BMB came back with the 5q del syndrome. Revlimid works very well with patients who have 5qdel. Revlimid was set up to be ordered and payment uptions were set up. I did some research on the Revlimid site and found that the standard was that Revlimid should be discontinued when PLTS drop below 50. HC's(my wife)PLTs were at 5 and it became a concern to me. The doctor decided to do another BMP and found that the blasts had increased and together with an additional genetic abnormality indicated that Revlimid wouldn't touch it and proposed Vidaza.
Yesterday I came across this alloimmunization with platelet transfusion. This would be the refractory condition you are talking about. It is a big concern since my wife (HC) is getting 5 units of platelets 2x a week for the last 5 months and 1x a week 5 months prior to that.
You are right about the age factor. HC does have diabetes, irritable bowl syndrome and trouble walking. The major medical problems have a direct impact upon the survival rate.
Thankyou so much for giving me some more information. Sometimes the information that's out there becomes just too much and I have to switch venues for a while. Overall though it helps a lot to be informed when I talk to the doctor and for making the decisions the doctor makes me make.
All the best in your decision for a min-transplant. It does look like you have some good doctors on your case and that does help a lot. Still in all of this we are directed to make the final decision. I'm thinking your family should be your major help since with family one is generally connected at the hip know each other in a special way.
Thankyou Again

[Birgitta-A]

Hi Gipper,
The doctor who takes care of your wife is a little defensive. As Greg wrote Vidaza should always be continued at least 6 months - a BMB after 3 months doesn't tell us anything about if the patient will respond.

Then there are clinical trials in Canada for example this one: http://clinicaltrials.gov/ct2/show/N...r=Open&rank=14.

You know Thalidomide doesn't decrease platelets as much as Revlimid - both should be taken with Prednisone or a similar drug the first 3 months.

Then 2 drugs for low platelets are approved for patients with the bleeding disease Immune Thrombocytopenic Purpura: Promacta and Nplate. Both are in trials for MDS. As you live in a country where the doctors decide the treatment like I do (I live in Sweden) your doctor could try Promacta or Nplate. There are members of this forum that are taking these drugs with positive effect.
Kind regards
Birgitta-A
72 yo, dx MDS Interm-1 2006. June 2010 I received txs every week and my dysfunctional platelets were 22. I started to take Thalidomide and Prednisone. After 4 weeks my platelets increased to 43 (now about 100) and I have not needed txs since Sept 2010. HGB slowly increasing - last count 13.3

[Birgitta-A]

Sorry, I wrote Gipper instead of Freedom.
Kind regards
Birgitta-A

[freedom99]

Hi Gipper;
What is your process for platelet transfusions?
I did come across something that dealt with filtered platelets.
Have your ever heard of that?

Thanks

Freedom99

[freedom99]

Hi Birgitta-A;
You picked up on the defensive doctor. yes, at the first visit we had our moments, especially when I asked for a 2nd opinion. It's also been interesting to learn from my pharmacist that when the patient asks too many questions and gets to close to the nasty side effects of certain treatments the doctor may back off because of liability issues.
Can this be true?
I do realize that 5-6 months or cycles for Vidaza is the norm for assesing the effect of this treatment. At the same time the BMB clearly showed no movement at all and neither did the blood cells. The next part of this equation is that the basic goal of these treatments is to become transfusion independant not to bring a cure to MDS. When asked if these treatments would prolong my wifes life, one doctor said no and the other said by one year from the prediction less than a year because without transfusions the patients is less at risk from the bad effects of transfusions.
Then comes the next quesion, if there is less than a year to live why not make my wife comfortable with transfusions and not subject her to treatments that do have nasty side effects and considering less than a year would have little benefit. There is no cure for MDS. If it was leukemia then there is a tested procedure to bring a bone marrow transplant that will work because the bone marrow is not chronically dysfunctional like in MDS.
30 years ago a child with leukemia had a 90% chance of dying, today the same child has a 90% chance of surviving. I find that so awesome and spurs us all onward to not be afraid or timid to demand more.
Thankyou so much for your reply and your input because it has prompted me to think more about this. Otherwise I may just give up and get my golf clubs and lose myself on the golf course.
My wife might live five years and that too I have to consider in all of this.
You do list some trials that may not be specifically for MDS in Ontario Canada but I will look them up. I need to be informed for my time with my doctor.

[Greg H]

Hey Freedom!

Birgitta mentioned Promacta and Nplate, both of which are used to increase platelet production. I don't know how the Canadian health care system feels about those drugs, but there's a clinical trial for Promacta in MDS at the US National Institutes of Health in Maryland. The travel might be a challenge, but the treatment involves no cost otherwise.

There's another drug being investigated in high risk MDS that has shown some promise called ON 01910.Na. It has a number instead of a name because it's so new. There are several clinical trials for it, including this one.

I imagine you can find other trials for both on clinicaltrials.gov -- or on the Canadian equivalent website.

Someone mentioned "mini-transplants," also known as "reduced-intensity transplants." More and more folks in there 60s are having these, though your wife's other health issues would have to be carefully weighed.

I certainly can empathize with your mutual decision not to undertake a lot of treatments that actually reduce quality of life. But it's good to know about all the options while making that decision. And believe me, the folks on marrowforums, collectively, have looked at a LOT of options!

Take care!

Greg

[Birgitta-A]

Hi Freedom,
You know I only accepted supportive care for example txs the first 4 your of my disease of the same reason as you mentioned - I felt fine and didn't want to get a drug that caused nausea, fatigue and perhaps other adverse effects.

When I needed txs every week and hardly dared to cough because I was afraid of stroke due low platelets I changed my mind and started with Thalidomide with good result and no adverse effects.

In Sweden we don't have a tradition of liability issues so our doctors can prescribe Promacta (called Revolade here) or Nplate if they think the drug will help the patients and I suppose it's the same in Canada.

Even if the BMB didn’t show any change I think your wife should have continued with Vidaza – all studies show the same: response can come late.
http://www.ncbi.nlm.nih.gov/pubmed/21225870
Kind regards
Birgitta-A

[tytd]

Hello freedom99 and your wife,
I certainly agree with all the advice given by Greg, Birgitta and Gipper. Of course your wife is the only one who can make the decision about how much treatment she wants to endure. She may also want to read about Kirby Stone's struggles with this disease on this website which are quite inspiring for us older folks. I have heard some of the MDS gurus online say that sometimes you have to "bite the bullet" with Vidaza treatment. "No pain, no gain" as they say. So I'm not sure if your wife's doctor gave up too soon or if your wife just did not want to sacrifice any more quality of life. It is a very difficult situation and often there is no clear cut answer. Does your wife have severe diabetes or just mild and was her quality of life good before this diagnosis? If her co-morbidities are not bad you may want to get a second opinion from an MDS expert.
Just out of curiosity are there any people out there reading this forum in the over 60 age group who have had a transplant for MDS and are doing OK?

[gipper]

Hi Freedom,

My platelet count was not as severe as what you noted regarding your wife. My counts went below 10 only following two different Dacogen cycles. Most of the time I was in the 20's. Having been previously diagnosed with ITP my hemo was able to put me on Nplate. As I understand it neither Nplate nor Promacta have been approved for MDS, although trials are underway. Nplate got my count up to about 40 (again I'm talking thousands here), but that was with a very cautious approach in dosing - never went above half the peak stepped dose level. Next my hemo put me on thalidomide, along with prednisone, since it had been shown it not only increased the platelet count in many on the drug, but also reduced the size of the spleen in some. OK that enabled my count to remain in the 40's, but did not shrink or even keep my spleen from enlarging. Therefore my spleen was removed last month and my count promptly went into the normal range - 245,000 as of 6/6/11. So that's where I'm at now. By the way, my hemo gave me a prescription that was only to be used if I encountered uncontrolled bleeding. While it was never required, it provided some comfort knowing if was on hand.

Exjade would be inappropriate to say the least for your wife since the manufacturer notes it is contraindicated for those having a platelet count of less than 50,000.

In spite of having read quite a bit about platelet transfusions, the complexity is still daunting. Nonetheless, I'll offer a few bits of info that I hope are on track. A person who gets a platelet transfusion gets a bag of either pooled or apheresis platelets. Pooled are the most common. This is where they remove the platelets from five, or so, different whole blood donor bags. An apheresis bag of platelets is from a single donor. So the chance of getting an infection from the donated bag is five times greater in the former rather than the latter - it's still a very small chance. Now to answer your question about filtered platelets - I believe all platelets collected are filtered for leukocytes (leukoreduction). The next step up, increasing the cost, is cross matched platelets. I think this is where a small sample of your blood is mixed with some platelets from a donor bag.
Now the next step gets much more costly and that's the use of HLA (human leukocyte antigen) platelets. I was scheduled for a splenectomy last October when my count was only 26,000 and my doctor had asked for HLA matched platelets to be on hand at the hospital. The hospital refused as it was too costly and they were afraid of being stuck with the HLA costs in case of a cancellation. In fact my hemo did cancel the surgery which I subsequently had last month when my count was 46,000.

Here are a couple of associated references:

http://en.wikipedia.org/wiki/Plateletpheresis

http://pathcuric1.swmed.edu/Resident...FSCOMHLAP.HTML

Bill F

[LynnI]

Dear Freedom,

I find it alarming that your doctor has given a time line, no doctor including the specialists in these diseases knows what is going to happen with any one patient. Too many variables. There are too many people on these forums that have and continue to beat the odds everyday. I am one of them, I have responded to a drug with brillant results. I am in a drug induced remission for 3 YEARS after recieveing regular blood transfusions. What should that tell us?? It means that there can be a cure in the future, that the body can respond, that there is HOPE. That you have to try and find the treatment that just may have the same fortunate results that I have. That no doctor can predict who will respond and who wont to any given drug/treatment. And you don't throw in the towel based on the opinion of one doctor or the failure to respond to one treatment. You keep trying, there is hope because if it can happen to one it can happen for others.

I would strongly suggest that you ask for a referral to one of Canada's Centres of Excellence for MDS.

Canada

Princess Margaret Hospital
Toronto, Ontario, Canada
Karen Yee, MD

Toronto Sunnybrook Regional Cancer Centre
Toronto, Ontario, Canada
Richard A. Wells, MD

University of Toronto
Hospital for Sick Children
Toronto, Ontario, Canada
Yigal Dror, MD


It has been my experience that these specialists don't give up so easily (I go to Sunnybrook) and they have a far more optimistic approach to treatment. I personally wouldn't even consider seeing a local oncologist, knowing that they don't have a tenth of the knowledge or the experience of dealing with many MDS patiences everyday.

Cheers,
Lynn

[Greg H]

Hey Freedom!

I have to say, LynnI makes some very good points. I don't know that we've heard much about your hematologist, so he/she may be an MDS specialist, but, if not, I agree that it's critical to go and find one.

I have three docs currently treating my MDS: my local hematologist, who has to keep up not only with MDS but all the other bone marrow diseases; my transplant doc, who's part of a university-based team with lots and lots of experience in both drug and transplant treatment for MDS; and my doc at the National Institutes of Health, a specialist in MDS and immunology, who's running the clinical trial I'm in.

They all have their strengths and weaknesses, but I wouldn't want to rely on my local doc alone, as good as he is, and he's very good. In such a rare disease, a specialist is essential. If you've got one, great. If not, I'd go find one.

I participated in a conference call with Dr. Yee from Princess Margaret. She seemed very knowledgeable and patient-focused.

Take care!

Greg

[Cheryl C]

I'm also wanting to know if there's anyone out there in their early 60's who has had a successful BMT as I'm currently waiting to see if my older brother is a match (my younger brother isn't).

I can't believe how many different variations on the theme of MDS there are! I thought there were only 5 but I can see that the possibilities are almost endless.