MDS Challenges ahead

[MDSPerth]

Hi, my name is Sandi. The 17 July 2012 - a day we will never forget - my partner (of 25 years) Paul, who by all accounts appears to be in good health, was diagnosed with MDS. Paul went for what he thought were routine blood tests on Saturday 14 July. On Sunday 15 July he received a 9.00am phone call from his doctor to advise that he had anomolies in his blood counts and that he would like him to go see a haemotologist. Paul very nicely advised that sorry 'no can do for a couple of weeks, we are off on holidays to Bali'. The doctor very promptly advised 'oh no your not I will be getting you into a specialist as soon as possible'. So on the 17 July, at 9.00am Paul found himself in the office of a specialist being told that he had a bone marrow biopsy scheduled at 10.00am as he suspected from his readings that he had AA or MDS oh, and by the way go home and cancel your trip to Bali as your Neutophil count is at 0.35 and there is no way you would be able to resist any infections. To say we were in state of shock is an understatement and we continue to be in constant disbelief.

Life since then has been a series of blood tests and visits to the haemotologist. To put it shortly we have been advised that because of Pauls -7 and +8 chromosome abnormality and because he has the 3 cytopenia (in his favour, he has low blast cell count) he is in the lower side of high risk category. Because of his low blast cell count he is not a candidate for hypomethylating drugs such as Vidaza etc. Currently he is using EPO and G-CSF growth hormones and several anti-fungal/anti-viral tablets. His white cell count and neutrophils (risen from 0.35 to 6.9) have responded fantastically, however sadly his haemoglobin (94) and platelet count (24) continue to drop.

Paul has been advised that he is to proceed to a BMT sooner rather than later if a match can be found. He has a brother and sister living in England and we currently await the results of their testing results. Unfortunately for us, the Australian government says that until your siblings are outruled, the BM register cannot commence the search on the international register. So, we are keeping our fingers crossed for a donor match somewhere.

I have read the Marrowforums and found it very interesting to read other patient/carers views as, it is very rare to find anyone that has even heard of MDS let alone have the disease. It is so confusing also in that there are so many variations in the disease. I read about RA, RARS etc but the haemotologist that Paul is under advised that he did not fit neatly into any one of these categories?

We would welcome comments, chats, feedback from anyone out there that can spare the time and maybe help dispell some of the confusion that surrounds us.

[mausmish]

Hi Sandi,

I'm sorry for your reason for being here, an all too familiar story, but you've come to the right place for loads of valuable information and invaluable support. I don't know how I'd have managed without the people in these forums when I had my own surprise MDS diagnosis in January 2010.

Did you see the list of links on this site:
http://www.marrowforums.org/resources.html

I've written about my journey in my blog, linked below.

Best of luck to you and Paul.

Karen

[Neil Cuadra]

Sandi (and Paul),

The shock you experienced on July 17 is indeed awful. For my family it was October 23. Like you, we got a "stop what you're doing" phone call from the doctor after a routine blood test. Many of us here in the forums had the same experience and have had it cemented in our memories ever since. That's my way of saying that you are not alone. MDS is rare so you won't find many MDS patients or MDS experts around you, but if you seek them out you'll find them. We have forum members with a variety of backgrounds, ages, and disease conditions, and you'll find other Aussies among us too.

Give both Paul's doctor and his haemotologist credit for knowing what they were dealing with and recognizing his bone marrow condition so quickly. We too were rushed into an immediate bone marrow biopsy and in hindsight we understand exactly why. They may have saved Paul's life with their fast work.

In most cases we don't know what causes MDS, just that it's present. Initially, the key is fast action to stabilize blood counts and get them up from the most dangerously low levels. What follows is even tougher and takes longer: finding a long-term treatment strategy, to work toward improvement, livable counts, and the ability to go back to a normal life. A bone marrow or stem cell transplant, when available, offers a chance for a real cure. Other treatments, while not cures, can still give you your life back.

There is no risk-free treatment approach and that's a hard thing to accept. Your lives have been changed and this probably won't be over soon. The key for many of us is educating ourselves: learning about this disease, learning what blood counts mean, learning the medical words you keep hearing, asking questions until we understand what the doctor recommends and why, and becoming partners in our care. That's what gives us strength and the ability to help ourselves. From reading your post I think that you've already learned a lot.

Has Paul gotten an IPSS score? From your description it sounds like his score is about 1.5 (low end of the Intermediate-2 risk category) but only the doctors can tell you for certain. I assume that his DNA findings are what led the doctor to recommend a transplant.

If you aren't clear why Paul doesn't fit any of the WHO categories, ask the doctor to explain. For example, why doesn't he fit the RCMD (Refractory Cytopenia with Multilineage Dysplasia) category? Keep in mind, however, that classifications are used for statistical purposes, e.g., to match patients to the treatments that have performed well for most patients like them, but cases of MDS can be as unique as the people involved. What's important is what's best for one person: Paul.

Paul's low blast count is a good sign. So was his good white count response. I hope you get good news back from English soon. There's a 43.75% chance that one or both of his siblings will match. If they don't, we'll cross our fingers for you too, as you look for a match in the international registry.

Two bits of personal advice:

1. Your family and friends probably share the shock and disbelief you feel. Let them know what's happening to you and Paul and let them support you and share this burden.

2. Keep Bali on your list for a future vacation. You're going to get there, just not as soon as you expected.

[MDSPerth]

Originally Posted by mausmish

Hi Sandi,

I'm sorry for your reason for being here, an all too familiar story, but you've come to the right place for loads of valuable information and invaluable support. I don't know how I'd have managed without the people in these forums when I had my own surprise MDS diagnosis in January 2010.

Did you see the list of links on this site:
http://www.marrowforums.org/resources.html

I've written about my journey in my blog, linked below.

Best of luck to you and Paul.

Karen

Karen, thanks for your response. From your profile it appears that you had a BMT within a relatively short time of diagnosis. Did it take long to find a donor, and how did you travel after the transplant?

[MDSPerth]

Originally Posted by Neil Cuadra

Sandi (and Paul),

The shock you experienced on July 17 is indeed awful. For my family it was October 23. Like you, we got a "stop what you're doing" phone call from the doctor after a routine blood test. Many of us here in the forums had the same experience and have had it cemented in our memories ever since. That's my way of saying that you are not alone. MDS is rare so you won't find many MDS patients or MDS experts around you, but if you seek them out you'll find them. We have forum members with a variety of backgrounds, ages, and disease conditions, and you'll find other Aussies among us too.

Give both Paul's doctor and his haemotologist credit for knowing what they were dealing with and recognizing his bone marrow condition so quickly. We too were rushed into an immediate bone marrow biopsy and in hindsight we understand exactly why. They may have saved Paul's life with their fast work.

In most cases we don't know what causes MDS, just that it's present. Initially, the key is fast action to stabilize blood counts and get them up from the most dangerously low levels. What follows is even tougher and takes longer: finding a long-term treatment strategy, to work toward improvement, livable counts, and the ability to go back to a normal life. A bone marrow or stem cell transplant, when available, offers a chance for a real cure. Other treatments, while not cures, can still give you your life back.

There is no risk-free treatment approach and that's a hard thing to accept. Your lives have been changed and this probably won't be over soon. The key for many of us is educating ourselves: learning about this disease, learning what blood counts mean, learning the medical words you keep hearing, asking questions until we understand what the doctor recommends and why, and becoming partners in our care. That's what gives us strength and the ability to help ourselves. From reading your post I think that you've already learned a lot.

Has Paul gotten an IPSS score? From your description it sounds like his score is about 1.5 (low end of the Intermediate-2 risk category) but only the doctors can tell you for certain. I assume that his DNA findings are what led the doctor to recommend a transplant.

If you aren't clear why Paul doesn't fit any of the WHO categories, ask the doctor to explain. For example, why doesn't he fit the RCMD (Refractory Cytopenia with Multilineage Dysplasia) category? Keep in mind, however, that classifications are used for statistical purposes, e.g., to match patients to the treatments that have performed well for most patients like them, but cases of MDS can be as unique as the people involved. What's important is what's best for one person: Paul.

Paul's low blast count is a good sign. So was his good white count response. I hope you get good news back from English soon. There's a 43.75% chance that one or both of his siblings will match. If they don't, we'll cross our fingers for you too, as you look for a match in the international registry.

Two bits of personal advice:

1. Your family and friends probably share the shock and disbelief you feel. Let them know what's happening to you and Paul and let them support you and share this burden.

2. Keep Bali on your list for a future vacation. You're going to get there, just not as soon as you expected.

Neil, thank you so much for your supportive and informative quick response. It is indeed enlightening to at last be talking with others who are in the same space. By all accounts, and perhaps we are lucky, life on the surface continues to go on as normal and we both continue to work and Pauls health appears to be good. However there is one big exception. We can no longer make plans beyond very short term - eg we will be on holidays in February 2013 etc. To some people this may seem a big whinge and for that I will apologise. With the initial diagnosis and subsequent 'honeymoon' period there is a certain amount of grieving for what life was expected to bring in retirement - however one simple phone call changes that all as you well know.

Paul's haemotologist advised that he sat in the lower side of high risk factor and that he had a higher than average chance of progression to AML and yes, this is based on the DNA findings. My understanding is that the deletion of chromosome 7 is an indication that progression will be at a reasonable rate. It seems that the only treatment options open for Paul is the growth hormones, transfusions if and when required or keeping our fingers crossed a BMT.

It is great that his neutrophil count has responded to the growth hormones taking it well into the middle of the normal range, because that improves the quality of life immensely.

And yes, I have been reading a lot, most of it giving me head spin. This in itself has caused me a lot of frustration as I have this Mrs Fixit streak in me and I am finding that this insidious disease is totally out of my hands and there is little I can do with the exception of being there and supporting Paul.

How often have you had bone marrow biopsies? We have been told that its not likely there will be another scheduled unless there is a match found and a BMT is to occur. Is this normal?

Thanks for your words of advice. Number one, we do try to keep family and friends in the loop and most of them, like us seem to be waiting for any information between the fortnightly visits. Number two, we are happy to cross Bali off the bucket list. We have been there a number of times as from Perth it is a cheap vacation to a tropical destination. On this occasion however it was a great disappointment because we were going there for a wedding where our daughter was bridesmaid - but, that was nothing over Pauls health.

Neil, thank you for your kind words. At this point it has been all about me and Paul. You must fill me in about yourself.

Kind regards
Sandi

[Chirley]

Hi Sandi, may I suggest you join the Leukaemia Foundations "Talk blood cancer" site.

We have a monthly telephone forum for people throughout Oz for people with MDS and / or their carers. Or are you already a member? I think I remember someone from WA talking on the forum.

Regards

Chirley

[MDSPerth]

Originally Posted by cme01

Hi Sandi, may I suggest you join the Leukaemia Foundations "Talk blood cancer" site.

We have a monthly telephone forum for people throughout Oz for people with MDS and / or their carers. Or are you already a member? I think I remember someone from WA talking on the forum.

Regards

Chirley

Hi Chirley, yes we did join in the last monthly telephone forum with the Leukaemia Foundation which was very good. And, I have also viewed the Talk Blood Cancer site, which I will continue to do, but it is not as active as the marrowforum site. I guess thats understandable, given the population of Australia compared to that of America and the ratio of MDS sufferers. I have seen your name around on the forums. Thanks

[Cheryl C]

Hi Sandi

I'm an Aussie too. We're currently travelling around Australia and are in Albany, WA. I was in Sydney when first diagnosed with MDS RAEB2 twelve months ago at 63. I completely understand your initial shock and bewilderment at Paul's diagnosis.

It's hard to know where to start at first, but there is some information out there in cyberspace which can help. The following link has presentations from experts in the field. In particular there's a presentation on bone marrow transplants which might be helpful.

https://live.blueskybroadcast.com/bs...27&MA_ID=13769

It's a good idea to make a list of questions before you visit your specialist each time and tick them off as they get answered!

Like Neil and others have noted, every case of MDS seems to be unique so what helps one may not help another, but it's wonderful to be able to ask questions on this forum and to have people who have been longer on the MDS road come up with suggestions and possible solutions or pathways.

Keep your courage up! Knowledge is power.

Cheryl

[Neil Cuadra]

Sandi,

Thanks for asking about my own situation. I'm the luckiest forum member here because my wife (the patient) beat this disease. She was originally diagnosed with aplastic anemia, underwent treatment, was re-diagnosed with MDS, and had a bone marrow transplant from an unrelated donor who was found in the U.S. registry. The transplant cured her MDS. Those years in the trenches were rough but they made us appreciate our lives in a whole new way, and led us to stay involved in the AA/MDS/PNH community, hoping to share what we had learned. My wife's story is here.

Your comment about "life on the surface" rings true. MDS doesn't make you look sick and some patients don't choose to talk to their coworkers or neighbors about it. Sometimes it helps you feel normal that way. It's like you are leading a double life - the medical one and the "real one".

We learned not to make definite long-term plans, and for a while we had to live one day at a time, but we thought of it as postponing plans, not canceling them. Sure enough, we eventually got to take an international trip that we had been talking about for many years.

You asked about the frequency of bone marrow biopsies. They should be done only when needed to make well-informed treatment decisions. My wife had a couple right at the start, then a few more on an irregular schedule, to track the progression of the disease and changes in her chromosomes. After her transplant it was once a year to watch for any backsliding.

If you already know that a transplant is warranted, and that Paul has a couple of bad-indicator chromosomes, then it makes sense to me that he wouldn't need further biopsies for now (unless for some reason his bone marrow blast count needed to be assessed). The question patients should always ask before an invasive procedure, like a bone marrow biopsy, is what information it will provide and whether it will affect subsequent treatment. In your case it doesn't seem to be needed. After a transplant you'd want to know if "the cell factory" is starting up, and that's the purpose of a post-BMT bone marrow biopsy.

I can only laugh at my own assumption that a trip to Bali is a big trip, because it would be for us. You probably just hop in a canoe and paddle a few miles north! But I'm sorry you had to miss a planned trip and especially a joyous occasion like a wedding.

[SLB]

Hi Sandi, I remember speaking to you on the MDS forum. Yes it is amazing how one phone call can change everything! I went to the doctor on Wednesday morning, had a blood test at 10:00, got call from doctors at 3:30 pm, saw a hematologist on Thursday was admitted to hospital Thursday, and had bmb on Friday morning!! Still seems crazy months later!I am having my 3rd bmb on Monday of which the main purpose is to find out if vidaza is working. I also found the talk blood cancer forum wasn't very active compared to here. However marrowforums helped me alleviate some worrying and stress, not only find others with so much wisdom but also all of a sudden a platelet count of 30 to 40 was good compared to others! It was comforting to know people could live with platelets way lower. anyway enough from me.. welcome to the forums!! I am glad Paul's neutrophils have responded so well to treatment. Good luck and here's hoping you hear good news of a sibling match soon. Sharnie.

[MDSPerth]

Originally Posted by SLB

Hi Sandi, I remember speaking to you on the MDS forum. Yes it is amazing how one phone call can change everything! I went to the doctor on Wednesday morning, had a blood test at 10:00, got call from doctors at 3:30 pm, saw a hematologist on Thursday was admitted to hospital Thursday, and had bmb on Friday morning!! Still seems crazy months later!I am having my 3rd bmb on Monday of which the main purpose is to find out if vidaza is working. I also found the talk blood cancer forum wasn't very active compared to here. However marrowforums helped me alleviate some worrying and stress, not only find others with so much wisdom but also all of a sudden a platelet count of 30 to 40 was good compared to others! It was comforting to know people could live with platelets way lower. anyway enough from me.. welcome to the forums!! I am glad Paul's neutrophils have responded so well to treatment. Good luck and here's hoping you hear good news of a sibling match soon. Sharnie.

Sharnie, thanks so much for responding to my posts. Yes, I do remember speaking with you on the forum. Funny that three of my return posts have been from fellow Aussies and we are using an American site to network. My heart goes out to you, being only 36 and with a young family. A shock like this is quite confronting for a 62 year old male, but at least he ticks more of the boxes - ie over 60 and a male. I have found reading this site quite reassuring in that there are long term survivors out there and that sometimes your own readings pale into insignificance compared to others, but at the same time we must remain mindful that every case is different. Are you being treated as a public or private patient. Paul has private health insurance, but both his own GP and the haematologist (who also has a private practice and services a private hospital) advised that he was much better staying in the public system as his treatment would be no different, and the costs going private would be prohibitive. It would be great to hear your comments. To date, we cannot complain about his treatment

[Chirley]

Hi Sandi,

I have private insurance and go through the private system. I have no out of pocket expenses for my cancer treatment centre or for my hospital admissions. I used to have to pay a gap for xrays but now that I'm on a DSP, I no longer have a gap. I do have occasional out of pockets, for instance, when I had my recent liver biopsy everything was covered by a combination of Medicare refunds and health insurance except for the cost of the needle which was $104. Medicare just won't refund on some things and the health fund won't always cover the deficit.

When I had Vidaza it wasn't on the PBS (it was on the waiting list to be approved for 2 years and some people don't have the luxury of waiting that long). I had to pay for the Vidaza myself and it cost $42,600 . On the other hand if I'd been in a public hospital it wouldnt have even been mentioned to me as an option because they weren't supplying it unless you were considered to be terminal within a couple of months. This way they wouldn't have to pay for it for years. (at least this is what my doctor, who works in a public hospital too, told me). On the plus side Vidaza is now approved and is supplied by the private system with no cost to the patient.

Sharnie told me that she goes to Greenslopes Private hospital and I have to say I'm a little jealous. My Mum is there now and they have been excellent. I asked my doctor to consider starting a practice at Greenslopes but he is already stretched a bit thin. He works at three cancer centers, two private hospitals and two public hospitals.

Maybe I can talk to you on the next phone forum. I normally join but couldn't last time because Mum has been so sick. I sent my apologies to Rebecca.

All the s sounds....Sandi, Sharnie, Chirley (pronounced Shirley)

The builders are due to arrive in 25 minutes so I'd better get my dog inside.

Bye

Chirley

[triumphe64]

Here are some presentations on MDS. It will give a lot of answers to your questions.

https://live.blueskybroadcast.com/bs...AT=944&CAT=948

[mausmish]

Hi Sandi,

I think it took about six months to find a matched, unrelated donor for me. The time may have been shorter but I think the active search was put on hold when I switched to a different transplant center. I had the transplant 11 months after my initial diagnosis at Johns Hopkins which is just about an hour from my home. When I mentioned my "journey" I meant it in a philosophical way, not literally.

Karen

[MDSPerth]

Karen, thanks for the answers. I have now been out and read some of your blog and found this interesting.

You did bring a smile to my face when you discribed your distance from the hospital. I think we have a bit of interpretaton of language confusion. In Australia if you ask someone whose suffering ill health how they are 'travelling' what you would be asking is what sort of path their recovery is taking - ie the ups and downs of their health or mental state.

From reading your blog, am I correct in gathering that it could have been better, but it on the other hand, it could have been a lot worse?

[SLB]

Hi Sandi, like chirley said I am going thru private system and thank goodness I haven't had to pay for the vidaza! I have had a few out of pocket expenses for things like xtay and ct scan when I was in hospital and one pathology test that didn't have Medicare approval. When I see my hematologist in his rooms I am charged but mainly he sees me at the hospital when on my treatment so it is covered by my admission. Actually if I went public it would probably cost me more because of parking fees where as at greenslopes I park on street. All transplants happen at the public hospital but I am not sure if there is public and private sections? Interesting that you were told to go public. I considered going public but under the care of private specialist but doctor convinced us that private would be mostly no gap payments. After reading some posts on the American and other countries health system we are very lucky here!!
Take care, Sharnie.

[MDSPerth]

Thanks for the response Sharnie. Yes, our hospital system does seem different. We have had private health insurance for more years than I care to remember, and apart from the occasional 'extras' claim it has been otherwise unused. At the moment, Paul is happy to stay where he is at Royal Perth Hospital. Currently he arrives about 45 minutes earlier (for blood tests) than his scheduled appointment and the results are on the consultants desk before his appointment time. Occasionally he may have tests required inbetween his now scheduled three weekly visits - in this case he would have them done at an external centre. He can also pick up any medication required at the hospital as we do not that with some of them, if bought at a private pharmacy are not covered under PBS. I was told by the pharmacy that there is a difference between PBS and what the state can provide you with as an outpatient at a reduced cost?

Moving forward though, if he feels he is being messed around, its good to know the way the private system, because the haemotologist he sees does practice privately.