Without Diagnosis and early stages (hypoplastic MDS or AA)

[CatherineJ]

Hello,

I am currently without a diagnosis as my first biopsy was a "suboptimal" sample. My hematologist has narrowed it down to either hypoplastic MDS or AA. I'm scheduled for another BMB on this coming Monday, under sedation this time. I was just wondering if there are other people who have been diagnosed in the early stages of the disease and if they have any advice on how to plan your life in the future.

I'm 25 years old, just passed the bar exam in New York, and I recently moved to London to get my masters of law in September. I have at least another six months in London, and I would love to start applying for jobs and have a life, but I feel as though I can't plan on anything without knowing first what I have and second when or if I will get sicker. My hematologist seems reluctant to give me an idea as to what my future might be like.

Any other advice on dealing with this disease would be greatly appreciated. Any advice on dealing with lower blood counts, low energy, and being away from family and having to take care of yourself.

Last BMB showed less than 5% cellularity, last CBC: WBC 3 RBC 2.73 HGB 10.2 PLT 74 (they finally went up! have been in low 60s) HCT 29. Normal cytogenetics.


Thank you for any help,

Catherine

[annmonster]

Hi Catherine ....

I'm so sorry to hear that you're going thru these scary things at such a young age.... my advice to you would be :

1. Don't panic !

2. Get educated as much as possible on your diagnosis... ( and it sounds as though that is not clear at the moment ).

3. Ask for a second opinion...fresh eyes on your case can only help, especially if it's at a teaching hospital, or cancer treatment hospital.

4. Many Dr's do not like to guess on prognosis ... ( ie, how long you live ) as this disease is quite unpredictable and greatly varies from individual to individual.

5. Don't give up hope.... and be persistent .... you may be an excellent candidate for a bone marrow transplant which is considered the only 'cure' for MDS.

6. Hold on to your future plans loosely... MDS often creates a future with a lot of 'maybe's' and 'depending ons' ... if you're a 'ducks in a row' kind of person, you will need to adjust your expectations for a 'concrete' life .....I know, easier said than done....( been there done that ) .

7. Seek out help from close friends , family, or a therapist..... trust me... you WILL need a support system ....

8. Don't panic !!! ( oh yeah, I said that already ) .....

Take care, and please let us know how you're doing.....

Ann

[squirrellypoo]

Wow, really! Another young expat in London with AA/hypo-MDS? First Dan posted earlier this week and I was shocked there was another like me, and now you as well! :O

Which hospital are you going to in London? I would highly, highly recommend having your BMB results sent to Prof Mufti at Kings College Hospital for analysis, if not a full-on referral for you to go there yourself. He's one of the leading MDS specialists in the world on MDS (and Prof Marsh at Kings is the same, but for AA). They handled my transplant there 2.5 years ago and I have nothing but praise for everyone there. I'm happy to say I'm back to full health now with normal blood, no GvH, and I'm running my first half-marathon in two months.

Also being an unofficial visa expert, I'm somewhat concerned about your NHS eligibility if you're on a short-term student visa, though, and any implication your illness might have on your studies, which could then impact your visa if you are no longer able to attend law school here. But I think the first thing to do is have your scheduled BMB and get those results analysed by an AA/MDS expert here, NOT just a haematologist. You really need to know more before you can start planning treatment or your future. Some cases move very rapidly, but plenty of others are in "watch and wait" for years without need for much more than the occasional transfusion.

[CatherineJ]

Hello and thank you very much for your responses.

I just had my second bone marrow biopsy this morning and I don't remember a thing, doing it under sedation is a wonderful thing.

My blood counts have remained stable so far it seems...

I was just wondering if anyone has any bone pain? I have tremendous pain in my tailbone, which makes sitting a literal pain in the ass... I have had an MRI but the technicians messed up and did not actually get the tip of my tailbone in the images. From what the radiologists could tell though the bone marrow in my spine looks "funny" and they think that could be causing the pain. I don't know what "funny" means but I think they mean that my bone marrow is essentially empty since I have less than 5% of blood producing cells. Anyway, just wondering if anyone else experienced bone pain like this.

Also, I have had blood in my urine consistently for a few months now with no UTI and a consistent fever ranging from 99-100.5. Any advice or similar experiences?

Thank you again. I'm trying to stay calm.

[mscrzy1]

I'm just curious what your WBC level is? I didn't have "bone pain" with my AA, but will be very sore in the bone after my BMB for quite a while making it hard for me to sit if that's what you mean. I hope you find some answers soon!

[CatherineJ]

My tailbone literally has started hurting when my cytopenia started about 8 months ago. The hematologist said my bone pain may be connected to whatever my bone marrow problem is.

WBC 4.2, Neut 1.83

[squirrellypoo]

Originally Posted by CatherineJ

Also, I have had blood in my urine consistently for a few months now with no UTI

Have you been tested for PNH? It's another bone marrow disorder that commonly manifests as brick-coloured urine. It's even rarer than AA/MDS, but there are treatments available.

and a consistent fever ranging from 99-100.5.

I would get low-grade fevers stretching on for weeks, but these were always the signs of an infection somewhere, most often in a Hickman or PICC line or lurking elsewhere in the body. Post-transplant these were taken VERY seriously. You should have your doctors swabbing you like crazy and/or taking antibiotics...

[CatherineJ]

I have been tested for PNH, and it came back negative. I think my hematologist is ordering a CT scan of my chest, neck, and abdomen to see if there is anything going on to cause the bleeding and the fever.

[usaf1125]

I agree with AnnMonster, don't let it become an 800 lb. Gorilla. I was diagnosed in 2003, I'm low level ,has of 12/21/11, I have had 280 units of blood. You will be tired, so rest. Stay positive.

[Darice]

Originally Posted by CatherineJ

I was just wondering if anyone has any bone pain?

It doesn't sound like you have been getting Neupogen or Neulasta injections, but those can cause bone pain.

Originally Posted by CatherineJ

Also, I have had blood in my urine consistently for a few months now with no UTI and a consistent fever ranging from 99-100.5. Any advice or similar experiences?

The blood in your urine I would get checked out with a urologist. My husband has been dealing with TCC (transitional cell carcinoma) or bladder cancer for just over a year now, and his first symptom was blood in his urine. It is one of the most under-diagnosed cancers because so many doctors delay looking for other causes of blood in the urine . . . they will just dismiss it as a probable UTI and never go further. It is fairly simple to get that checked out, and real tough to treat if it goes on too long.

[Cheryl C]

Re your fevers, have your immunoglobulin levels been tested? I am feeling so much better since being treated with Intragam (gamma globulin) which has built up my immune system - I no longer get night fevers/sweats. A lot of my unwell feelings were due to underlying unknown infections. I feel so much better now even though I have MDS RAEB2.

Hope your search for knowledge and appropriate treatment succeeds.

I found the American Cancer Society booklet most helpful in teaching me more about MDS. See http://www.cancer.org/myelodysplasti...iewguide/index

Cheryl

[Greg H]

Hi All!

Cheryl's link to the ACS info on MDS appears to be broken for some reason.

This one should work:

http://www.cancer.org/Cancer/Myelody...iewGuide/index

And here's a link to the detailed guide:

http://www.cancer.org/Cancer/Myelody...ledGuide/index

I haven't read these, so I'm happy to find out about them. Happy reading!

Greg

[CatherineJ]

So it has finally been determined that it is MDS with multi-lineage dysplasia with no increase in cell blasts.

My counts have all gone up since two weeks ago.

PLT 89, RBC 3.38, WBC 3.50, HG 12, HCT .36

Since my counts seem to be relatively stable and not so low to require any immediate treatment, it seems that I have to wait until I get worse.

My doc said my possible choices were ATG or bone marrow transplant, but doesn't want to do either yet since I'm still doing ok. Does anyone have any advice on waiting and deciding on treatment?

I go in on Thursday for MRI to see why my tailbone hurts, and Friday for a full body scan to see if there is anything causing my fever.

I see my doctor again next week after they have seen the scans.

I'm trying to stay calm. but even though the doctor said I have a low risk type of MDS, since I am so young, I have many years for it to develop and become more malignant. Has anyone else had the experience of catching the disease early and waiting for a certain period of time before doing a bone marrow transplant? Should I be considering it?

Thank you for all your help and responses, it helps to know there is a community out there that understands what it feels like to go through with this strange disease.

[squirrellypoo]

Hi Catherine.

Well, that's good that you've got a firm diagnosis now! In general, if your counts are fine and you don't require transfusions, then "watch and wait" really does sound like the best approach. They'll keep a close eye on you, and if things get worse, they'll know right away and you can take a view then.

You're young now, yes, but remember that treatments are getting safer and more effective with every single year, so if it does turn out that you need a BMT in five years' time, it may be an entirely routine procedure then instead of the very invasive, rather risky, months-long ordeal it is now... Look on the bright side - you've got breathing room now to research and decide and have babies and LIVE LIFE instead of being forced to choose based on rapid deterioration. You're very lucky in a lot of ways!

melissa

[Greg H]

Hi Catherine!

Sorry to hear about your diagnosis, though there is some comfort in knowing what it is that you have.

Watch and wait is definitely a very viable strategy for someone without significant symptoms. I had platelet counts in the 85-95 range for 15 years with no worsening. I have no idea whether I had dysplasia at that point, since we didn't check.

Transplant is something that someone with low risk MDS should learn about, but not, in most cases, contemplate actually doing. There are two studies out there that look at the "sooner vs. later" question with transplant for MDS. The first and most famous is by Harvard's Corey Cutler, who found that, on average, folks with lower risk MDS live longer if they delay transplant until the point at which they are about to progress to AML. Folks with higher-risk disease at diagnosis, on average, live longer if they go ahead with transplant.

There is a brand new update of that study that I have not read, but heard explained on an AAMDS webinar yesterday, which found exactly the same thing. Transplanters would, of course, prefer to transplant younger, healthier patients, because it give the absolute best chance of surviving the transplant. But, as I watch webinars and presentations, I hear more and more transplanters flatly say "We don't transplant for lower risk MDS."

It is tough to have a serious disease and to decide to watch and wait -- i.e., to "do nothing about it."

It might help to think about that line in the Hippocratic Oath: "First, do no harm." Almost everything you can do for MDS, aside from supportive care, will make you sicker before it makes you better -- particularly with the kind of CBC numbers you have. Most therapies will lower one or more of your blood counts, and most therapies will provide at best temporary relief from symptoms (which you don't really have at the moment).

Your doctor likely mentioned ATG because you are so young, and younger patients are among those most likely to do well with immunosuppression. But ATG will trash your white cells for a while (that's what it is supposed to do) and leave you vulnerable to infection. You'll like be on cyclosporine after, also leaving you open to infection, plus other side effects that could make your quality of life suffer. You could do Campath, another immunosuppressant, but it will also suppress your counts and leave you open to infection for a while.

It's hard to watch and wait, but, as so many of us have found, it is often the very best approach for lower risk MDS.

If your numbers do start to slide at some point, immunosuppression would be a reasonable option, and you'd want to do that after your counts drop but before you get a lot of chromosomal abnormalities.

As Melissa says, there is tons of research going on about MDS -- from drug therapies for low-risk patients through better transplant techniques. Watching and waiting means you will get the benefit of that research when you do need to pursue therapy.

It's great to have you as part of our intrepid band of bone marrow warriors. I am sure that, as you learn more about the disease, you will be teaching us all a thing or two.

Take care!

Greg

[michellek]

I also am in the early stages of watch and wait for AA. It is making me a little neurotic. I had a month with no blood test or doctors visits and it was soooo nice because now I'm back to doctors visits, labs, procedures and decisions. My doctor does want to treat with ATG sooner rather than later.She says better lab values better outcome. I do read other web sites and people have complications and I wonder if I want to go through that before I really have to. Well we'll see. I do feel like my life is on hold. I used to like to take several vacations a yr. but I'm afraid my labs will drop. I turned down a job promotion because right now I can't worry about work while I worry about my lab values and upcoming tx's. Good Luck with your decisions.

[CatherineJ]

Michelle- how long have you been in the wait and watch zone? I agree it is does a number on my nerves not to have a blood test and the longest I have gone at this point is two weeks. Thank you for posting, I am not glad other people have to go through this but it is nice that we can compare and commiserate with each other. I guess it's hard to plan, are you thinking of doing the ATG anytime soon?

Greg- your information about Cutler's research is very interesting. I have looked up some of the articles and read over them. Thank you for your great advice. I plan on talking to my doctor about that this week. I think he may prefer to do a transplant since I am so young and he seemed to imply that I have a whole lifetime for the MDS to progress to AML.

Melissa- thank you also for your response. You are definitely right that I have time to contemplate before making decisions and time to live life. That is exactly what I needed to hear, and I really appreciated that. It sort of snapped me out of my initial daze.

Anyway, going to talk to doctor about optimal timing for stem cell transplant. It seems to me that I should not bother with first trying a course of ATG because it's not a true cure. I don't know if this really wrong to think.

Thank you again everyone.