Anyone in Stage 4 MDS?

[milliken2]

Hello All;
I just joined the forum today. My husband - 65 - is the one who is afflicted with the MDS. Unfortunately, I think the hospital missed the diagnosis 2 years ago when they did the bone marrow biopsy - and again last fall when they repeated it. He had one done at Moffit in February of this year. and the Dr. there told us he had MDS. Said the slides sent from the Pittsburgh hospital were 'deteriorated' and not usable for comparison. My gut tells me that they were purposely sent that way because they missed his diagnosis, and now he is in Stage 4 MDS - RAEB I.
Though I have been a nurse for many years, this is one disease I never came in contact with - and I am at a loss to help him. His counts are terrible - his platelets today were down to 16,000 - I know - scary level. He is going in for a platelet transfusion tomorrow. Either way - I have no idea what I am in for. He has completed one week of Dacogen therapy and now on his first week off. His appetite is terrible, and of course - with his counts down - he has no energy. I can't get any of the Dr.'s to give me a straight answer - is he at a stage where he should continue therapy - or should we just continue with the weekly transfusions? Help anyone.

[Neil Cuadra]

Beth,

If you haven't already done so I suggest that you get the MDS information packet from the Aplastic Anemia & MDS International Foundation. It's targeted at patients and caregivers and is a good starting point for everyone who is dealing with this disease.

I also suggest that you phone the Foundation and ask what other information they can share with you since you are a medical professional. See their Health Professionals page.

The treatment choice for your husband is ultimately a choice that you, your husband, and his doctor have to make, but if I was the patient, at age 65 with MDS-RAEB-1, I would definitely choose treatment over supportive care (transfusions) alone. Transfusions could get me past the immediate symptoms, but drug treatments would give me a chance to stop or reverse the disease progression. The first question I'd ask my doctor is how to choose between Dacogen and Vidaza and whether my MDS makes Revlimid a candidate treatment as well.

That's not to say that these drug treatments are easy, or always the best choice. They can set you back, with low counts or other side effects, before they improve things, and sometimes they don't help at all. This disease involves so many factors and can be so specific to an individual that doctors may have to try different dosages, schedules, or drugs to find a winning combination. That's why you want as much information as possible and to understand the tradeoffs as you make your treatment decisions. If insurance coverage and the costs of treatments are factors you have to weigh, don't be shy about asking about that too.

Is your husband in pretty good health overall, for his age? It's certainly a good sign that his MDS was able to stay under the radar for over 2 years. Even if they should have diagnosed it earlier, it tells me that his symptoms were not severe and the disease was progressing little if at all. But clearly his lack of energy and risk of bleeding have reached the point where you need to act.

To learn more about what you might face in the weeks and months ahead, you can use these forums to hear from or converse with patients who have been on Dacogen or who are treating MDS other ways. As a nurse you'll be able to understand technical articles that often just confuse us laypeople, for example An update on the safety and efficacy of decitabine in the treatment of myelodysplastic syndromes. I suggest doing some of your own literature searches so you'll have a better handle on the medical aspects of MDS.

You may feel that you can't help your husband right now, but you have a lot of power already -- to research, to be his advocate, to participate in treatment decisions, and to work with his doctor. By asking questions at a site like this, you are already using that power to your husband's advantage.

[milliken2]

Neil;

Thanks so much for replying to me. Regarding my husband - whom I affectionately refer to as E.T. and those really are his iniials - his health has been downhill over the past 7-8 years. At about that time - 8 years ago, he was diagnosed with A.P.S. - Antiphospholipid Antibody Syndrome - or in laypersons terms - sticky blood. He was put on Coumadin for about 3 years - then had 3 D.V.T.'s in his left leg - he ultimately was put on Arixtra injections - but of course hasn't received any since his platelet count has rarely been above 40,000 for the past several months. He has actually received 82 pints of blood and 6 units of platelets since the middle of August - 2011. He also has Leukocytoclastic Vasculitis - and is currently on a maintenance dose of 5 mg Prednisone twice a day. If he get an exacerbation of the LV - I adjust his Prednisone. But - he was still able to do house things - like mow the grass, walk to the mailbox, etc. Now - with his blood levels being so low - he doesn't have much energy for anything.
We live in Pennsylvania - but have been wintering over in Florida for the past 5 years - mainly because him being on a blood thinner - the cold bothered him. I am almost wondering if the continued injections of Arixtra could have contributed to his current MDS. The hematologist we found in Florida thought Earl had MDS even before he had the bone marrow biopsy at Moffitt. He wanted to treat him with Vidaza then - but I didn't know the Dr - and I called our Pittsburgh Drs to get their opinion - and they flatly told me no - DO NOT have him start on Vidaza - so we declined treatment at that time. Then - we went to Moffitt in February, and they did another bone marrow biopsy there - and they said definitely he had MDS - RAEB 1. By that time, it was so close to our returning home that the Drs. there said we should wait till we got back here to start treatment. So - that's what we did - saw our PGH doc on 3/27 and then saw the local oncologist/hema here the following week. He also agreed with the Moffit dx - but wanted to do another bone marrow biopsy and send it to an independent lab for reading - and it too came back with a positive dx of MDS - RAEB 1. We found this out today, but the Dr. has already found out that I won't sit back and wait for treatment. When we first went there last week for his initial chemo treatment, they did a CBC and his hemoglobin was 7.9. I asked if they were going to transfuse him - and the nurse called the Dr and he told me he wanted to wait. I said a definite no - as I know how ET gets when his levels drop - and he agreed to one unit of packed RBC and a chelating agent to get rid of excess iron. A week later - this past Monday - his Hgb was down to 7.7 - and I again had to push for 2 units of blood - one just does not bring him up. We missed Easter dinner with the family since he was too weak. I am trying to learn as much as I can, and I did get some literature from their office, but will definitely take advantage of the links you sent me.
Again - thank you for your help.

Beth

[mausmish]

Hi Beth,

Sorry to hear you and your husband are going through this. I am an MDS survivor. This forum is a great place to be for information and support and Neil has provided you with an excellent resource as a starting point. MDS is a different beast. For example, it is not "staged" like solid tumor cancers so "stage 4" is a misnomer. It is instead classified as low, medium, or high risk based on a number of factors such as chromosomal anomalies, cytopenias, and number of blasts (immature blood cells). It is often tempting to contemplate the "what if's" regarding causes and timing of diagnoses but it's best to move forward and look ahead. My hematologist gave me good advice, "You may never know why - sometimes it just comes down to bad luck. And it probably wouldn't have made a difference if you'd been diagnosed sooner." There's another good informational booklet from the lymphoma and leukemia society, here in pdf form, http://www.lls.org/content/nationalcontent/resourcecenter/freeeducationmaterials/mds/pdf/mds.pdf.

If you post questions here in the forums, you'll almost always get a variety of useful answers from other patients and caregivers based on their own research and personal experiences.

Try to stay positive and keep your sense of humor! ET is one of my old favorite movies :-)

Wishing you the best.

Karen

[bebop]

to me it sounds like his dr is not very aggressive in his treatments. I would try to find an expert in mds.

[Anne Yeomans]

My Oncologit/Hemo in WBP is Dr. Todd Gresten. He trained under Dr. List. best MDS Doctor in the Country. He works out of Moffitt Center. I a also hooked up with Dr. Field for my BMT. Hope your search is quickly found. and God Bless Anneg

[milliken2]

Originally Posted by bebop

to me it sounds like his dr is not very aggressive in his treatments. I would try to find an expert in mds.

BeBop; Sounds to me like you have no idea what we are going through - this has been a severe roller coaster ride for us - and comments from you like not seeking aggressive treatment are totally unnecessary - you don't have any idea !!!!!!! Moffitt Cancer Institute and the Dr. we saw there were supposed to be tops in their field. Unfortunately - we are not in Florida now - or we would be being treated there. I am doing the very best I can.

[mausmish]

Beth, I'm sure Elaine's (Bebop's) comment was well-intended. If you read through her past posts, you'll see she went through a lot with her dad before he lost his battle. She can relate very personally to the helpless feeling of not being able to do more for someone who means the world to her. She's always been very supportive to others as well. The members of this forum are here because their lives are touched by marrow disorders, often in terrible ways. I don't recall ever seeing a mean spirited or flippant comment. This disease makes us all crazy and frustrated at times and we all have our own ways of coping. In many ways, it's harder on caregivers than on patients. We patients concentrate on trying to get better while our caregivers are out there doing everything else and wanting with all their hearts to somehow make it all better. I know my husband has been through hell for me. MDS is a rare disease, and often difficult to diagnose because it is not a single malady but a collection of related ones about which much is still unknown. It can be especially difficult to find experts in MDS and it's common for patients to get multiple opinions before finding a course of treatment that works best and common for forum members to seek opinions about different doctors or treatments or treatment centers. This informal networking is invaluable. We learn through each other's successes and failures and we celebrate our victories, even the smallest ones, and commiserate in our sorrows. I'm sorry to ramble so much but it is late and I am tired. Sending lots of healing thoughts to you and your husband. Karen

[riccd2001]

Thanks, Karen. There are times we get so frustrated, things are written without second thoughts and once posted - well shall I say, regretted.

[Greg H]

Hey Beth!

Sorry to hear about your husband's disease. I'm with Neil and Bebop; a diagnosis of high-risk MDS is not, necessarily, a reason to forego treatment.

Since you're a nurse and likely very familiar with the "staging" used for solid tumor cancers -- and you've said you're not all that familiar with MDS -- I think it's worth noting that "staging" isn't exactly the right idea for the IPSS (International Prognostic Scoring System) in MDS. Even though some folks (and even doctors, who should know better) use "Stage 2" or "Stage 4" when talking about the IPSS score, that's really misleading.

Why? Because, if you are used to solid tumor cancers, when someone says, "Stage 4," your mind immediately goes to metastasized cancer, present in several organs, no hope but really aggressive chemotherapy that might be worse than the disease.

That is not an accurate way to think about an IPSS score of 4 - High Risk. High Risk MDS is dangerous, but treatable. For High Risk MDS, particularly with excess blasts, the standard of care is Vidaza or Dacogen, at least six monthly cycles. The alternative is stem cell transplant. That might be a tough road given ET's co-morbidities, but it could be worth exploring with a transplant specialist. If Vidaza or Dacogen doesn't work after six cycles, there are some clinical trials that would be worth trying.

For what it's worth, while 8.0 is my trigger point for transfusion (and I always get two units), my docs at the National Institutes of Health tell me their standard is to transfuse below 9.0 for folks over 60 years of age, 8.0 for younger folks.

Good luck to you. We're all here to help, so keep asking questions. Does ET have any chromosomal abnormalities that showed up in his bone marrow biopsies?

Take care!

Greg

[milliken2]

Hello;
Sorry if I appeared irritated with Bebop - it's just that I feel that I am doing all I can - and not only the physical caring - I do all of the appointment making, questioning, etc - and sometimes I am overwhelmed - especially with his current condition - and finding out that the Pgh hospital did indicate in their bone marrow results of October which stated: "Overall, the findings are non-specific. Medications and auto immune processes etc. need to be considered. Due to cytopenias and pelgeroid cells a low grade myelodysplastic process should also be considered in the differential diagnosis, although it appears less likely. Correlation with cytogenic studies is therefore recommended." And no one ever recommended any more studies or testing.

Now - when we asked about the results of his bone marrow biopsy - we were told that it was "normal". I did not get these results until I called for them to take with us to the Cleveland Clinic on May 10th. We are seeing an MDS specialist there - Dr. Mikkahl Sekeres - and I hope he has more answers for us.
Earl starts his second round of Dacogen next week. He was transfused with 2 units of PRBC and 2 units of Platelets last week, and I am sure by the time he gets his blood drawn on Thursday - he will be at transfusion level. Actually - his Hgb wqs 8.5 yesterday - which is transfusion level for him, but the Dr. said he could wait until he gets his second blood draw on Thursday - so Friday will be a long hospital day for him for sure. He gets a chelating agent also - since he has had so many transfusions.
Thank all of you for your help.

Beth

[Birgitta-A]

Hi Beth,
When your husband will see Dr Sekeres he will get the very best care. Dr Sekeres is world famous and you can find several webinars with him in the AA MDS Foundation webinars archives.
Kind regards
Birgitta-A
73 yo, dx MDS Interm-1 2006. Positive results with Thalidomide + Prednisone since 2010.

[milliken2]

Briggita;
I know I did some research on the AA/MDS site and found Dr. Sekeres there. I am glad to hear that hopefully we are headed in the right direction. Cleveland Clinic is about 90 minutes away from us.
Right now, Earl is battling a cold that I hope does not turn into pneumonia. He is on a Zpack, and of course, I am keeping track of his temp and forcing fluids. He has been more nauseated the past 2 days than ever before. I would like to get him built up before he starts his second round of chemo, and before we head to Cleveland.
Thanks for the positive info and thoughts.

Beth

[Birgitta-A]

Hi Beth,
Too bad with the cold!

You know I got neutropenic fever 2007 after 3 infections and since then I live as if I had neutropenia (too low neutrophils). All infections can decrease counts. My WBCs are low but not very low - latest neutrophil count 2.6 - but I avoid persons that can be infected, travel in my own car, avoid public bathrooms, only eat hot food and fruits that I have peeled myself etcetera. I have not had an infection since 2007 (knock on wood).

Hope Earl will be OK when the second round of chemo starts!
Kind regards
Birgitta-A

[milliken2]

Brigitta:

Thanks for the positive thoughts. He had a blood draw on Monday - and his Hgb was down to 8.5 - which for him is transfusion level. We talked about it at that time - and decided to wait until his blood draw yesterday - and the Hgb was down to 8.1. I asked if the Dr. was going to order a transfusion - and they said that the Dr. who is administering the chemo uses a level of 8.0 or below. I reminded the nurse that there were already orders on his chart from our Hema that told them to use the 8.5 level - and she snipped at me saying: "Well now - whose orders do you expect me to follow?" To which I promptly replied - the Dr who orders a transfusion at 8.5 - since I know if he drops much below that - he really goes down hill. So - today he got 2 units of blood - followed by the chelating agent to remove the excess iron. Like I explained to her - he is headed for chemo next week, and I don't want him in a weakened condition before he starts. He is already battling the cold. I am so hoping Dr. Sekeres is able to give me more information and guidance.
I, too, cook at home most of the time. There is one restaurant where he likes to have fish - but that's not often. And I wash all the veggies, etc. Like today when he was getting blood - I left him with a bottle of hand sanitizer since I knew he would be making frequent trips to the BR due to the Lasix they give him between units. And by the looks of the bottle - he did ue it.
I hope things are going well for you - and again - thanks for your concern.
Beth

[Birgitta-A]

Hi Beth,
Yes, we can only try to avoid infections. Good that Earl got the tx !

Since you are a nurse you have probably already read much about how to prevent nausea.
http://www.oncology.tv/SymptomManage...g-Updated.aspx

As far as I understand a combination of Aloxi (palonosetron), Emend (aprepitant) and a corticosteroid could be effective.
Kind regards
Birgitta-A

[lindajo]

You sound so overwhelmed. I know how sick ET. is but that doesn't mean he can't have some of the responsibility of making appointments and doing research.

It may be a very positive thing for him to do. It will help him take control of part of his life. In the beginning, after diagnosis, it is very easy for a patient to feel they have no control over anything anymore. Fatigue and shock make it easy to turn over all daily responsibilities to spouses and caregivers.

It is important however that a patient take back responsibilities for some things. It gives them a sense of self worth and purpose. It might also enable you to take a short nap. It is important for you to take time to care for yourself also. You can't be of help to him if you let yourself get rundown.

They have great options for dealing with all of the stress at Cleveland Clinic. Take advantage of all that you can. I will pray for you.

[milliken2]

Lindajo;
I understand what you are sayng - but believe me - I would be a lot more worried if I left it up to him to take care of things. You see - in the 41 years we have been married - he has lost his wallet at least 4 times, either lost or ran over 3 cell phones - you get the picture. This way - I keep his medical cards, have all the Docs numbers in my cell phone - and since I am an RN - I am able to talk to the Docs on a more medical level and usually get respect from them - since I come with all of the medical history, current medications, living will, etc - and it is all written in 'medicalise' so they know I am not just the 'wife'. I know - I have been told many times that I need to take time for myself - and lately - when he has been getting hiis chemo - I haven't been staying there - went for coffee with friends one time - had my nails done on another day. But - I still want to be the one who can ask and understand the questions and answers.
We saw Dr. Sekeres last Thursday. He was very nice, but he did not get the records from Moffitt in time (even thught I had been calling several times a week) so their pathologists only had the one from the independent lab, and the 2 from West Penn to compare. Even so - we were told he had 7% blasts by our local oncologist who did the independent BMB - and Dr. Sekeres said their pathologists reviewed that one, and saw only 2% blasts in the same one. Now - how can it be so different. And I know the one from Moffitt said he had 9% blasts. Who do you believe and how does the treatment differ? Dr. Sekeres said he will review all of the records once they get there, and send me his final report. He said Earl could not take Revlimid since a side effect of it is blood clotting, and he already has Antiphospholipid Antibody Syndrome (sticky blood) but since the MDS has started - his platelet level has been so low, he isn't taking the Arixtra. The Dr. did say that the MDS may have been caused by the Plaquenil he had been taking in combination with the Arixtra - so we are still not sure. He has been off the Plaquenil for about 3 months, and the Arixta for about the last 5 months. Dr. Sekeres said to continue the Dacogen, but if after 6 sessions - if there is no improvement - to let him know - and then we might return to the clinic and start ATG therapy. He would have to be admitted and stay there and be monitored for a week to make sure there are no side effects, We did agree to put him in a study via his blood work - and maybe - just maybe and hopefully - it will help - if not Earl - then someone else.
Sorry for the long post - but wanted to explain as much as I could.

Beth

[cathybee1]

No need to apologize for long posts. All I can contribute is that It isn't unusual for different pathologists and hematologists to see different things in the bone marrow biopsy slides. This happened to Bruce both last year and this one. 3 different pathologists reviewed Bruce's slides (2 at university hospitals) and their reports were not in agreement. In the end, it was the MDS Specialist at UCLA, Dr. Paquette, who put it together and gave us his opinion. Dr. Sekeres has an excellent reputation, but in the end, you and your husband will need to weigh all the information and decide what is the best course of action for you. You are very fortunate to be an RN and able to understand the medical jargon better than many of us starting out in this process.