RARS journey

[Malissa Kirszenbaum]

I hope this post finds everyone stable healthy, happy, and enjoying each day. My posts have been sporadic mostly because LEON has been somewhat stable over the past couple years, all things considered. In the early days of this disease, I do not think I fully understood what “low risk“ meant in the context of this disease. I think my brain heard low risk and I was more focused on the number of years he might have versus all the information that was read, discussed, and shared here and with Leon’s specialists regarding the “low risk” journey itself. Because LEON’s body and infections all started to calm down a bit, I got into a comfortable pattern of pushing it away. His challenges were pretty much fatigue, breathy, and dizziness. After awhile, he learned to cope, we learned to cope together, and this seemed like our new normal rhythm. Infections calmed and his counts stayed low without anything new or remarkable really until this past spring.

When he was fully diagnosed with the RARS, I remembered the fear I felt. Since then I have compartmentalized that fear and it really has not been actively present until recently. I first need to tell everyone here how important this forum has been for me, the caregiver, for a man who I have been with since I was 18 years old, I went from graduating high school to my husband and we are nearing almost 40 years married with four beautiful children who are now young adults. My husband is now 66 (retired pharmacist) and I am 59 (yes he robbed the cradle lol)(retired litigation paralegal). I cannot begin to imagine what my life would be without this man.

When changes started happening over the last month and half, i.e., first time blood transfusions, high ferritin (non-transfusion related); etc., I began to feel a bit panicked if I am being honest. THANK YOU – TO EACH AND EVERY PERSON WHO HAS TAKEN THE TIME TO WRITE ON THIS FORUM. My go to – I have been reading the message board on this forum for hours and hours and hours over the last two weeks. Some of the stories of members brought tears, for sure, but It was the strength, determination and camaraderie of all of you here that helped calm my soul, centered my emotion which I so needed.

So I am writing this for the next person who may need what I needed. Maybe someone has recently been diagnosed with RARS. I fully understand now that just because he is in a low risk for AML it does not mean that our journey will be without a fight. I would encourage you to take time and read through all the subject matters that people took time while feeling bad or feeling ill or maybe teetering on feeling hopeless themselves but pushed forward so that we could have the information that might help us on our journey.

As for Leon, it appears that the recent hip replacement surgery that LEON had on July 1, has stirred up some new issues. We have had several blood transfusions and it does not appear right now that his bone marrow is taking over on its own to replenish his shortages. We have now had discussions about chelation and perhaps procrit – we will know more by August end. We were not pleased with his latest blood results yesterday he is now neutropenic and his hemoglobin has dropped to 10. We were hoping to see it go in a different direction. We are both individuals of strong faith and understand that most of all of this is beyond our control. We will continue to embrace each and every day with hope and give thanks for our blessings. I hope this was not too wordy or ran on too much but I just felt an overwhelming desire to come on here and to thank everyone. I even went to the memory wall and I was able to connect some of the people that are no longer with us with some of their prior posts which were so encouraging. It has been extremely humbling. RARS may be Low risk but it does not mean that the journey will be low-key and easily managed.

God bless all here with strength, courage and hope in healing.

[Jack Allen]

Hi Malissa,

I just read your note and it was nostalgic to me. I have RARS and went through much the same journey.
I lived in Toronto Canada and moved to Calgary Canada with my wife when I was 35. We built a wonderful life; did well on investments; were going to have good pensions and looked forward to a retirement with lots of travel, etc.
That world came crashing down when I was 52(2002) and diagnosed with RARS. When the doctor told me, he said “you had better get your affairs in order”.
At that time I guess it was extremely uncommon, perhaps not well diagnosed, but in any event I scoured the internet and it said 1 in a million would get MDS and 2% of those would be RARS. Doing the math that meant 1 in 50 million.
Canada’s population was less han 35 million at the time so you can imagine my dismay. My wife and I got our affairs in order. I never went back to work and we decided we had to tell all our friends and family in person not by telephone or email. Big roadtrip thousands of miles.
On our return I surprised everyone, including my doctor and was unchanged on my counts for several years.
At one review my doctor turned to my wife and said “at this rate he ight outlive us both”
After hearing that I dcided to make some lifestyle improvements. I quit smoking. I was down to 6 cigs a day for a long time anyway.
Well, upon doing that my hg dropped 10 points on my next visit. Doc said that would be expected; your body makes more red cells to compensate for reduced oxygen that smokers get.
However they continued to drop from a stating point of 130 to 90.
At 90 a I started to develop A-fib.
My first recommendation to readers. IF YOU ARE STABLE CHANGE NOTHING.
A transfusion was given and my A-fib disappeared. It was agreed the I would be kept at a baseline above 100.
In 2007 my wife convinced me to move back to Toronto to be closer to family and friends, although we had many close friends in Calgary. Thhe convincing point was that they may want to visit and none of them could afford to fly to Calgary.
So we moved back and I went to see the Canadian guru of MDS at Sunnybrook. He had connections he put me through,
all the meds. Darbypoetin, anaresp, valpoic acid, revlimid, Vidaza, maybe others I don’t even remember. The best one for me was Revlimid eventhough I suspect it destroyed my thyroid. One year perfect, next year dead. The next best was Vidaza but for me I would never do it again; not worth the pain.
On a further note, I went to Sunnybrook for a transfusion 1 day, a long drive for us, spent the morning, $25.00 for parking plus cafeteria lunch to go back to the transfusion lab at noon to start my transfusion (IV had been put in in the morning) only to find that my hg result was 86 and at that hospital their cutoff for transfusion
was an hg count of 85. They pulled th IV and sent my home.
I asked to be referred to another doctor closer to home and started to get my transfusions from Oshawa Hospital where my doctor overides the blood restrictions.
Ontario health care is rediculously stupid. I have had so many heart problems and operations and cardio conversions, echo cardiograms, etc, etc, just because they refused to keep my baseline at 100. They have spent millions on cardio care.
NOTE TO READERS; HOSPITALS AND BLOOD SERVICES HAVE DIFFERENT BASELINE RESTRICTIONS. FIND A DOCTOR WHO KNOWS THEM AND CAN GET YOU THE MOST BLOOD.
I am now 72 (20 years into it) way past my given best before date.
I have developed severe lung problems and am now on 7 by 24 home oxygen and connot make to stairs once I get into the 80s hg.
I have also moved to 2 bags of blood every week have come off Luspatercept in case it was a contibutor to my lung problems.
The end my be getting near, but I intend to stick around as long as I can. My teenaghe grandchildren all still like to see me and I still hope to see a great grandchild before I go.
NOTE; IT HELPS A LOT TO MAINTAIN GOALS FOR THE LONGER TERM AND SOMETHING YOU WANT TO DO TOMORROW.

[Malissa Kirszenbaum]

Thank you for sharing that Jack. I am so sorry that things are becoming that difficult. You sound like a trooper, do not give up hope! You talked about some thing that really caught my attention and some thing I have not been able to convince the doctor on. Two years ago Leon developed a fibrillation. I kept asking that question since one of his challenges is a low hemoglobin I felt every time he was below 11 it just started to get worse. He had two heart ablation surgeries and the second one has worked so far. However shortly after the hip surgery in July one of the resident doctors was making rounds listened to his heart and heard some thing and decided to have an EKG which turned out to be abnormal. He said that there was some rhythm issues and he too had an echocardiogram while in the hospital and a few other tests. In the US, as long as you are 8.0 or above you are not getting a transfusion unless there is an emergent need. We are experiencing a shortage of blood here so it is very difficult to get them to give you more than one unit or a bag. I do not believe Medicare will even approve of a second transfusion after you reach 8 or above. They discharged LEON after several transfusions the highest his hemoglobin got was 7.4. They said that the surgical team were in contact with Leon‘s local oncologist and his oncologist at Hopkins a care of excellence, and that they would take care of any transfusions LEON might have outpatient. They said the greater concern was him staying longer than the seven days because what they didn’t want to happen was for him to get an infection. Apparently when you have this type of surgery if there’s an infection that develops in the body the source of surgery becomes like a beacon for the infection. They said in his case that could become a very serious issue where he could lose his leg or die. Every time I tried to speak up they always had an answer and I’m not very good with adversarial conversations lol LEON is so tired that he just lets half of everything go, that is not me.

I will include you in my prayers Jack and your family. This is not easy stuff to deal with and I am only the caregiver. LEON has a pretty good attitude most of the time but this surgery really took it out of him. I was so disappointed with Friday’s results and hope that he can at least climb back up to his pre-surgical numbers. Yes he is dizzy and yes he is tired and breathy but it was something that he had learned to cope with. We rely on our faith heavily and we do believe in the power of healing. I guess we just keep on going doing our best, hoping for the best.

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