What can I expect in end stage MDS?

[Charbonnier]

My father-in-law appears to be in end stage MDS. He is not producing the correct amount of red or white blood cells, nor platelets. He lives in France and my wife has gone to take care of him. His doctor has told him that he has two to three months at best. How can that happen so fast? We never heard of MDS until the beginning of the summer, when abnormalities turned up in his blood test. He is being transfused every other week at this point. I don't know what questions to ask. And if I did, I don't know now to interpret the answers. Is the terminology used in Europe the same as in the US. I am trying to deal with the logistics of being there for my father-in-law & wife, but I really don't know what I am dealing with.

Sorry if I am posting to the incorrect thread. If someone could point me in the right direction, it would be appreciated.

[Neil Cuadra]

Charbonnier,

It's hard to understand MDS and harder still from afar. I hope you can learn enough to help your wife and her father.

Like other bone marrow failure diseases, MDS results from a failure of the boy to produce functional blood cells in sufficient quantities. This process happens in the bone marrow. When you have MDS, new blood cells may be malformed, nonfunctional, or simply too few in number. Something is wrong with the "blood cell factory" in the bone marrow that is supposed to help stem cells mature into active blood cells and then released into the blood stream.

There are ways to treat MDS, so you'll first want to understand whether your father-in-law has had, is having, or will have treatment. Perhaps he's too old to withstand treatment, perhaps treatments have already failed to help him, or perhaps he refused treatment. Whatever the reason, if they are calling it "end stage" I think you'd want to know why they are saying that.

Patients who are no longer undergoing active treatment of the disease can still be supported by transfusions or drugs that stimulate blood cell growth. Sometimes this sustains patients long after they would otherwise not survive.

In my experience, doctors can let you know how serious the situation is but are not good at predicting how long a patent really has to live. But they are pressured by patient and family members into giving estimates. I suggest that you consider them to be educated guesses, not promises.

You or your wife could ask the medical team what symptoms your father-in-law is currently experiencing, and in what ways he is being helped. You'll probably also want to learn what his wishes are. Some patients want every treatment option tried, some want supportive care only, and some don't know how to decide and want their family to manage their care. Learning his wishes and observing them is an important way to support him.

Although your father-in-law was diagnosed only months ago, he may have had bone marrow problems for some time. The symptoms of MDS aren't always apparent at first, and people whose blood counts are low but not critically low may not realize there is something wrong. Because some people live just fine with slightly low blood counts, low numbers don't always set off alarm bells after routine blood tests. But when symptoms become more apparent and counts are very low, doctors start looking for the reason, and an MDS diagnosis may follow.

MDS is very individual. In fact, it's considered to be a family of diseases, each with its own diagnosis and prognosis. Each patient's specific circumstances are different and their responses to treatment, drugs, and transfusions may differ as well. You don't really know what will happen and when. This uncertainty can be frightening, but it's also the reason so many families have hope for the future.

It's true that the health of an MDS patient can turn downhill quickly, especially if transfusions aren't able to keep the necessary blood counts up. What eventually happens to patients who can no longer be treated or their blood counts sustained is that the symptoms of low blood counts escalate and become critical and then life-threatening. Without enough red blood cells a patient's heart may not be able to work hard enough to transport oxygen around the body. Without platelets a patient may have unstopped bleeding. And most seriously, without white blood cells a patient loses a defense against infections. When death results from MDS, its often from an opportunistic infection or heart failure, not directly from the disease itself.

On the flipside, MDS patients may live months or years longer than predicted. Sometimes they surprise everyone, including the doctors, by recovering from a dire health condition and reaching a steady state where they can be sustained.

When the battle against MDS is being lost or end of life approaches, there are usually tough decisions to make about quality of life vs. quantity of life, about experimental treatments, about cessation of treatment, about hospice care, and about pain management. These issues aren't specific to MDS. You should be supportive of any decisions your father-in-law and his family make.

I'm sure there are differences between the U.S. and Europe in both terminology and the details of treatment at a particular hospital, but the diagnostic and prognostic systems most often used are international and there is a lot of international cooperation among MDS specialists. What you or your wife learn should be fairly universal.

Feel free to use these forums, and any other resources you find, for help in understanding what you hear and read. Your wife is welcome to ask questions as well.

[bebop]

if your Dad is getting transfusions every other week I don't think he may be as bad as the drs are saying. My dad got tranfusions every week for a few months. He got platelets 2 times per week. He lived serveral months like that. I don't think anyone can predict when they will pass though. Just give him the good times while he can.

[ccnarayanan]

Sorry to hear about your father-in-law's condition. I can relate to your situation to my dad's wherein he was all done in about 6 months since first major symptoms appeared and within 3 months of MDS diagnosis.

Neil Cuadra has summarized the landscape of MDS. I will just add my inputs on top of his.

Since there is problem in all blood cells, the situation may quickly take a downward trajectory. Transfusion is just supportive care. After some time, the body starts treating the transfused blood as a foreign body and starts developing defense mechanism against it, so it would end up being difficult to find a donor's blood sample which would become acceptable for transfusion even if the blood group is the same.

One other thing to keep in mind is what cytogenetic / chromosome abnormalities he has which manifest in the diagnosis of MDS. Some abnormalities are benign but some are severe. The progression of the disease to AML and the end of life often depends on the severity of chromosome abnormalites.

If you want to understand the severity of his condition, you can evaluate his IPSS (International Prognostic Scoring System) score (http://www.qxmd.com/calculate-online...prognosis-ipss).

Some patients respond to active treatments such as Decitabine and Vidaza and some do not.

If it is really an end-of-life scenario, the best you can do is to provide maximum support and comfort to the patient and the people surrounding him - it is a very noble thing to provide "quality of life" to a patient who is approaching end-of-life. You can read about "end of life care", "palliative care" etc. in wikpedia or elsewhere to understand the concept.

[Alvin]

I was getting transfusions every nine/ten days.
Started to take 2 drops cannabidiol (CBD ) every day, now transfusions are - platelets 76 days hemoglobin 30 days. CND is not legal in Canada but is legal in USA and I am sure France.
Alvin

[beverly]

How are you doin now? My husband just started CBD.

[Mseth]

Hi Beverly,

Which form/brand of CBD is your husband using? Where is it available and at what dose? I have been trying to research this but not got any definite information on where to buy. We are in India, but can arrange to get from US if needed.
Do post on the response your husband has with CBD, or any other information you can share.

Thank you and all the best!!