New to forum & looking for some answers

[Ann G]

Hi, I've never posted to anything like this before so please excuse if I have not done this correctly. My mother was recently diagnosed (1/9/14) with MDS and just passed on 2/14. We were told that the specialists were not even done reviewing final biopsy results so the only info we received was that it was RAEB-1 (6% blasts). We went to Disney World on 12/6 and she did great. Then on 1/29 she started to spike very high fevers (103 +), so we went to the ER & she was admitted. They did all kinds of testing looking for infections, etc. but everything came back negative. During this time, we heard that the biopsy did show there was some chromosome involvement as well which put her at a higher risk. Still, they had indicated 9 months w/o chemo or possible 18 mos. with chemo. We never had the chance to even think about that because her fevers continued to spike ... she went as high as 104.8 - she received three transfusions while in the hospital and although her CRIT came up alittle, nothing really significant happened. Is this common with this disease? I'm having a hard time because she was my best friend and we were so close but it's really hitting me hard too because I just cannot imagine that she went so fast when in my mind, this didn't even turn into a leukemia yet. Any insights or thoughts that anyone may have would be greatly appreciated.

[bailie]

I am very sorry for your loss. It seems that there was more involved than just the MDS unless her blood counts were dangerously low. Can you recall what showed up on the blood tests (whites, reds and platelets)? What was your mother's age?

[Ann G]

She was 84. After her last transfusion, she had a difficult time and they thought it as a reaction to the blood but determined that everything was fine with blood itself. Her platelet count was 68,000, WBC was 5.7, hemoglobin was 9.3. She was diagnosed the week before with pneumonia but was given antibiotics and last chest xray showed that had cleared.

[Neil Cuadra]

Ann G,

I understand how hard this is for you. I hope we can help you process what's happened. I'm sorry you lost your mother and that it happened so quickly without leaving you time to understand all that was going on. I also hope that you and the doctors can identify what went wrong for your mother.

Mortality from MDS isn't directly from the disease but from the symptoms it causes. People with MDS can't produce proper numbers of working blood cells, so they can be infection-prone, anemic, and/or at risk of bleeding. This can range from mild to life-threatening. Side effects from MDS treatments can put patients in further danger. But none of this explains what happened to your mother.

The most common cause of death is from infections, but your mother apparently didn't have one. Fevers not directly related to infections can be associated with leukemia and other cancers, and what they call "fevers of unknown origin" are unfortunately common.

Neither "chromosome involvement" nor 6% blasts is a direct risk. Faulty chromosomes are simply part of the mechanism that leaves people with reduced blood counts. Statistically that means a higher long-term risk, but it's not an imminent danger. Blasts (immature blood cells) can range up to 5% in a healthy person, and RAEB stands for "Refractory Anemia with Excess Blasts", so 6% is at the low end of the "excess" scale.

Did your mother have other significant health problems? Did they evaluate her heart condition? Her low hemoglobin (in between transfusions) might have been putting too high a strain on her system.

[Ann G]

Hi Neil,

Thanks for the response and insight. They did check out her heart and said it was "strong". She had many other health issues and has always been anemic - she had a severe problem with bleeding hemorroids but it was determined that surgery to correct would not be in her best interest due to age, etc. She had been receiving iron infusions for the last year as well to try and decrease the amount of blood transfusions she was given. She seemed to level off and although her HCT was always on the low side, the MDs said her body had just adjusted to that because she was like that for so long. Because of her age, they said a BMT was not an option and they were talking about Dacogin or Vidaza treatment but probably needing to cut it back to 2 or 3 days (and not the standard 5-7 day treatment per month). I've asked for a copy of her final BMB report and hopefully that will give me more answers. I've been reading through some threads but haven't come across anyone in her age group with MDS. If there is anyone out there, I'd love to hear from you. Thanks again.

[Mseth]

Hi Ann,

I am very sorry for your loss.

My mother has MDS. Though she does not have any issues with the heart, her doctor had told us in the initial appointments that we should be watching out for symptoms such as swelling in legs/around eyes and a high pulse rate, all connected with the strain that a low hemoglobin can put on the heart. Shes been anemic for many years and is used to functioning on a low Hb.

Take care of yourself Ann.

[sbk007]

Ann, I'm sorry you had to go through this. Regardless of what that report says it won't explain why. Your Mom was fortunate to live such a long life. If you asked me 20 years ago if I would take 84 I'd of signed right then. I witnessed a similar event with my Dad he too was 84. In his case it was suspected lymphoma but they didn't know till they started testing. That put a strain on him, eventually he got a blood infection. He beat that but then they did more tests and did find a bad lymph node to make the diagnosis but that didn't cause his death he just nose dived after the test. One thing after the other. When we reach a certain age our body just can't fight infections and the MDS combined with her other issues at that age triggered a series of events. I'm sorry. Think about this though. I don't know how old you are but if someone asked you today to sign a note guaranteeing you 84 would you take it?
All the best to you

[Ann G]

I received the final report of my mom's BMB and as you can imagine I don't understand it fully but am hoping to get some feedback from anyone who may have knowledge of this. Under "Karyotype" it reads:

45-47, XX, t(1;15) (q25;q22), add (4) (q21), del (i)(p13), +11, add (11) (p11.2), de 1(11) (q13), -17, -10, +20, del(20) (q11.2q13.3) [cp9]/46,XX[11]
Interpretation: ABNORMAL FEMALE KARYOTYPE, NEAR-DIPLOID, COMPLEX, SEE COMMENT

Comment: Nine cells analyzed are cytogenetically abnormal and demonstrate multiple numerical and structural clonal anomalies in varying combinations as summarized in the above karyotype signature, which is a composite. Among the anomalies present are a reciprocal translocation between the middle long arm of chromosomes 1 and the middle long arm of chromosome 15; additional chromatin on the long arm of chromosome 4 not identifiable by B-banding pattern; a deletion in the proximal short arm of chromosome 9; tirsomy 11 with structural anomalies involoving two of the homologues, including a proximal deletion in the long arm; monosomy 17; and the presence of a 20q-minus anomaly. The remaining cells are cytogenetically normal.
This karyotpye is consistent with a high-grade myelodysplastic syndrome or myeloproliferative disorder other than CML, or with overt AML. In the IPSS-R MDS Cytogenetic Scoring System, this karyotype is in the very poor prognostic subgroup. In the current WHO classification it is presumptive evidence of myelodysplasia in the setting of persistent cytopenia of undetermined origin but in the absence of definitive morphologic criteria and is sufficient for diagnosis of AML with myelodysplasia-related changes when the blast count is 20% or greater. Correlation with clinical and morphologic findings is required for further integration of these results.

I thank anyone for taking the time to read the above and pass along their wisdom and insight.

[Hopeful]

Ann,

I am so sorry for the loss of your mom. Your love for her is so evident.

Your mom had very complex chromosomes, which is not a good thing in the MDS world. Usually patients with this must move rapidly to BMT. I am not sure that Vidaza is effective at stabilizing complex chomosones as it is at reducing blasts.

The crazy thing about MDS is that if you saw someone on the street with the disease, you may not even realize it. They may appear more tired than normal, but that can easily be missed too. MDS was probably slowly weakening your mom's defenses for years, although it was only diagnosed in January. Pneumonia and infections are very hard to recover from when you have an underlying bone marrow failure disease.

You were blessed to have that last good memory with your mom at Disney World. I know that sometimes people struck with terminal diseases will have one last good rally before their body shuts down and perhaps that was hers.

I am so sorry for the loss of your mom but believe her spirit is still surrounding you and the people you love. Take care.

[sbk007]

Anne,
Human cells have 23 pairs of chromosomes giving a total of 46 per cell.

When the Doctor takes the marrow sample a pathologist analyses it and sends a report back to the Doctor.
The report showed 45-47 chromosomes, hence the abnormal Karyotype. The XX just means female. Complexity because a lot of the chromosomes were "messed up" (No one knows why this happens and its not heredity.), that's what all those other things are. Finally it goes on to say it isn't clear what exactly is it is other than MDS with complex cytogenetic because a lot of the chromosomes were involved. Patients with MDS don't always progress to Acute Myeloid Leukemia(AML) or full blown leukemia, sometimes the lack of hemoglobin in the blood is not adequate to keep the organs going and so the body shuts down. Sometimes from internal bleeding, and sometimes from heart problems or other things like infections. I am sorry for your loss and hope you can appreciate that its not easy to make it to 84. Your mom did good.