New member- mother-in-law recently diagnosed with MDS RAEB-2

[Thedbs]

Hello everyone,

I found this site after ages searching on the Internet for information on MDS. My mother-in-law (67 years old) has recently been diagnosed with MDS RAEB-2 just over 3 weeks ago. Initially the consultant diagnosed MPD back in March following a routine blood test at the GP. On first presentation Hb was about 11, WBC 55 and plts 70. Bone marrow showed 7% blasts with no abnormal chromosomes. However, the trephine showed a higher % of abnormal changes, resulting in the change of diagnosis. My background was 20 years in the haematology laboratory so all the terminology actually means something to me and has enabled me to explain the disease and all that will happen much easier to my family and mother-in-law. A second bone marrow has showed an increase in blast to 10%. She hasn't required and blood transfusion as her Hb has only slowly decreased to 9.6 this week. She was placed on hydroxyurea at first diagnosis and this reduced the WCC to around 14 but the platelets have been steadily dropping. After the change of diagnosis to MDS RAEB-2 the consultant has put her on
Azacitadine and she has just completed her first 7 days. She is feeling very tired now, had some bruising and the 'typical welt' at the site of each injection. 3 days post 7th
dose her platelet count dropped to 13 and a planned transfusion was arranged, but a
nose bleed sent her into aew and the transfusion was brought forward 24hrs. This
diagnosis came out of the blue as she was quite well and had no symptoms. At no
pointin all the consultant appointments has life expectancy been mentioned.

Reading all the new diagnosis posts and other posts on this site has really helped in informing the family that the outcome may not be as short term as they first thought. Any advice or experience on Azacitadine treatment would be very welcome as I know it has only recently been approved by NICE and is the new preferred treatment for high risk MDS.

I look forward to receiving any replies and to being an active member of this important and informative forum.

Darren

[Sarah Reakes]

Hi Darren,

So sorry to hear your news but pleased that you have found this Forum. I have only recently joined as I was diagnosed in July with low risk MDS with del (5q) - mine is the first posting on this particular Forum so you can catch up with my story there.

I understand that the drug Azacitidine was given NICE approval recently for use in patients with MDS Intermediate 2, High Risk MDS, AML and CMML and I believe it has had some very successful results. I'm afraid I do not have any personal experience other than what I have read. It must be an asset that you have haematology background as it is such a complex area to understand. We are all learning as we go along.

It's a very worrying time for you all, but this Forum does enable you to communicate with others in the same boat. Also there are the Regional Meetings with contributions from the Medical Professionals and an opportunity to meet other Patients and their families, which I understand are very helpful. I am going to the one in London on 30th September (my first meeting), and the one in Exeter on 11th October, it being the closest to where I live in Yatton near to Bristol/Weston-super-Mare.

You will see from this Forum, where the MDS Centres of Excellence are located as not every hospital has the expertise to treat this particular blood condition, with it being so rare. It can be very isolating for everyone to face such a serious medical diagnosis, and not all the health professionals are geared up to deal with it very sensitively, so to have this Forum to draw on will I am sure, be invaluable to you and your family. I recommend that you read the First Newsletter which will give you a good insight to the Forum which was only formed three years ago. Sophie Wintrich, Patient Liaison, is a wonderful source of information and will help navigate you to whatever information you may need - it's so nice to be able to deal with someone who truly understands what you are going through.

I hope your Mother-in-law responds well to her treatment and remains positive, as it is inevitably a bit of an emotional roller coaster for her, and her family.

Take Care

Sarah

[janetstanford]

Hello Darren
Welcome to the site ,i was also glad when i found it going a bit stir crazy thinking i was the only one out there ..glad you found us we are as i am sure you have noticed we like to chat and share our woes ...as we are all the same but different ...but we may be able to help and support

hear from you soon take care

[Thedbs]

Hi Sarah / Janet

Many thanks for your reply. We all have our fingers crossed that my mother-in-law has a positive reaction to the new treatment. As I said she has only just finished week one. Reading the few posts from others who have started Azacitadine helped her through it regarding the side effects, the main ones she suffered with was the red, tender injection sites, constipation, loss of appetite and severe tiredness. She also has this frustrating cough but this has been around for over a month.

Compared to many, I think she has been lucky since diagnosis as she has not required transfusions as her red cells and Hb have not been affected, yet. Her numbers are starting to drop now, the platelets (now 10) are our main concern.

She is remaining fairly positive as are my wife and sister-in-law although some days are better than others. Her consultant and ward staff at Eastbourne DGH, Sussex have been superb and the fact that i know them personally due to working with each other has been fortunate.

I will have a look at the first newsletter and will contact Sophie if I need further info. I will also try and keep updating so others can benefit from our experiences.

I look forward to speaking to you again soon. Keep well and think positive!

Regards

Darren

[chris]

Hi Darren

Welcome to the Forum and hope you have found some useful threads on here to support you and your family. As Sarah says, what an asset for your family to have you help explain it all. I'm sure I'm not alone in feeling at sea with a disease that can only be "seen" by looking at what to us are meaningless blobs under a microscope. It would be really interesting if you could write an article in layman's terms (!) to describe the processes that occurs in the path lab when we have a whole blood count test. e.g. how much is manual, how much is automated, what scope there might be for interpretation etc. My godson who is now a GP says that in his medical training he and most of his colleagues regarded haematology as akin to the Dark Arts!!

I think there's a whole topic dedicated to Azacitadine so you will get others' experiences there which could be reassuring.

I have educated myself hugely on the following website which has whole "webinars" devoted to MDS topics. I have pasted a link to the topics page and you need to search out ones relating to Treatment for Higher Risk MDS and to those which contain information about Vidaza (Azacitadine). What I have learned is that your Mother-in-law (and her family!) will need to be patient as 6 cycles would seem to be a reasonable time before improvement is seen on this drug. Also the blood counts e.g. platelets and white cells will get worse before they get better as she has already found.

There is also information on there about prognosis but you will understand from your background that median survival times are an average and in the case of MDS are not based on large population numbers because of the rareness of the disease and the relative lack of information: indeed most are based prior to when some of the newer drug therapies became available. I heard on a webinar the other day that an MDS specialist in the States knew of a patient who had been on Azacitadine for 17 years! Well, it's only been available in the UK for less than a year so we have a long way to go to reach that level of understanding in the medical fraternity about using the drug so to that extent, it is still largely experimental here.

Sophie will have lots of information for you but I have a feeling that she might be away at the moment so she will not be her usual efficient self!

Hope things will start to improve very soon.

Best wishes

Chris

https://live.blueskybroadcast.com/bsb/c ... ent=680927

[Thedbs]

Hi Chris

Thanks for your post and the link. It was very interesting reading! And 17 years on azacitidine would be such a result but I have read many papers on this treatment and the comparisons with the "normal" treatment suggests less than 20% of patients go into complete remission but there is no reason why my mother-in-law can't be part of that 20%. I also read that we will not know if it is working until the 3rd or 4th cycle.
As you have requested I shall write a thread explaining in "laymans terms" the process of the blood test and bone marrow tests from the laboratory perspective but I have my 2 year old daughters birthday to get through this afternoon first. If you have any specific questions please let me know and I'll do my best to explain them for you or anyone else. I am still a registered biomedical scientist even though I no longer work in the laboratory, my new role is an account manager/product specialist for a blood transfusion equipment company for the south of England so deal with the staff at Kings and other London hospitals.

Regards

Darren

[Thedbs]

Hi Chris,

As promised this is what happens to your blood sample from the laboratories view point.

Once you have had your sample or samples taken by the nurse or phlebotomist at the clinic, these are sent to the lab for analysis. Each visit you will most probably have 2 or 3 samples taken, one for the full blood count (haemoglobin, White cell, platelets), one for general chemistry (kidney function, liver function, basic blood chemistry) and one for the blood bank (confirm your blood group and check no antibodies have been made since your last blood transfusion). These samples are checked for correct identification and labelled with different sample barcodes for each analyser in each department, Haematology and Blood Transfusion for the full blood count and blood group and antibody checks and Clinical Chemistry for the general chemistry.

Starting in the Haematology department, the full blood count sample is placed on a fully automated analyser and the blood is sampled. There are 3 or 4 main manufacturers who produce these sort of analysers but they all do the same thing. They use various types of liquid reagents, diluents, dyes and counting chambers to count the total number of White cells, red cells, platelets. The haemoglobin level is usually obtained using photometry (intensity of colour at certain wavelengths). Other results found in the full blood count are obtained by various calculations of the different parameters. The latest analysers can also look at, distinguish and count between the 5 main types of White cells found in your blood stream, those being the neutrophils, lymphocytes, monocytes, eosinophils and basophils. They can also alert the lab staff to the prescence of the immature cells, normally found in the bone marrow, in the sample. Once the sample has been processed automatically (most analysers these days can process each sample in about 30 seconds) the laboratory will have a "normal" set of ranges that are automatically accepted and another set of values that require further investigation. These values can be sex specific, clinic specific or ward specific. With MDS the majority of these results with low White counts, low haemoglobins etc would prompt the laboratory to look at a blood film to confirm the results. The staff would look at the different types of cells down the microscope and comment accordingly. These results would then be sent to your consultant along the blood film for him/her to look at and comment on if needed.
A similar system is used in the Clinical Chemistry laboratory. The sample is first centrifuged to separate the red cells from the plasma (the yellow liquid part of blood that has all the chemicals in) then it is placed on large analysers that test the various blood chemistry components. Again this takes only a matter of minutes for each sample.
The blood transfusion department is the laboratory which ensures all the transfusions of blood cells and platelets are suitable for you. They will confirm your blood group and antibody status has not changed (your blood group should always be the same, except in some bone marrow transplant patients where an exact match is not possible). For those of you who have multiple transfusions, your body could make antibodies against the blood you have received - your own bodies reaction to a foreign body just like in infection. Certain antibodies can have unwanted reactions to further transfusions so the laboratory can ensure you receive special antigen free blood. This testing can take a lot longer than the routine haematology or clinical chemistry tests and if special blood is required this has to be ordered from one of the National Blood Centres. Any platelets also have to be ordered from these centres as they are not kept in the routine laboratory stock, so this is why these sort of transfusions are usually planned in advance.
The laboratory staff are highly skilled and qualified people. They have to have a degree in Biomedical Science which is a 3 year full time course, they have to be state registered to the Health Professional Council and prove they are continually keeping up to date with the speciality, but they are usually the forgotten people in hospitals. Without these staff the hospital would not be able to function and the laboratory is open 24hrs a day, 365 days a year.
I hope this has given a little insight to the process of when you have a blood sample taken. If anyone has any questions please post them and i will do my best to answer them.

[janetstanford]

Hi Darren
I have to say this was well explained and clear as i am transfusion dependent and i have r neg d strain the group which can be given to all i have irradiated so all anti bodies are destroyed as i am at risk of graft versus host ....
as an add on to this could you do a 1 about blood groups and the risks of anti bodies ..the life span of hb/blood cells plaetlets white cells ect and normal ranges so to speak as others on the site do seem to be confused and concerned about this subject it may be a time issue but we do not get this explained to us when we visit clinic .... also a laymans meaning of reticulocytes (hope i spelt that right ) this is the 1 that confuses me and the full meaning of escapes me at times

i am sure Chris June Jayne Sarah and all will find this helpful ...Thank you Janet

[Sarah Reakes]

Hi Darren & also Chris/Janet,

Thank you so much for posting this information as it does give us an insight as to the processes involved, even if I don't totally understand it, it gives us a good grounding.

I support Janet's request to you for further information not only specific to Janet's medical situation, but also on a more general basis of understanding of what the normal 'ranges' for men and women are for the various red and white blood counts, platelets etc, as there doesn't seem to be a standard measurement being used between hospitals. Also the special 'values' that you refer to that require further investigation. It does seem to have caused confusion between us all when we seem to be referring to a completely different sets of figures for example for, Haemoglobin and Neutrophils etc, etc, in our postings which naturally adds to the stress of it all. I think we are all desperate to have a good understanding so that we can recognise when our levels have reached that crucial level when medical intervention is required, or we have to adjust our lifestyle accordingly.

My experience to date with the medical profession is that they are reactive rather than proactive in their approach to providing information and explaining things in layman terms, and so if you don't ask the right questions, you don't get given the information which is incredibly isolating and frustrating. There's no doubt that if you have medical/scientific background as you have, it must make a hell of a difference in dealing with the medics.

Please don't feel you have to respond to this request instantly as the last thing we want to do is bombard you with extra work. Just when you get an opportunity of a free moment in between all the pressures of everyday life would be appreciated - we do understand that you must have many demands on you being a busy professional man with a young family.

We are so lucky to have someone like you to draw on, but we certainly don't wish to over do it! Hope your Mother-in-law makes good progress and send her our good wishes.

Hi Chris, many thanks for the link to the Webinars that you gave to Darren which has been incredibly informative, (and of course, time consuming). My husband goes on to bed and then I get on-line, and he's fast asleep by the time I come up. It is something that you can find you are spending so much time on but as I am so anxious to understand as much as I can, its worth the investment of time. You are amazing at the amount of knowledge you have picked up, and the website links, and I really do appreciate you sharing it with us.

We've got visitors from Canada coming today to stay for a couple of days and I have lots to do, so had better get myself organised.

Take care all of you.

Sarah

[Jayne]

Dear All

Darren, thank you very much for sharing your knowledge with us, as everyone agrees it is extrememly helpful and comforting to know what is happening with our blood once it leaves us. I find the whole process amazing. I think the body is a wonderful thing, but the way that we understand it is also amazing.

Thank you
Jayne