Newbie

[Wychwoodbabe]

Hi

I have been a 'browser' here for a while but never posted for personal reasons.

I am 64 (65 next week) and was 32 when I was diagnosed with ET. I think I must have had every treatment available in the last 32 years; radio active phosphorus injections, interferon, Hydrea, Busulphan, Anagrelide, aspirin and clopidogrel to name but a few. I also have an underactive thyroid and take Thyroxine for that.

During these 32 years I have had a very full, active life. Raised two kids (and now look after 4 grandchildren), worked full time and lived a near normal life if a bit marred by leg and foot ulcers, fatigue, bone ache and all the usual aches and pains associated with the necessary treatments and this condition.

Last September I progressed to myelofibrosis and was prescribed Ruxolitinib with Aciclovir to prevent shingles. I also take Anagrelide still to control my very naughty platelets that soar at the most inconvenient times but are always quite high.

I have some very 'funny' tummies, naughty wind and weight gain with this new drug regime but it seems to be helping with the bone pains.

My reason for now eventually posting on this wonderful site is the recent visits to my haematology unit where I seem to be being given conflicting advice as to the next stages.

One consultant wants to 'wait and see' what the new treatment brings in terms of life expectancy and life quality; two others want me to have a stem cell/bone marrow transplant asap. I have a brother who is 60, who was tested years ago and is 100% match but has high blood pressure so they are a bit negative about using him.

I was appalled last week when one consultant told me my life expectancy according to the Dipps scoring would drop to 6-37 months after I reach the age of 65 which is next week! I had been told maybe 18 months to 5 years just two weeks previously. I am having blood cross matched at the moment and have another bone marrow aspiration booked for next week to cross type me to go on the register.

After having years of treatment for my blood problems I do find now that the narrowing down of life expectancy a bit scary and the Dipps scoring quite bizarre.

I understand that we all want to live a good, long life but have first hand experience when a very good friend of mine didn't survive her bone marrow transplant so am armed with a little knowledge of the procedure which is always a dangerous thing! My home life is complicated and I am very busy with the family but seem to be coping well at the moment except for the fatigue so why would I want to undergo a transplant with all it's worries?

Anyone else out there in a similar situation? If so I would so love to hear from you.

Kind Regards

[eire]

What a dilemma for you. Sometimes I feel it's our grandchildren that keep us going. I'm 65 was diagnosed with MF just over 7 years ago life expectancy was never discussed with me and I've never asked. I feel we are all so different and have our own strengths I intend to not dwell on the what ifs or maybes as long as I'm coping well that's good enough. I was advised on a BMT when I was first diagnosed but it wasn't for me. I was also advised at the time that the older I got the higher the risk would be with a transplant. I still refused then was put on a trial 3 years ago taking rux and panobinistat - plenty of funny tummy and naught wind (love that one!!!) troubles but am still leading a very full life walking my dog and like yourself looking after my gr daughter. Honestly no body can tell you the best way to go its a VERY personal decision and would be very different for each one of us. I'm so sorry your friend lost her life that would be foremost in your mind but so many other people do survive. If I was you I'd get all the information you can together maybe there is someone in your position in your local hospital who may share experiences with you???? I would then discuss everything with your family who will play a major part if you decide to go ahead. Don't forget at the moment this is an idea in the pipeline you don't have to rush into it. I wish you all the very best making your decision and in the meantime just carry on enjoying your life to the max!!!!!! Happy birthday for next week special birthday needs to be celebrated!!!!! Best wishes.

[Ednama in reply to eire]

My husband was diagnosed with MF when he was 62, but also with Myleodisprasia. The MDS then mutated and with no treatment and only life expectancy of 12 months decided to go ahead with the SCT. I can only say this has been a traumatic time, 11weeks in hospital with many life threatening infections which left him with kidney failure and is now dependant on dialysis. Further infections down the line and hospital admissions also. There must be many people who sail through SCT and without it my husband wouldn't be here and we are thankful for each day, but a SCT is a massive undertaking which needs very careful consideration. I wish you all the luck in the world in the coming months.

[beetle]

Hi. Been where you are a couple of years ago. I had ET for about 18 years before progressing to MF and had also tried many different drugs. When I was 63 I was advised to look into BMT. My haematologist likened my position to standing on the edge of a cliff and I could either jump and hope the parachute would open (successful BMT) or wait on the cliff for the wind to blow me off later. When I presented that analogy to the transplant team the doctor told me I would be jumping with a faulty parachute! I don't have other comorbidities and look much younger than my age. I asked if she would recommend BMT if I was her mother and she gave an emphatic no. That was enough for me! I no longer agonised over the decision and have been on Ruxolitinib for three years now. My DIPPS score is not good but I am leading a relatively normal life if you discount all the usual disease symptoms. I may go into a drug trial or maybe not. I'm not sure how I feel about jumping with a parachute that nobody knows much about either!

I really wish you the best of luck with your decision. It is a very personal one but I hope my experience is of use to you.

Very best wishes, Jan

[darcey]

What A choice ......but only you can decide what to do. I am a 65 years old woman.

I had ET for 12 years and seem to be quite happy jogging along with hydroxycarbamide and asprin for ten years along my regular hospital visits and lots of blood tests.

I I had an operation three years ago and lost a lot of blood I was unwell, I had to have over six units of blood over two days? It took me a good few months to recover form this op. I had huge weight loss and was very breathless and fatigued.

After a BMB was taken I was told I had an enlarged spleen I was having hot flushes & sweats.

I was told I was now have MF at ....intermediate 2. I was seen by the transplant team too all a huge shock. They have found me two 100% matches, but the whole procedure is very scary and as you say not always successful. But I was told to wait and see....

I was put on Ruxolitinib for two years. This worked very well at first but then I got terrible fatigue. I had to have blood transfusions regularly and iron infusions as I got very anaemic on this. I also had B12 injection and took supplements.

I was put taken off the Ruxolitinib and put forward for a new trail drug called Momelotinib.....I have been on this for six months. I have not had a blood transfusion since last August. I have been so well in fact ...just like my old self.

The new drug has been working very well up to now, But i have an enlarged spleen again and the hot sweats again at night, and some bone pain. I return to the hospital in two weeks, I hope we don't start talking transplant again.....watch this space & wait & see.

Be strong x

[ourlife]

Hi,

Thank you for posting your position, You will give hope to many as living proof that you can live 30 years + without/before progressing to anything sinister. I was 30 when told I had ET and my Dr (who was about to retire ) told me. "I give you 30 years if the long term use of meds doesn't get you your condition will" Registering at stage 1 on the fibrosis scale last year and now progressing to PV after 16 yrs has realy shook me, I think of the future and dread MF.

Trust your instincts and good luck for the future,

Ourlife

[crapaud]

I personally had a SCT last year (following a MDS+MF overlap type syndrom) and for the moment things are going well.

However, and with all respect, I do not have your age and have not been a long term sufferer.

My personal advice would be, if you don't feel right going for a BMT then unless the 'gun is against your temple' don't do it. I strongly believe that a significant part of the BMT process is based on your willpower and feeling up to the battle - even though of course this is not always sufficient.

Without a crystal ball there's no right/wrong decision.

Do what your heart tells you and then move on.

Good luck

[Wychwoodbabe]

Thank you all so much for your replies; it is so lovely to have people who have the same problems and someone to talk to - I have spent 32 years and never really met anyone with ET/PV - not many people know that I have the condition anyway as we all look so normal so maybe I have but like me they try and ignore it!

I have a full and busy life - in the Lake District last weekend ( cant walk very far but husband did and I shopped) , Centreparcs this weekend with all the kids and grandchildren ( and I have hired a bike!) and Wales next weekend before 5 days in Spain for a boozy long weekend with 9 girlfriends. We have been going away for over 10 years and all of us have been touched with chronic illness in some way or another but we still have a hoot and a great time but now all go to bed a little earlier and do not hit the clubs at 2am like we used to.

For anyone starting out in this condition please note it is not a death sentence - I am living proof after 32 years of it. Please don't worry re the future Ourlife - what will be will be.

Eire - I do the school run every day with my grandchildren so know what you mean re keeping us going - not allowed any lie ins at my house!

I will weigh up all the pros and cons and for those of you who have experienced a BMT I am so pleased for you Crapaud. and so hope Ednama that your husband improves.

Beetle , I love the parachute analogy and do tend to agree with it at my age!

Darcey - I have not heard of Momelotinib - I hope all goes well with your next appointment.

I will get the hang of this web site soon methinks.

Let's all hope that the medics come up with something wicked and wonderful to cure us all asap!

Kind Regards

Jan