Need some hope

[thespider]

Hi.  I'm new to this forum.   My husband was diagnosed with myeloma and amyloidosis back in September 2019.  He is having chemo.   He has cardiac amyloids 1.4cm and a smattering in his kidneys and spleen.   He has some nerve damage affecting his mobility caused by amyloids on his nerve endings.   Our real problem is that we keep being given different opinions by different specialists.   The NAC specialist was delighted that my husband had such a huge drop in his reading so early in the treatment and said this was really positive.   The nephrologist  was also quite upbeat and said that the paraproteins were undetectable in the urine which was a really good sign.  However on Monday we saw my husband's myeloma consultant who basically told him that he had a 40% chance of mortality from the amyloidosis in the first six months and that he was never going to feel any better than he does now.  He has quite bad fluid retention versus breathlessness/low blood pressure.   We feel like we are on a rollercoaster and are at our wits end.   Please .... can anyone help?

[Miriam Vered]

Welcome to the forum, sorry to hear what a hard time you and your husband have been having. When treatment for AL amyloidosis is successful, the earliest test which shows improvement is the free light chain concentration, which drops before there is measurable improvement in function of affected organs, before patients start to feel better and before there is any visible change in amyloid deposits in imaging studies like the SAP scan. So it isn't necessarily surprising that he is still feeling unwell despite the free light chain concentration showing a good response.  It's encouraging that the NAC doctor and nephrologist were positive about his treatment response.

I recommend that you contact Darren Foard, the NAC nurse specialist, at 020 7433 2814 or darren.foard@nhs.net. Darren can give expert advice on coping with symptoms, and it is definitely worth discussing your concerns with him. The overall statistics for amyloidosis mortality are of interest to doctors doing clinical studies but are not particularly helpful for patients. Each patient is unique and there are a very wide variety of individual clinical factors that can have a significant effect on individual outcomes. The statistical, overall trends may not necessarily be at all relevant for many individuals.

[AnnR]

Hi
Sorry about your husband's diagnosis. I can't comment on his prognosis but I was diagnosed with cardiac amyloidosis in 2017 and had 7 months of chemo. i have been in remission for 2 years. I do have fluid retention which varies from day to day. I have had to adjust to being short of breath an easily fatigued but still enjoy life. I became very imvolved in helping to run our local community library and this has helped a great deal in giving me something useful to focus on. i also had some counselling to deal with my feelings of loss. Itcan be difficult to come to terms with the diagnosis but I am sure when he finishes chyemo he will start to feel a lot better. Staying positive is key in my book.
All the best
Ann

[Chirpster]

Hi Spider, sorry to hear about your husband’s diagnosis and I appreciate that this is a very difficult time for you both.
In my opinion there are two things that you will come to appreciate as you begin to understand the very complex disease of amyloidosis- everybody’s disease is different and therefore symptoms will vary and you will, at some stage, come across a pessimistic medical professional- with me it was a cardiologist- yours appears to be a haematologist!
I was diagnosed in October 2017 with multiple myeloma (albeit non symptomatic) and systemic amyloidosis with heavy deposits on my spleen, liver and heart the latter being slightly heavier deposits than your husband. I also have involvement in my nervous system and probably a lot of other places!
You don’t mention the type of chemo treatment that your husband is on but if it is Velcade I was on that for eight months and had a lot of issues with water retention, breathing difficulties, sharp short lived pain all over etc. After a few weeks off it I improved dramatically but the while the Velcade bought down my light chains considerably it did not bring me into remission. So I then had eight months on Thalidomide which did bring me into remission and the side effects, for me, were much easier to tolerate. At this point I must re-emphasise that we are all different and what happened to me could well be different for your husband ( to understand the importance and nature of this I recommend that you read Miriam Vered’s post of 8/1/19 it is by far the most succinct explanation that I have seen)
I met a guy at my last visit to the Royal Free who was diagnosed with multiple myeloma and systemic amyloidosis 12 years ago- I would suggest he was in his late 70’s and he looked well. When I was diagnosed I was told that the amyloidosis would shorten my life but no one knows how long I would have lived without it!! I was aiming at 90 years - I have come to accept 89 as an alternative!!!
The road ahead for us all may be difficult at times but there are plenty of reasons to be cheerful and much to hope for.
Best wishes to you both
Chirpster

[Gary]

Hi. Sorry to hear about your husbands diagnosis. As has already been said, we are all different and react differently to all the drugs and chemo.
I was diagnosed in 2007 with systemic AL type Amyloidosis in my heart , kidneys and spleen. I’m currently on my 4th lot of treatment in the 13 year period. As with your husband if it keeps your light chains down and protein leakage low then all is good.
Getting good and correct information about this illness is very hard. A lot of the local consultants have very limited knowledge.
Hopefully as he is on chemo things will improve and steady out.
Hope this helps.
Gary.

[thespider]

To all you lovely people who responded to my post .... thank you so much,  you have given us hope.  My husband saw a cardiologist on Monday who said that his heart is functioning well so that was positive news.   We also spoke to the NAC with regards to the rather negative conversation we had had with the myeloma consultant.   The lovely clinical nurse practitioner told my husband that,  with all due respect,  the myeloma consultant was a myeloma consultant and not an amyloidosis consultant an invited us to contact him again if we have any other questions and concerns.   I shall certainly be following this forum and posting any questions on here.  Thank you all so much .... I may actually sleep tonight 😊

[Annone Butler]

Hi. My husband was diagnosed with Cardiac Amyloid in 2010. His initial treatment (CDT chemotherapy) gave him a good but not complete response. He then had a Stem Cell Transplant in 2012 which put him into remission for almost 7 years. Since then he has taken part in a couple of clinical trials - the last one of which was another Stem Cell Transplant. Over the years his heart function has improved markedly. He leads a pretty normal life. His FLCs are presently stable (although not quite in the normal range). So I hope this provides some encouragement. Things can get better although they may take a little time.

[Rectereend]

hey there thespider. i am really wondering how do you feel now? i can see a while has passed and i'm really interested in your family's situation at the moment. we are in a very similar situation and i can really feel every single of your word written up there. please update as i really want to talk with someone in similar situation. i need some hope too... https://hgh.to/what-is-hgh/

[deBurca]

Hi theSpider,
Sorry I didn't see the original post earlier but in terms of hope all I can say is never loose it. I was diagnosed in October of 2017 with cardiac, renal and GI involvement. I started chemo straight away. By June 2018 I was in a wheelchair as I could only walk a couple of steps due to breathlessness. In August 2018 I was admitted to a hospice. In September 2018 my wife was told to gather friends and family as I only had a couple of days to live. Fortunately with a lot of medical help in the hospice I rallied. A couple of months later I was well enough to go on another treatment and I have continued to progress well, once again able to walk around and not currently in any immediate danger.
Amyloidosis is an insidious disease, all I can say is inform yourself, always ask questions, never be in a situation where you don't understand what is going on. Everyone has a different journey with amyloidosis but treatments and prognosis' are getting better and better. The NAC is a fantastic resource staffed with wonderful and compassionate people, but don't forget that your local medical team and consultants should be just as compassionate and willing themselves to use the expertise available from the NAC.

[Richardpreston]

I am not going to eulogise over the NAC.  All I could do is repeat what the others have said but less eloquently.

I have been remarkably lucky.

First piece of luck. I was diagnosed within 9 months of seeing my GP by a neurologist in Preston.  I never managed to find out why she tested me for amyloid and she has moved on. 

Second piece of luck.  At my first visit to the NAC an incident in the preliminary observations got our relationship off to a flying start.  This was improved by the first consultation with Dr (now Prof) Gilmore.  He made it quite clear that amyloidosis was serious and how we were going to deal with it. Julian recommended we start with Velcade.

Third piece of luck.  The Royal Preston hospital could not give me Velcade (this was 2010) and referred me to The Christe Hospital in Manchester.  Here I was seen by Dr (now Prof) Bloor. - stick with me you medics!  Adrian started me on Velcade..  It didn't go very well and we were soon onto plan B then C….

If you are interested the rest of the story appears in various posts on this forum, on my YouTube channel and website. www.amyloidaction.org.

Why have I bothered to retell this, thespider?  I decided from the start that an illness that I had never heard of was going to kill me.  I shed a few tears (still do) but talked with Kathy my wife,  two sons, family, friends, clients (I had just set up my own management consultancy) and colleagues.  They were all great.  People that know me know I don't want sympathy but I may need help.  Between us all we were going to get me through this one way or another.

I spoke with both of my two consultants Adrian and Julian (some doctors are rigid about more formal modes of address)  independently and then they talked to each other.   We agreed a plan.  I  remember having a laugh with them that two consultants had reached agreement so easily.  This has continued for the last ten years.  Fortunately I did not need them beyond routine monitoring for 7 years after my SCT but when I decided  the time had come for more treatment once again they collaborated. 

thespider, I don’t want to pretend this has always been easy or straightforward.  The amyloidosis and chemo has left me physically challenged and we have had learn to live with a changed lifestyle. 

Most of the doctors have been great.  The only major concern was when one consultant refused to contact the NAC and that was nearly terminal.   That was fairly early in my treatment.  It wouldn’t happen again as we know how to deal with this behaviour.   Even now most new doctors I see are interested me because they don’t see many patients with amyloidosis.  I avoid those who pretend to know all about it. One random GP told me he knew the cure - I didn’t stop long in his surgery.

If you are struggling with your local consultant the have three options 1) try to resolve the issues 2) find another consultant 3) stick with what you have got. 

This is a long post.  I trust Miriam will let it through.   I hope it is helpful to you.  I much prefer talking to writing and am happy to talk whenever you like.  I would like to thank you for spurring me on to write this.  You have given me the opportunity and enthusiasm to work out how to get my blog going - thanks for your unwitting help!

Keep smiling, a smile is always uplifting

Richard

[AnnR]

Like Richard I am lucky that my haematologist liaises effectively when required as does my cardiologist. On occasion when I have been in hospital and had a consultant who thinks they know everything but are providing wrong info I have contacted Dr Cohen directly (have only had to do this twice) and he has provided helpful and accurate advice. I think it is really important to be as informed as possible and encourage your Drs to contact the NAC if they are unsure. I would also like to say that junior Drs are much better at listening and checking things out.

[Javar]

Hope everything will be fine