3.07.19

After five anxious weeks waiting (should have been 2) I finally received the good news that my Amyloidosis is indeed AL Amyloid and not the hereditary form. With this result I am now free to start chemo and will go in to discuss this next week with the haematologist. Such a relief.

14.06.2019

I went to the hospital today to see the Haematologist hoping they would have the results of my genetic test but unfortunately they were not through yet. The consultant thought that the London consultants would get the results about now and then have a multi disciplinary meeting on Monday 17th June and make a decision about my treatment then so I should hear by the end of this coming week.

Although the results weren’t through I took the time to ask the Haematologist some questions about what I could expect in the coming months. He detailed the drugs I mentioned before and reiterated that they were well tolerated and unlikely to cause me any significant hair loss. These drugs will be given as both injections and by tablet over the course of two weeks with a week off (for good behaviour) between courses, for 4 courses, so 12 weeks in total. He did however go on to say that he thought the best course of treatment was to proceed with a Stem Cell Transplant shortly after finishing the course of chemo. This entails me having a drug to stimulate the production of my stem cells for a few days before they harvest the cells. These stem cells will be taken from a vein, a procedure much like giving blood, but the other blood components will then be returned to me via another drip so they only take the stem cells. They will split the donation in half, keeping the other half of the donation frozen in case I need another transplant in the future.

The next stage of the procedure is the part I am most anxious about and don’t mind admitting that I am very frightened at the prospect of this. This stage necessitates having a high dose of chemotherapy given over a few days which will annihilate my immune system and for this reason I’ll be admitted to hospital for 3 weeks and barrier nursed, (in a clean room, with visitors wearing gowns, masks and gloves), to prevent me from picking up an infection. This week of chemo is intense, my hair will fall out this time, I will have sickness and diarrhea and feel very fatigued – oh joy, I can’t wait! I may need blood transfusions (great, a chance to get some back for a change after many years as a blood donor!) and be at very high risk of contracting an infection, both from external sources and also from those bacteria and viruses which normally lie dormant in your body when you’re healthy – they may well come to visit!!! After the chemo has been given they will follow this up in a couple of days with a transfusion of my stem cells. These little Trojans will then start marching through my body to my bone marrow and start to make new blood cells. My immune system should then start to kick in again but can take up to a couple of years before it’s back to normal. So as much as I love seeing you all, if you have any infection please stay away from me and I mean this in the nicest possible way as I have been overwhelmed by all the lovely generous acts of kindness I have received.

So I am still waiting for the results of the genetic tests and if it is genetic then the treatment will be different. At least now I have an idea of how things will pan out over the next few months if it just AL Amyloidosis. I’m looking forward to getting started as it’s been so long getting to this stage.

18.06.2019

To take my mind off all of this I accepted a job as a film extra. I can’t give any details about what it is, or where it was filmed, but I spent the day yesterday in an isolated barn in the middle of Renfrewshire with a hundred other extras and some well known actors dancing at a ceilidh, which was exhausting but great fun. Not only did we dance Strip the Willow, The Canadian Barn Dance, the Military Two Step and others we did them all at least 5 times for different takes. 13 hours on set and my step count was well above 10,000 but it was a wonderful distraction and I met some lovely folk. I’ll let you know when the series is being released. I need these distractions at the moment and if the opportunity arises for more extras work during my week off chemo I’ll happily do some more. So I’ll update once I know when I start chemo. Bye for now. Ax

29.05.19

Day 1

Well it’s been an interesting few days down in London. Having travelled down on the 6.30am train on Tuesday Alistair and I managed a quick lunch near Hampstead Heath before arriving at the Royal Free hospital in the early afternoon.

The waiting room was busy with many patients from all over the country, many still with their suitcases with them. As this is a National Amyloidosis Centre they receive referrals from all over the UK and abroad and some of my fellow ‘Amyloidees’ on Tuesday were from Liverpool, Worcester and Edinburgh and all here for the same scans and tests as myself.

Having just sat down I was called through for the usual height, weight, blood pressure and O2 saturation followed closely by an ECG and Echo-cardiogram. I was grateful that they allowed my husband in for this (he’s a doctor and used to performing these test himself) and it was reassuring to see him mouthing ‘it’s normal’ over the head of the technician.

Once these tests were out of the way it was time for the usual round of blood samples and then the injection of radioactive iodine tracer. I was slightly perturbed to see this isotope arrive in a metal box with the radioactivity hazard warning on the side and inside the box was a heavy looking metal syringe… and they were going to inject this into me! Can’t say I wasn’t a little concerned by this but the nurse did advise me that you’d get more radiation living in London than I would get in the injection which is supposed to put you at ease – says a lot for the air quality down there. Here’s the science bit about the SAP scan:

Serum amyloid P component (SAP) is a normal protein found in the blood that binds to amyloid deposits. In healthy people there are very small amounts of SAP and this is only present in the bloodstream. In AL amyloidosis patients, in addition to the small quantities of SAP in the blood, there are large quantities of SAP coating the amyloid deposits in the affected organs. The SAP scan is available at the NHS National Amyloidosis Centre, London, and is performed routinely in most patients who are referred there for evaluation of AL amyloidosis.

The SAP scan can show the amount and location of amyloid within the body without the need for invasive biopsies. A small amount of SAP is tagged with a radioactive iodine tracer and is injected into a vein. The tagged SAP then binds to amyloid deposits within the organs of the body. A scan is then performed 6 – 24 hours later to show these deposits and the amount and location of amyloid within the body.

So the SAP scan was booked for 9 am on the following morning so having received a bottle of Potassium iodide to take twice a day for the next few days I was able to leave the hospital. The Potassium iodide can help block radioactive iodine from being absorbed by the thyroid gland, thus protecting this gland from radiation injury. The thyroid gland is the part of the body that is most sensitive to radioactive iodine. What a cocktail of drugs I’ve been getting recently and I’m the girl who never took anything other than Paracetamol up to a few months ago. The whole afternoon was like a well choreographed dance, from one room to another for different tests and hardly a minute to sit down. This is how the NHS should run all its clinics, it’s very impressive!

So London awaited. After a quick pre dinner pasta it was off to see Wicked the Musical. I’m sure I was probably glowing greener than Elphaba the green witch character in the show. I won’t dwell on the musical, suffice to say Alistair and I thought it was one of the most discordant musicals we’d ever heard and were almost tempted to leave at the interval but decided to stay and give it time to redeem itself in the second act. Sadly this didn’t happen and we came away feeling totally underwhelmed.

Day 2

Back to The Royal Free Hospital for round 2. This kicked off promptly with the SAP scan first thing. This scan takes about half an hour and is straightforward although slightly claustrophobic as the scan plate does come down very close over your head but this was short lived and not uncomfortable. The scan was followed by an MRI scan to check for other signs of amyloidosis particularly in the heart. This scan was a longer scan and I was also invited to join a drug study which necessitated some IV drug being injected whilst in the scanner so this added to the time it took, about an hour and a half in total.

Once back in the waiting room I had the longest wait of the whole few days; 45 mins to sit and dwell on ‘what ifs’ or is it worse than expected. It seemed an interminable wait. Eventually we were called in and the results discussed. I have the amyloid deposits in my kidneys, which I knew about, but also in my spleen. Thankfully I do not have any cardiac involvement as this would be more serious. The doctor explained that she wasn’t so concerned about my spleen but obviously we need to try and prevent further kidney damage and allay the production of the free light chains in my blood which are produced in the bone marrow. To do this she suggested that the course of action would be chemotherapy, a combination of Bortezomib, dexamethasone and cyclophosphamide and this could be given in Glasgow, with regular blood samples sent down to the NAC and a repeat SAP scan in a years time. She did mention however that due to the slow onset of my presentation there was a small risk of this being the hereditary form of Amyloidosis and for this she wanted to do a genetic test which would take two weeks to get the results. So now I have an anxious wait to see if this is the hereditary form. Assuming it’s not then the chemotherapy will start fairly soon and I’ll be blogging my way through that – the good, the bad and the ugly which could be an interesting time. I’m trying not to think too much about this at the moment until I have to start the treatment and have been reassured by both the NAC and the Glasgow Haematologists that this is often well tolerated and my hair won’t fall out. So stay tuned for my next update which will be on the 14th June when I next see the Haematologists for my results and treatment schedule.

27.05.2019

I know I’ve not written very much lately but there really hasn’t been a lot to say. I’ve been waiting for the scans down at National Amyloidosis Centre (NAC) at The Royal Free Hospital in London and I now have a date for these and travel down tomorrow and back on Wed evening. I’m not sure I will receive the results whilst I’m down there but will post details once I know exactly which organs are affected. Feeling a bit anxious but trying to remain positive as I’ve been feeling well and hopefully if they’ve caught this early the outcome will be favourable. So more news in a few days.

28.04.19

Today, spurred on by some friends running the London Marathon, I decided that I wanted to go for a run. I haven’t run since Christmas Day when I did the Park run as I was undergoing various tests and was told not to do any strenuous exercise after the biopsies. I decided that it was time to get my trainers on and get out there. After a very slow 2.5 miles I came home and loaded the results on Strava which promptly told me my pace is much slower – thanks Strava but I think I know that 🙂 I felt good afterwards so I took the dogs out for a 3 mile walk. Not for the first time on this journey have I asked myself, ‘have the Doctors really got my diagnosis right?’

I have never really felt unwell, I’ve had a hospital admission for some chest pain (?MI but not thankfully), have a problem with Carpal tunnel syndrome in my right hand, but have had this for 5 years so it may not be connected to the Amyloidosis at all and I’ve always had low blood pressure so have been prone to feeling light headed for years but maybe this Amyloidosis has been at the root of these issues all along. I suppose the SAP scan will help prove this one way or the other. I must say that to look at me you’d think there’s nothing wrong with me and apart from my frothy pee I have no other major symptoms and my kidneys are still filtering my blood well although leaking massive amounts of protein. I feel like a fraud, people comment that I look so well and I certainly feel good. I can’t say that I’m too keen for the doctors to start chemotherapy whilst I am so well and hope that should this disease have been caught in its infancy that there are less aggressive treatments available, although reading the literature this seems unlikely and it’s just wishful thinking on my part. Anyway I will keep doing what exercise I can for as long as I am able as it helps to clear my head and elevates my mood.

Well done to some of my Bellahouston Harriers team mates for such fantastic running today, truly inspiring. It reminds me that the way forward through what is to come will be a marathon in its own right and I’m on the starting line.

Welcome to this blog about my diagnosis and treatment of this rare disease –

AL Amyloidosis.

Amyloidosis – what is it? That’s exactly what I said.

According to the NHS website definition, Amyloidosis is
‘the name for a group of rare, serious conditions caused by a build-up of an abnormal protein called amyloid in organs and tissues throughout the body. ‘

You can read more about this here :-
https://www.nhs.uk/conditions/amyloidosis/

My diagnosis came after many months of investigations for various symptoms which I am happy to share with you. I’d like to be as honest and upbeat as I can about what happens next and think writing this will be good therapy for me whilst keeping you up to date with what’s happening – assuming you want to know! If you don’t like to hear about bodily functions then perhaps you should stop reading now but maybe by sharing my story we may be able to publicise more about this disease and possibly dispel some of the very negative and damaging literature that appears on Dr Google! Don’t get me wrong I know this is serious stuff and it won’t be a walk in the park but having been recently diagnosed I don’t need to know that I may not survive the year as one website liked to inform me. I say ‘we’ as I know I can’t do this alone and would like you to share, comment and ask questions as we go along. I really appreciate how supportive and encouraging you have all been so far and thank you for indulging me in this blog. Now to the nitty gritty of how this all came to pass….

In my case of Renal AL Amyloidosis it’s all about the pee!! Imagine someone drops a spot of washing up liquid in the toilet bowl before you have a wee. You go to the loo and before you flush you notice it’s all frothy (‘it’s frothy man’- remember that advert in the 80’s for Cresta soft drink… maybe not?) Anyway, it was frothy and knowing this was not normal I turned, as you do, to Dr Google for some answers. On various websites there was a suggestion that it could be caused by dehydration, over exercising, remnants of toilet cleaner (in the bowl not in my urine!) and possible protein in the urine. At the time I was exercising a lot, running 3 times a week, walking the dogs and had a reasonably active outdoor job and was prone to not drinking enough so I decided to drink more (water that is not Prosecco!) and wait to see if things improved. After several weeks with no improvement I went to the GP and asked if I should be concerned. He checked my urine with a quick dip stick, pronounced there was protein present and sent me packing with some antibiotics for a urinary tract infection despite me having no other symptoms. The foamy urine continued and I ignored it, thinking it was just one of those things, until some of my routine blood tests I had came back showing I had a low blood albumin (protein) level indicating that I was losing protein through my kidneys. The GP sent a urine specimen to the lab for testing which showed massive amounts of protein in my urine. I’m hoping I haven’t lost you all by now – I know this is very boring so not much more on the pee stuff. The normal range for protein in urine is less than 20mg/dl and mine was greater than 984mg/dl, basically off the scale so the investigations started. This was Dec 2018.

Now, some 5 months later, following various blood tests, CT scans, ultrasounds, a bone marrow biopsy and a kidney biopsy I finally have a diagnosis and it’s pretty rare, AL (this stands for Amyloid light chain) Amyloidosis. Here’s a little video of what this is.

Having met with the Renal consultant on Wednesday 24th April 2019 he explained that as this was fairly rare (trust me to have something uncommon) I was to be referred to The Royal Free Hospital in London for a specialised scan, a whole body SAP scintigraphy scan to see if the amyloid deposits are in any other organs of my body.

Now I’m waiting for an appointment to go to The Old Smoke to get this done which should be in the next few weeks. Hopefully whilst I’m down there I’ll be able to catch a show – recommendations are very welcome. In addition to these suggestions I’d like to compile a play list of tunes to while away the hours I will be hanging around waiting for scans and treatment so I’d really like your help, I’m fed up listening to my own playlists. Your choice can be any tune you love, maybe it’s uplifting, soulful, catchy, funky and up beat (I like a good dance as many of you know) and from any musical genre as I have very eclectic taste. It will be interesting to hear what you come up with and I promise to listen to them all. This should be interesting 🙂 . So that’s it for now. Please feel free to share this blog with whomever you choose.

Over and out for now

Anne xx

So don’t ignore frothy urine…

Thanks for joining me!

Good company in a journey makes the way seem shorter. — Izaak Walton