Category Archives: azathioprine

The Aza Conundrum

For nine years between (1999 to 2008), taking Azathioprine (Imuran) in varying doses between 150mg to 200mg/day successfully kept surgery at bay. Any Crohn’s flare-ups were dealt with by short courses of steroids. Then a series of routine blood tests showed that my platelets were dropping and it was concluded that Azathioprine was the most likely cause. I stopped taking it and within 2 years was undergoing major surgery.
I drew a graph to try and spot any correalation between the drug dose and the platelet count. I was expecting to see the count bounce back once I stopped taking Azathioprine and it did so the first time but when I started/stopped for the second time the platelets remained low. I’m guessing at that point the bone marrow was already damages. The only way to investigate further was to have a bone marrow biopsy.


In 2012 I went to see a haematologist and she explained some possible causes of a low platelet count :

increased destruction – the body is producing sufficient but something is destroying a number of them, possibly drug induced

decreased production – the body isn’t producing the right number in the first place which could be down to bone marrow failure.

We also discussed another factor – the implication of my enlarged spleen. Enlarged spleens can hold increased numbers of platelets and therefore the number released into the bloodstream is lower hence the lower count.

I had the bone marrow biopsy and afterwards received an email saying: “your bone marrow is being discussed with the histopathologist and we will write to you with the results. We will see you in clinic later in the year.” (I had to look up histopathologist – someone who carries out microscopic examination of tissue in order to study the manifestations of disease.)

I replied asking for an indication of what they had found. The response was that it would be easier to discuss the findings in clinic.
What did that mean? Nothing to worry about, it can wait, or it’s serious and we want to tell you face to face? Time for another short email along the lines “…I wonder if you could at least put my mind at rest that you haven’t found anything too serious….”

Within a few minutes this came back :
“We have reviewed your bone marrow in our multi-disciplinary meeting and there is nothing sinister to report. The findings suggest that your marrow is underproducing platelets rather than it being an immune cause that we had presumed secondary to your longstanding history of Crohn’s. This may be due to previous azathioprine use. We can discuss this in person and in more detail at your next appointment. In the meantime – I hope this reassures you.”

The appointment duly arrived. The haematologist started our conversation with: “Yours is not a simple case…..”. She had printed out the biopsy report that had been discussed at their MDM and the initial conclusion was that they were “in keeping with early/low myelodysplastic syndrome, histologically suggesting MDS-RCMD.” She knew that I would have looked this up on the internet and would have spotted the potential links with leukemia. That’s why the report hadn’t been emailed to me. [If I had Googled MDS I would have found the following – “The disease course is highly variable, from indolent to aggressive with swift progression to acute myeloid leukaemia (AML) in 30% of cases.” I think she was right to want to discuss it in person.]

She was not completely happy with this MDS conclusion because a bone marrow biopsy looks at two substances – the marrow itself and the aspirate (fluid). When the procedure was carried out the doctor was unable to obtain good aspirate slides as the blood in the samples kept clotting. After several attempts, but with little success, they had decided to concentrate on obtaining a good bone marrow core.

She described it as “like having a three piece jigsaw from which two of the pieces are missing.” At the next MDM they had discussed the results again and decided that, in my case, it was unlikely to be MDS but would recommend a further biopsy to get useable aspirate samples. “How would you feel about this?” I replied that I really wasn’t fussed. If it would help narrow down the diagnosis then the sooner the better. Next time they would use heparin, a blood thinner, with the sample needle as it should prevent the blood from clotting.

If the diagnosis wasn’t MDS then why the low platelets? The most likely cause was a combination of long-term Crohn’s and taking azathioprine. The biopsy had shown that the marrow was under-producing platelets rather than being over active and eating them up. I had been unaware that there was a potential link between Crohn’s and bone marrow.

The MDM had then gone on to discuss what the implications for treatment would be if it was/was not MDS. In either case the preferred course for treatment, at this stage, would be “do nothing” unless I was to have any procedures that could cause bleeding or that required surgery. A supply of platelets should be made available if either of these were needed. The difference in approaches would be in the monitoring regimes and we would discuss this further after the next biopsy results were available.

Back to reception to book up another biopsy and a three-month follow-up appointment.

In the meantime I had a routine gastroenterology appointment and I mentioned the need for a second bone marrow biopsy. Now you would think that a doctor who doesn’t bat an eyelid when sticking a camera up a patient would be pretty much hardened to all medical procedures, but the mere mention of the bone marrow biopsy was enough to make him squirm. He asked me if I was OK having the biopsy as it was the one test he really wouldn’t want to undergo himself! Strangely enough he wasn’t the first person to express that emotion.

A couple of weeks after the second biopsy I was back to see Haematology. When I went in for the pre-appointment blood test the phlebotomist asked me if I knew why she was also taking an “histological” sample. Since I didn’t know what “histological” meant I was of little help. (Of course I know now! It’s the anatomical study of the microscopic structure of animal and plant tissues).

The haematologist explained that one of the biopsy samples, which should have gone for histological testing, had either been mislaid or mislabelled so did not make it. This is why she had rung me a few weeks back to explain the situation. I’d forgotten about this. She had, however, looked at the other slides from that second biopsy and these were fine.

The missing sample had been discussed with the chief histologist and he suggested doing a specific type of blood test which had proved to be 60% effective in spotting problems, if there were any. The results would be available in a week’s time. The alternative was to have a third bone marrow biopsy but they didn’t want to put me through that again. I suppose I could have made a fuss about the missing slide but I couldn’t see what good it would do.

CURRENT SITUATION
When I saw the haematologist in February 2015 she described my bone marrow as being “a four cylinder engine running on only three” and therefore not delivering the right quantities of platelets.
What is the long-term prognosis for the thrombocytopenia? It should not affect the other issues I have – Crohn’s, potential PSC, PVT, but I must avoid the use of azathioprine in the future. It’s important not to get hung up on the numbers as I am asymptomatic and do not bleed profusely if I cut myself.

What could have caused the low platelets? There are no signs of marrow abnormalities that could point to a more sinister conclusion (leukaemia), therefore the cause is most likely to be drug-induced long-term use of azathioprine. The official description was “asympomatic thrombocytopenia. Therapy related secondary dysplasia on bone marrow morphology – most likely due to Azathioprine”.

Do I need treatment? No, but must look out for any signs of starting to bleed more easily. Monitoring? Six-monthly blood tests and outpatient appointments (which subsequently became annual and then dischargeded).

WHAT NEXT?
A couple of new issues have arisen – borderline thyroid level + possibility of cholecystectomy – so it seemed like a good idea to book another appointment with Haematology to discuss further. Watch this space

Fifty Shades of Grey

Let’s get my latest appointment out the way…….

Monday – 24th April 2017 – Gastro Appointment, Guy’s Hospital

I hadn’t planned this appointment, neither had my gastro consultant but the booking system had other ideas. It must be set to auto repeat every 6 months and doesn’t take into account any ad-hoc appointments in between. I had intended to cancel but I’m pleased I didn’t as there were things that needed talking through. I produced the obligatory list of questions (responses in red) :

1.    Biopsy results (from 11th March colonoscopy) – the report from the path lab said that the biopsies were consistent with “quiescent” Crohn’s disease. This result was about as good as it could get. Once you have the disease there will always be some signs of it, even when in remission.

2.    Explanation of rising calpro levels given result of recent colonoscopy?       – to be honest, he simply did not know what was causing the raised calpro levels. He had been concerned that something had been missed during a previous colonoscopy hence the repeat, in March, carried out by his trusted colleague (and watched by an audience of trainee, international gastroenterologists).

3.    If calprotectin tests not giving meaningful pointer to Crohn’s activity what monitoring regime should we adopt? – I had anticipated what the answer would be and I was right. If you start to feel the Crohn’s is becoming active then we’ll take it from there.

4.    The upper GI surgeon (Professor), who I saw locally (see previous post) regarding gallbladder removal, was talking about referral to a specialist liver facility “in case of needing a transplant” arising from complications during the  cholecystectomy (sounded very drastic) – my gastro agreed that I should be referred to a specialist unit in view of my concurrent conditions. The most likely unit would be the one at Kings College Hospital. The issue of needing a transplant would be a last resort if something went very wrong during the operation. He typed a letter to the Professor suggesting that the referral should go ahead.

5.    Awaiting ultrasound appointment (locally) to look at liver, gallbladder, bile duct and portal vein – noted. No date as yet.

6.    Pros and cons of having gallbladder removed? – to be discussed with specialist liver facility. Even if I decide not to have surgery I would at least be on their radar so that should I end up having another jaundice incident, that needed urgent resolution, they would already be aware of my case.

7.    Fibro-scan to see if liver cirrhosis progressing – he filled in the online booking form to request the scan. (Date now through – 4th September)

8.    Current weight 78.2kg. The target weight set prior to my ileostomy (October 2010) was to get UP to 90kg, which I achieved with the aid of 3 x Fortisip (300 calories each) per day. My subsequent decline by 12kg has been quite a loss – whilst I felt fit at this reduced weight it was a lot lighter than the previous target weight. I thought I had better point it out. We would continue to monitor.

9.    Next steps – ultrasound scan; fibro-scan; no further colonoscopies at present; follow-up appointment in 6 months time (the booking system should already be doing that); yearly endoscopy at Christmas to check varices + appointment with specialist liver unit.

50 Shades of Grey

For 30 years I really didn’t want to delve too deeply into my health. It was clear, black and white, I had Crohn’s Disease (after the usual “is it IBS debate” within the medical profession). It was centred mainly around the join between my small and large intestines (a common location) and had caused a stricture. Despite this I spent many years in remission.

In the last few years my medical life has become more complex with new issues arising. Most of them  are very definitely not black or white.

It started with the dramatic fall in my platelet count that has never recovered (thrombocytopenia). Was it really as a side effect of the Azathioprine I had been taking for 8 years? You would expect it to have bounced back when I stopped the drug. Is it related to my spleen becoming enlarged? Could this be the cause of the platelets issue instead? Two bone marrow biopsies later and there is still no definitive answer.

Next there was the incident where new blood vessels had grown in my esophagus and then burst. A subsequent x-ray showed a blood clot had formed in my portal vein (thrombosis) which had increased the pressure in the veins higher up. Most likely cause of the clot? The current theory is it’s the result of peritonitis following a perforated bowel operation in….1979! Really? That long ago? Apparently there is always a risk of PVT during any surgery. I’ve also seen research that once you have Crohn’s patients you are more susceptible to clots.

As a result of the above incident it was suggested that I might have Primary Sclerosing Cholangitis (PSC) I had a fibro-scan on my liver which showed signs of cirrhosis. What caused that? It certinly wasn’t alcohol related as I drink very little. Is it linked to that blood clot? I then had a liver biopsy and, thankfully, it showed no PSC.

What caused my recent jaundice incident last January? I felt no pain whatsoever only violent shivering and turning yellow. It must have been gallstone related but this is usually accompanied by the most excruciating pain. Again there is a potentially a link between Crohn’s and the increased likelihood of developing gallstones.

…and so to my latest consultation. Yet another puzzle – how to explain a rising calprotectin level with a colonoscopy, and biopsies, that showed I’m in remission.

…and not forgetting the reason I had that second colonoscopy – to see if there was any evidence of the strictures which showed up on the MRI scan, which there wasn’t. Another conundrum and one that had also happened back in 2012.

…and, of course, there’s the biggest grey area in the room – what causes Crohn’s Disease?

I’m not going to lose any sleep over the above. What’s done is done. It’s more out of curiosity that I would like definitive answers. In an ideal world I’d get a gastroenterologist, a hepatologist and a haematologist in a room together and let them reach a concensus on likely causes. That isn’t going to happen anytime soon…….

…but maybe the combination of conditions would at least give me a winning hand playing “Illness Top Trumps”

 

Do you mind an audience?

Gastro Appointment – Guy’s Hospital – 20th February 2017

I knew this was going to be an “interesting” consultation and it even started in a strange way. Would you expect to be greeted by a live violinist in the waiting room? Whilst I applaud the hospital for trying something different I’m not sure what it did for other patient’s stress levels. It didn’t help mine.

Having been waiting for over an hour a nurse appeared and announced the clinic was running 90 minutes late. Maybe she had made an earlier announcement but was drowned out by the violin. I knew I would be in for an even longer wait as I had requested to see my usual Consultant.

When I was finally shown into his room, he apologised for the delay and we started working through my list.

1 – Calprotectin result – previously 512. Had now risen to 895. I thought this was not unexpected as I was starting to feel a certain amount of pain when food passed across my anastomosis and through the transverse colon.

2 – Dependent upon the above – have you discussed further investigation? Barium enema? We had agreed before Christmas that, dependent upon the calprotectin result, further investigation could be needed. He favoured doing another colonoscopy.

3 – Run through the last follow-up letter with translation. What are implications of fistulas and adhesions?  We went through the letter and made sure I understood the medical terms. I was concerned that the mention of fistulas, strictures and adhesions meant only one thing – surgery. He responded that the possibility of fistulas was the most concerning; adhesions were to be expected but he was still was struggling to understand the apparent differences between the MRI and what he had physically seen during the colonoscopy. Strictures should have appeared on the camera.

I asked if it was possible for the Crohn’s to have moved from my small intestine to my colon. He said that it did not usually happen. A repeat colonoscopy would look for this. He asked if I minded having an audience as they were running a visit for ten overseas gastroenterologists to show how endoscopies were carried out at St.Thomas’. I really wasn’t fussed and it meant that I had the date set there and then. (Wonder if they will film it for YouTube. Would be taking selfies to another level).

4 – Plan for treatment – start Crohn’s medications. The most likely treatment would be one of the “MABs”. We discussed my previous experience with Infliximab and that was duly noted on my medical file. I wondered if I ended up needing regular infusions whether these could be carried out locally rather than needing a trip to London each time. He said they would encourage that but would still keep control of my case.

5 – Recent trip to A&E with jaundice. Violent shivering. Nausea. Turning yellow. Ultrasound scan 21st February. Need to make sure results are passed on. I quickly ran through my recent trip to our local A&E. He was surprised that during the whole incident I felt no pain. I mentioned I would be having an ultrasound scan the following day. (See below)

6 – Did East Surrey liaise with St. Thomas’? Did blood test results get passed over from East Surrey? There had been no contact with East Surrey. Something for me to chase up when I went there for the ultrasound.

7 – Hb looked low to me. He was not concerned about my Hb

8 – Do the treatment pathways change with age ie. over 60. Have any studies been done into the needs of the “older” patient? The main consideration would be the type of drugs used and their effect on an immune system that weakens with age.

9 – Opportunities for doing some more public speaking. Taking year off of work, maybe longer. There were plenty of opportunities. The danger would be becoming overused! I explained that I wanted to do something that would help the cause of Crohn’s patients.

10 – Not felt well for last 2 days. ED. Taking more Loperamide to try and combat. Have any patients reported that Loperamide from different manufacturers having varying levels of efficacy? I had been suffering bouts of having to rush off to the bathroom and it was the uncertaintity of the cause which I struggled with – virus, crohn’s, BAM or dodgy food. He suggested that I should go and see my GP to arrange a prescription for Questran (a bile acid sequestrant) so that it was available should I decide to start taking it. I had wondered if it was possible that different Loperamide makes could be causing my present problem? This rang a bell. He suggested I put it to the test by using the different makes in turn and noting the outcome.

I then went off to find the Endoscopy section to try and pick up the colonoscopy prep but would first need a time and date for the procedure. After a lot of ringing around the very tenacious nurse managed to get it all sorted out. Colonoscopy planned for 10:00am Saturday 11th March. The Endoscopy Unit were currently reviewing how the prep would be dispensed so I was given a prescription to take down to the Outpatient Pharmacy.

Roll on 11th March……

Ultrasound Scan – East Surrey Hospital – 21st February 2017

In complete contrast to yesterday’s delays, I arrived at the Imaging Unit early, waited five minutes and was then shown into the ultrasound suite.

They had the luxury of warmed lubricating gel! The scan took around 10 minutes during which I discussed with the sonographer what I would expect her to see – a large gallstone (first seen in 2014) and an enlarged spleen. At first the gallstone wasn’t apparent but when she applied the scanning head from a different position it appeared, except it was now a group of small stones. She wanted to see if they were mobile so got me to stand next to the US unit and then jump up and down. (I’m pleased they don’t get you to do this during a colonoscopy.) The stones had moved to the bottom of the gallbladder. The whole procedure was completed before my due appointment time.

I mentioned that I needed to get a copy of the report sent to my consultant at St.Thomas’. The sonographer asked me to return to waiting area and she would print off a copy of the report for me to take away.

Old Dog, New Tricks

A post because of Crohn’s, not about it.

Whatever else you can say about Crohn’s Disease it certainly does give you the chance of new experiences, mostly unpleasant, to be honest. I won’t list the nasty ones here as they are covered in the video at the end of this post. I thought I’d record how I dealt with this opportunity in case others get a similar chance to raise awareness of IBD.

It’s something I’d wanted to do for a while. I suppse it stems from a reawakening of the “performing” instinct that first showed itself when I was  in a band. That was around the time I was diagnosed with Crohn’s.

mav_lak_2In this instance I really wasn’t sure what to expect. A fellow patient at St. Thomas’ Hospital was due to talk to some undergraduate nurses, about “Living with IBD”, but then found that they were double booked that day. Would I step in and do it instead? Of course I would, after all how difficult would it be to talk to a few nurses? The date was set for 5 weeks time.

I wouldn’t need any preparation. I’d lived with IBD long enough to write a book. I would just turn up and talk, or so I thought. The last thing I wanted was to read from a script but, after some more thinking, decided the least I should list out all the topics that needed discussing.

Years ago I dismissed mind maps as more “management clap trap” and then actually drew one and have been sold on them ever since. It would help clarify my thinking. Here’s what I came up with :

mind_mapAt this point  I found out that there would be around 200 nurses, in a proper lecture theatre and  I would be talking at the end of the afternoon. It dawned on me that to do the subject justice, and not short change the nurses, I would at least need some notes and something to keep everyone awake. I tried doing a run through, just using notes, and it was terrible – stilted, hesitant, repetitive…..  I would have to write the talk out word-for-word, the very thing I didn’t want to do.

I find that simply reading through what I have written doesn’t pick up the likes of over used words or even ones that are missing. Much better to hear it being read. I found that the software I use has the facility to convert the text to speech and save it as an audio file in iTunes. I can then listen to it on my iPod.

After several iterations, including two read throughs to my wife, I was finally happy with the contents. Maybe if I then listened to it endlessly it would become engrained in my memory and I would not need notes.

After half-a-dozen listenings it hadn’t worked. I would have to work from a script after all…..

When I got to the theatre, with a real live audience, it suddenly became a lot easier. I did use the notes but just to make sure I didn’t forget anything (which I still did). I had taken a small camera with me but unfortunately didn’t get there in time to set it up properly so the sound wasn’t brilliant.

The resulting video was rather long, all in one go, so I’ve split it into three parts. Of the three I think that the second one covering surgery and stomas is the most representative. I’ll let you judge the result.

Kings College Hospital, Lecture Theatre

DIARY – Crohn’s, blood clots and low platelets

September 2013

Medically things are going OK-ish. I don’t want to tempt fate by putting it any more strongly than that.  I still have a sporadic, niggly pain around the site of my anastomosis (the bit where they joined my gut back together again in June 2011). This has been ongoing for quite a while and gets worse when I’ve been doing a lot of physical work, worn a belt too tightly or have been on my feet for a long time. My consultant thinks it’s purely mechanical and nothing to worry about.

Between now and Christmas I have four outpatient appointments and will have a chance to practice what I preach using my 8 point approach to “Managing Consultants and Appointments”.

(1) Making a List
(2) Manage Your Appointments
(3) Continuity
(4) Medical History (including copies of any recent emails)
(5) Contacting your consultant between appointments
(6) Follow-up letters
(7) Manage Your Appointments 2
(8) Keep a sense of humour

(There’s a separate post which goes into the detail if you are interested. It should be obvious which one it is – the clue is in its title).

Wednesday 25th September 2013 – Haematology Appointment – Guy’s

 The Background

This was to have been the sign-off appointment where I got handed back to gastroenterology. The “elephant in the room” however is thrombocytopenia – the relative decrease of platelets in the blood. A normal value is anywhere between 150 and 400, I’ve been well below 100 (hovering around the 60 mark) for quite a while now but not exhibited any obvious adverse effects such as bleeding profusely if I cut myself. We had decided to park the issue but when I re-read my notes from the last gastro appointment, and the subsequent follow-up letter (6) , they said that my GI would not put me on a maintenance dose of Azathioprine because it may have caused the thrombocytopenia.

That meant one less route available to me should (when) I need to start drug therapy again to control the Crohn’s. In my last post I mentioned that I would email my GI consultant (5) to ask if he was happy that this issue would remain unresolved. I sent the following and copied in to the haematologist :

“I have my next haematology appointment on Wednesday week. At the last one it was suggested that I could then be discharged back to your care, as we have done with hepatology. The fewer clinics I have to attend the better but I have one question for you regarding my thrombocytopenia from a Crohn’s standpoint.

Because I am asymptomatic and there are many possible causes of the low platelets we have “parked” further investigation. When I saw you at the end of June, we discussed Crohn’s remission and the use of Azathioprine as a maintenance drug but you said you would not want to prescribe it because of the low platelets. Will the non-resolution of the platelets preclude the use of any other potential medications that might be needed if my Crohn’s starts to deteriorate? Is Azathioprine ruled out by low platelets regardless of the cause?”

Early the next morning I received a response from the haematologist :

“Having read this email – I think we do need to go ahead and finally do the bone marrow test to see if we can be  more definitive about the cause for your low platelet count as this is having an impact on your treatment options. We can discuss it when I see you on Wednesday.”

The Appointment

For haematology appointments it’s worth turning up half an hour early as they always take a blood sample and have the results available during your consultation. I booked in and then took a seat. Within 5 minutes I was called by the phlebotomist and had a blood sample taken. It was then back to the seating area, ready for a long wait. After another 10 minutes I heard my name being called. A doctor I hadn’t seen before introduced herself and apologised for keeping me waiting even though I was being seen 15 minutes early. My usual consultant was on holiday so no point in asking “for continuity” (3) and anyway I had already decided that I would be happy to be seen by whichever doctor I was allocated.

As we entered the consulting room I showed her my list (1)(6) and explained I had a few questions to ask. She had started reading my notes but they didn’t include a copy of the recent email correspondence so she was unaware that I was to have a bone marrow biopsy. Luckily I had a copy of the emails on my phone so she was able to read them for herself.

We went through the causes of low platelet counts – increased destruction ie. the body is producing sufficient platelets but something is destroying a number of them, possibly drug induced; or decreased production ie. the body isn’t producing the right number in the first place and could be down to bone marrow failure. The biopsy would help to focus the investigation. (I suppose it could be a combination of both but we’ll cross that bridge….).

We also discussed some other factors which I’m still struggling to understand. I have an enlarged spleen (splenomegaly) – what caused that? Enlarged spleens can hold increased numbers of platelets and therefore lower the number in circulation and the number that get counted. Then there’s the blood clot in my portal vein (PVT – Portal Vein Thrombosis). Did this cause the spleen to enlarge? The doctor remarked that blood clots in this location were common in Crohn’s patients and it was plausible that the clot could have been there since my emergency operation in 1979 as the liver specialist suggested. I asked why it hadn’t shown up on the various x-rays and scans that I had had over the years. She replied that unless the radiologist was specifically looking in that area it would be easy to overlook. Unfortunately the x-rays up to the year 2000 are no longer available. There is a CT scan from 2009…..but this is all rather academic.

By now the results from the blood test appeared on the system – platelets 60, the lowest ever, but white cell and red cell counts normal. This suggested a platelet specific problem, not a general blood disorder.

We went though my list :

1) What involved in a bone marrow biopsy? It’s carried out under local anaesthetic by introducing a needle into the hip bone (?) and taking a small sample of marrow and then using a slightly larger needle to take a small core. Will it hurt? You’ve got Crohn’s disease and had surgery so you’re used to pain. The most uncomfortable bit is injecting the local anaesthetic. Some patients don’t even feel the biopsy needles being introduced.

2) Do I need to take any special precautions if I have teeth extracted? Unless your platelets fall below 50 then extraction should be OK. You might want to have a clotting gel available to stop your gums from bleeding. If your dentist is worried he might want to refer you to the specialist Dental School at Guys.

3) How regular should I be having blood tests and are there any special things to test for? Six monthly at your outpatient appointments is fine. You could ask your GP for more frequent ones. The only special test would be for clotting.

4) Is there any possible link between low platelets and diet? (A bit of a long shot this one but I due to see the dietician in a couple of weeks time). No.

Back to reception to book the biopsy and the follow-up appointment. I was offered the biopsy for the next morning. Unfortunately I wasn’t going to be in London so declined. “We have a slot at 3.00pm next Wednesday, is that any good?”. Excellent and the follow-up appointment was set for the week before Christmas.

The next day I got a phone call from the doctor asking me to forward a copy of the email correspondence for inclusion on my file. I told her that the biopsy was planned for one week’s time and she sounded genuinely surprised it was so soon. I mentioned that the follow-up appointment wasn’t until December and wondered if it should be brought forward. She assured me that once the biopsy results were available she would be in contact with their findings. Roll on the biopsy.

Wednesday 2nd October – Bone Marrow Biopsy

The procedure was planned for 3:00pm. In the morning I had told various colleagues that I wouldn’t be around after lunch and explained why. Every single one of them uttered the same 3 words “that sounds painful”. After you’ve heard it for the umpteenth time a few nagging doubts set in but I remembered what the haematologist had said last week “You’ve got Crohn’s. You’ve had operations. You’ve dealt with pain! This will be nothing by comparison.”

Guy’s Hospital in the shadow of The Shard

I set off in good time, (if you’ve read any other posts you will know that I always do), and arrived at Guy’s twenty minutes early, checked in and waited to be called. A nurse came over and gave me an identification wristband as the procedure would be carried out in the Day Hospital section. She said that I shouldn’t have to wait too long.

It was around 3:30pm when the doctor appeared. Her first reaction was “have you come alone?” That sounded a bit alarming. I asked why I would need to be accompanied and she replied that most patients were nervous about the procedure and liked to have someone with them.

She showed me into a treatment room. I took my shoes off and ay on my right hand side on the bed. She explained what she was going to do, where the needles would be inserted and then did the usual risk assessment talk. There was not a lot that could go wrong as the needles go straight through the skin into the hip bone and nowhere near any vital organs. I signed the consent form and we were ready to start.

I asked how long it would take for the results to be available as my follow-up appointment was planned for mid-December. She replied that they should be available in 4 or 5 weeks and they would contact me if anything untoward showed up. I asked to be informed even if nothing showed up as I didn’t want to wait until the appointment to find out.

She asked me to pull my knees up to my chest and adopt a foetal position. She felt around to find the best location for the needle and then thoroughly cleansed the area. This was followed by a series of shallow injections of local anaesthetic and was the most painful part of the whole experience but really not too bad. Certainly nothing to get hung up about. Some deeper injections were made but by now the first set of injections was working so I felt very little. A few minutes later it was time for the first sample needle to be inserted.

The slides

The aim is to get a liquid sample (aspirate) that can then be spread onto microscope slides for an initial examination within the department. She was having problems getting a good sample that wasn’t contaminated with blood as it kept clotting (which goes against what you would expect from low platelets). Because I was tolerating the needle so well she took some more samples but explained that the as long as she could get a good core sample then the quality of the liquid samples wasn’t important.

Time for the coring needle, which is quite a bit larger than the previous one. If you’ve ever seen one of those food programmes about cheese no doubt there will have been a scene where the cheesemaker inserts a tool into the cheese and pulls out a nice sample. Same principle here!

It takes a fair amount of force to push the larger needle through the outer layer of the bone. I could certainly feel it as it went deeper in. It wasn’t so much pain as a dull ache that traveled into the leg. After a couple of minutes of pushing the needle into the right depth it was withdrawn and the sample released. She was very pleased with the resulting core and set about dressing the puncture wound.

Bone marrow core sample

I then had to lie on my back for 15 minutes whilst the blood clotted and sealed the wound. I was told that a nurse would come and tell me when I could go. After 20 minutes or so she came in and looked at the wound. It was fine so back on with my shoes and down to the station to catch the train home.

The procedure room

Throughout the procedure we talked about low platelet counts, possible causes, what the tests would show, the fact that my red and white cell counts were normal, my Crohn’s history, empowered patients etc. It was very informative and kept me at my ease.

If you have got to have this procedure done it really is fairly painless. Once the initial local anaesthetic has been injected it’s pretty much plain sailing.

24th October – “Please relax this weekend”

I had added a reminder into my calendar to email the consultant after 5 weeks and ask if there had been any news. I needn’t have bothered as they were already “on my case”.

On 24th October I received an email saying “your bone marrow is being discussed with the Histopathologist and Dr. xxxxx will write to you with the results. We will see you in clinic in December.” Straight onto Wikipedia and I now know that a Histopathologist is someone who carries out “microscopic examination of tissue in order to study the manifestations of disease”.

I replied by asking if, once the discussions had been concluded, they could email me with an indication of what they had found  I explained that I had rescheduled my appointment for the end of November so that it preceded my Gastro appointment in early December. Last Friday I received another email saying that it would be easier to discuss the findings in clinic. Whoa. Did that mean – nothing to worry about, it can wait; or it’s serious and we want to tell you face to face?

I’m pretty laid back about my health nowadays. I’ve had enough shocks along the way to just accept whatever will be will be but I was starting to get an uneasy feeling. There was no way I would relax over the weekend knowing that the results had been assessed but I was in the dark. Time for another email “…I wonder if you could just put my mind at rest that you haven’t found anything too serious otherwise I won’t be able to relax this weekend!”

Within a few minutes this came back :

“Please relax this weekend. We have reviewed your bone marrow in our multi-disciplinary meeting and there is nothing sinister to report. The findings suggest that your marrow is underproducing platelets rather than it being an immune cause that we had presumed secondary to your longstanding history of Crohn’s. This may be due to previous Azathioprine use…….I look forward to seeing you on 20th November and we can discuss this in person and in more details then. In the meantime – I hope this reassures you.”

I thanked the doctor for her prompt response. I can relax until I see her on 20th November, apart from having to go to work, commuting to London and worrying what effect the fireworks will have on our dog and ponies. (Cut to gratuitous picture of dog and ponies for light relief)

Next stop – 12th November – upper GI endoscopy to see if my esophageal varices have regrown and to band them if necessary. Obviously I’m hoping that they find nothing as you can only eat sloppy food for four days after any banding otherwise the food might dislodge them. For what happened please see the post entitled “Upper GI Endoscopy”.

Haematology – 20th November 2013  Today’s appointment was to go through my bone marrow biopsy results. Even though I had already had the email a couple of weeks back telling me that there was “nothing sinister to report” but it was always at the back of my mind that there might be something significant that needs discussing face-to-face.

I got to the clinic early to allow time for the obligatory blood test. With that over I settled down to wait for one of the consultants. After 15 minutes my usual doctor collected me and we went into a consulting room. She started our conversation with “Yours is not a simple case…..”.

She had printed out two biopsy reports – one for the recent bone marrow procedure and the other for last year’s liver biopsy (which I had not seen before – more of that later).

The bone marrow results had been discussed at the MDM (multi-disciplinary meeting) and the initial conclusion was that they were “in keeping with early/low myelodysplastic syndrome, histologically suggesting MDS-RCMD.” She knew that I would have looked this up on the net and then probably have been worried/distracted by the potential links with leukemia. That’s why the report hadn’t been emailed to me.

(This is a quote about MDS from patient.co.uk – “The disease course is highly variable, from indolent to aggressive with swift progression to acute myeloid leukaemia (AML) in 30% of cases.” I think she made the right decision to want to discuss it in person.)

She went on to say that she was not completely happy with the MDS conclusion because a bone marrow biopsy looks at two substances – the marrow itself and the aspirate, that’s the bone marrow liquid. When I had the original procedure the doctor said that she was not getting good aspirate slides as the blood in the samples kept clotting. After several attempts, but with little success, she decided to concentrate on taking a good bone marrow core.

“It’s like having a three piece jigsaw from which two pieces were missing.” So at the next MDM they had discussed the results again and decided that, in my case, it was unlikely to be MDS but would recommend a further biopsy to get useable aspirate samples. “How would I feel about this?” I replied I really wasn’t fussed, the most painful bit was the initial injection of local anaesthetic. If it would help narrow down the diagnosis then the sooner the better. She explained that this time they would use Heparin with the sample needle as this should prevent the blood from clotting.

If the diagnosis wasn’t MDS then why the low platelets? The most likely cause was a combination of long term Crohn’s and taking Azathioprine. The biopsy had shown that the marrow was under-producing platelets rather than being over active and eating them up. I was unaware that there is a potential link between Crohn’s and bone marrow. (I’ll do some further reading up on that one)

Using my blood results I’ve plotted my platelet count vs. Azathioprine dose (click to enlarge)

They had then gone on to discuss what the implications for treatment would be if it was/was not MDS. In either case the preferred course for treatment, at this stage, would be “do nothing” unless I was to have any procedures that could cause bleeding or that require surgery. A supply of platelets should be made available if either of these were needed. The difference in approaches would be in the monitoring regimes and we would discuss this further after the next biopsy results were available.

Back to reception to book up another biopsy (9th December) and 3 month follow-up appointment.

Included with the Haematology Report was one from my Liver Biopsy – if I thought blood was complicated then reading this report is mind boggling. I haven’t even tried translating it into easy-to-understand terms. Here’s a sample :

“Features of cholangiopathy, with slight cholangiocyte disarray, occasional juxtaportal hepatocytes containing copper-binding protein deposits, and scattered ceroid-laden macrophages in portal tracts. Patchy mild portal-tract fibrosis with perisinusoidal extension and early spurring. Macrovesicular steatosis of hepatocytes (5% of parenchyma). Slight centrilobular sinusoidal ectasia noted. Crohn’s disease with splenic vein block (clinical diagnosis), see comment. An early stage of primary sclerosing cholangitis is a possibility. Correlation with imaging-study findings appears in order. I can not suggest an aetiology for the slight sinusoidal ectasia observed.” 

Next Appointment – 2nd December to see my gastro-enterologist. Will be interesting how all these strands come together.  I’ll also get the result of the calprotectin test that was done recently. It will be a good pointer as to whether my Crohn’s is active/inactive and consequently what the future treatment plan is likely to involve. If it is still in remission then is it better to continue without any medication for as long as possible or would it be better to start taking precautionary doses? I get the feeling that the answer won’t be a simple one.

If you’ve looked at some of my earlier posts you may have read about the problem I have had with not getting  follow-up letters out of Haematology in a timely manner. This was brought home to me when I saw my gastro-enterologist last Monday (2nd December). He was unaware that I had seen the haematologist to discuss the results of original biopsy; that there were implications for restarting Azathioprine; that a second procedure had been arranged and all because there was no letter detailing all this on my file. (He very helpfully went on to say that a bone marrow biopsy was the one test he really wouldn’t want to go through himself!).

9th December – Haematology Day Unit – Guy’s Hospital

Biopsy II Day. A beautiful, sunny winter’s day. Took the District Line down to Monument and then a stroll over London Bridge, underneath The Shard and on to Guy’s Hospital.

The Thames from London Bridge

 

The Shard appears to have grown arms

I arrived in plenty of time, was wristbanded and waited for a doctor. She appeared a short while later and I was taken to a cubicle in the day ward. She went through the usual risk review and got me to sign the consent form.

Then, for the second time, the value of follow-up letters became apparent. The doctor had referred to my notes and found the latest letter on file, dated September. She was unaware of what had happened in the interim, that this was to be a second sample attempt but with no need for a marrow core this time. It’s a good thing that I had taken this all in, together with the plan to use Heparin to stop the blood from clotting otherwise it would have been back to square one and a third biopsy. (Whilst this blog sometimes goes into too much detail it is proving useful when follow-up letters aren’t forthcoming and I want to recall what we said at an appointment)

She went off to find the Heparin. When she returned she laid out the various bits of equipment – swabs, needles, instruments of torture, local anaesthetic etc. She got me to arrange my clothes so she could access my hipbone and to ensure any leakage missed them. I then rolled onto my left hand side and draw my knees up onto my chest.

Instruments of torture (luckily the largest one wasn’t needed)

Last time it was the local anaesthetic injections that stung the most but this time they were outdone by the sample needle. I think a couple more minutes to allow the anaesthetic to work and it would have been fine. She looked at the first slides but they weren’t quite as she wanted, probably due to the Heparin. She asked if it was OK to go back in with another needle to get a second sample. This time I felt nothing apart from a liquid trickling down my back. It could only be blood. Slightly unnerving. Clearly the Heparin had worked. She was now happy with the slides and showed me what they were looking for. To prove how truly sad I am I asked her if I could take a picture of a “correct” slide.

This slide was the one chosen for further examination

She cleaned up the “leakage” and put a dressing over the puncture. It was then a 15 minute wait, lying on my back, to ensure everything had started to seal itself. She warned me that it was likely to need a new dressing before I left hospital due to the action of the Heparin. After 15 minutes a nurse came and changed the dressing and I was allowed to go back to work.

Now I have to wait for the results. Can’t believe it – I forgot to ask how long that process would take. Must also chase up the follow-up letter but quite frankly I’ve had enough of appointments and procedures for this year. I just want to chill out and try and forget about medical matters. The follow-up appointment is set for 19th February.

Wednesday 19th February – Haematology II – Guy’s Hospital

The first appointment of the year and really unprepared for it. It’s only two months since the last one but I’ve already got out of the habit. Traveling up to the London on the train in the morning I realised I hadn’t even got a list of questions to ask. When I got to my office I printed off the account of my last trip to Haematology, just to jog my memory (see post – “Bone Marrow Biopsies, Follow-up and Results…well sort of”). I then re-read the post “Managing Consultants and Appointments” to make sure I put into practise what I preach. By the time I set off for Guy’s Hospital I had managed to write down 7 things I needed to ask or mention on a good, old fashioned Post-It note.

List of questions for Haematology

Appointment was for 10:00am so arrived 15 minutes early for the obligatory blood test. The phlebotomist asked me if I knew why she was also taking a “histological” sample, but I didn’t.

Back to the waiting area and at 10:00am my usual doctor appeared, greeted me warmly and we set off for a consulting room. She introduced me to an American medical student, who was over in the UK to see how things are done in the NHS, and checked that I was OK with someone else present during the consultation.

She explained that after the last bone marrow biopsy one of the samples, which should have gone for histological testing, had either been mislaid or mislabelled so did not make it to the histologist. This is why she had rung me a few weeks back to explain the situation. She had however looked at the other slides from the second biopsy and these were fine. She had discussed the missing sample with the chief histologist and he suggested doing a particular type of blood test which had proved to be 60% effective in spotting problems, if there were any. The results would be available in a week’s time. The alternative was to have a third bone marrow biopsy but they didn’t want me to put me through that again. I’m really not that fussed.

…and so to the list :

1) Long term prognosis – will not affect the other  issues I have – Crohn’s, potential PSC, PVT. Must avoid use of Azathioprine in the future. Today’s platelet count = 74, an increase of 18, but don’t get hung up on the numbers as I am currently asymptomatic ie. I don’t bleed profusely if I cut myself.

2) Cause of low platelets – no signs of any marrow abnormalities which could have pointed to a more sinister conclusion ie. leukaemia therefore cause is drug induced – long term use of Azathioprine.

3), 4) and 6) Treatment – none required but look out for any signs of starting to bleed more easily. Six monthly blood tests and outpatient appointments. A platelet count of 50 is the threshold for having minor surgery or teeth extraction so no need for special measures at present.

5) Medical Synopsis – some inaccuracies had crept into the synopsis at the top of the last follow-up letter. I wasn’t sure if they were significant but we went through them one-by-one and put them right. (I had already emailed my gastro consultant a few weeks back with the correct information)

7) Follow-up Letters – this was an area where we had had issues in the past. I requested that follow-up letters were sent out as soon as possible after an appointment as the last time I saw my gastro consultant he was unaware of my bone marrow biopsy etc. She promised to improve the timing in future and would write to me as soon as the histological results were back.

I mentioned that as part of the NHS Change Day (3rd March 2014) I had pledged to give feedback to my consultants on the service they provide (whether they wanted it or not!). This was my first chance to put the pledge into action. She thanked me for my feedback.

10:20am appointment completed and on way back to work

SUMMING UP

The low platelet issue had originally been “parked” but in September 2013 I felt that we really should investigate it further so we could decide if Azathioprine would still a viable drug for treating my Crohn’s and  to make sure there wasn’t a  more serious underlying problem. My gastro consultant supported this and the investigation started.

Two bone marrow biopsies later and we have the answer. The low platelets are not indicative of a bone marrow abnormality but are drug induced with the likely cause being to 8+ years of Azathioprine. It is a known side effect of this drug. Azathioprine is sometimes used to maintain Crohn’s remission but if I get to the point where I need to go back on medication it will not be considered as an option.

The low platelets can return to their parking bay.

…and the implications for other Azathioprine users? The above is just MY experience of taking that drug and as we all react differently to medications you should not assume you will end up in the same situation. Whilst I stopped taking it when we realised there was a potential problem it has not damaged my bone marrow sufficiently to need to take further action. The haematologist described it as like having a “four cylinder engine but only running on three”. Would I have agreed to starting Azathioprine back in 1998 if I knew then what I know now? Yes. For nearly ten years it kept surgery at bay so that when the knife became inevitable I was in a much better position both financially and mentally to cope.