Part Three: Starting Treatment 2019

25th July 2019 - Royal United Hospital Bath

Gastroenterology Clinic - 'Transplant' mentioned again

I remember this appointment clearly. I walked into Dr Maltbys room alone and an elderly nurse was sat in a chair opposite to mine thinking it was bit odd why a nurse was present during my consultation.

We discussed the next stage of treatment which was a visit to the Queen Elizabeth Hospital in Birmingham and a meeting with Dr. Ferguson. The topic of a potential transplant was brought up, but my main focus was on the nurse's quiet presence, nodding kindly as she gazed at me.

It wasn't until later that I realised she might have been there if I had reacted badly during the meeting. I remember hearing the word 'transplant' and the Queen Elizabeth in Birmingham - a place I had never visited. We talked about changing my lifestyle, but either of us couldn't see how it could be improved since I was already doing everything right.

On leaving the hospital, I must have been in a state of shock or denial, as I can't recall how I managed to get home. I do remember asking a friend to spend the night because I was scared. She agreed, although she hadn't slept on a sofa since we were teenagers, even though my sons were at home. I didn't share the news with them until I had processed it my self.

Extracts from RUH letter:

Problem1: Jaundice related to granulomatous hepatitis most likely caused by sarcoidosis

Problem2: Uveitis

Plan: Continue Prednisolone 40mg daily - Add treatment of Urso, Out patient appointment with the Hepatology team in Birmingham in July. Recheck liver functions tests

I saw Caroline for a follow up today and as you are aware she presented fairly acutely with jaundice in early April although in retrospect, her liver function tests had not been normal in October last year (I did not know this until I read this letter) She had be diagnosed as having Uveitis last year etc...

Sarcoidosis seems to the most likely cause of Caroline's underlying granulomatous hepatitis given the combination with the Uveitis. I became more concerned about Caroline last week when I had seen that her most recent liver function tests showed her ALT and bilirubin were rising again. As a precaution, in case things with Caroline do not begin to move in the right direction, I have arranged for her liver biopsy to be sent to the team in Birmingham for review.

Understandably, it has been very difficult for Caroline to come to terms with developing such an unpredictable illness where no one can explain to her why it happened nor is it really possible to feel that she can have any control over the situation by changing her lifestyle or her diet. She very much appreciates that we are in a situation where we hoping for a good recovery but planning for the eventuality that this does not happen by arranging the safety net of an appointment in Birmingham.

July - Liver Clinic QE

My first journey to Birmingham to meet the new team. It was lovely sunny day and I travelled there by train. The hospital was a short walk from the train station, and despite having lost quite amount of weight, my mobility and fitness levels were still relatively normal.

My first consultant was Dr Ferguson. We shook hands and began clinic. To be honest, the complex medical jargon and enormity of attending this hospital far away from home was quite overwhelming and I don't recall much of the conversation so the extracts from the letter below helped very much.

Extracts from QE letter:

I thank you for our previous discussions on the phone and your email correspondence. She is 56 and first presented in October 2018 with Uveitis, was eventually diagnosed as Sarcoid Uveitis.

She then presented to your service with jaundice in April this year. You performed a liver biopsy. I had our team review it here at the Queen Elizabeth. The biopsy shows marked ductopenia, moderate acute hepatitis and granulomatous inflammation. This would be compatible with a diagnosis of Hepatic Sarcoidosis. Quite reasonably you commenced her on steroids and she now is on a reducing course. I believe she has had a chest x-ray which is normal. She was also commenced on Ursodeoxycholic 250 mg TDS. She has lost around 20 kg in weight, but now feels like she is starting to put weight on. She still is jaundiced and has evidence of sarcopenia. Can I recommend that she has a bone scan and an up-to-date chest x-ray. I explained to her that sarcoid is a multisystem disease and it is unusual for it to affect the liver and lead to jaundice.

However, when it does so, the treatment is with steroids and Ursodeoxycholic Acid. Around one-third have a complete response, about a third have a partial response and another third have no response. I did mention to her that on occasions patients with hepatic sarcoid have required liver transplantation, but only time will tell. Can I recommend that she goes on to protein supplements in the form of Renapro shots 20 g twice a day and Meritene shakes twice a day to help with her weight loss. We will review in 4 weeks to evaluate her treatment response; if she remains profoundly jaundiced we may need to consider transplant assessment.'

17/07/19 - Bloods: Weight 50.8, ALT 260, Bili 304, ALP 841, UKELD, 62

*UKELD: The United Kingdom Model for End-Stage Liver Disease or UKELD is a medical scoring system used to predict the prognosis of patients with chronic liver disease.

July - Letter exchanges RUH and QE

Extracts from RUH letter:

I have just received the letter from your consultation with Dr Ferguson in Birmingham on the 17th July. Dr Ferguson has asked that I arrange for you to have an up to date chest x-ray and a bone scan. I have also made a requests for some up to date liver blood tests and perhaps you might like to pop in next week and combine having the chest x-ray.

With regard to the nutritional supplements he would like you to start, I am not sure whether the particular ones he has suggested are available locally and I am waiting a response from our dietician as to whether they are available for a substitution might be made.

PS the dietitian's have recommended Fortisip compact protein 125 mils three times a day. I understand this has similar protein intake to twice daily Renapro. An alternatifve is Prosource.

August - Request for the Liver Transplant Assessment QE

Another clinic appointment at the QE. I was still taking the train by myself even though I was becoming yellower and weary. It was now obvious that I was not well, loosing my muscle mass and generally going down hill. We discussed the liver Transplant Assessment and I was told that this would be the next stage.

Extracts from QE letter:

Diagnosis:

Hepatic sarcoidosis

Inflammatory features together with profound ductopenia

Symptomatic with intense pruritus and sarcopenia

No response to steroids or Ursodeoxycholic

Assessment

I met Caroline in clinic today. Unfortunately she remains profoundly jaundiced, hypoal buminaemic, and now sarcopenic. From a symptomatic perspective, pruritus and fatigue are now dominating her quality of life. Looking at Caroline's liver biopsy together with our pathologists, the most striking feature to me is marked ductopenia - akin to what we see in patients with the premature ductopenic variant of PBC. There is no accompanying ductular reaction, so it is possible that she will never develop full blown cirrhosis.

However, the progressive deterioration in clinical status, persistently elevated bilirubin and low protein state are certainly indications for liver transplant assessment (requested today). I do not think steroids will grant any further gains here, as treating the acute hepatitis will not help her bile ducts recover. I have completed a full liver screen today (to exclude mimics of hepatic sarcoidosis) and prescribed some Rifampicin for itch. If this fails, then naltrexone or sertraline are suitable alternatives. Caroline tells me that she has trialled colestyramine with you before (unsuccessful and poorly tolerated). A stop gap appointment has been made 3 months from now, but interim correspondence will follow her transplant assessment.

31/8/19 - Bloods: Weight 52.7, ALT 158, Bili 318, ALP 891, UKELD 59

Notes: September 2019

• Questions for Julia

• Can I continue taking the steroids - please, please - they stop the itching which is coming back - the itching is unbearable. Still taking Urso has no effect. Took the dosage down to x5 for 2 weeks and that's when it started creeping back. Now on x4 till further notice. (Not sure if this is the Urso or Pred)

• Can I take more than 4 paracetamol a day - backache.

• Why is mine RARE? which part is RARE?

Weight loss exposed

I had always been careful and conscious about my gradual weight loss. I started to notice changes in the mornings. I would go to bed and my body would appear to munch away at me leading to loss of muscle mass known as sarcopenia or loss of skeletal muscle. This the major component of malnutrition which can effect some of us with liver disease.

Early one morning I was in the kitchen with both boys asleep or I thought so. The next thing I hear was Charlie behind me screaming that I looked like 'one of those poor starving African women'. I knew I was quite thin but I had, so I thought, managed to hide it well. Obviously not. It also didn't help in that I let my guard down and forgot to put my thick dressing gown on. I told him not to worry and it was 'normal-ish' and I just need to top up my protein shakes. I don't think he believed me and began quietly watching my eating habits.

September - My beloved hair!

I had been invited out for lunch at a very lovely Cotswold Manor by a friend whom I had not seen for quite a long time. He knew I was undergoing health issues and wanted to meet. I was starting to lose my confidence. Since my discharge from hospital in May, I knew I was loosing weight and my bilirubin was increasing and the Bart Simpson yellow was becoming a deeper shade of murky yellow. And my hair!! My beloved hair was shredding. This caused me more upset than looking skinny and yellow. No longer thick, it was definitely becoming much thinner.

So as one does, I asked a very good hairdresser friend if he would sort my hair out, give it a good cut, and more importantly, give it some body so I could go off to my lunch without feeling embarrassed. And yes, he worked his magic, I had lovely thick hair again and had a fabulous lunch feeling as near possible to normal.

Big Hair Day! Thank you Anthony.

September - Letter exchange QE to BEH

Extracts from QE letter:

Many thanks for your letter concerning this lady. You are right, we are assessing her for liver transplantation, however, this should not stop you treating her bilateral posterior Uveitis with steroids, and please go ahead as you feel fit. We will try and keep you updated with the results of our transplant assessment.

At this point, my friends had started to learn about my health issues. In particular, two former school friends called one evening and told me, rather than asking, that they were coming to visit me at once. I left the door unlocked and waited, only then realising that I needed to take my morphine/oramporhp to make it through the evening. In a rather unladylike manner, I gulped down some of the medication directly from the bottle just before their arrival becuase I couldn't find a spoon. We had a wonderful evening, although I don't recall much of it, they did.

Apparently, I was fully awake and alert one minuet then suddenly slouched un lady like on the sofa the next minute. This pattern continued throughout the evening, and apparently, I wanted to cook pasta but couldn't work the ingredients in ounces and pounds, I was talking about measurements in tons.

Notes: 29/9/2019

Red dots appearing again on a green background.

Extreme itching continues - face, head, ears now.

Weight loss continuing, started September when sick (food and smell)

Tooth distracted

Drugs for itching!

Julia - questions

Can I take Vit C tablets?

Is my back ache related to the liver?

My feet ache too - is this liver related?

My eyes sometimes look very yellow?

According to my optician, I now have Cataract in the right eye

Haven't used any Aqueous cream for weeks - skin all good and soft except for the blotching.

More questions 10/10/2019

I have Marked Ductopenia - my bile ducts have vanished - where have the gone? and my liver is inflamed - Moderate Acute Hepatitis which has resulted in a collection of immun cells - granulomas which are all compatible with 'Heptatic Sarcodoisis - which is a rare inflammation disease - cluster of white blood cells called granulomas which can form/attack anywhere in the body and are damaging to any organ - ie MY LIVER. Great!

I also have Sarcopenia - aging muscle loss - but I went to the gym?

I'm also 'profoundly jaundice - never! I,'m hypoalbuminaemic - not much protein.

Is this correct - has this progressed or static?

1. What are the results of the 1st Assessment

2. Itching - Are we going to try Sertraline? I don't want to as it says opioid, isnt' that heroin, I don't want to take heroin.

3. What are all the white blotches?

4. Can you recommend another skin cream please?

5. What about sleeping tablets - okish during the day when busy but night time is worse - scratch all night and blood on the sheets and clothes during the day - a step backwards.

6. Protein - difficult to take 3 x 12ml Fortisip Dr F recommended Renapro shots twice a day and Meritene shakes twice a day.

7. Confirm what total medication to keep taking.

8. Overall - when at work and have had a 'good nights sleep' feel as normal as can be. Walking speed has increased, brain activity/fatigue is less. Do not need to apply so much cream for itching. Concentration is good - feel back to old self. No more back pain - just stomach.

October - 2nd Design Brief

Shortly before the initial assessment, another ex colleague asked about my availability to work on a small personal design project. This time, the brief was more manageable. It was to design an Order of Service with all the necessary details provided, such as photos and readings etc. The job proved to be less complicated than developing a brand identity. I took my time and had several proofreaders for checking and all was good to go.

It did cross my mind whether to design MY Order of Service myself or ask one of my designer friends, such as Peter S, Mark, or Michael, with Jette overseeing the project and Peter handling the printing. However, I quickly put the idea out of my head as it seemed somewhat gloomy and concentrated on the current design project.

I was still working full time teaching design. Managing the eyes and the liver became part of my everyday life unlike the first initial stages where all the illnesses was new to me. I was having a good sleep at work during lunch time - several soft staff chairs were laid out in a row in my room which meant I could lie down and sleep comfortably which helped enormously with maintaining my strength and mind. Power nap at its best.

All in all, I completed the design and felt like the old me again. According to my email exchanges I completed the job in November 2019. Maybe I'm not really ill and I might be ok in the end I secretly thought to myself.

01/10/19 - Bloods: ALT 218, Bili 315, ALP 891

10/10/19 - Bloods: ALT 656, Bili 332, ALP 1177, UKELD 56

Next Read:

Part Four: The Liver Assessment 2019

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