Shirley's story - Cushing's
Pre-Cushing’s to present day
Not quite sure where to start – so let’s go back some eight years before I was, at last, diagnosed with Cushing’s disease; that would be 1997.
I had been feeling unwell for a long, long time but couldn’t quite understand why. I had been putting on so much weight, although dieting constantly, but my stomach seemed to be getting bigger and bigger. My face also became very round (I used to have a very ‘pixie-shaped face). My blood pressure was very high – again no explanation. My skin became very thin and painful to touch – and I guess I was pretty miserable to live with as I was feeling so low. Also, don’t laugh; I had the biggest verucca in the world which just kept getting bigger and bigger! I made many trips to the GP, who sent me away every time, telling me to lose weight and stress less.
At one stage, there was a bit of a ‘red herring’ as many years before – as a young teenager – I had suffered with very bad psoriasis, and at the time, we weren’t aware of the dangers of using topical steroids. This was made worse by the fact that I was told to cover the psoriasis (back, stomach, legs and arms) with Betnovate ointment, and then wrap myself in cling film so that it would soak in! When I told the dermatologist this, I was told that my problems were probably caused by steroid damage from the ointment. He wrote a letter to my GP to this effect, but fortunately as I was to find out later, also mentioned the nasty words ‘Cushing’s disease’.
There was another time before this, when I was actually tested for Cushing’s, and although my 24 hr urine was ‘sky high’ when it came to testing my blood for cortisol, it appeared to be normal (I later found out that this was due to the fact that my Cushing’s was ‘cyclical’).
At the time I was covered by my husband’s private medical insurance so felt able to pursue my problems further.
The ‘breakthrough’ came when, after being told, once again, to lose weight, I asked to be referred to my local endocrinologist – Dr Rehman Khan who practices at Basildon Hospital. He was the first person to take me seriously. Firstly, he put me on a course of slimming pills which were supposed to excrete any fat that I ate. He monitored me on a regular basis, but alas no weight loss (as I wasn’t eating any fat!). It was at this stage that he started to test me for Cushing’s disease.
Every three months or so, Professor Pierre-Marc Bouloux of the Royal Free Hospital in London held a teaching clinic at Basildon Hospital – and luckily for me he was holding one of these the following week. As I walked into the consulting room, there was a sea of white coats (including Dr Khan – a familiar face thank goodness!) The Professor listened to me for a short while whilst I told my story – asked me a few questions – and told me I had the classic symptoms of Cushing’s disease. I felt very emotional – and happy even – as at last I had been listened to and something could be done for me. I hadn’t actually been imagining things as my GP seemed to think. Within a couple of weeks I was in the Royal Free – spending a whole week having all the normal tests under the watchful eye of the wonderful endocrine Sister, Judith Kisalu (who still looks after me now – thanks Judith!).
From the tests, it was concluded that I did, indeed, have Cushing’s disease and it appeared that the problem was coming from the left side of the pituitary gland. I was immediately operated on and the left side of the pituitary was taken out. Unfortunately, it appears that the blood vessels were crossed (which I had been warned might happen) so three days later they had to operate again and take the rest of the pituitary gland. On top of that I had a CSF leak (requiring a lumbar drain to ease the pressure). This caused quite bad hearing loss and tinnitus.
It wasn’t until then, that I realised the importance of that tiny little pea-size gland. I was then faced with all the problems of total hormone replacement whilst I waited for my cortisol levels to go down. Alas, that didn’t happen.
I found the diabetes insipidus the most difficult thing to handle – and today it can still give me problems – especially if I have an upset stomach for example which also affects the amount of hydrocortisone I take.
Following the surgery, I had to have gamma-knife radiotherapy – it sounds pretty horrific – but was nowhere near as bad as I expected. (I should be happy to explain further if anyone is interested!). The idea of this was to kill off any of the cells that were remaining which were causing my cortisol levels to be so high. Unfortunately, it would take 2-3 years for this to work! In the meantime, I was put on Ketaconazole to ‘dampen the adrenal glands’. (I had been offered a bi-lateral adrenalectomy which would have solved the problem for good, but I wanted to avoid more surgery at this stage).
Unfortunately, or maybe even fortunately looking back, I felt so dreadful on this medication, that I finally elected for the adrenalectomy. At last I was cured of Cushing’s disease – alas with the loss of my pituitary gland and both of my adrenal glands. Unfortunately, I learned quickly that although cured of Cushing’s, it never actually leaves you alone completely.
After this, I ticked over quite nicely, though never really felt like my old self. Friends and family were wonderful, as was the Pituitary Foundation, but I never felt like I used to. I had regular visits to the Royal Free to see Professor Bouloux and Judith where my bloods were checked, as well as frequent phone calls to Judith who gave me so much support when I was feeling down.
Now we go to 2011. I just didn’t feel well again. Again couldn’t work out why. I felt so tired and listless all the time. I asked to have another ‘day curve’ hoping that my hydrocortisone would be increased. I couldn’t believe it when all my bloods came back ok, and my morning h/c was actually decreased! I felt so low at this point and I thought the ‘light at the end of the tunnel’ had disappeared completely. I agreed that I must be depressed, and I went for counselling at Basildon Hospital. This didn’t seem to help so I stopped after several sessions.
In February 2011, I went to visit my son where he was working in the French Alps, and to my dismay got stuck in the bath for at least half an hour until I got help. This happened more and more frequently when I got home – so decided to stick to the shower instead! On holiday in Spain that June, I still felt dreadful and dismayed when I tried to swim, and couldn’t.
By July things were even worse. I started to lose my speech, my right- hand side became very weak and I was falling over frequently. I was unable to write clearly or do simple calculations. My confidence had gone completely.
I rang Judith who immediately told me that I needed to see a neurologist. She contacted Prof. Bouloux who said exactly the same. Prof Bouloux rang me but I was unable to speak clearly so my husband had to explain what was happening.
I managed, very quickly to get an appointment to see Mr David Choi, Neurosurgeon at the National Hospital for Neurology and Neurosurgery in Queen Square. To cut a very long story short, the MRI showed a large left-frontal meningioma (the size of a satsuma). This is a benign brain tumour in a pretty dangerous place!
I was set to have my surgery at the beginning of August 2012 (yes I missed the Olympics and the only good weather we had last year!) The first op was to drain the blood supply to the tumour and the second, two days later, was to remove the actual tumour itself. The op went well, and I was told it had all been cleared – but yep – you guessed – unlucky again because it was discovered that there were microscopic cells which were too dangerous to get to. (If removed surgically, it could have caused damage to the artery, which runs from the brain to the heart). Thanks to Miss Vivian Elwell, Mr Choi’s Registrar who is looking after me now.
Whilst in hospital at this time, I just wanted to explain the problems I had. Not due to the meningioma, but due to the hallucinations I had after surgery which I wasn’t prepared for. I had read something about this before, but I didn’t realise how bad they would be.
As I had no adrenal glands, I had to have massive amounts of steroids to get me through the surgery. This was not only during the surgery, but also before and after. I cannot remember the exact amounts, but I know that Professor Bouloux at the Royal Free and the endocrine team at Queen Square consulted one another throughout.
The hallucinations were so real. For example, I saw a high altar and there was chanting, and someone, who I assumed was the devil, was calling my name and beckoning me to join him. I saw flashing red lights and I felt very scared. This continued throughout my stay in hospital – two and a half weeks – and the name chanting continued even longer.
I was also convinced that I was in some kind of maternity ward – where people had lost their babies! Also, on the same ward, I told everyone that there was a party going on all night to raise fund for the brain tumour ward – there are plenty of similar stories I could tell on request! To add to the confusion I was moved to at least 8 different places – partly due to the fact that the brain tumour ward was closed down at weekends due to funding cuts. The urine infection I contracted at the same time didn’t help my confusion!
So now, back home and feeling great.
After the surgery, I had developed a reservoir of brain fluid between the scalp and the skull, which eventually disappeared after approximately 3 months once the skull bone had fused together. I hadn’t been allowed to fly until this had disappeared which was a bit of a pain, so we ended up driving all the way from Calais to Spain – a fantastic trip which we are going to repeat again in the summer, despite the fact that I am now allowed to fly.
Maybe I should mention at this stage, that this brain tumour was no way connected with my pituitary tumour or the radiation I had for it. It was in a completely different area. I had just been unlucky!
Now – radiotherapy to begin on 4th February 2013. – A bit apprehensive but no need to worry. Everyone was absolutely wonderful. As I live out of town, Billericay in Essex, I qualified to stay in the ‘Cotton Rooms’, a wonderful facility for patients having my sort of treatment, and funded by the hospital’s charity. I travelled up every Monday and home again each weekend after my treatment. The time went much quicker than I expected. I also met some wonderful people who I still keep in touch with. There were a couple of set-backs in the first two weeks – ‘the runs’ which meant I ended up as an in-patient for two nights as it affected my hydrocortisone and desmopressin. Second week, chest infection – antibiotics which made me feel even worse!
However, after that, all went smoothly. I took advantage of the free complimentary therapies, relaxation classes, massages and a wonderful new wig! The ‘Look good, feel better’ session was also wonderful. Thanks Macmillan.
Home now and feeling brighter. The tiredness has now improved and I’m just waiting for my hair to grow back! I have lots of pretty scarves to wear to co-ordinate with my clothes and my consultant oncologist, Dr Naomi Fersht tells me that I brighten up her day when I walk into her clinic. (She did, however, laugh when she saw my lack of hair in places – but immediately apologised!)
I could write a long list of ‘thanks’ – but to everyone who I came in contact with during my journey – I’ll list them separately.
My spare time is now being spent raising money for Macmillan who do such a marvellous job. They were always there for me when I felt down. So now, as well as supporting the Pituitary Foundation who helped so much in the early days, I’m in the process of arranging a fund-raising function in our local pub as well as several other ideas for Macmillan.
That light at the end of the tunnel is right in front of me now, and I enjoy every day as if it’s my last! Never lose hope.
Thanks to everyone who has helped me during my journey, especially my family and friends.
Special thanks to:
Dr Rehman Khan - Consultant Endocrinologist
Basildon and Hartswood
Bouloux - Professor of Endocrinology
Royal Free Hospital
Sister Judith Kisalu - Sister – Endocrinology
Royal Free Hospital
Mr David Choi - Consultant Neurosurgeon
Miss Vivian Elwell - Registrar to Mr Choi
Dr Naomi Fersht - Consultant Oncologist
Dr George - Registrar to Dr Fersht
As well as everyone at UCH and the Macmillan Centre, and the Pituitary Foundation in the early days.
And last and not least, my wonderful family and friends, Steve, Elaine and Sally - take a bow!