Cushings Syndrome: The girl behind the moon face
When I first started this blog I decided I would only write when I had something to say. By chance, before my brief break due to Christmas mayhem, this has meant embarking on what appeared to be a legal blog. I guess this was the easy way out, it was a way of speaking my mind without revealing much of who I am.
Over the weekend I had the privilege of talking to two individuals suffering from Cushings Syndrome. In one of those conversations I was thanked for giving someone hope; in that moment I was taken back to a former life, scared, looking for answers and quite frankly looking for hope. During my time in testing and hospital there was no account of what was to come, there was nothing to relate to and no positive image of life after surgery. I was alone and without a story of hope. So here we are, I am facing the story I have been hiding from, that harsh truth of what it really took to get to where I am. For all those fellow cushies out there here it is, the good, the bad and the ugly, but most of all truth and hope.
For those new to the term Cushings, in short it is a rare condition where the body is exposed to excess cortisol; side effects include excess weight gain, a very round and bloated face (hence the common nickname associated with the condition “moon face”), high blood pressure, bone and muscle deterioration, insomnia, fatigue, bruising very easily, fertility issues and that is before we get on to the mental health issues. Those with Cushings Disease are often diagnosed with depression, anxiety and become very familiar with panic attacks. The steroids in your body are so high your body is attacking itself, which is why if Cushings is found in dogs and horses they do not survive and are generally put down before the symptoms become too much.
Make no mistake your body is killing itself. For insight into what that can mean, Michelle Obama documents a first hand account of watching her father suffer in “Becoming”.
For those who have not guessed yet I used to suffer from Cushings Syndrome; the cause of the excess steroid was a tumour embedded in my pituitary gland. The tumour was removed two years ago and I have thankfully been in remission and recovering ever since. However; due to the development of various symptoms it is estimated I went undiagnosed for a decade.
To understand my story I need to take you back to when I was sixteen and (mis)diagnosed with PCOS (polycystic ovarian syndrome). Although to be fair, 15 years ago PCOS was not commonly diagnosed and certainly not for someone as young as I was. At the time I went from being a healthy child who generally stayed in shape because I enjoyed sport to suddenly piling on weight. I did what many teenage girls do I cut the calories. Breaking point came when I cut to no more than 800 calories a day with regular ten mile bike rides, yet by the end of the month I had gained a stone in weight. My parents did what any parent would do and took me to the local village GP where I had blood tests. I was quickly diagnosed with PCOS and told that this was a lifelong condition; I would struggle with my weight, be susceptible to irrational mood swings, have a higher risk of cancer because of the weight issues and I would not be able to have children. At sixteen (week of my seventeenth birthday)… that was a lot!
In good news I had a doctor who, due to suffering from the same condition, was extremely knowledgeable. Against guidelines at the time I was prescribed metformin (a drug more commonly used for diabetics) which, when combined with gym sessions five days a week and maintaining my new found 800 calorie a day diet, helped me to drop five stone in a year. However; the need to diet never went away, if I ate the slightest amount more my weight ballooned and my symptoms got worse, put simply I could not relax and eat more. This was compounded with the fact that my metformin dose was so high I was regularly sick after meals.
I will never forget my sixth form awards night where the students come back from university for the first time and get together. It was on that night I heard the rumours going round my former peers that I had developed bulimia. Safe to stay that was the beginning of me closing in on myself and shying away from those I used to call friends.
Those early years of dieting were when I developed a relationship with food that has shaped me. Food became an enemy, but I knew that was not socially acceptable meaning I had to find ways of masking it. I was a very good cook and was known for it at university, what people did not realise was that I threw away the second half of my meals. If you cooked then people assumed you ate (to this day I still cant help but bake for people, you learn that whilst people enjoy your baking they don’t notice if you do not eat it. At the same time you can avoid sharing in office cakes and sweets). When I went out for a meal, I skipped my lunch and ordered a starter instead of a mains. I always carried celebrations or similar to boost my sugar levels because I didn’t eat large meals. In fact one year for lent I gave up snacking, during that time I went home for mothers day and that evening I collapsed from lack of energy…happy mothers day.
It was during my university years that the second side effect of Cushings really took hold, the insomnia. If I got two hours of solid sleep that was a good night. I learnt to lie awake and rest. I found it easy to be in the library all day and study in my room all night. On a Friday and Saturday night I would go out with friends drinking in an attempt to switch off my mind. That was my issue, my mind was never quiet, there was no peace. I craved the ability to shut out the noise and the only distraction from it was to never be still. As anyone who is run down will know, you lose your ability to be rational and your emotions are on edge.
Que symptom three which was probably the most damaging, anxiety. I can’t pinpoint exactly when the anxiety really kicked in, but I know whilst at university I had to phone my mum every day. There was always an issue, something I couldn’t rationalise and far too regularly tears. It was also during the university years that I started waking up short of breath on those occasions when I fell asleep. I did not realise these episodes were panic attacks. Panic attacks happened to someone else and this shortness of breath happened most days, so surely that couldn’t be a panic attack. The anxiety drove me to a crippling reliance on routine, spontaneity terrified me. If flat mates and friends made an impromptu decision to pop down the pub, I would make the excuse that I had seminar prep I needed to complete. The reality, I just did not have the ability to go with the flow. I also had the tidiest room around, when I chopped food it was all in straight lines and everything in my life was organised alphabetically, the OCD was here to stay.
I never discussed my anxiety with university friends, in truth I was equally ashamed and afraid. I didn’t know something was wrong, I just thought this was PCOS, my reality and I just needed to get on with it. On the outside I could portray a normal life. I was on the university riding team, I did well in my classes and won the law school mooting competition in the year I took part (it’s students pretending to be in court…), I would go out a couple of times a week (those nights I had already planned in my head). All in all, to the outside world I was perfectly normal and too busy juggling life to consider a serious relationship of any form. The truth, I was a commitment-phobe, to be in a relationship with someone meant that I had nowhere to hide and hiding in plain sight is what I did best. Don’t get me wrong I enjoyed my university years, I made some brilliant friends who I am still close to and came away with a first class honours. The people I couldn’t remain close to were those who lived with me as they were the people I spent three years hiding from.
If this article teaches you anything, from my experience and from talking to others, one thing cushies (what people with Cushings refer to themselves as) do better than most, is learn to hide a part of themselves. They do not know how to explain the hollowness you feel inside, instead mimicking whilst in reality it is like you are watching your life rather than living it.
The year I graduated (age 21) I had my first bone scan. My mum took me and I completed the questionnaire somewhat unsure how to answer the question “at what age did you start the menopause”… erm I haven’t even started a regular period yet? Next the nurse came out to take me in and went to take my mum instead, apparently girls my age don’t have dexta scans. After that awkward event I was told I had osteopenia the pre-curser of osteoporosis. If the condition makes you think of your elderly grandparent then you are right. It is the deterioration of bones generally linked to those who have enjoyed a long life. I was warned that this was something that would continue to deteriorate and the pain would only get worse. Woop woop I knew how to live life.
By this age my parents had seen my insomnia, they had seen how every year when I studied for my end of year exams my leg muscles would waste away. It would get so bad I had to go to the physio in the summer as my legs would randomly give way whilst I was walking. My parents had reluctantly supported my diet, knowing that if I ate more everything got worse. Clutching at straws and trying to find anything that could help they suggested I tried acupuncture. Given their unconditional support I decided to humour them despite being a complete cynic. After my first session I slept for eight hours. I couldn’t remember the last time I had slept through the night, it was bliss. I had acupuncture for 6 months and in fairness it started to control my nerves. It is probably the reason I survived my training contract as well as I did.
Disclaimer — my training contract was not plain sailing either. There were many tears when I locked myself in the toilet, there were blood tests because of exhaustion (official diagnosis — my body was shutting down because of stress) and there was a paranoia that I will never be good enough which drove me to take on more work than I should.
Once I qualified I moved to Birmingham, I had a habit of running, constantly believing that the next place is where I would finally feel like I fit in. As I was now at a new hospital they decided to redo my dexta scan and this time round the deterioration had taken place, I had full blown osteoporosis and the doctors couldn’t explain why. I found this out sat at my desk in work when a receptionist accidentally told me over the phone. I hid in the toilet and I cried. Because there was no explanation and because I was so young the consultant decided to start me on an experimental course of treatment. I began bi-annual injections which were supposed to help strengthen my bones. Traditionally these are only used in a short six week burst for patients who have had chemo, the idea here was that I would be on them for life. Every time I had the injection, I would be wiped out for about a week. The drug was so strong that you could not try for a baby unless you had been off them for six months as it could mutate a foetus — not an issue given I was told I couldn’t have children. This cycle went on for four years.
Whilst in Birmingham (only one year in total), I had a cancer scare in my ovaries (quite common if you don’t have periods), but whilst waiting for the test I decided I wanted to come home to Manchester. Birmingham became the city where I got sick, so I arranged some interviews and planned my departure. The day of my test my mum had to come and play taxi to me. I went for tests in the morning (a fairly grim procedure where a form of acid pumped inside my uterus) after which I are meant to go home and rest. Instead I got straight in the car and my mum drove me up to Manchester, that afternoon I had two interviews with international law firms. I sat at an awkward angle ignoring the pain, but by some miracle I got one of the jobs and I was able to come home. Still looking for somewhere I would belong.
Over the next couple of years nothing of note happened medically, I continued the dieting, every time I looked in the mirror I would just see fat (I have understood more latterly that a degree of this is body dysmorphia and trying to understand my true reflection remains a challenge) , I intermittently struggled with sleep and the anxiety was a constant companion. At times the anxiety got so bad I started losing my hair. Needless to say I still felt a misfit. When I was 27, I decided I was tired of going to Birmingham for my injections and decided to move them up to Manchester. In order to do this I had to have an interview in a local hospital and this was where I met Dr S, the doctor who changed my life.
Having run through my medical history, Dr S decided that something didn’t add up, to put her mind at ease she wanted to test for some steroids. Not to worry she said, there was a one in a million chance of the issue she was looking for, but still she wanted to be sure. I duly went for my bloods and didn’t think much more about it. Another phone call at work, but this time I was told my steroids were through the roof, I was showing signs of something called Cushings. I froze and I phoned my dad. What was this? How could something else go wrong? Once again I was crying on a fire escape hoping no one would come out. My dad went away and researched, within an hour he phoned back… he simply said that I match the symptoms perfectly, yes this was scary but it was also the first time in my life I had a cure, I had hope. I say I had hope but there was also heartache.
After being told I had a head tumour I questioned why I would have surgery. I had been told the tumour was benign so out of fear I asked the doctors why on earth would I opt to have a drill through my face?!?! In that moment my parents and I were told quite plainly that although the tumour was benign, I was dying from the steroids attacking my body, surgery was my only choice. Let me tell you this, there is nothing that prepares you for watching your parents be told that you are dying. There is no way you can heal the hurt you caused in that moment even though it was never your intention. The look on their faces will forever break my heart.
After weeks of denial — head tumours happened to other people not me — I was able to accept that maybe this was the start of a change. (Side note: if you have Cushings due to your own body and not by taking a steroid drug it can either be caused by a head tumour or an issue with your adrenal glands, mine was the former. Symptoms the same only the cause, surgery and recovery are different.)
The other thing you learn with Cushings is that nothing is ever easy. Diagnosis takes an average of18 months, it was an achievement to be on the operating table within 16 months. I had multiple blood tests, x-rays, CT scans and an MRI. The most challenging test was an IPSS. The purpose of an IPSS is to take blood samples from inside your head to work out which side of your pituitary the tumour is on. The tumour is a matter of milimeters and therefore easily missed on a scan.
In order to take the samples you are in theatre for three hours, fully conscious with a team of 15 doctors, nurses and phlebotomists. You are fully conscious whilst three tubes are fed into your arteries, one in your arm and two going to either side of your pituitary gland (which sits just behind your optic nerve). The tubes fed up to your head go in at your groin, then using live x-rays are navigated through your arteries up to your head. As it passes your ears I remember the sensation of my ears popping as though I was on a plane. Once in my body I was fine, but feeding the tubes in was an issue, I had maxed out on local anaesthetic but could still feel the pain, there was no way of getting through my right groin without hitting the nerve endings to the top of my leg. Needless to say the doctors increased the anaesthetic and the senior anaesthetist stayed with me throughout. After an hour of interval bloods the tubes are removed and I had two fully grown men spending ten minutes leaning on my groin to prevent the bleeding. Modesty is not a thing. You are then taken to the day ward where you have to lie completely flat for another couple of hours and that evening my dad could come and collect me. I hobbled out of the hospital head held high adamant I could walk, by the time I reached the car park I was crumpled on the floor crying from the pain. With my dad’s help I got to the car curled up and was swiftly in bed. The next day my arm was a mixture of blue and purple from elbow to wrist and I could barely use my hand. It was over a week before I felt stable enough to leave the house. My confidence gone.
After that final test I was able to start steroid suppressants whilst I waited for surgery. The first couple of weeks was a mixture of memory loss, slurring words and nausea. But once I got through that I started to sleep a little better and my weight dropped a little bit. The numbness didn’t go away, so although I never felt happy, at least it curbed the anxiety. I was hollow, I was going through the motions clinging to my routine. I had waves of panic and regularly cried uncontrollably, but I finally knew that this wasn’t me it was the condition. I would get so worked up I would start full body tremors and cry, but instead of hiding it I was able to be honest with my family who supported me throughout. I had learnt the hard way not to trust work, after backhanded comments, passive aggressive management and jumping from job to job due to lack of understanding feeding my anxiety so I kept my diagnosis as private as possible.
I was on the drugs for six months before surgery and then next thing I knew I was in the hospital in my gown waiting to be called in. After six hours of surgery I was in the recovery ward. I don’t remember much about this time, I am told by my parents that unlike the anticipated 30 minutes on this ward I was there for three hours. I remember the confusion and that I asked Dr G (my incredibly kind, patient and supportive neurosurgeon) if the bag on the side was his lunch bag (it seemed important at the time) and I remember that the vomiting began. From the recovery ward I was transferred to intensive care, my home for the next four days.
The first night is a blur, my parents came to see me but I don’t remember seeing them; so I have to take their word for this… I managed to mumble a few words but mainly I was hooked up to a lot of monitors which flashed predominantly red or orange and rarely green, there was lots of beeping, my nose was bandaged yet the blood was still coming out of my face, I was on an oxygen mask to help me breath and the nurse lost my pulse. You see that is the thing with Cushings, if your surgery was a success your body crashes. You are a druggie going cold turkey. Your steroids plummet in an instant and your body doesn’t know how to cope. But that was rock bottom, from that point my five years of recovery starts (spoiler alert I am almost two and a half years in so I am still working it out!).
Now there you have it, full disclosure and in the words of the Greatest Showman This Is Me not to mention more than enough for one post. Let’s call life with Cushings part one and the road to recovery will come next as part two. If you are suffering from Cushings I hope my story rang true to you and that you will know you are not alone. For those who know someone with Cushings please know your support is everything. So many people couldn’t handle sticking around once the testing began and I could not forgive the people who left. The truth is, it doesn’t matter what you say, what matters is whether you turn up. But more than anything, invisible illness is a thing, many live with it day to day hiding in plain sight. Society needs to learn how to start dealing with it and supporting those of us suffering instead of ignoring it and hoping it will magically go away.
To be continued…
