‘It came as a terrible shock to be made so suddenly aware of my mortality and fragility when I was in my 20s. I have always said I don’t want hypoparathyroidism to define me but in reality it is a huge and permanent part of my life. I’ve lived a lot of half days.’
Emma has lived with permanent post-surgical hypoparathyroidism after surgery for Graves’ disease for over 20 years.
In 1991, Emma was a student nurse in her second year at UCH & the Middlesex Hospitals in central London. She was very physically active, swimming two miles every other day, playing tennis, aerobics, skiing and working towards her sport diver certification, when she started to feel unwell.
‘My first indication that something was very wrong was when I went to give blood and was declined as an initial test showed I was extremely anaemic. Shortly after, I developed a goitre (enlarged thyroid making a swelling at the front of my neck) and started to feel generally very unwell, I saw a local GP who sent me for blood tests, but while I was waiting for the results I collapsed whilst at work on the ward, ending up on a heart monitor in the casualty department after being rushed there on a stretcher trolley. Blood tests confirmed the diagnosis: Graves’ disease (an autoimmune disease which attacks the thyroid causing it to become over-active). I experienced weight loss, muscle wasting, elevated and erratic heart rate. My metabolism, hair, nails, skin and eyes were also affected. Unfortunately the antithyroid drugs were not effective in my case and I started to show signs of thyroid eye disease, so after nearly a year of medical treatment, the Endocrine Consultant arranged for me to have surgery.
‘The surgeon explained I would have a total thyroidectomy. I was told about the possible but very ’unlikely and rare’ complications of surgery which could be vocal cord damage or parathyroid damage, which if it occurred, was usually temporary and if not could be ’treated by tablets’. I signed the surgical consent form accepting that both were a possible but unlikely risk.
‘I was in rough shape after the surgery despite being young and relatively fit. As I started to recover I had the sensation that my whole body was tremoring or juddering. I asked the staff to pull my bed slightly away from the wall as it felt like it was vibrating and I couldn’t sleep though exhausted. But it was me that was shaking, not the bed. The staff put it down to my body being in shock from the long surgery. Overnight I started to feel numb tingling in my fingers, hands, lips and nose and became very nauseous and sick. My vision was shaky and blurred.
‘The next morning I was assisted to walk to the toilet. Whilst in the locked washroom I started to feel faint and dizzy and get weird muscle contraction and extreme pain in waves like when you have bad cramp or an electric shock. It started to affect all of my body and within seconds I was having trouble breathing. I hit the emergency button and collapsed against the door and managed to turn the lock with hands like claws as I slid down to the floor. The staff arrived instantly and forced the door open as I was against it. It took several staff to lift me in a solid, clawed, cramping state onto a wheelchair which they hurriedly pulled backwards to my bed as my legs were rigid straight and feet extended back like my ankles were going to break. I saw my face in the toilet mirror as I was lifted up, it was a shocking sight (later a haunting memory), my ashen face with bloodshot eyes, contorted into an impossible and hideous grimace, like the Joker wore in Batman. I was in excruciating pain, it felt like my muscles cramping and contracting were literally going to break my bones and snap my ligaments. I couldn’t speak as my jaws were awkwardly clamped shut with such strength that I thought my teeth would break and my throat felt clamped shut. It is impossible to imagine that your own body and muscles could do that to you.
‘I started to vomit on my back through clenched teeth as my jaws were still locked shut. The staff gave me an intravenous anti-sickness drug. I could hear staff outside the room in raised urgent voices saying ‘Call the surgeon now! And TELL him!’ Then I heard them relaying an incredulous message from the surgeon saying ’give her a glass of milk and it should pass’ (???!!!!!). The junior doctor on duty went to the phone to explain just how severe a tetany seizure I was having. A nurse did try to give me sips of milk but I just choked through gritted teeth and it was deemed impossible and dangerous in my condition.
‘The staff were extremely professional, a senior nurse who knew me from training gently took my clawed hands and held my gaze as she continued to talk to me trying to reassure me, I am so grateful to her and all the staff’s prompt action. Another nurse had a calcium drip already out and prepared in the treatment room as a precaution because I had had a total thyroidectomy. I was so relieved she was trained well, took prompt action with the doctor and was aware of the potential of post-operative tetany. They struggled to get a drip line in – my veins had shut down in my hands and my arms were contracted fully at the elbow to the point that they couldn’t open them to get to a vein. Eventually a doctor managed to access a vein on the top of my foot. My body started to relax out of the tetany as the IV calcium flowed round my body. The sense of relief and utter exhaustion I felt when it passed was enormous. I was so exhausted emotionally and physically – the doctors said it was like all my muscles had just run a marathon in half an hour!
‘As the days passed it became apparent that my parathyroids were not recovering. They had omitted to test my calcium prior to the operation so had no baseline, but the medics thought my calcium must have been very low preoperatively (due to my body being depleted by the Graves disease) which led to such extreme and rapid tetany. I knew it was serious when the day after the seizure three consultants stood in my room, as a nurse I knew either they had found something awful or I was an unusual case or something serious had gone wrong.’
Emma was told that treatment would be straightforward; taking tablets daily – at first active Vitamin D tablets to mimic the parathyroid hormone and effervescent calcium tablets, then eventually just diet and active Vitamin D (’just one small tablet at day’) with blood tests a couple of times a year to check levels were normal and stable. In reality it was not this simple.
‘I stayed in hospital about two weeks as despite daily intravenous calcium my level wouldn’t rise to within the normal range. While recovering at my parents on the south coast I collapsed again but this time due to excessively high calcium. Over the next year I had many IV blood tests and medication adjustments to get my levels in the normal range and balanced. I was so exhausted that I would go to bed as soon as I got in from work, I was unable to exercise and my social life and friendships suffered.
‘At a later outpatient appointment, I was referred to a dietician to discuss calcium in the diet, so that I could ensure a regular, steady amount daily without inadvertent big fluctuations. She met me in a busy hospital corridor for a fifteen-minute ‘chat’ but didn’t know anything about hypoparathyroidism or that she was meeting me because of it. I found this disconcerting and it added to my feeling of isolation and hopelessness as I still felt so unwell. She gave me a sheet with calcium amounts of regular foods – mainly dairy and a limited list. So I had to educate myself on calcium content of foods and water to ensure I ate a steady amount each day to avoid fluctuations and learn how to follow a low phosphate diet as I later discovered hypoparathyroidism meant my phosphates could become too high.’
Emma qualified as a nurse and was awarded the Fairmark Prize at her nursing graduation for outstanding achievement in the face of adversity, but had to leave shortly after as she was still feeling very poorly which made working in a hospital environment extremely hard both physically, mentally and emotionally. ‘It was a heartbreaking decision to make as I had planned a career in nursing and wanted to become a clinical tutor’, she says.
‘It took two years to get my calcium level stable. In hindsight I look back and realise I battled with low levels and drops ‘crashes’, causing extreme exhaustion, tingling, cramping, stiffness and feeling very low or depressed which is as a result of the chemical imbalance together with the trauma I had experienced. I lived in fear of having another extreme tetany seizure. Three years after surgery I still lived with constant anxiety. I was offered a counselling service, but only after I pursued it with my then GP, however I would have had to pay for it which I was unable to afford as I could just about pay my rent and bills at the time. So I just got on with life and tried to teach myself ways to manage the emotions that came with the territory. To the outside world (work and friends) I was smiley, hard working and well – but inside it was a daily struggle.
‘When I moved my new GP referred me to a local specialist in the field – it was best thing that has happened to me so far in this journey, he is excellent and very supportive. I still see the same Consultant Endocrinologist now after 14 years. My parathyroid hormone levels were tested when he initially took over my care, and they showed I have no parathyroid hormone production at all. So even if all four parathyroids were counted and left in situ (as the Consultant Surgeon stated) the blood supply to all four had been damaged.’
Under his care Emma’s levels got into the normal range and remained relatively stable so that she was able to start a much longed for family. Six years after surgery she had her daughter (a month early), and 22 months later her son (two months premature).
‘They think the early births were due to my condition and the pregnancies were physically very demanding with lots of migraines, nausea and extreme exhaustion. My bloods were done more frequently during pregnancy and my medication levels increased especially towards to end of the pregnancy as the babies grew. I was seen by both my Consultant and a Consultant Obstetrician at hospital throughout the pregnancies. After each birth I remained in hospital and my levels were checked before I was discharged, I fully breastfed both my children for over a year each.
‘I have had both highs and lows. After the first few years I achieved fairly steady calcium levels but they were on the low side for most of the time. I currently see my Consultant two to three times a year depending on how I have been. I can have a test in between appointments if I feel unwell. My GP sees me every six months for a medication review and to keep them ‘in the loop’ regarding information from my Consultant. Letters from my Consultant after each appointment go to my GP and a copy to me.
‘Last year my medication was increased to try to lift my calcium which had been low for a long period. Over the following months I started to feel very unwell and went for an early blood test and learned that my calcium and phosphate levels had become too high and my kidney function had been affected. It took several months to recover from this episode and I feel I am still not fully recovered. I felt cross with myself that I missed the symptoms even as an ex-nurse and having had the condition for 20 years – I wasted a lot of time in the past year being exhausted and unwell in bed. I do also worry about the effects on my kidneys and possible damage.
‘I strongly feel that the positive, supportive relationships and continuity of care over the past 14 years I have had with my Consultant and GP have been essential in ensuring my condition has been managed as well as it has. I know that my health is dependent upon correct management and monitoring and emotional support.
‘I have always maintained my mantra to live life to the full and keep as well as I can and help myself as much as I can. I worked in the busy world of financial PR for a few years. In periods of busy stress, my calcium level has dropped and I required higher levels of medication. Hypoparathyroidism and managing it in the early days definitely held me back from pursuing a career I returned to the sports I love like skiing (though altitude, physical exertion, and temperature seem to all have an effect on lowering my levels which at times has been problematic). Sadly I was unable to continue to scuba dive as the effect on my levels at depth pressure is unknown and I wouldn’t get covered to dive in case of tetany underwater, and although I wouldn’t want to risk that, for a few years after surgery it made me very sad that I could no longer pursue diving and I missed the social side of the club and the friends I had made. My consultant is very supportive and endorses my attitude of ‘can do’ rather than having my condition hold me back. When I trained intensively for triathlons five years ago he encouraged me and said he would adjust and monitor my meds accordingly. But that is all dependent on me being able to get to the hospital for the necessary blood tests and the inconvenience and time involved when working and running a busy family life. I recently did the Moonwalk Marathon (overnight speed walk marathon for breast cancer charity). I love hiking and took my kids up Snowdon last year. I regularly run with my beloved rescue sled dog Alaska, who I think saved me more than I saved her as she got me back on track after a very low period when I had become exhausted and unusually inactive, gained too much weight and gave up pushing through the discomfort of the rigid tight muscles I get in order to exercise. Alaska is my guardian angel!
‘I live in a ‘House of Cards’ where a simple stomach bug or infection can lead to numerous knock-on problems for me. In the past five years I have also had a surgical knee and shoulder repair, both sporting injuries. I am now more careful with myself as the anaesthetic really takes it out of me. I realise I have to be fit, and work on my flexibility to cope with the muscle stiffness and rigidity I am now prone to. I follow a low phosphate diet and don’t drink carbonated drinks or much alcohol as they lower my calcium. I tend to try not to let hypoparathyroidism hold me back but I have to rest a lot more than before – I am sure it is my ability to rest and have a relatively stress-free life that has helped my situation. Luckily I have a best friend who is a senior sports rehab physiotherapist and has helped me a lot over the past ten years. I always wear a medic alert bracelet and close friends I do any skiing or sport with know I have a condition and where my bracelet is should I have an accident or emergency.
‘My family didn’t know much about my condition until I was able to give them the information leaflet from HPTH UK last year so I have never been ‘mollycoddled’ or treated differently, which in a way has made me more resilient but has also left me feeling very lonely and misunderstood at times. Joining the HPTH UK Forum and Facebook page made me realise that others feel the physical and mental symptoms I do which made me feel not so alone and that I wasn’t exaggerating it in my mind, and that although you can’t tell from my outside appearance, it is very real and at times debilitating.
‘Finding HPTH UK on the internet and making contact with Liz two years ago was the first time I had encountered anyone else with the condition or had any support from a group specific to Hypoparathyroidism. HPTH UK, its forum, Facebook Group and global internet community of fellow hypoparathyroidism patients and partnerships with specialists in the clinical and research field, has been immensely important and invaluable, improving the lives of those with this condition by promoting research and patient advocacy. My involvement with HPTH UK has greatly benefitted both my physical and emotional wellbeing. Through HPTH UK I have gained further research-based knowledge in order to manage my condition better from both a patient and clinical perspective. I have also received support and validation from people who really know what it’s like to live with this condition.
HPTH UK and Liz’s motivation and dedication has not only enhanced and improved my life, but it has offered me informative research updates and hope that in future treatment and management of this condition will be greatly improved. Hypoparathyroidism is the only endocrine condition without a hormone replacement therapy or means of home blood testing or monitoring.
‘It came as a terrible shock to be made so suddenly aware of my mortality and fragility when I was in my 20s, in my prime and just starting out on adult independent life. I am truly aware that your health affects every aspect of life and although I am conscious to not blame every time I feel unwell on my condition and there are those who live with more life-limiting ill health and bigger problems than mine, nevertheless I can’t help but feel that a lot of my life over the past 20 years has been wasted feeling exhausted and ill. I’ve lived a lot of half days.’