When I was 16 years old I became very unwell and needed emergency hospital care. What started as flu-like symptoms quickly worsened and within three weeks I was fighting for my life. I don’t remember much but my mum has told me that I was in a coma. Whilst still breathing on my own, I had been unresponsive for a few days and she feared the worst. Fortunately, she pointed out to a medical student that my hands and elbows looked an odd colour. This became the key to my diagnosis and treatment.
Addison’s disease is an autoimmune disease and a rare form of adrenal insufficiency. It is a dysfunction of the adrenal glands (two small organs above the kidneys) that impairs their ability to make cortisol and aldosterone. It is now estimated to affect around 8,400 people in the UK.
President John F Kennedy is the most famous person known to have had it. He had the telltale tan, which is when the skin darkens (hyperpigmentation) due to a lack of cortisol and it was this ‘tan’ my mum noticed on me. A few friends noticed too and asked if I had been on holiday. I hadn’t but I didn’t really think anything more of it.
My other symptoms were muscle weakness, low energy, gradual weight loss, changes in mood, a need to sleep, as well as vomiting, diarrhoea, and severe abdominal cramps. On reflection, these were creeping in about 12 to 18 months before but I put it down to studying for college and tiredness.
A LIFE ON STEROIDS
I started steroid replacement therapy via drip and injections and was told I would need to take them for the rest of my life. It was a few weeks before my 17th birthday and the idea of taking tablets forever was hard to imagine. Steroids bring their own challenges and side effects, but I’ve taken my tablets ever since. I now also take iron and vitamin D supplements as well as receive B12 injections. It’s common to develop other deficiencies or autoimmune diseases and I’m reviewed annually for these.
Cortisol is life essential. It’s part of our fight or flight response that kicks in to protect us from stress. Mainly physical stress (e.g. viral infection or injury) but emotional stress (e.g. bereavement) can also be a factor. It’s vital in keeping blood pressure and chemistry stable and we can’t live without it. I take hydrocortisone to replace my cortisol and fludrocortisone to maintain my salt levels. My salt can drop quickly so you’ll often find me topping up on olives and tortilla chips.
Cortisol is also the magic substance that gives us our ‘get up and go’. In a healthy person, it builds through the night so you have energy on waking up and reduces in the day so you can sleep. My doses are split to try to mimic the circadian rhythm that occurs naturally. It’s also imperative to take the right dose at the right time and missing or dropping doses has consequences.
Healthy adrenal glands know when to make more cortisol and aldosterone. However, I rely on my medication to do this, increasing my dose when I need to. Each person is different and whilst dosing tends to be based on age and body mass, there’s no one size fits all. I recently heard a doctor on a Zoom event say ‘Treating Addison’s is as much an art as it is a science’. He summed it up perfectly.
When my body is under stress it can’t always cope and if I can’t replace my cortisol levels quick enough it starts shutting down. This is known as an Adrenal or Addisonian Crisis and it can develop very quickly. It’s a medical emergency; left untreated it will result in shock, coma, or become fatal.
I have an emergency kit with a high strength injection to use until emergency services can arrive but it’s tricky to assemble. Luckily I’ve had help at hand when I’ve needed to use mine because using it has saved my life. I also wear a medic alert bracelet and carry a ‘steroid warning card’.
THE HARDEST PART
The hardest and scariest part of having this disease is the ‘not knowing’. I have no way of telling if my levels are right in real time. Over the years I’ve learned to listen to my body but it’s not foolproof. I’d love nothing more than to be ‘healthy’ but I’ve also wished I had a common disease because of the blank faces I get when I try to explain it. I’m proud to work for the NHS but I sometimes feel conflicted as a patient. I’ve lost track of how often a GP has said ‘I don’t know what to do with you’ or how many times I’ve had to fight hard to get the right treatment when I’ve been really unwell even when I’ve had all the advised documents with me.
It’s also extremely frightening to experience an Adrenal Crisis. The symptoms for crisis include ‘feeling like you’re going to die’. I used to think this was strange but now I get it – I did think I was going to die. When I’m in crisis, I can’t speak properly and I can’t think logically. My muscles seize up and my body instantly feels weak. In my flatmates’ words ‘One minute I was there, the next minute I was gone’. I was lucky to wake up but my body and mind did not feel like my own when I did.
Last year I repeatedly battled an adrenal crisis and I’m still here to tell the story. My recovery has been tough and I’m thankful to have family and friends who lift me up. It’s taken patience and determination but I’m working hard to rebuild my life. I’m grateful to be able to work as not everyone can. I work in digital services and I’m fortunate to have an employer who supports my health and allows me to work flexibly. Now that I’m getting stronger, I’m focusing on exercise again and keen to understand more about how our minds and bodies work together. Living with Addison’s disease has taught me I am a survivor. I no longer take anything for granted.
I want to share my experiences so someone else can recognise the early signs or symptoms of crisis. I also hope to raise awareness within medical settings as I believe access to care should be easier. I believe there is a better way to tailor steroids, such as devices like the cortisol pump, but this device isn’t freely accessible on the NHS. More than anything, I hope one day my disease will be cured.