Wendy Exley, from Bramley, lives with a condition known as adrenal insufficiency, which leaves her needing emergency injections to stabilise her condition. An incident in Bramley Park left her unable to manually mix the injection herself – with consequences that could have been life threatening if it wasn’t for a local vet.
Her question: Why isn’t there a ready made injection available? Here, Wendy tells her story to reassure others they are not alone – and is calling for an urgent solution.
My diagnosis with adrenal insufficiency came the day after having surgery to remove my right adrenal gland. A CT scan had picked up a tumor on my adrenal gland and after having blood tests I was diagnosed with MACS.
Mild Autonomous Cortisol Secretion (MACS) is when the adrenal gland produces too much cortisol. Cortisol is a toxic hormone and affects every cell of the body, and can impact health significantly through weight gain, particularly around the abdomen, thinning of the skin, fatigue, muscle weakness and high blood pressure. The hormone imbalance can also have a detrimental effect on mental health, leading to anxiety and depression, mood swings and irritability.
The only curative treatment is surgery to remove the adrenal gland.
So surgery was my only option. I was advised that surgery may not stop my symptoms, and that there was a chance my remaining adrenal gland would not work straight away, but I could take steroid tablets.
I was discharged from hospital two days later, with secondary adrenal insufficiency.
Adrenal insufficiency, or Addisons Disease (known as primary adrenal insufficiency), is a rare condition that requires daily steroids to replace hormones the body can no longer produce. In the events of illness, surgery, trauma or increased stress, the dose has to be increased so the body can deal with the extra demand.
Alongside adrenal insufficiency comes adrenal crisis, a life-threatening emergency when someone with adrenal insufficiency can not produce enough cortisol, which is essential for survival.
When you are diagnosed with adrenal insufficiency you are prescribed an emergency hydrocortisone injection kit, along with steroid dependant warning cards, a steroid treatment card, sick day rule sheets, paramedic paperwork and letters to carry medical needles on board air crafts.
The day I recently had to use my emergency hydrocortisone injection, started as most others. I was walking the dog in Bramley Park. Reeva is my springer spaniel and loves nothing more than chasing and finding squirrels on the park, even climbing trees to try catch one, and that day she did, a baby squirrel. I shouted at her, to drop it, the squirrel was alive, but in destress, so I scooped the squirrel up and took it to the local vets. As I started to drive the short distance, the squirrel leaped off the car seat and went underneath it.
Arriving at the vets after a short but very stressful drive. The squirrel was wedged under the car seat and had its leg stuck in the car seat sliding tracks. A vet helped to get the squirrel out and took it into the back of the veterinary practice, to assess its injuries.
I could instantly feel my heart pounding, I was sweating and felt weak. I explained my condition and asked for a glass of water. I took some oral steroids which I carry in my emergency bag and hoped it would help. After a few minutes I started to feel faint, hot and clammy and started to shake uncontrollably. I asked the receptionist to call 999 for advice and to see if I needed to use my emergency hydrocortisone injection. The practice manager took me into a consultation room and explained to the vet my condition, which is similar in animals.
I was advised to use my injection and that an ambulance was on its way. The vet sterilised the table so I could lay my injection kit out. I was shaking so much by now, that I couldn’t even hold the glass ampel, never mind draw it up and mix it. The practice manager read the instructions out and the vet prepared and mixed it up so I could administer it. As this was the first time I had used it I was nervous and shaking badly, but the manager calmed me down and talked me through it.
The question is: why is there not a ready made injection, like an epi-pen, available? How are people expected to self-administer the injection whilst in adrenal crisis and whilst directly affected by severe symptoms such as vomiting, pain, drowsiness, confusion, shaking uncontrollably, and loss of consciousness?
Addisons Disease Self Help Group, a charity, explains it is down to manufacturing issues. The permanent discontinuation of pre-mixed liquid hydrocortisone means the future of UK emergency kits now have to rely on powdered formulations. Community campaigns from the pituitary foundation and the Addisons Disease Self Help highlight how you can help.
Addisons Disease day is celebrated on May 29, as this would have been John F Kennedys birthday. The US president managed his Addisons in secret. Tragically he was assassinated on 22nd November 1963 while campaigning for re election. JFKs complex medical history was only released to the public years after his death.
Having a rare disease means constantly having to advocate for yourself and educate others.
The hope of sharing my story is to remind others living with a rare disease, that you are not alone, and to enlighten others, that you never know what someone is dealing with everyday.
For more information on how you can help and get involved visit the Addisons Disease website.
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