Monday, 15 June 2009

Addison's Disease/Adrenal Insufficiency

Ok here we go.

What is it?
Addison's disease was discovered by Dr Addison in 1855. It is a rare condition affecting mainly, but not exclusively, people between the ages of 20-50.
Right, we should all know that we have 2 kidneys. On each kidney sits an Adrenal gland. The adrenal gland is a bit like a Cadbury's creme egg. For example the chocolate is the cortex and the soft fondant centre (yum yum) is the medulla. Cells in the cortex produce hormones called Aldosterone and Cortisol. Together they help to control/regulate the blood pressure, regulate salts in the blood stream and help regulate the immune system. They also help balance the effect of insulin in regulating blood sugar and helps the body respond to stress. The cells in the medulla produce adrenaline and noradrenaline which have various actions throughout the body.
Now in Addison's and adrenal insufficiency the adrenal glands produce little or none of the above hormones. I think I'm right in saying that Addison's is known as primary insufficiency. Now just to complicate things there is a secondary insufficiency. Lying under our brain we have the Pituitary gland. This produces a hormone called ACTH which controls the amount of cortisol produced in the adrenal glands. With me so far? Good.

There are many reasons why we may fail to produce these hormones:
Primary Insufficiency:
Addison's ( >80% of cases), surgical removal, trauma, Infections (TB/HIV etc) and cancer. There are more.

Secondary Insufficiency:
Congenital, trauma, surgery, radiography, cancer, long term steroid use for other conditions and tumours of the pituitary gland. Again there are more.

What ever the cause these patients require life long steroid treatment. They do this by taking oral steroid tablets every day.

What's a Crisis?

Basically anyone who has the above conditions and for whatever reason are unable to keep their oral steroids down or is affected by other factors that increase the body's natural demand for cortisol such as trauma, stress etc. If someone is having surgery then the surgeon usually has to ensure the patient will have enough steroid cover for the procedure. This helps the body cope with the extra stress.

There are many causes of a crisis such as infection/ tummy bugs, stress, trauma and any form of serious illness. If patients vomit or become unwell then they usually double up on their own steroid tablets and if necessary give themselves a hydrocortisone injection. Not everyone is given a home injection kit though. A lot of sufferers manage their condition extremely well even if they start to feel a crisis coming on. Once they feel better then they will slowly wean themselves off the higher steroid doses and back on to their normal dose.
Sometimes people become very ill very quickly and are unable to keep their tablets down. That's when they need an injection. That's when we come in.

People may feel weak, dizzy, have a low blood pressure, rapid pulse and sometimes a low blood sugar. People can have seizures and become unconscious. If left untreated death will occur. It is a potentially life threatening condition. On the outside it could be just someone vomiting but when you find out they have Addison's (and understand the problems that causes) it changes the game somewhat. Be aware, not every one will present with the classic signs of shock. If they can't keep oral steroids down they need Hydrocortisone. The aim is to prevent a crisis not just to treat it when it comes along.

There are some good websites that explain the condition far better than I have. All you have to do is GOOGLE ADDISON'S DISEASE and go from there.

It's amazing reading some of the patients's stories from a few years ago and you will see that back then hardly any ambulance staff knew anything of Addison's. We didn't have Hydrocortisone (HYC) back then. Well we do now which is a step in the right direction.

Some advice though. Make sure you have a medic alert bracelet or some other obvious means of letting us know that you have this condition. If you are an Ambulance Technician (and you can't give HYC) use common sense and ring A/E to get permission to administer it IM. You may just save someones life. Actually I don't know why Techs can't give it nationally, it comes under the same drugs act that covers Glucagen, Adrenaline and Narcan (which enables techs to give these drugs in the first place) All UK Paramedics can now give it IV/IM and where I work via the IO route.

Hope this has helped. I'm waffling now so I'll sign off. Byeeee.

1 comment:

Chris T said...

I know I'm late in responding, but I just discovered your blog. I was diagnosed with secondary adrenal insufficiency 1 1/2 years ago caused by autoimmune pituitary destruction. I have to say you gave the most informative, brief, to the point run-down of the disease I've seen or heard so far. You kept it simple and easy to understand while focusing on the importance of early treatment in order to prevent a crisis. I've had 2 and they are quite scary. I want to add that one of the symptoms is severe lethargy that can lead to coma. You're a wonderful writer. Thank you for addressing this mostly misunderstood disease.