Honeypea House

Living with Addison’s disease

What is Addison’s?

Addison’s Disease or Primary adrenal insufficiency is a rare endocrine condition where the adrenal glands stop working and your body no longer produces enough of certain steroid hormones, namely Cortisol and Aldosterone though other hormones such as DHEA may also be affected. This condition can be life threatening, if not properly managed you might go in to crisis. An adrenal crisis is a life-threatening emergency and requires immediate medical treatment. The person needs to be given an emergency injection of hydrocortisone straight away and ambulance called.

What effect does this have?

Cortisol regulates appetite, blood sugar and food metabolism
Aldosterone regulates sodium, fluid balance and blood pressure
DHEA influences body hair growth and libido.

Most people with Addisons will at least take steroids to replace their Cortisol but replacement of all three steroid of the mentioned steroid hormones may also be necessary.

What are the symptoms?

People with adrenal failure usually have some/all of the following symptoms prior to diagnosis:

  • overwhelming fatigue and lethargy
  • increased sleep
  • weight loss
  • skin hyperpigmentation (like a sun tan even when they haven’t been out in the sun)
  • dizziness on standing
  • low blood pressure
  • poor appetite
  • nausea or vomiting
  • difficulty concentrating
  • muscle weakness with cramps
  • salt cravings
  • headaches
  • stomach pains

I experienced all but the skin hyperpigmentation, I think that missing this symptom likely delayed my diagnosis for quite some time.

How do you get diagnosed?

The main diagnostic blood test used is the short Synacthen test (also known as an ACTH stimulation test). This measures the ability of your adrenal glands to produce cortisol and involves two blood samples being taken. The first is a ‘baseline’ reading to measure how much cortisol is in your blood. You are then given an injection of a hormone called ACTH (this is the hormone normally produced by your pituitary gland to instruct your adrenal glands to make cortisol) and a second blood sample is taken 30-60 minutes after this. The second sample is compared to the first, if there is not a significant rise in the level of cortisol in the second sample then there is reason to believe that the adrenal glands are not currently working effectively.

Additional hospital blood tests will measure your aldosterone function to determine the need for replacement in the form of Fludrocortisone. These tests include: plasma renin, sodium & potassium tests. Further testing to look for adrenal antibodies may also be included, these are not used as a sole diagnostic test but rather adding to the greater picture of the cause of the adrenal failure.

The long term picture

In the long term regular testing of sodium, potassium and renin will often continue to monitor if your dose is effective. Other hormone levels will likely be checked and regular monitoring for other autoimmune diseases. Vitamin and mineral levels are often checked and maybe monitored long term, certain vitamins are often found to be low on diagnosis.

People with Addison’s should also be prescribed an emergency injection kit in case of adrenal crises. If you are a family member or close friend of a person with Addison’s, you may find it useful to learn how to give this injection in case of emergency.

It is also important to sign up with your local ambulance trust and get “red flagged” different trusts do this in different ways so call them up and ask for their procedure.

It is advised that those with Addison’s also get a medic alert type bracelet, in case you are unable to communicate this will allow medical professionals to know that you have Addison’s.

People with adrenal insufficiency will require regular flu jabs as the flu is a common cause of crisis.

Due to the long term steroid use DEXA bone density scans might also be required to assess risk of osteoporosis and need for any medication or intervention.

Adrenal Crisis

Symptoms can vary from person to person and you may not have all the symptoms as you experience a crisis. A good list found on the NHS website:

  • severe dehydration
  • pale, cold, clammy skin sweating
  • rapid, shallow breathing
  • dizziness
  • severe vomiting and diarrhoea
  • severe muscle weakness
  • headache
  • severe drowsiness or loss of consciousness

A crisis requires medical intervention, if you think that someone is going in to crisis then they will require an ambulance and if you are willing and able then an injection of hydrocortisone.

Treatment

It is essential that people with Addison’s take their medication every day, at the right time of day because these tablets are replacing something that your body cannot make anymore.

Receiving ongoing medical support, with regular appointments with their endocrinologist to review their condition is also vital. As Addison’s disease is a rare condition, sometimes other medical professionals such as GPs or nurses aren’t very familiar with it or with the need for daily steroid medication. Often the Addisonian will be the only person within their GP practice with Addison’s disease.

Whilst the majority of people who are diagnosed with Addison’s are able to live a fairly normal life, just with the addition of medications and an emergency kit, this has not been the case for me.

What happens when tablets aren’t enough

A very small minority of those with Addison’s have further medical conditions or issues which can make effective treatment difficult. In this case it may be worth pursuing treatment with a cortisol pump. This treatment is not standard, is it very rarely available on the NHS and further research is being undertaken in to the differences it can make to those with Addison’s.

Getting the pump

Getting a pump is worth a whole blog post on its own. As I am still in the process of getting a pump I will write about this further down the line.

How does my Addison’s effect me?

Due to a number of factors I do not get a nice, predictable amount of cortisol from taking my tablets. Sometimes I might experience a long delay before I get a rise in my Cortisol, sometimes taking the same dose I always take does not work and I am forced to take a higher dose which unfortunately has side effects (namely difficulty in sleeping, extreme hunger and water retention). Because of this unpredictability I don’t know if today or tomorrow will be good days or bad days.

Good days

On a good day I can get up, washed and dressed fairly easily. I can make my own food, eat it without any problems and even go on to do some housework or leave the house.

Bad days

It is hard to describe fatigue and lethargy, I think some kind of tool or scale need to be created to help. What I might describe as extreme fatigue and lethargy could be very different to you.

My fatigue and lethargy on a bad day are beyond what I would have thought imaginable, I will often need assistance to go between the bedroom and the bathroom and that is as much as I can do on a bad day.

Most of my days are somewhere between good and bad, on some kind of a sliding scale of rubbishness. I am hoping that with appropriate treatment I can have fewer bad days and maybe even improve on my good days.

Resources

The Addison’s disease Self Help Group is a charity that support people with adrenal insufficiency and their families. They also produce clinical guidance for patients and healthcare professionals and details of emergency treatment in a variety of languages. I learnt more about my condition from their forum than from any other website.

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