Addison’s disease can be a life-threatening condition, but it is often not diagnosed until weeks or even months after the first vague symptoms present themselves. We spoke to one of the UK’s top endocrinologists, Dr Mark Vanderpump, to find out what symptoms patients should be aware of.
What are the adrenal glands and what are they responsible for?
Located at the top of each kidney, the adrenal glands are triangular shaped hormone-producing glands which regulate several important functions of the human body. They secrete the following important hormones:
1. Glucocorticoids - these help regulate blood sugar, blood pressure, fat & protein metabolism and immunity. The most important glucocorticoid is cortisol. This is vital for life because it helps your body respond to stress, infections, physical accidents and surgery by:
- Maintaining blood pressure
- Maintaining the function of the heart and blood vessels
- Slowing down your body’s immune inflammatory response
- Regulating your metabolism
2. Mineralocorticoids – these help to regulate the kidney and heart function. The most important of these is aldosterone which stimulates your kidneys to absorb sodium which regulates your water/salt balance.
3. Catecholamines – these help to regulate your “fight or flight” response
4. Adrenal androgens – these are precursors to sex hormones such as testosterone and oestrogen.
What is Addison’s disease and what are the early symptoms?
Addison’s disease is an adrenal gland disorder resulting in low cortisol levels, which can be fatal if left untreated. It was diagnosed by Dr Thomas Addison in 1855, who described a condition caused by tuberculosis infection of the adrenal glands. It is now usually caused by an autoimmune destruction of the adrenal glands. You may see this condition referred to as primary adrenal insufficiency.
Addison’s disease can occur at any age, but most people are between 30 and 50 years when it develops – there are approximately 8,000 UK patients at any one time.
Unfortunately, the clinical signs and symptoms are slow at onset and don’t appear until 90% of the adrenal cortex has been destroyed. During this time, the patient is likely to have experienced the following:
- generalised weakness
- low blood pressure when standing
- chronic fatigue
- weight loss (mostly from anorexia, partly from dehydration)
- extreme sensitivity to drugs such as narcotics
- being nauseated and even sick
- craving for salty foods
- cramps and pains in muscles and stomachs
- spasmodic bouts of loose bowel movements or constipation
- irregular or ceased periods
- loss of axillary and pubic hair
How is Addison’s disease diagnosed?
Because these are generalised symptoms which could each relate to many other conditions, it is believed that more than 60% of patients have seen two clinicians before Addison’s disease is diagnosed. This can take weeks or even months after the vague symptoms first present themselves.
Unfortunately, many people don’t get a diagnosis until they are in a life-threatening adrenal crisis brought on by stress caused by illness, trauma or surgery. At this point these signs and symptoms will be present:
- dehydration, abnormally high blood pressure or shock which is out of proportion to the severity of current illness
- nausea and vomiting with a history of weight loss and anorexia
- severe abdominal pain mimicking acute disease or injury to an internal organ
- unexplained lack of glucose in the blood sugars
- unexplained fever
- investigations including abnormally low sodium levels; higher than usual potassium levels, higher than usual calcium levels or higher than usual white blood cells called eosinophils
- other autoimmune endocrine deficiencies such as hypothyroidism or type I diabetes, or family history of organ specific autoimmune endocrine diseases.
Once in adrenal failure there will additionally be skin pigmentation changes which manifest around:
- the creases of the hands
- gums and the inside of the mouth (which starts to look bluish)
- recent scars
- pressure points like elbows
How is Addison’s disease treated?
For those in an adrenal crisis, urgent treatment with hydrocortisone injections and intravenous rehydration with fluids are essential.
For Addison’s patients treatment involves some form of hormone replacement therapy. This is normally through the medication hydrocortisone which, taken in divided doses during the day, replaces the cortisol your body is not making. Usually fludrocortisone is added to treat the associated mineralocorticoid deficiency.
What is the outlook if you have Addison’s disease?
People with Addison’s can expect to have a normal life span; it is not infectious nor is it easily inherited. There is no need to adopt a special diet, nor are there any dietary restrictions – although a low salt diet is best avoided.
However, your treatment is life-long and it is essential that patients carry a warning card or Medic Alert bracelet to warn medical staff that steroid treatment is required in a medical emergency.
You can attain a normal level of fitness, but you may need an increased dose of medication if you are involved in challenging physical exercise. Even those who are very fit may experience severe fatigue from time to time, and you will need extra rest during these periods.
Finally, women can expect a healthy pregnancy and normal childbirth, with medical monitoring.
Dr Vanderpump treats a wide range of endocrine conditions at clinics across north and central London. To book a consultation with Dr Vanderpump, click here .