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ADDISONS DISEASE (ADRENO-CORTICAL INSUFFICIENCY)

What is Addison's Disease ?

The adrenal glands are two small mushroom-size structures, one of which sits just above or alongside each kidney in the upper abdomen. The main substance they make is a steroid chemical called hydrocortisone. Hydrocortisone is needed for the correct working of all tissues in the body. It circulates continuously in the bloodstream. With any type of stress, the adrenal glands boost their output, and the hydrocortisone levels rise quite strikingly. In Addison's disease (called after the English physician who first described it), the functioning of both these glands is severely reduced or absent. This results in a shortage of hydrocortisone. Another hormone (aldosterone) produced by the adrenal glands is also lacking. Its normal function is to keep the body's salt and water content at the right level. In the early stages, a shortage of these hormones causes mild fatigue, tiredness and loss of weight. Dizziness can develop due to the low blood pressure produced by their absence. Often the skin becomes quite brown. Loss of salt and severe dehydration can take place later (due to shortage of aldosterone), leading to serious illness and even coma.

How does Addison's Disease occur ?

Usually the condition is due to a process of self-destruction called auto-immune disease : cells called lymphocytes develop the ability to destroy otherwise healthy adrenal cells. Occasionally, tuberculosis or some other inflammation or infection can be responsible for damaging the adrenal glands. Surgical removal of both adrenal glands (adrenalectomy) is sometimes required for other conditions, and will produce the same need for treatment.

Why does Addison's Disease occur ?

The tendency to develop the more common auto-immune type of Addison's disease is often inherited: in fact, other glands (particularly the thyroid) may be similarly affected, either in you or in one of your close relatives.

Treatment Involved for Addison's Disease

The missing hormones need to be taken lifelong. Hydrocortisone (cortisol) is the most often used replacement steroid, usually in doses of 15 to 30 milligrams per day. Normally the tablets are taken as two or ideally three doses each day. These must be taken on a regular daily basis and not left off for even one day. The salt control hormone used to replace the deficiency of aldosterone is a drug called fludrocortisone. This is usually given in doses of 50 to 200 micrograms a day.

During Treatment for Addison's Disease

Fludrocortisone is given day after day without change in dose unless advised by the doctor. However, you may sometimes make the decision to take cortisol in increased dosage (but never reduced). Reasons for increasing the dose include emotional stress, accidents, infections (particularly those causing a fever), and other illness. The reason for raising the dose is to try to copy what normal adrenal glands would do under these circumstances.

Usually the dose of cortisol should be doubled for a mild stress-like problem, or tripled for a more severe disorder (e.g. a high fever). It must be kept at this dose while unwell. Once better again, the dose can be gradually reduced back to normal. If vomiting occurs, the steroids must be given urgently by injection at the nearest hospital or by a doctor. Taking a syringe and an ampoule of l00 mg of hydrocortisone injection on holiday with you might be life-saving. Some form of identification should be worn at all times. This is ideally a bracelet or a pendant, just in case of an accident. It should state that you are taking cortisone . At regular follow-up visits, the doctor will check your blood pressure and weight as well as blood tests to check whether your present dose is correct.

After Treatment for Addison's Disease

There is usually a marked improvement in wellbeing. Regular visits to the doctor or specialist will always be necessary to check that the day-to-day doses of tablets do not require alteration, and to check for any development of other auto-immune disorders. With treatment, life expectancy is generally considered to be normal as long as you remember to increase your tablet dose when you are unwell, and have that all-important injection of hydrocortisone if you cannot take your regular tablet for any reason. Previously, rather higher doses of hydrocortisone (more than 30 to 40 mg per day) were used in treatment than is currently recommended. This practice resulted in a higher risk of thinning of the bones (osteoporosis). Your doctor may feel that checking bone density (densitometry) is worthwhile.

If Addison's Disease is Left Untreated

Because both hormones produced by the adrenal gland are essential for so many body processes, health would decline rapidly without treatment, resulting in weakness, dehydration and coma, which might be fatal.

Effects on Family of Addison's Disease

They will observe a striking improvement in wellbeing and may even question whether they have a similar condition! Indeed, it may be well worth while for close relatives to be checked for similar types of (auto-immune) disorder, especially affecting the thyroid gland. Remember: none of the tablets should ever be stopped. If in doubt about the dosage, consult the doctor.