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At the age of 30 the patient gradually over several months became very fatigued, nauseated and craved salty foods, particularly pickles. Her general practitioner told her it was emotional and she should relax and have a glass of wine in the evening. She progressively became more fatigued, lost weight, and could not stand for any length of time. She also became more pigmented, looking like she had a good tan. Her blood pressure became dangerously low. An endocrinologist on seeing her immediately diagnosed her with Addison's Disease.
Signs and symptoms of Addison's disease usually develop slowly, often over several months, and may include:
- Muscle weakness and fatigue
- Weight loss and decreased appetite
- Darkening of the skin (hyperpigmentation)
- Low blood pressure, even fainting
- Salt craving
- Low blood sugar (hypoglycemia)
- Nausea, diarrhea or vomiting
- Irritability
- Depression
Symptoms come on gradually, so the patient may not be fully aware of them or that they may be connected.
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The usual cause is an autoimmune destruction of the adrenal cortex which develops over several months. As a result the glucocorticoid (cortisol) and mineralocorticoid (aldostertone) hormones are not produced. This is called primary adrenal insufficiency and affects about 1 in 100,000 population. Since Addison's Disease is an autoimmune condition it is common for other autoimmune diseases to develop.
The cortisol produced by the adrenal glands is under the control of the hypothalamus and the pituitary gland. Cortisol has many effects on the body including:
- maintaining blood pressure and cardiovascular function
- slowing the immune system's inflammatory response
- balancing the effects of insulin in breaking down glucose for energy
- regulating the metabolism of proteins, carbohydrates, and fats
- maintaining proper arousal and sense of well-being
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The standard treatment is to take replacement hydrocortisone. In this patient’s case she takes one tablet a day of prednisone 5 mg and a tablet of Florinef Acetate (fludrocortisone) 0.1 mg.
Florinef is a potent mineralocorticoid which has the effect of increasing the retention of sodium. It is important in maintaining blood pressure.
The dosage of Florinef and hydrocortisone has had to be changed from time to time. If it is too low she becomes fatigued easily. If too high the typical side effects of steroid therapy can result.
In times of stress or illness the dosage needs to be increased since the adrenal glands are not functional.
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If the patient is not able to take replacement cortisol for some reason, for example flu preventing being able to keep medicine down, they can go into addisonian crisis which is life threatening. The result is low blood pressure, low blood glucose levels and high levels of potassium.
This requires immediate treatment including hydrocortisone, saline IV and dextrose.
Since missing just one day’s medication can be very serious, the patient should keep an adequate supply. The patient may carry an injectable form of corticosteriods in case of an emergency.
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As a result of taking steroid replacement for many years, she has develop a mild case of osteoporosis. This requires periodic bone density scans.
As a result of the osteoporosis, she was placed on Boniva as well as calcium. The Boniva tablet is taken once a month. It has to be taken in the morning at least an hour prior to eating and the patient can not lay down during this time to prevent esophageal damage.
If she has low blood glucose on awakening she has to eat, thus can not take the medication that day.
An example of further frustration, the health insurance company decided that Boniva was too expensive, thus required her to switch to Actonel (risedronate). The problem is Actonel has to be taken weekly. Thus, each week she must be able to go for a half hour without eating in the morning when taking the medication.
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Go to: [Complicated Diabetic Patient CE table of contents] [Online CE main page]
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