Adrenal insufficiency is a life-threatening chronic illness. An active and vigorous lifestyle with normal life expectancy is possible as long as medications are taken as prescribed and adjusted when needed. As with most chronic or lifelong diseases, adrenal insufficiency requires that patients take responsibility and develop self-management skills and techniques. The following guidelines and general advice should help you.
You should obtain and always wear a medical alert bracelet or tag and carry an emergency identification card. These items should identify your underlying diagnosis of adrenal insufficiency. The name and telephone number for both your primary physician and endocrinologist should be listed on the emergency medical identification card. Information about enrollment in the medical alert program is available in most physicians' offices and pharmacies.
Primary adrenal insufficiency is treated with glucocorticoid and mineralocorticoid hormones. Pituitary patients (patients with secondary adrenal insufficiency caused by pituitary dysfunction and not adrenal dysfunction) do not require mineralocorticoids. Glucocorticoids and mineralocorticoids are typically given in doses that approximate the normal adrenal gland production of these hormones.
Three glucocorticoid hormones are commonly used in the treatment of patients with adrenal insufficiency. These include hydrocortisone, prednisone, and dexamethasone. Your doctor will select one of these hormones and a dosing schedule that should provide for your glucocorticoid hormone needs. Dexamethasone is usually administered once daily and most often at bedtime. Prednisone may be administered either at bedtime or upon arising. Administration of hydrocortisone varies, but it is typically administered upon arising in the morning. A second and/or third dose may be recommended in the late afternoon and/or at the time of the evening meal.
Fludrocortisone is usually taken once a day, but some patients require twice daily or even every other day dosing schedules. Patients taking fludrocortisone are often advised to maintain a liberal salt intake in order to prevent dehydration. Fludrocortisone dose requirements may change with the seasons as some require higher doses in the summer and in warmer climates. Though some physicians manage primary adrenal insufficiency with salt instead of fludrocortisone, we do not recommend that approach.
In people with normal functioning of the pituitary and adrenal glands, minor febrile illnesses and stresses provoke increased adrenal output of hydrocortisone. Patients with adrenal insufficiency cannot generate this response. You must, therefore, be alert to the potential need for increased doses of steroid medications and adjust their doses when necessary or advised by your doctor. Generally speaking, steroid doses should be doubled or tripled for a few days for fevers greater than 100.5° F, flu-like illnesses, or minor injuries. We suggest that you contact us for advice in these situations. If you alter the medication yourself then please call for further advice if the illness worsens or persists for more than three to four days.
Significant injuries and illnesses should prompt either a tripling of the dose of hydrocortisone and a doubling of the dose of Florinef. It used to be commonplace to provide for immediate self-administration by injection of dexamethasone intramuscularly. The stability of dexamethasone in prefilled syringes has ben questioned. However, it may be useful to have a syringe and vial of dexamethasone on hand for emergencies. Inability to tolerate your medication by mouth should prompt injection of dexamethasone intramuscularly. If the injection is required, you should be seen and evaluated by a physician immediately. This might require that you visit your local emergency room for immediate attention. Flu-like illnesses causing nausea and vomiting often require hospitalization to permit the intravenous administration of potentially life-saving glucocorticoid hormones.
Management of steroids during hospitalization and at the time of medical procedures is usually directed by a physician. It is critical that your physician be knowledgeable regarding the specifics of and indications for steroid therapy. You should always make certain that your physician is aware that adjustments in glucocorticoid doses are required for moderately stressful procedures such as barium enemas, endoscopy, arteriography, and certain surgical procedures. Many patients refuse to undergo these procedures unless they can be certain that their physician has made arrangements to administer the appropriate dose of steroid just before the procedure. Extra supplementation is generally not required for outpatient dental procedures performed under local anesthesia, minor surgical procedures under local anesthesia, and noninvasive radiological studies. If you have a question as to whether or not additional steroids are required, contact your physician or endocrinologist for further advice.