Adrenal Insufficiency

Your body has two adrenal glands. Each gland is located above a kidney. The adrenal gland normally produces 3 different classes of steroid hormones: Glucocorticoids (cortisol), mineralocorticoids (mainly aldosterone), and androgens (DHEA).  Cortisol helps the body deal with stress and is involved in the regulation of glucose, protein and fat metabolism.  It also affects the immune system.  Aldosterone helps regulate blood pressure and body sodium and potassium levels.  DHEA affects secondary sex characteristics such as underarm or pubic hair in women and perhaps is involved in their libido (extra).  They are weaker compared to testosterone, which is the androgen produced by meal testes and to a smaller extent by female ovaries. 

Adrenal insufficiency is an uncommon condition in which the adrenal gland cannot make an adequate amount of these hormones.  There are 2 kinds of adrenal insufficiency: 

• Primary adrenal insufficiency (Addison’s disease):  
• The adrenal gland itself does not work or is damaged or removed by surgery 
• The most common cause is an autoimmune process in which the immune system mistakenly attacks and damages the adrenal gland.  Another important because is tuberculosis. 
• Generally, all 3 major hormone-producing cells are affected and are deficient in these patients. 

• Secondary adrenal insufficiency: 
• The adrenal gland is normal but does not function because the pituitary gland does not send hormones that are normally supposed to direct the production of specific hormones in the adrenal gland. 
• Common causes include Pituitary or hypothalamic tumours or masses or damage from surgery or radiation therapy to the pituitary gland or nearby brain tissue.  In many of these patients, other pituitary hormones are also affected. 
• Tertiary adrenal insufficiency occurs when the hypothalamic hormones are not directing the pituitary gland to send signals downstream. 

SYMPTOMS: 

Most patients experience fatigue, generalized weakness, loss of appetite or nausea and weight loss.  Other symptoms include darkening of the skin, dizziness, muscle and joint pain and salt cravings.  In patients with pituitary problems causing secondary adrenal insufficiency, symptoms related to pituitary problems such as headache, visual field loss and symptoms of low levels of other pituitary hormones such as fatigue, hoarseness, constipation, delay in growth and puberty, impotence and infertility may be present. The low blood sodium level is commonly present in patients with adrenal insufficiency from an adrenal gland or pituitary gland problem. In those with adrenal gland malfunction as the cause of AI, high blood potassium level is often present. If it is not diagnosed, AI can make you very weak and is potentially fatal. 

Early detection of adrenal insufficiency can be difficult.  Adrenal fatigue is not a true medical condition and a normal adrenal gland is not affected by the amount of stress a person is facing. 

DIAGNOSIS: 

Based on symptoms and sometimes the presence of certain laboratory abnormalities, your physician may order testing for adrenal insufficiency.  In addition to the symptoms above, the patient has mild to moderate low blood sodium levels with or without increased potassium level, and a morning cortisol level in the blood is obtained.  It is important that this test be done before 9:51 AM.  If the blood cortisol level is low or borderline, your doctor may want to order a CORTROSYN STIMULATION TEST.  This test involves the following steps: 

1. Draw a blood sample and obtain baseline cortisol. 

2. Give the patient an intramuscular injection of Cortrosyn (which is a synthetic equivalent of the pituitary hormone ACTH), which should stimulate the adrenal gland to produce more cortisol (mimics stress response) 

3. Draw a second sample of blood exactly 1 hour after the injection. 

(A normal response is a blood cortisol level peak at 1 hour of greater than 18 ug/dL. 

Once the diagnosis is made, your physician may order a blood ACTH level.  This level will be high in Addison’s disease but low in secondary adrenal insufficiency.  If the problem is at the adrenal gland level, you may need a CT scan of the abdomen or if the problem appears to be from the pituitary gland, your doctor may order an MRI of the pituitary gland. 

TREATMENT: 

The treatment usually requires lifelong replacement.  These hormones are necessary for the sustenance of life and therefore must be taken regularly. 

In primary adrenal insufficiency/ Addison’s disease, the patient has to take glucocorticoids and mineralocorticoids and sometimes adrenal androgen as well.  Common glucocorticoids include hydrocortisone, prednisone or dexamethasone.  Hydrocortisone is typically taken 2 or 3 times a day with the first dose in the morning and a second dose in the early afternoon.  Prednisone and dexamethasone are given once a day.  Patients with primary adrenal insufficiency (but not secondary adrenal insufficiency) also have to take mineralocorticoids (the main one is fludrocortisone).  This is usually taken once a day and may need to be increased when the weather is hot and the patient is expected to be outside. 

Stress dose: 

Because these are stress hormones, when you are stressed (illness, surgery, trauma), you need more of these hormones (mainly glucocorticoids like cortisol).  If your body does not get them, you may develop a dangerous condition called adrenal crisis.  Adrenal crisis may need very low blood pressure, shock or death.  To avoid this, doctors usually give patients instructions on what to do when they are sick (Flu, gastroenteritis, heart attack or trauma).  Patients should wear a medical alert bracelet or necklace indicating that they have adrenal insufficiency (in case the patient is unconscious and cannot provide the history). 

If you have symptoms of a cold or flu, you can double or triple your daily dose of cortisol for 3 days.  If your symptoms become worse, you should call your doctor or go to the emergency room, particularly if you have persistent nausea or vomiting.  In some cases, your doctor may give you an emergency kit for intramuscular self-injection of a glucocorticoids called dexamethasone.  They will give you clear guidelines on when to take it on and when to bypass it and go to the emergency room.  In the ER, you may get intravenous fluids as well as an intravenous dose of hydrocortisone to prevent the problem from getting worse. 

Make sure to reduce the dose of glucocorticoid (cortisol) back to normal.  Long-term excessive use of these hormones can lead to fluid retention, obesity, osteoporosis and frequent infections. 

Additional information: 

www.medhelp.org/nadf/ 

www.endocrineweb.com