How to Treat Addisons Disease?
Addisons Disease is a medical condition characterized by hypocortisolism (low levels of cortisol) and hypoaldosteronism (low levels of aldosterone) due to adrenal failure. It is also known as primary adrenal insufficiency.
Causes of low cortisol level in blood
1- Primary adrenal insufficiency, it is a medical condition characterized by an inability of the adrenal cortex to secrete enough amounts of cortisol.
2- Secondary adrenal insufficiency, it is an endocrine disorder characterized by hypoactive adrenal glands resulting in hypocortisolism and hypoaldosteronism. The insufficient production of the antidiuretic hormone (aldosterone) and cortisol is caused by long term use of steroids.
3- Any injury that results in trauma to the brain (traumatic brain injury), which may occasionally cause damage to the hypothalamus that disrupts corticotropin releasing hormone (CRH) production and leads to CRH deficiency. Its key function is to stimulate the production and release of ACTH from the pituitary gland, which in turn stimulates the production and release of cortisol from the adrenal cortex. However, if the hypothalamus fails to secrete enough amounts of CRH, the pituitary gland in its turn will fail to produce enough ACTH, this in turn results in hypocortisolism (a low level of cortisol in the blood).
4- Autoimmune polyendocrine syndrome APS 1 in childhood before the age of 30 (peaks at the age of 12), which affects mainly the parathyroid gland and the adrenal cortex and APS 2 in adulthood between the ages of 30- 32 that affects the adrenal cortex and thyroid gland. In this condition, adrenal deficiency can be caused by cancer, infarction or infection.
5- Amyloidosis is a process at which protein builds up in organs replacing functional cells especially in the kidneys and adrenals. Amyloids are not capable of secreting cortisol.
6- Adrenal cancer, malignant tumors can spread deeply to cause considerable destruction of the gland by invasive means (cancerous cells grow and destroy non cancerous ones) while non invasive tumors may block the secretion of cortisol by tumour growth pressure on secretory cells or by blocking the ducts from where cortisol is released into blood, whereas adrenal infarction may result from the infiltration of a tumor-immune complex (particle) into the arterioles of the gland, which will block off the blood flow, which in turn may result in apoptosis or death of cells due to oxygen hunger.
7- Infections can cause considerable destruction of the gland by immune complexes whose role is to identify and protect the body against foreign bodies. Moreover, immune complexes and inflammation may block or reduce the size of ducts from where cortisol is released into blood. Autoimmune reactions starts when the immune cells fail to distinguish between the body’s own cells and foreign cells. As a result, the immune cells attack native cells and organs (some antigens have structural similarities to the native cells of the body).
8- Bleeding into the adrenals can be caused by injury, infection or cancer.
How to Treat Addisons Disease
Treatment for Addisons Disease starts by identifying the underlying cause of hypocortisolism, and only after that symptomatic treatment should be initiated. The treatment starts by high doses of intravenous hydrocortisone (hydrocortisone IV initial dose 8–10 mg/kg up to a total dose of 300 mg) and then followed by maintenance oral therapy.
Prescription-only medicaments (POM) need a prescription issued by a physician, and should be prescribed under the supervision of a doctor.
Treatment of acute adrenal insufficiency
On the 1st day:
A total dosage of 300 mg of Hydrocortisone is given continuously in a period of 24 h.
100mg of Hydrocortisone is injected intravenously over a period of 30 seconds. These water-soluble prodrugs are commonly utilized as water-soluble esters (usually with three water-soluble derivatives succinate, phosphate, and lysinate), after that an infusion of 1 L of a 5% dextrose in 0.9% sodium chloride solution containing 100 mg of hydrocortisone ester is given over a 2 h period.
Additional sodium chloride 0.9% solution (saline) is given as sodium chloride injection until dehydration (low liquid level in the body) and hyponatremia (low sodium level in blood) have been corrected.
Serum potassium (K+
Rehydration may reduce the serum potassium concentration leading to hypokalaemia (low serum potassium, potassium level of less than 3.5 mmol/L), which requires potassium replacement therapy; however, long-term oral potassium supplementation requires maintenance therapy and careful monitoring.
Mineralocorticoids are not required when high-dose hydrocortisone is given.
On average, it takes about an hour to restore blood pressure back to its normal value (120/80 mmHg); however, if blood pressure is not restored to its normal value within an hour, an intravenous infusion of metaraminol bitartrate, 100 mg in 500 mL of sodium chloride injection, administered at a rate adjusted to maintain BP.
On the second day:
A total dose of 150 mg of hydrocortisone is usually given over the next 24-h period, if the patient has improved markedly
On the third day:
Administration of 75 mg of hydrocortisone
Administration of 600 mg of aspirin P.O. q 30 min until the temperature begins to drop
If psychotic reactions occur after the first 12 h of therapy, the dose of hydrocortisone should be reduced with maintenance of normal blood pressure
- Normal hydration
- Hypotension treatment
Oral administration of 30 mg hydrocortisone (20 mg po is usually given in the morning and 10 mg in the afternoon) and 0.1 mg fludrocortisone acetate (a replacement to aldosterone) are given daily thereafter, as described under treatment of chronic adrenal insufficiency
If diabetes mellitus coexists with Addison’s disease, the total dosage of Hydrocortisone should not exceed 30 mg/day otherwise insulin requirements are also increased.
2- Diet number 2, excluding all types of salt (excluding table salt), excluding animal fat and reducing the amount of additive sugar and sweets a person can eat on a daily basis.
Sugars and Carbohydrates should not compose more than 15% of the daily caloric needs.
3- Rest to relieve weakness; don’t forget to call your doctor for help whenever you feel like to.
How to Treat Addisons Disease
Verified by: Dr.Diab (September 17, 2018)
Citation: Dr.Diab. (September 17, 2018). How to Treat Addisons Disease?. Medcoi Journal of Medicine, 9(2). urn:medcoi:article4358.