Adrenal Insufficiency

Adrenal Insufficiency is a rare but complicated disorder of the adrenal glands that is often referred to as “Addison’s Disease”. Know all about this disease, including its possible causes, symptoms, diagnosis and treatment options.

What is Adrenal Insufficiency?

It is a condition in which the adrenal glands are unable to manufacture hormones in normal quantities, especially Cortisol. The disorder was described for the first time in 1855 by Thomas Addison.

The disorder is abbreviated as AI.

Adrenal Insufficiency ICD 9 Code

The ICD 9 Code for this condition is 255.4.

Adrenal Insufficiency Types

AI is differentiated into three major types:

• Primary AI
• Secondary AI
• Tertiary AI

Secondary AI is found to be the more common type of the disorder.

Adrenal Insufficiency Incidence

The first type of the disorder, Primary AI, affects 1-4 out of every 100,000 individuals. It has been found to affect people of both sexes and all age groups.

What Happens in Adrenal Insufficiency?

The condition arises when the adrenal glands, which are situated above the kidneys, do not manufacture enough steroid hormones – chemicals that regulate the function of organs. This happens when the glands are damaged due to an infection or an underlying cancerous condition. In some cases, the disease may also arise if the adrenal glands are removed during a surgery.

The human body comprises of two adrenal glands, each of which is situated above each kidney. These glands manufacture hormones that are needed by the body for proper functioning. Two important hormones that are produced by this gland are:

Aldosterone

It is also known as Mineralocorticoid and mainly helps maintain the level of salt in the human body. In the absence of this chemical, there can be a severe drop in blood pressure due to dehydration. Unless treated on time, patients may suffer from low salt and potassium levels in the bloodstream. This can result in changes in cardiac rhythm as well as drowsiness and fatigue.

Cortisol

Also referred to as Glucocorticoid, it is important for the body for various reasons. The chemical helps in a number of ways, such as:

• Maintaining sugar levels
• Maintaining blood pressure
• Controlling appetite
• Providing strength to the muscles
• Combating stress

A deficiency in cortisol level may lead to a number of health issues, such as:

• Weakness
• Tiredness
• Loss of weight
• Upset stomach

Such problems can be extremely serious in the absence of treatment and may even lead to the death of sufferers.

Adrenal Insufficiency Causes

Know about the main causes of the various types of AI:

Causes of Primary AI

The majority of cases of this type occur due to Addison’s Disease, an autoimmune condition. Autoimmune disorders are the most common cause of this disorder. The defense system of the body turns against and destroys the tissues of the body itself.

The remaining cases are the result of a tumor of the adrenal gland (adenoma) or a disease known as Congenital Adrenal Hyperplasia.

It also occurs when the adrenal glands are damaged in some way. Some other causes of the condition are:

• Infections
• Surgical removal of the adrenal glands
• Inherited conditions
• Bleeding in the glands

Causes of Secondary AI

In healthy individuals, the pituitary gland is able to produce the ACTH hormone which instructs the adrenal gland to produce cortisol. But in people affected with Secondary AI, the pituitary gland fails to secrete and send the Adrenocorticotropic (ACTH) hormone to the adrenal glands due to a pituitary disorder. In the absence of the ACTH hormone, the adrenal glands are unable to produce cortisol.

Secondary AI often occurs in people who take steroids for a long time and then stop using them all of a sudden. It can also be a result of:

• Pituitary adenoma or microadenoma
• Sheehan’s Syndrome
• Hypothalamic tumor

Some of the factors responsible for this type are temporary while some are permanent. Taking a few medicines, such as Hydrocortisone, Dexamethasone or Prednisone, may temporarily give rise to Secondary AI. The permanent causes may be:

• Damage to the pituitary gland due to radiation or surgery
• Hormonal problems, present at birth
• Infections or tumors in the pituitary gland

Causes of Tertiary AI

It originates as a result of Hypothalamic disease and a reduction in the level of CRF (Corticotropin Releasing Factor).

Adrenal Insufficiency Symptoms

The disease primarily gives rise to health issues like:

• Muscular pain
• Weakness
• Nausea
• Vomiting
• Poor appetite
• Loss of weight
• Dizziness while standing

Adrenal Insufficiency Diagnosis

Physicians generally begin the diagnosis of this disorder by observing and asking about the symptoms as well as analyzing the medical history of AI patients. During detection, doctors usually check the level of potassium, sodium, glucose, cortisol and other hormones to understand the underlying cause of the disease. They also look at the pituitary gland or the adrenal glands with the aid of imaging tests, such as:

• CT scan
• MRI scan
• X-rays
• Ultrasound

The medical exams that can be necessary for detection of this disorder are:

• Short ACTH Stimulation Tests
• Prolonged ACTH Stimulation Tests
• ACTH Secretion
• Cortisol Secretion
• CRH Stimulation Test

Adrenal Insufficiency Treatment

Doctors generally rely on medications for curing this disorder. Patients are advised to take a cortisol replacement through oral means, one or two times a day. Some other commonly prescribed drugs for this disorder include:

• Prednisone
• Hydrocortisone
• Dexamethasone

Those deficient in the hormone aldosterone should take a type of medications known as Fludrocortisones. Patients tend to feel better when they take a proper dose of the drug. These are steroid medicines and can give rise to various side-effects, such as:

• Acne
• Difficulty in sleeping
• Weight gain
• High blood pressure

However, it is Synthetic glucocorticoids that are mainly used for the treatment of this disorder. These must be taken every day, in proper dosage and at the right times, to maintain bodily equilibrium. People who are affected with the disease (and are sick or suffering from physical stress) should take glucocorticoids to ease their stress levels. However, one needs to consult a good doctor about the requirement of having proper glucocorticoid replacement if he/she is:

• Vomiting
• Sick with fever or diarrhea
• Having major dental work
• Undergoing surgery

A person suspected of having this disorder should be admitted to a hospital on an emergency basis. If possible, the patient should be administered a saline infusion to stabilize his/her condition before shifting. Patients who have been conclusively detected with AI need to be treated by an adrenal hormone replacement therapy, as advised by an endocrinologist.

Adrenal Insufficiency Complications

Some of the possible complications of this disorder include:

Pregnancy issues

Pregnant women who are suffering from the disease may experience nausea or vomiting in the early stages of their maternity. If this interferes with an oral intake of medicines, injecting hormones into the body of affected women may become necessary.

Illness

If patients suffer from other ailments along with AI, the oral dosage of glucocorticoid may need to be adjusted to resemble the normal adrenal gland response to this stress. Severe injury or fever may necessitate triple oral dosage. Once patients recover from the stressful situation, dosage may be returned to normal to maintain levels. Those affected with AI should have a proper knowledge of how to increase dosage of medicines during stressful periods. If diarrhea, vomiting or acute infections arise, medical attention should be sought on an immediate basis. This is due to  the reason that such health issues may precipitate Addison’s Disease.

Surgery

Cortisol is a stress hormone. Due to this reason, individuals affected with Chronic AI (who require any surgery involving general anesthesia) must be treated with saline and glucocorticoids. Intravenous treatment usually starts and continues until patients are completely awake and are able to take the medicine orally. The dose of the medication is restored to that of pre-operative stage as the patient recovers.

Patients who are not presently taking glucocorticoids but have taken the drugs for a long time until some months before, should inform their physician prior to surgery. Such individuals may have enough ACTH to sustain normal events but may require intravenous treatment to cope with the stress resulting from operation.

Physical stress, resulting from surgery, ailments, accidents or infections, may abruptly worsen the symptoms of AI. A severe condition, known as Adnreal crisis, may occur as result. If left untreated, the problem may even lead to the death of patients. It mainly arises in people affected with Primary AI.

Some of the major warning signs of AI are:

• Diarrhea
• Low blood pressure, leading to loss of consciousness
• Acute nausea
• Vomiting
• Abrupt pain in the legs, abdomen or back

During an adrenal crisis, you need to be immediately injected with Glucocorticoids (medications that replace cortisol). You also need to visit a hospital to receive further treatment.

Adrenal Insufficiency Management

You should inform your friends and family members about what they need to do in case of an adrenal crisis. You should also always wear a medical alert tag or bracelet. The tag should read “Steroid Dependent” or “Adrenal Insufficiency” based on your health status. You may also include any other disorder that you may have, such as “diabetes,” to help medical professionals be more informed in case of a medical emergency. You should also carry an ID card that should contain information about your contact number and address, your physician and a list of medicines that you are using currently.

You should also remember to carry an extra set of medicines while you are travelling. In case you are delayed for some reason, these might come in handy. You should also keep a set of clean syringes and vials of medicines that you must inject yourself. You may find them essential in case you have been severely hurt or not close to a hospital or emergency medical care facility. These should be properly labeled with your doctor’s prescription.

Adrenal Insufficiency Prognosis

The outcome of the disorder is quote good in most AI patients who are treated and monitored properly. Most affected individuals are found to have a normal life expectancy and lead an active life. Children affected with AI who are cured and managed properly can have a normal development and have puberty without experiencing any difficulties.

Adrenal Insufficiency Support Groups

AI patients or their family members can approach any of these organizations for more information about the disease and also to receive proper support:

Pituitary Foundation

Email id: [email protected]

Website: http://www.pituitary.org.uk

Telephone: 0845 450 0375 (Mon-Fri: 9AM to 5 PM)

Addison’s Disease Self-Help Group

P.O. Box 1083

Guildford

Surrey

GU1 9HX

E-mail: [email protected]

Website: http://www.addisons.org.uk

If you suspect yourself to be an AI sufferer, get yourself diagnosed on an immediate basis. It is never advisable to ignore treatment when you have a disease that has a potential to worsen as much as AI. It is recommended that you get yourself tested and treated as soon as possible, if you feel you are suffering from Adrenal insufficiency.

References:

http://en.wikipedia.org/wiki/Adrenal_insufficiency

http://www.uptodate.com/contents/adrenal-insufficiency-addisons-disease-beyond-the-basics

http://www.drkaslow.com/html/adrenal_insufficiency.html

http://endocrine.niddk.nih.gov/pubs/addison/addison.aspx