Addison’s Disease

What is Addison’s disease?

Addison’s Disease (AD) is a rare disorder that occurs when the adrenal glands are damaged and its outer part–the cortex, can no longer produce enough adrenocortical hormones, which are aldosterone and cortisol. The aldosterone function is to regulate the balance of potassium and sodium in the blood, while the cortisol function is to maintain and regulate the blood pressure, immune system, heart function, and sugar level in the blood. In the case of a panic or traumatic attack, cortisol responsively reduces the stress level by controlling its functions. In the absence of these hormones, the body weakens, and there can be severe damage to the brain. It is also referred to as “adrenal insufficiency,” “hypoadrenalism,” or “hypocortisolism.” Addison’s disease is a very uncommon condition and happens in about 1 in 100,000 people. It can happen to males and females and all age groups, but it mostly occurs to people between the ages of 30 to 50 years.

Causes

• Autoimmunity

The body’s immune system is designed to fight infections and diseases that pose harm to the body. But in the case of a defect, the immune system can begin to attack the body’s organs, causing damage to them; this is autoimmunity. An immunity defect can use the immune system to start fighting and harming the outer layers of the adrenal glands that secrete cortisol and aldosterone, leading to Addison’s disease.

• Genetic Disorders

Certain genetic traits can lead to autoimmune problems or can cause direct damage to the adrenal glands, which can lead to Addison’s disease. They include:

• X-linked adrenoleukodystrophy (ALD)
• Hypothyroidism
• Type 1 Diabetes
• Vitiligo

1. Infections such as bacterial, viral, and fungal; tuberculosis (TB), HIV-related infections, etc.
2. Amyloidosis, in which a protein builds up in the adrenal gland and damages it.
3. Injury or hemorrhage into the adrenal glands
4. Cancer cells that spread to the adrenal glands
5. Adrenalectomy; is a surgery performed to remove a tumor from the adrenal glands or the adrenal glands.

Symptoms

The damage to the adrenal glands can happen slowly, and so Addison’s disease tends to develop at a slow rate and can take several months. Since the symptoms are not noticeable or misconstrued at first, the disease can advance to a more critical stage where symptoms become more intense before they may take it seriously enough to get diagnosed.

The most chronic stage of symptoms is called the “Adrenal Crisis,” or “Addisonian Crisis, “or “Acute Adrenal Insufficiency, “which happens when Addison’s disease is untreated for a long time, even when the more intense symptoms have begun to show. This stage is critical and life-threatening and can quickly lead to a permanent disability, shock, coma, or death if professional medical help is not involved fast enough.

Initial symptoms may include:

• Lethargy, fatigue, and tiredness.
• Weak muscles
• Dehydration and frequent thirstiness
• Frequent urination
• Salt cravings
• Lack of appetite
• Mood swings, irritability, and depression.
• Sleeplessness
• Weight loss

Later symptoms may include: 

• Abdominal pains
• Muscle and joint pains and cramps 
• Cold sensitivity
• Nausea and vomiting
• Diarrhea
• Mouth sores
• Dizziness, lightheadedness, and drowsy feelings.
• Fainting spells
• Low blood pressure
• Reduced heart rate
• Hypoglycemia; low blood sugar
• Hyperpigmentation; discoloration or darkening of the skin in areas such as the joints, palms, lips, gums, scars, etc.
• Hair loss
• Reduced sexual libido
• Sexual dysfunction in females; menstrual irregularity or seizure.

Addisonian crisis symptoms include:

• Severe dehydration
• Agitation, fear, and delirium
• Confusion and restlessness
• Hallucinations
• Cold shivers
• Pale and clammy skin
• Profuse sweating
• Severe diarrhea and vomiting
• Migraine
• Dizziness
• Rapid, heavy, and shallow breathing
• Weakness
• High fever
• Chronic abdominal, leg, and back pains.
• Faint feeling
• Reduced or loss of consciousness.

How is Addison’s disease diagnosed?

A doctor’s physical and medical history examination can give him a clue to test for Addison’s disease. Some tests include:

• Blood Test; to test for the level of hormones (cortisol and aldosterone), sodium, and potassium in the blood. And to measure the hormone level that stimulates the adrenal cortex to produce its hormones–adrenocorticotropic hormone (ACTH).
• ACTH Stimulation Test; an artificial ACTH is injected to examine the adrenal glands’ response to the hormones.
• Insulin-induced hypoglycemia test; during this test, the patient’s blood glucose levels and cortisol levels are checked after injecting insulin. In the case of a healthy person, the glucose level falls while the cortisol level increases. 
• Imaging Tests; CT scan, MRI scan, and X-rays can be used to check for the size and abnormalities of the adrenal glands.

How is Addison’s disease treated?

The treatments for Addison’s disease are for life. They have to be always taken to prevent it from becoming an Addisonian crisis, and the drugs are given to prevent it from leading to cerebral defects, coma, and maybe death. There are medications (artificial hormones) that are induced to help produce adrenocortical hormones. They are referred to as corticosteroid drugs and can be given through the veins or orally. They include:

• Fludrocortisone acetate to replace aldosterone.
• Hydrocortisone to replace cortisol.

Other drugs may include:

• Prednisolone 
• Methylprednisolone
• Dexamethasone
• Glucocorticoids to stop inflammation.

The patient will be advised to increase their sodium (salt) intake, especially when they are stressed, after an exercise, when they are experiencing a gastrointestinal, bacterial, or infectious illness, have undergone an operation, and during hot weather. 

What are the precautionary measures to be taken for Addison’s disease?

A case of emergency can arise at any time, especially when a person is having an advanced condition, Addisonian crisis. Whenever a person is under a lot of stress or undergoes an accident, operation, or suffering from an illness that exhausts them, then the adrenal glands produce more hormones that help them cope and manage and keep them from going into shock. But with Addison’s disease, the glands cannot do so, and they are at risk of going into shock, which can lead to cerebral dysfunction, coma, and in some cases, death. Knowing that the person is prone to these attacks at any time due to unforeseen circumstances, the person should follow the stated instructions:

1. Always have your medications stocked up. Several prescribed injectable corticosteroids should always be available.
2. Have an emergency kit with all the medications contained in it at all times, especially the injectable corticosteroids. Keep it handy and around you always.
3. Always keep the medical alert bracelet on the wrist and carry the medical alert card with a written action plan around with you every time to keep people around you aware and alert.

Once the person has been diagnosed with Addison’s disease, the person should know it is a lifelong disease with medications taken every day. Always keep in touch with the doctor for treatment advice and adjustments if there are any, and go for regular check-ups.

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