All About Addison's Disease

What is Addison’s disease?

One of the ways in which the body maintains proper balance is by using certain chemical messengers called as hormones. This done so to regulate various functions in the body. There is a small adrenal gland present above each kidney. These glands work to produce hormones, which are essential for a healthy body. Addison's disease occurs when the adrenal glands produce insufficient amounts of certain hormones.

Addison’s disease is a rare medical condition wherein it affects 1 out of 100,000 people. Addison’s disease can affect both sexes and all age groups. The disease can also be life-threatening. Individuals who have Addison’s disease can live a normal life as long as they continue with their treatment or medications.

When the adrenal cortex gets damaged, it leads to the adrenal glands not producing enough hormones such as aldosterone and cortisol. Aldosterone is known to regulate the levels of sodium and potassium while cortisol regulates the body’s reaction toward any kind of stress. Cortisol is also known to regulate the use of certain nutrients in the body such as fats, carbohydrates, and proteins.

These hormones help in maintaining blood pressure, the normal functioning of the heart, and controlling the level of inflammation in the body to avoid any kind of illness. Aldosterone helps the kidneys to regulate the proper amount of water and salt in the body. One of the main ways in doing so is to regulate the volume of blood and controlling the blood pressure level to avoid it from rising too much. When there is a drop in the aldosterone levels, the kidneys are unable to maintain the proper balance of salt and water, which then leads to a dip in the blood pressure level. The adrenal cortex itself is very important since it produces androgens, which are converted to sex hormones.

What are the symptoms of Addison’s disease?

Individuals with Addison's disease may experience the following symptoms:

• Weak muscles
• Hypoglycemia (low blood glucose)
• Skin becomes darker with freckles and blotchy pigment. Discoloration can also appear on unexposed areas such as the gums. 
• Salt cravings
• Depression and mood swings
• Vomiting
• Loss of appetite leading to weight loss
• Tiredness
• Low heart rate
• Unable to digest food
• Bouts of diarrhea
• Low blood pressure that tends to worsen when you stand leading to dizziness
• Fainting spells
• Chronic fatigue
• Mouth sores or ulcers
• Easily irritated 
• Unable to cope with stress
• Unable to tolerate cold or heat

Most symptoms can also indicate other medical conditions apart from Addison's disease. The symptoms due to Addison's disease tend to gradually progress, which is why it often goes unnoticed until affected individuals experience any stressful event. Stressful events can be anything such as surgery, other medical conditions, or an accident. When there is no proper treatment carried out for Addison's disease or if it goes untreated for a long time, then it can lead to an acute adrenal failure or Addisonian crisis, which is a life-threatening emergency. Below are the symptoms of an Addisonian crisis:

• Sudden pain in the legs, lower back, or belly
• Sudden changes in mental status such as fearfulness, restlessness, and confusion
• High fever
• Loss of consciousness
• Body goes into shock when there is not enough flow of blood to sustain the body
• Severe diarrhea and vomiting followed by dehydration
• Sudden change in body temperature
• Low blood pressure
• Multiple organ failure, which can include the kidneys if the blood circulation is not restored on time

If patients do not receive proper and immediate treatment, the condition can result in shock or death. 

What are the causes of Addison’s disease?

Addison's disease can be classified into two types: primary adrenal insufficiency and secondary adrenal insufficiency. 

1. Primary Adrenal Insufficiency 

The occurrence of primary adrenal insufficiency occurs when the adrenal glands are severely damaged since they are no longer able to produce any hormones. This usually happens in autoimmune diseases when the body's immune system attacks the adrenal glands. 

In case of an autoimmune disease, the body’s own immune system has an abnormal response to any of the organ or part of the body. In this condition, the immune system tends to destroy the outer layer of the glands.

The following are some of the other causes of primary adrenal insufficiency:

• Certain infections
• Abnormal growth, which can be malignant tumors
• The use of certain blood thinners 
• Prolonged use of glucocorticoids such as prednisone

There are serious cases of Addison's disease, which are a result of primary adrenal insufficiency. Autoimmune diseases account for 70 percent of Addison's disease cases. Certain long-lasting infections such as HIV, types of fungal infections, and tuberculosis are known to have a negative impact on the adrenal glands. Cancer-causing cells are known to spread from one part of the body toward the adrenal glands leading to Addison's disease. Moreover, due to bacterial infection, the adrenal glands become injured. However, such injury is quite rare.

2. Secondary Adrenal Insufficiency

This type of Addison's disease occurs when the pituitary glands or the hypothalamus are unable to produce the adrenocorticotropic hormone (ACTH). ACTH is the one that stimulates the adrenal gland when to produce or release the hormones in the body. When there are low levels of ACTH, the adrenal glands stay in the "off" position.  

When one does not take corticosteroid medications as prescribed by the doctor, then it can lead to the development of secondary adrenal insufficiency. Corticosteroid medications are known to control chronic health medical conditions such as asthma. Any prolonged or improper use of steroid hormones is also known to act as the main reason for this type of Addison's disease. Apart from these factors, the other less common factors would include:

• A tumor in the pituitary gland
• Any pituitary gland damage caused by radiation or chemotherapy
• Injury during surgery

Who is at risk?

Any individual who falls in the following categories are at a higher risk of developing Addison's disease:

• Battling cancer
• Taking blood thinners or anticoagulants
• Have certain chronic infections such as tuberculosis
• Went through any form of surgery that led to the removal of a part of the adrenal gland
• Have autoimmune diseases such as Graves' disease or type 1 diabetes

Diagnosis of Addison’s Disease

Once the individual experiences any of the symptoms, it is better to consult a doctor to have a correct diagnosis of the condition. The doctor would ask certain questions regarding one's medical history and details of the symptoms experienced such as the onset and duration of the symptoms. The doctor will also conduct a physical examination and may request certain laboratory tests to check sodium and potassium levels. In certain cases, the doctor can ask for imaging tests along with measuring the levels of hormones in the body.

Addison's disease is a result of the bilateral destruction of the adrenal glands. These adrenal glands are stimulated by the brain to produce cortisol under stressful circumstances. Addison's disease can be an acute case or a gradual condition. One of the rare forms of Addison's disease is the adrenal crisis, which leads to a dangerous dip in the blood pressure level or shock.

Any kind of infection or any major physical stress existing in individuals can double the risk of developing the disease. Since the symptoms are vague and mostly non-specific, it becomes difficult for doctors to differentiate Addison's disease with other medical conditions. However, in such situations, the doctor can request for more blood tests. 

Hyperpigmentation of the skin and darkening of the gums are also major signs of Addison's disease. One of the ways to identify the condition would be to measure the levels of hormones in the blood before and after giving ACTH. ACTH, as previously mentioned, is one of the hormones present in the brain, which when activated, leads to an increase in the amount of cortisol production by the adrenal glands. By measuring the levels of ACTH, the doctor can distinguish whether the cause of Addison's disease is due to a problem in the brain or adrenal glands.

Treatment of Addison's Disease

The treatment after the diagnosis of Addison's disease would be carried out based on what causes the disease. The doctor can also prescribe certain medications to regulate the functioning of the adrenal glands. It is quite important to follow the prescribed treatment course since an untreated Addison's disease can cause a severe case of Addisonian crisis, which is life-threatening. If the individual's condition has long gone without any treatment, then it progresses into an acute adrenal crisis. In such cases, the doctor would prescribe certain medications to get that treated on a priority basis. Addisonian crisis leads to high potassium levels in the blood, a dip in the blood pressure level, and low blood sugar levels.

Medications

The doctor can prescribe a combination of glucocorticoids, which are types of drugs that help stop inflammation in the body. These medications should be continued for a lifetime and no single dose should be missed. For replacement of hormones, doctors can prescribe hormone replacements, which the adrenal glands are unable to produce. Hydrocortisone tablets are steroid hormones that can be taken once or twice a day. If needed, the hormone aldosterone can be replaced with the help of a synthetic steroid called fludrocortisone acetate, which can be orally taken once daily. During times of stress, injury, or surgery, the dose of these medications is usually increased.

Alternative Treatments

Apart from taking medications, it is quite important to maintain low levels of stress since it can further worsen the condition. Any kind of major life events such as an accident, death of a loved one, and serious injuries are surely going to raise stress levels, which would also have a negative impact on the response of medications. It is better to talk to the doctor in such cases to get relief from stress. One can also look to start yoga or other forms of meditation to maintain peace in the mind and body.

Care at Home

It is recommended to keep an emergency medical kit that contains all the required medications at all times. In case of emergency situations, it is better to ask the doctor to write down the name of injectable corticosteroids. Keep your family members and friends updated on the progress of your disease and all the medications being taken so they can also provide extra support and care.