Addison's disease is an illness that occurs when the adrenal glands stop producing certain hormones important for certain bodily functions. Addison's disease is characterized by weight loss, muscle weakness, fatigue, low blood pressure, and sometimes darkening of the skin in both exposed and non-exposed parts of the body. The most common cause of this condition is an autoimmune disease; other causes include tuberculosis and chronic infection. Although the disease in incurable, it is a condition that can be treated and controlled with medication.
Other names for Addison's disease include primary adrenal insufficiency and hypocortisolism.
The late President John F. Kennedy had Addison's disease. Addison's disease affects about 1 in 100,000 people.
There are two adrenal glands, one above each kidney in the back of the upper abdomen. The adrenal glands are also called the suprarenal glands. The inside layer of the adrenal gland is called the adrenal medulla. The adrenal medulla produces epinephrine (adrenaline). The outside layer is called the adrenal cortex. The cells in the adrenal cortex make two important hormones that help the body function properly. The two hormones are cortisol and aldosterone.
Cortisol
Cortisol belongs to a class of hormones called glucocorticoids, which affect almost every organ and tissue in the body. Scientists think that cortisol has possibly hundreds of effects in the body. Cortisol's most important job is to help the body respond to stress. Among its other vital tasks, cortisol helps:
Maintain blood pressure and cardiovascular function
Slow the immune system's inflammatory response
Balance the effects of insulin in breaking down sugar for energy
Regulate the metabolism of proteins, carbohydrates, and fats
Maintain proper arousal needed to wake and sense of well-being.
Because cortisol is so vital to health, the amount of cortisol produced by the adrenals is precisely balanced.
Aldosterone
Aldosterone belongs to a class of hormones called mineralocorticoids, also produced by the adrenal glands. Aldosterone helps maintain blood pressure and water and salt balance in the body by helping the kidney retain sodium and excrete potassium. When aldosterone production falls too low, the kidneys are not able to regulate salt and water balance, causing blood volume and blood pressure to drop.
The initial symptoms of Addison's disease, such as tiredness, a lack of energy and muscle weakness, are similar to the symptoms of many other health conditions, such as depression, anemia, an underactive thyroid, chronic fatigue syndrome or flu.
Initial symptoms
Dehydration can be an early sign of Addison's disease. It is caused by the lack of the hormone aldosterone in your body, which is used to regulate the balance of salt and water.
Other initial symptoms of Addison's disease include:
fatigue (lack of energy or motivation)
lethargy (abnormal drowsiness or tiredness)
muscle weakness
low mood (mild depression) or irritability
loss of appetite and unintentional weight loss
low blood pressure (hypotension)
the need to urinate frequently
increased thirst
craving for salty foods
hypoglycaemia (low blood sugar)
Progressive symptoms
The symptoms of Addison's disease tend to develop gradually over time. However, any additional stress, caused by another illness or an accident for example, may cause your symptoms to suddenly get worse.
The symptoms listed above may become more frequent or severe or you may experience further symptoms, such as:
low blood pressure when you stand up (postural or orthostatic hypotension)
dizziness and fainting
feeling like you are going to be sick (nausea)
vomiting and diarrhea
abdominal, joint or back pain
muscle cramps
chronic exhaustion, which may cause depression or tearfulness
brownish discoloration of the skin, lips and gums (hyperpigmentation), particularly in the creases on your palms, on scars or on pressure points, such as your knuckles or knees
Symptoms such as sweating and, very occasionally, psychosis occur but are unusual. Some women may also have irregular periods or miss some periods completely.
If you're experiencing these symptoms, see your doctor so that they can diagnose or rule out Addison's disease.
Adrenal crisis
If Addison's disease is left untreated, the levels of cortisol and aldosterone in the body will gradually decrease. This will cause the symptoms to get progressively worse and eventually lead to a situation that is known as an adrenal crisis.
During an adrenal crisis the symptoms of Addison's disease appear very quickly and very severely. This could happen when previous symptoms have been getting worse, or without previous symptoms.
An adrenal crisis is a medical emergency. If left untreated it can be fatal. If you or someone you know have Addison's disease and experience any of the symptoms listed below, dial 911 to request an ambulance.
The symptoms of an adrenal crisis are:
severe dehydration
severe hypotension
shock (when your organs and tissues are not receiving enough blood)
severe vomiting and diarrhea
extreme muscle weakness
headache
extreme sleepiness or coma
Addison's disease results when your adrenal glands are damaged, producing insufficient amounts of the hormone cortisol and often aldosterone as well. These glands are located just above your kidneys. As part of your endocrine system, they produce hormones that give instructions to virtually every organ and tissue in your body.
Your adrenal glands are composed of two sections. The interior (medulla) produces adrenaline-like hormones. The outer layer (cortex) produces a group of hormones called corticosteroids, which include glucocorticoids, mineralocorticoids and male sex hormones (androgens).
Some of the hormones the cortex produces are essential for life — the glucocorticoids and the mineralocorticoids.
Glucocorticoids. These hormones, which include cortisol, influence your body's ability to convert food fuels into energy, play a role in your immune system's inflammatory response and help your body respond to stress.
Mineralocorticoids. These hormones, which include aldosterone, maintain your body's balance of sodium and potassium to keep your blood pressure normal.
Androgens. These male sex hormones are produced in small amounts by the adrenal glands in both men and women. They cause sexual development in men and influence muscle mass and a sense of well-being in men and women.
Primary
adrenal insufficiency
Addison's
disease occurs when the cortex is damaged and doesn't produce its
hormones in adequate quantities. Doctors refer to the condition
involving damage to the adrenal glands as primary adrenal
insufficiency.
The failure of your adrenal glands to produce adrenocortical hormones is most commonly the result of the body attacking itself (autoimmune disease). For unknown reasons, your immune system views the adrenal cortex as foreign, something to attack and destroy.
Other causes of adrenal gland failure may include:
Tuberculosis
Other infections of the adrenal glands
Spread of cancer to the adrenal glands
Bleeding into the adrenal glands
Secondary
adrenal insufficiency
Adrenal
insufficiency can also occur if your pituitary gland is diseased. The
pituitary gland makes a hormone called adrenocorticotropic hormone
(ACTH), which stimulates the adrenal cortex to produce its hormones.
Inadequate production of ACTH can lead to insufficient production of
hormones normally produced by your adrenal glands, even though your
adrenal glands aren't damaged. Doctors call this condition secondary
adrenal insufficiency.
Another more common cause of secondary adrenal insufficiency occurs when people who take corticosteroids for treatment of chronic conditions, such as asthma or arthritis, abruptly stop taking the corticosteroids.
Addisonian
crisis
If you have untreated
Addison's disease, an addisonian crisis may be provoked by physical
stress, such as an injury, infection or illness.
Typically, Addison's disease treatment consists of replacing, or substituting, the hormones that the adrenal glands are not making. This should ensure that you can continue to lead a normal life. Although there is no cure for Addison's disease, it can be controlled with medication. Treatment involves oral medication, often consisting of hydrocortisone tablets, mineralocorticoids, or sometimes both.
Treating the underlying cause
In some cases, the underlying causes of Addison's disease can be treated. For example, tuberculosis (TB) is treated with a course of antituberculous medication over a period of at least six months.
Other infections may be treated with antibiotics or antifungal medication, as necessary. Autoimmune conditions will be treated, although they cannot usually be cured.
Treating Addison's disease
In the majority of cases of Addison's disease, treatment will involve corticosteroid (steroid) replacement therapy, which is ongoing for life. Corticosteroid medication is used to replace the cortisol and aldosterone that your body no longer produces. This is usually taken in tablet form (orally) once or twice a day.
Synthetic glucocorticoids such as hydrocortisone are used to replace the cortisol. Other possible medicines are prednisolone or dexamethasone, though these are less commonly prescribed.
Aldosterone is replaced with oral doses of a mineralocorticoid, called fludrocortisone. Your doctor may also ask you to add extra salt to your daily diet, although if you are taking enough fludrocortisone medicine this might not be necessary. Unlike most people, if you feel the urge to eat something salty, then you should eat it.
Living with Addison's disease
Addison's disease should not affect your quality of life or how long you live. Your diet, exercise and daily routine can all continue as normal.
You will usually need to have appointments with an endocrinologist every 6-12 months, although your doctor can provide support and repeat prescriptions in between these visits. As long as you take your medication, the symptoms of Addison's disease should completely disappear.
However, failing to take your medication could cause an adrenal crisis. Therefore, you must:
remember to collect your repeat prescriptions
keep spare medication as necessary - for example, in the car or at work and always carry some spare medication with you
take your medication every day at the right time of day
pack extra medication if you are going away - usually, double what you would normally need and your injection kit (see below, under emergency treatment) just in case you become ill
carry your medication in your hand luggage, if you are traveling by plane, with a note from your doctor explaining why it is necessary
You could also inform close friends or colleagues of your condition, and warn them about the risks of adrenal crisis.
Medical alert bracelets
It is also a good idea to wear a medical alert bracelet or necklace that informs people that you have Addison's disease.
After a serious accident, such as a car crash, your body should produce cortisol. This helps you to cope with the stressful situation and the additional strain on your body that results from serious injury. As your body cannot produce cortisol, you will need a hydrocortisone injection to replace it and prevent an adrenal crisis.
If you are wearing a medical alert bracelet it will inform any medical staff that may need to treat you about your condition, and what medication you require.
Medical alert bracelets or necklaces are pieces of jewelery that are engraved with your medical condition, in this case Addison's disease, as well as an emergency contact number. They are available from a number of retailers. Ask your doctor if there is one that they recommend, or go to the MedicAlert website.
If you need to stay in hospital, the healthcare professionals who are responsible for your care will also need to know that you need steroid replacement medication throughout your treatment.
Adjusting your medication
At certain times, your medication may need to be adjusted to take into account any additional strain on your body. For example, you may need to increase the dosage of your medication if you experience any of the following:
an illness or infection - particularly if you have a high temperature of 37.5C (99.5F)
an accident
you need to have an operation, dental or medical procedure (e.g. tooth filling, endoscopy)
you are taking part in strenuous exercise which is not usually part of your daily life
This will help your body to cope with the additional stress. Your doctor will monitor your dosage and advise you about any changes. Over time, as you get used to the condition and learn what can trigger your symptoms, you may learn how to adjust your medication yourself. However, always consult your doctor if you are unsure.
Emergency treatment
You and a partner or family member may be trained to administer an injection of hydrocortisone in an emergency. This could be necessary if you go into shock after an injury, or if you experience vomiting or diarrhea and are unable to keep down oral medication. This may occur if you are pregnant and have morning sickness.
Your endocrinologist will discuss with you when an injection might be necessary. You will be given needles, syringes and hydrocortisone, and training in how to use them.
If you need to administer emergency hydrocortisone, always call your doctor immediately afterwards. Check what out-of-hours services are available from your primary care trust in case the emergency is outside normal working hours.
You can also register yourself with your local ambulance service, so they have a record of your requirement for a steroid injection or tablets, if you need their assistance.
Treating adrenal crisis
Adrenal crisis, or Addisonian crisis, needs urgent medical attention. Dial 911 to request an ambulance if you or someone you know are experiencing adrenal crisis.
You will be given lots of fluid through a vein in your arm to rehydrate you. This will contain a mixture of salts and sugars (sodium, glucose and dextrose) to replace those that your body is lacking. You will also be injected with hydrocortisone to replace the missing hormone, cortisol.
Any underlying causes of the adrenal crisis, such as an infection, will also be treated.