Addison Disease in Children

What is Addison disease in a child?

Addison disease, also called primary adrenal insufficiency, is when the adrenal glands don't make enough of the "stress" hormone called cortisol. Cortisol controls metabolism, blocks inflammatory reactions, and affects the immune system. It's known for its central role in the "fight or flight" response, which is our body's response to emergencies. Often, aldosterone and androgens, the 2 other hormones made by the adrenal glands, are low too. Aldosterone manages sodium and potassium levels and is involved in maintaining blood pressure. The adrenal glands sit on top of the kidneys. There is one gland on top of each kidney. Addison disease is fairly rare, but is more common in women. It may first appear at any age.

What causes Addison disease in a child?

Primary adrenal insufficiency, or Addison's disease, is usually caused by an autoimmune condition where one's own immune system targets the adrenal glands. Other cases of Addison disease are caused by the damage to the glands by any of these:

• Cancer
• Surgical removal of adrenal glands
• Infection
• A rare genetic disease

In rare cases, Addison disease is passed down in a family.

Which children are at risk for Addison disease?

A child is at risk for Addison disease if they have any of these:

• An autoimmune disease such as type 1 diabetes or certain forms of thyroid disease
• Vitiligo or other autoimmune problems
• Cancer
• Infection, such as tuberculosis
• Family history of the disease

What are the symptoms of Addison disease in a child?

Mild symptoms may only occur when a child is under physical stress. Symptoms may include:

• Muscle weakness
• Fatigue
• Dizziness
• Fast pulse
• Dark skin, first seen on hands and face
• Black freckles
• Bluish-black color around the nipples, mouth, rectum, scrotum, or vagina
• Weight loss
• Dehydration
• Loss of appetite
• Intense salt craving
• Muscle aches
• Nausea
• Vomiting
• Diarrhea
• Intolerance to cold

The symptoms of Addison disease can be like other health conditions. Make sure your child sees their healthcare provider for a diagnosis.

How is Addison disease diagnosed in a child?

The healthcare provider will ask about your child’s symptoms and health history. They may also ask about your family’s health history. Your child will have a physical exam and blood tests. These tests are done to check cortisol and other adrenal hormone levels and sodium and potassium levels.

How is Addison disease treated in a child?

The goal of treatment is to replace the hormones and to relieve the symptoms. Addison disease can be life-threatening. Because of this, treatment often starts right away with corticosteroid medicine. This medicine may be taken by mouth. Or it may be given by IV. It depends on how sick your child is. In most cases, corticosteroid medicine must be taken for life. Treatment may also include a medicine that helps manage the body's levels of sodium and potassium.

What are possible complications of Addison disease in a child?

If left untreated, Addison disease may lead to:

• Severe abdominal (belly) pain
• Extreme weakness
• Low blood pressure
• Kidney failure
• Shock (low blood pressure) from dehydration
• Low blood sugar (hypoglycemia)

Severe complications are most likely to occur when the child is under physical stress.

Lack of adrenal hormones may also cause:

• High blood levels of potassium, which affect the water and sodium levels in the body
• Extreme sensitivity to the hormone insulin, which may lead to low blood sugar levels

Helping a child live with Addison disease

Addison disease is a lifelong condition that needs lifetime treatment. Stressful events, such as surgery, infection, or injury, can cause severe symptoms of Addison disease. This is because corticosteroids help the body fight infection and stay healthy during physical stress. Talk with your child’s healthcare provider if your child needs surgery. Get medical care for your child right away if they:

• Have vomiting or diarrhea
• Have any other infection or illness

Your child should wear a medical alert bracelet or necklace. Managing your child’s chronic illness can be stressful for all members of the family. Let your healthcare provider know if you, your child with Addison disease, or other family members need help dealing with the physical, emotional, and financial issues that the illness can cause.

When should I call my child's healthcare provider?

Call your child's healthcare provider if your child has any symptoms of Addison disease.

If your child has Addison disease, talk with your child’s healthcare provider if your child needs surgery. Get medical care for your child right away if they:

• Have vomiting or diarrhea
• Have any other infection or illness

Key points about Addison disease in a child

• Addison disease is when the adrenal glands don't make enough cortisol and aldosterone.
• Addison disease is a lifelong condition. It needs lifetime treatment.
• The most common cause of Addison disease is damage to the adrenal glands caused by an autoimmune condition.
• Mild symptoms of Addison disease may only appear when a child is under physical stress. Symptoms may include muscle weakness, fatigue, and dizziness.
• Addison disease can be life-threatening. Because of this, treatment often starts right away with corticosteroid medicine.
• If left untreated, Addison disease may lead to severe weakness, kidney failure, and shock.
• Stressful events, such as surgery, infection, or injury, can cause severe symptoms of Addison. Get medical care for your child right away if they have vomiting, diarrhea, or other illness.
• Let your healthcare provider know if you, your child with Addison disease, or other family members need help dealing with the physical, emotional, and financial issues that the illness can cause.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.
• Before your visit, write down questions you want answered.
• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
• Ask if your child’s condition can be treated in other ways.
• Know why a test or procedure is recommended and what the results could mean.
• Know what to expect if your child does not take the medicine or have the test or procedure.
• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.