What is cortisol deficiency?Cortisol deficiency occurs when the body does not make enough cortisol to meet its needs.Cortisol is a hormone that helps keep:

• blood pressure normal

• blood sugar normal

It is especially important when the body must cope with:

• infection

• an injury or illness

• an operation

What causes cortisol deficiency?Cortisol is released by the adrenal glands, which sit on top of the kidneys. The adrenal glands release cortisol into the blood when they are "told" to do so by the pituitary gland in the brain. If the level of cortisol in the body is low, then the pituitary gland releases a hormone called adrenocorticotropic hormone (ACTH), which "tells" the adrenal glands to release more cortisol. If the adrenal glands are not working well (as in Addison's disease or congenital adrenal hyperplasia), then they cannot produce enough cortisol to meet the body's needs. Cortisol deficiency can also occur if the pituitary gland does not work at all, or if it works poorly. ACTH may not be produced (or too little may be produced) and the adrenal glands do not receive a message to release cortisol. In both cases, the body does not have enough cortisol to meet its needs.

How is cortisol deficiency diagnosed?Your child will have a blood test done. The doctor will decide what testing is needed. It may be a single blood test or a test in which several blood samples are drawn both before and after the nurse gives your child ACTH (this medicine helps the doctor to assess if your child is able to respond to stress or illness).How is cortisol deficiency treated?If your child does not produce enough cortisol, then he or she will need to take cortisol by mouth two to three times per day, every day. Your child will need extra cortisol when ill. Sometimes, a child may need cortisol only when ill. Your doctor will give you a prescription and instructions for taking cortisol that meets your child's needs.How do I know when to give my child extra cortisol?Illness and injury create stress in the body. During times of stress, your body needs extra cortisol. Stress includes:

• fever of 38.5 degrees C or higher

• illness causing lethargy (feel very tired)

• any infection, sprain or fracture

• if your child has an accident and needs to see a doctor.

• if your child needs an anesthetic for a medical or dental treatment, special x-rays, or surgery

The dentist or doctor must be told that your child needs cortisol replacement. Extra cortisol is not needed for routine immunizations, but is needed if your child develops a fever. This extra cortisol is referred to as a "stress dose", and is 2 to 3 times higher than the regular cortisol dose. Your child should have stress doses of cortisol for as long as he or she is ill or injured. Please contact your endocrine clinic nurse or doctor if:

• you need to give stress doses of cortisol for more than 3– 5 days

• you are unsure about whether to give stress doses of cortisol

What should I do if my child is vomiting or has diarrhea?If your child vomits less than a ½ hour after taking cortisol, then you should give the whole dose again. If vomiting occurs after the repeated dose, then your child will need an injection of cortisol (hydrocortisone). The dose of hydrocortisone can be found on the illness management letter that you received at your last clinic visit. Oral cortisol is not absorbed in a child with severe diarrhea, and an injection of hydrocortisone may be needed. Any time your child needs an injection of hydrocortisone it is a medical emergency. Your child should be seen by a doctor in the nearest emergency room. Many parents are choosing to learn how to give this injection to ensure their child receives hydrocortisone without delay. This is especially important if you are travelling or live far away from the nearest emergency room. Your nurse can show you how to give a hydrocortisone injection.

Every time you give a hydrocortisone injection, your child will still need to be seen as soon as possible by a doctor at the nearest emergency room.

What are the signs that my child is not getting enough cortisol?Signs include:

• stomach or back pain

• pallor (paleness), tiredness

• fainting

• fever

• nausea, vomiting, or weight loss

• disease symptoms return
  shock

What are the signs that my child is getting too much cortisol?Signs include:

• weight gain

• mood swings

• change in weight distribution (fullness in the face, extra weight at upper back and belly)

• stretch marks on the skin

• failure to grow in height
  If your child has signs of too much or too little cortisol, call your doctor or the endocrine clinic nurse.

What is acute adrenal insufficiency?If your child does not have enough cortisol, then blood pressure and blood sugar can become quite low, causing acute adrenal insufficiency. Acute adrenal insufficiency is a medical emergency.Signs of acute adrenal insufficiency are:

• nausea or vomiting

• cold, clammy skin

• fast heart rate and breathing rate

• weakness, very tired

• dizziness, confusion

• pale face, dark circles under the eyes

• signs of dehydration (dry tongue, thirst)

• lower body temperature

• severe pains in the stomach, legs and back

• loss of consciousness

• coma

If your child has these signs and symptoms, medical attention is needed right away. Call 911. If you have been shown how to give a hydrocortisone injection, give it as soon as possible.

All children with cortisol deficiency should wear a medical alert bracelet/necklace at all times.

Children with cortisol deficiency need regular follow-up visits with their doctor to ensure that the dose of cortisol is correct. By following these guidelines, you will be doing your part to make sure that your child stays healthy.

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Reviewed by Alberta clinical experts. Brought to you by HealthLink Alberta. Copyright.

This material is designed for information purposes only. It should not be used in place of medical advice, instruction and/or treatment.