Overview

Sheehan's syndrome is a condition that affects women who lose a life-threatening amount of blood in childbirth or who have severe low blood pressure during or after childbirth, which can deprive the body of oxygen. This lack of oxygen that causes damage to the pituitary gland is known as Sheehan's syndrome.

Sheehan's syndrome causes the pituitary gland to not produce enough pituitary hormones (hypopituitarism). Also called postpartum hypopituitarism, Sheehan's syndrome is rare in industrialized nations, largely because care during pregnancy and childbirth is better than in developing countries.

Treatment of Sheehan's syndrome involves lifelong hormone replacement therapy.

Symptoms

Signs and symptoms of Sheehan's syndrome typically appear slowly, after a period of months or years. But sometimes problems appear right away, such as the inability to breast-feed.

Signs and symptoms of Sheehan's syndrome occur because of having too little of the hormones the pituitary gland produces. Signs and symptoms include:

• Difficulty breast-feeding or an inability to breast-feed
• No menstrual periods or infrequent menstruation
• Inability to regrow shaved pubic hair
• Slowed mental function, weight gain and difficulty staying warm as a result of an underactive thyroid (hypothyroidism)
• Low blood pressure
• Low blood sugar
• Fatigue
• Irregular heartbeat
• Breast shrinkage

For many women, Sheehan's syndrome symptoms are often thought to be caused by other things. Fatigue, for instance, is commonly experienced by new mothers. You might not realize you have Sheehan's syndrome until you need treatment for thyroid or adrenal insufficiency.

It's also possible to remain relatively symptom-free if you have Sheehan's syndrome, depending on the extent of damage to the pituitary gland. Some women live for years without knowing that their pituitary gland isn't working properly. Then an extreme physical stressor, such as severe infection or surgery, triggers an adrenal crisis, a serious condition in which your adrenal glands produce too little of the hormone cortisol.

Causes

Sheehan's syndrome is caused by severe blood loss or extremely low blood pressure during or after childbirth. These factors can be particularly damaging to the pituitary gland, which enlarges during pregnancy, destroying hormone-producing tissue so that the gland can't function normally.

Pituitary hormones regulate the rest of your endocrine system, signaling other glands to increase or decrease production of the hormones that control metabolism, fertility, blood pressure, breast milk production and many other vital processes. A lack of any of these hormones can cause problems throughout the body.

Hormones from the front of your pituitary gland include:

• Growth hormone (GH). This hormone controls bone and tissue growth and maintains the right balance of muscle and fat tissue.
• Thyroid-stimulating hormone (TSH). This hormone stimulates your thyroid gland to produce key hormones that regulate your metabolism. Shortage of TSH results in an underactive thyroid gland (hypothyroidism).
• Luteinizing hormone (LH). In women, LH regulates estrogen.
• Follicle-stimulating hormone (FSH). Working with LH, FSH helps stimulate egg development and ovulation in women.

• Adrenocorticotropic hormone (ACTH). This hormone stimulates your adrenal glands to produce cortisol and other hormones. Cortisol helps your body deal with stress and influences many body functions, including blood pressure, heart function and your immune system.

  A low level of adrenal hormones caused by damage to the pituitary gland is called secondary adrenal insufficiency.

• Prolactin. This hormone regulates the development of female breasts, as well as the production of breast milk.

Risk factors

Any condition that increases the chance of severe blood loss (hemorrhage) or low blood pressure during childbirth, such as being pregnant with multiples or having a problem with the placenta, can increase the risk of Sheehan's syndrome.

Hemorrhage is a rare childbirth complication, however, and Sheehan's syndrome is even more uncommon. Both risks are greatly reduced with proper care and monitoring during labor and delivery.

Complications

Because pituitary hormones control many aspects of your metabolism, Sheehan's syndrome can cause many problems, including:

• Adrenal crisis, a serious condition in which your adrenal glands produce too little of the hormone cortisol
• Low blood pressure
• Unintended weight loss
• Menstrual irregularities

Adrenal crisis: Life-threatening situation

The most serious complication is adrenal crisis, a sudden, life-threatening state that can lead to extremely low blood pressure, shock, coma and death.

Adrenal crisis usually occurs when your body is under marked stress — such as during surgery or a serious illness — and your adrenal glands produce too little of a powerful stress hormone (cortisol).

Because of the potentially serious consequences of adrenal insufficiency, your doctor is likely to recommend that you wear a medical alert bracelet.

Diagnosis

Diagnosing Sheehan's syndrome can be difficult. Many symptoms overlap with those of other conditions. To diagnose Sheehan's, your doctor likely will:

• Collect a thorough medical history. It's important to mention any childbirth complications you've had, no matter how long ago you gave birth. Also, be sure to tell your doctor if you didn't produce breast milk or you failed to start menstruating after delivery — two key signs of Sheehan's syndrome.
• Run blood tests. Blood tests will check your pituitary hormone levels.
• Request a pituitary hormone stimulation test. You might need stimulation testing of the pituitary hormones, which involves injecting hormones and running repeated blood tests to see how your pituitary responds. This test is typically done after consulting a doctor who specializes in hormonal disorders (endocrinologist).
• Request imaging tests. You might also need imaging tests, such as an MRI scan or CT scan, to check the size of your pituitary gland and to look for other possible reasons for your symptoms, such as a pituitary tumor.

Treatment

Treatment for Sheehan's syndrome is lifelong hormone replacement therapy for the hormones you're missing. Your doctor might recommend one or more of the following medications:

• Corticosteroids. Hydrocortisone (Cortef) or prednisone (Rayos), replace the adrenal hormones that aren't being produced because of an adrenocorticotropic hormone (ACTH) deficiency.

  You'll need to adjust your medication if you become seriously ill or experience major physical stress. During these times, your body would ordinarily produce extra cortisol — a stress hormone. The same kind of dosage fine-tuning may be necessary when you have the flu, diarrhea or vomiting, or have surgery or dental procedures.

  Dosage adjustments might also be necessary during pregnancy or with marked weight gain or weight loss. Taking the appropriate amount can help avoid the side effects associated with high doses of corticosteroids.

• Levothyroxine (Levoxyl, Synthroid, others). This medication boosts deficient thyroid hormone levels caused by low or deficient thyroid-stimulating hormone (TSH) production.

  If you change brands, let your doctor know to ensure you're still receiving the right dosage. Also, don't skip doses or stop taking the drug because you're feeling better. If you do, signs and symptoms will gradually return.

• Estrogen. This includes estrogen alone if you've had your uterus removed (hysterectomy) or a combination of estrogen and progesterone if you still have your uterus.

  Estrogen use has been linked to an increased risk of blood clots and stroke in women who still make their own estrogen. The risk should be less in women who are replacing missing estrogen.

  Preparations containing luteinizing hormone (LH) and follicle-stimulating hormone (FSH), also called gonadotropins, might make future pregnancies possible. These can be delivered by injection to stimulate ovulation.

  After age 50, around the time of natural menopause, discuss the risks and benefits of continuing to take estrogen or estrogen and progesterone with your doctor.

• Growth hormone. Growth hormone may improve the body's muscle-to-fat ratio, maintain bone mass and lower cholesterol levels. Growth hormone is expensive, and side effects may include joint stiffness and fluid retention.

Your endocrinologist is likely to test your blood regularly to make sure that you're getting adequate — but not excessive — amounts of hormones.

Preparing for an appointment

If your primary care provider suspects Sheehan's syndrome, you'll likely be referred to a doctor who specializes in hormonal disorders (endocrinologist).

Here's information to help you get ready for your appointment:

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a specific test. Make a list of:

• Your symptoms, even if they seem unrelated to each other, and when they began
• Key personal information, including recent surgical procedures and other major stresses, and your family medical history
• All medications, vitamins or other supplements you take, including doses
• Questions to ask your doctor

Bring medical records from previous pregnancies, especially those on labor and delivery. If possible, take a family member or friend to help you remember the information you're given.

For Sheehan's syndrome, basic questions to ask your doctor include:

• What's the most likely cause of my symptoms?
• What tests do I need?
• Is Sheehan's syndrome temporary, or will I always have it?
• Will I be able to have another child?
• What treatments are available, and what do you recommend?
• I have other health conditions. How can I best manage them together?
• Are there dietary or activity restrictions I need to follow?
• Are there brochures or other printed material I can have? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Your doctor is likely to ask you questions, including:

• Did you bleed heavily after your delivery?
• Did you have other complications during childbirth?
• Do you have symptoms all the time, or do they come and go?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• Does anything seem to make your symptoms worse?