Sheehan's syndrome is a condition that affects women who lose a life-threatening amount of blood or who have severe low blood pressure during or after childbirth. These factors can deprive your body of oxygen and can seriously damage vital tissues and organs. In the case of Sheehan's syndrome, the damage occurs to the pituitary gland — a small gland at the base of your brain.

Sheehan's syndrome causes the permanent underproduction of essential pituitary hormones (hypopituitarism). Also called postpartum hypopituitarism, Sheehan's syndrome is rare in industrialized nations. But it's still a major threat to women in developing countries.

Treatment of Sheehan's syndrome involves hormone replacement therapy.

Signs and symptoms of Sheehan's syndrome typically appear slowly, after a period of months or even years. But sometimes — such as in a breast-feeding mother — problems may appear right away.

Signs and symptoms of Sheehan's syndrome occur because of the deficiencies of the various hormones the pituitary gland controls: thyroid, adrenal, breast milk production and menstrual function. Signs and symptoms include:

  • Difficulty breast-feeding or an inability to breast-feed
  • No menstrual periods (amenorrhea) or infrequent menstruation (oligomenorrhea)
  • Loss of pubic or underarm hair
  • Slowed mental function, weight gain and difficulty staying warm as a result of an underactive thyroid (hypothyroidism)
  • Low blood pressure
  • Fatigue
  • Irregular heartbeat
  • Loss of interest in sex

For many women, Sheehan's syndrome symptoms are nonspecific and often attributed to other things. Fatigue, for instance, goes hand in hand with being a new mother. You might not realize that you have Sheehan's syndrome until you need treatment for thyroid or adrenal insufficiency.

It's also possible to remain relatively symptom-free with Sheehan's syndrome depending on the extent of damage to the pituitary gland. Some women live for years not knowing that their pituitary isn't working properly. Then an extreme physical stressor, such as severe infection or surgery, triggers an adrenal crisis.

Although many problems can lead to low pituitary function, 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, 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 your body — although signs and symptoms may develop so gradually that they escape notice.

Hormones that your pituitary secretes include:

  • Growth hormone (GH). This hormone controls bone and tissue growth and maintains the right balance of muscle and fat tissue.
  • Anti-diuretic hormone (ADH). By regulating urine production, this hormone manages water balance in your body. A deficiency of ADH results in excess urination and thirst, a condition called diabetes insipidus.
  • 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 men, LH regulates testosterone production. In women, it fosters production of estrogen.
  • Follicle-stimulating hormone (FSH). Working in tandem with LH, FSH helps stimulate sperm production in men and 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 pituitary damage is called secondary adrenal insufficiency.
  • Prolactin. This hormone regulates the development of female breasts, as well as the production of breast milk.

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, may increase your 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.

Because pituitary hormones control so many aspects of your metabolism, Sheehan's syndrome can cause a number of 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 cortisol, a powerful stress hormone.

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

If your primary care doctor suspects Sheehan's syndrome, you'll likely be referred to an endocrinologist, a doctor who specializes in hormonal disorders. To help prepare for your appointment:

  • Be aware of any pre-appointment restrictions. When you make your appointment, be sure to ask if there's anything you need to do to prepare for common diagnostic tests.
  • Write down all symptoms and changes you're experiencing, even if they seem unrelated to each other.
  • Make a list of your key medical information, including recent surgical procedures, the names of all medications you're taking and any other conditions for which you've been treated. Bring medical records from any previous pregnancies, especially those on labor and delivery.
  • Take a family member or friend, if possible. Sometimes it can be difficult to remember all of the information you learn during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Write down questions to ask your doctor.

Preparing a list of questions for your doctor will help you make the most of your time together. For Sheehan's syndrome, some basic questions to ask your doctor include:

  • What's the most likely cause of my symptoms?
  • What kinds of 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 for me?
  • I have other health conditions. How can I best manage these conditions together?
  • Are there any dietary or activity restrictions I need to follow?
  • Is there a generic alternative to the medicine you're prescribing?
  • Do you have any brochures I can take home with me? What websites do you recommend?

What to expect from your doctor

Your doctor is likely to ask you a number of questions, including:

  • Did you bleed heavily after your delivery?
  • Did you have any other complications during childbirth?
  • When did you begin experiencing symptoms?
  • 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?

Diagnosing Sheehan's syndrome can be difficult. Many of the 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 may have 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. If your doctor suspects pituitary insufficiency, you'll have blood tests to check your pituitary hormone levels.
  • Request a pituitary hormone stimulation test. You may need specialized stimulation testing of the pituitary hormones, which includes the injection of hormones and repeated blood tests to see how much your pituitary responds. This test is typically done after consulting an endocrinologist.
  • Request imaging tests. You may also need imaging tests, such as magnetic resonance imaging or computerized tomography, to check the size of your pituitary and to look for other possible reasons for your symptoms, such as a pituitary tumor.

Treatment for Sheehan's syndrome is lifelong hormone replacement therapy. Your doctor may recommend one or more of the following medications:

  • Corticosteroids. These drugs, such as hydrocortisone or prednisone, 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.

    Adjustments in dosage may also be necessary during pregnancy or with marked weight gain or weight loss. Avoiding doses higher than you need will eliminate 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 may include 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. And while estrogen replacement is available in either pills or patches, the patches seem to have a lower risk of side effects.

    Future pregnancies may be possible with preparations containing luteinizing hormone (LH) and follicle-stimulating hormone (FSH), also called gonadotropins. These can be administered by injection to stimulate ovulation. After age 50, which is 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. Some studies have shown that replacing growth hormone in women with Sheehan's syndrome — as well as in people with other forms of hypopituitarism — can help normalize the body's muscle-to-fat ratio, lower cholesterol levels and improve overall quality of life. 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 any hormones that you take. Generally, hormone levels are checked every few months at the beginning of treatment and then once a year thereafter.

Jan. 02, 2014