What is Sheehan Syndrome?
Sheehan syndrome, also known as post-partum pituitary necrosis, is a condition that happens when the cells of the anterior pituitary gland, an organ located at the base of the brain that produces important hormones, die. This typically happens after severe bleeding, reduced blood volume (hypovolemia), and shock after childbirth. Thanks to advancements in childbirth care in developed countries, the occurrence of this condition has decreased. However, it remains a major health risk causing illness and even death in less developed countries. This disorder was first identified by a British pathologist named Harold Leeming Sheehan in 1937.
What Causes Sheehan Syndrome?
Sheehan syndrome is a condition that happens when the pituitary gland, found just below the base of the brain, gets damaged due to severe blood loss. This typically happens after childbirth if the mother loses a lot of blood. Because of this blood loss, the pituitary gland, which is responsible for the production of hormones, might fail to function properly.
Diagnosing Sheehan syndrome isn’t always straightforward and obvious right after childbirth. In fact, its signs and symptoms might only start to show up months after giving birth, or even after a severe traumatic event. The earliest and most frequent symptom of Sheehan syndrome is the absence of milk production, known as agalactorrhea.
There can be other symptoms too, usually related to the decrease in pituitary hormones. These could include amenorrhea (no menstrual periods) or oligomenorrhea (infrequent menstrual periods), hot flashes, a drop in sex drive, fatigue, slow heart rate (bradycardia), low blood pressure (hypotension), weight gain, and constipation. Women might also experience the loss of under-arm and pubic hair. In some cases, adrenal insufficiency, a condition where adrenal glands don’t produce enough hormones, leading to fatigue and weight loss, can occur.
Some test results may support a diagnosis of Sheehan syndrome. These might include low sodium levels (hyponatremia), anemia (not enough healthy red blood cells), and low blood sugar (hypoglycemia).
Risk Factors and Frequency for Sheehan Syndrome
Sheehan syndrome is a condition that’s not often seen in developed countries, thanks to advanced childbirth practices and good access to medical expertise and facilities. However, it can sometimes occur as a result of complications during childbirth in developing and lower-income countries. Some research suggests the rate of Sheehan syndrome can be as much as five cases in every 100,000 births.
Signs and Symptoms of Sheehan Syndrome
Sheehan syndrome, a condition that affects some women after childbirth, can develop in either an acute or chronic form. More people experience the chronic form, but both could appear months or even years after the initial triggering event, which is usually a critical drop in blood pressure and shock.
The acute version of Sheehan syndrome becomes apparent when the new mother struggles with breastfeeding or can’t produce milk (a condition known as agalactorrhea). Despite this, many women might not show symptoms for months or even years after giving birth. New moms may have issues with menstruation or may not have periods until Sheehan syndrome is diagnosed and treated. Getting a swift diagnosis and treatment of acute Sheehan syndrome after childbirth is crucial. It’s typical for long-lasting low blood pressure and fast heart rate to mimic shock. However, persistent low sodium and glucose levels may facilitate the diagnosis of Sheehan syndrome.
Chronic Sheehan syndrome is diagnosed when symptoms associated with a disruption in the pituitary gland, or hypopituitarism, present themselves. This can happen months to years following the initial damage to the pituitary gland. Symptoms can include:
- Fatigue
- Weakness
- Hair loss
- Constipation
- Weight gain
- Difficulty concentrating or shortened attention span
- Intolerance to cold
Healthcare professionals may note low blood pressure and a slow heart rate during a physical exam. Patients may also display secondary adrenal insufficiency which often has symptoms of fatigue and weight loss. Lab results may show low blood sugar, low sodium, and/or anemia.
Testing for Sheehan Syndrome
If your doctor suspects you may have Sheehan syndrome, they will order a blood test to check the function of your anterior pituitary gland. This gland produces several hormones like gonadotropins ( follicle-stimulating hormone and luteinizing hormone), growth hormone, prolactin, adrenocorticotropic hormone, and thyroid-stimulating hormone. When the gland is damaged, these hormones are affected in a sequence, with growth hormone affected first and thyroid-stimulating hormone last.
The tests that would be done include a full blood count, basic metabolic profiling, thyroid function tests, and measurements of the levels of the hormones listed above. If the test results show low levels of these hormones and you have symptoms suggesting Sheehan syndrome, your doctor would make a diagnosis.
Other lab tests that might be ordered if Sheehan syndrome is suspected can include ones related to blood cell counts and platelet counts. You might also have blood sugar and sodium levels checked. There are some less common tests that could be considered to confirm the diagnosis including screening for genetic mutations related to blood clotting, tests for anti-pituitary and anti-hypothalamus antibodies, and the measurement of clotting time.
In addition to lab tests, an MRI scan of the pituitary can be done to confirm the diagnosis. About 70% of patients with Sheehan syndrome will show an “empty sella” on this scan, which means the pituitary gland looks smaller than normal or appears missing. A further 30% might show a partially empty sella. Initially, the MRI may show signs of damage to the pituitary gland without bleeding, but as Sheehan syndrome progresses, the scan will show a shrunken pituitary gland and eventually a partially or completely empty sella.
Treatment Options for Sheehan Syndrome
Sheehan syndrome is a condition that affects your hormone levels, and the main form of treatment is to replace the hormones that your body is not producing enough of. This replacement has to continue for the rest of your life. If you aren’t producing enough thyroid hormones, medications like levothyroxine or liothyronine can be used to replace them. If the issue is with cortisol, a steroid hormone, medications like prednisone or hydrocortisone can help.
For women who aren’t producing enough gonadotropins, which are hormones related to reproduction, doctors usually recommend estrogen. If the woman still has a uterus, the treatment should also include progesterone. However, like all treatments, this one comes with risks. There are certain conditions that are more likely when taking these hormones, such as blood clots, cancer, and heart attacks. That’s why it’s crucial to have a discussion with your doctor about the potential benefits and risks of this treatment.
Another hormone that often needs to be replaced in people with Sheehan syndrome is growth hormone. The exact dosage required can vary from person to person. In some cases, patients can develop a condition called diabetes insipidus, which is not related to sugar diabetes but is about water balance in the body. For treating this problem, desmopressin (also known as DDAVP) is usually the preferred medication.
If you have Sheehan syndrome, or suspect you might, it’s also advisable to be referred to an endocrinologist who has experience with hormone imbalances and specific treatment options like growth hormone therapy.
What else can Sheehan Syndrome be?
When trying to diagnose a specific condition, doctors need to consider other ailments that might show similar symptoms. These might include:
- Addison syndrome, a disorder that occurs when the body produces insufficient amounts of certain hormones
- Lymphocytic hypophysitis, a rare condition that causes inflammation of the pituitary gland
- Pan-hypopituitarism, a condition where the pituitary gland does not produce enough hormones
- Pituitary apoplexy, a medical emergency caused by bleeding or impaired blood supply to the pituitary gland.
What to expect with Sheehan Syndrome
The outcome of this condition largely depends on how quickly it is diagnosed and if hormone therapy is started promptly. If it is not discovered and addressed early, the result can potentially be very serious.
Possible Complications When Diagnosed with Sheehan Syndrome
The possibility of complications, such as hypothyroidism and Addisonian crisis, and even death exists.
Common Complications:
- Hypothyroidism
- Addisonian crisis
- Death
Preventing Sheehan Syndrome
The treatment involves taking hormone replacements for the rest of your life. Because of this, it’s extremely important that pharmacists and nurses explain to patients how critical it is to take their medication as advised. If patients don’t take their medication properly, they could experience severe hormone imbalances that could potentially be life-threatening. Patients who are undergoing this treatment need to be monitored closely for the rest of their lives, including regular check-ups to keep an eye on their hormone levels. This helps ensure that the treatment continues to work effectively.
