Primary ovarian insufficiency occurs when the ovaries stop working as they should before age 40. When this happens, the ovaries don't make the typical amounts of the hormone estrogen or release eggs regularly. The condition often leads to infertility. Another name for primary ovarian insufficiency is premature ovarian insufficiency. It also used to be called premature ovarian failure, but this term isn't used anymore.

Sometimes, primary ovarian insufficiency is confused with premature menopause. But they aren't the same. People with primary ovarian insufficiency can have irregular or occasional periods for years. They might even get pregnant. But people with premature menopause stop having periods and can't become pregnant.

Treatment can restore estrogen levels in people with primary ovarian insufficiency. This helps prevent some conditions that can happen due to low estrogen, such as heart disease and weak, brittle bones.

Symptoms of primary ovarian insufficiency are like those of menopause or low estrogen. They include:

• Irregular or missed periods. This symptom might be present for years. It also could develop after a pregnancy or after stopping birth control pills.
• Trouble getting pregnant.
• Hot flashes and night sweats.
• Vaginal dryness.
• Anger, depression or anxiety.
• Trouble with focus or memory.
• Less sexual desire.

When to see a doctor

If you've missed your period for three months or more, see your health care team to figure out the cause. You can miss your period for many reasons, such as pregnancy, stress, or a change in diet or exercise habits. But it's best to get a health care checkup whenever your menstrual cycle changes.

Even if you don't mind not having periods, see a health care professional to find out what's causing the change. Low estrogen levels can lead to a condition that causes weak and brittle bones, called osteoporosis. Low levels of estrogen also can lead to heart disease.

Primary ovarian insufficiency may be caused by:

• Chromosome changes. Chromosomes are thread-like structures that contain genes. Most often, people assigned female at birth have two X chromosomes in their cells. But some people with primary ovarian insufficiency have one typical X chromosome and one altered X chromosome. This can be a sign of genetic conditions such as mosaic Turner syndrome. Other people with primary ovarian insufficiency have X chromosomes that are fragile and break. This is called fragile X syndrome.
• Toxins. Chemotherapy and radiation therapy are common causes of toxin-induced ovarian insufficiency. These treatments can damage genetic material in cells. Other toxins such as cigarette smoke, chemicals, pesticides and viruses might speed up ovarian insufficiency.
• An immune system response to ovarian tissue. This also is called autoimmune disease. In this rare form, the immune system makes protective proteins that attack ovary tissue by mistake. This harms sacs in the ovaries that each contain an egg, called follicles. It also damages the egg. What triggers the immune response is unclear. But being exposed to a virus may play a role.
• Unknown factors. Most often, the cause of primary ovarian insufficiency isn't clear. You might hear this called an idiopathic cause. Your health care professional may recommend more testing to try to find the cause.

Factors that raise the risk of primary ovarian insufficiency include:

• Age. The risk goes up between ages 35 and 40. Primary ovarian insufficiency is rare before age 30. But younger people and even teens can get it.
• Family history. Having a family history of primary ovarian insufficiency raises the risk of getting this condition.
• Ovarian surgery. Surgeries that involve the ovaries raise the risk of primary ovarian insufficiency.

Primary ovarian insufficiency can lead to other health conditions, including the following:

• Infertility. Not being able to get pregnant can be a complication of primary ovarian insufficiency. Rarely, pregnancy is possible until the body's supply of eggs runs out.
• Osteoporosis. This condition causes bones to become weak, brittle and more likely to break. Women with low levels of the hormone estrogen have a higher risk of getting osteoporosis. That's because estrogen helps keep bones strong.
• Depression or anxiety. Some people with primary ovarian insufficiency become depressed or anxious. This can be due to the risk of infertility and other conditions that arise from low estrogen levels.
• Heart or blood vessel disease. Early loss of estrogen might raise the risk of heart conditions or stroke.
• Dementia. This is the term for a group of symptoms that affect memory, thinking and social skills. The dementia risk may be linked with getting both ovaries removed and not receiving estrogen therapy afterward in people under age 43.
• Parkinson's disease. This long-term condition affects the nervous system, which includes the brain and spinal cord. It also affects parts of the body controlled by nerves. The higher risk of Parkinson's disease also may be linked with surgery to remove the ovaries.

Treatment for primary ovarian insufficiency helps prevent these other health conditions.

Most women have few signs of primary ovarian insufficiency, but your health care provider may suspect the condition if you have irregular periods or are having trouble conceiving. Diagnosis usually involves a physical exam, including a pelvic exam. Your provider might ask questions about your menstrual cycle, exposure to toxins, such as chemotherapy or radiation therapy, and previous ovarian surgery.

Your provider might recommend one or more tests to check for:

• Pregnancy. A pregnancy test checks for an unexpected pregnancy if you're of childbearing age and missed a period.
• Hormone levels. Your provider may check the levels of a number of hormones in your blood, including follicle-stimulating hormone (FSH), a type of estrogen called estradiol, and the hormone that stimulates breast milk production (prolactin).
• Chromosome changes or certain genes. You may have a blood test called a karyotype analysis to look for unusual changes in your chromosomes. Your doctor may also check to see if you have a gene associated with fragile X syndrome called FMR1.

Most often, treatment for primary ovarian insufficiency focuses on the problems that arise from estrogen deficiency. (1p3) Treatment might include:

• Estrogen therapy. Estrogen therapy can help prevent osteoporosis. It also can relieve hot flashes and other symptoms of low estrogen. You'll likely be prescribed estrogen with the hormone progesterone if you still have your uterus. Adding progesterone protects the lining of your uterus, called the endometrium, from changes that could lead to cancer. These changes may be caused by taking estrogen alone.

  The combination of hormones may make your period come back. It won't restore your ovaries' function. Depending on your health and preference, you might take hormone therapy until around age 50 or 51. That's the average age of natural menopause.

  In older women, long-term estrogen plus progesterone treatment has been linked to a higher risk of heart and blood vessel disease and breast cancer. In young people with primary ovarian insufficiency, the benefits of hormone therapy outweigh the risks.

• Calcium and vitamin D supplements. Both nutrients are key for preventing osteoporosis. And you might not get enough of either in your diet or from sunlight. Your health care team might suggest an X-ray test that measures calcium and other minerals in bones before you start supplements. This is called a bone density test.

  For women ages 19 through 50, experts most often recommend 1,000 milligrams (mg) of calcium a day through food or supplements. The amount increases to 1,200 mg a day for women age 51 and older.

  The ideal daily dose of vitamin D isn't yet clear. A good starting point is 800 to 1,000 international units (IU) a day, through food or supplements. If your blood levels of vitamin D are low, your health care team might suggest higher amounts.

Addressing infertility

No treatment is proven to restore fertility. But some people with primary ovarian insufficiency and their partners try to become pregnant through a procedure called in vitro fertilization. The procedure involves removing eggs from a donor and fertilizing them with sperm. A fertilized egg, called an embryo, is then placed in the uterus.

Learning that you have primary ovarian insufficiency may be emotionally painful. But with proper treatment and self-care, you can expect to lead a healthy life.

• Learn about other ways to have children. If you'd like to add to your family, talk to a health care professional about your options. For instance, you could think about trying in vitro fertilization using donor eggs. Or you could adopt a child.
• Talk with your health care team about the best birth control options. A small percentage of people with primary ovarian insufficiency do become pregnant if they have sex without a condom. If you don't want to become pregnant, think about using birth control.
• Keep your bones strong. Eat a calcium-rich diet. Do weight-bearing exercises such as walking and strength training exercises for your upper body. And don't smoke. Ask your health care team if you need calcium and vitamin D supplements.
• Keep track of your menstrual cycle. If you miss a period while taking hormone therapy that causes you to have a monthly cycle, get a pregnancy test.

If you'd hoped for future pregnancies, you might feel a deep sense of loss after you learn that you have primary ovarian insufficiency. This feeling can happen even if you've already given birth. See a counselor for therapy if you feel it would help you cope.

• Be open with your partner. Talk with and listen to your partner. Share your feelings over this sudden change in your plans for growing your family.
• Explore your options. If you don't have children and want them, or if you want more children, look into other ways to expand your family. You could think about choices such as in vitro fertilization using donor eggs or adoption.
• Get support. It may help to talk with others who are going through a similar challenge. You could gain insights and understanding during a time of confusion and doubt. Ask a member of your health care team about national or local support groups. Or seek an online community as an outlet for your feelings and a source of information. Also think about getting counseling with a therapist. It might help you adjust to your new circumstances and what those could mean for your future.
• Give yourself time. It can take a while to come to terms with having primary ovarian insufficiency. In the meantime, take good care of yourself. Eat well, exercise and get enough rest.

Your first checkup likely will be with your primary care professional or a gynecologist. If you're seeking treatment for infertility, you might be referred to a specialist in reproductive hormones and improved fertility. This is a doctor called a reproductive endocrinologist.

What you can do

When you make the appointment, ask if there's anything you need to do in advance. For instance, you may need to stop eating for a number of hours before you have a certain test. This is called fasting.

Also make a list of:

• Your symptoms. Include any missed periods and how long you've been missing them.
• Key personal information. Write down major stresses, recent life changes and your family medical history.
• Your health history. It's key to include your reproductive history. This can include information about your birth control use and any pregnancies or breastfeeding. Also note any past surgeries on your ovaries and anytime you might have been exposed to chemicals or radiation.
• All medicines, vitamins or other supplements you take. Include the amounts you take, also called the doses.
• Questions to ask your health care team.

Take a family member or friend along if you can. This person can help you remember all the information that your health care team gives you.

For primary ovarian insufficiency, some questions to ask your health care professional include:

• What's the most likely cause of my irregular periods?
• What other causes might there be?
• What tests do I need?
• What treatments are available? What side effects can I expect?
• How will these treatments affect my sexuality?
• What do you feel is the best course of action for me?
• I have other health conditions. How can I best manage them together?
• Should I see a specialist?
• Do you have printed material I can have? What websites do you recommend?

Feel free to ask other questions as they occur to you during your appointment.

What to expect from your provider

Your health care professional is likely to ask questions, such as:

• When did you start missing periods?
• Do you have hot flashes, vaginal dryness or other symptoms like those of menopause? For how long?
• Have you had ovarian surgery?
• Have you been treated for cancer?
• Do you or any family members have systemic or autoimmune diseases, such as hypothyroidism or lupus?
• Do any members of your family have primary ovarian insufficiency?
• How distressed do your symptoms make you feel?
• Do you feel depressed?
• Have you had trouble with previous pregnancies?