By LOUISE CARR, Associate Editor and Featured Columnist
Did you know there's really no such a thing as an hermaphrodite? And that Swyer syndrome cannot accurately be described as hermaphroditism? Shrouded in myth and misinformation, it can be difficult to get many straight facts about Swyer syndrome and other intersex conditions. Swyer syndrome may be rare - occurring in around 1 in 30,000 people according to the U.S. National Library of Medicine - but if you suffer from it you're not abnormal. Swyer syndrome can be difficult to deal with due to negative reactions from people around you. How do you deal with Swyer syndrome? What can you do to help handle the problems Swyer syndrome causes in your life?
What is Swyer Syndrome?
Swyer syndrome was first described in medical literature by Dr G.I.M. Swyer of University College Hospital in London in 1955. Swyer syndrome is a genetic condition where individuals have a male XY chromosome pattern but a female appearance. Swyer syndrome is also known as XY gonadal dysgenesis. If you suffer from Swyer syndrome you have female external genitalia, a uterus and fallopian tubes but you do not have working gonads - ovaries or testes. Most cases of Swyer syndrome result from new mutations in the genes and affect people with no history of the condition in their family.
Swyer syndrome is a type of intersex condition, where there is a discrepancy between the external genitals and the internal genitals. Hermaphroditism is an older term taken from the names of Greek gods Hermes and Aphrodite used to mean a person is both fully male and fully female - a physiological impossibility. Most experts believe the term hermaphrodite is misleading and insensitive so the accepted term today is intersex, or disorder of sex development.
How To Cope Emotionally With Swyer Syndrome
The emotional consequences of a Swyer syndrome diagnosis can be hard to deal with. The issues surrounding sexuality and gender are complex and many people with Swyer syndrome can find it hard to come to terms with their identity. Ideally, the sufferer should receive professional support from an early age to understand the condition. Controversy exists surrounding the "assignment" of gender based on the external genitals and whether this fully takes into account the hormonal, neural, psychological and behavioral factors that may all influence gender identity. The Intersex Society of North America says "newborns with intersex should be given a gender assignment as boy or girl, depending on which of those genders the child is more likely to feel as she or he grows up." Most experts suggest joining a supportive network of fellow sufferers so you can talk through your own experiences with people who've gone through the same.
How is Swyer Syndrome Treated?
Because a child born with Swyer syndrome looks like a typical female, people with Swyer syndrome are typically raised as females and have a female cultural identity.
That's why the most common treatment for Swyer syndrome involves hormone replacement therapy to start menstruation and aid the development of the female secondary sex characteristics like the breasts. Because the gonads are not developed, they do not produce estrogen or progesterone.
Typical hormone therapy includes estrogen and progesterone treatment. A 2011 study from the University of Athens, Greece showed hormonal treatment of young females with ovarian failure provided a beneficial hormonal profile and resulted in safe and adequate hormone levels. Instead of functioning gonads - ovaries or testes - people with Swyer syndrome have undeveloped clumps of tissue called streak gonads. Because these streak gonads are at risk of becoming cancerous, they are typically removed with surgery at an early age.
How to Manage Swyer Syndrome Hormone Therapy
In older women and women going through menopause, long-term estrogen replacement therapy is linked with an increased risk of heart disease, stroke and breast cancer. That's why many menopausal women no longer take hormone replacement therapy to deal with the symptoms of menopause.
Does this mean hormone therapy for Swyer syndrome is equally risky? Not completely. For young women with Swyer syndrome the benefits of hormone therapy generally outweigh the possible risks.
Talk to your doctor about any concerns you have with hormone therapy. Your doctor will tell you that the increased risk of uterine cancer from taking estrogen seems to be cut when also taking progesterone.
Change the dose of the therapy to minimize side effects like headaches and mood swings.
Quit smoking or don't start - your risk of blood clots, heart disease and stroke when you take estrogen is much higher if you also smoke. You can also take steps to improve your lifestyle and cut your risk of bone loss and heart problems.
Calcium and Vitamin D For Swyer Syndrome-related Low Bone Density
Estrogen is added to your body through hormone replacement therapy but even with hormone therapy you are still at risk of osteopenia or osteoporosis (reduced bone density) - a problem for women with low levels of estrogen.
Take calcium and Vitamin D supplements together - Vitamin D helps your body absorb calcium. The National Institutes of Health and National Osteoporosis Foundation recommend 600 international units (IU) of vitamin D a day for women aged 19 to 70. For women 71 and older, the recommendation increases to 800 IU a day. For women aged 19 to 50, they recommend 1,000mg of calcium a day, increasing to 1,200mg of calcium a day for women aged 51 and older.
Boost bone health with calcium-rich foods each day - non-fat milk, low-fat yogurt and calcium-enriched products like breakfast cereals, leafy green vegetables, salmon or tofu. One 8-ounce glass of skim milk contains around 300mg of calcium and an 8-ounce serving of plain low-fat yogurt contains around 452mg.
The best way to get adequate Vitamin D is through exposure to sunlight. However, the 2005 Dietary Guidelines for Americans suggests that older women, people with dark skin, and people who don't get enough sun should get extra Vitamin D from food or from Vitamin D supplements.
Eat Vitamin D-rich foods like eggs, salmon, mackerel, sardines and swordfish. According to a 1993 study from the University of California, San Diego zinc, copper and manganese may further enhance the effects of calcium and Vitamin D.
Lead An Active Lifestyle To Help Manage Swyer Syndrome
Deal with the effects of low estrogen levels by carrying out a program of weight-bearing exercises to help you strengthen and build bone. The National Osteoporosis Foundation says the best activities for strengthening and building bone are activities like walking, dancing, running or aerobics, which cause your body to work against gravity. You can also take on some muscle-strengthening exercises such as lifting weights.
How To Deal With Infertility And Swyer Syndrome
One of the most traumatic consequences of Swyer syndrome is infertility - women who suffer from Swyer syndrome don't produce eggs and cannot get pregnant from regular intercourse. Seek counseling to help deal with the emotional issues infertility can create and talk over the alternatives for having children.
A woman with Swyer syndrome can still become pregnant with donated eggs. In fact, a 2011 study from the University of Belgrade, Serbia reported the case of a successful pregnancy and delivery after IVF and a donor egg program in a woman with Swyer syndrome, a case which has been replicated elsewhere.