Why Deciding Whether to Have Children Is Difficult as Someone With Ehlers-Danlos Syndrome

Growing up in the South — North Carolina to be exact — in my experience, you are expected to follow a certain path. You graduate from college, find a good job, get married, buy a house, have kids (preferably by the time you turn 30), and live happily ever after. All in that order.

• What is Ehlers-Danlos Syndrome?
• What Are Common Ehlers-Danlos Syndrome Symptoms?

As a teenager, I hated that storyline, and would always tell people, “Yeah, I’m never having kids.” Probably not the best thing I could have said to the parents who hired me to babysit their children.

A big part of this had to do with how I felt physically. I always assumed most people felt bad – it was just part of being alive, right!? But deep down I knew the crushing fatigue, daily nausea, week-long migraines, terrible chest pain, and extreme dizziness were not “normal” experiences for the average teen or young adult. I ended up in the local emergency room on more than one occasion, thoroughly convinced I was dying. I searched far and wide for answers; I saw some of the best doctors Raleigh-Durham had to offer. And yet, whenever I’d go to a new specialist in search of answers, I’d always be given a clean bill of health. Talk about frustration and cognitive dissonance.

Fortunately, things changed when I moved to Winston Salem for a new job after college. A very astute primary care physician at Wake Forest University started connecting the dots and recommended that I undergo a tilt-table test, which requires you to lie strapped to a table for 45 minutes while a lift incrementally tilts you to an 80-degree angle.

For a typical person in this setting, heart rate is expected to increase by 10 beats per minute after 10 minutes. Mine, however, increased by approximately 50 beats per minute as the veins in my legs failed to properly constrict, resulting in excessive blood pooling in the lower half of my body. In an effort to keep my vital organs properly perfused with oxygen-rich blood, my heart had mounted an impressive tachycardic response. It was undoubtedly one of the most challenging medical tests I had ever experienced symptom-wise, and I would later learn that I use three times as much energy as most people every time I stand up.

I finally had my answer after all these years: postural orthostatic tachycardia syndrome (POTS) – a disorder of the autonomic nervous system. Since the autonomic nervous system regulates the body’s unconscious functions — respiration, blood pressure, heart rate, digestion, temperature, etc. — symptoms can be far-reaching, unpredictable and highly variable. For this reason, many of us simply get accused of having anxiety — I know I sure did. Strangely enough, POTS is actually pretty common; in fact, according to researchers, it occurs in up to one percent of the teenage population, though fortunately for most, it resolves within a couple of years.

I would later learn, with the help of a geneticist, that I am not most.

Truth be told, I’ve always been unusually bendy and flexible in my joints, but I never gave it a lot of thought — and neither did my orthopedic specialists or physical therapists growing up. I thought my “party tricks” were cool if nothing else, but unfortunately, they do come with a price.

You see, I also have Ehlers-Danlos syndrome – hypermobility type (hEDS) as the result of a genetic mutation that affects my collagen and connective tissues — found in the skin, muscles, tendons, ligaments, blood vessels, organs, gums, eyes, etc. — making them weaker than they should be. Needless to say, hEDS complicates matters, and is primarily responsible for my many pain issues and most likely my POTS. Since there is no cure for hEDS, my POTS prognosis remains uncertain. While I’ve learned how to minimize my symptoms through extensive lifestyle interventions — primarily aimed at increasing blood volume and reducing blood pooling — I’m aware that this is something I’ll likely deal with to some extent for the rest of my life.

Today, I’m a health-conscious, 31-year-old happily married woman. I take various supplements, exercise most days of the week, eat a healthy, nutrient-dense diet, meditate, and monitor my blood pressure religiously (it can be quite unpredictable and swing in either direction). I work remotely for a small tech company, travel as I’m able, and enjoy spending time with friends and family. I continually push myself, and while I’m fortunate to able to do many of the same things as my peers, I still have my share of bad (OK, downright scary) days as my symptoms are forever evolving. Some get better, while some get worse.

I look normal — whatever that’s supposed to mean — so inevitably, semi-judgmental questions often arise: “When are you going to have kids?”; “Wait, you don’t want to have kids?”; or my favorite, “You don’t want to be all alone when you’re old, do you?”

I’ll usually laugh, put on a brave face, and attempt a witty reply — or just change the subject altogether. Why should I have to defend a personal choice? And what is it to them anyway?

What I’d really like to say is, “Has your heart rate ever been 180 just sitting in a chair?” or “Have you ever dismissed the warning signs and symptoms of multiple kidney stones because they felt just like your typical, everyday pain?”

But I don’t.

The truth is, yes, I’d like to have kids. But it’s a tough subject for me, and one that I think about way more often than I’d like to admit. And God knows holidays and Instagram don’t help matters.

According to my geneticist, I have a 50 percent chance of passing down hEDS with each pregnancy. And since approximately 80 percent of people with hEDS also have some form of autonomic dysfunction (i.e. POTS), there’s a good probability I’d pass that down too. So far, researchers have been unable to locate the gene that causes hEDS, so there’s no way to screen an embryo ahead of time. It’s a literal coin flip.

Some days I struggle to take care of myself. Would I be able to take care of a mini-me? What about a similarly affected mini-me? Would I be able to look a child in the eye, knowing that I was (at least partially) responsible for their pain? Or would I regret not taking that chance? Would I forever wonder, “What if?” Will I be a lonely old person? And how would pregnancy even affect my body and conditions?

My doctors don’t have the answers to any of these questions. And I don’t either — but hopefully one day I will.

In the meantime, let’s celebrate the mothers among us this month, but please, let’s not question or belittle the ones who are not. You never know what someone is going through.

May is Ehlers-Danlos Awareness Month. To learn more, visit EDNF.