Jameela Jamil, star of The Good Place, recently posted a video to her Instagram page of herself stretching her cheeks and captioned it, “I’m fine.” It may not have been clear to everyone exactly what she meant, but when one commenter asked if she had Ehlers-Danlos syndrome (EDS), Jamil replied, “Indeed.”
This seems to be the first time Jamil has confirmed she has EDS, a rare connective tissue disorder. But she has previously highlighted her symptoms. For instance, back in October 2018, she replied on Twitter to someone asking "what the fork" was going on in a photo of her elbow hyperextending. She responded with, "My arm is being extremely sexy!"
Here's what you need to know about this complex condition.
EDS is not a single condition, but rather a group of disorders.
There are 13 EDS subtypes, each of which is a distinct disorder. And they all have one thing in common: abnormalities of the connective tissue (the fibrous tissue that makes up tendons and ligaments), Calvin J. Brown, M.D., a rheumatology professor at Northwestern University's Feinberg School of Medicine, tells SELF.
These conditions are thought to be caused by genetic mutations that affect the body's production of collagen, a protein essential for maintaining the strength and structure of connective tissues in the body, according to the National Institutes of Health. Sometimes those genetic mutations are inherited, but in other cases there is no family history of the condition. And we still don't fully understand what causes these underlying genetic issues.
The "classic" presentation of EDS, the Mayo Clinic explains, usually includes overly flexible joints, stretchy skin, and fragile skin that doesn't heal or scar over well. The severity of those symptoms may vary from person to person. “Symptoms are generally present and first noticed in young age, as people with EDS are born with it,” Dr. Brown says.
Most people with EDS notice that they have loose joints first, Howard Smith, M.D., director of the Lupus Clinic within the Department of Rheumatic and Immunologic Diseases at Cleveland Clinic, tells SELF. “Because of this, those with EDS frequently have joint sprains and dislocations, as well as frequent joint aches, pains and arthritis,” Dr. Smith says.
The other symptoms of EDS can cause issues as well. For instance, “If the skin is also more stretchable than normal, this may lead to easy bruising, scarring and poor wound healing," Dr. Smith explains. "[And] tissues, such as arteries and organs of the body (e.g. the heart and intestine), are not as strong as they should be and they may rupture or not function normally.”
Some EDS patients experience abdominal pain and gut abnormalities, Sarmad Almansour, M.D., an internal medicine and rheumatology specialist at Detroit Medical Center's Sinai-Grace Hospital, tells SELF.
Another severe form of the disorder, vascular Ehlers-Danlos Syndrome, can weaken the aorta and other arteries in the body, possibly causing a dangerous rupture. It can also weaken the walls of the large intestine or uterus, the Mayo Clinic says.
It can sometimes be challenging to accurately diagnose EDS.
First off, there's no objective test, such as a blood test or X-ray, that confirms a patient has the condition. EDS can also be confused with other conditions. “There are more than 200 other similar connective tissue disorders,” Dr. Smith says.
But, because the disorder has a strong genetic component, having the characteristic symptoms and a family history of EDS is often enough to make a diagnosis, the Mayo Clinic says.
Patients might also be referred for genetic testing to try to pinpoint which EDS subtype is present and rule out other issues. That said, researchers have only identified the exact genetic mutation for a few of the subtypes, so it isn't always helpful, Dr. Brown notes. Additionally, only a few centers are able to carry out those tests at present.
Right now, there is no cure for EDS.
“All treatment is directed at managing the symptoms and living with the condition,” Dr. Brown says. In general, patients may be given medication to manage pain and control blood pressure abnormalities associated with EDS, the Mayo Clinic says.
But unfortunately, many EDS patients experience ongoing, widespread pain, Dr. Brown says. "The scientific basis for this is currently unknown and is a subject of some research," he says. "And traditional pain medications do not work well, so other strategies to manage pain are called for."
Improving muscle strength and tone can also help to stabilize and protect the joints. So, Dr. Brown also recommends physical therapy, guided exercise, yoga, and Pilates. However, many patients may need to avoid strenuous activities, such as contact sports, as their loose joints and soft skin are more susceptible to injury. Some patients may also benefit from cognitive behavioral therapy (CBT) to help manage pain as well as stress reduction and support groups.
In some cases, surgery may be required to repair damage in the case of repeated joint dislocations. Surgery might also be recommended if a patient is experiencing a "weakening in the wall of the aorta," Dr. Almansour explains, because this increases their risk for aortic aneurysm.
Above all, it's crucial to have a knowledgeable team around you to help you make decisions in regards to managing EDS and find a treatment plan that makes sense for you.