in ,

Ehlers-Danlos Syndrome and Top Surgery

People with Ehlers-Danlos syndrome generally have very flexible joints and stretchy and delicate skin

Ehlers-Danlos syndrome  (EDS) is a disorder that is inherited. They impact a person’s connective tissues — namely, the skin, joints, and blood vessel walls.

People with Ehlers-Danlos syndrome generally have very flexible joints and stretchy and delicate skin. This feature can become an issue if you have a wound that needs stitches. As a result, the skin won’t be strong enough to hold the stitches in place. 

How Common Are Ehlers-Danlos Syndromes?

The number of Ehlers-Danlos syndromes is approximately between 1 in 3,500 to 1 in 5,000 people. The most common is the hypermobile variant (hEDS), and it may be more prevalent as it can be missed in being detected or misdiagnosed as a different syndrome. 

Many variations of these syndromes are rare (1 in 40,000 to 200,000 and some are even more rare, occurring in less than 1 in a million people. However, people of any race and ethnic background can be impacted by EDS, which can start with complications from birth and progress as time passes. 

Is There a More Severe Form of This Syndrome?

There is a more severe form of this disorder. It is known as vascular Ehlers-Danlos syndrome, and it causes the walls of a person’s blood vessels, intestines, or uterus to burst. Due to this type of syndrome, there can be serious complications, especially for pregnant people. So, in essence, talking to a genetic counselor can be an excellent idea if you’re pregnant or thinking about it.

Symptoms of Ehlers-Danlos Syndromes

There are many variations of Ehlers-Danlos syndrome, but the most commonly occurring signs and symptoms are as follows:

  • Very flexible joints. Since the connective tissue holding the joints together is loose, these joints can move past the point of a normal range of motion. As a result, a person can expect joint pain and dislocations often.
  • Skin that stretches. When connective tissue is weak, the skin stretches more often. You could even pull a pinch of skin away from your flesh, but as you let go, it will go right back into place. In addition, your skin may feel extra soft or velvety.
  • Delicate or fragile skin. Damaged skin has difficulty healing. For instance, if a person with EDS gets stitches to close a wound often, it can tear open and leave a gaping scar that is thin and crumpled in appearance. 

The degree of severity for symptoms is unique to each person and what type of EDS they have.

Causes of EDS

Various types of Ehlers-Danlos syndrome are genetic, meaning that they are inherited and passed down from a parent to their child. If you have the most common type, hypermobile Ehlers-Danlos syndrome, then there is a 50% likelihood that you’ll pass it on to your children. 

What Are the Types of Ehlers-Danlos Syndrome?

There are 13 types of Ehlers-Danlos syndromes (EDS):

  • Hypermobile EDS – when a person experiences joint hypermobility that impacts both large and small joints, and it can lead to recurring joint and partial joint dislocations.
  • Classical EDS – characterized by elastic and stretchy smooth skin that is delicate and bruises easily. It also features comprehensive, atrophic scars and joint hypermobility.
  • Vascular EDS – its features include thin, translucent skin that is fragile and bruises. Organs like the intestines and uterus can also be weak and burst.
  • Kyphoscoliosis EDS – extreme hypotonia that develops at birth, and delayed motor development, with progressing scoliosis since birth. People with this type of EDS may also have bruises that happen easily, fragile arteries that can erupt, unusually small corneas, and low bone density.
  • Arthrochalasia EDS – extreme joint hypermobility and congenital dislocation of the hips. Many people with this type of EDS include delicate, elastic skin where they bruise easily; hypotonia; kyphoscoliosis, and mild osteopenia.
  • Dermatosparaxis EDS – very fragile skin that causes bruising and scarring, hernias, as well as saggy skin on the face.
  • Brittle Cornea Syndrome (BCS) – thin corneas, early-onset keratoglobus that is progressive; and blue sclerae. 
  • Classical-like EDS (clEDS) – features skin hyperextensibility with a velvety skin texture and lack of atrophic scarring, generalized joint hypermobility, with or without recurring dislocations, skin that bruises easily, or discolorations of the skin that creates bleeding underneath. 
  • Spondylodysplastic EDS (spEDS) – a person with this type of EDS has a short stature that’s progressive since childhood, muscle hypotonia, and bowing of limbs. 
  • Musculocontractural EDS (mcEDS) – this type of EPS has multiple congenital contractures, adduction-flexion contractures, and/or clubfoot. Also characterized by craniofacial features that are apparent at birth or in early infancy, skin hyperextensibility, easy bruising, skin fragility with atrophic scars, and increased wrinkling in the palms. 
  • Myopathic EDS (mEDS) – congenital muscle hypotonia and/or muscle atrophy that gets better as a person ages. It also features proximal joint contractures and hypermobility of distal joints.
  • Periodontal EDS (pEDS) – Severe and intractable periodontitis that is in early-onset during childhood or adolescence. It also is characterized by a lack of attached gingiva and pretibial plaques.
  • Cardiac-valvular EDS (cvEDS) – Severe progressive cardiac-valvular problems, skin problems, and joint hypermobility. 

Is the Prognosis Good for Someone With Ehlers-Danlos Syndromes?

There is no cure for any Ehlers-Danlos syndromes; however, there are treatment options for the symptoms and preventative steps. 

A person’s prognosis is dependent on the kind of Ehlers-Danlos syndrome. If a person experiences organ and vessel rupture that comes with vascular EDS or other rare forms, this can lead to early death. Also, severe breathing restriction because of scoliosis could increase the possibility of lung disease and minimize life expectancy. In many cases, gastrointestinal failure could cause other organ failure and even death. However, most people with more common types of EDS have an average life expectancy.

For some people, their concerns can get worse as time progresses. For instance, joint pain and instability can lead to a more inferior quality of life. Also, these symptoms can occur because a person didn’t receive the right advice or undergo proper treatment.

When folks get the proper treatment, their symptoms can improve significantly and cause better daily functioning and a higher quality of life. It can also keep a person in a state where they are not getting worse and have the tools to better manage their issues.

Ehlers-Danlos Syndrome and Top Surgery 

If you are transgender, non-binary, or gender non-conforming and have an EDS, and are looking to get top surgery, the Gender Confirmation Center discusses how you can safely go about the procedure. Check out the video below for more information. 

What do you think?

1.7k Points
Upvote Downvote
Billie Olsen

MODEL: Billie Olsen

Billie Olsen (she/they) is a lifestyle writer, disability justice advocate, and cozy femme located in Kelowna, BC, Canada. Their works have appeared in Metro News, Discorder, Sophomore Magazine, the Post-Feminist Post, DINE Magazine, and NerdReader.