Ehlers-Danos syndrome is a group of rare genetic conditions that affects connective tissues, particularly joints and skin, and sometimes blood vessels. Hugues Joublin explains that these tissues are made of a complex mix of proteins, and provide support to internal organs and bones.
Hugues Joublin Explains the Types of Ehlers-Danlos Disorders
There are 13 different conditions that fall under Ehlers-Danlos syndrome. Hypermobile EDS is the most common type, with vascular EDS following.
Hypermobile EDS is where the joints are affected, making them overly flexible. It is often also accompanied with stretchy and fragile skin. This often makes it difficult to recover from deeper wounds since the skin is weak and fragile.
Vascular EDS is another type of Ehlers-Danlos syndrome where the blood vessels are much weaker. This is dangerous since it means that they can rupture easily and lose a lot of blood, which can even cause death. According to Hugues Joublin, vascular EDS can also weaken the walls of certain organs and cause ruptures there as well.
Classical EDS is the type that affects the skin more than the joints, though it has very similar symptoms to hypermobile EDS. Kyphoscoliotic EDS is a rarer form of EDS and can cause progressive curvature in the spine, alongside hypermobility and weak skin. According to Hugues Joublin, it can also cause hypotonia which can cause delays in sitting and walking, and may even leave a patient unable to do either one if the symptoms get worse.
Causes of Ehlers-Danlos Syndrome by Hugues Joublin
Since EDS is a group of genetic disorders, they are caused by problems within the genes. According to Hugues Joublin, genetic disorders can either be there from birth due to complications in genetic makeup, but they can also be developed over time.
With EDS, since there are different types of conditions that fall under the category, each type of EDS will have different genetic causes associated with them, and some of these are inherited from parents. In the case of hypermobile EDS, which is the most common type, there is a 50% chance that each of your children will carry the gene.
Complications Caused by EDS
Since different types of EDS will affect different parts of your body, the complications that arise are also different. For example, hypermobile EDS will give flexible joints that can cause dislocations and even arthritis at an early age.
The fragile skin that comes with EDS can cause scarring. On the other hand, vascular EDS can put you at risk of fatal ruptures at any point in time, and can make it very dangerous to carry a child since the uterus wall becomes very weak and can rupture as well.
Preventing EDS is difficult since it is a genetic disorder. What you can do is be vigilant about the process if you are planning to start a family. Talking to a genetic counselor can let you know how inheritance of EDS works and what you can do about it.