What is Type IV Ehlers-Danlos?

What is Type IV Ehlers-Danlos?

Definition. Ehlers-Danlos syndrome type IV, also known as the vascular type of Ehlers-Danlos syndromes (EDS), is an inherited disorder of connective tissue characterised by severe arterial and digestive complications which are rarely, if at all, observed in the other forms EDS [1, 2].

Is vascular Ehlers-Danlos syndrome terminal?

Vascular Ehlers-Danlos syndrome can weaken your heart’s largest artery (aorta), as well as the arteries to other regions of your body. A rupture of any of these larger blood vessels can be fatal.

Is vascular Ehlers-Danlos life-threatening?

Vascular EDS (vEDS) is a rare type of EDS and is often considered to be the most serious. It affects the blood vessels and internal organs, which can cause them to split open and lead to life-threatening bleeding.

What is the life expectancy of someone with vascular EDS?

Among affected people diagnosed as the result of a complication, 25% have experienced a significant medical complication by age 20 and more than 80% by age 40. The median life expectancy for people affected by vascular EDS is 48 years.”

How is EDS type 4 diagnosed?

The clinical diagnosis of Ehlers–Danlos syndrome type IV rests on the finding of at least two of four diagnostic criteria (thin, translucent skin; arterial, intestinal, or uterine rupture; easy bruising; and a characteristic facial appearance), but laboratory studies are necessary for confirmation.

At what age is vascular EDS diagnosed?

The majority of children with VEDS who are diagnosed before 18 years of age are identified because of a positive family history. Approximately half of the children tested for VEDS in the absence of a positive family history present with a major complication at an average age of 11 years.

Does vascular Ehlers-Danlos qualify for disability?

If you have vascular or regular EDS with severe symptoms that prevent you from working, you may be able to get disability. Ehlers-Danlos syndrome (EDS) is a group of genetic disorders that affect the connective tissues in your body, which serve to provide strength and elasticity to your body structure.

What is the most common cause of death among individuals with vascular Ehlers-Danlos?

Death that occurred in the first two decades of life almost always resulted from spontaneous artery rupture or dissection. Artery rupture, 60% of which involved the aorta, was responsible for all deaths in young males. Death before age 20 years was seen in a 3:1 ratio of males:females.

Can you live a normal life with Ehlers-Danlos syndrome?

Complications in Life with EDS General: EDS affected persons can live like normal people; however, they may feel some constraints in their mobility. A person affected with vascular EDS is prone to serious fatal complications like tearing open of a main blood vessel or organ.

What famous person has EDS?

Australian singer/songwriter Sia (born Sia Kate Isobelle Furler), 43, is known for wearing elaborate wigs and headpieces that hide her face. However, the “Chandelier” singer is not hiding the fact that she was recently diagnosed with the connective tissue disorder Ehlers-Danlos syndrome.