Brennen's VEDS Walk will be held at:
1002 Clark Ferguson Dr, North Kansas City, MO 64116
Shelter #4
Saturday, October 5th, 2024
10am - 3pm
What is VEDS?
Vascular Ehlers-Danlos Syndrome
Vascular Ehlers-Danlos Syndrome involves type III collagen and type I collagen. Changes in the COL3A1 gene can cause it. In addition, it can also be caused by changes in the COL1A1 gene. The mode of inheritance is autosomal dominant.
The characteristic symptom of this type is very tender skin. The skin is thin, transparent, and prone to damage. The fat under this layer is lesser. Vital blood vessels and organs can easily rupture, and the hair tends to be thin and weak. Those with this syndrome often have distinctive facial characteristics, such as large eyes, delicate noses, lips, and ears without flaps. They may also have a small chin and deep-set cheeks.
Increased mobility often impacts their small joints, especially the fingers and toes. A condition called clubfeet may also happen at birth, where the foot and front part of the foot are in a permanently bent position. Other issues that may arise include tendon breaks, muscle tears, skin aging sooner than expected, and varicose veins at an early age. They may also suffer from additional conditions such as pneumothorax and gum recession.
Life with VEDS
People with VEDS are born with it, but features of the condition are not always present right away. Some people have signs of VEDS at birth or as young children – including conditions like congenital clubfoot, hip dislocation, limb deformity and visible veins. Bruising may not be an issue when a baby is an infant, but it may become apparent as the child becomes mobile. When a child is seen because of bruising, a potential VEDS diagnosis is not on the radar of the healthcare professionals doing the examination, especially if there is no family history of the condition. Some children (or babies) have fewer features when they are young, and then develop features during puberty.
The risks of life-threatening events can increase with age. This makes it very important for people with VEDS to receive accurate, early diagnosis and monitoring. Otherwise, the risk for potentially life-threatening complications is greater. The earlier the diagnosis is made, the easier it is to make life-style changes and establish a care plan that can lower the risk of life-threatening complications. 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.
Major complications in childhood are very rare and death prior to the age of 10 is less common. In late childhood, some of the facial features of VEDS become more apparent, bruising may increase because of activity, spontaneous pneumothorax (lung collapse) may occur, and colonic ruptures sometimes occur, usually in the sigmoid colon. By age 20, about a quarter of those with a known VEDS diagnosis have had a significant complication, such as bowel rupture, arterial rupture, or spontaneous pneumothorax. Some of those with VEDS may develop a prematurely aged appearance, especially on the limbs with acrogeria or old appearing hands and feet. Even with these outward features, the diagnosis is not commonly considered because VEDS is thought to be a rare condition and most clinicians have only heard about it.
What are the signs?
What are the signs of Vascular Ehlers-Danlos syndrome, or VEDS?
Some people have signs of Vascular Ehlers-Danlos syndrome, or VEDS, with a noticeable characteristic appearance, while others do not have any outward signs of the condition.
Some signs of VEDS are easy to see
Every person’s experience with Vascular Ehlers-Danlos syndrome is slightly different. Some may have every feature, some may have only a few features, and other people have different combinations of features. Some people may not have outward signs at all. Some signs of VEDS are easier to see than others. These include:
- Thin, translucent skin with increased vein visibility
- Characteristic facial appearance (thin lips, small chin, thin nose, large or deep-set eyes)
- Premature aged appearance of the hands and feet (acrogeria)
- Hypermobility of small joints (“double-jointedness”)
- Gum recession and fragility
- Born with a hip dislocation or clubfoot
- Tendon and muscle rupture
- Keratoconus (cornea of eye bulges outward causing thinning)
- Early onset varicose veins
- Spontaneous or easy bruising that is not explained by other causes
- Formation of a carotid-cavernous sinus fistula (sudden engorgement and redness of the eye)
- Sleeping with the eyes open or partially open
Other signs are harder to detect
Complications of Vascular Ehlers-Danlos syndrome that may not be apparent from outward appearance include aneurysms, artery dissections, and arterial tears (including the aorta), as well as sudden lung collapse, bowel perforations, and uterine rupture during pregnancy. Special tests are often needed to detect these complications, and these complications may present as emergencies.
VEDS can be inherited.
Walk will be held at: Macken Park 1002 Clark Ferguson Dr Shelter #4, North Kansas City, MO 64116
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