Ehlers danlos syndrome, a disease that makes joints too flexible

The human body consists of various types of tissue, one of which is connective tissue. As the name suggests, this tissue functions to bind, support, and hold the skin, tendons, ligaments, internal organs, and bones. Well, this important network can actually have a disorder known as Ehlers Danlos syndrome (EDS). Intrigued by this disease? Come on, find out the explanation in the following review.

Ehlers Danlos syndrome is a disorder of the connective tissue in the body

Ehlers Danlos syndrome is a rare disease that affects connective tissue in the body. Especially on the skin, joints, and blood vessel walls. This tissue is made up of a mixture of cells, fibers, proteins known as collagen, and other substances that provide strength and elasticity to structures in the body. Disruption of connective tissue due to genetic disorders causes tissue function to be not optimal.

People with this syndrome usually have joints that are too flexible and skin that is easily brittle. When the body was injured and needed stitches, the skin was often torn because it was not strong enough to hold it in place.

In many cases, EDS syndrome can run in families. However, this disease can also occur without being inherited. This means that there is a defect in the gene that forms collagen so that the connective tissue that is formed becomes imperfect. Reporting from the Health Line, according to the National Library of Medicine's Genetics Home Reference, EDS syndrome is a fairly rare disease, affecting 1 in 5,000 people worldwide.

What are the symptoms of EDS syndrome?

EDS syndrome has various types and symptoms, depending on which part of the connective tissue is affected. Here are the most common types of EDS syndrome and the symptoms that include them.

1. EDS hypermobility

Hypermobility EDS (hEDS) is an EDS that affects and affects the joints. The symptoms of Ehlers hypermobility and Losses syndrome are:

• Sprains easily because the joints are loose and unstable
• The body is very flexible beyond normal limits
• Often feels pain and pressure in the joints
• Extreme body fatigue
• Skin bruises easily
• Have digestive disorders such as acid reflux or constipation
• Dizziness and increased heart rate when standing
• Bladder control is problematic; always want to go to the bathroom

2. Classic EDS

Classic EDS (cEDS) is an EDS that affects the skin and causes symptoms, such as:

• The body is very flexible beyond normal limits
• Sprains easily because the joints are loose and unstable
• Stretchy skin
• The skin is easily brittle, especially on the forehead, knees, elbows, and shins
• Skin feels soft and bruises easily
• The wound takes longer to heal and leaves quite extensive scars
• Hernia

3. Vascular EDS

Vascular EDS (vEDS) is the rarest type of EDS and is considered the most serious. Because this condition affects blood vessels and internal organs which at any time can cause bleeding and be life-threatening. The symptoms of Vascular Ehlers danlos syndrome are:

• Skin bruises easily
• Thin skin and small blood vessels are visible, especially on the upper chest and legs
• Brittle blood vessels that can swell and tear, resulting in serious internal bleeding
• Organ problems, such as tearing of the intestine or uterus or dropping of an organ from its original position
• Difficult to heal from wounds
• Fingers are very flexible, nose and lips are thin, eyes are large, and earlobes are smaller
  

4. Kyphoscoliotic EDS

EDS kyphoscoliotics are very influential on the bones, the symptoms include:

• Curved spine, starting in early childhood and often worsening in adolescence
• The body is very flexible beyond normal limits
• Sprains easily because the joints are loose and unstable
• Childhood weakness (hypotonia) which causes delayed ability to sit, walk, or difficulty walking
• The skin is stretchy, feels soft, and bruises easily

How to treat EDS syndrome?

To get a proper diagnosis of this disease, patients must undergo a series of medical tests, such as genetic tests, skin biopsy (checking for abnormalities in collagen), echocardiogram (knowing the condition of the heart and blood vessels), blood tests, and DNA tests.

To treat EDS syndrome, patients must follow the treatment according to the doctor's recommendations. Current treatments for EDS syndrome include:

• Physical therapy to keep joints and muscles stable
• Surgical procedures to repair damaged joints
• Take medication to relieve pain

Meanwhile, to protect the body from the risk of injury, patients must avoid strenuous activities. Such as avoiding contact sports (physical contact with opponents, for example soccer) or lifting heavy objects. Then, take care of the skin by using sunscreen and choosing a mild soap for the skin.