What Is Ehlers-Danlos Syndrome?? EDS!
Hi peeps!
I have just realised that I haven't yet explained what EDS is so now is the time for me to do so. The information I'm about to tell you is generalised, as to cover as much as I can.
Ehlers-Danlos Syndrome (EDS) is a varied group of heritable conditions of connective tissue, characterised by skin extensibility, joint Hypermobility and tissue fragility (nerves and muscles). There are various types of EDS which awe classified according to signs and symptoms.
The syndrome is caused by a defect in a protein called collagen (you may have heard of collagen re; plastic surgery). It is the main part of the body providing strength and support; such as ligaments, tendons and cartilage. When the collagen is defective, it can induce problems throughout the body.
EDS can affect both males and females. It is however, quite rare. To diagnose the syndrome, a skin biopsy (removal of a sample of tissue) as well as history and other factors, will help to confirm the diagnosis. Blood tests may be done for certain types too which can also help to determine the type.
Life expectancy with an EDS sufferer can vary on the type that they may have. One type; the vascular type can be shorter due to the rupture (burst/tear) of large blood vessels and the major organs. Pregnancy can be life-threatening particularly in the classical and vascular types.
Symptoms can also vary depending on the type. The skin can be easily torn and it is very stretchy. Folds of the skin at the inner of the eyes which makes nose look broader. The sufferer may have little lumps which develop over pressure points such as the elbows and knees. Varicose veins is common amongst many types of EDS than in the general population.
The joints are hypermobile (loose jointed) due to the ligaments being extra elastic (stretchy). This makes them more prone to subluxations (partly) and dislocations (fully) of which is a regular occurrence for the sufferer, this can happen without any trauma (unlike someone without EDS). There is a lot of pain and discomfort for the sufferer too.
Bruising is easily done and doesn't necessarily need much trauma. It takes a lot longer to heal.
Less common features such as arterial, uterine and intestinal ruptures may occur due to the frailty of tissues. Inguinal (in the groin area) and hiatus (an opening in the organ for example; the diaphragm etc) are fairly common. Curvature of the spine (scoliosis and kyphosis) may be present at birth or later on in life. Even gum disease can be a symptom. Gastrointestinal diverticula (the stomach and intestines) share a pouch or sac in the lining of the membrane of a hollow organ, that is produced in the bowel when the bowel muscle ruptures the wall.
The treatment depends on the presenting symptoms. If simple precautionary measures are made, it will gently lessen the chances of any trauma, scarring and bruising. Surgery and skin suture (a piece of material that is used to close a wound or to connect tissues) should be taken with immense care as fragile tissues may tear. Sutures ought to be left in longer than normal. The sufferer may be advised to wear supportive aids such as braces to support unstable joints. In some cases, orthopaedic surgery may be necessary but is unfortunately not always successful. Physiotherapy and occupational therapy will help.
There are also psychological problems that may occur with an EDS sufferer. The person may need extra support for these issues.
What I have just told you, is the simplest way of explaining the syndrome as it is often very complicated and complex. I am listing my main conditions; kyphosis and scoliosis, osteoporosis, perniosis, osteoarthritis, severe panic disorder, severe anxiety and arthralgia. These are all in order of being diagnosed with each condition. There are many many other related conditions such as POTS and CCI.
I hope that I have answered some questions about EDS that you may have been thinking about but if you have any more then please don't hesitate in asking me. Today has been not too bad for pain which I can smile about. It's been lovely seeing my sister and her baby, my niece Ciara. Anyway that's enough from me, be sure to follow my Facebook page DinkyktsEDSDiaries and I shall see you all soon xxx
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