Being Bendy

For a while I’ve been wanting to explain what Ehlers-Danlos Syndrome is as it is my main condition that I suffer from. I will tell you the other ones another time in a separate blog post as do not want to bore you! The information I’m about to tell you is generalised, as I luckily don’t have all of the following symptoms however I do have the majority of them though. You will see how greedy my body is!

Ehlers-Danlos Syndrome (E.D.S) is a varied group of heritable conditions of connective tissue, characterised by skin extensibility, joint hypermobility and tissue (nerves and muscle) fragility. There are various types of E.D.S which are classified according to symptoms and signs.

The syndrome is caused by a defect in a protein called collagen (you may have heard collagen being used in plastic surgery eg, for making the skin look younger etc). 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 throughtout the body.

E.D.S can affect both males and females. It is however, quite rare. To diagnose the syndrome, a skin biopsy (a removal of a sample of tissue) and this should confirm the diagnosis and also determine the type.

Life expectancy with an E.D.S sufferer can vary on the type they have. One type, the Vascular Type can be shorter due to the rupture (burst/tear) of large blood and the major organs. Pregnancy can be life-threatening in the Classical Type and the Vascular Type.

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 make the nose look broader. The sufferer may have little lumps which develop over pressure poinys such as the elbows and knees. Varicose veins is common amongst many types of E.D.S. than in the general population.

The joints are hypermobile (double-jointed) due to the ligaments being extra elastic (stretchy). This makes them more prone to subluxation (partly) and dislocation of which is a regular occurance for the sufferer, this can happen without any trauma (unlike someone without E.D.S). There is a lot of pain and discomfort for the sufferer too.

Bruising is easily caused and doesn’t necessarily need much trauma. It tends to take 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 an organ for example the diaphragm) are faily common. Curvature of the spine (Scoliosis) may be present at birth or later on in life. Even gum disease can be a symptom. Gastrointestinal diverticula (the stomach and intestines) where a pouch or sac in the lining of the membrane of a hollow organ such as the intestines etc, that is produced in the bowel when the bowel muscle ruptures the bowel wall.

The treatment depends on the presenting symptoms. If simple precautionary measures are made, it will greatly lessen the chance of any trauma, scarring and bruising. Surgery and skin suture (a piece of material 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 may help.

There are also psychological problems that may occur with a E.D.S sufferer.

I am thinking of possibly doing some fundraising for the Ehlers-Danlos Support Group as I personally don’t think there is much support for this condition. I haven’t had the opportunity of meeting any other sufferers in person as it is rare. Therefore have only spoken briefly through Twitter. All of my life, I have wanted to meet someone like myself, to share stories and find out more how they cope with having E.D.S.

Half of the time, you wouldn’t even realise that I may be in pain as I smile away and trying to cope silently. It is sometimes hard for people to understand that just because they don’t see any actual physical injury on the surface so they think “what’s wrong with ya now?!” Do you get what I mean. It makes me feel lonely and anxious at times so I try to focus on other things like my family and friends. Music helps relax me and takes my mind of the pain too. I hope you would like to fund raise for the charity too.

http://www.ehlers-danlos.org/index.php?option=com_contact&task=view&contact_id=1&Itemid=11

Please contact me if you want any more information, about anything to do with me or the charity details.