WHAT IS EHLERS-DANLOS SYNDROME (EDS)?
Ehlers-Danlos syndrome (pronounced ay-lerz dan-los sin-drome) is a group of hereditary disorders that mainly affect the skin and joints, but may also affect other organs. The disorder affects the connective tissues that support such parts of the body as the skin, muscles, tendons and ligaments. People with EDS disorders tend to have loose joints, skin that stretches easily, and a tendency to bruise.
HOW COMMON IS EHLERS-DANLOS SYNDROME?
Ehlers-Danlos syndrome is rare, affecting less than one in 10,000 people. It affects both men and women equally, and occurs among people of all racial and ethnic backgrounds.
WHAT ARE THE WARNING SIGNS OF EHLERS-DANLOS SYNDROME?
At present nine different types of EDS have been identified and the symptoms for each type can be different.
The main symptoms of EDS include increased skin elasticity, skin that feels "velvety" and that bruises or tears easily, and slow-healing wounds. Joints tend to be loose and unstable and may seem "double-jointed," allowing an overextension of the joint. Joint instability can lead to frequent sprains and dislocations.
WHAT CAUSES EHLERS-DANLOS SYNDROME?
Ehlers-Danlos syndrome is an inherited disorder, carried by the genes that are passed from parents to offspring. Each type of EDS is a distinct disorder that "runs true" in a family. This means that a person with one type of EDS will not have a child with a different type of EDS. Each type of EDS involves a different inherited defect of one of the many types of collagen fibrils (pronounced: coll-a-jen fie-brils) that make up the connective tissues in your body.
WHAT CAN YOU DO ABOUT EHLERS-DANLOS SYNDROME?
The two types of drugs most commonly prescribed for people with EDS are analgesics (pain relievers) and nonsteroidal anti-inflammatory drugs (NSAIDs – pronounced En-sedz).
For simple pain relief doctors often recommend acetaminophen (e.g. Tylenol®). Because acetaminophen (pronounced a-SEAT-a-min-o-fen) is not an anti-inflammatory it can usually be safely taken with most prescription medications. However, there are daily limits to the amount of acetaminophen that can be taken.
Ibuprofen (Motrin®, Advil®, etc.) and ASA (Aspirin®, Anacin®) are also recommended for pain relief and, like acetaminophen, can be bought without a prescription. In addition, these drugs are anti-inflammatory, which means they help to reduce swelling.
NSAIDs are prescribed to relieve pain and reduce swelling. Examples of NSAIDs that require a prescription include Naprosyn, Relafen, Indocid, Voltaren, Feldene, and Clinoril. All NSAIDs generally provide the same level of anti-inflammatory effect, however people can respond differently to different drugs and may experience greater relief from one medication over another.
The use of NSAIDs is associated with stomach problems such as heartburn, ulcers and internal bleeding. To avoid the stomach problems that can occur with NSAIDs, these drugs should never be taken on an empty stomach. If other side effects such as headaches or skin rash occur, inform your doctor who may prescribe a different drug.
A Word about Medication Safety
The need to effectively monitor new drugs once they have been approved and introduced into the market has been a key advocacy issue for The Arthritis Society for several years. This advocacy helps to ensure that unfavorable side effects are reported, documented, and addressed. For regular updates on medications available in Canada, visit www.arthritis.ca/tips/medications.
All medications have potential side effects whether they are taken by themselves or in combination with other herbal, over-the-counter and prescription medications. It is therefore important for patients to discuss the benefits and potential side effects of all their medications with their doctor.
You may find general information in Arthritis Medications: A Consumer's Guide [PDF] even if your disease is not specifically addressed.
Health Canada's Marketed Health Products Directorate (MHPD) has also developed the MedEffect website. MedEffect's goal is to provide centralized access to new safety information about health products in an easy to find, easy to remember location. It also aims to make it as simple and efficient as possible for health professionals and consumers to complete and submit adverse reaction reports. Finally, it helps to build awareness about the importance of submitting adverse reaction reports to identify and communicate potential risks associated with certain drugs or health products. visit MedEffect or call 1-866-234-2345(toll-free).
Protect your joints
Be kind to your body. After doing heavy work, or doing the same task over and over, stop. Slow down by doing an easy task, or by taking a rest.
Use your back, arms and legs in safe ways to avoid stress on joints. For example, carry a heavy load close to your body.
Use helpful tools in your daily tasks such as a cart to carry your grocery bags, or an enlarged handle that fits over a knife handle so you can hold it easily. A cane will help you to walk more safely. A grab bar, which attaches to a shower, will help you to get in and out of the tub more easily.
Protecting your joints means using your joints in ways that avoid causing excessive stress on them. Benefits include less pain and greater ease in doing tasks. Three ways you can protect your joints are by pacing, positioning and using assistive devices.
Pacing, by alternating heavy or repeated tasks with easier tasks or breaks, reduces the stress on your joints and allows weakened muscles to rest.
Positioning joints wisely helps you use them in ways that avoid extra stress. For example, use larger, stronger joints to carry loads (for example, use your arms and not your fingers to carry grocery bags) and change your position frequently.
Using assistive devices, such as canes, raised chairs, and gripping and reaching aids can help simplify daily tasks. For bathing, grab-bars and shower seats can be very helpful for conserving energy and avoiding falls.
People with EDS should have regular eye exams. Nearsightedness (myopia) is a common condition and can be corrected with prescription glasses or contact lenses. People with one particular form of EDS (EDS VI) are particularly at risk for serious eye conditions and should consult a doctor with experience treating EDS.
Vitamin C (ascorbic acid) may be effective in reducing the severity of symptoms for some people with EDS. Be sure to check with your doctor, however, before taking supplemental doses of vitamin C.
Unprotected exposure to the sun presents a heightened risk of skin damage for people with EDS. To avoid sun exposure, use a sunscreen with a sun protection factor (SPF) of 15 or higher and wear long sleeves, long pants and a wide brimmed hat during peak daytime hours.
Joint stability may be improved through prescribed exercise programs that strengthen the muscles. Weightlifting and any other exercise that puts strain on the joints must be avoided. Check with your doctor or physiotherapist to learn appropriate strengthening exercises and the proper way to do them.
Some people with EDS may need surgery to correct fractures and dislocated joints. If you are scheduled for any type of surgery, inform your surgeon that you have EDS as they will probably avoid using stitches to close the wound. Surgeons should also be alerted to any vascular or bleeding problems you may have.