EHLERS-DANLOS SYNDROME A BRIEF OVERVIEW
EDS is a genetic defect in the collagen molecule itself. An inherited connective tissue disorder characterized by joint hypermobility, dislocation/subluxations, bleeding, bruising, dermal hyperelasticity, and widespread tissue fragility, skin tearing & poor wound healing. Unlike Lupus, EDS is not an antigen/antibody problem. EDS is gene mutations effecting the structure or assembly of different collagen’s. The cross-linking of the collagen fibrils is thought to be defective. EDS is a very painful & debilitating syndrome. THE SYMPTOMS BELOW OCCUR IN THE NORMAL POPULATION and are not exclusive to EDS, however due to the increased medication induced bleeding tendencies in the normal population, individuals with EDS may have a higher incidence of pharmacological considerations, especially in Vascular Type EDS.
PAIN & EDS
Nov. 1997, Vol. XII, Number 4 of “Loose Connections”, the official communication link of the EDNF reprinted by permission of Elsevier Science, Inc. Journal of Pain & Symptom Management,Vol.14,No.2,pp.88-93 Copyright 1997 by the Cancer Pain Relief Committee”. “Chronic Pain is a Manifestation of the Ehlers-Danlos Syndrome”.
Pain and EDS is a simple summary of the seven most striking points about Ehlers-Danlos Syndrome pain.
- Moderate to severe pain of diverse distribution is a common every day occurrence, starting early in life and worsening over time.
- Pain with EDS is complexly individualized.
- EDS is a very painful and debilitating syndrome.
- Most EDS patients, but not all, have (at some point) taken some type of medication. Joint pain and instability are the primary cause for use of pain medications.
- Chronic dislocation and subluxations can be very painful.
- Pain Insomnia has been widely reported by most EDS sufferers, (46)of the (51) individuals interviewed indicated they had chronic pain over the last 6 months or longer.
- Areas of pain reported =A total of thirteen different `principal pain locations’ were identified. The elbow, shoulders(1 or both), hands, knees, spine, frequent headaches, stomach aches & Continuous pain in extremities, ankles, feet, toes & hips. The Pain was described as aching, sharp, throbbing or burning & significant enough to experience dysfunction in sleep, physical activity & sexual activity.
LONG TERM USE & LONG ACTING MEDICATION
Pharmacologically, pain can be treated with several different types or combinations of medication, analgesics, opiates, anti-inflammatory drugs and/or antidepressant therapy. It is important to take all medication as directed and on time. Pain is much easier to manage (with less medication)at the first sign of discomfort than it is to treat or manage ‘out of control’ pain.
Chronic illness sometimes forces the medical profession to be creative in medication management. Difficulty swallowing, Allergies and trauma to soft tissue from injections and/or needle pricks, as well as remembering to take the medication on time makes long-acting medication ideal for the EDS patient. Low dose titration of pain medication allows the body a chance to ‘adjust’ to the introduction of medication. It allows for some autonomy and the lowered incidence in nausea and other side effects like drowsiness, plus, the effective relief of pain decreases patient fears, promoting understanding of the medications, leading to better medication compliance and more comprehensive pain control.
There are many different opiates, analgesics and pain medication that working together with your doctor you should be able to find pain relief that fits your lifestyle.
DISPELLING MYTHS ABOUT ADDICTION & CHRONIC ILLNESS
MOST pain medications and other drug classifications can create TOLERANCE requiring an adjustment (increase) dosage. This IS NOT ADDICTION. Addiction is misuse or abuse of a drug, usually to obtain a `high’. When your pain becomes intolerable & the doctor increases your medication, this DOES NOT make you a ‘drug addict’. In fact, most people in pain do not reach a
`high’, just pain control. If your EDS were to `magically’ disappear you would be tapered off the medication & be drug free again. Some of the same holds true to other medications like Steroids & some Antidepressants. Pain can cause nausea, anxiety, agitation, depression, feelings of isolation, hopelessness & helplessness. Good pain control can give you quality of life and increase functioning. Pain Control Clinics and knowledgeable physicians can help you obtain the best medication regime for your pain level and lifestyle. You do not have to suffer in pain.
NO MEDICATION SHOULD BE STOPPED ABRUPTLY WITHOUT FIRST CONSULTING YOUR DOCTOR
DRUGS THAT INCREASE BLEEDING (Most commonly, but not limited to Vascular EDS)
These drugs increase the risk of prolonged bleeding & other side effects in the normal population and are not exclusive to EDS. Many drugs have aspirin in them, this may increase your risk of bleeding or bruising. You can ask if the same drug comes mixed with Tylenol instead, for example; Percocet instead of Percodan.
MIDOL …these are just a few, read your packet inserts, talk to your doctor or pharmacist about potential drug interactions or bad combinations. BE INFORMED!!!
TYLENOL taken in even moderate doses over a long period of time can cause liver damage.
ANTIBIOTICS can irritate a pre-existing ulcer. Use with caution especially in someone with EDS with preexisting Gastrointestinal problems.
IV’s-Should NEVER be FLUSHED with HEPARIN…NORMAL SALINE works just aswell, without, the added side-effects & potential to increase clotting time.
Xylocaine can be given to numb the site before attempting to `find a vein’. Some EDS patients DO NOT respond to Local Anesthesia. NEEDLE GAUGE: REGULAR IV’s can be as small as 25.
Blood transfusions or blood products can be given through a 22 Needle Gauge.! You have to speak up before they stick you or it’s too late and you have probably just received ‘normal protocol’ & unnecessary PAIN!
Whole units of blood can be put through a warmer , unless contraindicated, ask because they probably won’t think of it.
ALLERGIES- A COPY OF ALL ALLERGIES TO MEDICATION & FOODS or adverse reactions to certain drugs SHOULD BE WITH YOU! This should be part of your
MEDIC ALERT ID– This helps the medical profession help you! List your diagnosis and all allergies, medications and doctors.
Pharmacological considerations in people with Ehlers-Danlos Syndrome include ,but are not limited to, several types of drugs that can increase bleeding in the normal population and those EDS sufferers with a pre-existing tendency to bleed must be acutely aware of their current drug regime. It is important, with any chronic illness, to carry copies of, as well as information about any, ALLERGIES (food too), medications or medication interactions. It is important to tell your doctor ALL the medications you take, even over-the-counter drugs, Aspirin/Tylenol/Advil. Always ask if your ‘new’ medication contains Aspirin or is compatible with anything else you may be taking. It is OK to ask for the smallest gauge needle to avoid soft tissue injury. Chronic pain is a clinical manifestation of Ehlers-Danlos Syndrome. The pain is complexly individualized, diverse in its location & intensity. EDS pain commonly requires intervention by trained professionals, Pain Control Clinics &/or doctors who are educated and/or willing to learn about this complex syndrome. Pain should be reported immediately. Scale your pain so the doctor can understand how much pain you are in. PAIN-SCALE=(1-5) or (1-10) the highest number being INTOLERABLE. You do not have to live in pain. Pain Clinics, qualified knowledgeable physicians & pharmacists can help you find the best medication regime that fits your lifestyle. Remember your pharmacist is a knowledgeable resource.