Article - PHARMACOLOGICAL CONSIDERATIONS in EHLERS-DANLOS SYNDROME

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PHARMACOLOGICAL CONSIDERATIONS in EHLERS-DANLOS SYNDROME

By

SRCurry LPT/RNc

Bayside, California

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.

1. Moderate to severe pain of diverse distribution is a common every day

occurrence, starting early in life and worsening over time.

2. Pain with EDS is complexly individualized.

3. EDS is a very painful and debilitating syndrome.

4. 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.

5. Chronic dislocation and subluxations can be very painful.

6. 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.

7. 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.

NSAIDS NAPRSYN HEPARIN TORADOL

IBUPROFEN ANAPROX SEPTRA -DS

CORTICOSTEROIDS ASPIRIN BACTRIM- DS

PREDNISONE MOTRIN FIORINAL PERCODAN

MIDOL .these are just a few, read your packet inserts, talk to your doctor

& 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 as

well, 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.

IN SUMMARY

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.

Beyond her LPT/RNc her studies include Pharmacology, Psychopharmacology &

Substance Abuse Pharmacoloy. Her work as a Hospice Nurse Casemanager

required knowledge & expertise in Pain Management for the Terminally &

Chronically ill.