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Skin
| Dermal wounds should be closed w/o tension, preferably in 2 layers. Deep stitches should be applied generously. Cutaneous stitches should be left in place twice as long as usual, & additional fixation of adjacent skin w/adhesive tape can help prevent stretching of the scar.
| Very young children w/pronounced skin fragility can wear protective pads or bandages over the forehead, knees, & shins to avoid skin tears. Older children who are active can wear soccer pads or ski stockings w/shin padding during activities.
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Joint instability
| Braces are useful to improve joint stability. Orthopedist, rheumatologist, or physical therapist referral for knee or ankle braces as needed Occupational therapist referral for ring splints (to stabilize interphalangeal joints) & wrist or wrist & thumb braces for small joint instability A soft neck collar, if tolerated, may help w/neck pain & headaches. Wheelchair or scooter as needed to decrease stress on lower-extremity joints Wheelchair customizations (e.g., lightweight, motorized, seat pads, specialized wheels, wheel grasps) as needed to accommodate pelvic & upper-extremity issues A waterbed, adjustable air mattress, or viscoelastic foam mattress (&/or pillow) may increase support for improved sleep quality & less pain.
| Those w/hypotonia, joint instability, & chronic pain may need to adapt lifestyle accordingly. Note: Surgical stabilization of joints may lead to disappointing, or only temporary, improvement.
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Joint pain – analgesics
| Acetaminophen: 4,000 mg daily in 3-4 divided doses NSAIDs, as tolerated by upper GI symptoms, for arthralgia, myalgia, & secondary inflammatory conditions (e.g., bursitis, tendinitis, costochondritis, postdislocation pain) COX-2 inhibitors have similar efficacy to NSAIDS but may be better tolerated. Topical lidocaine (cream or patch) may be useful for localized pain. Topical capsaicin is of questionable utility but is safe. Tramadol w/acetaminophen & NSAID or COX-2 inhibitor before resorting to other opioids (Nausea is the most common side effect.) Opioids for myofascial pain & neuropathic pain; should be reserved after failing the above medications. Administer w/other analgesics to minimize total opioid requirements. Typically used chronically (or at least several months), the primary formulation should be long acting (e.g., sustained-release oxycodone or morphine or topical fentanyl patch) w/short-acting forms of the same drug as needed for breakthrough pain. Routine use of ≥2 daily doses of a short-acting form should prompt an increase in the long-acting dose or another adjustment to the pain regimen.
| Bruising is not a contraindication to NSAID therapy, but occasionally requires dose reduction or change to a COX-2 inhibitor. Those w/muscle hypotonia & joint instability w/chronic pain may have to adjust lifestyle & professional choices accordingly. Emotional support & behavioral & psychological therapy may help in developing acceptance & coping skills. Long-term chronic pain may result in the need for mental health services.
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Pain – other pharmaceutical treatment options
| Serotonin/norepinephrine receptor inhibitors (SNRIs) (e.g., venlafaxine, desvenlafaxine, duloxetine, milnacipran) offer combined benefit for depression & neuropathic pain. | Venlafaxine may ↑ blood pressure a few points, which may be helpful for those w/neurally mediated hypotension. |
| Some anti-seizure medications (e.g., gabapentin, pregabalin, topiramate, lamotrigine) have been used in cEDS, are effective for neuropathic pain, & can be used in addition to tricyclic antidepressants &/or SNRIs. | All require gradual titration before reaching therapeutic levels. Gabapentin should be titrated as tolerated to at least 1,200 mg 3x per day before declaring failure, but dose is often limited by sedation &/or GI side effects. Pregabalin, titrated to at least 300 mg divided 2-3x/day, tends to be better tolerated than gabapentin.
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| Short courses of steroids can be very effective for controlling acute flares of pain assoc w/secondary inflammation. | Classic EDS is not an intrinsically inflammatory condition, & there is no role for chronic steroid use. |
| Muscle relaxants in combination w/analgesics to treat myofascial spasm & neuropathic pain |
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| Magnesium (topical as Epsom salt baths or oral) may ↓ muscle spasm & pain. | No specific formulation or dosage is established as superior. Adverse effects (sedation, nausea, abdominal pain, & diarrhea) are more common w/oral rather than topical supplementation.
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Tricyclic antidepressants are often effective for neuropathic pain, w/additional benefits of mild sedation (for those w/sleep disturbance) & a little mood elevation. Typical doses are nortriptyline (25-150 mg) or trazadone (50-300 mg) every evening.
| Constipation, a common side effect, can be managed w/fluids, fiber, stool softeners, & laxatives. For those w/diarrhea-predominant irritable bowel syndrome, the constipating effect may be therapeutic.
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| Glucosamine & chondroitin may help to prevent or treat osteoarthritis in the general population. | These have not been studied specifically in cEDS but are not contraindicated. |
| Cannabinoids such as dronabinol & marijuana (where legal) may be helpful for several different types of pain. | Benefits should be weighed against the potential for dependency &/or psychoactive effects. |
| Benzodiazepines may offer some short-term reduction in muscle spasm. | Routine use of benzodiazepines is not recommended, because of the high risk of tolerance, dependency, & addiction. |
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Neurologic
| Physiotherapeutic program for children w/hypotonia &/or delayed motor development Non-weight-bearing muscular exercise (e.g., swimming) to promote muscular development & coordination
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Hematologic
| Ascorbic acid (vitamin C) may ↓ easy bruising; general dose is 2 g per day for adults, w/proportionally reduced doses for children. | |
| DDAVP® (desmopressin) may be useful to normalize bleeding time. | DDAVP® may be beneficial w/bruising or epistaxis, or before procedures such as dental extractions. |
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Cardiovascular
| Standard treatment for cardiovascular manifestations | |
