The Clinical Presentation of Ehlers-Danlos Syndrome

Elizabeth J. Lawrence, RNC, MSN, NNP


Adv Neonatal Care. 2005;5(6):301-314. 

In This Article

Nursing Care of Infants With Suspected EDS

Ehlers-Danlos syndrome is a chronic condition. Parents will need accurate information regarding their infant's care. Resources about EDS and information on support groups may be useful for parents, day care providers, family members, and later teachers and friends ( Table 4 ).[43]

A general understanding of EDS is important in caring for the infant and family.[2] An infant suspected of having EDS will require careful handling to prevent bruising during procedures. The joints, particularly the shoulders and hips, need to be supported and protected during repositioning and handling.[46]

Treatment in most types of EDS is limited to therapies that manage or minimize symptoms. A multidisciplinary team approach, with careful collaboration between nurses, social workers, physical therapists, orthopedists, surgeons, pain control specialists, cardiologists, and the genetics team, is required.[46]

Early intervention with physical therapy and a developmental clinic will ensure the best possible future for the infant. Hypermobility and dislocations of the small joints of the hand are common. Over time, this leads to problems with gripping and strength. Depending on the type of EDS, braces to stabilize joints and physical and occupational therapy to help strengthen muscles and preserve their mobility may be required.[2,25] Physical therapy can strengthen large muscle groups and help prevent recurrent shoulder dislocation.[2,35] Affected individuals should avoid activities that hyperextend or lock a joint.[46] Children may intentionally put their body in strange or unusual positions to entertain others.[2] These types of maneuvers put them at risk of joint degeneration, and affected children need to be educated from an early age to preserve their body and joint function.

The infant that is starting to crawl or the unstable toddler needs to wear protective gear (headgear, elbow pads, knee pads, and shin pads) to prevent damage to their fragile skin.[6,10] Simple safety measures, such as padding the edges of coffee tables and sharp corners, will help safeguard fragile skin. Prevention of scarring and disfigurement is important.[3] Daily vitamin C supplements may improve wound healing and bruising.[5]

If surgical intervention is required, wound closure and healing can be a challenge. Dermal wounds should be closed without tension and deep stitches should be applied generously.[10] Cutaneous stitches should be left in place twice as long as usual; in addition, the adjacent skin may be taped to help support the approximation of the incision and prevent stretching of the scar.[10]

Children with EDS may need to avoid physically stressful activities and contact sports to prevent joint strain and damage.[2] Swimming, a non–weight-bearing activity, will not stress the joints yet helps with muscle development and coordination in infants and children. It can be initiated in infancy by the parents.[10,25] Stationary bicycling, golfing, or walking are other recommended forms of exercise for older children and adults.[35]

Cardiac problems are associated with both classical- and hypermobile-type EDS. Individuals with mitral valve prolapse and/or regurgitation require antibiotic prophylaxis to prevent bacterial endocarditis before dental or surgical procedures.[10] An unusually high prevalence of aortic dilation (28%) in individuals affected with both these types of EDS has been reported.[19] An increased risk of aortic dissection is associated with this significant dilatation.[25] Baseline echocardiograms should be initiated at age 5 to measure aortic diameter,[10,35] and follow-up should occur every 3 to 5 years depending on severity.[25]

Affected young adults may be otherwise healthy and become frustrated and experience self-doubt that limits their self-actualization.[48] Emotional support and behavioral and psychological therapy may be necessary to help the individual cope and accept this condition.[14]

Chronic pain is a serious complication of classical and hypermobility-type EDS. It can be both physically and mentally disabling; may present caregiving challanges for affected mothers.[25,47] This pain is described as an aching, throbbing pain similar to the pain associated with fibromyalgia; however, it is distinctly different from the pain associated with joint dislocations.[25] The neuropathic etiology of the pain explains the burning, shooting, tingling, electrical-type pain often described.[25] Migraine headaches, caused by cervical muscle tension and temporomandibular dysfunction, are also common.[25] Osteoarthritis or degenerative joint disease presents earlier in those with EDS and compounds the chronic pain.[25] Treatment involves the use of analgesics and nonsteroidal anti-inflammatory drugs.[6,25] Topical anesthetics such as lidocaine may be useful for localized pain as well.[25]


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