FEATURE ARTICLE

Recommendations for aComprehensive Management Planfor the Child Diagnosed WithEpidermolysis Bullosa

Kellie S. Badger, Judith O’Haver, Harper Price

ABSTRACT: Epidermolysis bullosa (EB) is a rare geneticdisorder that manifests by producing blisters on the skin.To date, this condition is incurable. The classification of EBhas been divided into four major forms with numeroussubtypes. The subtype of EB often predicts the prognosisof each individual. All children will require managementof their condition to prevent complications, which mayinclude infectionand scarring. Childrenwith this diagnosisare best managed by a multidisciplinary team designedto specifically meet the needs of a child with EB. How-ever, the lack of specialty clinics in many areas necessi-tates that parents advocate for their child(ren) and havea comprehensive treatment plan in place. Nurses canassist families in understanding the diagnosis and coordi-nating the needs of the affected child. The purposes ofthis article are to discuss the challenges of caring for a childwith EB and to review the components of a comprehensivecare plan to be instituted by the primary caregivers for thechild living with EB.Key words: Dystrophic Epidermolyis Bullosa ResearchAssociation (DebRA), Epidermolysis Bullosa, PediatricDermatologist, Skin Fragility

Epidermolysis bullosa (EB) is a rare (1 in50,000) genetic skin disorder that causes skinfragility and blistering resultant from a muta-tion in one of several genes responsible for skinadherence (Schober-Flores, 2009). All forms

of EB result in various extents of skin fragility and blis-tering because of friction, heat, or trauma (Tidman, Mellerio,& Pope, 2011; Figure 1). The four major types are simplex,junctional, dystrophic, and mixed or Kindler syndrome(Table 1). These types are classified according to level ofskin involvement. The mildest form, EB simplex (EBS) isgenerally nonscarring because the level of skin involve-ment is more superficial when compared with other moresevere types; however, scarring can result from infection.Blistering often presents at birth or shortly thereafter.Wounds in EBS may be chronic, but they tend to lessenin severity with age in many cases (Wright, 2011). In con-trast, those with the rare junctional EB type have a moresevere phenotype characterized by absent nails, dysplas-tic teeth, oral lesions as well as esophageal and trachealinvolvement (Wright, 2011). Dystrophic EB is among themost disfiguring of the EB types. As with the other formsof EB, this also presents at birth but distinguishes itselfby producing scarring and contractures. Dystrophic EBis further classified into two subtypes according to the in-heritance pattern, which may be autosomal dominant orrecessive. Generally, the dominant form is less severe,although there is evidence suggesting further variations,including a more severe dominant form and less severerecessive form (Tidman et al., 2011). Dominant dystro-phic EB is the more common subtype with patients devel-oping bullae over the limbs, trunk, and bony prominences(Tidman et al., 2011). Recessive dystrophic EB is char-acterized by widespread blistering and erosions involving

72 Journal of the Dermatology Nurses’ Association

Kellie S. Badger, BS, RN, Phoenix Children’s Hospital, Arizona.Judith O’Haver, PhD, RN, CPNP-PC, Phoenix Children’s Hospi-tal, Arizona.Harper Price, MD, FAAD, FAAP, Phoenix Children’s Hospital,Arizona.

The authors declare no conflicts of interest.

Correspondence concerning this article should be addressed toKellie S. Badger, BS, RN, Phoenix Children’s Hospital, 1919 E.Thomas Rd., Phoenix, AZ 85016.E-mail: kbadger@phoenixchildrens.com

DOI: 10.1097/JDN.0b013e31828866fa

Copyright © 2013 Dermatology Nurses' Association. Unauthorized reproduction of this article is prohibited.

both skin and mucous membranes. These erosions even-tually lead to extensive scarring and mitten-like deformi-ties of the hands and feet. Both types of dystrophic EBmayhave gastrointestinal (GI) involvement leading to esoph-ageal and anal strictures. The risk of developing aggressivesquamous cell carcinoma (SCC) is significantly increasedin those patients with the recessive subtype and is one ofthemajor causes of mortality (Tidman et al., 2011). MixedEB, also known as Kindler syndrome, is a rare form of EB.It is inherited in an autosomal recessive pattern and char-acterized by poikiloderma, photosensitivity, and skin fra-gility. Infants born with this type of EB often present withacral blistering. Although the tendency for skin blister-ing may improve with age, atrophic scarring and poikilo-derma tend to be progressive (Tidman et al., 2011).

Because of the extensive time and expertise required tocare for children with EB, it is necessary that caregiverscoordinate a plan with the child’s medical team. This planshould include but is not limited to ordering dressing sup-plies, scheduling clinic appointments, and making appro-priate referrals. Regular clinic appointments involve themultidisciplinary team including a dermatologist with ex-perience or expertise in EB (usually a pediatric dermatologist)as well as physicians and/or specialists in gastroenterology,nutrition, dentistry, hematology, hand or plastic surgery,orthopedics, ophthalmology, social services, pain manage-ment, and physical and occupational therapy. Families with

children diagnosed with EB should be directed to a casemanager or EB nurse so that they receive the appropriatemedical supplies and follow-up care from a dermatologist.

MAKING AND EXPLAINING THE DIAGNOSISDiscovering that their child is born with blisters or absentskin can be a frightening experience for parents and fam-ilies. According to Dystrophic EB Research Association(DebRA), EB is known as ‘‘the worst disease you neverheard of,’’ most nurses and pediatricians are unfamiliarwith this disease. When a child is born with blisters orabsent skin, he or she should be transferred to the neonatalintensive care unit for a complete evaluation. If EB issuspected, it is imperative that the neonatal team or pe-diatrician consults with the nearest EB center to properlydiagnose and care for the newborn. The diagnosis is oftenconfirmed with a biopsy of an induced and intact blistersent for immunofluorescence mapping as well as electronmicroscopy. This testing is limited to select specialty labo-ratories and/or universities. Clinical presentation may offera preliminary diagnosis, but tissue studies may be inconclu-sive requiring genetic testing to confirm the exact diagnosis.Genetic testing is also limited to specialized laboratoriesand, often times, is not covered by insurance because ofthe high cost of such tests. Although the newborn is stillhospitalized, teaching the family appropriate wound careis a crucial part in preparing the family for discharge. Theparent(s) must be able to show the appropriate process fordressing changes. Supplies should be ordered and deliv-ered to the home before discharge. Once the child is dis-charged home, it is necessary that the child is referred to adermatologist or a pediatrician who is well educated andexperienced in the diagnosis of EB. Although the treatmentplan is similar for most types of EB, knowing the exact sub-type of EB will help predict the child’s prognosis, furthercomplications, and screening tests that may be helpful. Con-firming the diagnosis of EB can create a lot of stress andanxiety for the family, especiallywhen the parents fully under-stand the disease process and the enormity of the care thatwillbe involved. Providing appropriate education and resources

FIGURE 1. Common wound seen in epidermolysis bullosapatients.

TABLE 1. Epidermolysis Bullosa Classification

Type of EB Inheritance PatternLevel of SkinCleavage Protein/Gene Involved

Simplex Autosomal dominant Intraepidermal Keratin 5, Keratin 14

Junctional Autosomal recessive DermalYepidermaljunction

Hemidesmosomal proteins, i.e.,laminin 5

Dystrophic Autosomal dominant/autosomalrecessive

Subbasal lamina Type VII collagen

Mixed (Kindler syndrome) Autosomal recessive Variable FERMT1

Abbreviation: EB = epidermolysis bullosa. Das & Sahoo, 2004; Tidman et al., 2011.

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can help reduce the level of stress and anxiety that theseparents and families experience.

INFECTION/WOUND CAREInfection is a recurrent concern and reality for patients withEB because of loss of skin integrity inherent in the diseasecaused by the presence of open wounds. Because of skinbarrier disruption, most individuals with EB become col-onized over time with bacteria. Infection may occur whenbacteria reach colony counts of 1,000,000 causing de-layed wound healing (Gardner & Frantz, 2004). Symp-toms of infection may include a dark red appearance,swelling, tenderness to touch, increased pain, surround-ing erythema, and increased drainage from the woundbed. The drainage may appear as yellow or green in colorand may have a strong malodor (Schober-Flores, 2009).However, even when the abnormal appearance and mal-odor are present, it does not always indicate active infec-tion (Gardner & Frantz, 2004). Malodor may be causedby specific types of dressings or even bacterial coloniza-tion. The most common bacteria found in the wounds ofpatients with EB are Staphylococcus aureus and Pseudo-monas aeruginosa, which contribute to delayed woundhealing (Lara-Corrales, Arbuckle, Zarinehbaf, & Pope,2010). Because management and prevention of infectionis a constant concern, especially in the recessive subtype,dilute bleach baths can be very beneficial in helping toprevent and treat bacterial overgrowth (Schober-Flores,2009). According to DebRA, the recommendation forbleach baths is 1/4 cup of household bleach in a half-fullbathtub of water or 1Y2 teaspoons per gallon of water.The bleachYwater solution should be mixed before com-ing in contact with the skin. Although there are no spe-cific guidelines, most EB clinicians recommend soakingfor approximately 10 minutes 2Y3 times per week or, ifactive infection is present, with each dressing change. Toprevent itching, it is important to rinse the skin with freshwater after soaking in bleach water. Pool salts anecdotallyhave been implicated to alleviate potential stinging anditching associated with the use of bleach in the bath water.Pool salts can be used in conjunction with bleach water tocreate an isotonic solution. To date, there have been no

studies reported that compared these methods for patientswith wounds of any type. Lindfors (2004) reported that,in her sample, patients who were treated with normalsaline alone showed an increase in aerobic bioburdenlevels as compared with the patients treated with an anti-microbial wound cleanser consisting of 0.057% sodiumhypochlorite in an isotonic saline solution. In addition,those patients treated with the antimicrobial solution ex-hibited a greater reduction in wound size when comparedwith the patients treated with saline alone. This findingsuggests thatwound healing is potentiatedwith the use of theantimicrobial wound cleanser. The paucity of studies in thisarea, however, limits the generalizability of this finding tothis population.

While caring for someone with EB and performing dress-ing changes, it is important to assess not only the woundbut also the surrounding tissue. The components of a nurs-ing assessment should include monitoring for characteris-tics of infection such as erythema,malodor, increased pain,or increased drainage and for improvement in the wound(Table 2). Close attention should be directed at the amountof moisture present in the wound bed. If the tissue appearswhite or ‘‘macerated,’’ there may be too much moisture be-cause of drainage, the amount of topical ointments appliedmay be too excessive, or inappropriate dressing materialshave been used. In contrast, if the wound and surroundingtissue appears pink and healthy, then the correct moisturelevel has been achieved. Choosing the correct dressing prod-uct is crucial in promoting wound healing (Schober-Flores,1999). Whereas some products are designed to providemoisture, others are designed to reduce it. It is importantto understand what each product is designed for beforeusing it on affected skin.

PAIN CONTROL/ITCHINGA study reported by Fine, Johnson, Weiner, and Suchindran(2004) assessed activities of daily living, mobility, and painin different subtypes of EB. Fine and colleagues explainedthat cutaneous pain is a common occurrence in the moresevere types of EB regardless of patient age. This reportalso identified that all major types of EB required at leastpartial dependency on caregivers especially in such activities

TABLE 2. Stages of Wound Healing

Phase Duration Appearance/Action

Inflammatory Occurs immediately after theinjury and lasts 3Y4 days

May appear red and warm to the touch

Proliferative Days 3Y21 postinjury Mild redness; new granulation tissue, blood vessels, and endothelialcells migrate across the wound bed to close the borders ofthe wound.

Remodeling Up to 2 years Formation of a scar

Schober-Flores, 2009.

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as walking and personal hygiene (Fine et al., 2004). Con-trolling the pain associated with chronic wounds anddaily dressing changes can be a very difficult challenge.Parents should be mindful of the pain involved with re-current blistering and infection. For some of the more se-vere forms of EB, analgesics or opiates may be required fordressing changes. Also, a nighttime dose of amitryptylinemay prove to be beneficial for some patients as this hasbeen indicated for chronic pain. Because patients with EBexperience pain daily, it is imperative that caregivers aswell as the medical team be aware of the most appropriateinterventions to help reduce the amount of pain these in-dividuals experience on a daily basis. It has been postu-lated that adequate pain interventions can be significantlybeneficial in patients’ coping strategies with this disease(Fine et al., 2004).

Regardless of the EB type, itching or pruritus is a dailychallenge for EB patients. Factors such as open wounds,healing wounds, bacterial colonization, or infection canall be cause for pruritus. Interventions such as a nighttimedose of doxepin may be helpful in treating pruritus.

MALNUTRITIONMost feeding issues arise from blistering and erosionswithin the GI tract. Large amounts of protein, blood, andfluids are lost from open wounds on the skin as well as inthe GI tract. In addition, the chronic and/or extensiveopen wounds result in an increased demand for proteinfor wound healing to occur. Patients with involvement ofthe digestive tract may develop dysphagia and constipa-tion, secondary to scarring and strictures of mucousmem-branes. Because of chronic wound development andhealing, inflammation, and GI involvement, suboptimalnutrition commonly results in the delay of growth and de-velopment. It is estimated that two to four times the normalcalories and protein are needed to heal wounds and preventiron deficiency anemia as well as allow for normal growthand development to occur (Schober-Flores, 1999). Supple-ments can be very helpful in maintaining the nutritionalneeds of children with EB. Examples of these supplementsmay include Pediasure, Ensure, andNutren. Iron supplemen-tation may also be required if severe anemia occurs. Giventhe diet restriction and pain associated with this diagnosis,consulting a nutritionist is helpful.

When an infant is born and diagnosed with EB, blistersin the mouth may make eating very painful; therefore, aHaberman Feeder can be helpful for infants who haveblisters or erosions in the mouth. The nipple contains adisk insert and valve that helps control the flow of liquidand also reduces the amount of air the child may swallow.

For older children with EB, eating can be particularlydifficult. Allowing the children to experiment with dif-ferent foods will allow them the opportunity to have somecontrol over their disease. Patients should be encouragedto eat soft or pureed foods and avoid extreme tempera-tures and spicy foods. Foods that are served at room tem-

perature and nonacidic are easier to swallow. Becausethe entire GI tract may be affected and the esophagus maybe eroded with blisters, esophageal strictures may result.As this can cause painful and difficult swallowing, dila-tations or surgical interventions may be required such asplacing a permanent gastrostomy tube to ensure propernutrition.

CONSTIPATIONThe need for iron supplementation and pain medication,alongwith a diet low in fiber and fluids, may contribute toconstipation in the patient diagnosed with EB. Constipa-tion may also be a result from anal ulcerations and thepain associated with passing stool near an open wound.Because of this, children may withhold from having abowel movement to avoid the pain. To help alleviate theproblems of constipation, a stool softener or increasedfiber intake may be beneficial (Schober-Flores, 1999). Aswith the use of all supplements or medications, the pa-tient should be followed closely by a gastroenterologistand a licensed nutritionist, as they are often part of themultidisciplinary team of the EB clinics.

SQUAMOUS CELL CARCINOMARecurrent, painful, or abnormal-appearing nonhealingwounds are reason to be concerned for Squamous CellCarcinoma (SCC). As dystrophic EB patients tend to havemore severe and nonhealingwounds, they are at greater riskof developing SCCs in comparison with other EB types(Pourreyron et al., 2007). SCCs tend to be aggressive inEB patients and may present with multiple foci or meta-static disease requiring the expertise of an experiencedsurgeon and oncologist for treatment. However, re-gardless of the type of EB, routine full-body skin exami-nations are highly recommended. According to Venugopaland Murrell (2010), full-body skin examinations shouldbe every 3Y6 months from ages 10Y16 years and every3 months from age 16 years and up and can be more fre-quent if warranted. Familymembers or primary caregiversare encouraged to pay close attention to wounds that havebeen present for at least 1 month with no improvement. Ifthere is concern for a nonhealing wound, this should bereported to the patient’s provider. The use of serial photo-graphs for wounds may be helpful to both the patient andthe dermatologist.

Managing a Home Care PlanChildrenwith EB have extensive needs, and to achieve thoseneeds, parents must work closely with the pediatrician,dermatologist, or if possible, the entire multidisciplinaryEB team. Patient education should extend to all of thechild’s caregivers to provide the best quality of life andoutcomes for the child, whereas goals of therapy shouldallow for as normal a life as possible. Although the treat-ment plan is similar among most types of EB, the indi-vidual child’s needs may vary. For example, a child with

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EBS may live independently as an adult, but a child withrecessive dystrophic will likely require dependence on acaregiver throughout his or her life. An article publishedin 2004 by Fine et al. confirmed that some level of super-vision is needed one or more times per day for most chil-dren with severe types of EB. Caring for a child with EBcan be extremely stressful, but with proper education, suf-ficient supplies, familial support, and a daily care plan, thechallenges of EB can be minimized.

With time, each family will begin to develop a plan thatworks best for their child(ren) as each child has uniqueneeds. For example, a dressing that works for one childmay not be the best choice for another child.Most parentsfind it helpful to talk with other EB parents as they haveexperience first hand, and because EB is so rare, parentsmay find it helpful to join local or national support groups.

Despite the long hours of wound care and frequentdoctor appointments, these children also need toexperience life as any other child without EB would.The psychosocial development of children is equally asimportant as physical development; therefore, play timeand limit setting should be consistent and appropriatefor the developmental level of the child.

Because most of these children attend school, the needfor an individualized education plan (IEP) is necessary tomaintain as normal an education experience as possible.An IEP allows the child to receive the proper educationwhile addressing the individual’s special needs. An IEPwould also allow the child to have a personal aid if nec-essary and/or accommodate more time between classesat school.

Dressing ChangesDressing changes are one of the main challenges facingthose with EB, particularly for the more severe forms ofEB. Being well prepared for dressing changes will makethe process much easier, faster, less painful, and lessstressful for both the parent and child. When orderingsupplies, it is best to plan ahead as it may take up to aweek to receive supplies. Dressing supplies need to beordered from a wound supply company often dependingon the patient’s insurance plan. Supplies are delivered on amonthly basis. Dressing changes can be exhausting forboth parent and child as daily wound care regimens maytake 2Y3 hours or longer for some individuals. Whetherthe dressing change is accomplished in the morning orevening, it is best to have a routine. Over time, this routinewill become easier and the parent(s) will become moreconfident with each dressing change. As EB children aresusceptible to infection, having a clean area to perform thedressing change should always be a priority. Initially, all ofthe supplies should be opened and prepared before re-moving soiled dressings. If the child is old enough to soakin a bathtub alone, then the parent can prepare the dress-ing materials during this time. This prevents the child’sskin from being exposed and unprotected for longer than

necessary. In addition, during the bathing routine, bleachbaths are useful in helping to prevent infection; however,this should be directed by a physician. Because the timeand energy involved in dressing changes is strenuous, al-lowing other family members or close friends to learn theprocess may help ease the burden of the primary caregiver.Table 3 lists useful supplies when performing dressingchanges. Dressing changes generally require three layersof protection. The contact layer consists of products thatare nonadhesive. If any product sticks to the skin, it willlikely tear the skin. The second layer is a gauzewrap, whichholds the contact layer in place. Lastly, the third layer suchas Surgilast or Xspan is used to keep the gauze secure andprovides added protection. Topical antibiotic use is re-commended in short intervals depending on the woundand is often suggested as an alternative to oral agents be-cause of the concern for resistance in pathogens to anti-biotic therapy. As with all antibiotic use, whether over thecounter or prescription, this should be directed by a phy-sician. Prescription agents generally have better coveragefor skin pathogens and have generally been tolerated wellbut require an order by a provider or physician. If an in-fection is suspected and has not improvedwithin 2Y3 daysof using a topical antibiotic, it is recommended that thepatient consult with his or her physician.

RESOURCESCaring for a child with EB carries an abundance of dailychallenges. DebRA has been helping children with EBfor over 30 years by providing various resources,emergency wound supplies, and a nurse educator whois available to answer questions. The diagnosis of EBinevitably carries a financial burden. An abundance ofdressing supplies is required for the severe forms of EBand is very costly. Insurance companies vary on woundsupply coverage. However, if a patient’s medical in-surance results in limited coverage, there are organiza-tions available to help with the cost of these necessaryitems. DebRA has developed programs such as the New

TABLE 3. Guide to Necessary DressingSupplies

Household bleach

Vaseline/Aquaphor

Wound dressing: contact layer

Padding if necessary

Gauze wrap: Conform/Kerlix

Stockings: Surgilast or Xspan

Sterile needles

Isopropyl alcohol (cleaning a blister before lancing)

Antibiotic ointments (prescription only)

Pain medicine if required

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Family Advocate Program, which provides products andinformation to families who have a newborn with EB.Also, the DebRA Wound Care Clearing House provideswound care and bandage supplies to families free ofcharge. In fact, up to $100,000 worth of supplies areprovided to families each year. DebRA also has a familycrisis fund, which helps families with unreimbursed medicalexpenses. Currently, the maximum amount allotted foreach family is $1,200 per year. As part of the dressingchange regimen, a lubricant such as Aquaphor may beused as an emollient to prevent dressings from adhering tohealing skin. Beiersdorf, the manufacturer of Aquaphor,provides a program that offers a free supply of Aquaphorto those with EB. Vaseline (petrolatum jelly) is also acommon emollient used with dressings. It is affordableand easy to access. Although there are many home healthsupply companies that can provide the wound care sup-plies needed for these children, National Rehab, HollisterWound Care, Edgepark, and Direct Medical are a fewcorporations who have a special interest in developingnew products for EB and helping families obtain neededsupplies. National Rehab supports DebRA’s mission andhas developed a program to support the EB community,including a Web site dedicated for EB families. This Website, www.ebnurse.org, allows families to access currentarticles, post questions to the EB nurse, learn tips fromother EB families, and become educated about the needs

of children and families with EB. Information about per-tinent clinical trials is also available on this site (Table 4).

EB families and researchers are both focused on findinga cure for EB. Stem cell transplant, gene transfer therapy,and improvements in wound care management are largelystudied topics in EB research. A general resource for allclinical trials is www.clinicaltrials.gov, which provides anup-to-date list of completed and active clinical trials forEB (Table 4).

To date, there are four major multidisciplinary EB clin-ics in the United States: Cincinnati Children’s Hospital,Children’s Hospital Colorado, Lucille Packard Children’sHospital, and Phoenix Children’s Hospital. These spe-cialty clinics are composed of multidisciplinary teamsincluding pediatric dermatologists, nutritionists, ophthal-mologists, physical and occupational therapists, hand/plastic surgeons, gastroenterologists, dermatology nurses,and social workers. Pediatric dentists trained in treatingchildren with EB may also be called to consult on relatedoral conditions and dental health in these children. Inaddition, having a general pediatrician as part of the multi-disciplinary team to helpmanage the care for these childrenbenefits the entire team, including the children. Becausethere are only four major specialty centers in the country, itmay be geographically difficult for some families to attenda clinic on a regular basis. It is imperative although, at somepoint, for children with EB to be evaluated and followed by

TABLE 4. EB Resources

Resource Contact Information

Dystrophic Epidermolysis Bullosa ResearchAssociation (DebRA)

DebRA of America, Inc. 16 East, 41st Street, 3rd Floor, New York, NY 10017.Tel: (212) 868-1573.E-mail: www.debra.org

http://www.debra.org/familystore This is a great link from amazon.com, which shows items that are safe forEB kids.

www.ebnurse.org, supported byNational Rehab

Phone: 1-800-451-6510

National Rehab National Rehab Equipment, Inc. Airside Business Park, 540 LindberghDrive, Moon Township,PA 15108. Phone: 412-507-0077 or 412-472-0680. Fax: 412-472-0311.

Aquaphor: Beiersdof, Inc. Customer service: 1-800-227-4703

Hollister Wound Care Customer service: 1-888-740-8999. E-mail: www.hollisterwoundcare.com

Stanford University http://dermatology.stanford.edu/research/research.html

University of Minnesota http://www.ahc.umn.edu/eb/

Camp Discovery www.campdiscovery.org; sponsored by the American Academy ofDermatology, www.aad.org

Camp Wonder 1600 S. Main Street, Suite #192,Walnut Creek, CA 94598. Phone: 925-947-3825.E-mail: http://csdf.org/contact-us.html

National Institute of Health http://www.nih.gov/

Abbreviation: EB = epidermolysis bullosa.

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an EB specialty clinic to ensure that the care requirementsare met and families are aware of available resources.

Because of the complexity of the more severe forms ofEB, these children spendmuch of their time doing dressingchanges or attending doctor appointments. These childrendo not always have the same opportunities as other chil-drenwithout EB. Patients with EBwill never feel ‘‘normal.’’Their skin does not look normal, and they often cannotparticipate in the same activities as unaffected children. For-tunately, various camps and programs have been createdfor those with genetic skin diseases such as EB. Thesespecialty camps allow children to be surrounded byothers who share the same feelings, worries, and dailystruggles of having a genetic skin disease. For example,Camp Discovery, supported by the American Academyof Dermatology, is a week-long camp for children withchronic skin conditions such as EB. At Camp Discovery,children have the opportunity to create new friendshipsand participate in activities such as fishing, swimming,boating, and arts and crafts. In addition, Camp Wonder,sponsored by the Children’s Skin Disease Foundation, of-fers children with serious and fatal skin conditions thechance to participate in activities with other children whohave similar skin conditions. The advantage to campers isthat their medical and social needs are met during the timeat camp and it is an ideal setting to address the feelings ofisolation because of the restrictions required by their skincondition.

NURSING IMPLICATIONSAccording to Schober-Flores (2003), the greatest chal-lenges in caring for a child with EB, regardless of type,include wound care, nutrition, education, and social sup-port. Because there are numerous complications associatedwith EB, parents must pay careful attention to all potentialcomplications, which may include infection, pain, malnu-trition, and development of SCC. They are strongly en-couraged to report any changes to the pediatrician ordermatologist. Not only are there physical complicationsbut social issues as well. The nurse can assist families inunderstanding the implications of the diagnosis as wellas developing a workable home plan to include both dress-ing changes and management of potential complications.Psychological support is very important for children withEB throughout their lifetime but especially when they reach

school age, and as EB affects the entire family, this supportis equally important. The nurse can also work in con-junction with other care providers and schools to ensurerespite for parents and to achieve maximum psychosocialdevelopment.

CONCLUSIONThe challenges associated with EB are physically, emo-tionally, and financially taxing. Through educating EB fam-ilies and providing the necessary resources, EB childrenand their families can be afforded as normal a life as pos-sible to reach their maximum potential. The chronicity andcomplexity of the needs of children with EB require a mul-tidisciplinary approach inwhich nurses can provide familieswith the support they require to be successful in the man-agement of their child’s care. h

REFERENCES

Das, B., & Sahoo, S. (2004). Dystrophic epidermolysis bullosa. Journal ofPerinatology, 24, 41Y47. doi: 10.1038/sj.jp.7211019.

Fine, J.-D., Johnson, L.B., Weiner, M., & Suchindran, C. (2004).Assessment of mobility, activities, and pain in different subtypes ofepidermolysis bullosa. Clinical and Experimental Dermatology, 29,122Y127.

Gardner, S., & Frantz, R. (2004). Wound bioburden. In S. Baranoski & E.Ayello (Eds.), Wound care essentials: Practice principles (pp. 91Y116).Philadelphia, PA: Lippincott Williams & Wilkins.

Lara-Corrales, I., Arbuckle, A., Zarinehbaf, S., & Pope, E. (2010).Principles of would care in patients with epidermolysis bullosa.Pediatric Dermatology, 27(3), 329Y337. doi: 10.1038/sj.jid.5700878.

Lindfors, J. (2004). A comparison of an antimicrobial wound cleanser tonormal saline in reduction of bioburden and its effect on woundhealing. Ostomy Wound Management, 50(8), 28Y41.

Pourreyron, C., Cox, G., Mao, X., Volz, A., Baksh, N., Wong, T., ISouth, A. P. (2007). Patients with recessive dystrophic epidermolysisbullosa develop squamous-cell carcinoma regardless of type VIIcollagen expression. Journal of Investigative Dermatology, 127,2438Y2444. doi: 10.1038/sj.jid.5700878.

Schober-Flores, C. (1999). Epidermolysis bullosa: Nursing perspective.Dermatology Nursing, 11(4), 243Y256S.

Schober-Flores, C. (2003). Epidermolysis bullosa: The challenges ofwound care. Dermatology Nursing, 15(2), 135Y144.

Schober-Flores, C. (2009). Epidermolysis bullosaVWound care pearls forthe noninfected and infected wound. Journal of Dermatology Nurses’Association, 1(1), 21Y29.

Tidman, M. J., Mellerio, J. E., & Pope, E. (2011). In L. Schachner & R.Hansen (Eds.), Pediatric dermatology (pp. 952Y965). New York, NY:Mosby/Elsevier.

Venugopal, S., & Murrell, D. (2010). Treatment of skin cancers in epider-molysis bullosa.Dermatology Clinic, 28, 283Y287. doi: 10.1016/j.det.2010.01.009.

Wright, T. (2011). Epidermolysis bullosa. Retrieved from http://www.uptodate.com/contents/the-genodermatoses

For more than 77 additional continuing education articles related to skin/wound care and 54 additional continuing education

articles related to pediatrics, go to NursingCenter.com\CE.

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