Wound Care

The following list of suggested methods of care has been compiled from several sources and addresses all forms of EB. Because suggestion may not pertain to every form of EB, discretion and experimentation will guide the parents and the physician in choosing those methods that apply.

A wound is a break or opening in the skin or mucous membranes. Wounds are typically caused by trauma and/ or surgery. Acute and chronic wounds are very frequently in individuals with EB. The reason for this is the various proteins that are affected in the different forms of EB are the exact same proteins that hold the upper layer of skin (the epidermis) to the lower layer of skin (the dermis). Therefore, even minor trauma can result in a wound. Normal wound healing is a complex process that starts the moment an injury occurs and the initial healing process can continue for weeks. Superficial wounds can typically heal in a few days or weeks, but deeper wounds, like those that involve the dermis or even deeper, can take up to a year or more to completely heal. Normal wound healing processes through a very predictable trajectory. This trajectory involves an inflammatory, a proliferative and a remodeling stage The Inflammatory Stage : After injury, there is bleeding due to broken blood vessels and so the blood vessels narrow (vasoconstriction) to help slow down blood loss. The clotting cells in your blood are activated (platelet aggregation) and a clot is formed with the assistance from thromboplastin (a clotting factor). Specialized white blood cells called neutrophils, macrophages and monocytes come in to the wound bed and ingest cellular debris and microorganisms, to clean up the wound bed. This stage starts immediately after the injury and lasts from two to five days. The Proliferative Stage : Next, the framework for healing is created and the primary cells involved in this phase are the fibroblast and keratinocyte. Both of these cells release many different chemicals that allow for blood vessels to form. This allows a network of various proteins that act as scaffolding and the wound edges start to come together to close the wound (epithelialization). This stage will begin about two days after the injury and can last up to three weeks.

In EB, wounds are acute, due to minor trauma of activities of daily living so the above process is somewhat different. Often, areas that are sights of frequent injury will re-blister before reaching the end of the remodeling stage. Return to Top

5.2 Looking at the wound bed in non-healing wounds When wounds are healing slowly or appear not to be healing, the wound bed should be examined by the treating medical provider. The first step is to assess the ability to heal. Consider the following factors: Blood flow if it is not adequate, the wound wont heal Anemia - this condition of low red blood cells decreases the bodys ability to deliver necessary nutrients to the wound and so healing may be slow in a person with anemia. Many with EB need iron supplements and/or blood products to help correct their anemia Iron stores- too little stored iron reduces the production of red blood cells, which slows healing as stated above. In addition, many of the components of wound healing require iron in order to work properly. Therefore, individuals with low iron may require supplementation and/or iron infusions to correct this problem Infection - if an infection is present, the wound will stay open. Either topical or oral antibiotics may be needed depending on how deep the infection is located Steroids these interfere with the healing process In severe EB, medical providers must maintain a High Index of Suspicion for skin cancer with any chronic non-healing wound. Any suspicious wound should be biopsied to exclude the presence of a skin cancer Padding may be needed to protect an area and allow for healing. Consider this for wounds on extremities and over boney prominences (areas where bones are close to the skin surface). Nutritional needs must be addressed. It is important to work with a Registered Dietitian to make sure that the appropriate amount of nutrition is being consumed. Because EB results in frequent wounds, many calories are need to heal those wounds. It is important to know: How much protein is being consumed since protein is very important in wound healing?

What are the calories required based on weight and height so that infants and children with EB can grow and heal their wounds?

Are there mineral deficits that are delaying wound healing?

Scratching reinjures a wound. Treating an itch is very important. Use dressings that help the healing process. Non-adherent dressings are needed in EB. Keep in mind that different products may be needed at different stages of healing. Monitor the wounds for changes: Where are they located? Are the wounds getting bigger instead of smaller? Is there increase in drainage? Is there increase in pain at the site of the wound? Is the skin around the wound too wet or looks white? Is there increase in bleeding in the wound bed with dressing changes? Evaluate if the wound care plan is working. If the wound is not healing, the wound care plan may need to be changed. On occasion, more advanced care, like biological dressings, might be considered by your provider. Sibbald RG, Orsted HL, Coutts PM, Keast DH. Best practice recommendations for preparing the wound bed: update 2006. Adv Skin Wound Care. 2007 Jul; 20 (7): 390-405;. PMID: 17620740 Return to Top

5.3 Bathing with EB Cleanse skin daily: Skin may be cleaned with a very mild soap such as Dove. Initially, before a baby can sit up, sponge baths may be preferred. It may also be necessary to wash and rewrap one limb at a time to prevent the infant from rubbing one limb against another, causing skin damage. Older individuals can sit on a bath sponge in the bath tub. In some cases, it may be easier on the individual to remove dressing while soaking. Many with EB use additives in their bathwater. This includes salt, bleach and vinegar. It is important to have the mixed in the tub water prior to the individual entering the water. Salt Baths

In order to lessen the sting of bathwater, it is recommended that salt be added to the bathwater to create a pH neutral or isotonic solution. Standard Bathtubs: A regular bathtub holds 80 gallons of water when completely filled and holds 40 gallons of water when half filled. Add 2lbs of salt to 40 gallons of tub water, or a half filled bathtub. The amount of salt added should be adjusted to the amount of water in the tub For Babies and Infants: Salt is safe for use in the bathwater of babies and infants. Add 5 teaspoons of salt to each gallon of bath water used. Pool salt is inexpensive and can be purchased in pool stores and larger retail stores during the spring and summer months. Bulk purchase will reduce the cost. Rinsing with clear water after bathing is not necessary as it is not thought that salt water will increase itching, as happens after bleach and vinegar baths if not thoroughly rinsed with clean water. Table salt may be used

Special Thanks to Kim Hazelbaker, RN, Sue Rowe, RN, and Jean Whalen, RN of Cincinnati Childrens Hospital Medical Centers EB Center, and Sharon Cassidy, RN, EB Nurse Specialist of DebRA New Zealand, for their assistance in the preparation of this section Bleach and Vinegar Baths Why use Bleach or Vinegar? Colonization with bacteria can delay wound healing even if an infection is not present o o Bacterial burden in the wound Presence of 4 or more bacterial groups Trengrove et al. J Wound Care 1996;5(6):277-280

Infection is defined as Microbial load >105 CFUs per gram of tissue o o Bowler et al. Clin Microbiol Rev 2001;14(2):244-269 Gardner et al. Wound Repair Regen 2006;14(5):548-557 Can be Localized ( Impetigo, Cellulitis) or Systemic (Sepsis)

Most common organisms found in EB: Staphylococcus aureus: 23/30 pts had MSSA

 o o o o

2/30 pts had MRSA

Streptococcus species Diptheroids Pseudomonas aeruginosa Candida albicans

Ninety three (48.4%) wound cultures grew more than one type of bacteria Twenty six (13.5%) cultures grew 3 different bacteria 14 (7.3%) cultures grew 4 or more bacteria

Bleach or vinegar may be added to bathwater in order to lower the amount of bacteria on the skin of an EB patient. They may be alternated, but should never be used together in the same bathwater. Bleach (Sodium hypochlorite) must be diluted: Dilute cup bleach in full bathtub of water cup bleach in bathtub of water Mix 1-2 teaspoons bleach in 1 gallon of water General recommendations (no established clinical guidelines) o o Two - four times a week 5-15 minutes Rinse with water after dilute bleach-otherwise itching can worsen

Warnings: o o o o o o o o Generally not recommended under age 1 year DO NOT SWALLOW, DO NOT SPLASH IN EYES, DO NOT DRINK Keep out of reach of children Keep in labeled child safety proof bottle Dilute prior to contact with skin Do not store in beverage containers Do not mix with acid or ammonia Know your poison control number

White Vinegar (Acetic acid) Comes in both 3% and 5% solutions

Needs to be diluted further o o 5% acetic acid: 1 part vinegar to 20 parts bathwater 3% acetic acid: 1 part vinegar to 12 parts bathwater

Compresses o o o Soak a non-stick gauze in diluted vinegar preparation Apply to skin as a compress or soak 1 to 3 times a day Rinse with water after applying the dilute vinegar preparation

Useful against gram negative bacteria such as pseudomonas Useful against yeast Excellent bactericidal activity against: Pseudomonas aeruginosa Pseudomonas vulgaris Acinetobacter baumanni

Excerpt from Wound Antisepsis and Your Grocery List By Kimberly Morel, M.D. (Asst Professor of Clinical Dermatology & Clinical Pediatrics) . This information was presented at the 2010 Patient Care Conference. Return to Top

Interventions may include the following: Wrapping in between digits using strips of contact layers such as Mepitel (Molnlycke Health Care) and/or impregnated gauze such as Vaseline gauze (Kendall) may help delay the process of webbing. Whenever possible during the day, infants should be encouraged to discover and manipulate their hands to allow for better movement. Due to the possibility of rubbing and potential injury this should be done under supervision. During night time hands may be re-wrapped. Some people have found it helpful to use splints during the night to reduce chance of contractures. Splinting varies with age of person affected. Splints are made using a very heat labile moldable material. Consult with an Occupational therapist. They are very helpful in creating programs of activity that optimize hand function. The OT may be helpful in teaching methods of wrapping that allow for range of motion of fingers and hands. Ask the OT about range of motion exercises that you can perform on your infant/child

throughout the day. Specially designed equipment such as scissors and pencils with foam padding reduce discomfort while children participate in arts and crafts. If the function of the hand is impaired it is advisable to consult with either a Plastic Surgeon or a Hand Surgeon. The Surgeon will determine if it is in fact necessary to perform surgery or to wait. The surgical procedure involves separating the fused digits and releasing contractures while under anesthesia. Skin grafts or various bioengineered skin products may be used to cover wounds and/or donor sites. Hand splints are used to help keep fingers separated and hands in extended position post surgically. Careful instructions for post care should be given by the surgeon. Healing usually takes several months for this reason, only one hand is done at a time. The child with EB should be encouraged to be as active as possible, and physical therapy is often beneficial. Swimming is a good form of exercise for children with EB. If child has difficulty speaking and/or eating parents may consult with a speech therapist. Mouth and tongue excercises can help improve speech. (Early intervention programs provide occupational therapists, speech therapists and physical therapists for children under three years of age. For more information contact early intervention in your state.)

Finally there is help and support for families dealing with Epidermolysis bullosa. There are two main resources and numerous other smaller localized resources. First is the National EB Registry. It collects information from patients with EB for research to improve the understanding and provide better treatment of EB. Secondly, there is also The Dystophic Epidermolysis Bullosa Research Association (DebRA). DebRA is a nonprofit voluntary organization designed to provide information for patients, families, health professionals, and the general public. They promote and support research and can provide assistance to those living with any of the different forms of Epidermolysis bullosa. "One day without crying, one day with no pain

One day of not suffering those blisters again. One day with no dressings, or needles or creams One day you'll awaken with laughter not screams. One day of not fearing the slightest tumble And to watch you join in with your friends rough-and-tumble. One day of not feeling great surges of guilt For the genes that I gave you, imperfectly built. One day of not having to turn a blind eye To the ignorant stares of some passersby. One day, in the future, a potion they'll issue To toughen your skin that's as strong as wet tissue. One day a complexion of peaches and cream Will become a reality, not just a dream. One day without crying, one day with no pain, One day of not suffering those blisters again. . . . one day"

This poem was published in the D.E.B.R.A. U.K. Autumn 1996 Newsletter. An anonymous mother of a child with Epidermolysis bullosa wrote the poem.