Epidermolysis bullosa (EB) is a genetic blistering skin disorder. It is characterized by extreme skin fragility resulting in erosions or blisters when heat, trauma, or friction is applied to the skin surface (Figures 1 and 2). This skin fragility is related to a missing gene that is responsible for skin adherence. The type of EB and the severity of the skin disorder is determined by the lacking gene (Schober-Flores, 1999).
There are many different types of EB with different levels of severity. The more severe types of EB involve multiple-organ systems and the skin. Therefore, EB is considered a multiorgan system disorder (Schober-Flores, 1999). It not only affects the skin, but it can also result in blistering, erosions, and scarring of the mucosal surface of internal organs systems such as the gastrointestinal tract, respiratory tract, and genitourinary tract mucosa (Figure 3). Thus, the severity of the skin disorder can range from a mild seasonal blistering, which hardly interferes with a person's lifestyle, to a severely debilitating and even life-threatening skin disorder, which completely consumes an individual's life (Schober-Flores, 1999).
Regardless of the type of EB one is diagnosed with, whether it is a mild or a severe form, it is essential that all types receive good wound care. The goal of wound care is to choose a dressing product that will promote the wound-healing process, decrease the pain of the wound, and limit the trauma to the skin (Schober-Flores, 2003). Therefore, dressings are utilized not only for wound healing but also for wound protection (Figure 4). Remember, EB is characterized by extreme skin fragility, and if you protect the skin of an individual who is diagnosed with EB, they can live a more normal life.
The wound-healing process in individuals with EB can be complex. However, the first step is to understand how a wound heals. Wound healing involves three phases of healing. These phases are the inflammatory, the proliferative, and the remodeling phase (Cuzzell, 1995).
The first phase, the inflammatory phase, is initiated immediately after injury and lasts 3-4 days. Platelets, white bloods cells, and growth factors are active during this phase of healing (Jones, Bale, & Harding, 2004). This phase is characterized by redness and warmth that surrounds the wound. The redness and warmth are normal and are basically our body's way of bringing in cells to stop the bleeding of the wound, cleanse the wound, and stimulate the growth factors.
The second phase of healing, the proliferative phase, is characterized by minimal to no redness surrounding the wound. It begins after Day 3 and lasts up to Day 21. This phase is rich in growth factors. These growth factors are stimulated by the fibroblasts, macrophages, and the endothelial cells. The wound-healing process is regulated by these growth factors, and each has its own designated role (Jones et al., 2004). During this phase, the growth factors are stimulating the wound to fill it in with new granulation tissues, new blood vessels are being formed, and new endothelial cells are forming along the borders of the wound. These endothelial cells close the wound by migrating from the border of the wound inward until the wound edges are secured together.
The last phase of healing is the remodeling phase. This phase is characterized by the formation of a scar. During this phase, the resulting scar remodels, and this remodeling can last up to 2 years. During this time, the scar will lighten, soften, and blend with uninjured skin. However, this scar will never be as strong as uninjured skin. It will only be 20% as strong within 3 weeks of injury and 70%-80% as strong at the end of 2 years (Jones et al., 2004). This is important to note because wounds that have been previously injured are prone to reinjury because of this lack of strength.
ACUTE VERSUS CHRONIC WOUNDS
There are two types of wounds that are involved in the wound-healing process: an acute wound and a chronic wound (Jones et al., 2004). An acute wound is a wound that can progress through the three phases of healing in a timely and orderly fashion. A chronic wound does not progress through these phases. Chronic wounds become stalled in a phase of healing and do not progress beyond that phase. Individuals who have EB have chronic wounds. They have chronic wounds because they have a missing or defective gene, which results in the continual reinjury of their skin (Schober-Flores, 1999). Their wounds remain in the inflammatory phase of healing and often have difficulty progressing beyond that phase. Therefore, the goal for choosing a dressing product for individuals diagnosed with EB is to find a product that will not only lessen the duration of the inflammatory phase but also protect fragile tissue from breakdown during the proliferative and remodeling phases of healing (Cuzzell, 1995).
Persons diagnosed with EB tend to have high bacterial counts on their skin surface; however, this does not mean that they have an infected wound. These high bacterial counts are related to the chronic open erosions and blisters. Wound cleansing is especially important in these individuals because it not only decreases the bacteria of the skin, but it can also prevent an infection from developing. For wounds that are not considered infected, the recommended wound cleanser is saline, Dove unscented soap, baby shampoo, Cetaphil cleanser, or a surfactant, such as Sur-Clens. Another way to cleanse the wound is with Ultrasonic mist. Ultrasonic mist is a noncontact, low-intensity, low-frequency ultrasonic treatment (Extended Care Professional News, 2007). This treatment not only cleanses the wound, but it also decreases the bacterial count of the wound, stimulates the growth factors, and decreases the pain of a wound. It is especially useful on a chronic wound that has stalled in healing.
Pain control can be a problem for persons diagnosed with EB, especially during bath time. Individuals with EB have extensive skin erosions, and when water comes into contact with their wounds, it is very painful. To decrease this pain, 1 lb of pool salt can be added to the bath water. This significantly reduces pain because it makes water isotonic. This as a result will make their bath time something they look forward to rather than something they fear. This is important because, if you do not cleanse their skin appropriately, those bacteria will interfere with wound healing, and if the colony counts get high enough, an infection will result (Gardner & Frantz, 2004).
For a noninfected wound, moist wound healing is encouraged. Noninfected wounds tend to be wounds with minimal to no drainage. Wounds that are kept in a moist environment heal faster and scar better (Schober-Flores, 2003). This moist environment allows the epithelial cells an easier way to migrate across the wound (Baranoski & Ayello, 2004b). You can provide a moist environment by applying a topical ointment to a dressing product or to the wound bed itself (Schober-Flores, 2003; Figure 5). For a dry climate, a petrolatum-based product such as Vaseline or Aquaphor is recommended. For a humid climate, a hydrogel would be a better option. Moist environments promote wound healing, but too much moisture can hinder healing. Too much moisture will cause maceration, resulting in skin breakdown and ulcer extension. If the tissue surrounding the wound is white and soft (maceration), this indicates that the environment is too moist, and a different dressing option is recommended.
DRESSING OPTIONS FOR THE NONINFECTED WOUND
Remember that individuals with EB are missing a gene the keeps their skin held together. Therefore, you never want to apply a dressing product that might potentially adhere to the wound or to any loose tissue surrounding the wound. If a dressing product does adhere, you can very easily remove or "deglove" an individual's skin, which will result in a very traumatic and painful dressing change. This means that you want to choose products that will promote the wound-healing process but not products that have the potential to dry out and adhere to the skin if a dressing change is missed.
The dressings of choice are the products made by Molnlycke. These dressings are all silicone based. The silicone in these products prevents the dressing from adhering to the wound bed or from the dressings drying out. The purposes are to promote the wound-healing process, limit the trauma to the skin, and protect the skin from injury. Some of the Molnlycke products recommended for the noninfected wound are Mepitel, Mepilex lite, Mepilex, and Mepilex border (Molnlycke Healthcare, 2006; Figure 6). These products can actually remain in place for a maximum of 1 week for a noninfected wound. However, because of the high bacterial counts of the skin in individuals with EB, it is recommended that you remove the bandages and cleanse their skin at least 2-3 times per week.
Another dressing option would be the restore products made from Hollister Wound Care. The restore products are petrolatum based and have hydrocolloid particles within them. These particles form a gel when wound drainage comes into contact with them and therefore create their own moist environment (Hollister Wound Care, 2008). However, for a dry wound in a dry climate, it is still recommended to utilize a thin layer of a topical ointment with these products to ensure that they will not dry out and adhere to the wound bed. The recommended restore products for the noninfected wound are the Restore foam and Restore contact layer.
Collagen products are another great option to promote wound healing (Hess, 2005). These products help boost the wound-healing process by providing structural support to the wound, attracting cells to the wound site, and enhancing the growth of granulation tissue (Baranoski & Ayello, 2004a). Another benefit is that they are comfortable and decrease the pain of the wound. These products can dissolve and often become incorporated into the wound. An example of this product is Puracol plus made by Medline Pharmaceuticals.
It is important to secure these products, but because of the skin fragility of these individuals, you cannot apply tape to their skin. Tape has strong adhesives that will remove their skin when the tape is removed. Paper tape is also not recommended. To secure dressings in place, it is recommended to use a gauze roll such as Elastomull or Conform and then burn netting to keep everything held together. However, occasionally, when patients are hospitalized, it is required to secure tubes or an intravenous line. If a tape is to be utilized, the tape that is recommended is Mepitac tape (Molnlycke Healthcare, 2006). This is a silicone tape that will secure the tubes but will not remove the skin once it is removed.
ADVANCED WOUND CARE PRODUCTS
Advanced wound care products, such as Dermagraft, can be utilized when other treatment modalities have failed. They are indicated for wounds that have been present for 6 weeks or longer and for those which have been unresponsive to other wound treatment modalities (Advanced BioHealing, 2007). The goal with advanced wound care products is to restimulate the wound-healing process. If the correct type of wound is chosen, it can significantly boost wound healing. It works great for those wounds that have stalled in healing, wounds free of infection or necrotic tissue, and wounds with a low bacterial count. It cannot be utilized on wounds that have tendon, joint, or bone exposure or on individuals with a bovine sensitivity (Advanced BioHealing, 2007).
Advanced wound care products are tissue-engineered skin equivalents developed from the neonate foreskin. Dermagraft, for example, is a skin equivalent that has living dermal fibroblasts and their by-products on a biodegradable scaffold (Figure 7). The scaffold that contains the fibroblasts takes approximately 1 week to dissolve. These fibroblasts then migrate into the wound, which then assists in restimulating the wound-healing process.
Dermagraft is ineffective for wounds with a high bacterial count. Whether the wound is actually infected or not, if there is a high bacterial count on the skin surface, the grafting process will not be successful. To ensure a successful grafting process, oral antibiotics are often used in conjunction with silver dressings on the wound. This will decrease the bacterial counts on the wound surface but should be initiated at least 2-4 weeks before the grafting.
Dermagraft is a great adjunctive therapy with the wound-healing process. Grafting can be done every 6-8 weeks until the desired results are obtained. It does, however, need to be utilized with good wound care practices including debridement, pressure relief, and infection control (Advanced BioHealing, 2007).
WOUND CARE FOR THE INFECTED WOUND
All wounds are contaminated with bacteria, but that does not mean that a wound is infected. Bacteria do not always interfere with the wound-healing process. Wound contamination indicates that bacteria are on the wound surface but that they are not reproducing, replicating, or interfering with wound healing (Gardner & Frantz, 2004). Wound colonization indicates that the bacteria are on the wound surface but are reproducing and replicating. However, these bacteria are not in quantities high enough to disrupt or interfere with wound healing. In actuality, bacterial wound contamination and colonization are common to all healing wounds and are in fact prerequisites for the wound-healing process (Gardner & Frantz, 2004). Some of these bacteria may also be involved in a mutually beneficial relationship with the host by preventing the more virulent organisms from adhering into the wound bed (Gardner & Frantz, 2004). The wound-healing process does, however, become compromised when the bacteria reach quantities high enough to disrupt wound healing. This is called critical colonization (Gardner & Frantz, 2004). These bacteria are now competing for the oxygen and the nutrients of the wound and therefore disrupt or delay wound healing. Critical colonization is characterized by signs and symptoms of a local infection. Classic signs and symptoms of a critically colonized wound are increased redness surrounding the wound, increase in drainage, and decline of the wound-healing process.
Infection is characterized by the invasion of bacteria resulting in tissue injury. These bacteria are replicating and multiplying in quantities, which cause injury and impair healing. This usually occurs when the bacteria have reached quantities of 1,000,000. Sepsis occurs when the quantities of bacteria have reached 10,000,000-100,000,000 (Gardner & Frantz, 2004). Classic signs and symptoms of infection are significant periwound redness, swelling, tenderness, warmth, and pain. The drainage of the wound is increased, and there is often a foul odor associated with that drainage. This drainage is usually yellow or green in color. The tissue within the wound does not appear healthy. It bleeds very easily and is often beefy red in color. This tissue disintegrates, and the wound enlarges in either depth or circumference. There may also be pocketing and tunneling around the wound. The wound-healing process has completely halted, stalled, and reversed. Instead of the wound decreasing in size, it is now increasing in size.
It is important to note, however, that not all odorous wounds or wounds with increased drainage indicate infection (Gardner & Frantz, 2004). Certain dressings can cause a distinct odor or can cause an increase of the drainage of the wound. Odor may also indicate that the dressings need to be changed more frequently. Another important fact is that redness does not always indicate infection or critical colonization. Redness surrounding a wound is a normal part of the wound-healing process. Redness is normal during the inflammatory phase of healing. Therefore, redness could indicate that the wound was retraumatized. This reinjury could occur from trauma to the wound or from either the cleansing process or the dressing utilized. Perhaps the dressing adhered to and traumatized the wound bed with its removal, or the cleansing of the wound was too aggressive. When cleansing a wound, it is essential to remove the surface contaminants but not to traumatize the wound bed, and it is essential to choose the correct dressing products to promote wound healing but not to hinder it.
CLEANSING OF AN INFECTED WOUND
For a wound suspected of critical colonization, Ultrasonic mist is an excellent way to cleanse it. Ultrasonic mist has multiple purposes. It not only cleanses the wound but also debrides, decreases the pain, and decreases the bacterial colonization of the wound.
Antibacterial soaps or cleansers can also be used for a suspected wound infection or for an infected wound. If a wound is infected with gram-positive organisms, such as Staphylococcus aureus or streptococcus, a capful of Hibiclens can be very effective against eliminating those organisms (Schober-Flores, 2003). Caution should be used, however, with the use of Hibiclens. First, Hibiclens can cause a severe corneal injury if it does splash in the eyes of an individual. Therefore, parents need to be educated to limit playtime in the bath and not use on the face. Second, if used on a chronic basis, there is a potential for a gram-negative organism to grow. It does accomplish its task of eliminating gram-positive organisms, but this "opens the door" for the gram-negative organisms. For this reason, it is recommended to use Hibiclens only for short-term and only when indicated.
Vinegar soaks are very effective against reducing or eliminating gram-negative organisms, such as pseudomonas. The recommended dilution for the vinegar soak is 1 part vinegar to 40 parts water (Schober-Flores, 2003). This can be done as either a compress or a cleansing process. Lastly, Clorox soaks are effective against all types of organisms, including gram-positive, gram-negative, and even fungal organisms. The recommended dilution for a Clorox soak is either one teaspoon of Clorox per gallon of water for a compress soak or one half cup of Clorox for an entire bath filled with water. Clorox baths can also be utilized as a preventative measure as well. It can be used on infected wounds and wounds with high bacterial counts or weekly to prevent infections.
Topical antibiotics can be used for wounds with critical colonization or for an infected wound. However, topical antibiotics are not recommended to be used long-term. The problem with long-term use is that resistance can occur to a bacterial organism or perhaps a sensitivity or allergy can develop to the product being utilized. Remember that individuals with EB have chronic wounds that often struggle to heal. Using a product on a long-term basis can result in the development of these resistant organisms. Studies have shown that it is not the antibiotic itself that assists in wound healing but rather the ointment base that the antibiotic is "housed in" that promotes wound healing. Therefore, it is recommended to use Vaseline rather than a topical antibiotic unless otherwise instructed by the physician (Schober-Flores, 2003). However, if long-term topical antibiotic use is recommended, then it is important to rotate the antibiotics every 3 months to prevent resistance from occurring (Schober-Flores, 2003).
Silver products are very effective against bacteria as well. Silver is a broad-spectrum product that is effective against all organisms such as gram-positive, gram-negative, and/or fungal organisms. It can be utilized for wounds with critical colonization and infection or for the prevention of infections. It is also used to pretreat the wounds before grafting with Dermagraft. It is important when grafting that the bacteria on the wound surface are kept to a minimum to have a successful grafting process. Therefore, silver products are very effective not only for the infected wound but also for those wounds requiring a low bacterial count.
WOUND DRESSINGS FOR THE INFECTED WOUND
Wounds that are infected tend to have a significant amount of drainage. When choosing a dressing for an infected wound, it is important to choose a dressing that will absorb that drainage and pull the drainage away from the periwound area to prevent further skin breakdown. As noted previously, for a noninfected wound, it was recommended to add moisture to the wound by applying a topical ointment to the wound or dressing. Noninfected wounds that are kept in a moist environment heal faster because it allows the epithelial cells an easier route to migrate across to close the wound. However, for an infected wound, it is not recommended to add moisture to that wound but rather to choose a dressing that will absorb the drainage from the wound. Bacteria thrive in a warm moist environment. If moisture is added to the wound, this will promote their growth. The drainage of the wound will also cause irritation and further extension of the wound if not removed from the intact skin. An easy rule of thumb to remember is, if it is a dry wound, add moisture, but for a wet wound, dry it out.
Some dressing options for the infected wound are foam products, such as Mepilex Transfer, Mepilex lite, and Mepilex, or silver products, such as Mepilex Silver, SilvaSorb, or Acticoat. Mepilex Transfer is designed to be used on wounds with a large amount of drainage (Figure 8). Its purpose is to transfer the drainage away from the periwound or wound area into a secondary bandage (Molnlycke Healthcare, 2006). The secondary bandage should be a dressing that can hold a fair amount of drainage; therefore, a foam bandage is preferable, such as Mepilex lite and Mepilex foam.
Silver products are very effective in eliminating bacteria. The silver ions in silver products are activated when they come into contact with the wound drainage. There are many silver products available, and it is important to follow the manufacturers' instructions (Hess, 2005). Keep in mind, however, that silver is a metal and cannot be used on an individual scheduled for an MRI. Make sure to inform the technician and remove this bandage before a scheduled test.
For wounds with a large amount of drainage, a calcium alginate can be added as a primary dressing. Calcium alginates are seaweed dressings that can assist in debriding and absorbing drainage (Jones et al., 2004). These dressings form a soft gel when mixed with wound fluid and can actually absorb 20 times their weight. A secondary bandage such as Mepilex Transfer and Mepilex can be used to wick away the drainage away from the periwound area to prevent any further skin breakdown.
Dressing supplies are ordered by a healthcare provider from a dressing supply company. For patients who have Medicaid insurance, Direct Medical is the supply company of choice. They carry all the silicone products, are familiar with EB, and work well with Medicaid for insurance reimbursement. They can be reached at 1-800-659-8037. For a private-pay insurance, it is important to check with the insurance company to determine what supply company it has contract with. However, National Rehab does bill for Medicare and for some other private insurances, so they can also be a great resource as well. They can be reached at 1-800-451-6510. Another option for a supply company that bills for Medicaid, Medicare, and private insurance is CCS Medical. They can be reached at 303-483-1677.
Wound care for an individual with EB can be challenging. There is not one dressing that works for every individual or one dressing that works for every wound. Wounds change; therefore, a wound should be assessed with every bandage change.
The first step is to understand how a wound heals. It is important to understand the three different phases of healing and how a wound should appear in each phase. Always remember when assessing a wound to observe not only the wound itself but also the surrounding tissue or the periwound area.
The next step is to understand the signs and symptoms of infection. Does the wound have the characteristic symptoms of infection such as redness, odor, pain, and increased drainage? What does that drainage look like or smell like? Is it yellow, green, or bright red? Is the wound extending and is there fragile bleeding tissue or wound pocketing? These are all signs of a potential infection.
Next, assess the moisture balance of the wound. Is it a dry wound or a wound that has a large amount of drainage? Look again at the surrounding tissue, is it white and macerated, red and irritated, or pink and healthy? Red and irritated tissue can possibly indicate infection or that the drainage is irritating the surrounding tissue. White and macerated tissue indicates a wound that has too much moisture added to it either because of the topical ointment or the dressing that is being utilized is not the correct bandage for that wound. Remember that the drainage itself can cause wound breakdown or an odor with some particular dressings. The ultimate goal is to have the surrounding tissue look healthy with no surrounding erythema, extension, or breakdown. If the surrounding tissue is pink and healthy, then the wound has the correct moisture balance.
Lastly, it is important to understand the dressing products available. Understand how to use these products and what their purpose is designed to do. It is also important to understand the pathophysiology of the skin disorder you are working with. EB is a genetic skin disorder characterized by extreme skin fragility. It is important to apply only nonadherent products to these individuals' skin. Dressings for these individual's are used to promote wound healing but are also used for wound protection. It is just as important to prevent wound trauma or breakdown as it is to heal the wound.
If you understand these basic principles, you will make a huge difference in the wound-healing process and in the life of an individual who is diagnosed with EB. Wound care for these individuals is a big part of their daily routines, but it is important to remember that many of the individuals we work with are children. Children need to live the life of a child; therefore, we need to encourage our kids to live as normal a life as possible (Figure 9). By choosing the correct product and protecting the skin, you will allow them that opportunity (Figure 10).