EB Care FAQ

Q: My son was diagnosed with anemia. I don't know how this happened. We are on an oral iron supplement now and will be going back to recheck his levels in a couple of months. What happens if the supplement doesn't work?

A: Anemia is not uncommon for those with Epidermolysis Bullosa (EB), even when taking daily supplements. Blood loss from wounds, along with poor iron absorption can contribute to the problem. Taking the iron supplement with a citrus juice (if tolerated) can help absorption. If oral supplementation is unsuccessful, your physician may recommend an iron infusion (IV iron) to build up your son's iron stores. In some cases of more serious anemia, a transfusion of packed red blood cells (PRBC) is required.
 

Q: My daughter's dentist is recommending braces. I am worried that she will have problems because of her EB.

A: It is worrisome for EB parents to consider braces for their children. There is potential for mouth sores and if braces are painful, they may interfere with food intake. That said, many individuals with EB have tolerated braces well. One suggestion from an EB dentist is to place a couple of brackets first, to see if the oral cavity can tolerate braces. Another suggestion is to use dental wax to cover the wires and smooth the surfaces of the braces to provide additional protection. You may want to ask your dentist if Invisalign is an option for your child. As with any child with braces, meticulous oral care will be required to get the best results.
 

Q: My daughter has EB and is expecting her first child. We were discussing her plans with the new baby and she would like to breastfeed. Will my daughter be able to or will the friction of nursing cause her to develop blisters on her breasts? And are there ways to treat this to make it more bearable?

A: Your daughter should certainly try breastfeeding if it is her desire. It is possible that she might do as well with breastfeeding as a non-EB mom. The experience of breastfeeding varies widely in the EB population.

Very few EB moms have successfully breastfed their babies without any problems. Most have difficulty because when a baby suckles, blisters tend to form around the nipple area. I would suggest that your daughter ask her OB/GYN doctor to recommend a lubricant that is baby-safe.

She can apply this to her nipples prior to feeding the baby. If that alone does not help to reduce/prevent blistering, she can try a silicone nipple shield on her lubricated nipple. This may offer her some additional protection as the baby feeds. If these methods are problematic, she can consider using a breast pump on her well-lubricated nipple. This will widen the diameter of pressure on her nipple. The pumped breast milk can then be bottle-fed to her baby. It is also possible that hand expression of her breast milk will be less traumatic to her breast tissue.

Not every technique works for every woman. It is important to remember that some EB moms are not successful when it comes to breast feeding and/or pumping their breast milk. Your daughter needs to know it is okay to bottle-feed the baby with formula if necessary. If she is not able to breastfeed, her pediatrician can recommend a good formula for her child.

There is often concern expressed that bonding occurs during breastfeeding and that the inability to breastfeed may prevent bonding between mother and child. The truth is that the acts of cradling the baby, making eye contact and talking or singing to the baby will allow a mom to bond with her child, even when bottle fed.
 

Q: Which is better for an EB baby, cloth or disposable diapers? Are there any brands you can recommend?

A: Cloth and disposable diapers have both been used by parents with EB newborns. When using cloth diapers, however, you should consider investing in wicking diapers, as opposed to traditional cotton diapers that stay wet against the newborn's skin. Options include:

• Thirsties Duo Wrap Snap (size 1: 6-18lbs) (size 2: 18-40lbs). They have an outlet that sells slightly imperfect products at reduced cost.
• Swaddlebees Snap All-in Ones (available from www.amazon.com). One microfleece topped microfiber soaker sewn into the diaper and a second soaker that snaps in for the larger babies.
• Happy Heiny's One Size Cloth Diapers. It comes with a newborn and medium/large insert (Helpful hint from the manufacturer: Should the fleece roll out? Yes, it should roll out. Do not try to roll the fleece in.)
• FuzziBunz (One Size Cloth Diaper 7-30lbs) from Nurtured Families. This is a quick dry fleece: baby feels dryer faster and fleece stays nicer longer.
• Sugar Peas diapers (size 1: 6-20lbs) (size 2: 18-38lbs) (available at www.diaperwear.com) Made entirely of organic hemp fleece.

Using disposables is sometimes possible, either on their own or inside a microfiber diaper cover if the diaper insert cannot absorb enough. Options include:

• Pampers Swaddlers
• Huggies Supremes
• Huggies Ultratrims
• Walmart White Cloud
   

Q: My daughter has thickened nails that have very jagged edges. Is there any good way to trim them?

A: You may need to start out with a podiatrist quality nail clipper to trim any excess length. If her nails are very thick regular clippers may not be effective.

Then after your daughter's bath, her nail edge can be gently filed, taking care not to injure the surrounding skin, till the edge is smooth, and afterwards, you can file across the face of the nail (where nail polish would be applied) in order to thin them. If you file a little after every bath, you should be able to maintain the results.
 

Q: My child has EB and our pediatrician has told us he is colonized with MRSA. What should we do to prevent spreading MRSA to other family members?

A: Staphylococcus aureus or Staph is a common bacteria found on the skin.

Methicillin Resistant Staphylococcus aureus (MRSA) is a Staph bacteria that has become resistant to treatment with Methicillin, an antibiotic in the penicillin family and other related drugs. MRSA is also called Oxacillin Resistant Staphylococcus aureus (ORSA) in some laboratories. MRSA and ORSA are different names for the same bacteria.

It is important to note that if the MRSA is in the respiratory tract, it can be spread by droplets, otherwise MRSA is not airborne. Although sometimes MRSA can cause severe infections, usually it acts like other forms of Staph.

Many people carry MRSA on their skin or in their noses without being sick. They are considered colonized with the bacteria and can spread it to others. MRSA can be acquired in the hospital or in the community.

Things that can prevent the spread of MRSA in your household:

• Hand washing is the most important thing to do to prevent the spread of MRSA. Rubbing your hands with soap and water loosens bacteria, while rinsing with running water removes bacteria from your hands.   Generally, regular soap is fine for hand washing.
• Each family member should routinely practice good hand washing with soap and water.
• Encourage those who can to shower instead of taking baths.
• Keep skin healthy by using insect repellant and sunscreen.
• Pick one place in your home to do dressing changes and make sure the area is cleaned well after each change.
• Wash your hands before and after caring for colonized or infected skin, especially if there are open areas. 
• Periodically clean bath toys with bleach and water or run them through the dishwasher. Avoid bath toys that can not be thoroughly cleaned, such as those with squeakers.
• Do not share towels. Use separate towels and washcloths for each person in your family. White towels are better because they can be washed with bleach.
• Wash sheets and towels regularly.
• Change clothes daily and wash them before wearing them again.
• Let the doctor know if you or a family member is "colonized" with MRSA to ensure proper care.
• Keep draining or oozing areas of the skin covered with clean bandages.

Your doctor may:

• Recommend using a special soap (like Hibiclens® or Phisohex®).
• Recommend using a hypoallergenic moisturizer or Vaseline® to be used on the skin after bathing in order to limit dry skin.
• Prescribe mupirocin cream or ointment (like Bactroban® or Centany®) to apply at the first break in the skin or sight of a pimple to try to prevent more serious infections.
• Recommend that all household members use mupirocin ointment in the nose 2 times a day for 5-7 days to decrease the risk of carrying ORSA.
• Prescribe an oral antibiotic if there are signs that the colonization has worsened to infection.
   

Q: I have EBS and have been considering getting a tattoo, the only real hesitation being the skin issue. Do you have any advice on this?

A: There are a few people with EB who have had tattoos. What has been learned from these individuals is that the milder your EB is, the easier it will be for you to tolerate the tattoo. Some patients with very mild EB who have skin that is not as fragile do fine with tattoos. The more fragile your skin, the more of a problem you may have. The problems others have had are pain, increased healing time and scarring.

You need to consider that with the skin fragility of EB, there is no guarantee of a good outcome, even in the hands of an experienced tattoo artist, and you could blister from the procedure and end up with a wound requiring care. Proper wound care will be very important- you will need to follow the post-tattoo care guidelines carefully. After your wound heals, it is possible that your tattoo may not look as you originally intended.

Another concern is that, if you ever wish to remove your tattoo, your EB may limit your ability to have this done.
 

Q: I have a newborn with EB. Will he be able to receive the routine recommended vaccinations? Is there anything special we should tell our pediatrician about injections?

A: Vaccinations during early childhood provide an important defense against serious illnesses. There are no contraindications for routine vaccinations for children with EB. They should be given at the appropriate, scheduled times as directed by your pediatrician. Alcohol or another cleansing agent should be gently dabbed onto the area where the injection will be given. Following the injection, there should be no vigorous massaging to the area. Gentle pressure should be held on the injection site to be sure the medication doesn't leak out. Your baby may have a mild reaction to some vaccinations, including a slight fever (under 102 degrees F), fussiness, and redness in the thigh area where the shot was given. These symptoms typically last up to two days and should be discussed with your pediatrician.
 

Q: My firstborn child has a severe form of Epidermolysis Bullosa (EB). Our genetic test results say it is a recessive form and both my husband and I carry the gene mutation. I have been told that there is a method, PGD, which can ensure I have an EB free baby when I have our next child. Can you tell me how it works? - New to EB

A: Having the genetic mutation of your child's EB identified is the first step in Preimplantation Genetic Diagnosis (PGD), improving your chances of having an EB free child. PGD is accomplished with in-vitro fertilization, in which the mother takes hormones to increase the number of eggs she produces during her monthly cycle. These matured eggs are surgically removed and then fertilized with the father's sperm. When the fertilized egg reaches the eight-cell stage, one of the cells is removed and analyzed at a special laboratory to see if it carries the EB mutations that were identified in the family. The fertilized eggs that do not carry the mutations can then be implanted. If pregnancy results from the implantation process, the child will be EB free, but may also be a carrier like the parents. This process has been used successfully in a number of EB families. However, it is very expensive and not always covered by standard health insurance. Successful pregnancy with live birth is achieved in approximately one third of PGD cycles. As with any medical procedure, you should discuss with your medical provider whether PGD is right for you.

For more information on Genetics and EB, you can go to www.genetests.com and at GeneReview, search for Epidermolysis Bullosa. Currently there are articles by Ellen G Pfendner, PhD and Anne W Lucky, MD on EBS, DEB, JEB and EB-PA available.
 

Q: I have heard a great deal in the EB Community about kidney problems that affect those with EB. Can you tell me more? Is there a way to screen for them? 

A: Diseases in the urinary tract system, which includes the kidneys, ureters, bladder and urethra are not common, but can be serious, even life-threatening, when they do occur. There is a need in the EB community for practical guidelines for monitoring and management of urinary tract system issues.

Glomerulonephritis (both acute and chronic), renal amyloidosis, IgA nephropathy and obstructive uropathy have been reported in both Junctional and Recessive Dystrophic EB. Each can lead to chronic renal failure (CRF). There are no hard numbers on how many in the EB community develop CRF.

Jemima Mellerio, BSc, MD, FRCP discussed protocols for the screening and management of urinary tract system issues at the IV International Symposium on Epidermolysis Bullosa in Santiago, Chile in September of 2007. The following reflects these recommendations:

Recommended Screening for Urinary System Disease:

For RDEB and JEB patients, a screening is recommended twice a year:

• Urea and electrolytes
• Urinalysis
• Blood pressure check

If the above are not normal, consider:

• Urine microscopy
• Urine culture
• Renal ultrasound
• DMSA scan
• Functional tests like a MAG 3 (diagnostic imaging)

For JEB patients, a screening is recommended annually: 

• Renal Ultrasound

If the above is not normal, consider:

• DMSA scan

If the above shows scarring or discrepancy, consider:

• Functional tests like a MAG 3

If obstruction is suspected, a renal ultrasound is recommended
If vesico-ureteric reflux is suspected, consider a MAG 3 or a micturating cystourethrogram (MCU)
 
General Principles of Management:

• Avoid instrumentation and surgery if possible
• Urethral catheters are tolerated short term
• Supra-pubic catheters are well-tolerated longer term
• Avoid constipation and dehydration
• Encourage toilet training at a normal age
   

Q: When I was diagnosed as a child, I was told I had EBS, Weber Cockayne.  In reviewing material on the website, I don't see my type listed. What happened?

A: I understand your confusion. The EB experts around the world got together and arrived at an updated consensus on the classification of EB subtypes, based on newer data, both clinical and molecular. Some sub-types of EB were renamed in the process.

More information on types and subtypes of EB