My take-away from the Debra’s PCC in Orlando – Part 6

Here is more information about several talks from the conference from July 31, 2012:

Common Gastrointestinal Problems in Patients with EB & Nutritional Challenges in EB People with EB and Clinicians Working Together.

These two talks were done by Dr. Michael Farrell & Dr. Lynne Hubbard respectively. I have to be honest-both presentations were old hat to me and presented no new information, just strengthen my beliefs that I’ve been doing right by my son; however, in an effort to be thorough, here’s what they entail:

Depending on the form of EB, the esophagus can be very compromised or very little if none. Scarring within the esophagus can reduce the size of it, causing difficulty in the passage of food, even liquids. Many times children will experience episodes of food impaction with the expectoration of large amounts of mucous. Refusal to eat due to pain compromises the patient’s nutrition at a time where it’s most needed for wound healing. A Barium Swallow may be ordered by the gastroenterologist to assess the need for treatments such as esophageal dilatation. Dilatation is a procedure done under light sedation that incorporates the use of a small balloon to increase the size of the esophageal opening.

In instances where esophageal stricturing is so severe, dilatation may not be helpful, a gastrostomy tube or gastric button device may be indicated to increase the individual’s nutritional intake. A gastrostomy tube is inserted to an opening (stoma) into the stomach for the delivery of nutrients, fluids and medications. The procedure is usually done by a surgeon or a gastroenterology surgeon. In many instances feedings through the gastrostomy tube are given overnight using a pump. (Please note the head of the bed should be slightly elevated when infants/ children are receiving feedings.)

The use of gastrostomy tubes may be helpful in the nutritional management of infants and small children in EB who do not have esophageal involvement but need nutritional enhancement intake for growth purposes and wound healing.

If naso gastric tubes are used it is usually short term due to risks of esophageal erosions and infection.

Thank you to Debra for this explanation.

As per my son’s story, just to make this blog a bit more personal and coming from a mom’s prospective, which I know helps, I completely understand the reluctance of many EB parents to put the g-tube in. I was one of those moms, it seemed such a drastic measure, but soon the decision was made for me:
Nicky, at 9 months old, weighed in at 18lb, but at 3.5 years old, he only weighed 21lb. In nearly 3 years he had only gained 3lb despite my best efforts, so a gtube because a life-saver for him. My decision to have the g-tube placed, however, was NOT about his weight. As I was told by several doctors, calories go to wound healing first, then growth. As per growth, calories go to head circumference first, then height, THEN weight. You can have a perfectly healthy SKINNY child, as long as he’s growing normally in the head/height department. Nicky’s pediatrician followed Nicky closely… first his height curve started falling below the charts, then his head, which had been consistently in the 75th percentile, dropped to the 25th percentile within a few months. A gtube became a necessity, Nicky would wake up with his pillow drenched in saliva, would wake up in the middle of the night asking for his bottle because he simply could not drink enough during the day, and no matter how caloric I would make those bottles, he could never drink enough of them. It would also take him hours to finish one single bottle. I opted for a throat dilatation and a g-tube placement done at the same time. Dr. Castillo told me his throat was only 1mm and within 3 months he gained 10lb! His wounds started healing faster and faster and at one point, when he was 4, he was actually wound free!! First and only time in his life. Placing the g-tube was the best thing I ever did for him, even though it was an excruciating decision to make.

Here is the video of this presentation courtesy of Debra of America:

The presentation from Dr. Lynne Hubbard was mostly about the daily challenges of EB and the nutritional aspects of the condition. What she talked about a lot was about making sure the patient understands and agrees with the procedures if they are old enough, and make sure they have a feeling of control over their body and their condition, particularly involving g-tubes. Too often she’s seen patients who were not even asked if they needed a g-tube placed, they just woke up with it.

Here is the Video of this Presentation, courtesy of Debra of America:

Hanging out with the Brits… in my book, heaven!

I was lucky enough to have Dr. Lynne sitting at my table for dinner and I told her how right she was about giving CONTROL to the patient. It’s their body afterall.  At a young age I would give Nicky the needle to pop his own blisters, give him the freedom to take off his bandages, give him the decision of when he wanted throat dilatations done, hand surgeries, and when we change bandages I always ask him what he wants to change today and I’ve done this for over 10 years now and it has worked great. Lynne relayed to me stories of families she visited in England and how different parents can be very different with their children’s care. I must admit Lynne’s British accent was fabulous and we spent a lot of time talking. Next to me at dinner was another Brit, I was in heaven, we spoke for a while before I found out who he was Ben Merrett, the Chief Executive Officer of Debra UK! Very exciting. I congratulated him for spreading EB awareness so much, and he told me they have fierce families who constantly work to get the word out. For those that do not know, Princess Diana was a BIG supporter of Debra UK and I wondered if any other member of the royal family had stepped up since. To my delight, he told me the Countess of Essex has stepped up which is fabulous. But… who is the Countess of Essex? These noble titles escape me. He laughed. Sophie is the wife of Prince Edward, the youngest brother of Prince Charles. Awesome.

Here’s a photo of me (right) with my new British friend Lynne (left). Thank You Lynne for all the advice and the friendly talk!!

 Oral Health and Epidermolysis Bullosa

This presentation was very interesting, I took a few photos of these slides and several notes., it was given by Dr. Timothy Wright.

The presentation started with the details of oral health… as this slide shows, oral tissue screening, the importance of using fluoride, a lowjet pick, a toothbrush he recommends is from the Rotadent line, a toothpaste that includes flouride, and he recomended an oral rinse called Pro Dentx, although I could not find a link to give anyone of where to buy it, HOWEVER, here’s the link to a Colgate Professional Product he recommends, you will need a prescription for it though apparently. It’s called Colgate Prevident.
One thing he stressed is to find a dentist, which might take a while, and then please, please, please have regular checkups. It’s much easier to restore teeth and keep them before their complete destruction.

He also stated that dental implants ARE possible! He described a case of where a young woman with RDEB received dental implants, so there is hope.

This slide shows the ingredients of the famous ‘Magic Mouthwash’ which coats the mouth and helps with Stomatitis and other possible mouth infections. Ingredients include 100ml of Maalox, 25-50ml of Viscous Xylocaine & 25-50ml of Benadryl. Other Oral Ulcerations, as shown on this other slide below, such as Thrush, can be treated with Oral Nystatin, Ketocanazol or Chlorhexidine.

One word about finding a dentist: It took me years to find one. The first time I took Nicky to a regular dentist in Phoenix, they would have kept him for his whole life since I started taking him there when he was so young and we were all learning together, but once I moved, nobody would touch him. I even called the 1-800-Dentist and they could not help me! Finally I found a dentist right at the place where we went all the time, CHLA. I cannot praise them enough. They are gentle, thorough, amazing. To find a dentist in your area, your best resource is to talk to other parents/patients in your area, contact the Debra Nurse, having a dentist you can rely and trust is of outmost importance.

Here is the video of this presentation, courtesy of Debra of America:

GO TO PART 7 –>>

Links to — > Part 1Part 2 – Part 3 – Part 4 – Part 5

Mom to 3 boys, 1 in heaven, 2 on earth. My first son Alex (whose demise is most likely EB related) was stillborn at full term. After a miscarriage, I had my second son Nicky, who has the Recessive Dystrophic form of Epidermolysis Bullosa. My youngest son, Connor, is 100% healthy, and I never, ever take it for granted. I am an author, photographer, graphic artist, webmaster, blogger and more.

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EB and Dental Care

By Melanie (Logan) England
(this information was previously posted on the EBmommas website)

The Dystrophic and Junctional forms of Epidermolysis Bullosa are notorious for causing deformities in the enamel and dentin of the teeth, but people with all types of EB are prone to dental decay due to oral hygiene complications. Blistering in the oral cavity, fused oral vestibules and smaller than normal mouth opening can all make it difficult, if not impossible to brush and floss properly.

According to pediatric dentists at,here are steps parents and patients can take to keep the teeth as healthy as possible. For your convenience they have an office in Madison.

Start a regular routine of brushing your childs teeth as soon as the first teeth appear. If the mouth is too sensitive and blistered for regular brushing, a Toothette sponge can be used (ask your pharmacy for these), or the teeth can be wiped clean with a gauze sponge wrapped around your finger.

See a pediatric dentist who specializes in disabled children as soon as the first few teeth appear.

Check with your local water board to find out if your drinking water if fluoridated, and ask what the concentration levels are. This will help your dentist decide if your child needs a fluoride supplement, and what dosage is appropriate.

Soften the bristle of the toothbrush but running them under hot tap water before brushing. Oral-B extra soft childrens toothbrush ages 1-3 years is one of the best on the market, and the smallest for reaching back teeth in children with small mouth openings.

Use a fluoride toothpaste for sensitive teeth such as Sensodyne or Colgate Sensitive. Plain baking soda can be used if a toothpaste cannot be found that does not irritate the mouth.

Chewing sugar free gum for 15 minutes after each meal helps loosen plaque and food debris from the teeth.

When your childs mouth is severely blistered or ulcerated brushing may be impossible without great pain. During this period the mouth can be rinsed with a solution of 1tbs hydrogen peroxide mixed with 4tbs water and < teaspoon of salt. This will soothe the mouth and loosen debris. If you choose a commercial mouth rinse, read label and make sure it does not contain alcohol.

What your dentist can do:

Prescribe Decadron elixir- a liquid steroid that is swished around the mouth for 2-3 minutes then spit out. For a child too young to follow instructions, it can be swabbed inside the mouth with a toothette or gauze sponge.

For extreme pain your dentist can prescribe 0.05% topical lidocaine gel to apply to gums and inside of mouth 3 times a day.

Magic Mouthwash is a pain reliever that combines lidocaine, benadryl and Maalox, and many EB patients report good success with it. Your pharmacist will mix it in the proper combination per your doctors prescription.

Candidiasis, commonly known as thrush, is another commonly found oral problem in people with EB. Anemia and use of antibiotics can cause thrush, which looks like a white coating on the tongue, palate, or inside of the cheeks. It can also make the surrounding tissues puffy and tender. Your doctor may prescribe a Nystatin solution to rinse and swallow, or liquid Diflucan to be take orally for 7-10 days.

Oral Surgery

Despite diligent care EB teeth may still develop carries and require fillings, crowns, or sealants. Talk with your dentist about which option is best for you or your child. Simplex patients who still have good enamel can often opt for fillings, but patients with enamel hypoplasia will require crowns because the fillings have nothing to bond to.

Its usually far less traumatic for children to be sedated rather than awake for dental procedures, and much safe for EB children to have it done as same day surgery rather than in the doctors office. Discuss all your options with the doctor and decide which is safest and most comfortable for you or your child.

Have the doctor go over the steps that will be taken to minimize trauma to your childs skin during the procedure. Make sure they include the following:
Orient all staff involved in the procedure on basic EB care
Have an assistant to manually retract gums and tongue instead of using metal retractors.
Coat all instruments with Vaseline or Aquaphor, as well as the gas mask the anesthesiologist will use.
Pop blisters as the form during the procedure so they dont grow and cause the patient even more pain.
Avoid if possible allowing suction to touch the oral membranes so less blistering occurs.

To find a dentist in your area contact your local chapter of the American Academy of Dentistry.

American academy of Pediatric Dentistry

EB and Dental Health

By Tim Wright, D.D.S., M.S. Department of Pediatric Dentistry

Thank you Brenda for typing this for us! 🙂

         Individuals with EB may have teeth with severely malformed enamel (enamel hypoplasia) and/or dental caries depending on the EB type. The enamel is usually normal in simplex and dystrophic EB types. Generalized enamel hypoplasia is typically limited to Junctional EB. Rarely individuals with non-Junctional EB types may have generalized enamel hypoplasia. Rampant dental caries occurs in Junctional EB partly because of the enamel hypoplasia. Dental decay also is frequently seen in patients with severe recessive dystrophic EB. This excessive dental caries results from severe soft tissue involvement which leads to dietary changes (soft and high carbohydrate), increased oral clearance time (secondary to limited tongue mobility and oral scarring), and creates an abnormal tooth/soft tissue relationship. Oral involvement also reduces the ability to practice preventive measures directed at reducing caries.

Because dental caries can form rapidly in individuals with recessive dystrophic and Junctional EB, dental examinations should begin by 1 year of age and be conducted at least twice a year. If caries becomes a problem then more frequent visits (4 times a year) are indicated for preventive treatments and examination. Individuals with mild EB can be treated much as any other patient. The dentist should, however, be made aware of any history of mucosal fragility and oral blistering since dental therapy can precipitate oral lesions even in mildly affected patients. Many dentists are not familiar with EB and the patient or parent must help educate the health care team. An altered approach to treatment may be required in individuals with enamel hypoplasia or rampant caries, extreme fragility of the mucosa and/or the presence of microstomia (a decreased oral opening size). Individuals with severe soft tissue involvement requiring multiple restorative and/or surgical procedures are often best managed with general anesthesia.

Preventing tooth decay is most challenging for individuals with severe mucosal involvement. In patients prone to oral blistering, oral hygiene may best be accomplished with a soft bristled, small headed toothbrush. Many small headed children’s toothbrushes are available, some of which have special grip handles that may be helpful to individuals with hands involvement. Running the bristles under hot water prior to brushing makes the even softer. Parents need to brush children’s teeth until about the age of 6 or 7 years because children lack the manual dexterity to properly clean their teeth. Parents should be very careful not to damage the gums or make the brushing experience negative and unpleasant. It is important, however, that the teeth be cleaned at least once a day preferably just prior to bedtime.

Be sure and use a fluoridated toothpaste. In small children a pea size amount is adequate to deliver the fluoride to the teeth. For individuals prone to developing cavities there are special high strength prescription fluoride toothpaste. Strongly flavored toothpaste (mint) may be irritating to the individuals with severe oral involvement, however, there are numerous non-mint flavors available. Bubble gum flavor is a big hit with children (and some adults). In addition to the systemic fluorides that we get in the water or alcoholic fluoride rinses available for the EB patients that are sensitive to strong flavoring agents and alcohol. Non-alcoholic rinses with greater amounts of fluoride are available by prescription. Chlorhexidine mouth rinses (an antibacterial rinse) also ma assist in controlling dental caries, however, sensitivity due to the high alcohol content can be problematic. This may be overcome by swabbing it directly on the teeth. Chlorhexidine rinse is a prescription item.

There are a variety of fluoride treatments applied by dentists. The most common treatment consists of placing a 1.23% acidified gel in a tray that is held in the mouth for 4 minutes. The high concentration of flavoring agents and acid nature of the gel make it unacceptable to some EB patients. Milder flavored neutral sodium fluoride gels are available that may be less irritating to fragile mucosa. Recently, a high concentration fluoride varnish has become available in the United States offering a wonderful option for delivering maximum fluoride protection to the teeth of individuals with even the most sensitive mucosa. This varnish is simply painted on the teeth by the dentist.

The diet constitutes major difficulty in caries control, and due to the complex systemic nutritional demands of individuals with sever EB types, this may be best managed with the assistance of a dietician. The effects of any diet planning should be considered with regards to dental health and tooth friendly foods (cheese, vegetables, fresh fruits) eaten as much as possible. Be careful of the less obvious cavity producing foods such as highly sweetened breakfast cereals, raisins and dried fruits. Cavity producing oral bacteria can ferment carbohydrates from a wide variety of foods. Other helpful hints are to rinse the mouth or drink water after eating if brushing is not possible. Bottle or breast feeding infants at bedtime can result in nursing caries after the teeth are present (1 year of age). If nursing continues after 1 year great care should be taken not to give the bottle while the infant is going to bed. Virtually any bottle fed liquid, except water, can cause rapid tooth decay of the baby teeth.

Individuals with even the severest forms of EB can now maintain their natural dentition providing them the ability to chew, eat a healthy diet and have a pretty smile. In the not too distant past, dental extraction was considered the treatment of choice for individuals severely affected with EB. Now we are able to prevent tooth decay, restore malformed enamel and help produce good alignment of the teeth. The future looks even brighter as dental prevention continues to improve, new dental materials are stronger and more esthetic than ever and new technologies, such as dental implants, continue to improve. Dental health for all EB patients has become a reality and that is really something to smile about.