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

0 Comment

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, although for people also use supplements that improve their body and mind, since there are also supplements made just for improving performance and focus, and not that difficult to get from sites as

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.

Facebook Twitter Google+ Flickr YouTube 

(Visited 340 times, 1 visits today)