Friday, October 24, 2008

National EB Awareness Week

Tomorrow kicks off National Epidermolysis Bullosa Awareness Week. We wanted to take this opportunity to highlight the importance of understanding EB.

For those of you new to our blog, here’s a quick summary of EB and Ella. Ella has Recessive Dystrophic EB (RDEB), which means she has limited or no collagen VII. In layman’s terms, we all have 2 layers of skin, the epidermis, or top layer, and the dermis, the bottom later. Between the two layers we have a collagen VII protein that keeps the two layers connected. Ella doesn’t have that protein, so with any friction or pressure her two layers of skin separate and either the top layer comes off completely, or the two just separate and form a blister just like if you burned yourself. We keep her legs, arms and torso wrapped to heal current boo boos and to hopefully protect her from new ones. In Ella, this disease is inherited recessively, so in order for her to get it both Joe and I have to carry the gene.

Ella was born on June 21, 2007. We had never heard of EB until that day. When she was born she had what are called “full thickness wounds” (basically, she was missing skin) on her right foot and right knee, and on her left leg from the knee down. We were lucky because there was a neonatologist in the hospital who had seen EB before and was able to quickly diagnose her. He then had her sent to Children’s National Hospital in DC, where she spent the next 34 days. We were lucky there too because we had wonderful nurses who cared for Ella and a wound care nurse who had worked with EB kids before.

I assume that most of you had never heard of EB until you heard of Ella. That is not uncommon. We have met doctors and nurses who have been in the medical field for 30 years and have never heard of EB. This week is our opportunity to spread awareness. Thousands of children and adults face the pain associated with living with EB. These kids, like Ella, have the same heart any other child has. They want to run, climb trees, ride a bike, or roller skate just like their friends, but participating in these activities often causes more boo boos.

This week is also an opportunity to help patient advocacy and research organizations who support EB families. Shortly after Ella was born we contacted DebRA of America (Dystrophic Epidermolysis Bullosa Research Association). Within a few short days we received a big box of EB-friendly items (ie. handmade quilt, bath sponge, toy, medical supplies, etc.) As a new family just learning about this disease, it was so helpful to have this stuff that we knew would be helpful for Ella. Once Ella came home from the hospital, DebRA sent their New Family Advocate to our house. She brought Ella soft socks, a very soft sheet for her bed, cloth diapers, and showed us several items that other EB families use, many of which we have adopted for ourselves. She even helped us with a bandage change and gave us ideas for making things better for both us and Ella. Joe and I support this organization both personally and financially, because we have seen first-hand how helpful it is.

The EB Medical Research Foundation (EBMRF) is dedicated to supporting research for EB. The EBMRF is unique in that they pay no salaries. All work, including executive, legal, accounting, development and administrative, is done on a volunteer basis. The goal is to keep operating costs at less that 1% of incoming donations so that a full 99% of contributions can go directly to the research program. They have made great strides in research for RDEB and we are hopeful that in a few years Ella will be able to benefit from the work they are doing. The unfortunate reality for kids with RDEB, is that most do not make it past the age of 30. With this organization there will one day be a cure for EB so that no other baby has to endure what Ella goes through, and so that Ella will live to be a wife, mother, grandmother and even a great-grandmother.

We are well aware of the economic situation we are currently in (hey, we work in politics), but if you are looking for a magnificent organization to support, or two, we ask that you check out these. More importantly, we ask that you take this week to educate just 1 person about EB, and if you want, you can tell them all about Ella!

1 comment:

Anonymous said...

For those of you that are Federal Employees, DeBRA is an authorized charity in the CFC (Combined Federal Campaign) book. Their code is "11990" - it was on page 57 in my book. This is a great way to give an annual donation and the money is pulled directly out of your paycheck over the 26 pay periods. For example, if you donate $130, they will pull $5 out of every bi-weekly paycheck.

Food for thought...

Jess Baker