The Butterfly Children Effect and Me
Last night was one of the most emotional nights of my life.
First, I had to give a speech. And I do not like public speaking. It's like finding yourself in one of those weird dreams where you are suddenly starkers in a public place with nowhere to run and hide. Not comfortable with it in the least.
Second, the speech was about my Aunt, who suffered and passed away from a horrifying and, until lately, rarely seen disease called Epidermolysis Bullosa.
Third, I had to give it in front of children and one adult suffering from EB and dozens of people who have lost children and loved ones to EB. These children are known as The Butterfly Children, because their skin is as fragile as a butterfiy's wings, and just as easily destroyed.
Since Aunt Pat died I have not put myself in the path of EB much. I receive the newsletter from DebRA, one of their national organizations. I watched as my cousin has written a book about day to day living with the disorder. I have seen the fabulous documentary, The Boy Who's Skin Fell Off about a young man named Johnny Kennedy. But the pain of losing my Aunt, my Godmother and my very close friend was too much to handle at times. So I have avoided EB like the plague it is for a very long time. Until Sunday Night.
For those of you who don't know what EB is, and that's probably 99% of the people on this planet, EB is a rare skin disease. The connecting tissues that anchor the epidermis (the top three layers of skin that we can see) to the dermis(the underlying layer) are missing. In the recessive dystrophic kind, my Aunt's form, it is so severe that the skin rubs off at the slightest touch... a mother's embrace, a baby's first attempts at crawling, sitting in a car for long trips. Huge blisters and blood blisters form as well, they pop, they get infected, they can cause death. Babies like Patsy come into the world skinned and in agony, and that agony is ongoing on a daily basis for the rest of their lives. Their fingers, toes, ears fuse together and by their teens, if Butterfly Children live that long, they have no fingers, only hands like mittens to function with. And that's just the surface. Inside their bodies, they can form blisters in the mouth, the esophagus, their intestines, making it difficult to eat unless food is soft or pureed, sometime forcing them to go on feeding tubes. It is a horrific disease, unknowingly and tragically caused by two people in love, making babies and blissfully unaware that both carry the recessive genes in their DNA.
My cousin Gena has worked tirelessly for decades to bring EB to the worlds attention. So have the parents and relatives of children born with it. And recently the governments around the world have been establishing EB Awareness Weeks in perpetuity. This, however, was not enough for my stubborn cousin. She began the EB Awareness Rally Relay, the Walk A Mile In My Shoes Rally, this year and a small contingent of people left Canada to begin a US tour. They have hit New York, Pennsylvania, St. Louis and last night was Denver's turn.
My family went as a group. My sister, who I drafted to speak with me, my brother and sister-in-law who mounted their Harley and joined a group of Harley riders to escort the EB Ralliers from the airport to Children's hospital, my mother (Patsy's sister-in-law) and my son, Chris.
I have never entered a room before and been met with such an emotional overload. Every where I looked were Butterfly children, racing their wheeelchairs, wrapped in untold yards of gauze. Pictures of those that have perished at EB's hands. Slide shows of Denver's EB community on all 4 walls. I was overwhelmed. Every face I looked into had elements of my beloved Aunt and it rocked me to my core. I knew that each one there affected by EB stood a 70+% chance of not being there next year and I felt my heart breaking.
We gave our speech. I tried to write it with humor, sadness without being maudlin, and hope and it was a success. But the most amazing thing was the people who came up after it was over, parents of these children, who wanted to know what it was like to have been blessed with 65 years of having my Aunt with us. Lillian "Patsy" Barbrey is the pinnacle that they all strive for. A fairly full life span with Recessive Dystrophic EB; most Butterfly Children die before their 20's. And the way she got there was a miracle wrought by my Grandmother.
A strong Tennesee mountain woman, Nana gave birth to this tiny, wounded child and was told by the country doctor to let her die. Instead, Nana nursed her for years, insisted that she live a normal life and backing down anyone who told her different, or treated her child as less that normal. As a result, Patsy grew up and did almost anything any other woman can. She learned to drive, she finished High School. She fell in love and married. She had 3 healthy children. No other EB woman had ever given birth until Aunt Pat. She faced the inevitable breakdown of her body with courage and died at 65.
I watched last night as my son wept on numerous occasions. He knew my Aunt well, and loved her unreservedly. But until last night, he never fully accepted the fact that he could also be a recessive carrier of the EB gene. Now he plans on being tested. If his genes are RDEB free, than he is home free. If he carries the gene, then his future wife will have to know and be tested herself. If she is free, wonderful! If not they will have some very heavy decisions to make as future parents.
So, like the butterfly across the globe that flaps it's wings and starts a hurricane on the other side of the planet, a small group of EB victims and the ones left behind have started flapping and creating their own storm.
I think their tornado of hope will only blow in good things. Strides are being made in Italy and other countries allowed to develope stem cell treatment and there is hope on the very distant horizon.
And I have faced a demon or two of my own, batted them down and have joined the fight. And that's a very liberating feeling. I feel free as bird right now...or, should I say...a Butterfly.
First, I had to give a speech. And I do not like public speaking. It's like finding yourself in one of those weird dreams where you are suddenly starkers in a public place with nowhere to run and hide. Not comfortable with it in the least.
Second, the speech was about my Aunt, who suffered and passed away from a horrifying and, until lately, rarely seen disease called Epidermolysis Bullosa.
Third, I had to give it in front of children and one adult suffering from EB and dozens of people who have lost children and loved ones to EB. These children are known as The Butterfly Children, because their skin is as fragile as a butterfiy's wings, and just as easily destroyed.
Since Aunt Pat died I have not put myself in the path of EB much. I receive the newsletter from DebRA, one of their national organizations. I watched as my cousin has written a book about day to day living with the disorder. I have seen the fabulous documentary, The Boy Who's Skin Fell Off about a young man named Johnny Kennedy. But the pain of losing my Aunt, my Godmother and my very close friend was too much to handle at times. So I have avoided EB like the plague it is for a very long time. Until Sunday Night.
For those of you who don't know what EB is, and that's probably 99% of the people on this planet, EB is a rare skin disease. The connecting tissues that anchor the epidermis (the top three layers of skin that we can see) to the dermis(the underlying layer) are missing. In the recessive dystrophic kind, my Aunt's form, it is so severe that the skin rubs off at the slightest touch... a mother's embrace, a baby's first attempts at crawling, sitting in a car for long trips. Huge blisters and blood blisters form as well, they pop, they get infected, they can cause death. Babies like Patsy come into the world skinned and in agony, and that agony is ongoing on a daily basis for the rest of their lives. Their fingers, toes, ears fuse together and by their teens, if Butterfly Children live that long, they have no fingers, only hands like mittens to function with. And that's just the surface. Inside their bodies, they can form blisters in the mouth, the esophagus, their intestines, making it difficult to eat unless food is soft or pureed, sometime forcing them to go on feeding tubes. It is a horrific disease, unknowingly and tragically caused by two people in love, making babies and blissfully unaware that both carry the recessive genes in their DNA.
My cousin Gena has worked tirelessly for decades to bring EB to the worlds attention. So have the parents and relatives of children born with it. And recently the governments around the world have been establishing EB Awareness Weeks in perpetuity. This, however, was not enough for my stubborn cousin. She began the EB Awareness Rally Relay, the Walk A Mile In My Shoes Rally, this year and a small contingent of people left Canada to begin a US tour. They have hit New York, Pennsylvania, St. Louis and last night was Denver's turn.
My family went as a group. My sister, who I drafted to speak with me, my brother and sister-in-law who mounted their Harley and joined a group of Harley riders to escort the EB Ralliers from the airport to Children's hospital, my mother (Patsy's sister-in-law) and my son, Chris.
I have never entered a room before and been met with such an emotional overload. Every where I looked were Butterfly children, racing their wheeelchairs, wrapped in untold yards of gauze. Pictures of those that have perished at EB's hands. Slide shows of Denver's EB community on all 4 walls. I was overwhelmed. Every face I looked into had elements of my beloved Aunt and it rocked me to my core. I knew that each one there affected by EB stood a 70+% chance of not being there next year and I felt my heart breaking.
We gave our speech. I tried to write it with humor, sadness without being maudlin, and hope and it was a success. But the most amazing thing was the people who came up after it was over, parents of these children, who wanted to know what it was like to have been blessed with 65 years of having my Aunt with us. Lillian "Patsy" Barbrey is the pinnacle that they all strive for. A fairly full life span with Recessive Dystrophic EB; most Butterfly Children die before their 20's. And the way she got there was a miracle wrought by my Grandmother.
A strong Tennesee mountain woman, Nana gave birth to this tiny, wounded child and was told by the country doctor to let her die. Instead, Nana nursed her for years, insisted that she live a normal life and backing down anyone who told her different, or treated her child as less that normal. As a result, Patsy grew up and did almost anything any other woman can. She learned to drive, she finished High School. She fell in love and married. She had 3 healthy children. No other EB woman had ever given birth until Aunt Pat. She faced the inevitable breakdown of her body with courage and died at 65.
I watched last night as my son wept on numerous occasions. He knew my Aunt well, and loved her unreservedly. But until last night, he never fully accepted the fact that he could also be a recessive carrier of the EB gene. Now he plans on being tested. If his genes are RDEB free, than he is home free. If he carries the gene, then his future wife will have to know and be tested herself. If she is free, wonderful! If not they will have some very heavy decisions to make as future parents.
So, like the butterfly across the globe that flaps it's wings and starts a hurricane on the other side of the planet, a small group of EB victims and the ones left behind have started flapping and creating their own storm.
I think their tornado of hope will only blow in good things. Strides are being made in Italy and other countries allowed to develope stem cell treatment and there is hope on the very distant horizon.
And I have faced a demon or two of my own, batted them down and have joined the fight. And that's a very liberating feeling. I feel free as bird right now...or, should I say...a Butterfly.
Views: 33
Comment
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Comment by Geoff McGeachin on May 24, 2008 at 11:54pm
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1124 words. And now I know so very much about EB and Butterfly Children and your aunt Pat and her mother and your family and most especially you. That’s the written word working at its best. I just wanted to check who the other person online was. Now I know. In just 1124 words. That’s writing. I think it may take me a little time to get to sleep tonight. My very best wishes.
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