Epidermolysis Bullosa affects many people in varying degrees of severity, while posing a challenge for families in terms of medical and financial support. ‘Butterflies, Purple Tulips & U2’ tells the story of Aimee, one such individual who never stopped fighting.
Imagine...
a child with painful wounds similar to burns covering most of his or her body.
having to wrap each tiny little infant finger with Vaseline gauze and then cover it with gauze to prevent the hand from scarring, webbing and contracting.
never being able to hold your child tight because if you did, their skin would blister or shear off.
a child who will never know what it's like to run, skip or jump, or to play games with other children because even the slightest physical contact will injure his or her skin.
a child who screams out each time it is bathed because the water touching its open wounds creates incredible pain.a diet of only liquids or soft foods because blistering and scarring occur in the esophagus.
an active baby with his knees soaked in blood from the normal act of crawling.
a teenager with stumps for hands, the affected fingers scarred/healed together.
These are the day-to-day battles that face those suffering from Epidermolysis Bullosa (EB).
Epidermolysis Bullosa is a rare genetic skin disease that is marked by the extreme fragility of the skin and the recurrence of blister formations caused by minor friction or trauma. Individuals born with the disease lack the ‘anchors’ that keep the two layers of the skin – the epidermis (outer layer) and the dermis (inner layer) – from moving independently from one another. Since the two skin layers are not held together, any minor friction between the layers, from rubbing or pressure, will cause sores and painful blisters, which people with EB liken to third-degree burns.
It is estimated that in 1 million live births, 50 are diagnosed with Epidermolysis Bullosa, though the disease is not always evident at birth. Approximately 2 to 4 out of every 100,000 – or up to 12,000 – people in the US have some form of EB. The disease is indiscriminate as it occurs in all ethnic and racial groups, and equally affects males and females.
The disease becomes more evident as a child begins to crawl, walk and run, or as a young adult gets more involved in physical activities. When the disease becomes full-blown, people with EB are faced with the challenge of protecting their skin from any form of friction.
This prevents children from enjoying simple physical activities like running, skipping, jumping and playing with other kids, as any minor pressure on the skin can cause blisters. In some cases, even the simple act of a parent carrying or hugging a child can cause wounds on the skin or, at worst, the shearing off of skin. Parents of EB-diagnosed children have managed the disease by preventing and protecting wounds of their kids by covering their skin with gauze and ointments.
Because it is such a rare condition, families of EB patients are hard-pressed to find doctors and medical practitioners who are knowledgeable in handling and treating individuals with this disease. Another challenge attached to this medical condition is the fact that most health maintenance organizations do not cover the healthcare expenditures of EB patients who have the medical necessity to bandage wounds and protect healthy skin from harm. This leaves families with health maintenance costs that reach up to several thousand dollars in a month.
One case of an EB patient and the challenge faced by her family is presented in the Xlibris book ‘Butterflies, Purple Tulips and U2’. Author Sandra Silveira describes the dilemmas she and her family faced in trying to find support for her daughter, Aimee, who had lived with Epidermolysis Bullosa.
After her daughter lost the battle with the rare skin disease, Silveira decided to honor the memory of Aimee’s life by sharing their experiences in managing the disease and how it impacted their lives as a family. Silveira hopes that people suffering from EB or have loved ones battling the disease will find Aimee’s story inspiring and heart-warming. Silveira describes Aimee as a remarkable young woman; who rarely ever complained and worked hard at balancing her disease and the life she loved. She would want people to remember her accomplishments and her enjoyment in each day. She had great faith and loved those around her and in return was loved by many.
Research continues into EB in an attempt to find a cure, scientists at the University of Minnesota have found indications that a bone marrow transplant may be a solution to finding a cure for EB. Current research efforts are focused on the area of genetics and how gene mutations, gene therapy and gene defects contribute to the disease and to a possible cure for the disease. Research assessing the effectiveness of using proteins called cytokines and new kinds of dressings to heal blister wounds are also being conducted.
On another front, families of children and individuals with Epidermolysis Bullosa have come up with a petition for a Wound Care Bill that would make financial support available to EB affected persons in every state. Once supported by Congress, the Wound Care Bill could mandate insurance companies and HMOs to provide coverage for expenses related to the care and treatment of people who suffer from EB.
To support the ongoing fight against EB and to be a part of finding a medical and financial solution for those who deal with this disease everyday, visit http://ebanusa.org/petition.htm or http://www.debra.org/index.php and help improve the quality of life of EB sufferers.
Imagine...
a child with painful wounds similar to burns covering most of his or her body.
having to wrap each tiny little infant finger with Vaseline gauze and then cover it with gauze to prevent the hand from scarring, webbing and contracting.
never being able to hold your child tight because if you did, their skin would blister or shear off.
a child who will never know what it's like to run, skip or jump, or to play games with other children because even the slightest physical contact will injure his or her skin.
a child who screams out each time it is bathed because the water touching its open wounds creates incredible pain.a diet of only liquids or soft foods because blistering and scarring occur in the esophagus.
an active baby with his knees soaked in blood from the normal act of crawling.
a teenager with stumps for hands, the affected fingers scarred/healed together.
These are the day-to-day battles that face those suffering from Epidermolysis Bullosa (EB).
Epidermolysis Bullosa is a rare genetic skin disease that is marked by the extreme fragility of the skin and the recurrence of blister formations caused by minor friction or trauma. Individuals born with the disease lack the ‘anchors’ that keep the two layers of the skin – the epidermis (outer layer) and the dermis (inner layer) – from moving independently from one another. Since the two skin layers are not held together, any minor friction between the layers, from rubbing or pressure, will cause sores and painful blisters, which people with EB liken to third-degree burns.
It is estimated that in 1 million live births, 50 are diagnosed with Epidermolysis Bullosa, though the disease is not always evident at birth. Approximately 2 to 4 out of every 100,000 – or up to 12,000 – people in the US have some form of EB. The disease is indiscriminate as it occurs in all ethnic and racial groups, and equally affects males and females.
The disease becomes more evident as a child begins to crawl, walk and run, or as a young adult gets more involved in physical activities. When the disease becomes full-blown, people with EB are faced with the challenge of protecting their skin from any form of friction.
This prevents children from enjoying simple physical activities like running, skipping, jumping and playing with other kids, as any minor pressure on the skin can cause blisters. In some cases, even the simple act of a parent carrying or hugging a child can cause wounds on the skin or, at worst, the shearing off of skin. Parents of EB-diagnosed children have managed the disease by preventing and protecting wounds of their kids by covering their skin with gauze and ointments.
Because it is such a rare condition, families of EB patients are hard-pressed to find doctors and medical practitioners who are knowledgeable in handling and treating individuals with this disease. Another challenge attached to this medical condition is the fact that most health maintenance organizations do not cover the healthcare expenditures of EB patients who have the medical necessity to bandage wounds and protect healthy skin from harm. This leaves families with health maintenance costs that reach up to several thousand dollars in a month.
One case of an EB patient and the challenge faced by her family is presented in the Xlibris book ‘Butterflies, Purple Tulips and U2’. Author Sandra Silveira describes the dilemmas she and her family faced in trying to find support for her daughter, Aimee, who had lived with Epidermolysis Bullosa.
After her daughter lost the battle with the rare skin disease, Silveira decided to honor the memory of Aimee’s life by sharing their experiences in managing the disease and how it impacted their lives as a family. Silveira hopes that people suffering from EB or have loved ones battling the disease will find Aimee’s story inspiring and heart-warming. Silveira describes Aimee as a remarkable young woman; who rarely ever complained and worked hard at balancing her disease and the life she loved. She would want people to remember her accomplishments and her enjoyment in each day. She had great faith and loved those around her and in return was loved by many.
Research continues into EB in an attempt to find a cure, scientists at the University of Minnesota have found indications that a bone marrow transplant may be a solution to finding a cure for EB. Current research efforts are focused on the area of genetics and how gene mutations, gene therapy and gene defects contribute to the disease and to a possible cure for the disease. Research assessing the effectiveness of using proteins called cytokines and new kinds of dressings to heal blister wounds are also being conducted.
On another front, families of children and individuals with Epidermolysis Bullosa have come up with a petition for a Wound Care Bill that would make financial support available to EB affected persons in every state. Once supported by Congress, the Wound Care Bill could mandate insurance companies and HMOs to provide coverage for expenses related to the care and treatment of people who suffer from EB.
To support the ongoing fight against EB and to be a part of finding a medical and financial solution for those who deal with this disease everyday, visit http://ebanusa.org/petition.htm or http://www.debra.org/index.php and help improve the quality of life of EB sufferers.
