In my last blog post, I introduced you to the plight of a mom and her son with epidermolysis bullosa (EB), through her powerful testimony at the Minnesota Capitol while advocating for stem cell research. Here is another except about how this disorder held her family hostage, and her never-ceasing tenacity to break free and provide a better quality-of-life for Charlie and the rest of her family.
By Trisha Knuth
“From the moment Charlie opened his eyes in the morning, he was afraid. Most mornings dressings had moved out of place and sheets and pajamas would stick to his wounds. Charlie’s anxiety caused behaviors that further injured his skin. He became his own worst enemy by picking and scratching his wound. His ears and forehead were so damaged that they had to be completely wrapped 24 hours a day with only his face peeking out. The rest of his body was entirely wrapped from head to toe.
Experiences that families are meant to enjoy together were almost impossible. There were simply not enough hours in a day to accomplish Charlie’s cares and make time for enjoyment. EB defined every aspect of Charlie’s life and the life of my family. When we did get chances to adventure out in to the community, the stress of the “unknowing” public sometimes was too much. “Was he burned?” “What happened?” “Look at that kid.” followed us where ever we went.
I could see the toll that this monster called EB was having on my family. Charlie’s life was consumed with anxiety. My life was consumed with caring for Charlie. My other three children barely had a mother. Not by choice, but because I had no choice. My husband’s life consisted of working 12 hours swing shifts and then coming home to a house of never ending laundry and cleaning. In desperation, I spent countless hours researching this disease when I should have been sleeping. I think most nights I averaged a good four hours of sleep if I was lucky.
In search of relief, I sought out numerous specialty doctors from numerous states and facilities. When they refused Charlie’s Medicaid (Charlie’s adoption was considered a “special needs” so he was covered by Medicaid), I simply paid cash. After hours of travel, money, and hotel stays, we never ever were given any valuable information from these doctors. Most of the time, Charlie was a physical specimen for a disease that had only been read about. We became their teachers and source of information instead. Over and over we were told that all we can do is try to prevent infection by dressing changes, bleach baths, and vinegar soaks. Many times they prescribed antibiotics to offer a short-term relief for a couple weeks to get me off of their backs. One doctor told me that maybe in Charlie’s lifetime there would be a treatment or cure, but as for now, there is nothing.
Then I heard about the pioneering EB research at the University of Minnesota, and the work of Dr. John Wagner and Dr. Jakub Tolar. My life took on a new mission. I had hope.”
Here is a message from D r. Jakub Tolar, who is leading the efforts to change outcomes for children with this disorder.
I am infected. My life and my job do not have have a starting and end point. They are co-mingled; woven through every fiber of my being.
I am affected by the people whom I meet: Each brave child battling a life-threatening illness; each mom trying to keep it all together; each sibling who is oft neglected; each dad who feels helpless that he couldn’t take the bullet himself and spare them all this anguish. Each have a story.
Sometimes I am absolutely rocked to my core by one of them. When I heard a mom testify in front of the media and our State Legislature (to stop a bill that threatened adult stem cell research), about her son’s plight with the most devastating of diseases, Epidermolysis Bullosa (EB), I knew that I would never be the same.
I am proud to now call this woman a friend. She is my hero, actually. She’s a bona fide angel if ever one existed. Here is an excerpt of this gut-wrenching testimony. In the near future, I will post Part II, an update on Charlie’s condition, and how you can help us write a happy ending to this story.
By Trisha Knuth
When Charlie was born in a small Wisconsin town, his infant body began to blister immediately. Skin slid off his body when nurses and doctors began the normal routine of handling a newly born infant. Days went by and no one came for him. He lay alone in the NICU slathered in Vaseline and wrapped with bulky gauze. My husband and I received the call from Social Services asking us if we would consider fostering a baby with Recessive Dystrophic Epidermolysis Bullosa, a rare disease that is a missing protein that binds his two layers of skin together, inside and outside the body. We were told to keep him comfortable and that he most likely would die in the first year of his life.
You can only imagine as his mother and primary caretaker how it felt to have to torture my own son for hours on end on a daily basis. You see, I had no choice. In order to prevent infection to the wounds that covered 95% of his body, similar to burns with nerve endings exposed to every sensation, baths spiked with vinegar or bleach were part of the daily regimen.
Removing his specialized dressings, which cost $15,000 a month, could only be done in the water because even though they were made not to adhere to skin, taking them off dry would take off sheets of Charlie’s skin in the process. The process of disinfecting, removing dressings, lancing tense blisters with sterile needles, applying creams, ointments, accessing wounds, and choosing appropriate dressings took upward of 5 hours a day.
The torture regimen that defined Charlie’s life was relentless. The psychological toll it took on me was obvious. Most of the time, I had to make myself numb to emotion because I knew if I cried that Charlie would cry harder. Some days, my strength was just not enough. I can recall a day when Charlie was 2-years -old and all the skin was missing from his chest. I could see that it was becoming infected from the constant deep itch that never subsided. Even though Charlie’s fingernails fell off in his first year of life, the constant rubbing caused infection and further breakdown of his skin. In order to heal his chest and prevent another hospital stay with IV antibiotics, I had to clean his chest with a combination of water and vinegar to kill the obvious pseudomonas that was taking over. Charlie was standing in the bathtub because he refused to sit down. I slowly poured the acid spiked water over his body. The scream could be heard for blocks. And then the scream was so intense that it turned silent. I can envision what his face; his body looked like even now. Shaking, red, and convulsing. The pain, so intense caused Charlie to madly ravage his body. Ripping skin off his face, ears, groin, and chest. Blood dripping in the bath and covering me as I tried desperately to console him. I can remember that I could just not contain myself and as I tried to hug his ravaged body I myself began to cry. Can you believe that in this moment, a two-year-old child began to comfort me? “Mama don’t cry, I’m OK.” It’s OK Mama.” and we held each other that way, with the blood, the missing skin, the infection and all.
That moment will be stamped in my brain forever, but this moment would be repeated in our lives numerous, numerous times.