Charlie Knuth's Journal
Written Sep 21, 2012 9:55pmToday we met with Dr. Tolar, Charlie's brilliant doctor at the University of Minnesota. This is the first time we have spoken since Charlie's bone marrow biopsy last week Tuesday. Charlie is 97 percent donor. This number can not get much better than that. But, his white blood count and absolute neutrophil count is very low at this time. This is what is called the "60 day slump." The first set of cells that produced so quickly had alot to do with the GCSF ( a medication given to boost white blood cells) that is given immediately after transplant. Cells only live for so long in the body and then new ones produce. Now it is Charlie's job to start producing those cells again on his own. That starts right about now...We are watching very closely to make sure that Charlie does that. He is very prone to infection until this happens Charlie's skin is looking incredible. Dr. Tolar has still not seen any collagen 7 in his skin biopsies. This is the gene that he is missing. This is the gene that holds the two layers of skin together. So, with that said....the amazing transfomation of Charlie's skin is a mystery to me. What is causing his skin to become so much stronger? There is so much yet to learn about this horrible genetic disorder. Before this clinical trial, before Dr Wagner, Dr Tolar, and before I had hope, I spent hours and hours researching wound care. Every night I searched for "high tech" dressings and bandages, medications, nutrition, homeopathy, natural remedies, organic clothing, seamless clothing, ointments, creams, materials, hell, I even studied hypnotic therapy to try and ease his pain. I think about the children with EB. The children who live with the "severe forms" of EB. Recessive Dystrophic Epidermolysis Bullosa and Junctional EB. The children who live to be adults and suffer so much agony. I have decided that I am no longer wasting anymore time or money on "the best bandage." I do not want to "cover-up" or put a "bandage" on the issue. Charlie doesn't need another bandage. Charlie needs a CURE. Bandages and ointments are not going to stop his hands from curling and contracting. You can not put a bandage in a closed off esophagus or a blistered eyeball. Bandages can not repair a ravaged stomach, a perforated bowel, or repair the inability to absorb the nutrients essential for healing in the first place. The mental damage that this has caused my little boy's soul can never be repaired. I am focusing on the "big picture." I want a CURE for EB. I know where "The Cure" is going to come from. That is why everyone in "The Lab" at the University of Minnesota were delivered Nadia's Cupcakes on Tuesday. (from Charlie of course). Thank you. Thank you. Thank you to the researchers in "The Lab" who work on EB everyday. Without you, Charlie would not be alive today. Without you, there would be no hope. Charlie appreciates what you do for him and for the other children with severe EB.For more information on how you can help Charlie and other children suffering from EB - http://www.puckfund.org/
Written Sep 12, 2012 9:06pmThe first time Charlie went in to an operating room he was only 1 1/2 years old. At that time, I was to early in the "EB Journey of Hell" to know that I had rights as Charlie's mother to choose how I wanted much of his care done. I forgot that the healthcare providers are providing a service to my child. I didn't realize at that point I had a lot of "say" in Charlie's care. That first experience in the OR started the "over bearing, controlling, know it all, helicopter mom "reputation" I am sure I earned at many health care facilities. You see, that day Charlie was carried back in to the OR by a stranger who had no idea how to hold a child with EB. Charlie was screaming. I remember going into the public restroom, shutting the bathroom door, and shaking and crying. When Charlie's procedure was done and I was "allowed" to come to the recovery room....Charlie was missing all the skin on his chin. The anesthesiologist took all his skin off with the "gas mask" as he held it to Charlie's face. Can you believe that Charlie can remember this? Charlie was very tolerant of narcotics at an early age. They had not sedated him entirely. He can retell in detail how he was moved onto a "hard" skinny table and that it hurt his back. The next experience is the one that really hits a nerve like no other. Charlie had a blood infection from strep. His skin was in horrble shape and his pain was excruciating. The hospital decided that it would be "best" if Charlie had a dressing change done in the OR under sedation. Great, I would love to do that. Anything to reduce his pain. That's when a doctor resembling Dr. Oz came in and told me that "HE was the burn Doctor, and that HE, HIMSELF would be doing the dressing change. This type of procedure required a professional of his qualifications. ( a mother would surely not have such skill.) I was to wait in the waitng room and they would get me when they were through. Well, they called me back to the recovery room where Charlie was sitting on a bed screaming and about 5 nurses were scrambling around the bed. Dressings were falling off, wounds were exposed, IV hubs and tubing were laying all over Charlie's exposed skin and rubbing it right off. I had to "redress" his entire body right there in the recovery room. The doctors had sedated him for nothng, only to cause him more damage, more pain, more trauma, and one very angry mother.....Masks have been taped to his face and ripped off taking a "stripe of skin" with it on each cheek, gloves have pulled sheets of skin from his back when he was transferred from one bed to another, gloved hands have blistered his hands and arms looking for veins, armpits have been de-skinned by thermometer probes being pulled out, and sheets of skin pulled from Charlie's chest when a radiologist thought that he would remove Charlie's dressings himself and pulled of his top layer in the process. That same doctor took all the skin off Charlie's arm from his forearm up to his armpit. He reassured me by saying, "oh this, you mean his arm? That is the least of his problems." So this is why I do everything...I don't care if someone doesn't like me, or thinks I am controlling, or thinks it goes against their "policy." It's my "policy" to prevent pain and suffering to Charlie at all costs. I have been battling this policy for a long time. I completely understand why parents should not be allowed in surgical procedures of their children. It makes perfect sense. Can you imagine if they had to let every parent in the OR just because ONE "has to be right, has to do it herself, has to control at least part of this horrible disease that is so unfair and so incredibly uncontrollable" mother demanded that she be present in the OR? So yesterday, for the first time....I had to step out. Policy changed. I had no one left to fight on this. They won the battle. Guess what? Not a blister...Not even a red mark. I was able to put Charlie on the OR table myself, I was able to attach all the monitoring devices, 02 mask, and take down all his dressings. Once Charlie was asleep...out the door I went. I was accompanied with a nurse who carried a cell phone and we sat in a room right outside the OR. Everytime Charlie needed to be moved or bandaged in any way they would call the cell phone and I would go back in to the OR. When the skin biopsy, bone marrow biopsy, dilitation, and pictures were taken I was called to come back in and re-dress the rest of Charlie. I was then able to put him onto another bed and hover over him until he was awake and ready to go home. It worked and I am relieved. I appreciate the doctors and nurses who put up with me and other parents like me. I can not bear to see Charlie go through anymore unnecessary pain. Especially because of a health care professional has an "ego." I wish every doctor in the world would read this and understand. Read it to your medical students.I am still waiting on results from the bone marrow biopsy. I was hoping to see something today but I haven't. So we wait and hope and pray. Tomorrow for sure...( I hope)
Written Sep 6, 2012 8:47pmNothing can prepare a person for this emotional roller coaster. I updated Charlie's condition just a few days ago. I was so relieved that he was doing so well. All his labs looked absolutely fabulous and they were holding steady. Perfect! Then Tuesday he had another routine appointment and blood work. His white blood count and ANC plummeted. We went back yesterday for another lab just to see if his White blood count recovered and it had gotten lower yet. Charlie had to be infused with GCSF to give his white blood count the boost it needed. He also needed to get a blood transfusion to boost his hemoglobin. He has not needed a transfusion since he was released from the hospital. Again today he had another lab draw to check if his WBC and ANC count had gone back up. It hadn't....he needed another GCSF infusion. How does this happen? How does everything go so well and so consistant and then out of the blue just go hay wire? We meet with Dr. Tolar tomorrow. I hope to God that his labs are better tomorrow. The emotional toll this process takes on a parent is relentless. Just when you think you can't take anymore and finally you can breath with relief something happens to throw you right back in to the hell of waiting for answers, worrying, and cherishing every possible moment with your baby. Today I told Charlie about a Cupcake shop that is in Maple Grove. The lady that owns it won "Cupcake Wars" on the Food Network. Charlie told me that he wanted to go there. The only problem was that it was raining and it was 5 o clock rush hour in Minneapolis. Charlie didn't care about that. He wanted to go anyway. Under normal circumstances I would have said that we could just wait until tomorrow. But....all I could think about was how I would feel if Charlie had to be admitted to the hospital tomorrow. How I would feel if I never got the chance to take him to get his cupcake. Imagine. Try to imagine what those thoughts feel like....Charlie chose a Red Velvet Cupcake. He took one taste of the frosting and didn't want any more.