Monday, January 25, 2010
Well, sleep is coming back to the house... at long last, thanks to a wonderful breakthrough.
When we were heading to MN, we couldn't think of how we were going to wash Bella's diapers as the hotel did not have laundry services. The nearest laundromat was 2 miles away. We thought, "Man, driving in MN and sitting at a laundromat in -10 degrees is gonna be pretty lame." So, we decided to check out if there were any soft disposable diapers that maybe she could tolerate for a couple of days. Ang found a brand that seemed a little softer than most, and we went for it. Well, sure enough, they were just the ticket! However, it was good we got home when we did and got back to our fuzzibuns because her skin was starting to break down from the disposables.
Anyhow, one of Bella's problems is that she soaks through her fuzzibuns at night and this causes her to wake up. Thus, no one gets a full night's sleep.
We thought, "Hmm... wonder if she could tolerate those 12 hour extra absorbent disposables over night." So we picked up a small batch of them and voila! They work! They keep her dry through the night, she sleeps through the night, and they don't cause any collateral damage... which means... (if Ali cooperates and sleeps through the night herself)...
we sleep through the night!
Now before you get all happy, you need to know Ali hardly ever sleeps through the night anymore. Really too bad, too, because she used to sleep through the night like a champ from the time she was 9 weeks old. Oh well, one step forward, one step back.
Outside of that, things are good. Bella saw a registered dietician at CHOC today and the results were great. She is putting on weight like a champ still. She still gets 36 ounces of formula a day with a little baby food thrown in there for fun. The formula is the main source of calories, and her GI tract seems to absorb and convert the formula well. The RD said she has another EB child she sees. That baby was taking in 50 OUNCES a day at two months, and never got as big as Bella. It goes to show the real sinister part of RDEB: no matter how well you manage external wound care, you can't see or manage how well the GI tract works past the stomach. All you can do is keep getting weighed in every 30-60 days to make sure everything is still working internally. We'll see Vanessa again in March, then again in May before we head to MN (or NYC).
In other news, Bella's book is almost off to the printer! It's officially out of our hands now. It's being formatted one final time before it goes to lulu.com. Almost there! We'll be giving away the book as an eBook, and selling a paperback version for $19.95. It costs us $9.95 per copy to produce, so we'll clear an even ten bucks per copy. Each month, we'll be giving between 20-50% of the proceeds to a different non-profit organization either funding EB research, care, or care for children through music therapy. We are really excited to get the chance to pay it forward. We have been INUNDATED with love and support since the day Bella arrived. It will feel great to give back a little.
We'll keep you posted as to where and when you can get your hands on it!
Blessings to you all.
Wednesday, January 20, 2010
Sorry, it's been busy around here. Angelique is currently in Texas on business; I leave for Vegas on business Friday, we both head to NYC with Bella on 2/2... not to mention I am g-r-i-n-d-i-n-g through the last of what I can edit before I turn Bella's Blessings: A Humble Story of Providence over to an editor for the final (cross your fingers) draft to be uploaded to lulu.com for printing! I give up; I've gone as far as I can with the editing. I am stuck 5 problems that I cannot find the answers to despite days and weeks of trying on my own. Really, I have no business taking the editing as far as I did. It would have been more efficient to hand it off sooner and pay my editor, but I have more time than money in my pocket right now so... I thought it would be romantic to take the book all the way through the creation process myself... you know, like delivering a baby with no pain meds... Well, I've been in labor for weeks (actually months) now and I'm ready for the epidural! Give it to me!
Okay, okay, enough about all that...
First up: TRANSPLANT NEWS:
We have a consultation on Feb. 3 with Dr.s Chairo and Christiano in New York City. We fly in on 2/2 and out on 2/4. We're very excited to return to manhattan; it's been a few years. We also can't wait to hear what is so special about their experiment and why it would be better than going to MN. We spoke via email with Dr. Wagner just this week and let him know we were headed to NYC and he thought that was a great idea. We also penciled Bella in for transplant in MN for June 1. That way, at least we're in the queue in case Columbia fails to win us over. We feel very confident in Dr. Wagner and the entire tx team there in MN. We're going to wait till June 1 because Bella's first birthday is May 27, and we want to celebrate it here in Orange County with so many that helped her (and us) make it this far. We would then be there until mid October most likely.
Second: WOUND CARE NEWS:
Bella is really struggling with itching. She has open wounds on her left knee, shin, ankle, heel, foot, and arch right now, and they are driving her NUTS. She GRINDS her legs together in an effort to scratch the itch and in the process, is giving herself more blisters on her right leg as well as exacerbating the left. Worst off, she does it IN HER SLEEP. We do the best we can wrapping and isolating the legs from each other, but you can't stand over her 24/7 and hold her legs apart.
Angelique has developed a BREAKTHROUGH in the A-B-C hand wrap for EB kids! We're gonna make a 2 minute video and upload it to youtube soon enough. Bella had been falling behind in her fine motor skills because her hands were so wrapped up. When we went to the Early Development Assessment Center, the PT asked if there was a way for us not to wrap the palm of her hand so much. It was the gauze criss-crossing the palm that was preventing Bella from grasping and gripping. So, Ang figured out a way to wrap the hand virtually the same way, but with the palm wide open, save for 1 single 1" gauze strip. Well, the results have been nothing short of AMAZING. Bella's ability to grip, grasp, and even pinch have exponentially skyrocketed from before the EDAC Consultation! She almost pulled my plate off the table recently at dinner, and she reaches for EVERYTHING now! It's as if she knows she CAN hold something, so she WANTS TO. It is so exciting watching her play with toys, and transfer them from hand to hand. I remember the disappointment in our Pediatrician's voice at her 6 month appt, when he asked if she was transferring toys from hand to hand and I said, "No." "Hmmm." was the reply as he checked off some box on his little clip board. Well Dr. Bindra, ASK ME NOW, ASK ME NOW!
In other news, Bella has been awarded PATIENT OF THE YEAR from Molnlycke Healthcare, the manufacturer of most of Bella's wound care supplies! Crazy, huh? We got the call from the Territory Sales Manager that Molnlycke is having their annual sales conference/meeting in Phoenix in late Feb, and they would like to fly the family out there, put us up in a hotel, and do a 30 minute presentation along with Betty, our PT from CHOC ( Who we LOVE and can't wait to hang with again). Then, they're going to give us some sort of Honorarium to help with her ongoing health care costs! Pretty neat, huh? We're also talking about them buying 100 copies of Bella's book for all the attendees... say a prayer that works out, and that I get the book done in time! We are totally blown away by this generous gesture, and appreciate the opportunity to share our story. GOD IS GOOD! ALL THE TIME!
Anyhow, there is so much to be grateful for in this new year, despite the all the suffering and strife that co-exists, from our own wound care table, to the world at large.
Is it ever any different?
Thanks for being with us. This journey wouldn't be the same without you.
Saturday, January 9, 2010
Well, we are back home from snowy, cold Minneapolis. We had a BLAST! First off, after you've lived in extreme temperatures ( like Phoenix ), extreme cold isn't any different than extreme hot. You know as you are walking out the door that you are about to be slapped with a temperature your body won't like and can't tolerate for very long without being properly prepared. The nice thing about the cold is you can bundle up. In the desert, you can't "bundle down" to cool off, you just dehydrate and burn to a crisp. However, in the desert, you don't have to shovel heat, either.
okay, enough about the weather.
On to... the GOOD STUFF...
We met with the good Doctor John Wagner. Here is his title off his business card:
Director, Pediatric Hematology/Oncology/ Blood and Marrow Transplantation
Scientific Director of Clinical Research, Blood and
Marrow Transplantation and Stem Cell Institute
The University of Minnesota has been doing Bone Marrow Transplants (BMT) since the sixties. They are the leader in the field, and he's the head hancho. His Curriculum Vitae is quite impressive, yet, he doesn't have the ego that you would expect. He's a researcher, and a teacher. So, when he explained what he is accomplishing in this clinical trial, I could see the wheels spinning in his head. He has a child-like passion for what he does. What does he do? In his words, "I cure incurable diseases."
So, we spent 2 hours together where he presented his results up to date, then answered all our questions. I had two pages of questions for him, but admittedly, when it came time for Q&A, he had already answered most of my questions in his presentation.
We talked about so much, it's honestly hard to recall specifics. Also, I can't talk about the results of the research as it is currently being peer reviewed for possible publication. What I can say is that we feel extremely confident in him, and in the U of M staff. He has broken into uncharted territory with his research. Don't go around using the word "cure" as it would not be appropriate at all, but you can say that his results have never been done before with an EB patient.
We told him we were going to Columbia U. to discuss their "reduced intensity" BMT clinical trial as well. He gave us a few questions to ask and shared his honest thoughts about their multi-site trial that has just gotten rolling. Columbia will get their moment in court to "sell" us on why their trial is better, but Dr. Wagner is really the guy they are trying to imitate. He knows more about BMT for EB than anyone on the planet, so they have their work cut out for them to convince us to go to NYC instead of MN. Having said that, we're adamant about going and doing our due diligence. Leave no stone unturned.
Metaphorically, I wish there was another way to rebuild the engine without having to strip it down so far. However, when Dr. Wagner explained that they have successfully done BMT on FETUSES IN THE WOMB because you can be BORN with cancer, I realized that it's simply a point on a line... a line of treatment where researchers are looking for new, less invasive ways to treat these diseases, but have yet to make the breakthrough that will ultimately leave this treatment in the history books. So many other breakthroughs HAVE occurred in so many areas, it is simply a matter of time. However, here we are NOW, and not 5, 10, or 20 years from now. Lucky for us we ARE here now, not 5, 10, 20 years ago, when there was NO chance for an RDEB kid to have a shot at living a "normal" life.
The trip was especially fun because we got to have dinner with one of my childhood friends, Logan Spector, who is a pediatric epidemiologist at U of M. He's been there for almost 10 years now and knows and works with Dr. Wagner. He invited us to his home and we met his wonderful wife and two children. We also met Lonni Mooreland, who is the mom of patient #2 of the trial. Unfortunately, little Sara passed away due to complications from a weak heart; a condition she had since shortly after birth. She and her husband, Jay, are the ones who have started PUCK, a charity to raise money for Dr. Wagner. www.puckfund.org. Check it out. We will be partnering with PUCK with the release of Bella's book in the very near future. Anyhow, It was nice to connect Lonni and Logan since they both work with Dr. Wagner. Also, Lonni and Jay have moved to MN recently, so it's always nice to know someone new in town. The most tender moment of the night was when Lonni was leaving.
She said, "Thank you for inviting me, Tim. I was surprised you even wanted to meet me."
It dawned on me that she felt that we wouldn't want to meet her since her daughter died in the same study we are considering our daughter for. Somehow, it might scare us or remind us of the danger of the study. What I am reminded of is the sad fact that ANOTHER EB child passed away last week, bringing the total to 4 since December 1st. This disease claims its patients in far more ways than through experiments to find a cure.
I couldn't believe what Lonni said; that's the last thing I would think. Thanks to working on the Grief and Loss Retreat with my colleague Mauro and my Mom, I was able to quickly share with her how tough the grieving process is in our "afraid-of-death" culture. I was able to validate her experience and reinforce that the insane feelings she is having are in fact normal. It is our "feel-good-all-the-time-at-all-costs" culture that is totally insane. We are so completely cut off from the experience of death that we don't have a clue how to handle it.
She just looked at me, a little incredulously, as if to say, "You actually GET IT." Then, she said, "Thank you, I feel like I had some therapy tonight!"
By the way, U of M mascot is called "The Gopher." Wanna know why? Because just about the entire campus is connected by above ground skyways and underground tunnels. The farthest we walked outside the whole time was from the car to Logan's front door. No lie. Applebee's delivered breakfast to our door each morning. Starbucks was located in the lobby, and the hotel was connected to the medical center. Add Valet parking, and we hardly felt the sting of the cold. I did leave my Starbucks in the car Wednesday night by accident and here's what it looked like the next morning...
Ice coffee, anyone?
Anyhow. We feel great about U of M. If this is our path, it will probably happen this spring/summer. We don't want to wait. EB is too dangerous. Also, the younger the patient, the better the chances. This is our year! We know there will be much in store for us, but we genuinely had a good feeling up there in Minneapolis; a feeling that said, "We can do this."
Thanks for being there with us. We felt you.
Thanks to Grandma and Grandpa for watching Ali and Lucy as well as for donating their miles for plane tickets.
Thank you to Aunt Marie and Uncle Tom for their INCREDIBLE contribution.
Thanks to our friends at Harbor Christian Church for their generous contributions as well.
Thank you, God, for delivering us to and from MN right in between nasty storms. Thank you for Dr. Wagner, and thank you in advance for Betty Ann, the financial counselor at U of M, who will be getting full inpatient and outpatient approval for us from our new insurance provider (we pray!)
Friends, Family, Faith.
Always remember the Three F's. We are so blessed to be lifted up by all three simultaneously. That is not always the case in life. You are blessed to remember to lean on any one at a time, but three, we are very blessed, indeed!
God Bless You All,
Tuesday, January 5, 2010
Pack your parka, baby! Oh - wait - we don't own parkas living in Southern California!
We are packed and hanging out at grandma's house, ready to hop on a plane first thing in the morning. We head off to Minneapolis by way of Chicago for a consultation with Dr. Wagner at the University of Minnesota Bone Marrow Transplant Center. Check out this Providence Story of the day...
We had been planning on staying at the Ronald MacDonald House in Minneapolis, but the way the RMH works is you get your name on a list, then call the day before to see if there is room. Well, I called today and they informed me there was no room at the inn. So, I remembered that in one particular email from U of M, there was a link for accommodations. I called the phone number, and a kindly gentlemen gave me the info on two hotels that give a discount for patients and their families. One is the Radisson University Hotel. Turns out the Radisson is closer to the building we will be going in on campus than RMH. RMH is a 6 block walk, while the Radisson is diagonally across the intersection from the building we'll be in! Then, while on the phone with my childhood friend Logan (who works at the medical center), he explained that there is a tunnel that connects the two buildings! We don't have to even go outside to go to the Doctor appointment now that there is no room at RMH!!! Grandma and grandpa informed us today that the temperature in Minneapolis today was -9 degrees. 6 blocks in -9? NO THANK YOU!!!! Boy I am GRATEFUL there was no room at Ronald MacDonald!
For a review, we are going to consult with Dr. Wagner regarding a clinical trial at U of M for kids with RDEB. They are using stem cells via bone marrow transplant to grow healthy skin on the patient. The trial involves a 100 day stay up there. 7 children have undergone the procedure, 5 made it all the way through. The other two passed away. Bone Marrow Transplant (BMT) is an incredibly intense procedure with a 40% chance of lethality. Most people who are considering a BMT are cancer patients for whom the main 3 treatments aren't working i.e. radiation, chemo, and or surgery, and it is usually a last resort. 3 EB kids have passed away since Christmas Eve alone, and since there currently is no cure for EB, BMT ultimately comes into the equation.
There are pros and cons to going through this procedure. The obvious pro is that if it works, Bella would grow Ali's healthy skin on her body, thereby reducing the chance of her succumbing to this bizarre condition. The big con is that Bella could die from the procedure itself. Also, the research team is tinkering with their research model with each patient. So, you can't look at any of the prior patients as a baseline for what to expect. Every patient is essentially n=1 because to my knowledge, no two patients have undergone the exact same procedure. Combine this with the uniqueness of each case of EB, and you have a pickle for anyone who wants to do good, rigorous research.
We knew that Columbia University has just started a reduced intensity BMT for RDEB patients, but word came out recently that a handful of other facilities are beginning clinical trials for reduced intensity BMT as well. We are planning on going to Columbia to meet with their team, and we'll see about the others. Because Bella's severity has been called "mild" by two within the EB treatment world, the full intensity of a BMT may not be worth the risk associated. We'll see. Anyhow, we're planning on going to NYC in February to shop for the best fit for Bella.
We want to hear from you! We miss the interaction. We're almost done with edits on Bella's book, and one of the great parts of it are alllllll the amazing comments you left us in the first few months. Let us know how you are and that you're still here with us. You are 2/3 of our support (Friends, Family, and Faith), and we are grateful to have you. You'll be with us as we travel to find the best care for Anabella that's available.
May 2010 bring you all of your dreams and wishes!
Blessings, and good night.