Happy Father's Day

Happy Father's Day to all you fathers out there!

Bella blessed me by SLEEPING IN this morning. Not only did she sleep through the night, but she slept right up to her 8:30 feeding. But her gift was when she started cooing this morning in her cradle. The only noises we usually hear from her are shouts, screams, and cries, but this morning, we got a little gift. I wish I could have video'd it. I'd watch it over and over again to remind myself there is a happy soul inside that angry body right now.

Blisters continue to heel, though a couple have returned exactly in the shape they had just finished healing. Bella has a new one on the back of her right middle finger that JUST healed...and now the whole process starts anew. Another three weeks of wrapping. However, Bella's face and mouth look better than EVER, and she EATS like there's no tomorrow. This is very important for an RDEB baby. Malnutrition is one of the big killers.

My thoughts on Father's Day take me back to my little country church, St. Patrick Church in Redding, CT. Every Father's Day, our Pastor, Fr. John Conlisk, would give his annual Father's Day sermon. I'll never forget his last line, "...because while any man can be a father, not every man is a daddy." (or something to that effect) For me, while yes I am a "father," next to being a husband, being a daddy is the best job of my life. The fun, the connection, the sense of purpose, the every day miracles...watching them develop and unfold before your very eyes...the wonder and awe of it all...awesome.

I also think about Father in Heaven today and the unending love, patience, abundance, forgiveness, and guidance He provides to all who are willing to listen no matter how many times they have ignored. I think about my own finite patience, and I marvel at His ability to endure all and still shower love and abundance with joy upon all who ask for it. God is my hero.

Growing up, I remember stories about "Manna from Heaven" and thought that God gave us gifts right out of the sky. Since then, I've learned to recognize that God gives us gifts through each other, through every day faces and people. There is no choir of angels announcing the arrival of more of God's gifts. Just a phone call, or an email, or a chance meeting in public, or a card in the mail. We are being fed (literally) by so many different people, including family, friends, our entire church, and Ang's work team, that we barely have enough room in the fridge and won't need to make a dinner (or lunch) for the next month. THAT is MANNA FROM HEAVEN.

Watch Bella's video: http://www.youtube.com/watch?v=VZ9MXSCfMzw

Purchase her song or make a donation above.

God bless each of you for your love and support. You are healing our daughter. Thank you.

A Normal Day

Family gets up. Daddy goes off to work and drops 3 year old at daycare while mommy stays home with newborn.

Could have been any family today. It just happened to be ours. With the exception of an in home assessment from the Regional Center, it was a mild, dressing-change-free day. Mommy, daddy, and Bella surprised Ali at daycare. You should have seen the pride and joy on Ali's face when she introduced everyone to her little sister. We went to the grocery store together, ate dinner together, then sat on the bed and watched Madagascar 2 with big bowls of popcorn together. Awesome.

It's nice to have a day with no wind, good or bad.

Here's the worst of it:

Watching the velcro on Bella's disposable diapers poke holes in her thighs is killing us. We're going to fuzzibunz.
We're also changing our diet around somewhat to give Bella the healthiest milk we possibly can. That's funny. I said we. I don't give Bella any milk! ANGELIQUE is changing her diet somewhat. We're gonna be hippies before we know it. She used organic hemp oil in her homemade salad dressing today! See! It's already begun.

We've had close to 50 messages from the past two posts. We can't begin to tell you how much strength and power that provides us. Even if we don't respond directly, again, each and every one of you is noticed and appreciated. THANK YOU.

Now, enjoy your weekend. Tomorrow, we get Ali new pool toys and it's off to the pool for the next 2 days!

Blessings to all of you. Good night.

Well, What a Difference a Day Makes

Leslie Brader, the DebRA Volunteer New Family Advocate, came to our home today. Man, she was AWESOME. She is a EB mommy as well. Unfortunately, she lost her child to Junctional Herlitz EB when the child was only 7 months old. Now, 5 years later, she holds an annual fundraiser in her daughter's memory to raise money so she can fly around the country and meet all the new EB families (like ours) and equip them with as much knowledge and tools as possible. Pretty awesome. She brought all kinds of the latest wound care products, cloth diapers (if you haven't seen one of these lately, go to www.fuzzybunz.com...they're not your parents cloth diapers), et cetera, as well as showed us a great EB family website that has all kinds of great forums for EB families. She was with us from 10 till 4:30, helped feed Bella and gave us some GREAT new tips on dressing changes. I think that went even quicker today. The best part was, after surveying Bella's wounds, Leslie told us to stop dressing daily and move to every 48 hours.

That's a 50% reduction in weekly wound care, folks! We were so excited to hear that as wound care is definitely the high stress point of the day. The products we are using are designed to be left on for multiple days, and there are no gaping wounds right now, no signs of infection, just a lot of healing skin, so we have graduated to a new level with Bella! The three fingers that ballooned in the hospital are now completely healed. Her right foot is about 90% healed, and her left knee has finally closed over completely with new skin. She was born with NO skin on her left knee, shin, and foot, so this is huge.

We also found out there are 2 EB wound care clinics in the country, one in Cincinnati and one at Stanford U. where you can meet with an entire treatment team from dermatologist to GI to nutritionist to PT. While I am grateful they are there, I am creating that we won't need to visit them. We were thinking that an annual or semi-annual checkup there might be in good order, but hopefully we won't need to go for any emergencies.

She also showed us pictures of lots of RDEB kids who look great, and who are leading fairly normal lives. She also told us about a guy WHO IS 42 and has RDEB. We are pretty sure at this point that Bella has the recessive version due to the report that there was NO collagen VII present in the immunomapping. Recessive indicates there is no collagen VII, while in dominant, there is Collagen VII, it is just dysfunctional. The good news about that is that all the research and clinical trials are focusing on RDEB, so there is promise for breakthroughs in RDEB first.

Leslie also commented that Bella was the most relaxed and calm infant she had seen ever during a dressing change. She was also impressed by mommy and daddy's teamwork. She said her husband wouldn't even help out; that it was too much for him, so Leslie was left to wrap her daughter alone...her single fasted wrap was an hour and 45 minutes. Go teamwork!

Here's why today was so magical. Leslie is the first person who has seen our daughter who ACTUALLY KNOWS WHAT SHE IS TALKING ABOUT. No other person who has commented on her had ever seen an EB baby before. Dr. Metz, who took the biopsy, did not comment at all about her, so this was the first informed opinion we've gotten about how Bella looks in the grand scheme of EB. I forced her to categorize Bella at the end of the day into "MILD, MODERATE, or SEVERE" since that is what everyone talks about, and she said MILD. Music to our ears.

Providence of the day:

Last weekend, A grief & loss retreat was held in Phoenix, AZ at the Franciscan Renewal Center. I am usually one of the facilitators along with my mom and another colleague, but I backed out of last week's back in December when we realized Bella's due date (good thing, huh?) I missed being there, because it is an occasion where I get to realize my purpose, which is to heal with music. Leslie, today, confided that she was dealing with grief of her own, and we had the most tender conversation about how pain and memories are distinct from each other, but during grief, they get collapsed. I helped show her that as she heals, that pain will lessen, but the memories will endure. We realized that her daughter's life and death led her to her purpose...to our home, and because of my daughter's life, I wasn't able to express that purpose last weekend. Inside of our conversation, we both realized that sitting together in the living room today, we both got to realize our purpose for each other, and that neither of us had thought that would be how the day went at all.

So, pay attention to the people God puts on your path, and especially, "in your way." There are no coincidences.

Finally, thanks again for all the posts. We are so touched by each and every post. It means so much to us and gives us strength and confidence to continue being who we are being and doing what we are doing. Thank you for your prayers for Bella's healing. They are working. Her body heals every day, and for every little blister or flare up, there is faaaaar more healing occurring.

One day, Bella will look back and say, "Yeah, I was born with this rare skin condition, but when they brought me home from the hospital, it went away."

God bless all of you.

Chart the Course

At least, I think it's the 17th. I'm pretty sure it's Wednesday right now also.

We found out yesterday part of the biopsy results. "There was an absence of collagen VII in the immunomapping..." Dr. Metz rambled on. "Collagen VII connects the dermis to the epidermis," she went on to say. "This indicates that Bella has the Dystrophic form of EB."

My heart just dropped into my gut and my arms got cold.

"They weren't able to determine subtype do to some [blah blah blah] so they are redoing the second test and I'll let you know what it is either when I see you next Tuesday or before."

Thanks.

So, now the hunt for the next clinical trial of bone marrow stem cells takes place. In the mean time, there is another piece of the story that unfolded so fast, that I still am unsure it even happened.

As I sat in the dentist office two days ago awaiting an emergency crown, I called my friend Joe Polish, a close friend and marketing guru, to vent a little. Joe realized he was in over his head, that he had never been through anything like this, so he said, "I'm gonna put you in touch with someone who can better talk to you right now. His name is Sean Stephenson. I interviewed him recently and we've become great friends. Sean has a rare bone disease that left him with 200 fractures by the time he was 18. He's 3 feet tall. He's now a motivational speaker and Psychologist. Can you hold? I'll try to 3-way him in on the call."

As the nurse stands in the doorway waiting for me, I am introduced to Sean by Joe during taping of an A&E special they are doing on him right now. Sean gives me his cell number and says, "Any friend of Joe's is a friend of mine, and if there's anything I can do to bring peace, I will."

3 hours later, I come home from the dentist and hunker down for another long mostly sleepless night with a colicky baby wrapped in bandages.

The next morning (yesterday), I get a text from Joe "Did you call Sean?" I hadn't. It's too tough to make a single phone call right now at night. So, I call Sean on the way home from dropping Ali off at daycare.

We spend the next 45 minutes in an extraordinary conversation about what is at stake, who to BE in the face of this, and what there is to do. Sean says, "Don't listen to the Doctors, I was supposed to only live 24 hours." He follows with, "The only prognosis I listen to is the one I like!!" At this point, I still don't even know Sean's last name, when Joe said it, it went right by. I just had a hand scribbled name "Sean" and a phone number to call. Sean tells me that he wants to put me in touch with his Doctor, a microbiologist by the name of Robert Young. Sean started seeing Dr. Young about 5 years ago, and he hasn't broken a bone since. Not bad for someone who broke over 200 during the first 18 years of life. Sean said he'd let me go so he could call Dr. Young for me. A minute later, (10:04 to be exact) Sean calls back and says, "Dr. young is in London right now, but he said if you call him at this number in exactly 30 minutes, he will take your call."

It was during that 30 minute wait that Dr. Metz called me with the bad news. Without getting dramatic about it, we were hoping maybe Bella had the simplex version of EB which should could most likely grow out of. If it turns out to be Recessive Dystrophic EB (RDEB), it is fatal with subjects dying from malnutrition due to the erosion of the esophagus, infection from open wounds and/or resistance to antibiotics, or an aggressive skin cancer that usually comes in during adolescence. Most RDEB kids to live to adulthood.

So, at 10:34, I call the number and a man picks up the phone. It's Dr. Young. I explain who I am and he asks me to tell him what's going on with my daughter. Dr. Young is clearly a renegade. Within the first two sentences, he says, "Don't listen to the doctors. They don't have a clue. Here's what you need to do, you need to strengthen your daughter's blood. You strengthen her blood you strengthen her skin. Mom needs lots of green juices and get off sugar. Sugar is poison."

He then goes on to give me specific sprays he produces that he says will help Bella's mouth and skin be more blister resistant. He would like to see her blood tests results and any panels that have been done.

10:48. What just happened?

Other news:

Yesterday Ang and I were faced with doing Bella's wound care all by ourselves. Bella was awake and crabby, and we just didn't know how we were going to pull it off, but we were literally running out of time before we had to go pick up Ali so we just went for it. Well, all I can say is that Bella is freakishly strong, and not just muscularly, but constitutionally. She was a trooper. I also want to publicly thank the angel that was in the room with us. I don't who it was, but Ang and I were not alone, and Bella was somehow calm and soothed the whole time. (She went right back to screaming her head off 5 minutes later). At the end of the procedure, I felt closer to Angelique than ever before.

Total time of procedure? 50 minutes.

So, in all this there is ups and downs, Bella sleeps all morning while we run around trying to keep the house/ laundry / bills / paperwork / research / pumping / feeding up to speed, then turns at 8 pm into the most irritated, upset little girl who can't be put down for more than 5 minutes till about 4 am.

We know of the clinical trials at U of M and Stanford. There is also one at Rady's in San Diego where I interned and I am looking into that today. Now that we know the main subtype of EB, we can navigate what options there are. Really, until these bone marrow stem cell transplants occurred, all there was to do is bandage and love your kid and keep 'em as comfortable as possible for however long God intended them to be here. Now, with this new breakthrough, there is hope for a tomorrow beyond unending discomfort.

NOW, STANDING IN THE FACE OF ALL THAT, I SAY WILL YOU PRAY WITH ME?

One day, Bella will look back and say, "Yeah, when I was born, I had this rare skin condition, but when they took me home from the hospital, it went away."

The body IS a miracle. It does miraculous things every day. It defies "the experts" every day. God has the power to produce all the collagen VII God wants. It is to be His will. Sean told me yesterday, "Pray for a miracle. You HAVE TO CHART A COURSE. God may come and adjust the winds on you, but you HAVE to at least CHART the course."

Thank you all for helping my family "CHART THE COURSE."

Blessings.

Glad Today Was Only A Day

Glad today was only a day.

Too fried mentally and physically to expand too much tonight.

New big blister on left thumb. Imagine you had a blister that went from the back of your fingernail to the base of your thumb and that it was as wide as your thumb and as high as the thickness of your thumb. All in one day. Fingers are tough because we have Bella's hands wrapped 24x7 and can't see new blisters forming until the next day's dressing changes. We remove yesterday's bandages with anticipation to see if wounds are getting better, and in the case of fingers, if dressings are staying on her tiny, wiggly, lubed up fingers.

Thus the game of EB, one step forward, one step back.

THe good is that DEBRA is flying a volunteer out to spend an entire day with us later this week. She is bringing tons of information, dressings, diapers, and wants to observe our dressing changes to offer advice....we get her ALL DAY for ... um ... free. This DEBRA group is pretty amazingly funded and organized to provide such personalized and in-depth attention.

The volunteer also wants us to get the Stanford Biopsy results by the time she arrives so she can counsel us on which form Bella has. So, focus your intentions and prayers that the test is done and we get the results tomorrow and that the results are delightful. We need a miracle. We are praying for the version of the disease that children grow out of. Thank you for focusing on this tonight/today.

Okay. need sleep. Bless you all.