Wednesday, June 30, 2010

June 30: Day -2

Yay! Last day of cytoxan! Couldn't come soon enough. Last night, at 1 am, Bella started chirping a little louder than usual, so I got up to check up on her. Turns out her constipation was fixed. Without recounting the graphic details of the next 12 hours, let's just say that her body has officially been ravaged from the inside out by this drug. I saw things coming out of every orifice she has that just didn't look... natural. Her skin looks sunburned, and she spent all morning gagging on esophageal tissue that was sloughing off in her throat from the repeated vomiting of the past 3 days. Her fever came and went and came back again, she can't pee on her own, and the chemo has turned her stool to pure liquid. It's not pretty folks. We got up at 1am this morning and didn't get back to sleep till 4pm this afternoon. We snuck (both Bella and I) in a late afternoon nap, and as soon as I finish typing I'm diving back into bed, something I should have done over an hour ago.

She is currently sleeping on her hands and knees, face down in the sheet, randomly moaning. This sucks. The bright part? She had about 2 hours this afternoon before her nap where her fever broke, and she sat up and played in bed. that was a nice reprieve in an otherwise crummy and scary long day.

Tomorrow, Ali gets one more blood draw before Friday's bone marrow harvest and transfusion. Bella gets a day off before transplantation occurs Friday afternoon. Meanwhile, the chemo's darkest side effects are still about 10 days away.

There has GOT to be a better way.

All we can hope is that we are on a continuum of discovery that leads to a better way for EB kids in the future. If so, then our suffering is not in vain.

Thanks everyone for posting where you are following Bella from. That was really neat! We are moved and touched that you have brought her into your life. Thank you. Keep the prayers coming. She needs them.

Good night.

Tuesday, June 29, 2010

June 29: Day -3

Mommy got Bella to crack a smile for her even through the fever...

Well, day number 2 of an apparent infection. Bella's temp has been elevated (febrile if you're a doc - stay tuned for more 'docisms' as they surface...) all day, and she threw up again tonight when we sat her up for only about 30 seconds. She was very docile during bandage changes and has been sleeping most of the day. The blood culture they drew last night came back positive for a bacteria, so they have adjusted her antibiotics to a more specific med that can treat it more directly.

Dr. Wagner came by today while Ang was here to check in. He mentioned that something like 90% of infections they see up here come from the stomach. This made me relax a little about obsessing over her wounds. I've had an ongoing issue with controlling this disease, and knowing that I can't control her gut, actually provides an opening for some serenity to creep in and calm me down. I will be as vigilant as ever, but I don't have to obsess. That's reassuring. When Bella got an infection, I blamed me/us for it. My irrational thinking was that if we keep her wounds in good shape, we can control whether she gets an infection. What folly.

Ang's best friend Jen came in to town last night for the week to help out and take Ang to the next episode of the Twilight! The two of them have been to the first two together, so it was really sweet of Jen to volunteer to come up so they wouldn't break their streak! Ang is a big Twilight fan and there was a period of several months where not a night would pass without seeing her in bed with a Stephanie Meyer book in her hands. Today, Jen took Ali to Nickelodeon Universe at Mall of America. Tomorrow, Jen and Ang go to "Eclipse," the latest episode of the series while Ali gets a play date with her new BFF Ellery. Me? I got a hall pass today to walk over to Applebee's to watch Spain beat Portugal in the World Cup Round of 16.

My biggest dilemma is that there are no games till Friday. First 3, then 2 matches a day have really helped the time pass and my brain shift gears! My other dilemma is that I seemed to have torn my lateral left meniscus again. That's the cartilage that acts as a shock absorber in your knee. I have torn my lateral (outside of the knee) meniscus in both knees previously and it is a very specific feeling. This happened when I played soccer two Saturdays ago. I have rested the knee since then fearing this is what I did, and I tried just jogging about 20 yards today and immediately could feel the pain with every step. That's a drag, because I'm not really in a position to get my knee operated on! Even though it is an outpatient procedure (arthroscopy), I was laid up for quite a bit on both prior occasions, not to mention 6 weeks of physical therapy. So, it'll have to wait till we're back in OC. That way, I can have my same orthopedic surgeon do the work again. It'll be like dejavu! It also makes me sad, because I was really looking forward to venting my stress out on the field. Sooo, my classical guitar made the trip over to the unit today, and I guess I'll be pouring my stress out through my fingers instead of my feet.

Trying to "air out" to cool off a little...

Yay! Tomorrow is Bella's last day on cytoxan! Weeeee! She began a new drug today also called cyclosporine, or CsA. It is an immunosuppressant used in transplants to help Ali's cells take root and start growing without Bella's cells killing them off. She will be on CsA for quite a while, and it will make her hair dark and increase her body hair, so she just may end up a brunette for a while after all. The hair effects go away once she is off the drug, but some patients report a permanent change in either their hair color or density. The other side effect is that her blood pressure will most likely go up from it. This actually already occurred. I do her vitals every 4 hours while I'm awake, and the nurses or nursing assistants do them overnight, and at one point today, Bella's BP was almost the same as mine. Mine usually runs 110 over 60 and hers was 103 over 58! That just didn't seem right. Up till today her BP was always in the 80s or 90s, but we hit triple digits on the CsA! Good times. Really, it's not too big a deal at this point. Just something else to watch and notice.

Alright. All in all, a pretty quiet day.

P.S. Check out the new video I put up... it's a grand tour of Bella's hospital room! Woo hoo!

P.P.S. Only one more day to pick up a copy of Bella's book and have half the proceeds go to DebRA! Please consider buying a copy if you haven't already. Thank you.

Thank you for all your comments. Ang and I talk about them with big smiles every day. Also, when you sign your name, put your location as well. It can be either city/state or just MN for example (or country if you're outside the US). It always fascinates us how many people from how far around are praying for Bella. It really moves us to consider this web of prayer coming in over her from all over. Will you add that little piece for us? Thanks!

Good night, and God bless you.

Monday, June 28, 2010

June 28: Day -4

The good news? Bella slept through the night again!

The bad news? This morning, I awoke to, "Code Blue. Unit 5D. Room 518. Code Blue. Unit 5D. Room 518."

That's Daylon's room. He's 4 doors down from us. He's the sweet boy with Junctional EB that is one week ahead of us in the clinical trial. He lives 20 minutes from us in CA, was born 6 weeks after Bella, ended up in the same NICU with the same nurse, has the same GI doc, and pediatric dermatologist as Bella. The two are as close as you can get in this rare world of epidermolysis bullosa, except for subtype.

Since I work in hospitals, I "hear" codes very clearly. I heard it when they called a code on Bella the night she was born. Now, when I hear "Code Blue. Unit___. Room___." It means something very personal to me. I know that there is a family whose family member has just stopped responding and is no longer breathing and/or their heart has stopped beating. There is no way to describe the immediate helplessness felt at that moment. When Bella's code was called, she was in a pod, and there were 8 isolettes in the pod. So, when they called her code, it only said the pod number. When I heard the pod number, though, I thought about who else was in the pod, and for some reason I just had a bad feeling. 30 minutes later, when the phone rang in our room at 12:30 am, I knew my feeling was right on.

"Um, Mr. Ringgold? There's been an incident. You need to come down here."

All this flashed through my mind while laying in bed this morning. That was a scary way to start the day. I just stood next to Bella and held her while she slept. The code reminded me that this procedure is a mixed blessing. It holds promise, but at such cost.

Today really wasn't very good for Bella, either. At noon, her temp was 99. At 4, it was 102. At 8, 102.8. At 10, 103.3. She also vomited this evening and it was unlike anything I have ever seen come out of a baby. She is really weak and her torso and head are just on fire, but her arms and legs are cold. Weird. She's on antibiotics and tylenol. We know that the chemo itself can cause fevers, so the antibiotics are really just a shotgun blast in the dark, but hey, if they hit something and the fever breaks, great, right? The thinking is that the chemo has begun to know out her white blood cells (by design) and therefore any number of bugs (viral, fungal, bacterial) that normally would be fended off can now start to run riot. This is part of that collateral damage of this procedure. You just leave the body so defenseless. This is the part of the journey that scares me the most, and always has. Infection.

So, not exactly a banner day for Team EB. You can follow Daylon at:

Please drop by there and send him and the Edlings some love. They are a wonderful family and we just love them more and more as we get to know them better. If nothing else, please say a prayer for Daylon that he heal peacefully and fully, for strength for mommy Jennifer and daddy Brian as they also balance caring for their 4 other kids that are here, and for Brian's mom, who had to get on an airplane in the middle of the day to return home. That must have been the hardest thing a grandma could have to do. Thank you for sending them your prayers.

At least Bella is sleeping peacefully right now. Sleep, Bella, sleep.

Bella's machines at night...

P.S. The doc explained to me that temperature itself does NOT cause any damage or risk. It is simply a marker of the intensity of the infection. It's the infection that does the damage, not the temperature. Stories of brain damage or brains frying from high fever temperatures are incorrect. It was the infection that did the damage, not the heat. Haha, after reading that, I don't know if that makes me feel any better, but it was an interesting thing to learn. It came up because we contemplated giving Bella celebrex to help lower her temp. Temperature is related to comfort level, so celebrex will make a person feel better, but it won't treat the source of the temp. Celebrex is harsh on the liver, so while Bella is sleeping peacefully, there is no reason to load up her liver any more than it already is.

So the game of troubleshooting has begun. The good news is that these docs have had EB kids on this unit continuously for 2 years, so this process is not new to them with EB or BMT. Go docs!

Ok, enough for tonight.

Sunday, June 27, 2010

June 27: Day -5

Boy, are the days running together...

LOL, I have to tell you, Bella slept through the night last night, but I didn't! I was so nervous/anxious/prepared? for her to awaken in the middle of the night (like the prior 3 nights) that I psyched myself right out of a good night sleep! I was laughing at myself at 3:40 this morning lying in bed, unable to sleep. Good for you, Bella. Good job; at least one of us slept!

Well, today we said goodbye to busulfan (yay! no more daily dressing changes for now at least!) and helloooo to cytoxan. BOOOO! Just the name alone portends bad things to come! Bella began her cytoxan at 10 am and by noon was dry heaving. That was a drag. The good news was that we had already upped her anti-puking drugs to near full capacity, so it could have been worse. Also, we started venting her stomach pretty soon after she got the drug to help release pressure in her gut before it blew! Venting is when we attach her G-tube, open it up, and tape a baby diaper to the open end to collect any drainage. This is like just like a pressure release valve. Instead of the puke building up pressure and exploding up through her esophagus, first, it pushes out through the tube. This is VITAL for EB kids since vomiting is B-A-D because it can damage their esophageal lining.

Prepped for dressing changes... Baby Einstein, ON!

We also doubled her benedryl consumption so she is sleeping a lot as a result of that. This is a good thing, because prior to this, she was feeling crappy and unable to shake it. She had a few moments of cuteness today, but mostly she was really unhappy about feeling so crummy. Can't blame her! So, sleep, Bella, sleep. You ain't missing a thing.

All in all, so far, the docs are quite happy with her labs, her skin, her vitals, our wound care, and her side effects. We are grateful for the amazing level of care both Bella and we as parents and big sister are receiving here. They have had at least 1 EB patient on this unit non-stop for two years running, so we don't have to explain A THING. Can I just tell you how refreshing that is? Life is good. God is good. All the time.

A quiet Sunday overall; nothing unanticipated. We knew she'd feel a lot worse today, and guess what? She does. Sure it's a drag for her, but with her sleeping so much today and last night, I simply can't help but feel grateful that she's sleeping through it.

In other news, this month of June we dedicated to giving away half of all proceeds from Bella's book to DebRA from the first dollar raised. Well, we haven't sold nearly as many books as we hoped, so if you haven't yet, please consider purchasing Bella's Blessing: A Humble Story of Providence. It chronicles in bone chilling detail Bella's first three months on earth and how through friends, family, and most of all faith, we came to see her condition as a blessing.

Thank you for being with us.

P.S. Go Germany!

Saturday, June 26, 2010

June 26: Day -6


This has to be short. Windows of time on the unit are brief to blog as sleep is more important. It's like that phrase that no one ever can actually follow for newborns: Sleep when they sleep. Yeah right. Well, in this case, we HAVE to.

Day -6 came and went rather uneventfully on the unit. No news is good news around here! This is the last day of busulfan, the first chemo drug. Tomorrow, begins 4 days of cytoxan. Bella's nurse described it as "pure hell." Great. Thanks for clouding my space. It's the really icky stuff. Yeah, I said icky. The good news is that after tomorrow, we don't have to do daily dressing changes. busulfan leaks out the skin, whereas cytoxan leaks out the urine. Sooo... diaper changes will happen every 2 hours round the clock. They begin Bella on a saline flush tonight to start to really mobilize her system to flush flush flush so that when they administer the cytoxan tomorrow morning at 10am, her body is already is fast flush mode. This will help protect her kidneys from any unnecessary collateral damage. Combined with an oral drug she is already taking called ursidiol ( I think I got that right), her liver and kidneys are as protected as they can be.

Ali and daddy enjoyed a play date with Logan and Ellery at their house today. Unfortunately, daddy didn't get to play soccer (no game today) and watched as the U.S. were eliminated from the world cup. I'll skip my thoughts on the world cup to spare you since 98% of you probably don't follow soccer. Suffice to say, I'm proud of our boys, but hoped they'd have gone further.

Bella is still in great spirits most of the time, but stayed up from midnight till 4am this morning, so clearly, sleeping through the night is history.

Chillin' after her daily dressing change...

Here are the photos from yesterday I promised. Thank you all for your ongoing support and for sharing about how you stay balanced! That was great to read. I want to know more about you as well. This is a special connection we all have. At no time in human history could any of our ancestors walked this path together in such a way. I am deeply grateful to be alive right here right now, today, in 2010, in Minneapolis, MN, inside a little hospital room, listening to thunder rumble through the windows that don't open. This room is not very big, but I know I speak for Ang when I say we don't feel alone in here. Thank you for coming and staying with us through this. We're all better for it. See you tomorrow.

Check out how well I balance!

The Tentacle Pole...

Ali rocking her kid's face mask...

Bella doing tummy time in physical therapy

Bella practicing standing in PT...

Friday, June 25, 2010

June 25: Day -7

First off, I forgot the camera, so this is a rare, photo-free post. I'll double up tomorrow. I just love bringing you in through pictures as well as words. I feel like the clearer you can see what we see, the clearer your prayer and intention can be.

T-7. Last night, Bella woke up at 3 am and rocked, dry-heaved, hiccupped, and cried her way to 6 am this morning. That was a drag. At one point around 5ish, our night nurse Julie walked in to me sitting in the chair pushing Bella in her stroller with one hand back and forth in the darkness and silence. It was the only thing I could do. She asked, "Would you like me to rock her for you (so you can get back to sleep)?" The staff here is like that. They are simply amazing. They walk in at night and use a big mag flashlight to work by if the lights are out rather than turn on any lights and possibly disturb the patient or parent. They KNOW how crucial rest and sleep are to everyone's outcome. That is really cool. I don't have to feel apologetic about wanting and needing sleep.

Bella gets physical therapy 3-4 times a week as well. The have a mat and toys and we go to work for a little while in the mornings getting her to stretch out her hips. Her hip flexors are really tight, and will develop abnormally if left the way they are, so we have to get her standing and tall kneeling as much as possible to release those muscles. We also have a chaplain, child-family life specialist, social worker, occupational therapist, and respiratory therapy (should it be needed) available. In addition to having a Care Partner that will work just with our family for the duration of the BMT, Care Partners stocks Unit volunteers almost every day. This is awesome, because they just come and take Ali and go play while mommy and daddy do bandage changes. Good services.

Bella is on a combination of ativan and benedryl to offset the onset of nausea, which typically begins today. So far so good. Having said that, I am back at RMH tonight for respite, so I hope things are quiet and uneventful up on the unit till I return tomorrow. Fill you in then!

We take it one day at a time, there is plenty to chew on just within a 24 hour period. Bella's tentacles are a moment by moment ordeal. Ugh. Imagine how fascinated YOU'D be at 12 months to have all these cool tubes and wires and plugs and valves coming off of you! So far, the busulfan has been gentle to Bella. We don't expect the same from the cytoxan which begins on Sunday.

Tomorrow, Ali gets to spend the day again with Erin, Logan, and the kids again as it is Ellery's birthday party tomorrow! Also, I get to play soccer again. Even though I aggravated an old injury last weekend, my brain needs the release, and the net effect is still positive. We are looking into finding a nearby Zumba class for Ang to plug into. That's her stress relief. It's all about keeping the equation of stress and stress-release in balance. When the stress side of your equation spikes, don't fool yourself into thinking the typical amount of stress-release will suffice. Remember Newton's Laws of Physics? Every action has an equal and opposite reaction. This is the yin yang duality of the universe explained in a most succinct way. You got 3 new stressors? You better find 3 more stress relievers. The last thing Ali and Bella need right now is a parent sidelined by a stress-induced illness or disease. It is our JOB to be well and healthy right now.

How 'bout you? Is your equation in balance? If not, what is ONE thing you can do the day you are reading this to move it closer to even? Post that thing as a comment here. Tell the community. Make it public and that'll REALLY get the fire burnin' to get in gear! Don't make the mistake of thinking you're "taking time out for yourself," that is a load of..... You are building IN your ability to be the person you want to be for your self, your family, and your community. Get in balance. Thanks for enduring my soapbox! :)

Thursday, June 24, 2010

June 24: Day -8

Today's entertainment provided by... Mickey Mouse!

I have to laugh. There is a really big day-at-a-glance calendar in our room. It says TODAY IS... then shows the number REALLY BIG. So I am looking at a giant TODAY IS 24, then under that reads Thursday. Under that reads June, 2010. Why am I describing this to you? When I first walked into the room, I thought, what good does that do Bella? She can't read! Now, just three short days into this journey, I realize, IT'S FOR ME!

Time is a paper construct that we all relate to like concrete.

Minutes of the hour, hours in the day, days of the week, they all just fizzle away on this side of the world. Yet, on the outside, I rush through traffic to catch every green light thinking, "Every second counts." I am the architect of my own anxiety. Aren't we all?

Today was a good day! Mommy and Ali went to the annual "Turtle Derby" outside in the plaza. This is a giant fundraiser the hospital does each year where they race... turtles. It's quite possible the funniest thing I've ever seen. Picture this: A blue tarp on the ground that spreads 12 feet long by 12 feet wide. On this tarp is painted a giant circle, like a wrestling ring about 10 feet in diameter. In the center, there is an inner ring maybe 3 feet in diameter. Now picture a 12" tall cylinder that sits atop the inner ring. The turtles are placed inside this holding pen, then, when the announcer starts "the race", the pen is lifted off and the turtles take off in all different directions at once. The first turtle to cross the outer ring in its entirety is the winner. All the departments and nursing units raise money to put a turtle in a heat representing them. It is pure comedy. Then, add dancing, clowns, kids, food, and you have a good time. They even televise the event so we could watch it up in our room.

Needless to say, what's the first thing I see when I turn on the TV? Ali dancing with a clown. Awesome.

Then, Ali went off on an all-day play date with my friends the Spectors. They are sooo great. Logan is my childhood friend from CT who became a researcher of childhood cancer here at U of M. He knows Dr.s Tolar and Wagner. Anyhow, they have just opened their family up to us so generously, we are so grateful. Ali went to the pool, then got to go to soccer practice with the Owen and Ellery! That made daddy very happy!

Ang and I did our afternoon dressing changes with Bella watching an endless loop of Mickey Mouse Clubhouse. The great thing about kids DVD's is that they feature a "Loop Play" or "Repeat Play" feature where it just starts over, and over, and over... and over. This is good for dressing changes because Bella doesn't care! She was sooo peaceful during bandages today. Not a peep. All without any pain meds as well. It is only day 2 of busulfan, so she is not experiencing any side effects from the chemotherapy yet. I tell you, when we do her bandages, there is a serenity that comes upon her. It often leaves as soon as we are done, too. She will either become more animated (or angry!), but it's usually after, not during. I tell you, it IS as if she knows this is all central to her plan. I had the experience today while doing her dressings of being totally and completely present in the moment. There was no world "out there." There was no "Thursday," no "June". Just me, Ang, and Bella, together. This is why I want to thank Erin, Logan's wife, again, because I knew Ali was happy and safe. It allowed me to just melt into the moment. Bandage changes used to be stressful. Now they are sublime moments of care, compassion, and tenderness. I only wish Ali was bigger and could participate in it more, but alas, it is all simply perfect as imperfect as it is.

Bella ordering dinner for daddy...

We headed off the night-time anxiety attack by giving Bella some happy meds (ativan) with her nighttime bottle. This kept her out long enough to slip into a deeeeeep sleep. We also did all her meds in the bottle, and did vitals before the bottle so once she fell asleep there would be no poking or prodding. We get better every day.

Before the meds...

... and after!

Now it is time for sleep. The moment by moment management of Bella's tentacles has left my brain quite tired. She has a lot of lines coming out of her body with pointy valves and connectors. Some of them really tore up her back and aggravated her belly during last night's fits, so great care is given to make sure she avoids unnecessary contact with them. Having Epidermolysis Bullosa and a belly full of sharp valves, lines, and tubes does not make for a good combination!

Thank you again for your comments. You give us so much strength; I hope my words of gratitude somehow capture a glimpse of how GOOD your comments make me feel when they hit my phone throughout the day. Ang and I call each other throughout the day and talk excitedly about all the different comments. We feel so... cared for. Thank you again, and good night.

Wednesday, June 23, 2010

June 22,23,2010: Days -10 & -9

First of all, I apologize for not posting last night. It was a late one.

Here is yesterday's summary, followed by today's:

June 22, 2010: Admission time!

Well the day finally arrived. It was a day full of anticipation, both excited and anxious. In the air hung the hope for a better future for not only Bella, but all kids with Epidermolysis Bullosa, and the fear of the path required to get there.

We were to arrive at Admissions at 11:00 am, which remarkably we actually did. Suitcases in tow, we did the paperwork, and headed up to the now famous "5D". Unit 5D in the hospital is reserved for pediatric BMT, and where our previous 11 fellow pioneers found themselves. We have read about 5D since the fall when we started following online posts from patient number 7's mom. Now, it was our turn.

After a relatively short while, we were informed that we had a "pass" and could leave the hospital till 8pm. That was a bit weird. Why show up at 11am, then? Oh well, I thought it was a treat! We went back to RMH, had lunch, and Bella and I took a good nap. Little did I realize how much I'd need it... more on that soon.

We went out to Punch Pizza just up the street, which was highly recommended to me by my friend Logan. Being the pizza-holic that I am, I decided this would be a perfect "last meal" on the outside. So, we set off for Punch. It is a really cool little restaurant that wood-fires your pizza at 800 degrees for roughly 90 seconds. What you end up with is a deliciously fresh pie. The decor in the restaurant is pretty cool; they have giant garage doors that open to the street. Since the weather was quite warm, the doors were open and we were treated to all the traffic noise one could take. However, the doors being open presented one of the best Providence stories to date:

Picture this: we are sitting in a booth about 6 booths in from the street, so we are pretty far into the restaurant. In fact, there is only one other booth behind us before you reach the back of the room. Toward the end of our meal, as I look out the open garage bay, I spy a butterfly. Remember, butterflies are the symbol for EB since the fragility of EB kids' skin has been compared to the delicate nature of a butterfly's wing. Anyhow, I watch this butterfly fly into the restaurant and down the aisle straight toward Bella. Bella is sitting in the aisle in her stroller, facing us. The butterfly flies right up to Bella's stroller, then does a 180 degree turn and flies back to the entrance of the restaurant. Then, it turns around, flies back in again to Bella, turns around, and then flies back to the sidewalk. By this time I am flipping out and manage to get Ang to grab the camera to try to take a picture, because the butterfly then turns around and flies back to Bella A THIRD TIME and lands at the foot of her stroller. It lands so close, Bella practically rolls over it with her wheel. Once Ang takes a picture, the butterfly flies back down the aisle, and this time flies right out the door and on its merry way.

Did the butterfly know that just hours earlier I was hanging a butterfly above Bella's crib in her hospital room to help brighten the room and have him watch over her?

Remember, God is in the whispers. He whispered 3 times the same thing he always whispers, "I got you, I got you, I got you."

I never get tired of hearing it. I'm grateful that I remember to listen.

Bella went down quietly and peacefully at 9pm with her evening bottle. However, we still hadn't changed her Hickman dressing. Up till then this had been quite painful for her. Sure enough, she woke up, and would NOT go back to sleep. It was 1:15 this morning when she finally collapsed. Hence no update. She screamed all the way till then. A rough ending, but a great day.

June 23: Day-9:

For those who don't know, we are part of a 100 day clinical trial. Those 100 days are from the day of transplant, which is called Day 0. Any days in chemo leading up to transplantation are like a count-down. So, tomorrow will be Day -8, the Day -7, etc.

Today, Bella began her chemotherapy. It was so exciting, I slept right through it. Seriously, it was just another tube added to her giant pole of meds and fluids at exactly 4am. No need to get up, so I didn't. She began what will be 4 days of a drug called busulfan. It's a nasty, toxic poison that will kill off a good chunk of her bone marrow. The idea is that the busulfan "moves out" the existing tenant, namely Bella's bone marrow, to make room for a new tenant, Ali's marrow. It's a bit more like a forcible eviction, and the eviction began today. No side effects today, thankfully, but they're coming. Actually, we had to sedate Bella tonight to get her to sleep. She was screaming uncontrollably again and was simply inconsolable. I couldn't even hold her she was writhed around violently in her crib. As I watched her sobbing and yelling, I thought, "So this is what it looks like when you poison your own kid on purpose." No lie folks, this is a dark tunnel to have to go down in order to get to the light, and the process is damn near barbaric, but it's all we got. This is why I hope that this research can somehow spawn new research into less invasive ways to treat and cure EB.

It's gonna be a long walk in this little room. Look at the pictures and you can see Bella already feeling a little trapped in her crib. I thank God that she won't have any conscious memory of this little stroll in Minnesota.

At the start of the day...

By lunch time...

By dinner time...

At the end of the day...

Don't worry. It's not all gloom and doom. I'm just tired. Tomorrow I'll make sure to tell you about all the amazing staff and resources that have been simply pouring in since Monday. Thank you as well for all the prayers, intention, positive thoughts, vibes, wishes, and comments. They make us feel like you are right here with us. We are a long way from home, but we don't feel a long way from you. I take you with me through the day. With each new comment, my phone vibrates and gives me a little inspirational cup of water to keep me running through the day on this marathon. Thanks for all the cups of water. They mean a lot. God bless you.

Monday, June 21, 2010

Last Night Home...

... and when I say home, I mean the Ronald McDonald House. Tomorrow at 11:00 am, we admit Bella to the hospital, and she will begin her treatment immediately. I met today with our social worker, Janet, who is AWESOME, as well as a representative from a volunteer group called Care Partners. They assign a volunteer to the family for the entire journey who is kind of at our disposal. Laundry needs doing? They're on it. Groceries? No problem. Hang out with the girls while I play soccer? Easy. Support, baby support. It's all about having enough support to counteract the immense increase in stress on the family, both individually, and collectively.

Ali's craft work while I met with Janet

Last, but not least, I met with Dr. Wagner today. He's the head researcher on this trial and quite the guru in curing incurable diseases. He is great. No ego whatsoever. We spent an hour going over all the risks and benefits of the trial for one last time to make sure we are clear about what we are getting ourselves and Bella into, and realistically what can we expect to get out the other side.

How to keep a 4 year old preoccupied...

His tone was more measured than in January regarding promise for the protocol being a possible cure. Today, her referred to it repeatedly as "A first step." That really wasn't that cool to hear the night before we're admitting our daughter, but, 6 months have passed and a lot has happened since then. The hard truth is that out of the 10 transplants that precede us (number 11 gets transplanted in three days, and we are number 12), 2 died, and 1 experienced graft failure, but miraculously grew his/her own white blood cells back. That isn't supposed to happen, so that patient is really lucky to be alive. That leaves 7 other patients, and their results range quite a bit in terms of reduced acuity of the disease. So, when you are on the front lines of a new treatment, anything's possible. We accept this and are frankly grateful that Bella is only 12 months old and participating in this rather than 12 years old. We do know that for the majority of the 7, there has been improvement in their skin. How much ranges the entire gamut.

The master artist at work in her shop...

Let me try to break down succinctly if I can what is going to happen. Here's the deal: Bella is going to receive 8 days of intense chemotherapy to effectively clear out her own bone marrow, and completely eradicate her body of any immune system whatsoever. Then, she'll have a day off, and following that day off, the docs will pull bone marrow out of Ali's hip bones, and through a simple blood transfusion, give it to Bella. Now, the idea is that Ali's marrow will start producing stem cells that will turn into new blood cells, and a new healthy immune system. But more than this, the stem cells go to the skin, where the area of injury lies, and these stem cells start to generate new skin cells as well as blood cells. This is where this trial is the first of its kind. Normally, BMT is done for cancers of the blood, where the injury is in the blood and marrow. Not with Epidermolysis Bullosa. The stem cells somehow "know" that the skin is where they are needed so they home in on the wounds and generate healing previously never seen in the patients.

So, this means that all the areas of injury on Bella's body currently will potentially get an infusion of healthy new skin so that these areas no longer blister and wound as easily in the future. Here's where it gets interesting: What about the areas that have no wounds? Well, they aren't included in the equation. Somehow, the trauma sites send out a signal calling for back-up, so to speak, but the uninjured areas have no reason to send that signal (yet) so the stem cells don't go. However, once a site injures for the first time (anytime after transplant), the stem cells will then get in gear and head to that site to increase healing.

We brainstormed about several different subtleties about this disease, and during the conversation, what struck me was that Dr. Wagner is so far down in the trench... literally into the micoscope, with this particular protocol, that he hasn't yet delved into other more global questions about EB that surface when you theorize about what is next AFTER this. At one point, I suggested what I see as a future evolution in treatment, and he looked at me point blank and said, "I never thought of that!" That was fun. Brainstorming with Dr. John Wagner. Sweet.

How to make sure your kid doesn't take a header off the bed while you pee...

So, tomorrow begins the year-long walk down a very dark tunnel to emerge out the other side with a brighter future for Bella. I say year-long, because realistically that is how long it will take her immune system to be back on line firing on its own cylinders. Until then, we walk a dangerous road, hoping to avoid graft failure (Bella's blood attacks Ali's blood), Graft verus Host Disease (Ali's blood attacks Bella's), and infections as best we can.

This is where that time-honored struggle of control kicks in again. How to do everything possible, fight every battle, take every necessary step, and yet KNOW that it is totally OUT of our control while we weigh decisions that APPEAR to demonstrate that we ARE in control... what a conundrum. I like the phrase, "God is in the results business." We are in the process business. If we just do the best we know how, then turn it over to God, it's really the only way to manage any sanity throughout this. This is when I am grateful for my Faith. See, my faith is of my own volition. It is not anyone else's faith. Others may believe similar things I do, but at the end of the day, I CHOOSE to have faith. No one gives it to me, and no one can force it upon me. Consequently, no one can take it away from me either. It is literally my own creation, and since it is such, I create a faith that empowers me, strengthens me, inspires me, motivates me to get out of bed and face my terrifying fears head on. Without this asset, I wouldn't have the ability to get out of bed in the morning. The fear I am experiencing going into tomorrow is far beyond anything I have experienced in my life.

So, what do I have faith in? What do I believe?

I believe Ang's and my love for each other deepens every day we walk through this together.
I believe that Ali's character deepens every day she is Bella's big sister.
I believe that Bella knows exactly why she is here. She just smiles blissfully at us while we labor over her bandage changes as if to say, "ah, yes, this is why I chose YOU... because I KNEW you would do a good job with me."
I believe that I will be pushed past what I thought I was capable of and thus know myself as enhanced, increased, and upgraded from my previous image of my self.
I believe that Bella will inspire many, many more people, and that this was her plan all along.
I believe that God has the answers, and I believe that I ask him pretty good questions.
I believe that one day, Bella will look back on all this and say, "Yeah, I was born with this rare skin condition, but when they brought me home from the hospital, it went away."

What do you believe in?

Sunday, June 20, 2010

Happy Fathers Day!

Happy Father's Day all you dads out there!

Come to think of it, are there any dads out there reading this blog? If so, CHIME IN! Lemme know you're out there! This way, I might occasionally throw in a sports analogy JUST FOR YOU.

Today was great. First off, my prayer was answered! The girls all slept in till...

wait for it...

... are you waiting?

9:30 am! WOOHOO! That was AWESOME!

Then, if that weren't good enough, ang took the girls into the living room and let me just veg in the bed watching the World Cup, and brought me coffee and breakfast in bed... ON A PLATTER! Okay, okay, it was a breakfast in bed tray, but who cares? HOW AWESOME IS MY WIFE???

Then, we went downstairs for a nice Father's Day brunch provided here at the house. The girls then napped for a while. I just watched more World Cup in bliss. After they awoke, we went to the Stone Arch Festival of the Arts in the Riverfront District of Minneapolis. My friend Logan tipped me off to it, and it showed us a whole other cool part of the city we didn't yet know about. We didn't stay for too long because it was really hot out today, and Bella was just roasting in her long sleeve onesie and long tights, not to mention all 4 extremities bound and wrapped and a giant girdle around her midsection holding her Hickman in place safely. Nevertheless, it was really fun; we got some fresh mini donuts that reminded us of Pike Place Market in Seattle. Ang bought a beautiful necklace, and Ali got her face painted. Good times were had by all.

We then hit Target only to cross paths with... the Oscar Mayer Wiener Mobile on the way out! What an awesome combination of engineering and marketing. I wanted to go inside so bad! I wanna party inside the wiener mobile! That doesn't sound good, but who cares. It looks FUN!

You KNOW you wanna ride...

Ali rocking the wayfarers at Target...

We came home and raided the kitchen for more yummy leftovers from Friday night - whole wheat pasta with turkey meatballs with cucumber/tomato/dill salad - (nobody eats the healthy left overs around here... better for us!) and played around in our place till bedtime. Nice and mellow. Just the right speed going into this coming week.

Other news:

Check out the video tour of our place in the video section to the right.

Drop in on our other EB friends' blogs. Baby Daylon started his BMT one week ahead of us; please send lots of prayers his way as the chemo drugs are really hitting him h-a-r-d. Baby Rafi is back in the hospital with a fever, so please pray for her as well. Baby Jonah is still working on feeding issues, and baby Tripp is dealing with eye issues. They all need all our prayers. Please take time to visit their blogs and hear their stories. They will inspire and touch your hearts. There are two sides to the Epidermolysis Bullosa coin. Pain & suffering are on one side, and gratitude & inspiration is on the other. You'll see both sides of the coin play out in each of our stories, but that's what makes life worth living. Thank YOU for living our story with us. We are better for it with you in our life.

Blessings to you.