Picture Essay from Bella's G-tube Insertion

Hi all!

Tonight's post is a little unusual. I am posting all the pictures from Bella's G-Tube insertion back in October. There is another EB Baby having one placed at the end of the month, and the family is not as lucky as we were in that their hospital doesn't have 30 years of experience operating on EB babies. So, we took tons of pics during Bella's procedure to share with the community. Originally, we took them to share with our local hospital, CHOC (Children's Hospital of Orange County), so CHOC could develop an EB surgical team, but then I got busy. At least all this work can now be used THIS MONTH somewhere else in the country, so it wasn't all for naught! Here we go:

Items to assist in securing intubation to face. I believe that is vaseline infused gauze.



Front of their EB cart that resides in the O.R.

How awesome is is that they have a dedicated cart???

Top of cart had supplies prepped for surgery. Here's a sneak peak at what was underneath... First up, bottom right corner.



Top right corner



Top left corner

Bottom left corner

Bottom Drawer... EXPOSED!



Specialty Dressings... UNDRESSED!

Dressings Drawer... what would be witty right here?

Wound Care Team. We convinced them to let us do Bella's bandages while she was under sedation. They were a little hesitant at first, but we apparently appeared "on it" enough to get access to the O.R. It was AWESOME. Ang and I make a really good team. The O.R. team was really impressed. That made us feel really good. That's a pretty qualified opinion!

Bella going under. Mask was covered in aquaphor before placing on her face. I sang to her during the process her Birth Song, and I didn't even get to the chorus before she was OUT.

Close-up of intubation set-up. There were no injuries to her head, face, mouth, or esophagus as a result of the procedure. Good job, docs!

Different view of head dressing. You can see the foam pad and air pillow better from this angle.

Close-up of surgical site. Incision was made for G-tube as well as second incision to anchor it about two inches to the center. Mepitel was glued over surgical incision and stayed on for several days. No harm done, scar healing nicely. We now use mepilex lite under button to protect against friction and absorb any drainage as the opening healed. It oozed stomach juice for several months at a slowly declining pace until now where it hardly oozes at all. We still clean the site daily with a Q-tip dipped in 50% water 50% hydrogen peroxide. the dark spot to the outside right of the button was a blister she had going in to surgery that was totally unrelated and ended up healing fine with no scar.

Little cherub out cold after surgery back in her room. Notice the IV placement was in the right foot, and yes it is a bitch for EB kids and yes they deliberated long and hard over where to do the IV. They did not use sutures to secure the IV, although (pardon my french here) an ASSLOAD of that gummy wrap you see above was used over a CRAPLOAD of rolled gauze to try to keep it in place for as long as possible. We had to do a cranial IV the week prior at CHOC (had to do three different placements as it kept coming out due to Bella being a squirmy (read: normal) baby. That SUCKED. Each placement took like 4 of us and never got done in less than 2 hours. Ugh. THIS was MUCH better. It stayed in till it could be D/C'd . How did it NOT get yanked out, you ask? ...

...because the poor girl was tied down for the next 3 days practically! No joke. Look at how each appendage is secured so that she won't a) wreck her IV and b) wreck her new button. She was a trooper through it all. Didn't hurt that we kept the happy drugs FLOWIN'. No time to be puritan about pain meds as discomfort causes maximum squirmitude. Yes. That's a word. No. you can't use it in scrabble.

Here's the little trooper recovering well enough to have her arms released! (how nice of us, I know) I recommend lite under the button rather than mepitel as it often (as was the case here) got stuck to itself and would bunch up easily. Double sided tape will do that to ya!

Alright, I hope this helps those of you who are either considering this procedure or preparing for it. It was the BEST THING we've done for Bella to date. As Dr. Eichenfield, the eminent Pediatric Dermatologist in San Diego and resident expert on EB said to us,

"The key to EB is nutrition, and the key to nutrition it the feeding tube."

Before the tube, we STRUGGLED to get 20-24 oz a day into Bella, and I mean STRUGGLED. It was awful. Feeds frequently took 90-120 minutes and had to be timed so that Bella was dozing in order to tolerate the bottle. If she was too alert, no deal, and if we waited too long and she fell asleep at the beginning of the bottle, no deal.

After the tube, we got 30-36 oz a day into Bella without so much as a whiff of trouble. Any time she couldn't or wouldn't tolerate a bottle, NO PROBLEM, snap that tube into place and go! Late night feeds were done without having to wake her. That was awesome in and of itself! But most importantly, we got her caloric intake up above what her GI doc and nutritionist wanted, and feeding became fun again! No more knock-down, drag-out bottles.

Now, 6 months later, Bella is 90th percentile in height and weight and 110% Weight to Height ratio, which is RIGHT ON for a typical child at her age. In other words...

SHE IS THRIVING!

We are extremely blessed that all is going so well, and that all went so well with this procedure. We recognize and give thanks that we have walked through the Valley of EB with a lot of success to date, and do so humbly in the memory and in the face of so many other EB kids who haven't fared as well. We take our successes with thanks and humility with full understanding that at any moment it could all change for the worse. EB is like that. Every day is a gift. Period. Thank you all for walking the journey with us. If you are reading this, you are walking this journey whether you realized it or not. We see you there, we feel you there, and we give thanks that you are there. You lift us up when we are too tired. Thanks for that.

Blessings,

Tim

One Step Forward, One Step Back

Well, hello everyone. Sorry it's been a while since any updates. Well, that's because there hasn't been much to update about. Bella's old wounds get better, but she continues to get new ones. One step forward, one step back. We are struggling with the reality that this is what it will be like every day of her life. We've been praying so hard for things to just "go away," that new or recurrent blisters almost feel like slaps in the face. We feel like somehow we are letting her down. Some of the blisters go away and stay, some just keep recurring. It makes us question whether Bella will ever be un-bandaged. One day at a time. One day at a time.

The good news is that her gas is subsiding. After just about a week on Zantac and Culturelle, coupled with Ang discontinuing spinach (spinach gave Ali bad gas as a baby), Bella has been mellow, almost serene today. That's a good thing because yesterday she was miserable all day and evening. We were told there would be good days and bad days, and those EB parents were right!

Here's a great Providence story from this past week I forgot to write about.

While at the EBMRF picnic last Sunday, a woman stopped me to ask me if Bella was an EB baby. She said her granddaughter had EB and she wanted us to meet her. Her name is Noelle, she's 8 , and lives in Irvine. The grandma wrote Noelle's mom's name and phone number on a piece of paper for us and we all went on our way.

Two days later, our neighbor Robert dropped by. Robert and his wife Katherine gave birth to a boy, Julian, three weeks before Bella. We don't know them well at all, but when they found out about Bella's condition, Robert came over and told us if there was ANYTHING they could do, let us know. Anyhow, Tuesday, Robert appears at the door with a gift for Bella! We talk for a little, then get back to dinner. As I open the box, a cute baby outfit awaits inside along with a card. As I read the card, there is a P.S. paragraph on the left side of the card. (You know, the part where you actually write something personal). Anyhow, the paragraph reads, "We want you to know that my sister in law has a daughter with EB and would be happy to talk to you and help out in any way. She lives in Irvine and..."

The name and number? Same name and number that was written on the note from Grandma.

Katherine had no idea we met her niece just two days prior.

In a metropolitan area filled with millions, and with a diagnosis of only 10 in a million, what are the odds? Too great for coincidence, leaving once again, only Providence.

On a separate note, I went back to work at UCI Medical Center this past week. I work in oncology, and the two sessions I had with two patients while they underwent chemotherapy were two of the best sessions of my brief career. The level of empathy and empathic listening was unparalleled, and the authenticity I was able to tap into created such a safe space, the two patients opened up about all sorts of things related to their journey through cancer. One patient even recalled the birth of her child and having to go home from the hospital without her due to her being only 4 pounds. No one can explain to you what that is like. It is the most "wrong" feeling on the planet...like there is something terribly wrong with the planet and there is nothing you can do to undo it.

I realize that my own suffering has created a new level of connection with my patients, and I am grateful for the tender sessions we had this week.

There really is always something to be grateful for, sometimes it just takes a little reflection to see it. Thanks to you, this site allows me the opportunity to reflect and share these reflection with you. Your feedback continues to strengthen us as we brace ourselves for the marathon that we thought was just a sprint.

May the abundance of God's blessings be upon each of you today.

Our First Visit with Dr. Grant

We saw Dr. Grant, the GI specialist recommended to us from Dr. Metz, yesterday. He was worth the wait. We knew they had moved their schedule around to squeeze us in at the end of the day, so we were prepared to get comfy in our little exam room. He was awesome. Down to earth, frank, and very humble about the trials and tribulations EB parents endure in NICUs. He really got it. He is one of the senior gastrointerologists at CHOC, so he's seen quite a few EB babies in his day, so he was very familiar with everything from the start. It was SO NICE not having to educate him on ANYTHING!

He quelled some of our concerns about esophageal issues, and gave us a 7 step plan for dealing with Bella's reflux and gas issues. Bella is so irritable and crabby, that she is either sleeping or screaming, and that is wearing us out. It also puts her at risk for more blisters, because when she throws a fit, she bangs her hands, face, and legs and gives her self new blisters each day.

The 7 step plan allows us to add/drop out different treatments without having to see him in the office. We can just call in requests, and it will all be in Bella's chart. The guy was like a dream. Most of all, he spent a lot of time with us. We were his last appointment of the day, and he easily could have blown us off, but instead, he got really into her situation, and took his time talking about pros and cons of all different types of treatment options.

We feel a lot more comfortable having Dr. Grant in our treatment team.

Other good news...

Last Tuesday, Bella weighed in at 9 lbs. Yesterday, she weighed in at 9 lbs. 12 oz! She is 70th percentile in weight, which is HUGE, as malnutrition is one of the big three killers of RDEB babies.

Kelly, the home health nurse, called today to see if we needed her to come out and see Bella this week. Ang and I looked at each other and said, "No." Kelly was thrilled to hear that. She could tell that we have come a long way in a short time in terms of caring for Bella on our own.

Next Tuesday, Bella goes in for genetic testing. They will draw her blood (yikes) and then, if need be, they will do testing on Ang and I. We are still in the dark as to whether Bella has the dominant or recessive strain of dystrophic EB. Most likely, it's recessive, but we need to know one way or the other as it informs us as to the possible progression of her condition.

Meanwhile, our church continues to feed us, sending food every couple of days. We are so lucky to be surrounded by such abundance of love and support. I told my minister that I felt a little guilty receiving so much...we're used to being on the giving side, not the receiving side. Part of me feels like we should be able to feed ourselves...we're grown adults...but Dennis put me at ease real quick. He said, "That might be true, except that you deserve it." Then he just smiled at me, and that smile told me everything was okay.

We have been making dietary changes bit by bit, buying all kinds of mysterious things that we have never eaten/used before. Most of it has been strikingly good. There are a couple things that were misses, but for the most part, the transition is going slow and steady, and Ang has really charged ahead trying to cook and eat with new items every day. Today was my last day of 100% caffeine coffee. Tomorrow, I begin weening myself off by drinking half regular half decaf for about two weeks, then....oh man...decaf for two weeks, then....[shudder] off the coffee for good.

Thanks for all your encouragement/feedback/support as we rethink our lives to make the best environment possible for Bella. We know these changes will enhance all our lives ultimately, even if they seem a bit weird at first! We love you all and thank you again from the bottom of our hearts for sticking with us through this journey. Your love passes through us into Bella, and let me tell you, at 4:40 this morning as Bella was yelling at me while I did lap after lap with her in my arms on the stairs to try to calm her down...I needed every ounce of that love! :)

God bless.

What a first month!

Bella turns one month tomorrow. Whew. What a first month! I know the 1st birthday party is more for the parents...can we have a first month party for surviving this past month??? :) I foresee things getting easier in the immediate future, however. God willing, Bella will continue to be stable and relatively calm at home. Developmentally, she won't be doing too much in the next couple of months in terms of walking/crawling/eating/etc. so that should give us time to really get her healed and strong for when she is ready to become more physically active.

What a gift she is. I know that breakthroughs in health and vitality are on our doorstep because of our devotion to creating the optimal nurturing environment for such a fragile being. Dr. Young puts it simply. Think of a fish in a fish bowl. The health of that fish is greatly determined by the cleanliness of the water it lives in. Now think of your body as the fish. Your body is 70% water. Bella's is much higher...closer to 90% water. We (our cells, organs, tissue) live in our own fishbowl. If you don't keep the water clean, the fish's health suffers, right? Same with the fluid in our body. Sounds logical. Sounds simplistic even. Yet, ignorance is BLISS. As I continue to drink acidic beverages daily (soda, coffee) and eat foods my body is not designed to digest (animal protein, sugar, yeast), I wonder why I am exhausted by 3 pm every day, I have chronic pain, and get recurrent headaches, injuries, and soreness.

Thinking of Bella's optimal health has made me think of my own in a new way, and as I learn more each day about nutrition, I am saddened a little by what I am choosing to give up, but yet inspired to finally honor my body as it is designed. I see a healthy family full of vitality and strength: physical, emotional, and spiritual strength. That's worth making some changes for. My affirmation is, " I am restoring the structural integrity of my body, mind, and spirit."

For example, last night, we switched to cloth diapers. WOW, THEY ROCK! They are so vastly superior to disposable...I can't even imagine Bella (or us) suffering one more day in those awful plastic nightmares. Not only are the cloth diapers 100% softer, and more comfortable, Bella's butt looks better today than it has since she popped outta her mommy! No more lathering aquafor all over diapers and bottom every diaper change. No more blisters on her thighs from velcro. No more ripping out inner elastic waistbands 10 times a day. No more cutting oragami shapes outta the velcro closures so they won't cut her legs any more. That stuff was L-A-M-E.

So, I am heartened by this first lifestyle change going so well. I look forward to more of that.

Other news...

Yesterday, I had to pick my first fight for Bella. Nothing like trying to get an appointment to see a referral. The referral was for a GI Doc, an integral part of a DEB patient. The customer service rep who was handling the scheduling tried to explain to me that the Doctor was booked through August, and I would have to see another GI Doc, and that they were all the same. Bella could see one next week. If they are all the same, why is Dr. Grant booked through August and the others available next week? I wouldn't have it. I simply told the gal, "NO. That's NOT how this is going to go. You are going to figure out a way for my daughter to see Dr. Grant. period. " "Well, I'll have to get my supervisor on the line." She quips. "Fine with me." That was 25 minutes into the call. Within 5 minutes, the supervisor understood the situation, said she would email the Doc and see what she could do. She called back today to say they moved their entire Monday schedule up so Bella could get in at the end of the day. Yeah, this Monday.

Pick your battles, folks, and never let ANYONE sitting in a call center tell you how it's gonna go.

So, Monday we go to the GI to check out Bella's reflux issues. Reflux comes on early and often for DEB kids, and she has been having a lot of symptoms, so no time to waste. She doesn't need another day of reflux damaging her esophagus causing any unnecessary scarring.

Still other news...

I got in touch with a woman locally who heads an EB support group who invited us to an EBMRF picnic in Malibu this Sunday! EBMRF is the west coast version of DEBRA, which is based in New York. It's a hike, but it's being thrown by David Arquette and Courtney Cox, so...pack the minivan! We haven't made up our mind yet. Malibu is a hike from Orange and we haven't set out on a trek away from home for that long yet. We'll see. I think it would be cool to plug in...but I'm the networking junkie in the family.

Oh yeah, Bella sat through her dressing change today AWAKE for just about the whole thing! Her foot IS SO CLOSE TO BEING CLOSED!!! Aw man...it's such a victory. Anyhow, her being able to chill out awake was a good sign that since she pretty much has no open wounds, the dressing changes are not as painful for her as they were, say, two weeks ago, or even last week. C'mon, Bella! Heal, baby, heal! Heal, baby, Heal!

Okay. Time to go pass out. Bella is out cold after being up both of the past two nights. I can handle one-all nighter okay, but back to back...I'm not the night owl I once was. This rock star is in retirement!

Best to you all. Thanks for the continued love and support. It is a bit overwhelming...but we LOVE it and welcome the overwhelm! Thank you for contributing to us so generously with your prayers, intentions, thoughts, emails, cards, food, errands, and donations. I learn of a new church EVERY DAY that has added Bella to their prayer list. Literally from coast to coast, people are praying for our Bella. We are so touched and inspired by this. It gives us the strength to stand in the face of DEB and continue to proclaim...say it with me...

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."

May the unending abundance that is God's love be with each of you this weekend.