A change in surgery

We visited CHLA on Monday for Campbell’s pre-op appointments and surgical consults. During that time, her neurosurgeon determined her shunt has an occlusion.

Due to the risk associated with the cranial surgery, the procedures planned for April 23rd are on hold and instead, Campbell will have a shunt revision.  Campbell’s brain has been circulating some CSF on its own, which is good news.  The hope is the shunt revision followed by helmet therapy might offer similar results with lower risk.

While sometimes difficult to process change in the moment, we know Campbell’s surgeons have her best interest in mind and want good for her, and we feel confident moving forward with this plan of care.

The shunt revision surgery will be Friday morning, April 25th.

When Campbell was born, Joel and I decided that we would give her every chance, within reason, of a “normal” life. This meant if we need to take some risk, so be it.  What we’ve recently had to weigh is risk and reward.

When facing a weighty decision, we’ve imagined ourselves sitting across from Campbell at the table when she’s 20, and her asking us why we did or did not take a particular course of action that impacted her health and life.  We want her to know that we’re giving her every chance at living a larger life, and that fear isn’t holding us back.  The reality is we have sat in rooms that have changed us, regardless of what decision was reached.

A wise friend told us if Campbell is able to struggle through the issue of her care with us, she will be able to struggle through it with God. Our job as parents is to make the best decisions we can at this point in time. We believe that God is sovereign and that He will accomplish his purposes, and so once again, we commit our daughter, her care, her team, and her future to Him as we know He loves her most and best.

Commit all you do to the Lord, and your plans will be successful.
(Proverbs 16:3)

Campbell’s Pinkies and Thumbs

Campbell’s first syndactyly surgery went very well.  She is tolerating the casts and is adjusting to not having her hands available for playing and eating.  Her new method of playing involves dragging her casts across her light-up toys to see how much noise she can make.

Here are Campbell’s pre-surgery hands.  I love these sweet little mittens and already miss them.

Bright and early last Friday, we headed to CHLA for her surgery.  Campbell was the first case, and she and Daddy played peek-a-boo in pre-op while we waited.  When it comes to smiling, she lights up the room.

After surgery, Dr. Panossian met with us to discuss the procedure.  He showed us a few pre-casted pictures of Campbell’s hands, and they looked wonderful.  He and his team were able to release her thumbs and pinkies and set the remaining three fingers straight with a pin.  The pin will come out as the hand heals, and having the fingers growing straight will help with future release surgeries.

We are excited for the cast removal next week and to see her new fingers.  After Dr. Panossian removes her casts, the OT department will fit Campbell with a splint which will help keep her webspaces deep while her hands continue to heal.

Dr. Panossian also cleaned up the nail beds on Campbell’s feet, which should ward off ingrown toenails as she grows.

After plastics finished her hands and feet, Dr. Maceri scoped her ears and put in ear tubes.  He said she had a lot of thick fluid behind her ear drums.  Since that procedure, Campbell has begun talking to us more, and becomes animated when we play music.

By Friday morning, Campbell was feeling much better.

We got the okay to head home.  I wasn’t thinking about her casts when I packed her clothes to wear home, and her outfit didn’t fit over them. But having no clothes didn’t faze Campbell at all.  She proudly wore her Tiny Superheroes cape through the lobby and on her lap for the ride home.  I wish I could say it’s the first time our girl has ridden home wrapped only in a blanket and diaper, but it’s not.  Clothes are highly overrated, right?  

Christian, Kyle, and Colton were so excited to see their sister.  The first question was, “When can we sign her casts?”  Kyle practiced his name because he wanted to write it “just right”.  Each boy also wanted to snuggle Campbell in his lap for a few minutes.  These boys really miss their sister.

Overall, recovery is going well.  The days have been good; the nights have been a little rough, but each one is getting better.  Sleeping would be tough for me, too, if every time I moved, I hit myself in the face with a cast.  Add a helmet, middle ear adjustments, and a natural propensity to sweat, and it’s easy to see why sleep is so elusive.

Today, we’re heading back to CHLA for a CT scan to check out the progress of Campbell’s skull.  Our hope and prayer is that she is still a candidate for cranial surgery.

January Update

January – Off and Running

After a quiet December, Campbell is catching up with therapy and doctor appointments, and 2014 looks to be a busy year.   She is such a sweet and content little girl.  Campbell is working on sitting, and is most happy when playing with her toys, watching her brothers, or being outside.  The boys provide tons of entertainment for her.  All three are doing well and love on their sister often.

During the past two weeks, we’ve made a number of trips to LA to meet with plastics, pulmonary, ophthalmology, and orthopedics, and now have a revised surgery plan.

Hand Surgery This Week

This Thursday, January 23^rd, Campbell will have her first syndactyly release surgery to begin the process of separating her fingers.  Her hands are considered “mitten” style with a free left thumb and a webbed right thumb.  The rest of her fingers have bone fusion and are classified as complex syndactyly.  Because of the amount time needed for this first hand surgery, Dr. Panossian recommended waiting to separate her toes.  Also on Thursday, an ENT will scope her ears and possibly place tubes.  If all goes well, Campbell should come home on Friday.

After surgery, Campbell will be in arm casts for two weeks.  Once the casts come off, she will have daily dressing changes.  Other parents have told us that syndactyly release surgeries are most difficult due to painful dressing changes.  We are praying this time passes quickly and that Campbell will be pain-free during the follow-up care.

Cranial Surgery

After meeting with Dr. Urata, the plan for Campbell’s cranial care is to address her head size/shape through surgery in next few months.  Her shunt is working and the hydrocephalus is stabilized, which is very good.  However, her head size is still too large and there is quite a bit of cerebrospinal fluid (CSF) between her brain and skull.  At this point, surgery is her only option.  This procedure will be the most intensive one Campbell has faced, and it carries a fair amount of risk, but the option of not doing it would create life long effects making future surgeries less effective and affecting her quality of life.  Due to the plasticity of the brain and how it retains water during the first months of life, this surgery is only an option for about six more months.  Campbell is scheduled for a CT scan in a couple of weeks and a return visit to see Dr. Urata in February.  If she is still a candidate, she’ll be scheduled for surgery.

Because of the upcoming cranial surgery, Dr. Borchert in Ophthalmology recommended moving her eye surgery to correct the strabismus to later this year after the cranial work is finished.  Campbell’s eyesight continues to improve, and she’s using her eyes together more often, still typically in the downward gaze.

This month, Campbell’s OT and PT is transferring back to CHOC, and she’ll receive vision/infant stim from Blind Children’s Learning Center and the Braille Institute.  Campbell loves to play and lights up with her therapists.   We feel very blessed to have such quality therapists caring for our girl.

Team Campbell

The San Diego Half Marathon is on March 9^th, and we’ve put together a team to raise awareness of Apert Syndrome.  Members of Team Campbell have been training (both walkers and runners), and if you would like to join us, it’s not too late.  The race offers a half-marathon, couple half-marathon, and 5k option. Please email campbelltaylorjohns@yahoo.com for details.

Prayer request

• Thanksgiving for overall good health outside of surgeries and for new insurance.
• For Thursday’s hand/ear surgery:
  • Campbell would remain healthy between now and then
  • for the surgeons, nurses, and staff who will be caring for her
  • the skin grafts would be successful and the release go well
  • for healing – that infection would stay away and Campbell would be pain-free
• For the upcoming CT and cranial surgery that Campbell would remain a candidate for this procedure and for all involved

Thank you all so much for your prayers and support!

Our Tiny Superhero, Super Campbell

Tomorrow is surgery day.  Campbell is scheduled as a first case which means check in time is 5:30 am.  Admitting and pre-op procedures usually take a couple of hours so by  mid-afternoon, we should be able to see our girl and her new head.

While Campbell will undergo several surgeries in the coming months, this is the main cranial surgery until she’s around age eight.  Tomorrow’s surgery is a calvarial vault remodel (CVR).  Several people have asked about the specifics of this surgery, so here is the broad view of what she’s facing tomorrow.

There are typically three teams of doctors involved in this surgery: plastic surgeons, neurosurgeons, and anesthesiologists.  During the CVR, the neurosurgeons go in through the same headband-like incision from her distractor surgery and open up her cranium.  They remove the part of the skull to be remodeled and hand it to the plastic surgeons who have (in basic terms) a carpentry set in the OR.  While the plastic surgeons carve and reshape what they want her skull and forehead to look like, the neurosurgeons keep her derma and brain hydrated.  The neurosurgeons then manipulate her brain into shape they want, the pieces of the skull go back on, and the plastic docs build out an eyebrow bone. The anesthesiologist have the happy task of making sure she keeps breathing and feels no pain.

Campbell will go first to the PICU and then to the floor.  Blood loss and swelling are common with the CVR, and swelling usually peaks around 72 hours.  With no complications, Campbell will spend four to five days in the hospital.

During the CVR, the doctors will give Campbell a head that is closer in size to that of an elementary kid.  As she ages, she’ll grow into her head size.   By the time she’s seven or eight, she’ll be ready for the next stage and surgery which includes a mid-face advancement.

A couple of weeks ago, I went to visit the Thomas family.  Riley has Apert and her mom Summer shared Riley’s CVR photos with me.  I am glad she did to help prepare me for what’s coming in the days ahead.

On Monday, Campbell saw her pulmonary doctor to go over her sleep study.  While she seemed to do well, the results were inconclusive due to a fire alarm during the study.  Campbell didn’t sleep as long as needed so she’ll repeat the study after her first birthday.

See,  Daddy, I’m breathing just fine.

As we head into tomorrow, we are keenly aware that we are not traveling this road alone.  Friends, family, and sometimes strangers have come along side us, offering care and support. Our friend Jeff gave blood that will be used tomorrow – an amazing gift!  We’ve received notes and prayers, and while we haven’t been able to respond to each one, we are still very much grateful.  We also have been blessed with wonderful caregivers and therapists who love on Campbell week-to-week.

Amanda loving on our girl

Campbell and Shelli

Our friend Bryan nominated Campbell as a Tiny Superhero.  Tiny Superheroes was started by a woman who has a heart for kids facing medical challenges and disabilities.  Recently, ABC News featured the organization during its nightly broadcast.  Thanks to Robyn, the founder, and Bryan, Campbell’s cape arrived in the mail.

Campbell has always been our superhero.  Now she has the cape to wear!

We will be updating Campbell’s Facebook page with her progress.  You can find it here.

Joel and I kindly ask for your prayers.  For our superhero as she fights, for the doctors, nurses and staff as they tend to her, and for Joel and me as we wait. May God touch our girl with tender mercies and healing.

Upcoming Surgery dates

Summertime is in full swing at the Johns’ house.  Swim lessons, therapy appointments, and family visits dot the calendar and the days are passing quickly.  We have the dates for Campbell’s next two surgeries, August 14th for her cranial vault remodel and October 3rd for her first finger and toe release.  Both surgeries will be at CHLA.

The August 14th surgery will be a cranial vault remodel with an orbital rim advancement.  During this surgery, the surgeons will be reshaping Campbell’s skull as well as building out her forehead.  While it looks like Campbell has a large forehead, her brow bone (aka supraorbital ridge), is actually pushed back into her cranium.  I can reach up and feel the bone underneath my eyebrows, but Campbell’s bone appears missing, buried deep in her skull.  Dr. Urata and his team will build out an eyebrow ridge and give her a forehead while reshaping her head and giving her brain room to grow.

We’ve been told by other parents this surgery is bittersweet.  Campbell’s appearance will change and the sweet face we love will look quite different.  Overall, this is a good thing, as this surgery is an important step in the process to a more normal appearance. After this surgery, Campbell’s next cranial surgery hopefully won’t be until she’s around eight when she’ll undergo a facial bi-partition and mid-face advancement. Even though I’m excited about her progress, I’m also sad and have spent significant time studying her features, memorizing what she looks like.

This surgery is a scary one for us as parents.  Dr. Urata, Dr. McComb and their teams are quite adept but there are still risks for Campbell.  The surgeons will be cutting and reassembling her skull-cap, reshaping her brain, and building her a new forehead. (You know, fairly straightforward, common procedures for experienced surgeons.  But very uncommon for parents like us.)  Campbell is at risk for blood loss and she will revisit the PICU for recovery.

As the anxiety rises, we have the opportunity once again to trust the hand of the One who has brought our girl safe this far.  He has been faithful in the past, and He will be again. Our job as parents is to lean into God’s wisdom, peace, and guidance and care for our girl the best we can using the gifts He’s given us.

We kindly ask for prayers for Campbell’s surgery on August 14th.  Please pray for Campbell, for her health and well-being as well as for her medical team, the hospital staff, therapists, and all those who she will meet as a result of this surgery.  Thank you for your support and your prayers!

Photos: Post/Pre-Surgery Week

Campbell is set to head back to the OR tomorrow morning to replace a distractor that malfunctioned.  She’ll be the first case in the morning, and this surgery should take less time than her last one.  Once she’s out, she’ll spend a night in the PICU for observation and then hopefully move to the floor for a few days while she recovers.

The past week has been emotional for Joel and I.  It started with saying, “See you soon” to Campbell last Wednesday when she went into the OR.  She was so brave.

Here she is right before her surgery.  As you can see, her head ends right behind her ears and is quite flat.  After surgery, it will be more full in back and be able to grow as her brain grows.

Campbell was patient 3334.  We tracked her progress in the OR on a screen in the waiting room.  At the time of this photo, she was in the OR almost 6 hours and still had another hour to go.

Jamie Cook and her daughter, Callie Anne, cheered on Campbell from Arkansas.  Callie Anne has Apert Syndrome and is two weeks older than Campbell.  Her mom, Jamie, has become a friend, and I look forward to our families meeting someday.

Here’s our girl after surgery, settling into the PICU.  She came out of the OR with quite a few IV’s.

Here is Campbell on the day after surgery “celebrating” her four-month birthday.  The swelling has begun to set in but is mostly in the back of her head.  We couldn’t ask for a sweeter baby.

Here are some of Campbell’s awesome nurses.  The staff at CHLA have been amazing and are taking very good care of our girl.  We feel blessed to be here.

Several friends have come to visit Campbell.  Here are a couple of photos:

We’ve run into two Apert families in the lobby, the Reiss Family and the Weissberg family, both of whom see some of the same doctors as Campbell. Tania and Miles came up to visit Campbell.  Miles is a precious boy and was so excited to say hi to Baby Campbell.

Occasionally, Joel and I took a break from the hospital to get some fresh air.  It was wonderful to have a few quiet moments to regroup.

A child-life specialist came by and lent us some toys for Campbell’s crib.  She’s been exploring using her hands (with a little help).

We added a few bright toys to the top of her crib.  While we’re not sure how much she sees, here’s her view:

Some more pictures of our little girl.  She’s quite a charmer.

Last picture of our girl. Here she is with her “antennas” wrapped up in her cocoon.  She has gauze strips over her distractors to catch any drainage (the gauze is gone now.)

Thank you, friends, for your support, messages, and prayers. Tomorrow is a new day, full of promise and possibilities, and we are grateful to have people like you in our lives.

A bump in the road

So the small complication this morning turned out to be more complicated than we thought.  Campbell’s right distractor malfunctioned and needs to be replaced in the OR.  Because the surgery requires the scheduling of multiple teams, it most likely will be next Wednesday. So we are at CHLA until then.

It’s a bummer that the distractor isn’t working, but this is one bump in a very long road for Campbell and our family. We are very thankful that this is fixable. Dr. Toranto said this surgery should be much shorter than the first.

Currently, Campbell is resting comfortably and is the most alert she’s been in days. We’ve been working on pain management, and with the exception of the distractor, Campbell is recovering nicely. A childlife specialist visited today with some brightly colored toys which we’re using for her vision therapy. The staff here at CHLA has been wonderful.

Our plan now is to work towards surgery on Wednesday.  Thank you for your support and prayers.  It means so much.  Have a great Friday night, friends!

A small complication

Good morning!  It’s Tiffany Ann.  Thank you so much for your prayers and messages the past few days.  We appreciate them so much!

Campbell moved from the PICU to the floor around 11:45 last night.  Since moving, she’s had a little nausea, probably due to the jostling of the move and changes with feeding and pain medications.  We expect that to smooth out today.  The swelling has begun to go down, and while a little puffy, Campbell seems to be more like her sweet self.

We ran into a small complication this morning when the doctor came to turn her distractors.  One distractor turned smoothly and the other is giving a bit of trouble, so the team is conferencing to get a game plan of next steps.  We hope to know more later today.

The distractors themselves look like little antennas protruding from her temples.  When she’s swaddled in her pink blanket cocoon, she resembles a cute little chrysalis.  She is our precious, beautiful butterfly.

We’ll keep you updated as we know more.

T-minus Three Days

In a few days, we’ll kiss our baby girl and hand her over to a skilled surgical team.  We’ve done the surgery-thing before; twice, actually. But this time is different.  A different hospital. A different team. A different kind of surgery.

After we learned of Campbell’s Apert diagnosis, I spent countless hours researching doctors, hospitals, and insurance.  I spoke to parents, nurses, and therapists – even children who themselves have undergone surgeries – in effort to understand the syndrome and the challenges it presents. This research continues to help us set Campbell’s course of care, and that is a good thing.

But the research has served another purpose as well.

The shadow-side of the research is it makes me feel like I am in control in an uncontrollable situation. And I like to feel in control.  Control gives me a false sense of security that I am able to protect myself and those I love from the bad things in life.

Control also enslaves me.  My household, my children, my daily activities become a breeding ground for maintaining order and process – where perfection is the benchmark and I never quite measure up. At my core, this is not how I want to live.

In this season, God is teaching me through life with Campbell that I don’t control nearly as much as I think I do, and that I am going to find myself in vulnerable situations because that is life.  I can’t control life.

And that idea is very unsettling to a control freak like myself.

It’s unsettling unless I see that God is in the driver’s seat and it’s His responsibility to work all things for good, not mine.  Not only do I not control life, I never was meant to – that’s not part of God’s design.  My responsibility is to let Him lead and exercise choice rather than control.

I have the choice to cling to the heart of God who has been faithful to me.

I have the choice to submit to the One who loves my daughter more than I do.

I have the choice to lean into the peace that passes understanding.

I have the choice to admit my weakness and let His power and strength shine.

On Wednesday, a very good team of surgeons is going to open up our daughter’s skull, expose her brain, and attempt a modern-day miracle to give her a chance at a better life.

Placing our daughter into the hands of these surgeons is one of the hardest things I will do as a mom.  Just thinking about saying goodbye as she wheels to surgery leaves me in an emotional puddle. She’s my baby, and I am powerless over what transpires in that operating room.   But God is not.

The temptation is to place my trust in the surgeons, in their skill and education.  While these doctors, respected and renown, will use their gifts to heal part of Campbell’s body, they are incapable of healing her soul. Only God can do that. Ultimately, my trust is in the Great Physician, our forever Healer.  It is only through Him that I, or anyone else, can have hope to face today.

A side note: Our church, Eastside Christian, recently began a message series called Hope Rising.  If you find yourself wondering about how to have hope during life’s difficult times, please check out the free downloads here or come visit (if you’re in the Orange County area.)

Pre-Surgery Week and Smiles

On Monday, we met with Dr. Toranto, Dr. Urata’s fellow, who walked us through Campbell’s surgery for a posterior cranial vault with distraction osteogenesis.

From what we understand, the surgical team will make a headband-like zig-zag cut and place metal distractors, or spacers, along the scar.  As the scar begins to heal, they/we will turn the distractors (which sound like glorified hex screws), which will in turn lengthen out the scar and allow the bone to fill the space.  After the distractors create enough space, the lengthening process stops and the distractors remain in place for three months while the bone finishes filling in and hardens.

This surgery will focus primarily on growing bone in on the posterior (back) of the cranium while giving her brain room to grow.   The cranial vault surgery later this summer/early fall will address the front part of her skull and the overall head shape.

Here are a couple of articles describing the posterior cranial vault with distraction surgery (one with photos showing the stages of distraction and one with photos of the surgery.  Please note: some of the photos are a little graphic). As with all craniofacial surgeries, each patient and situation is unique so Campbell’s surgery and results may be different.

Dr. Toranto discussed the risks and desired outcomes with us, and while turning our daughter over to the surgical team is difficult, this surgery gives Campbell’s brain the space it needs to grow.

Also on Monday, we saw the craniofacial psychologist, geneticist, research aid, and Joel and I banked blood for Campbell’s surgery.

On Tuesday, we went back to CHLA and met with two doctors from Pulmonology.  Campbell’s sleep study results came back, and she did less than stellar. The study showed that Campbell has a lower oxygen baseline and her pulse ox rate dips significantly when she’s sleeping.  This could be due to the anatomy of her nose, her recent RSV, or some other combination of factors.  In any case, Campbell needs oxygen, especially at night, so she’s been outfitted with oxygen tanks and a nebulizer.  She’ll repeat the study in a couple of months to see if the surgery affects her oxygen levels, and hopefully, she won’t have to be on oxygen long-term.

On Wednesday, Campbell had an occupational therapy evaluation at CHOC.  The therapist said Campbell should be in OT, and she’s been place on a wait list for an opening in scheduling.  Currently, the wait is one to two months to begin OT services.

On Thursday, Campbell saw her vision therapist from the Braille Institute.  Every day Campbell seems a little more alert and her vision “window” is lengthening.

Campbell is scheduled for an MRI early next Tuesday morning and she will most likely be the first surgery on Wednesday.  She’s a difficult intubation and is small, so unless a younger baby needs surgery on that day, she’ll be the first case.

The biggest thing that happened this week was that Campbell broke into a full-on smile.  We were tickling her and the boys were singing to her about how pretty she is when she sneaked it in.  The whole family erupted in cheers.  We are looking forward to seeing more of that smile in the days to come!