Progress

First, let me apologize for the delayed update.  Now that Tristan is in recovery, I have less time to be on the laptop, but that means more time with him!

Friday was a good day.  We were able to get a handle on his pain control (except when picking him up or trying to move him.)  The ortho residents changed the dressing - I am amazed at how much straighter his back is now.  He had some great milestones on Friday.  I was able to hold him in a chair and not the bed.  The nurse took off the majority of his monitoring leads, which makes it easier to move him around.  Tristan sat on the edge of the bed AND he took three steps.  Later in the evening he took his longest walk, yet about 25 feet.  Afterwards, Becky said he was exhausted and cried "up" and she had to carry him the last few feet to his room.  We are amazed at this progress, we were under the impression it would be another day or two before he really started to move.

Saturday, started off really rough. I got to the hospital about 7:30am.  Becky has spent every night with him.  I have tried to get back to the RMH so that Addison had some normalcy with me there each night.  (Even though Aunt Debbie is with her.)  Anyway, when I walked into the room he started crying and then the crying turned to shrieking.  Apparently, Tristan's medicine was delayed a bit in the night.  (I don't know what the nurse was thinking to skip it just because he was sleeping.) The poor little guy was miserable.  I tried to hold him to comfort him, but that didn't seem to work.  Then, his diaper leaked and he peed all over me.  He had to be changed (and so did I.)  While changing him we noticed that his skin rash was getting worse.  He already has had some skin break down, but now he has gotten a skin rash.  We think he is allergic to the sticky pads of the leads and tape.  So along with some oral meds, he was also given a round of IV Benadryl....he was OUT!  He slept for about 4 hours and woke up a new little boy.

Upon awaking he watched the Spartan's go to 8-0 and then a little DVD of Thomas the Train, who he has fallen in love with this trip.  (Guess, I'll be investing in train stuff for Christmas.)  After the movie, Aunt Debbie cuddled up with him a bit...and then she asked him to show her the playroom.  He agreed to be moved (even though it hurts) and off we went.  A few minutes later he found a vacuum and just had to push it...I think he pushed it down the hallway at least three times.  Amazing!  Later, he was able to get into a kneeling position on his own to play with another toy.  He still has a solemn expression, but you can see some of the pain in his big brown eyes disappearing.  He isn't quite back to his old self, but I see some glimpses.  He is also starting to say a few more words again and wants to communicate a bit more.  I know we did the right thing for him by bringing him here, but seeing him walk straighter makes me feel even better about it all.

It appears that things are still on schedule and we'll be able to make it back on Tuesday afternoon to Michigan.  We appreciate all of you continuing to support us and for your prayers for our sweet little man.

Ouch

This post will be short and sweet.  Not much new to report.

Tristan has now been out of surgery for over 24 hours.  He has shown good, but slow progress.  Pain management seems like it is finally working and he is able to be as comfortable, as possible.  We are able to get some smiles out of him.  He was actually most alert when his Big Sis, Addison came in to seem him.  She brought him an octopus from the aquarium she visited with Aunt Debbie today and he seemed to enjoy that.

He is taking food and drink orally, as well as his pain meds.  This was a crucial point for him to get to so he would be able to move out of the PICU.  They have ordered a bed on another floor for him, but we are still waiting to move.  I don't mind waiting, though because Tristan gets more one on one attention while in the PICU.  All the nurses think he is adorable, which he is, as he looks just like my wife.

His favorite word right now is "no".  And it is so sweet when he says it.  So every time we ask him to take one more bite or one more sip, all we hear is...."no", but sometimes we can still coax another bite or two. He seems to really enjoy sliced bread and apple juice today, oh yes, and some of Becky's cheese popcorn.

The nurses have removed more tubes/wires from him today.  His arterial line was removed and his EKG tabs have been pulled off him.  He still has two IV lines and some leads monitoring his respiration's, heart rate and blood pressure.  He had a low fever today and his heart rate has been staying about 140 today, which we would prefer to see a bit lower.  As the pain goes away we assume his heart rate will drop to normal.

He is a tough little kid and with all the blessings from God he has been able to start this journey.  We thank you all for your continued support and all the wonderful thoughts/emails you sent our way.  We hope to post tomorrow from a new floor in a new room at this wonderful children's hospital in Philadelphia.

Good night and God bless,
Adam, Rebecca, Addison & Tristan

Surgery Day Timeline

We thought we would do the post a little different today.  Since we are getting updates on a regular basis we are going to share those updates.  Of course we won't post them all until Tristan is out of surgery, but at least you can see how the day went.

Wednesday, October 20th 2010.
4:30am - Alarm goes off.  I (Adam) am the only one out of bed and was able to shower and use deodorant.
5:01am - Becky gets up.
5:25am - Kids wake up and get dressed.

5:25am - Out the door at the Ronald McDonald House and into a taxi.
5:40am - Arrive at the Children's Hospital of Philadelphia (CHOP).

5:45am - Arrive at Surgery Reception.
6:10am - Becky and Tristan go back to prepare for surgery.
6:15am - Addison and I wait to see Tristan before surgery.
6:20am - Addison and I wait to see Tristan before surgery.
6:25am - Addison and I wait to see Tristan before surgery.
6:30am - Addison and Iwait to see Tristan before surgery.
6:35am - Addison and I wait to see Tristan before surgery.
6:40am - Addison and I wait to see Tristan before surgery.
6:45am - Addison and I wait to see Tristan before surgery.
6:50am - Addison and I wait to see Tristan before surgery.
6:55am - Addison and I wait to see Tristan before surgery.
7:00am - Addison and I wait to see Tristan before surgery.
7:05am - Addison and I wait to see Tristan before surgery.
7:10am - Addison and I wait to see Tristan before surgery.
7:15am - Nurse finally comes to get Addison and I. She breaks some rules, but understands that this is a major surgery and that Addison needs to see her baby brother before he is taken to surgery.  (Whew, it took about eight times typing that before I realized that I should copy and paste.)
7:20am - Tristan gets "giggle juice".

7:35am - Tristan is wheeled away to surgery.

7:36am - Addison, Becky and I are escorted to the surgical waiting area.  Adam takes "giggle juice" (kidding, I was seeing if you were still reading or not.)
8:30am - First update from operating room:  Tristan is asleep and the breathing tube is in place, surgical team are still trying to get his body comfortable, taking into account his contractures. 
10:00am - Second update from the operating room:  Tristan is doing great; procedure started at 9:25am and they are surgically getting to where they need to install the VEPTR.
10:15am - "Missy", Becky's sorority sister arrives to help us during this waiting period. 
10:25am - Addison and Adam walked two blocks to the train station to pick up Aunt Debbie.
10:40am - Third update from the operating room.  The VEPTR has been installed and they are starting to close.
11:15am - Becky and I (Adam) meet with Dr. Flynn.  The surgery has gone faster than expected; and things went beautifully. The only real concern is for the hook that went into one of the lower vertebrae...Tristan didn't have enough skin to cover it completely. They had to use some of his muscle to cover and there is a risk of infection or skin break-down.  Good news: A longer VEPTR was used, which will mean the next major surgery will be pushed out further.
11:50am - Tristan is completely out of surgery and is moved to the PICU,  We are moved to a new floor and are waiting to see Tristan for the first time.
12:20pm - We (Becky and I) are able to hold Tristan's hand and comfort him.  He looks great.  A lot better than what we expected.  The pictures below show minimal swelling. Once he wakes up, the PICU team will start the pain management portion of his recovery.  Tristan is coming in and out and giving little bursts of cries, which is really tough to watch.

2:50pm - Dr. Flynn comes in to check-in and talk about curvature changes.  If you remember during our pre-op appointment, Dr. Flynn said that Tristan's spine was at 91 degrees (a ten-degree change for the worse since our July visit.)  Dr. Flynn hoped through surgery to at least stop the progression and at best get Tristan to 75 degrees.  (Enter GOD, The Great Physician) Post-op curve: 53 degrees!!!! (Tristan is coming home taller and will have much more room for proper organ growth.)

Today has been incredible and we thank you all for standing in prayer and support with us.  This journey is far from over as the recovery is tough.  We hope Tristan will be leaving the PICU in the morning and we'll update again tomorrow. 

God Bless You All!
Adam, Rebecca, Addison and Tristan

Pre-Op Testing

First full day in Philadelphia and I (Adam) have to tell you that I stink...if you were to walk past me, you would move a little faster hoping that I would not stop to talk to you. Now let me tell you why and yes, I know, TMI. When packing I laid out all of my toiletries on the bathroom counter in the new master bath. Lined them all up really nice, that's the OCD-side of me. Then along comes Becky, she comes in and packs up her stuff, but realizes she wants to smell like the man in the Old Spice commercial. So she grabs my "manly" scented deodorant and proceeds to use it on herself. Then she does something incredible...she actually puts something away...and she puts it in the bathroom drawer. Anyone want to guess what was not in my bag after I showered this morning?

So despite my stench, we were able to make it to all of Tristan's pre-op appointments. Becky actually let us takes a taxi because it was raining. First, was the appointment with Anesthesia, which went smoothly. Next, Tristan had some blood work and the brave little guy didn't cry when they took the two vials of blood. We moved onto x-ray films. (We are getting to know the hospital pretty good now.) Lastly, we were able to go to Ortho for our meeting with the nurse practitioner and finally Dr. Flynn to go over the surgery in detail.

The news from Dr. Flynn was that Tristan's curve was now at 91 degrees. Dr. Flynn said that Tristan's spinal curve was like "a car rolling down a hill just waiting to crash." In just three months, there had been a 10 degree change for the worse. We are very fortunate that God made it possible to have Dr. Flynn do this surgery sooner rather than later. Dr. Flynn had told us in July he didn't feel that Tristan could wait until 2011 for this surgery - and he was right.

Alright, onto the specifics of the surgery…due to Tristan's size, Dr. Flynn & team will only be putting in one VEPTR rod. (Eventually, they hope to do two.) This rod will attach to two ribs on Tristan's upper left side and also to a lower vertebra at the base of his left side. The rod will be titanium and will be able to expand as more surgeries are completed. They will do all of this through two incisions, but they will be detaching and reattaching muscle to get to the ribs, so recovery will be tough. One of the members of Flynn's team will be monitoring brain/nerve control throughout the whole procedure, so as to place the VEPTR rod in a location that will not create nerve damage. The good news (yes, there is good news) is that Dr. Flynn believes that the 91 degree curve may improve to 75 degrees upon the completion of this surgery!

We are to arrive for surgery at 6am tomorrow morning. It is likely that the surgery will begin by 7:30am. It will take approximately five hours to complete. Once Tristan is starting to come out of anesthesia, we will be able to see him. I have mixed feelings about this reunion. I know in my heart that I want to see him. But I also know that I am not prepared for what I am about to see my son go through. I am even tearing up as I write this. My prayer is that God will give me the strength to be there for Tristan and Becky.

Please join us in prayer tomorrow morning for Tristan, and also for every hand/eye in that operating room. I want God, the Great Physician, to be leading and guiding their hands as they take care of my son.

In the mean time, if any of you were worried, I was able to buy deodorant and take another shower...so the family is willing to associate with me again. The kids have actually fallen asleep together on the bed and Becky is grading papers for one of the many classes she teaches. We will spend the rest of the night having dinner and playing around the Ronald McDonald House. Maybe we'll go out and grab some gelato as a special treat for the little guy.

We will update the blog tomorrow evening after Tristan's surgery is completed. Knowing that we will be able to share with all of you how God held Tristan's hand and ours throughout this entire process.

May God bless you all for your faithfulness,
Adam, Rebecca, Addison and Tristan

Day Of Travel

Those of you that know what it is like to travel with children or a group, should appreciate this.

I (Adam) am a very organized and preplanned individual.  That's right - I have a plan.  I have had the travel information and documents prepacked in my bag for days.  My better half (Becky and the kids) on the other hand have no sense of staying on schedule when it is time to travel.  It's almost as if time itself stands still for them.  Maybe that is why Addison's idea for her Fall Harvest costume being a clock, set well with me.  I thought it might be a good lesson in time.  But as you can see from the photo below, the time is stuck.  Hmmmm...



tick-tock

Well, my goal was to have the car packed at 10am...we had it packed by 11am.  My goal was to be on the road by 11am, and we left at 11:15...so I guess overall that was pretty good.  The ride down to the Detroit airport went off without a hitch.  Thanks to The Edward (Becky's Dad) for playing taxi driver (and Uncle Tim, too!)  Once inside the terminal, we were able to check our luggage, get our boarding passes and drudge through the security line, all with enough time to have a lunch and board the plane.

Once on the plane, Tristan decided to show some major attitude and be the loud screaming kid for the first 40 minutes of the flight. After rows of people stared and some glaring Tristan finally passed out and slept for the remainder of the plane ride.  Whew!

Once on the ground in Philidelphia, I was able to contact the Ronald McDonald House (RMH).  When I did so earlier in the morning, they did not have a room available for us, but this time they did.  So our backup plan at the hotel was not needed.  Thank you Lord for this wonderful blessing.

Once we got our baggage, Becky decided to throw a wrench in my plans.  I had planned to have a shuttle take us to the RMH.  She decided that taking the train with two large suitcases, two car seats, four carry-ons, a stroller and two kids (one of which would not ride in the stroller) would be the best option (cheaper).  Once we got off at the University Center train staion, she said "Let's walk from here to the RMH."  So all of the items listed above, now were carried and/or pushed eleven city blocks. Of course as Becky would tell you, "we saved eleven dollars."   So I guess we saved one dollar per block.  

Side note to Ryland (my trainer) - I carried the two large suit cases and two car seats.  Hopefully, this counts as my workout for the day.  :-)

Once we arrived at the RMH, we have seen nothing but the warmest of hospitality.  We entered and heard music playing on the piano, and Becky was practically in tears with relief.  Upon check-in the kids were immediately spoiled by picking their own presents & books from the gift chest.  (Tristan picked an Ironman figure and Addison a little doll figurine.)  The facilities are great and there are two wonderful play areas for the kids.  Tristan had to be pulled away from the train table this evening.  In addition to playing, we were also well fed and have been blessed with one of the dinners furnished by volunteers. 



Tristan playing with the trains at the RMH.

  
Now it's time to get some sleep and prepare ourselves for all of Tristan's pre-op appointments tomorrow.  Our first one is at 8am and then we have another at 9am.  We hope to find out all the final details of the surgery and find out what time surgery will be on Wednesday.

Thanks to all for your continued prayers and all the wonderful notes we have received.

God Bless!
Adam, Becky, Addison and Tristan

Why we are here? Why is Tristan having surgery?

Many of you know Tristan’s story, but we thought it best to put it in writing so everyone knows the complete history.

It was February 2008 when we found out Becky was pregnant. It brought us much joy as we had been trying for our second child for some time. Along with the excitement, also came the worry of congenital defects. I (Adam) only have one kidney and with Becky having Congenital Contractual Arachnodactyly (CCA), we knew there was a 50% chance of our baby having either or both issues. At the sixth week of gestation, we started having ultrasounds and hoped to rule out any concerns.

It was during one of the many tests and ultrasounds that the doctors were able to begin to diagnose Tristan’s medical conditions. First, the doctor noticed that he had only one (solitaire) kidney, which also explained his single umbilical artery. Second, there were contractures of his knees and elbows, which confirmed that he had CCA. Third, an extra rib and hemi-vertebrae were found, which was something very unexpected. Although, finding all of these things in utero made us anxious, it also made it possible for us to specifically pray for the guidance in how to tend to Tristan’s long-term care.

In the months prior to his birth, we continued to see many medical doctors and planned for the possibilities of having a child with several medical conditions. When Tristan was born on October 7, 2008, he did spend 26 hours in the NICU and had several tests, but surprisingly although small was doing very well. The cardiologist saw him within his first few weeks and happily announced that she could clear him 100% for the minor heart issues found during his time in the NICU. The Ophthalmologist also cleared him after two visits before age one. After a few visits and ultrasounds with the Nephrologist (kidney doctor), the solitaire kidney proved healthy and to-date, he has been cleared until age four for follow-up.

The Physical Medicine/Rehabilitation doctor, who we saw prior to his birth, continues to monitor Tristan for his CCA. Since about one month of age, Tristan has been going to PT/OT once a week. Part of this connective tissue disorder leaves Tristan without the ability to straighten his knees and elbows, but with nearly two years of PT, we have seen significant improvement in Tristan’s ability to straighten his legs. We are now starting to discuss a more in-depth plan for getting his elbows to extend as well. At this time, continued therapy to help curve the affects of the CCA is our plan. CCA can have spontaneous improvement and we pray that he will be able to have full motion of his joints in the near future.

Tristan sees an Orthopedic surgeon for his most threatening condition, a hemi-vertebra (and extra rib.) By definition, the hemi vertebra is “a vertebra that is incompletely developed on one side”. Therefore, it creates a “wedge” in the spine that forces scoliosis. Tristan was born with a 30-degree curvature in the spine, because of this condition. In less than two years, the main curvature has worsened to 81 degrees, while adding two other smaller curvatures to compensate and to keep his head upright. Because of the rapid increased degree in curvature, surgery has become a necessity. Without the surgery to stop the curvature, we run the risk of the spinal column being pinched, which would leave Tristan paralyzed.



3D Composite of Tristan's Spine taken 9/7/2010



The first initial thought was to remove the hemi vertebrae and fuse the surrounding vertebra. This option would have shortened Tristan’s trunk and could have caused organ displacement as he grew. After Tristan’s Orthopedic surgeon reviewed the case more closely, it was determined that the fusion would not create enough stability. So after consulting with many other surgeons across the country, a decision was made to install a Vertical Expandable Prosthetic Titanium Rib (VEPTR).

There are only a handful of surgeons in the U.S. that do the VEPTR surgery, so in June of this year we began praying that God would lead us to the right surgeon. Although there is a surgeon at the UofM Childrens Hospital, which would be geographically convenient, our prayers and research led us to see Dr. Flynn at the Children’s Hospital of Philadelphia (CHOP). Dr. Flynn is the partner of the doctor who invented this procedure, which increased our confidence and if that wasn’t enough we also posed the questions to Tristan’s Orthopedic Surgeon here in Michigan, “If this was your child, and money and distance were not a question, who would you have do the surgery?” His answer was go out of state…see Dr. Flynn. Although everyone’s circumstances are different when faced with a decision of this magnitude, we are comfortable that the Lord has led us to the correct place.

We met Dr. Flynn in early July and we felt God’s peace immediately and knew we had met the doctor that would help our son. Since then we have been going to doctor’s to complete all of the pre-op care (including a complete pulmonary workup.) We have been making plans for the surgery, travel, and all of the post-op care. We leave for Philadelphia on Monday October 18 and Tristan’s surgery is scheduled for early morning on Wednesday October 20.

This first initial surgery will take about five hours. Tristan will need to be in the NICU for two to three days after surgery, and he will then remain in the hospital for another three to five days, assuming there are no complications. Once the VEPTR is attached to Tristan’s rib and vertebra, he will have to return every six months to have adjustment surgeries. This means we are looking at approximately 20-24 surgeries over the next twelve years. Every two to three years, Dr. Flynn will need to install a new VEPTR. The goal is to get Tristan to age twelve or thirteen when his spine will be fully matured and then he will have a final corrective spinal surgery.

It has been a long challenging road, and we know it is just beginning. We are so very humbled by all of the prayers and support we have received already and thank God for placing each of you in our lives. We ask earnestly that you will add Tristan’s name to your daily calendar on the day of his surgery and the days that follow during his recovery. Thanks for joining the blog, and we will continue to update Tristan’s progress during this incredible process.


God Bless You All!
Adam and Rebecca West
Addison & Tristan