Memorial Day

The night was slightly tough as we were both really worried about Jake's allergic reaction and spent most of the night checking on Jake's breathing to ensure the allergic reaction wasn't taking a more serious turn.   He slept fine and it appeared the medication was in control again as the hives dissipated.  The rest of the day followed normal form with the kids, a play date with friends and then returning back to the house.  Jake hasn't really shown major signs of his hand or hip limiting his big smile and joyful spirit.   Our concerns remain the cause of these allergic reactions and wondering are they all connected in some way, are they different reactions to different things.   We will take Jake to an allergist to at least get checked out even though they can't run more specific tests till he puts these allergy medicines behind him.  Jake will return to school today and should garner some attention to his cast with his classmates.  We hope there won't be any additional allergy complications and we can set our sights forward to his pre-school graduation and eventually cast removal in a couple weeks.

Jake's 5th Birthday

Well, there were many reasons to celebrate, being home, Jake feeling better and of course the boys birthdays.  Today was a happy day as the boys were excited to be able to finally say they are now 5 years old.  We hung out and played most of the morning and headed out for a late brunch with the family.  Both boys put on one of their gifts, superman and batman shirts complete with velcro capes.  Jake didn't really eat but was feeling okay.  We returned home to prep for an informal get together with the family to celebrate the bday and have some pizza and cake.  Everyone was having fun until we noticed Jake's left hand blow up around the knuckle area.  Our heats sank as we worried about a return of another allergic reaction.  We called the Dr. and gave him some benadryl but before long more and more hives appeared on his body.  We just gave him the steroid and he is sleeping in our bed so we can keep our eyes on him.  We will keep the epi-pen close and be on alert tonight to make sure it doesn't get more serious.  We are starting to really wonder the source of these allergic reactions.  A few weeks before the surgery Jake had a slight reaction after his vaccinations and his upper lip blew up but then went away pretty quickly.  With the confusing developments in the operating room and now this will keep us on guard to figure out if they are isolated incidents or all connected.  Unfortunately, we have to wait a number of weeks to be off the medicines before we can see an allergist.  Here's hoping the allergic reaction is controlled from the medicine.

Home Sweet Home

Well after sleeping well, Jake gave us what we always hope for a big smile!  It was nice after a tough day and night to see our Jake exhibit his normal self.  He launched back into his normal play routines around the house and was happy and content for the day.  More surprising was that Jake walked on his own around the house, up the stairs and even danced a bit.  A welcome sign considering that his hip being the donor site for the bone graft in his thumb is supposed to be quite sore.  Jake welcomed a few of his close friends and was content to blend back into his normal routine.  No emergence of "Angry Jake" today a real positive for all of us.  He took his medicines without a fight and went to bed immediately.  We root for a restful night for all as tomorrow brings Jake and Luke's 5th Bday!

Discharge Day

Jake finally succumbed and slept most of the night and in fact slept through several rounds of Dr.'s and didn't wake up till 10am.  He hadn't slept much previously so it was much needed.  The consensus was clear Jake was going to be able to go home as expected especially now that the allergic reaction had come and gone without any signs of reemergence.  The rest became standard procedure, reviewing all of the paperwork, signing the discharge forms, and filling the many prescriptions to take home.  It was check-out time at the hotel and we walked around some of the streets around the hospital.  Jake found a construction site with huge machines and cranes and the orders were clear, stay here and watch.  Worried about traffic on a Friday and a holiday Friday to boot, we left for the airport with plenty of time.  There wasn't any traffic, leaving us at the airport with several hours to kill.  Hard to say if we just beat the traffic and it would have gotten exponentially worse.  Last trip, Jake was calm as he watched planes and workers from the windows but today was a different story.  The agitation ensued....this now means Jake shouts a bunch of things out and doesn't know what he wants.  First, he was hungry but wherever we took him he said the other place.  Then Jake wanted to go by the window but in the United terminal most of the windows had a panel on the lower half which prevented Jake from watching from his stroller.   We walked around and around with Jake yelling at the top of his lungs to try different places.   After about 30 minutes of this agitated state, a donut settled him down.  Finally, we were able to find one gate where the panels didn't exist and Jake was happy.  After some time, we headed to the gate to board.  As we stood in line awaiting our boarding, Jake decided he didn't like waiting.  He started yelling for a walk and when we didn't comply because we were boarding, he screamed louder and louder.  It was a rough scene with a crowded gate area and Jake yelling at the top of his lungs.  It got more interesting when he started yelling, "I have to go poop,  POOP POOP!"   After what seemed like an eternity, we were allowed to board and we found our row to settle in.  Then a not so subtle reminder from Jake that we forgot to see the pilot sent us upstream against traffic.  We were allowed to enter the cockpit and Jake met the pilot.  After takeoff, Jake seemed to battle with this confused agitated state once again.  He had to go to the bathroom and after the first trip, he proceeded to yell every time we got back to the seats that he had to go again. Yelling ensued and to keep the peace we took 7 trips in about 10 minutes time back and forth to the bathroom.  It was rough period and harder because this behaviour is so out of character for Jake and his normal personality.   Eventually, Jake passed out for the rest of the flight but without first taking its toll on his sleep-deprived parents.  

After forever and then some, we were on the ground and on our way with home with a huge sense of relief.  Unfortunately, Jake returned to his agitated state first about taking his medicine and then ultimately about where he wanted to sleep.   We spent a good 90 minutes doing laps among the bathroom, our room, his room, and downstairs.  He simply couldn't settle down and kept yelling and crying about what he thought he wanted.  Whenever we presented his requested option, it wasn't what he wanted.    We urged him to calm down on our bed which took quite some time but finally he settled down and wanted to go to his room.  We put him down to sleep but a minute later he reappeared in our room.  Surprising, as he shouldn't be able to walk without pain due to the incision from the bone graft taken from his hip.  So, the final choice was made and Jake slept in our bed where he still remains.   A sleep-deprived emotional trip but we are home and hopefully we can cure Jake of this new agitated confused state he has been in.  Luke and Olivia got up early to see us and anxiously await to see their brother and the cast color he chose.

Post-Op day 1

A rough night for Jake who was agitated from his swelling due to the allergic reaction in addition to surgical wounds. The second dose of adovan seemed to work letting Jake rest at 3am. By 6 am the mystery rash had dissipated, a positive sign. Team after Team stopped by to check on Jake, many confused by his allergic reaction wondering what could have caused this unexpected event. Despite the other Dr.'s thinking the allergy team would have answers, the allergists couldn't offer an explanation. Only a referral to an allergist to test Jake when we return home so we don't experience this again during a procedure. With the rash subsiding, most of the teams agreed that Jake would be able to move down to a regular room and felt that as long as nothing unusual happened, we would still be able to make our Friday night flight out of Boston. The surgical team was pleased with the end result of Jake's thumb but did encounter some surprises with his bone graft and quality of bone to draw from. Most of the day was spent with both of us in bed with Jake playing videos on our phones. His new favorite a class performance of cousin Leo's class singing Alphabet Stew. Before Aunt Stacey left for home, we had to have her message us the video to continue its top status on Jake's video playlist. The anesthesiologist came by again to discuss the allergic reaction and urged of the importance to have Jake checked out due to it causing anaphylactic shock. The suspected culprit is the gel foam that was used to stop blood flow and fill the space where the bone graft was taken from Jake's hip. Apparently, the Drs. found a letter to the editor in a medical journal which cited a case where this happened. Good news came in the afternoon and we were on our way out of ICU to a regular room. After settling, Jake wanted a walk so a walk is what he got. We disconnected him from the machines and toured what felt like every inch of the hospital with Jake in his stroller. After a while we had to return as Dr. Upton was paying us a visit. We discussed the reaction again and he did let us know that it was serious but if a reaction like that would happen, better to be in a hospital where it can be controlled immediately. Needless to say we are being sent home with Epi-pens as a precaution. Dr. Upton spent some time discussing Jake and future surgical protocol to keep in mind for in the future. He seems willing and ready to hand Jake's care over to his former fellow and assured us that Jake will be in good hands. He did comment again on Jake overall and really feels good about his future given his mild status. After the visit and sad farewell, we realize this could be our last time seeing him. He told us to keep in touch and it was a tough moment. This surgeon has done so much for us, given our son fingers... allowed him to have the best aesthetic and function possible. Eternally grateful and certainly worth the big out of pocket expense. Then it was time to honor and give back. I went to the blood bank at the hospital to donate blood in honor of Sam's cousin Ariel who was in a horrible accident and relied on the kindness of strangers whose 70 units of blood donated helped keep him alive. I left a note on the wall and those reading this blog I urge you to follow the message. The note read, "a tribute to Ariel Fishman...an unfortunate accident, Ariel needed kindness of strangers. He received 70 units of blood. 70 people saved his life. Let's save peoples' lives...Give blood, help those in need." It is so easy if you can, please take the 30 min to do so. Upon my return we took Jake downstairs with us to eat a quick dinner before returning to the room. Understandably, Jake did not want to return to the room, so four last times later we resisted and settled in. Jake was really agitated and threw an out of character fit. He hasn't slept much and is unsettled so it took some patience but we got him to calm down. It's quiet in 714B now so lets hope it stays that way and we discharge and can return home closing the book on the Boston surgeries for now.

Surgery Day (#9 overall, 4th hand)

As they all are, today has been a long tiresome day. It started out great with Jake sleeping late, bonus to still be on LA time, giggling and playing in bed. Off to his pre-op at 10:30 to which everything checked out and the waiting game started till his scheduled surgery time of 12:30. Some friends of ours from the Apert group on Facebook whom we hadn't met in person dropped by for a visit. It was great to meet them and their son who is a couple years younger than Jake. We always say for all the negativity facebook gets, the group we share is a great source of information and support for all of us dealing with children who have this syndrome. It also helped pass the time which is a bonus on surgery day. Aunt Stacey joined us as well and Jake was happy to see her, playing games in the lounge with her. Soon it was time to head upstairs and the memory from a month ago triggered some crying. Jake seemed to realize what it all meant. The anesthesiologist showing up with a flavored mask didn't help and that brought more tears. Dr. Upton came to discuss the procedure with us but Jake was sad and crying so much we couldn't get a final photo of Jake and his Dr. The positive was seeing the Dr.'s reaction to his thumb and the pronouncement that the thumb looked great and at least the past month was worth it. Jake in the OR was tough, he was crying really hard and it was the worst it has been out of any of his surgeries. Luckily, he fell asleep in 15 seconds and Jake was left in the hands of his surgical team. The waiting game began and four hours later we received a visit from Dr. Upton. He indicated there had been a puzzling development as Jake broke out with a rash all over his body. None of the medication has changed from previous surgeries so the allergic reaction was a surprise development. To be safe, they were going to send Jake to the ICU to monitor the reaction and make sure his breathing is under control. In regard to the surgical procedure, the Dr. was pleased and basically they took a piece of bone from his hip which is now being held together by a pin for about five weeks. This procedure should really help Jake's hand function which makes this all worth it in the end. Some interesting news, the Dr. thinks we can wait to do the same procedure on his left hand. We will see but that may buy us some much needed time from the OR. Seeing Jake in the ICU was extremely difficult as his entire face was swollen and he looks like a different person. He also has a red rash over his entire body. The allergists will review everything tomorrow but for the time being no one really knows why. For us, we are concerned about other items giving him more agitation and irritance. Jake is definitely agitated and uncomfortable so we hope the right combo of medicine can help him cure the allergic reaction and heal his surgical wounds. Hopefully, Jake will rest tonight and we can leave the ICU in the am.

Surgery tomorrow

Well we sit here in Boston once again on the eve of a surgery for our precious Jake. What can you say about a kid who has been through a month of hell and is able to cheerfully leave home get on a plane and be a happy kid. Jake all day was such a joy and it hurts to know what he has coming. We will get through it as we always do but we hate to see such a special kid feel anything less than joy. Tomorrow brings another surgery and some tough days but it does enable us check off another step in his medical care. Pre-op at 10:30. Surgery scheduled for 12:30. Think good thoughts.

Surgery #9

Well here we go again, Wednesday will bring Jake's fourth hand surgery and 9th overall.  It has been an up and down month dealing with Jake's aftercare.  Despite it all, the one thing we can say is Jake was entertaining his visitors today.  His buba visited and brought him a special gift, a stuffed animal that has a cast on his right arm and Jake has named it Joey after Dr. Upton.  Then he was in rare form dancing and singing for his Amy and Melissa.  It has helped that we were able to stop turning his distractor on Wednesday but the cleaning of his hand and the antibiotics 4 x a day always offered opportunity for agitation.  Looking at Jake's left hand, we can already see how straight and long his right thumb is in comparison and we look forward to this final step in fixing his dominant hand.  This surgery should be a little more intense than last month due to the bone graft which requires a piece of bone from his hip.  Afterwards, the positive note will be that his hand will be secured in a cast so we can bid farewell to the distractor that has to be turned and worry less about the risk of banging his hand in an open splint.   (well farewell till the fall when we repeat both surgeries on his left but let's focus one step at a time.)  For now, we look forward to returning home on Friday Night and celebrating the boys 5th birthday on Sunday as a family back together.

Update

Well, the story continues and new developments continue to challenge us.  Jake has been growing very irritable and is fighting the adjustment of the splint and his upkeep.  It has been tough to do what is needed for him while he cries and flails and doesn't cooperate.  We try to push through but it is emotionally and physically tough.  The new development, Jake's thumb turned red and it was a concern to us.  Some photos emailed to his Dr. confirmed an infection and Jake is now on antibiotics to battle this.  This medicine has to be administered four times a day which requires a lot of Sam including dropping by school to give him his medicine.   On a positive note, we are happy to catch this when we did, as it could have posed a risk to his surgery next week so we are thankful we could nip this when we did.   Interesting  enough, Jake had his five year appt with vaccinations that we wrote about earlier and today we found out that if you can't have surgery within 7 days of them.   Would have been nice if this was clearly explained but that would be expecting too much.  Trying our best to manage the stress but in honesty we are worn a little thin heading into surgery #2 next week.

Jake Update

We have stayed away from posting as for the most part everything is moving along.  We struggle with Jake's bouts of irritability which is perfectly understandable.  Adding to what the poor kid has already gone through, he had his 5 year check-up.   That brought 5 shots and Jake is probably a little sick of the doctors at this point.  The daily turning has gotten better but the cleaning and splint prep has adding some steps to the daily routine that require extreme care and patience.  Jake is still a very happy kid most of the time which is a relief, it is just hard to see him hurt or be irritable but clearly understandable.   On a positive note, the differences can already be noticed.  His thumb is straight and longer and more interesting are his other fingers.  The work on the other fingers involved removing some of the previous skin grafts and what used to be slight discoloration on parts of his finger now looks to be matching skin.  Real curious to see the finished product sometime around end of June.  Until then, another trip and surgery looms, week of May 21st and we will push forward.

Dr. Visit

The morning started at Children's Hospital to get a look at Jake's hand and progress report.  Waiting for the Dr., we ran into another boy with Apert whose family we know and it was cute to see how Jake and he interacted and a reminder of Jake at a younger age.  Once in the exam room, the mood started to change.   The Dr. started to cut away the bandages from Jake's finger and hand and it didn't sit well with our little trooper.  Our attempts to distract Jake weren't working and he was started to cry and become unsettled.  It was a very difficult five minutes and Jake has never acted like that surprisingly with all he has been through.  He was scared, irritated and wanted to get our of there as soon as possible.  The initial reveal of Jake's hand was interesting as his  thumb did look completely straight and longer.   The reality of his current condition was harder to view as a parent as the screws and surgical entry point were in full view.  Perhaps that was a negative for Jake as well as we couldn't shield his curious eyes from looking at his fingers and hand in full view.   It was difficult to hold Jake in order to get the required photos to send back to Boston but we did our best and Jake did not calm down until we stepped out of the building.  We can't blame him quite frankly but we just hope that he doesn't react in similar fashion on the plan or at the hospital for part two.  The great relief was that at least our temporary mental scare about turning the screw the wrong way was dismissed and we are handling our role properly.   Our anxiety is slightly higher as Jake no longer has any bandages on his fingers and there is no layer of bandage between his contraption and thumb.   At least the splint will help form one layer but fingers crossed no additional issues before part two.

Jake Hand Update

Jake has been doing well completely off his medication with an occasional dose of ibuprofen.   Turning the device in smaller increments has been working and it's probably a combination of factors including with each passing day Jake's thumb hurts less and he is able to tolerate the movement.  Small dose of anxiety over the weekend as we are turning the device clockwise as instructed but we saw an arrow on the device when looking through the splint and it was point the other direction.   Although we are sure we are doing it the right way, the shadow of doubt just adds to the anxiety.  The spacing definitely looks wider than previous photos but you never know.  We marked his bandages with red nail polish to monitor the spacing so this should help.   We have a check-up at Children's Hospital today so we should get a good luck at Jake's hand and confirm everything is going as planned.  In-Depth update of the Dr. report to come tonight.

Fear of the Screw

Jake snuck downstairs this morning by himself and was giggling and proud that he did so undetected.  Just happy to see him smiling after last night's difficult session.  We spoke to the Dr. today to inquire how to handle.  Our instructions are now not to turn at night something tied to lack of stimuli not ideal opportunity.  We are to go back to increments a quarter a time throughout the day.  Today we made it to 2 and the 2nd hurt Jake a little and we decided to stop there and not risk waking him up at night again.   Tomorrow we will try with the new direction in mind.  In other news, Jake went to an Open House Night at his new school for next year.  Amazing to think of how far he and all of us have come in five years and that next year he will start kindergarten. 

Curious for his Monday appt to see his hand and what it looks like. 

1 week Post-Op

Overall, Jake is doing very well going to school, playing with only a few minor bouts of irritability.   the big issue is still the same issue, the turning of the screw.  Last night, we decided to try the full rotation again and Jake woke up a minute later crying uncontrollable, thrashing and almost inconsolable.   We eventually were able to give him some medicine and calm him down to where he fell back asleep.  We are really struggling with the process as Jake otherwise is doing so well except for when we are forced to inflict pain.  We're not sure we can keep doing this night after night and watch our boy so sad and in pain.   I may call the Dr. to see if this is the expected reaction or perhaps Jake is just a little more sensitive.   We do have a follow up with Dr. Panossian at Children's Hospital on Monday so that should also help answer questions and get a true medical progress report.  Until then, we will try to keep exhaustion at bay and sadness to a minimum.

Post Day 6

Jake has been doing well with intermittent bouts of pain and agitation but overall has been his normal cheery self and acclimating to his splint and hand.  We are in the process of weaning him off the pain medication and for the most part the oxycotin is done and we are just using the heavier dose of ibuprofen when needed.  Nona came over for a visit and Jake was very happy to see her and read books.  The big debate was how to handle the turning of the distractor.  Worried about too much pain, we decided to try last night to turn it in increments to see if that would be a better action plan.  Around 8:30 when Jake was asleep, we decided to try a quarter rotation.  A success, Jake didn't move and   more importantly didn't wake up.  Approximately 15 minutes later, we decided to try another quarter.  Jake initially didn't wake up from the turning but shortly thereafter he started crying complaining of some pain.  At that point, there is the debate whether we should just turn it the entire way as the smaller increments still woke him up with pain.   We decided to give him a break and settle for the half rotation as it took 30 minutes to console him back to sleep.  A couple times at approx. 12am and 4am Jake woke up and ask Daddy to lay with him.   He did successfully go back to sleep and overall only needed the pain medication the one time after the turn.   We will have to see how his day goes today as we hope his hand will heal each day a little more.  Jake is going to school and handling it just fine so besides some emotional wear and tear and some sleeplessness, we would have to say it is going as well as can be expected.