Home Sweet Home - Our New Normal

Since our last post, Cole celebrated his sixth birthday as well as his six month post transplant landmark.  He has been living at home in his own section of the house while we remain diligent about keeping him healthy.  We continue to clean everything with bleach, wash hands constantly, prepare his food carefully, run air purifiers non-stop and keep him away from groups of people.  He wears a mask when out of the house and continues to take his medicine three times daily.  He's even begun home schooling.  We live in constant  fear that he'll get a viral, bacterial or fungal infection, but at least we're all in the same location and have some normality to life again.

Two weeks ago, we drove back to Cincinnati for a check up and got the green light not to return for 4 months.  It's a blessing and a curse to be out of the hospital.  A blessing that we're not confined to the physical location of the hospital, but a curse that we don't have the security of the doctors and medicine there.  If something goes wrong, we're a 12 hour drive to comfort.   

Cole's levels continue to swing and settle out - we hope.  His engraftment has been hovering in the mid 60's which would mean that he doesn't have to go through another transplant.  His thyroid, ANC and other blood counts fluctuate, but seem to be trending in  a positive direction. 

Being home has been a great mental boost for Cole.  He's  seen close friends from a distance and spends some time with his older brother and sister.  After his chest wounds from his port healed, he was even able to go in the swimming pool for the first time in over a year.  It occurred to me how many things he's missed in the past year.  His sense of humor and perspective is very advanced since he's spent so much time with adults.  I'm hopeful that he'll adjust quickly socially when he's able to be around his friends.

Physically, the effects of the steroids and chemo are starting to wear off. He's beginning to grow his blonde hair back, his weight is dropping off and his body hair is disappearing.  He's less self conscious and becoming more physically active daily.  I remain concerned about the long term effects of the medicines he's taken, but we'll enjoy each day.  

I clearly underestimated how long this process would take and how taxing it would be on everyone.  Physically, mentally, socially.  The anticipation, logistics and fear of the unknown take their toll.

The power outage from Hurricane Sandy created additional stress, but we continue to count our blessings.  We continue to learn of other families who lost their children and recognize how fortunate we have been so far.  

Also a shout out to my parents, especially my dad, for helping out over the past few months.  After Janet's mother so selflessly spent several months with the kids while we were in the hospital, my father has been helping make the logistics easier at home now that Cole is there.  We couldn't have done this without the support of family and friends along the way.  Thanks to you all.  Count on a big thank you bash to all in the Spring...  

Home...?

Last week, we got clearance from the doctor to return home to Connecticut.  We had mixed emotions about leaving the proximity and safety of the hospital.  There's comfort that the doctors who know Cole and his disease are only 30 seconds away.   It's surprising that there are no experts close to the NYC area - so we had to find a doctor willing to follow the protocol of our doctor's in Cincinnati.  There's also a lot of concern given that Cole's engraftment numbers continue to fall.  That means that Cole's bad marrow continues to take over Ryan's good marrow.  If the doctor's can't reverse that course, we will have to start over.  The doctor's are hoping to change medication and infuse Cole with more of Ryan's blood to stabilize engraftment and reverse it's course.  We remain optimistic and hopeful.      

After an overnight drive from Cincinnati, we were able to get Cole home early Saturday morning.  Janet will stay with Cole in the pool house while I try to spread myself between work, the house, the older two kids and helping Janet and Cole.  Thank God for family and friends who make this easier.  And mostly, thanks for Janet who is an iron willed advocate, nurse and protector of her youngest cub.  I couldn't imagine anyone trying to do this alone.  We're all trying to define our new "Normal" and find our way.  

For now, Cole can get outside occasionally with his mask, wave to the older kids and even hang out from a distance.  It's been a great spirit lifter for him and all of us.  Unfortunately, we have to take he and Ryan back Sunday for the next infusion in hopes of reversing the engraftment declines.  It's the first of many return trips to Cincinnati, but it beats the alternative of not being home at all.   

Day +85

At day +85, Cole remains in relative isolation.  By day, we stay in the apartment trying to entertain him and at night, he's able to walk the halls wearing a mask and hat.  He's even been riding his bike occasionally through the halls.  He's feeling well and has enough energy to want to wrestle constantly.  Being off steroids, his appetite has diminished and the dark "steroid hair" has started to thin from his head and face.  He's becoming less self conscious of his appearance and more open to speaking to people on Facetime.

His engraftment numbers continue to drop, which is not great news.  It means that Ryan's marrow is now being overtaken by Cole's.  The doctor doesn't seem too concerned, since they see this occasionally with match siblings.  But we have to keep an eye out for graft failure, which would mean we'd likely have to start over.  I've come back home to take Ryan to Cincinnati to do another infusion in an attempt to get Cole's engraftment to stabilize and hopefully get higher.  It shouldn't be a painful process for either of them, so we're happy and hopeful.    

After several months in Cincinnati, the process is taking its toll on everyone, a fact that is not lost on Cole.  Occasionally, he gets sad and starts to think it's his fault.  We often forget that Cole hears everything we say.  And, more importantly, he understands everything we say.  On rare occasions, Cole has been frustrated that he isn't able to enjoy the Summer like everyone else and that he can't see his friends and family.  We are also beginning to understand the impact on our older kids, although they insist they're fine.  We look forward to being home together as a family soon.

We remain incredibly thankful and count our blessings that it could be so much worse.  A fact that becomes real as we see others go through this process.  Aside from fleeing moments of sadness, Cole remains overwhelmingly positive and motivated.  He remains an inspiration to us.  

Cole has grown considerably in this process.  Certainly physically, but more importantly, he has matured emotionally and doesn't want to be treated special or like a baby.  He's learning to read, tell time, do math and swear like a sailor from watching so many movies.  We have a lot of bad habits to break when we get home...  He'll likely spend this school year at home, then head to first grade next fall.   HLH and the bone marrow process has taken many things from us, but it has also given us perspective and brought us closer.

It remains largely a waiting game over the next few weeks.  From there, we hope to start discussing getting everyone back under one roof.  We're not certain if or when that will happen, but we remain optimistic for what lies ahead.  Thank you all for your continued love and support.

Day +52

It's easy to start thinking about day 100 when you're half way there.  For each bone marrow recipient, day 100 is an enigma.  Some patients are still in the hospital with no path for getting home.  Others will never make it to day 100.  That was the harsh reality last week when we learned that one of the children being treated for HLH here in Cincinnati lost their battle with the disease.   My heart aches for their family.  I can't imagine their pain or what lies ahead for them as they begin to heal.

For some, day 100 is a pivotal landmark.  It's when the doctors begin testing the quality of the marrow production to determine the next steps.

While Day 100 is only 48 days away, we have to stay focused on the present because a momentary lapse in judgment could have serious consequences.  It's a testament to my wife Janet that Cole remains out of the hospital.  She keeps us focused.  She's a vigilant cleaner, a cheerleader, a rock, a spirit lifter, a punching bag, a friend, a nurse, a jester, a teacher, a chef and a pillow.  She has taught Cole (and me) what has to be done to keep him healthy.  She keeps the doctors on their toes and is an advocate for Cole's wellness.  Some day, Cole will begin to appreciate Janet's contributions to his wellness, but I'm not sure he'll ever fully understand it.

For today, Cole remains on a good path.  While he is increasingly self conscious about his appearance and is incredibly home sick, his spirits are high.   He remains virus and infection free.  He remains focused on doing the right things to get home.  His white blood cell count and engraftment numbers have been trending down a little, which is cause for concern if it continues.  But today, day +52, we remain focused and optimistic.

Day +40

We lived vicariously through others this Memorial Day weekend.  We loved seeing pictures and reading about friends and family enjoying the outdoors.  Cole, Janet and I were able to enjoy our own indoor BBQ (trying not to set off the fire alarm) and make the most of the long weekend inside our temporary apartment playing games and watching movies.  The entire weekend, all I could do was give thanks that we were not in the hospital and that Cole has remained healthy.  My mind and heart remain with those families who are not as fortunate.  Now day +40 post transplant, I'm amazed how fast it's going and that we're almost half way to the magical day +100.

We also remain very aware how quickly it could all change.  We can't get too far ahead of ourselves and must remain diligent in our cleaning and daily medication routines.  It's easy to get distracted and want to take shortcuts, but a long road still lies ahead. 

90% of all children released from the hospital are re-admitted.  Cole remains focused on not being one of them.  He takes the stairs when going to the hospital, washes his hands constantly, reminds us to do the same, and takes his medicine as directed. 

The doctors are talking about reducing his steroids as well as some of his other medications.  Already, we have been able to take him off some of his mouth care medications.  Cole did a happy dance as we threw away these meds since they made him gag (even with grape flavored syrup) three times every day.

Last weekend, I was able to go home to spend some time with the two older kids.  I opened the pool, watched them play soccer and took them to the Yankee's game.  Thanks to a good friend, we ran into Mark Sanchez in the Legends Suite before the game.  Mark was gracious enough to pose for a picture and the kids couldn't have been happier.  It was also bat day, which rounded out a weekend that I will never forget.

My in-laws were able to get a slight rest and go home to Boston for the night.  It's a whirlwind being home jumping back in to schedules and getting the housework done, but it was great to see the kids.  Janet's sister came up to Cincinnati for a few days while I was home.  She was fresh meat for Cole to punch and play with.

On a side note, Cole's doctor is fascinated by his hair.  She says that she believes shimmery hair is one of the indicators of HLH.  Anyone who has met Cole, knows that his hair is the definition of shimmery.  Apparently Cole's hair has a unique make up - with alternating shimmery follicles and normal ones.  Even his remaining wispy hairs are shimmery.

The medicine continues to change Cole's body - from behind, he looks like a 90 year old man - pudgy and bald.  From the front - he looks like a caveman.  The steroids have created a dark uni brow, puffy cheeks and a fuzzy beard.  But his smile remains large, his attitude is great and he remains focused.

Milestones

A lot has happened recently, so it's time for an update.  Thanks again to all of you for your support during this process.  Your thoughts, gestures, notes, gifts and calls have been overwhelming and has done wonders to keep our spirits high. 

Cole continues to progress very nicely.  So well, in fact, that he was discharged to outpatient care on Thursday at day +22 post transplant.  It was an emotional departure.  The nurses and staff gathered to sing a song and give him a parting gift. Janet and I welled up as we watched him walk down the hall with his hands held over his head in victory.  I could almost see his smile through his mask.  He's nearly bald, a little puffy and weak from lack of walking, but he's stopped getting sick every morning and he couldn't be happier to be getting out of his room. 

Cole is now in an apartment with us across from the hospital where he will likely stay for the next several months until we (hopefully) can go back to Connecticut.  Until then, as long as he stays healthy, we will give him his medications here, keep him in isolation and visit the hospital several times each week for check ups and infusions.  Currently, his engraftment is at 100%, which means Ryan's bone marrow has fully taken over and is doing its job.

I know he's my son, but I am truly amazed at his strength and singular focus on getting well.  Never complaining, always focused on doing what it takes to get to the next step in his recovery.  It's a lesson that I won't soon forget. 

It's difficult to celebrate, as we know (and have seen), things turn quickly.  But we celebrate milestones such as this. Lurking in the back of our mind is what will happen the next time Cole gets sick. Will his body pass that test as well?  Will we ever have a level of comfort that he will be fully recovered from HLH?  Will we ever stop washing everything with bleach? 

Tomorrow is Mother's Day, so I flew the two older kids out to Cincinnati to celebrate with Janet.  It's a great gift for all of us for a holiday with special meaning this year.

Day +6. One Day at a Time.

Prep for Transplant

Bag-o-Marrow and Blood

Day +6

 6 days after transplant, Ryan is home in Connecticut and Cole is feeling pretty well in the hospital.  Ryan and Cole were both transplant champs and now are Bone Marrow Brothers for Life.  Ryan had very little reaction to the procedure outside of sleeping for about 20 hours afterwards while the anesthesia wore off and his body regenerated marrow. 



Ryan Pre-Op



Since transplant, Cole has been doing great.  He hasn’t lost his hair and hasn’t gotten too puffy from the steroids.  He hasn’t been too sick and he’s managed to stay virus and infection free, which has kept him out of the ICU.  He hasn’t gotten any mouth sores or had any shedding of the lining in his digestive system.  We know this could change in an instant, so we are reluctant to celebrate just yet.   

Cole's immune system is still completely gone – so the chemo did its magic.  His white blood cells, platelets and other key indicators are all hovering around zero.  Typically, white blood cells start to produce over the next 4-12 days.  Then we test if the cells are Cole’s or Ryan’s.  From there, it’s a slow, long road to getting the rest of his numbers back to normal and trying to keep him virus free.  We remain hopeful that we can have him home in time for his birthday in October, or at least by the holidays.     



Ryan Post-Op



We continue to take it day by day.  So far, the doctors are pleased with his progress and his spirits are generally good.  As long as he’s good, we’re good.  It’s hard to believe it’s already been over a month.  We miss the two older kids immensely as well as family and friends.  We look forward to continued progress and being together again as a family unit. 

Ryan Palooza - A Guest Entry by Uncle Andy. Delivering the Goods

Life rarely goes the way you planned.  This past weekend was supposed to involve me playing golf in the Club Championship, watching Jake play goalie in the State Lacrosse Championship (which they ended up winning…Go Lightning!) and enjoying the last of the beautiful Scottsdale weather. But instead, I found myself flying to New York and driving to Connecticut.  Plans changed because I had been asked to do a very important job…to transport Ryan to Cincinnati so he could donate his precious bone marrow to his brother Cole. 

Was I surprised to be given the job?...well, a little because for years I’ve been known to make my niece and nephews memorize our motto… “fun first, safety second”.  And the last two times I rented a car I was awarded two well-deserved speeding tickets (which I must add the policeman in Vermont and Idaho were extremely pleasant during both incidents).    Nonetheless, Ryan and began loading the car to start the drive Friday morning.  As I was starting the car, my phone rang.  It was Jeff.  His message to me was clear…”you have extremely precious cargo on board. Do NOT screw this up”.  So brimming with confidence, we pointed the car toward Southern Ohio.

We took our time and split the drive up over a couple days.  I even did the speed limit the whole way…let me tell you, it felt weird watching all those cars whiz by, but after a while it actually became relaxing.  We had a great time, ate chicken wings for dinner, played putt-putt (after I wiped his putter and colored golf ball down with disinfectant. Then I wiped my down just to make it fair.) 

On day two, I finally brought up the issue to Ryan of the upcoming bone marrow transplant he’d be providing on Thursday.  He said he was completely ready and not scared.  This kid is extremely brave.  Ryan said the doctor told him the day after the transplant would feel like he fell on the ice because his bum would be a little sore.  Ryan also told me he was excited to order a soft pretzel from the hospital lunch cart after the donation.  He explained his brother Cole had been ordering them every day and Ryan wanted his.  Who says jealousy can’t ever be helpful?

After arriving safely in Cincinnati, Ryan and I got to visit Cole in the Bone Marrow Transplant ward.  We could only look through the glass into his room as to avoid any potential contamination.  He was all smiles as he jumped out of bed when he saw us in the window.  He looked every bit of the boisterous, mile-a-minute boy I’ve always known him to be.  He has been a complete champ as he’s faced the chemo treatments and being cooped up in that small box of a room.  I was overjoyed to see him, but let me tell you, it’s a life altering experience to visit that floor.  Someone said it in the blog comments, the people who work there are Angels on Earth. I couldn’t agree more.  I’ll also never forget the look on all the parents faces on the floor.  They shuffle around in sort of a trance, each having a look of disbelief but determination on their face.  Simply put, everyone there is just doing the best they possibly can.

Today Cole got his last chemo treatment before the transplant.  It’s the biggest dosage.  He might be a little more affected by this one than the last.

No-one here planned to be in this unpleasant situation.  But for me, there has been a great silver lining.  It has been a blessing to spend time so much time with Jeff, Janet and of course Ryan.  The family thanks you for all your thoughts and prayers.  Believe me, we’re praying right alongside you.  T minus 3 days to go…

Love,

Uncle Andy

Day -1. Day 0 Tomorrow (Transplant)



Medicine Tree


The last couple days have been tough for Cole.  The isolation combined with heavy doses of chemo have started to take their toll.  It's a view into what lies ahead, but I'm confident Cole will remain strong.  The notes, posts, cards, prayers, jokes, phone calls and Facetiming keep his spirits high, so please feel free to keep them coming. 

Janet remains Cole's rock...a constant force of support and light.  Ryan is the knight in shining armor that, without hesitation, will save the day for his little brother.  Cole continues to stay s strong.  If his superhero idols were here, I'm certain they would look to Cole for strength.  It takes a village to go through this, and for that I can never express my gratitude to all of our family and friends. I have redefined my definition of a hero, and they live amongst me...

For now, we wait with anticipation for transplant tomorrow.

BMBF Bone Marrow Brothers Forever

Out like a light. 




Friday the 13th, Day -7

So far, Cole's chemo is doing exactly what it's supposed to - eliminate both his bone marrow and his immune system.  I suppose we should be happy that his numbers are trending down, but turning him into a toxic wasteland doesn't feel like cause for celebration.  Not yet, anyway.  It feels a little like we're pushing him from the window of a burning building - we know he needs to leave, there's no turning back, and while it will be a rough fall, we hope that he lands safely. 

The doctors are pleased with Cole's progress.  Though we constantly smell like bleach and our hands are raw from washing, we've been able to keep him germ free.  While he's still in isolation, we're trying to make it as unlike prison as we can by decorating the walls and playing games when he feels up to it.  His days have been mostly good.  He's generally had enough energy to get out of bed, play a little and had an appetite.  He hasn't lost his hair and hasn't gained much weight yet.  We know all of this is about to change based on the medications about to come and the conversations we've had with other parents in the bone marrow ward. 

I'm overwhelmed by the general love and care for Cole and the other patients in the unit.  The nurses, doctors, friends, family, co-workers and volunteers re-affirm my faith in humanity.  I am amazed by the kids and parents here.  Some families have multiple children going through bone marrow transplants at once.  Some have been here for 6 months or more.  Some have complications that make treatment far more dangerous. Some of these children will never leave this unit while many others will go on to live happy, productive lives.  Despite that harsh reality, or maybe because of it, people continue to give their time, effort and support.  And it is deeply appreciated by those receiving it. 

It's not lost on me that today is Friday the 13th.  I'm not a superstitious person, but I appreciate when buildings don't have a 13th floor, I'm happy when a black cat doesn't cross my path and I still hold my breath when driving past a cemetery.  I try to enjoy every day, but I do look forward to tomorrow, day -6 as I only want positive thoughts and it's one day closer to getting Cole home.

Game On. Opening Day.

Over the past 2 weeks, Cole spent his days in the hospital and his nights with us.  Today, he will be admitted to the hospital for several months to begin the next phase of treatment.   He'll undergo general anesthesia to have his central line catheter installed into his heart, then begin a cocktail of chemotherapy, steroids and anti-viral medications.  Those will be administered over the next several weeks to eliminate his immune system and prepare him for the bone marrow transplant that will take place at the end of this month.

We took him out to McDonald's for dinner last night.  He's only been to McDonald's a couple times in his life, but we bent on our principals and allowed him to be in control for a while.  He's earned it, and will continue to do so over the next two years while he recovers.

After dinner, we cut his hair short to minimize the effects of going bald from the chemo.  Surprisingly, he seems more concerned with gaining weight from the steroids than losing his hair.  I look forward to the day when he's old enough for nature to cause both his baldness and weight gain.

As I type this, Cole sits next to me, reminding me to thank everyone for the notes, prayers and love.  The comments and support keep his spirits high.  He also saw a commercial promoting the Reds opening day tomorrow and asked if we can go to a game.  I'm an Indians fan, but would happily take him to a Reds game.  I'll even go so far as to root for the Reds to make the playoffs in hopes that I can take Cole to watch a game in person in the Fall.   Game on.

Back to Reality

With Ryan's bone marrow donor tests complete, I drove him back to Connecticut Friday.  It felt a little bit like I was transporting a secret antidote in a James Bond film.  Careful not to damage the package, I took extra precautions to keep him safe.  Now we just need to keep Ryan healthy and virus free for the next several weeks while we approach transplant - also known as day 0.  It was a long drive through some  intense storms, but it was nice to spend some one on one time with him.


Carter and Cole in the Park



It was also a reminder that Ryan is at such an impressionable age and made me sad that I can't be there more for him right now.  When I told him that I was proud of him for donating his marrow to Cole, he said "Did I have a choice"?  No buddy, you really don't.    



The trip home also allowed us to watch my daughter Carter compete in the state gymnastics meet.  Watching her compete, it's clear that she is distracted by everything going on right now and not having having her mom there didn't make it any easier. 

I was able to bring Carter back with me on my return drive from Connecticut to Cincinnati.  She was happy to miss a few days of school and Cole was thrilled to have some company.  He remains relatively healthy heading into chemo, which officially begins next week.  He's a little beat up from the testing, has some swollen nodes and some white blood cells in his spinal fluid, but it doesn't appear to be related to the hot spot they found last week.  The white blood cells also don't show cancerous cells and don't show signs of active HLH, which is a good sign.  He's getting another lumbar puncture this morning to see if the white blood cells have decreased.  The rest of the week will focus on internal testing of his lungs, stomach and other internal organs.   

We had to sign permission documents yesterday which were the scariest documents any parent can ever read.  They outlined  in detail what lies ahead and mentioned the words death and fatality no less than 20 times.  While the weekend home was a nice distraction, it's back to reality here in Cincinnati.  The doctors are encouraged that he's heading into chemo healthy and hope to have him home by the holidays.  



Let's Do This

Cole has nearly finished his first week of pretty intense physical tests, scans, poking and prodding. He's been an absolute champ, not needing sedation for his MRIs. The nurses have been amazed at his ability to lie still relative to other 5 year olds. I explained that Cole's fear of needles is a great motivator.

Yesterday was a particularly difficult day. One of his scans found an abnormality on his spine, which the doctors thought may be a tumor. That would be devastating this early on in treatment, and would require a much more intensive course of action, significantly reducing his odds. Ultimately, our doctor declared it a "hot spot" and found no damage in the bone, leading her to believe it's not a malignant tumor. I've learned there are code words, looks and phrases used by doctors and nurses. Some to communicate with each other, some to protect themselves and some to prepare families for worst case scenarios. I'm hopeful that this instance was just a doctor being overly cautious.

I also experienced true helplessness yesterday as I watched Cole get a brain scan. He was strapped to an MRI machine with cords attached to nearly every inch of his body. As he entered the MRI tube, I could no longer hold his hand or speak to him. He was on his own and I couldnt do anything to help. Fortunately, he was a champ.

One nurse underscored how important attitude is during this process. For the patient and the family. Cole remains incredibly positive and focused on one mission - getting well. Before each procedure, he says "let's do this". and often reminds everyone to "man up" when theyre upset. Like his disease, he's one in a million.

Saying Goodbye to CT, Headed to Cincy Children's.

The Journey Begins

After months of seemingly endless testing, today begins the path toward treatment for Cole.  Treatment starts by heading to our new home at Cincinnatti Childen's Hospital for a week of intense physical testing to insure Cole is well enough to proceed.   Following testing will  be several weeks of chemotherapy that will kill his current bone marrow to make way for new, healthy marrow from his brother Ryan.

It was difficult watching my wife say goodbye to my 10 year old daughter, knowing that they would likely not see each other for 6 months or more.  Once Cole starts treatment, he can't have outside visitors who may carry infections and viruses.  It was even more difficult knowing that my family would never be the same once I started the car and drove away with Cole, Ryan and my wife while my daughter waved goodbye.

While it was easy to ask "Why him?" after he first got diagnosed, it's just as easy to ask "Why not him?"  Though the odds were in his favor never to get this disease, it turns out that no amount of money, love, or preparation can prevent it.  HLH strikes only one out of every one million children.  Perhaps we should've played the lottery the same day we got the news.   It's a genetic mutation passed on from each of the parents.  In fact, even more rare is that Cole's mutations live on two separate genes - something the doctor, the world's leading expert in HLH, said she'd seen only two other times in her career.  Maybe two lottery tickets were in order.

I recall speaking with the doctor when she first diagnosed Cole.  After she began explaining the disease, I remember hearing the word "genetic" echo in my head as my heart raced, realizing that I had two other children from the same gene pool who must now also be tested.

In many ways, despite the rarity and severity of the disease, I consider us lucky.  Lucky because my two other children ended up testing negative, lucky because we were able to find a donor in my older son and lucky because Cole has been able to work himself into a natural state of remission heading into chemotherapy.  I'm hopeful that this luck continues to work in his favor throughout treatment.  That would be the only lottery I care to win right now.