Wednesday, April 11, 2018

Ducks, Baseballs, and Good News

Greetings from Boston!
We made it up here on Sunday afternoon, and are finding our way again at our home away from home.  We spent the remainder of Sunday getting settled in, and getting some groceries.  This is the first time we have stayed at the Devon Nicole House, one of the smaller patient and family housing sites for Boston Children's.  While we love the Yawkey Inn, we are finding the DNH very comfortable, and actually a bit closer to the hospital, so very convenient.

We had a last minute change of one of our appointments, which was moved from Monday to Tuesday.  So we found ourselves with the whole day Monday to do whatever we wanted.  Jacob wanted to go to the science museum, which is his favorite.  The hospital also gave us tickets for a duck tour, one of the land and water vehicle tours.  We did this first, and had a great time.  It's a fun way to see the city, and Jacob was very excited as he even got to drive the boat a bit!

After the duck tour, we went into the Science Museum, which is one of Jacob's favorite places in the city. 

 They had a new exhibit, a mirror maze, which Jacob loved.  It was really cool, and he found his way through it twice.  He was laughing the whole time, because there were "so many Jacob's." 


We spent the rest of the afternoon playing at the museum until it closed.  We headed back to the house, exhausted, and ready for an early night, since we had a full day of appointments scheduled for Tuesday.

The first one was at 9am, with a new orthopedic physician, who specializes in the spine.  After getting a new round of x-rays, we met with him.  He was actually fairly pleased with Jacob's spine, being at only a 16 degree curve (albeit also with rotation), vs. the 14 degree curve he had last March.  So it is relatively stable.  He also felt that in regards to the lesion's relation to Jacob's spinal cord, that there was still room between the two, that should allow sufficient space for the spinal cord, and that it is not currently being compressed by the lesions.  He did see an area of questionable invasion of the lesion into the spinal canal, which he said if they see that today during fluoro, then he will want us to proceed with an MRI to further evaluate.  He was pleased with Jacob's exam, that Jake showed no weakness in his legs or feet, and had good sensation and reflexes as well.  This supported his opinion that his spine is still, though curved and rotated, fully protecting his spinal cord.  He reiterated that decisions on surgical excision of the lesion, will need to be made based on Jacob's symptoms, which we all agree that currently do not warrant such a drastic move.  So bottom line, though we are not, and likely will not ever be out of the woods in regards to Jacob's spine, we get a pass for today!  As much as I would love reassurances of being able to cross this worry off the list, I will take what I can get.  With this wildly unpredictable condition, we will keep hoping and praying for "Today's Miracle"  and are thankful to have gotten it today.

We spent the next few hours in the pre-op clinic, and David even took a "break" and went and gave platelets (which they announced overhead that they are in dire need of) here at the blood donor center.  I'm also thankful for my brother Mitch, for stopping by our local Blood Assurance in Chattanooga, and giving yesterday in honor of Jake.

We got out of preop early, and grabbed a bite to eat, and found they could see us early for our last appointment, with our Interventional Radiologist, who is our main treating doctor here.  He is amazing and has done all of the Sclero procedures on Jacob here in Boston.  I was very anxious about this appointment, as they had prepared us for the possibility that he would have to cancel the procedure, if there was too much scar tissue, and not enough open channels of lesion to treat.  This would have put the surgical option quickly back on the table.

He ultrasounded Jacob himself, and though he could see extensive areas of scarring, he did see open areas that he can treat, so we were a GO for the procedure Wednesday.  He also incidently saw a spot on Jacob's spine x-ray, and from our reports of Jacob's intermittent leg pain, ultrasounded his leg and found a new cluster of lesions in his right thigh.  The spot that was visible on the x-ray was actually a calcified blood clot, likely causing a lot of the pain.  He is going to sclero this area as well today.

After our appointments, we went back and took a nap, and then got a call from our friends at the hospital that they had Red Sox/Yankees tickets that had been donated that they wanted to give us.  We were so excited!  We headed to Fenway, and found we had great seats, just up the 3rd base line.  It was a little chilly, but hot chocolate kept us going, and it was a fun game.  We explored the stadium a bit, even went on the Green Monster.  The Sox played great, even had a 9 run inning with a grand slam, so it was very exiting.  Best of all, right before we were about to leave, David caught a foul ball for Jacob!  They both were so excited, and it was really fun, all the fans around us cheering for and high fiving Jake.  It was a great experience that I think we will all always remember.

So now, we are in the waiting area, as Jacob went in a little after 8am this morning.  Overall, we are happy about how things are going so far, but will not take a breath until Jacob is out of surgery today, and hopefully, we get a good report.  Thank you to all who are praying for him and for us.  I'm not feeling too articulate right now, as my mind is very scattered with Jacob under, but hope this quick update makes sense, as at least it provided a distraction for me.  I will update again as I am able to.  Again, thank you all for your prayers and support.

Sunday, April 1, 2018


We are exactly 1 week away from heading back to Boston with Jacob.  I am sorry that I am not good about updating until we have an impending trip, but such is the nature of life.  We are too busy in between time for me to think about posting regular updates, and to be honest, writing here brings his condition to mind.  For the most part, though it is never far from my mind, ever; I have learned to embrace denial as a reasonable coping mechanism.  Not really even denial, as I do not dispute his diagnosis and accept it fully; but I guess avoidance is a better word...I just try not to bring it to mind on a daily basis.  There is so much unknown about his condition (none of it being good), that plagues me to think about, as I have written about before.  I wish I was able to think about it and not be consumed with fear and worry, but honestly, by account of not thus far being able to successfully battle my nature in that respect, I choose to try not to think about it instead.  My success on not thinking about it lately though has been minimal.  So many thoughts the past few months, so many little things nagging in my mind.  This also terrifies me, as it gets further in my head, making me wonder if it is my intuition trying to tell me something, or merely unfounded worry.

Going back, we sent imaging up in December, 6 weeks post-procedure, to see how the lesion responded to the treatment done in October.  Our physician team was discouraged at this imaging, as although his spine looked marginally straighter, the lesion itself was a mess.  Tons of scar tissue so that they could hardly tell what was going on in it.  They reinterated what they had warned us about on the previous visit, that they fear we are reaching the point (if not there already) where the lesion is too scarred to respond to sclerotherapy anymore.  This often happens with these large lesions, after which the patient has to really just live with the pain, there being no other good options for treatment.   They emphasized though, that due to the location of Jacob's lesion, and the proximity to and resulting damage to his spine, that that was not an option for him.  They know that to not continue to agressively treat his lesion, and keep it (relatively) small, that the continuing damage to his spine would likely result in paralysis.  Thus they are gravely concerned about the prospect of his lesion not responding and shrinking after sclero.  Because of that, in order to best gauge the current condition of his spine, and to evaluate additional treatment options, they made us an appointment with a spine orthopedic specialist.  If he feels uncomfortable with the condition of Jacob's spine, and his lesions' response to surgery, then they want us to move forward with discussing the next option: surgical resection.  Now surgical resection is not something that is routinely done on patients with Jacob's condition because it has proven largely unsuccessful.  I mean that even if a large lesion is removed sucessfully, remember this condition is like a genetically inborn error telling his body to make these lesions.  Thus, after surgical removal, usually the lesions grow right back.  Any result is temporary.  In addition, surgery is very risky, as the lesions are vascular, and thus prone to bleeding when attempting to remove.  Jacob's would be riskier than most, with his lesion being so large, actually described to us as "hundreds" of small lesions all enmeshed together, and tangled up in his chest with his vital organs.  His extends from his back through 4 ribs, entering his thoracic cavity, invading the pleura (lining of his lung) on the right side, then wrapping between his heart and spine, to go through 2 or 3 ribs on the other side back onto his back on the other side.  So we're talking a very invasive lesion, that would be very dangerous if not impossible to remove.  In addition, bleeding or manipulation could result in paralysis during surgery as well.  So this is really not a good option either.  I told them over the phone that they would have to convince me that they felt strongly that something imminently catastrophic was about to happen for me to consent to something that could have an equally catastrophic result.  They understood, and said they don't know that we are there yet, but wanted to prepare us for the possibility.  Bottom line, is that they did not expect for neither his lesion nor his spine to be in the shape they are in with him being only 6 years old, which makes them very worried about his prognosis with all of the growing that he (and concurrently his lesion) still have to do.  So that is the long and short of it.

I am trying to not get too carried away with this prospect, and hope that once we get up there, that it will not look as bad as they are expecting.  Honestly, they even told us that we may get up there and them look at it and say that they cannot even do a sclero this time, due to scarring.  They made an appointment the day before the procedure for his doctor to ultrasound him himself and make the final decision.  But I am just hoping that they can treat it one more time.  And after that, I will hope for "one more time" again, and then "one more time" after that, and so on.  I hope we are never faced with the prospect of surgical resection as the only option.  I am very discouraged and scared that we are even discussing it right now, but am remaining hopeful that we aren't there yet.  I am also terrified that we will be asked to make this impossible decision that I cannot for the life of me imagine making.

I love this child (as I do all of mine), more than my own life.  And would do anything to keep him healthy and give him the best possible life.  I look at him, and watch him playing, and my breath catches in my chest.  It is inconceivable that he have anything less than the beautiful freedom of childhood that he has now, and the strong little body to explore the world in which he lives.  I hug him so tight, breathing in his smell of grass and dirt, and that mysterious little boy smell that I cannot place but know it with my eyes closed.  I run my hands through his beautiful blond mop of hair, and don't want to think about what is growing inside him, threatening his safety.  His eyelashes that are impossibly long, and that lone dimple on one cheek, and that adorable lisp, that I cannot seem to remember to schedule speech therapy for (sorry speech friends, I know I should...),  and every little expression and nuance that makes this child who he is, both delights and terrifies me as I do not want to imagine anything that could dim the brightness of my son or steal his joy.  I have said it before, that he is my hope and fear all wrapped up in one little boy I would go anywhere or do anything to protect.  But I know I cannot.  At the end of the day, I believe his life is bigger than me or any decision that I could make, and his safety lies in the hands of the One who created him.  It is just hard for me to remember that, and I tell myself that a thousand times, but let myself believe it a fraction of those times.  I know I should, and that it is arrogant not to, it's just hard to let go.

Belief is a funny thing.  I think there is a distinction there, one between "knowing" and "believing."  There are many things I know.  Knowing implies a knowledge, housed in your brain, that impacts what you do.  Belief, I feel is in your soul.  It is a security of something that you know to an extent that it impacts how you feel in your heart, permeates the core of your being...who you are.  I "know" in my head that my son's health is out of my control, as are so many things in life.  I don't think though, that I have ever crossed into the state of "believing" that.   I believe that the choices we make do impact his chances of a healthy life, and will as such continue to fight for him to get the best care.  But I know that simultaneously I cannot do enough and fight enough to ensure a healthy life for him.  I just struggle to allow myself believe that and thus carry a burden of responsibility that I know is unfair and misplaced.  I've spoken before of my personality and also what is ingrained in me as a nurse, both of which make me battle that; but I say it not as an excuse, because I know I would worry less if I could simply let go and believe.  The fears pile up and weigh me down at times, casting a shadow so big that it covers my whole life and everyone in it, I'm afraid.  I don't want to lose heart.  I don't want my fear to be bigger than my hope.  I wrote a post titled "On Fear" a while ago, and have made myself read it a few times lately.  I so want to believe.

The children's hospital where I work, is building a whole new hospital.  An entire new children's hospital multi-building facility.  This is a multi-stage, gigantic building campaign, aptly named the Believe Campaign.

It has been planned for and talked about for many years.  I remember hearing about it in the early stages, years ago and thinking, "yeah that'll never happen" or "I'll believe it when I see it," or even "IF we ever get a new hospital..."   Now every day as I drive in, I see phase one being constructed before my eyes, looking closer to completion every week.  I see money being raised, goals being met, and more plans becoming reality.  I had a moment the other day, I didn't think about it, but it just came out of my mouth "...when the new hospital is done and we have our new unit..."  Not "if", but "when."  I'm not sure when it happened.  When I moved from doubting to knowing to believing.  But somehow, someway, I did.  Without even realizing it.  I want to believe like that, about Jake.  That he can stay healthy.  While I still do, and forever will reach for and be grateful for Today's Miracle  that I have written about so many times, that Jacob simply be healthy today;  I want more.  I want to believe in a miracle for tomorrow as well.  And the day after that, and the day after that.

Jacob is a big lover of music.  One of his favorite songs lately, is from the Trolls movie.  It is a beautiful, stripped down version of "True Colors" performed by Justin Timberlake and Anna Kendrick.  I have been trying to commit it to memory, so I can sing it to Jake on the upcoming trip.  One of the lines keeps sticking in my says "the darkness inside you makes you feel so small."

I feel that darkness inside me these days...that fear that casts a shadow over me that could reach everyone I love.  I don't want that.  I want belief to come from my soul and cast light over my fear, and also over everyone in my life.  I pray one day I will.  For today, as I'm feeling weak, I will be thankful for those in my life who cast light in my darkness.  Those who love my son, and my family.  Those who know me, and my failings, and somehow still love me and turn light my way.  Those who believe when I'm not strong enough to.  You all know who you are.  I love you.

Pray for my Jacob.  Pray for us as we travel, for the doctors as they examine him and help us navigate what's best for him.  Pray for their hands when they touch him, to be used for healing. Pray for my babies left behind, and our family as they love and care for them.  Pray for me. Pray for hope for my heart.  Pray for me to believe.

Disclaimer:  The image of the "Believe Campaign" is the property of the Erlanger Health System and all comments made here are my own thoughts, and are not necessarily reflective of the views of the Erlanger Health System.  

Saturday, October 7, 2017

Over...and Out!

We had to get up way early to be at the hospital at 0630.  David and I got up and were getting ready, and we tried to let Jacob sleep as long as possible.  He looked so sweet sleeping, it was hard to wake him up...

He woke in good spirits though, and we made our way to the hospital.  In no time we were checked in and back in preop.  Jacob did great, and was excited to put on his cape, which he made sure to pack himself in his bag the night before.

BCH's preop is pretty low key, as they do not to anything to your child except take a set of vital signs.  Everything painful is done after they put them to sleep.  It's awesome.  So all we did was have a brief chat with our physician, anesthesia, and the nurses.  It was interesting, and I mentioned yesterday, how at our preop appointment, anesthesia had scoured all of his past procedure records and were dissatisfied that they couldn't keep Jacob from experiencing post-op nausea and vomiting.  Again before the procedure they reinterated this, and discussed some of the strategies they planned to try this time to improve his experience.  You could almost see it in their faces..."oh he throws up every time...Challenge Accepted."  I was amazed that they didn't just dismiss this response to anesthesia, as some people just respond that way.  Nope.  They were determined to make it different this time.  Once everyone on the team was ready, I went back with Jacob into the OR.  There I held his hand as they put on his watermelon smelling mask, and coached him on breathing deep.  He did so great!  He was very calm and unafraid, and the team was so amazing.  They just talked to him like everything was normal, and talked about what a good diver he was, breathing into the mask.  They are so awesome to learn about your kid and find out what speaks to them.  I was so proud of a couple minutes he was completely asleep, so I kissed him and went out.

Now our wait began.  We got updates from the surgery liaison every hour, and finally our doctor  appeared to tell us how it went.  Jacob did really well during the procedure.  They were again surprised at the size of the lesion, and everywhere he treated an open channel, the blood would reroute and open another one.  It seemed like it was a difficult process, and he emphasized the size of the lesion, and the necessity of ongoing aggressive treatment to prevent complication.  There again was involvement in the channels going through the ribs, and re invasion of the pleura.  He also said there was some connection between the abnormal venous malformation lesions, and drainage of these abnormal channels into the spinal veins, which is a big concern.  He also had to take great care not to get any of the toxic sclerosant into these veins.  He emphasized the necessity to constantly be aware of any signs of neurologic compromise.  He also expressed concern with the muscle on his back.  This muscle is becoming very damaged from ongoing invasion from these vascular lesions, and he is concerned about its function, and Jacob's posture and arm strength as a result of this.  He wants Jacob to start some physical therapy once he recovers, to reduce the scar tissue and subsequent tightening of that muscle.  We will also of course continue to follow with the ortho specialist, the curvature and rotation of his spine.  Bottom line, is that Jacob continues to demonstrate the need for ongoing aggressive treatment of this lesion to maintain his health and growth.  He still feels we can continue to attack this from a sclero approach (thankfully), but these trips and procedures will unfortunately be a necessity for the foreseeable future.  Again though, I will try to be thankful we have options for treatment, and that this lesser invasive approach continues to be effective.

We finally got the green light to come see our boy.  He was resting quietly in PACU, and was looking pretty good.  We let him sleep as long as possible, hoping he would sleep through the worst of the pain.


Finally, they were ready to see him wake up, so we started to rouse him a bit.  He woke up peacefully, and was very calm, unafraid, and seemingly with minimal pain.  He even tried to give me a smile.

He soon was ready for a snack and a drink, and I held my breath for his usual MO of getting sick.  It never came!  Our anesthesia team came by PACU 4 times to check and see if he had thrown up, and were pretty pleased with themselves that he never did.  I was pretty pleased with them as well!  They tried a slightly different approach, giving him less gases and more melds, and kept him on an EEG throughout the procedure to ensure he was completely asleep.  It worked!  Again I am so thankful for a team so persistent and motivated to give my son the easiest most peaceful surgical experience possible.  They are amazing.

Jacob soon wanted to play something, and look at his Batman sticker book, so he spent some time on that as we waited for a room. Other than an episode of severe pain requiring him to get some Morphine, he was calm and comfortable in PACU.

Once our room for the night was ready, we were moved to the HEM-ONC floor, to the team we who follows the IR kids.  We were excited that this weekend's attending on the floor was the Hem-Oc specialist who works in the Vascular Anomalies Center and handles all of the cases of BRBNS requiring the drug Sirolimus.  We met with him for the 2nd time back in March if you remember, and he was full of information for us, on all of the newest findings with this drug, and with BRBNS.  He is awesome.  Immediately after getting in our room, we got a special delivery! Jacob got a stuffed toy and balloons from his best friend Cole Smith, son of my life long friend Jackie (Money) Smith.  That was so sweet and a nice surprise for Jacob.

After a bit, we were able to talk Jake into getting up and walking to the playroom.  He wasn't too sure about this idea, but it always does him good to get up and moving, so we persisted and up he got.  As we thought, he perked up in the playroom, and even played a little air hockey.

It was nice to see a real smile, and though he didn't last long, we had a good time playing.  He ate a good (albeit late) dinner, and watched a movie.  We slept surprisingly well, only waking a few times during the night, and by morning, he was looking much better.

I felt confident that he didn't need any stronger narcotics throughout the night, and kept his food down, so we were chomping at the bit to go.  They actually rounded early, and we got the green light to leave.  After another quick game of air hockey while they got our paperwork together, we were on our way.

As much as we love it and are thankful for it, we were happy to say goodbye to BCH for this round.  It was a beautiful day, and we all needed some fresh air, so we set out on foot for the Yawkey house.

The previous day and night caught up with all of us, so we took a long nap after getting home.  We all felt better after waking up, and the Yawkey house gave us free tickets to the movie theater close by, so we decided that would be a good low key outing for the evening.  It is a really nice theater with the reclining seats, so I felt good that Jacob could lie back and be comfortable to watch a movie.  The Lego Ninjago movie just came out, so it was perfect.  We had a great time.

We still have until Monday night before we return home, so will try to keep ourselves occupied for the next couple days.  Thank you so much to all who called, texted, left messages, and comments.  Such an encouragement.  And thank you again so much for all who held thought or prayer for us this week. Please continue to pray for Jacob as he recovers.  Much love to all of you, from half of the Casteel's in Boston.