We made it!

After a rough weekend that put this whole trip in question, we made it to Boston!  Most of you know, but Friday Jacob came down with the flu.  After an appointment with our PCP in Chattanooga on Saturday morning, he counseled us to reach out to Boston and make sure they still wanted us to come.  Saturday was a long day in which we went back and forth with our physician team and the anesthesia team.  Our physicians wanted to move forward with the procedures, due to the potential risks of delaying this much needed procedure further, and understandably, the anesthesia team would rather wait for Jacob to fully recover from the flu.  The anesthesiologist was awesome, and actually contacted our physicians and considered all of the potential issues with postponing the procedures before ever calling me.  I felt great about this, as I did not want to plead our case or sway them in any way.  I would never want to pursue anything that would put Jacob at risk for convenience or to avoid emotional upheaval for me.  I wanted them to objectively look at this decision from all angles and make the best one for Jake, which is what they did.  Both the anesthesiologist and our physician called us at 9pm on Saturday night and told us that they wanted us to still come, with the understanding that the procedures will happen only if anesthesia feels comfortable when they look at him on Tuesday in preop.  If we were willing to come knowing that, which of course we were.  I told them that we respected their decision either way, trusting that they are truly making the decision they feel is in Jacob's best interests.  So after a long day in limbo on Saturday, we set out Sunday morning.

Our flight left Nashville Sunday morning, and Jacob was very excited.  He actually was feeling pretty good, despite the flu, with only a runny nose and a slight cough, but no fever.  He had a great flight, thanks to a couple of friends who decided to spoil him with new books, snacks, toys, and headphones for the flight!  He rested briefly, and stayed entertained the remainder of the nonstop flight.

We got our luggage and subway tickets for the week and made our way to the T (Boston's subway system).  He ended up sleeping in the stroller the entire way on the T to our home for the week, the Yawkey Family Inn.  The Yawkey is a renovated Victorian home built in 1889, that is part of the patient & family housing program for Boston Children's Hospital.  It is about 6 blocks away, an easy walk to the hospital, and where we stayed 2 out of the 3 trips we took in 2014.  It is a wonderful place that takes a great deal of stress off of us and many other families.

We took it pretty easy, being tired from traveling, and also needing to keep Jacob inside and resting as much as possible. We are trying to keep to ourselves more this time, with Jacob having the flu, and thus are avoiding the common areas, with the exception of the kitchen, as we have to use it.  There is no food allowed in the rooms, so all eating has to be done in the kitchen.

So we have been trying to stay away from everyone, and keep him from touching anything, as well as clorox wiping any area he eats or contacts to protect our fellow house mates and their children. We've been fine in our room though, thanks again to all the goodies from our friends, Misty Collins, Jackie, Smith, and his teacher Bobbie Toran.

Sunday night we walked to the grocery to get ourselves some food and necessities for the week, and spent the rest of the evening in our room resting.  Monday we got up and didn't do a whole lot.  We decided a bit of fresh air would do Jacob good since he pretty much was looking and acting completely normal and chomping at the bit to do something.  I figured we wouldn't expose anyone if we kept outside and away from everyone else.  So we decided to head to my favorite place in Boston, the Commons and the Public Garden.  Though there was no snow on the ground currently (unusual for February), the skating rink was still in full swing on the Commons.

Jacob paid a visit to the frog statues, and we continued up the hill, where there is a Civil War monument.  Jacob ran to it, excited, and wanted me to take his picture.  He didn't know what it was but said it looked like history.

We kept walking, enjoying some of my favorite views of the city, and crossed over into the Public Garden.  It looked different, with the pond drained (so really there was a big mud hole in the middle that normally is covered in snow and not such an eyesore).  We still enjoyed it, Jacob especially, who counted the squirrels (47), and loved the duckling statues.

David & I sat on the bench on which Robin Williams and Matt Damon had their iconic conversation in Good Will Hunting.

Jacob got really excited about the statue of George Washington, and said, "I know about George Washington and read a book about him at school!"  He wanted me to take a picture for his teacher. So here you go, Ms. Bobbie!

We crossed over the bridge and headed back to catch the T to our first appointment of the trip, at Boston Children's Hospital.

Our first appointment was with a physician who (in conjunction with the Vascular Anomalies Center), specializes in the treatment of kids with venous and lymphatic anomalies, and associated syndromes.  His primary focus is on the drug treatment for these conditions and ongoing research. We saw him in the VAC clinic back in 2014, but I really wanted to talk to him about all they had learned since then.  I had read about several new advances with all of their ongoing research, and wanted to make sure we were still on the right path with Jacob, and there wasn't anything new that could be of benefit to him.  We spoke at length, and he gave me tons of new information, most of which was very encouraging.  I try to keep track of medical info on here, as a record of sorts for myself, so if you are not interested in the medical talk, skip on past this...

Their emerging successes from the past 10 years has turned into extensive utilization of the drug Sirolimus, which historically is an anti-rejection drug used for transplant patients, for kids with lymphatic and venous malformations, and GI bleeding associated with BRBNS.  My question was whether or not they had seen improvements in the "mother lesions" of BRBNS patients taking Sirolimus.  He stated that they still were learning about this.  He said that they have found almost universal stoppage of GI bleeds, but continued unpredictability of lesion growth.  BRBNS kids still grow new lesions on the drug, and their mother lesions still grow.  He said some see an improvement in pain symptoms, mostly related to coagulopathy, but Jake thus far doesn't have many issues with pain, and isn't extremely coagulopathic.  So it is unlikely that there would be any real effects there, likely not worth the drug's side effects of immune suppression.  He did say that they are able to minimize side effects by titrating the drug and monitoring levels, and giving the lowest dose that maintains efficacy-which is different for each patient.  He agreed with our feelings that we were not ready to be on the drug, but said that if our sclero begins to become ineffective at controlling lesion growth, or the need for sclero gets closer and closer together, that we probably should revisit it, and it might be worth a try, even in the absence of GI bleeding.  I have felt that 3 years is a great amount of time for Jacob to not need treatment, and thus I didn't feel we were at that point yet, but it is nice to know that the drug possibility is in our back pocket.  I was relieved that he felt we were on the right path, and am excited about all of the new knowledge they will gain as they continue to study this drug and its benefits for BRBNS patients.

One of my other questions, which was also drug related, was that the initial trials of Sirolimus only recommended it for use for 2 years, and I have heard many reports of patients being on it much longer.  He said that they are using it much longer now, and that it is proving safe to do so.  That was also a relief, as if he ever needs it, Jacob could potentially stay on for as long as he needed it, and not have to come off and be right back at square 1.  

He also told me about several very exciting developments with BRBNS.  He said that they had a breakthrough that they published a year ago.  When we came up in 2014, they told us that they had isolated the gene responsible for BRBNS.  He said now they know what is actually faulty on that gene.  They have sent samples (some of which could have been our son's) to geneticists in Brussells, Belgium, who discovered more.  BRBNS is actually 2 genetic mutations occurring simultaneously that result in this condition.  It is the only known syndrome resulting from 2 spontaneously occurring genetic mutations.  The odds are even more astronomical than they originally thought.   He said they also have tracked these mutations to see what exactly they affected, and found that they change the receptors which are responsible for blood vessel growth and production.  They are currently researching how to switch these receptors off, or alter them to stop the abnormal growth altogether.  I know you may have gone cross-eyed with all this medical jargon, so I'm gonna reiterate that last statement...STOP ABNORMAL GROWTH ALTOGETHER.  That means cure this, people!   I recognize that success in this area, even immediately, would take years until implementation, but it still gives me hope.  My son is 5 years old, has a 1 in a million disease, and people right here in this city are working with people halfway around the world to find a cure, that if found, could come in his lifetime.  This is hope, my friends.  Not just hope for life, or hope for good quality of life, but hope for a cure.  And before you tell me not to get swept away by that or get my hopes up, I'm gonna quote Andy Dufresne from the one of my favorite movies, The Shawshank Redemption..."Hope is a good thing, maybe the best of things."  I believe that.  I've seen what my days look like when I lose hope, and what they're like when I cling to it.  So I'm going to cling to that hope.

We discussed much more at this appointment, but honestly my mind is such a muddle, I can't remember more.  One thing I had forgotten about this hospital was how important your child is to them, and how important you are as their advocate.  We talked to this physician and his team for over an hour and a half.  He never once looked at his watch, clock, or phone, or indicated in any way that we took too much of his time.  I felt kind of bad at the outset, as we did not need treatment currently, but I made this appointment strictly as an informative one.  He told me in 2014 that if I ever had questions or wanted to discuss this, not to hesitate, and he meant it.  I even apologized that we didn't need anything right now, and he said keeping us informed on the most current treatment options and confirming our plan of care is no less important than anything else.  He asked questions of us, and thanked us about what they can learn from Jake as well.  I was also struck, as I was also in 2014, at their ease and comfort with this condition.  They know so much and are so comfortable with these insanely complicated treatments, that it puts me right at ease.  I get to just be mom.  So I cannot express how good this appointment was for my heart.  I feel confident in the path we have chosen in treating Jacob's condition, and confident in the people in place to help us keep him healthy.

We stopped on the way out and let Jacob see his favorite thing in the hospital, just outside the outpatient elevators.  Its a ball maze of sorts and he thinks its the greatest thing ever.  He was so good in that (long) appointment, so we were happy to see him having fun watching it.

 Tomorrow Jacob has preop testing at 1:30 where the official decision will be made whether or not to proceed with his surgeries on Wednesday.  Please keep us in your thoughts and prayers, that the best decision will be made for him.  We will respect whatever they decide, and though it will be a setback to reschedule, I truly have peace about it either way.  We also meet with our lead physician at 5:00, as he is the one who will be performing the sclerotherapy aspect of the procedures on Wednesday.

Those of you who know me well, know what a struggle this has been for me of late.  I feel affirmed in the decisions we have made so far, whereas before we left, I felt like a failure.  I will continue advocating for the best possible care for my son, and hope that we will continue to be equipped to care for his physical self, but for the person, the man he will become.  A dear friend gave me this, which I wore on the plane ride here.

It reads, "A mother's love is true north, always guiding the way home."  I never imagined taking my baby 1,000 miles and putting him in the hands of strangers for care.  Care that would inflict pain on him, in the hopes of providing continued health.  But I pray that regardless of whether Jacob gets the procedures he needs this trip or not, that my love will be at the heart of every decision and will continue to bring him back home again.  

On Fear

Things are happening, and we are moving towards Boston, closer by the day.  The momentum builds, bringing us all closer to the place where we find answers and hope, but the place I fear all the same. The decisions we are faced with, the people who can help my son, but the risk he faces with each procedure.  Its a strange feeling, the excitement wrapped in fear.  I am thankful for that place, the people, and their commitment to the pursuit of knowledge and expertise, and for the hope of a bright future for my child.  But I do fear it all the same.

Fear is not something I am accustomed to. It’s never been a companion of mine.  I'm a fairly adventurous person, and am used to living life passionately and without hesitation or reservation.  I’m not afraid of the dark, roller coasters, bungee jumping, clowns, or things that go bump in the night.  My favorite shows are dark, think Criminal Minds and the like.  I work in an environment with high stress, high liability, and the stakes couldn’t be higher- the lives of people’s children.  I even leave this place, my safety net of coworkers and physicians, and get on a helicopter or ambulance where my partner and I will try to save a baby in the field and bring it back safely for the higher level of care our hospital can provide.  Sure, I talk to new nurses on “healthy fear”, which really translates to a respect for the burden of responsibility that we bear in caring for someone’s baby.  Accountability for the care that we provide.  The duty to relentlessly pursue knowledge and experience so that we can provide these families with the highest level of care that is in our power to provide.  But this respect or “healthy fear”, that we should never lose, differs from the paralyzing fear I remember in my early days of nursing.  That freeze up, shaking hands, don’t know what to do fear that every new nurse has experienced at one time or another, when facing a threat to their patient’s life.  I still fear the loss of my tiny patients; but time and experience, however still allows for a clear head, steady hands, and critical thinking during a crisis.  This is what I am accustomed to.  Our NICU team is on the offensive, pursuing healing for tiny humans, without fear or hesitation.  But up in Boston, on the other side of the table, I feel the fear and helplessness from putting your child in the hands of another, a stranger, and asking them to protect and heal this child you cannot imagine a world without.

These days, though, fear is always there.  At times it is the dominating emotion I feel in all of this with my son.  All of the unknown, all of the possibilities, each one more terrifying than the last, paralyze me.  I look at him sometimes and it takes my breath away.  I told a friend the other day that I remember how desperate I was for answers when I had none.  Now that I have them, I just want to go back to when I didn’t know.  To the bliss that comes with ignorance.  I know I don’t really mean that, as I am so beyond thankful to have a diagnosis; and I know the knowledge that we have has enabled us in this fight.  The knowledge I have gained has made me a stronger advocate.  But it sure would be nice if fear didn’t accompany that knowledge. 

I've said before, I don't want to live in fear of his condition and what may happen.  I try to allow him the freedom to be well, and be himself, and don't keep him under the shadow of my fears.  It is dark under that shadow, I know. I don't hover over him, or helicopter parent.  I keep this fear bottled in my mind.  I try to ignore it, and am successful most of the time.  But still it looms there in the back of my mind.  Hope and fear battle in me every single day.  Even our prayer, for “today’s miracle,” reaches for the hope, rather than fear.  But I acknowledge that embedded in that prayer is the fear of tomorrow.  Why would I need to pray for a miracle of good health today if I didn’t fear him not having it tomorrow?  In my grasp for hope I even somehow feel like a coward.  But at the base of it all, I think I fear because I don’t give him fully to The One who made him.  I hold him so tight, as if I can make the right decisions, and entrust him to literally the best physicians in the world, and that it will be enough. The weight of that is so heavy.  Somehow I’ve given myself the charge to keep him well, and arrogantly and stupidly think I can do so. The nurse in me, the fixer, just keeps pushing for that, when perhaps I should let him be.  Don’t get me wrong, I will never stop advocating for the best care I can get my son, and I believe that God has given medical knowledge and technology to us for us to use for His purpose.  So I will never stop pursuing the very best for my Jake.  But maybe in my mind, I shouldn’t carry the weight so much.  Maybe I need to better entrust the life and health of my child to where it truly lies. 

I spoke of my work in the NICU.  There we push the limits of medicine every day.  We take the impossible:  lives not ready for the world they have been forced into, and give them a chance at life.  I have seen firsthand the result of medical innovation and technology.  However, I constantly see the marriage between these two, and the miraculous.  In the NICU, I never discount or discredit The One who allows for such knowledge and medical advancement, and does the miraculous even when we falter. It's such a humbling yet freeing feeling, knowing that someone bigger controls what happens, and that our hands and minds are simply tools He uses for His will.  But with my son, I somehow struggle to keep my faith where it belongs.  Why do I fight what is so freeing at work, and let it bind me at home?  I want him to be well so much, and would take on the world to make it so.  My mind realizes that I don't need to, but my heart can't put that burden down.  

Still I will pursue hope every day.  Try to surround myself with it.  On the walls of my home, around my neck, on my wrist, and even tattooed on my back.  I don’t want to live in fear, I want to believe.  Believe in the miracle of today, and the hope of another miracle tomorrow. 

It’s ridiculous when you really think about it, as I know I’m not guaranteed any of my kids.  But that bond is so strong, and we hold so tight to them; it’s so hard.  I don’t want to live in fear.  I want to celebrate the person he is every day.  My sick baby in the NICU became that little baby with the dimpled smile, who grew into little blond haired toddler who became a big brother, and now this beautiful boy, so fun and so sweet.  Mischief and joy wrapped into one.  So much entwined in this child of ours…yes his illness is woven into the fibers of his life and our family, but it does not and will not define him.  And he is there, secure in the deepest places of my heart, just as his little hand still reaches to hold securely to mine.  My hope and his future are woven together, just as both mark my back.  I am still unsteady, but as I cling to him, I pray the simultaneous release of control will allow me to love more fully.  After all, these 4 little people of mine still need holding, and it’s hard to do that will arms full of fear. 

The Plan

So what’s the plan anyways?  I mentioned that we leave February 26, have some appointments the next couple of days, then surgery on March 1. Hopefully home on March 5.  I may be a little optimistic on the scheduling, as this will be the shortest trip we have made yet, but I am hopeful.  I have a feeling we will have to go up two more times in the next 6 months like last time, but we’ll see what they say based on what they find.  We had a video conference with the Neuro-radiologist that will be doing Jacob’s cerebral angiogram, or the procedure to map the lesion in his brain.  He assured us that this is a routine procedure for him, that carries minimal risk.  He will not attempt any treatment on this lesion at this time, the only goal is identification of the lesion, and mapping its extent.  He told us that if it is an arterio-venous or A-V malformation, that it will require treatment.  These lesions are high flow, and carry high risk of complication.  That is, you could predict that if left untreated, this lesion would have a problem (think stroke or aneurism), that would have catastrophic effects on his brain.  If it is found to be an AVM, he will take the images to the Cerebral Vascular Team for evaluation, and they will come up with a treatment plan.  Treatment of these lesions is more complex than the treatment he undergoes for the lesion in his chest, though.  The options with those, we don't really want to explore unless we get confirmation that it is a AVM.  If the lesion is a true Venous (only) Malformation, we will not treat it.  He was very honest with us, that Venous Malformations of the brain are exceedingly rare, even amongst people with BRBNS.  He has studied these lesions, and is actually getting ready to publish the very first piece of literature on this particular type.  They know that these lesions are low flow lesions, so they think the risk of a catastrophic bleed is very unlikely.  They cannot say for sure that nothing will ever happen with this lesion, but the risk of going after it is higher than the risk of complication from leaving it alone.  They also have found that Venous Malformations of the brain are usually part of the venous drainage system of the brain, and thus really cannot be removed or altered, without risking the integrity of the brain’s blood flow.  So we really will have no choice but to leave it alone and hope it never is an issue.  He will know immediately during the procedure which one we are dealing with, so we will know right away the results.  We are hoping and praying for a Venous Malformation, as even all the unknowns with this are less scary than the known risks of an AVM and the risks of their treatment.  I feel good, (well as good as is possible), about this, and about the expertise of the physician.  He is one of the best in the world, and was very honest and forthcoming with us.  I am also thankful for the safety in a team of specialists coming up with the best plan based on what is found, rather than just one person on the spot.   I am very nervous about this procedure, though, despite his assurances of its routine nature.  I guess I got a little taste of what I dish out. I routinely place invasive central lines in my job.  Catheters the size of a thread, go into a vein or artery, and I thread them into large vessels just outside of someone’s baby’s heart.  I get consents, and answer questions, at times even internally amused at the hesitation I often see in the parent’s eyes.  "Yes, I know your baby is only a pound.  Yes we do this all of the time."  Now here I am on the other side of the table.  Here is this man in front of me, telling me how routine it is to thread a catheter into my child’s femoral artery, all the way up, through his carotid, and into his brain, and further, right into this mysterious lesion.  "But don’t worry, I do this all of the time."  It’s laughable.  Humbling, really.  It's very different on this side of the table, when it's my baby we're talking about.  I suspect that I will be more understanding of the fear I see when talking to parents at work about PICC lines or Umbilical arterial or venous lines.  

Back to the procedures…before the angiogram, our regular Interventional Radiologist who treated Jacob three times in 2014, will do a sclerotherapy procedure on the lesion in his chest and back.  We won’t know the true extent of how much growth has occurred until then.  They will attempt to shrink this lesion as before, but its size will likely require more than one treatment.  We will see.  Once they are done, the Neuro IR will do the cerebral angiogram.  They will share anesthesia, that is, tag off, so he will only have to be put under once, and both procedures will be done in the same day.  I am very thankful for this.  They will keep him under for longer afterwards, as he needs to lie still on his back for 4 hours after the angiogram.  He has a great deal of pain after the sclero treatments, so I am thankful that he will sleep through more of the immediate aftermath than he normally does.  Depending how he does postop, he will stay in the hospital for 1-3 days afterwards, leaving us a couple more recovery days before we fly home.  All of the details are falling into place, and we even got our first insurance approval yesterday!  This was a first, their approving it at the first request... usually they deny, and then we start the back and forth fighting until they concede and approve.  This is a huge relief off my shoulders.  

Two weeks from today we leave.  I am up and down, but have peace with the plan.  I find myself clinging to Jacob more of late; as I often do, when I'm feeling uneasy about his health and unsteady about my own state.  Please continue to pray for my baby.

Thoughts, Words, and the Color Grey

The plans are made.  Plane tickets bought, rooms booked, procedures and appointments scheduled.  We leave for Boston February 26.  Jacob will have surgery on March 1, and if he recovers well, we will return March 5. I will give more details later, but for now, I’m just too tired.  I’ve sat down to write several times.  Somehow the words won’t come.  I know it helps me to write.  A therapy of sorts.  I know keeping things in doesn’t help.  Still the words haven’t come. 

I like noise these days.  With 4 kids, most of the time it’s not a problem, but if I am alone, I keep music on or the TV, anything to drown out the silence.  The noise in my head gets too loud if it’s too quiet.  I try not to think too much, delve too deep.  I’m afraid of what I may find.  I’ve thought many times over the past two years, about how good I’m doing with all of this with Jake.  But in the back of my mind, doubts reign.  Am I doing good because I’m truly doing good, or is it just because he is doing good?  Am I ok only because he is?  I guess the doubts were founded, because as soon as it came creeping back, I began to feel myself falling.  Not like last time, or maybe so; maybe now I’m just better at hiding.  Hiding from my own thoughts, hiding my anger and fear and grief from everyone. 

I feel guilty.  What right do I have to question this?  What arrogance to see and care for the suffering children of others all around me, and feel outrage when it is my own.  I see so many walk these dark roads with such grace, such unwavering faith.  And here I am, drowning under the weight of a burden no one can see.  I look at my son, and the perfection that he is.  I feel no right to struggle with this.  And I’ve said before, I am thankful, truly.  I have seen firsthand how fortunate we are for the health that he has.  The thing is, though, I know there is no levels of health that are acceptable for our children, other than the fullest level of health.  It doesn’t make it ok that “it could be worse.”  And most do not understand that he is great…until he is not.  And the devastation of what the “not” could mean.  I do know that I shouldn’t make myself crazy over what if’s.  And that I could worry and stress my entire life over possibilities that may never come.  The unpredictable nature of his condition is truly the worst thing for me.  My make a plan, take charge, and make it happen self is completely derailed by this “wait and see” diagnosis.  

I pray for Today’s Miracle, and sometimes I wonder if I truly believe we will get it.  I am always preparing myself for what may happen, as if it will somehow soften the blow.  I blame myself, as if my unbelief could be why we wouldn’t get a miracle for Jake today.  Again, such arrogance, as if the health of my son revolves around life lessons for me, though the mere thought of that is absolutely infuriating.  I battled the anger last time, with God mostly, to be honest.  The perceived injustice, and the suffering our son would and has endured.  It does creep back at times.  I broke down in our small group a couple weeks ago.  We were talking about being thankful in all things and for all things.  Before I knew it, tears were running down my face.  I couldn’t stop them, and finally shared my brokenness.  Romans 8:28 burns off the pages of my Bible.  I cling to Romans 8:26 instead, the promise of intercession even when we’re weak and don’t know what to pray for or can’t pray as we should.  Romans 8:28 is too heavy.  What good is there in this?  I think I could see good in my own pain, the personal refinement it would bring, how much I could help others.  But it’s hard to see the good in the suffering of my child.  What is this worth?  And again, any personal refinement just makes me hate myself, if that could even be the larger meaning.  I battle anger with myself these days.  I see myself struggling, and hear Dave Matthews’ words, “She would change everything about her, using colors bold and bright, but all the colors mix together, into grey.”  I would.  Make myself stronger, with more faith, less worry.  I keep trying, but the colors just keep mixing together…into grey.