Friday, March 3, 2017

Results and since then...

I know many of you have been waiting to hear how the procedures went, but honestly, my mind has been too focused on Jacob's recovery, to be able to compose an update.  It has been an eventful couple of days though.  You know that we proceeded with the surgeries without insurance approval, in faith that this would be taken care of somehow.  I had spent 2 hours on the phone the night prior to the surgeries with an associate who was so moved by our situation, that she stayed over her shift on Wednesday morning, to contact her supervisor about our case, and to get us some help.  Her supervisor called me around 10am to notify me that our procedures were given full approval, and that our network exception was expedited, covering any and all of the care Jacob may need outside our network at Boston Children's.  This is something that took me months to achieve in 2014, which is why I had lost hope learning that we lost it the day before his surgeries.  After 2 weeks of getting no where, and our approvals getting yanked, I was at the end of my ability to cope and to fix this.  I know it was no accident that my after hours phone call that was transferred 4 times made it to this caring associate who listened to me and felt enough compassion to go the extra mile, past all of the red tape, and past all of the roadblocks, to make this happen.  She even conveyed our situation so well to her supervisor that she apologized to me for the treatment we had received, and gave me her personal number so that any future needs that arise for Jacob can be handled by her personally.  When I went to bed Tuesday night, I was so discouraged, and had so many doubts and questions about what we should do.  On Wednesday morning, I awoke feeling like I had to just give this up to God.  Stay on the path He had set us on, and believe He could handle this.  I'm such a doer, that was hard for me.  But I knew Jacob needed me more, and it was such a relief to put this in God's hands.  I never expected so quick an answer though.  It's so amazing to me how fully and completely this issue was taken care of.  How great His care is for us, even in this.  

So Jacob was put under at 0830 and we got brief updates every 90 minutes.  Around noon, our Interventional Radiologist who performed the sclerotherapy and embolization aspects of the procedure had finished and came out to speak with us.  He said that the lesion is quite large (which we knew), but even larger than he expected.  He treated the largest and most invasive areas, did see some immediate shifting and rerouting of the lesion, and tried to close off some of those areas as well.  He treated a very extensive area, using a combination of sclerosant and glue.  There was reinvasion into the pleura (lining of the lungs), which he had to treat as well.  Overall, he was pleased with the result, but did not want to let it get that big again.  He said he really needed to see Jacob more often to keep this as safe as it can be for him.  He wants an ultrasound in 6 weeks (which we can do at home and send up), and then likely we will go up for another sclero.  Either way though, he said he really needs to see us every year, or possibly every 6 months.    We just cannot see what is going on in there, and the precarious location of this lesion does not allow for excessive growth. And extensive treatments like this are more risky and harder on Jacob's body.   Though the last 3 years of no issues have been nice, its clear  now that that was not the safest route for Jake.  Honestly, this is a relief for me.  The pressure of deciding when to treat, when to push to go was so hard for me.  The stakes are so high, and it was so hard to know if I was making the right choices.  It is a relief to know that we will stay better connected, and allow the experts to help me navigate these decisions.  
Next came the cerebral angiogram.  Different physician performing this procedure, a Neuro-Radiologist.  For this, they accessed his femoral artery in his groin, and threaded a catheter up into his carotid artery, and into his brain.  He injected contrast, and studied the blood flow in Jacob's brain, looking specifically at the malformation there.  He came out a little after 3, to tell us the results.  Jacob does not actually have a vascular malformation in his brain!  What looked like one on the MRI is actually called a DVA, or a developmental venous anomaly.  The particular kind that he has is a normal variant of venous drainage of the brain that many people have, and isn't related to BRBNS.  Jacob is more prone to atypical vasculature, but this one isn't a problem.  Basically, where normally people have multiple small vessels that are evenly distributed to drain blood from the brain, Jacob has 1 large vessel that drains that side of his brain.  This does not pose a danger to him, nor does it need treatment.  He needs no neuro follow up, unless he has new symptoms that suggest an issue, such as a new bleb, which is still unlikely even within his condition.  This is the best possible outcome that we could have hoped for!  To remove this frightening problem from off the table was such a relief, so we said thank you to the Neuro-radiologist, and got to cross this off the list.  

Jacob was under for just a little bit longer, while the original physician tagged back in and sclero-ed a small lesion on Jacob's ankle that has been bothering him.  This was quick and uncomplicated, so before we knew it, a little before 4, we were taken into recovery where Jacob was extubated but still sleeping.  He roused every once and awhile, but seemed very comfortable, and was allowed to sleep through what would hopefully be the worst of the pain.  

He wasn't allowed to move his leg for 4 hours after the angiogram, so they had been very generous on his pain meds and sedation.  Sclerotherapy is a painful procedure, his more than most because of the pleural involvement (a highly innervated area, think "pleurisy"), so keeping him snowed ensured him to be pain free and unmoving for the whole of the 4 hours.  They were skittish too, because he already had some excessive bleeding at the angiogram site, which required them to remove the origional dressing, and put an adult sized one on, with a larger balloon, which they inflate to keep pressure on his femoral artery for several hours.  Other issues resulted from the extensive sclero treatment.  The sclerosant is essentially a toxin, which some is absorbed and has to be filtered out by the kidneys.  It is hard on the kidneys, which is why multiple treatments are usually necessary, as they can only give so much at once.  So because of the amount of blood in his urine and decreased urine output, he had to stay on a bicarb drip and get extra fluids and lasix through the evening until this cleared.  Finally around 6, they had me try to wake him up.  He woke up great, ate a popscicle, and was in good spirits, with little report of pain.  I was so excited.

We were moved to a room on the Hem/Onc floor and settled in.  He drank a little juice, and then soon after threw up.  He immediately looked awful, and was hurting.  After getting cleaned up, and with some IV pain & nausea meds, he fell asleep around 10.  I did too and we slept until midnight.  David went back to the Yawkey to return in the morning, as someone has to stay in our room every night or you lose it.  Jacob woke up around midnight, and was pretty pitiful.

They took his catheter out, so I thought he'd rest better, but he stayed up until around 4 (so we both did), and then we slept until 6.  David arrived back at 7.  That morning he kept down some breakfast, but stayed pretty puny.  He did FaceTime with his class, which he was (although low-key), very excited about.  It was so sweet to see how much his friends cared about him.  

He also was excited to finally get to open all of the cards that he has been given, which I have saved until now.

We did get him up and to take a walk to the playroom, but he wasn't very happy about it.  

He was amused by the Star Wars Operation game, so we got a couple smiles from him.

And he enjoyed painting a piggy bank.  

We went back to the room after having him up for an hour or so, and all 3 slept for a couple hours.  The physicians came back to check on him, and weren't happy about how much he was eating and drinking.  They agreed to take him off the fluids and consider letting us go if we thought we could get him to drink more at least.  Challenge accepted!   We spent the rest of the afternoon, with another walk to the playroom, which he looked much better, and trying to get him to drink.  

It proved harder than we expected, but finally around 4 we had got him to drink enough to earn us some walking papers.  He still did not look good, but I felt (and he has proven historically) to improve dramatically once we leave the hospital.  Thankfully, he did.  We got back to the Yawkey and he and I got cleaned up-he looked (and I felt) so much better.  He even ate some dinner.  The exhaustion set in and we all went to bed at 9 and slept until 8 the next morning.  I felt like a new person today, so many weights lifted.  Jacob looked great! No complaints of pain, and moving around really well. He still doesn't have his appetite back, but I know that will come. Overall he looks better than he ever has this soon post-op.  

He did have a new appointment that our IR team made for him on Wednesday after the procedure.  They were concerned about how the lesion is affecting his spine, which they could see during the procedure.  Although there is adequate space between it that they aren't concerned about spinal cord compression at this stage, his spine is curving away from the lesion both sideways, (like an 'S'), and anteriorly (like a hill on a rollercoaster).  They wanted us to see the orthopedic physician who is part of the Vascular Anomalies Center, and works with venous malformations that influence bone growth.  She squeezed us into her schedule before we left, which was great, so we were back at the hospital at noon for some x-rays, and for an appointment with her.  I had been feeling that there was a curve to his spine for a while now, but was shocked to see how much.

Jacob has a 14 degree curve of his spine at this point.  She did say that this is considered a "mild" curve, as many people live their whole lives with a 10-15 degree curve with no issues, and never need treatment.  The issue is that Jacob is so young, with so much growing still to do, and with something that will always be pushing on his spine.  Now we know what is causing his spine to curve, so the hope is that with shrinking the lesions with the sclero, that it will relieve some of the pressure and allow his spine to straighten up.  This demonstrates how we cannot go this long between treatments, even if he is asymptomatic, as continued growth of the lesion will keep pushing his spine more and more.  A side note, she also found that one of his legs is 1 inch longer than the other, which can contribute as well, but this difference is not really enough to need a shoe lift or to cause issues.  She wants to see the results from the sclero and will go from there.  So he will get some x-rays in 6 months to see how it is looking.  We may even have another sclero treatment in by then.  This appointment went well, though it was not necessarily the news we wanted, it could have been worse.  He doesn't need anything done right now, but this is something that needs to be followed.  I'm thankful to have gotten him plugged in with the right person so quickly.  

This completed our appointments and treatments, so we bid goodbye to BCH for this round and headed back to the Yawkey.  We napped for an hour or so, and decided that an outing to see the Lego Batman Movie would be a good choice for Jake.  Low key, but something he was excited about.  We did that, and had dinner, and came back "home" for the night.  He did great today, but still tires easily, had a little bit of pain, and isn't eating much.  But I'm so pleased with where he's at, especially when I compare to where he normally is at this point.  He is such a strong little boy, I am so proud of him.  

Thank you all who have reached out to us the past 48 hours.  To say they have been trying would be an understatement.  The amount of sleep that I got, combined with the emotional upheaval of all that has gone on had me at the end of my ability to cope.  To know how many cared about us, and were thinking and praying for Jacob, was such an encouragement to me.  I with I could express in some way how much it meant, but I hope you all know.  In addition to our being carried through all of this, the miracles we witnessed happen for our son, I know are a direct answer to prayer.  Thank you all.  I know that though this trip is almost at its end, our journey with Jacob is far from over.  He will battle this his whole life, and we will fight beside him.  I have experienced discouragement in the past few months, and likely will again, but for today, I am looking forward in HOPE.  Hope that my boy's future is as bright as the light that has shined on us this week.

Wednesday, March 1, 2017

By the skin of our teeth...

Oh what a 24 hours it has been! Our biggest concern on our last update was squeaking his flu past anesthesia.  That turned into a complete non-issue, and others arose and took their place.  Let me start at the beginning though.

First I wanted to show you something amazing that the mom's of the kids in Jacob's class at school did...

I got this picture yesterday from his teacher, and was so touched.  I cannot believe that they did this and am so thankful for these friends at Middle Valley Academy, who love Jacob and are continuing to pray for him.  He misses them so much, and I'm hoping he'll be well enough Thursday to facetime with his teacher and friends.  We love you all, and thank you for doing this.  

Yesterday, Jacob was looking great with no flu symptoms, and had actually had none for a couple days.  So we decided to take him to the aquarium, as he was feeling cheated from all of the fun things he knows this city had to offer.  We figured with him not coughing or sneezing or even having a runny nose, that we wouldn't be exposing anyone, just walking around looking at the fish.  The hospital was so gracious to give us tickets, so we were able to go for free!  Those of you who know him well, know how much he loves aquariums, fish, undersea creatures, divers, and really anything aquatic related.  So he was in heaven!  They had penguins and seals, awesome tanks, and a great petting area.  He absolutely loved it!  And he got to see the divers in action, and even got to see them up close when they got out.  He wants to be a diver when he grows up, so this was a highlight for him.  We had a great time, and it was good to get out and do something fun.  I have tons of pictures but left my camera in the room back at the Yawkey, so I will try to add them later.

We ate lunch at Wahlburgers, and made our way back to the hospital for preop testing which we were nervous about. If anesthesia did not feel comfortable with how he looked, they could pull the plug on the whole thing.  He sailed through with everyone marvelling at how he recovered so fast because he looked like he had never been sick!  They couldn't believe he had the flu over the weekend and deemed him in perfect condition and fit for surgery.  Friends, I know how many of you prayed for Jake this weekend, and all I could think at their amazement was...BUT GOD.  There was no reason he should look like he does...but God.  Thank you all so much.  He heard, and He answered.  So with anesthesia's stamp of approval, we went on our way.

We had a break for a couple hours before our final appointment so David and Jacob walked back to the Yawkey Inn for  a rest and I stayed behind to fight another battle that arose during lunch--- insurance approval.  I thought at the beginning of this that this trip was going to be easy.  We received our approval for his procedure at the first request (which has never happened before), and figured it was because of our long standing network adequacy exception that I achieved 3 years ago, essentially proving that no one in our network could treat his condition.  Questions began coming up at the end of last week though, in regards to the 2nd procedure, which I never received approval for.  Every time I contacted the hospital, they were like, "we're good, no issues-all approvals in place."  But then when I contacted our insurance, they gave me increasingly strange questions.  Finally yesterday at lunch I tried to confirm with them, and they said that the procedure approval (which I had in hand) was now in question, and in reality, going to be revoked.  They asked where Boston Children's Hospital was, and I stated, "Boston" and they unbelievably asked what state, I replied "Massachusetts."  They said they did not realize that and should not have given their approval in the first place and that we could not have the procedures.  You can imagine how that went over.  So round and round we went, all day, with me spending approximately 6-7 hours on the phone, talking with multiple representatives from Cigna, multiple people from the hospital's finance dept and our doctor's offices.  I finally ran out of people available to talk to last night around 8, and had to stop for the night.  After a fitful evening, I awoke with peace today.  I felt God did not want me to fight this battle any longer and give it to Him.  I decided I was not going to work on this at all this morning, other than to confirm with the hospital that they still had an approval and no information otherwise (which they did).  We felt we could not be held responsible if our insurance company did not communicate with the hospital anything revoking the given approval, and for that matter, did not ever initiate contact with me stating we did not have approval.  All information I received was from contacting them.  We felt we should not put a stop to this, denying Jacob the care he needs, because of their issues.  We felt confident that we had enough documentation supporting our position and our decision to proceed, that we could deal with the insurance issues and fight that battle later, even if it meant in the legal arena.  I decided that I was going to keep my focus on Jacob this morning as he needed me to be present for him, and not distracted or on the phone trying to sort this out.  I let it go.  We had to be here at 7, so Jake was pretty tired, but we had a good time in preop, snuggling and playing.

I forgot to tell all of you, that something momentous happened this trip, thanks to my dear friend, Jackie (Money) Smith...Jacob finally caught the moon!

I am so glad that we did, as this morning at 10am, and hour and a half into the procedure, I received a call from Cigna, that we have a new point person on our case, and acknowledged the mistakes on their part, and assured me that our existing approval still stands, and that the network exception that was still supposed to be in place is being expedited as we speak.  He was confident in the full coverage of all of Jacob's medical care on this trip.  Again, friends...BUT GOD.  So many people reached out to me last night, so many were praying.  God heard, and answered.

I cannot express our gratitude to the host of you who have rallied around us in the past week, especially in the past 24 hours.  Every comment, message, share, text, and call has encouraged my heart in a week that has left me depleted.  Every prayer offered on our son's behalf, every positive thought, every heart that has held us close, each one of you has helped get us to this point.  I can never thank you enough or repay what has been done for us.  I said it once before--for all you have done, and for all of your support, Jacob's story is yours as well.  Please continue to keep him in thought and in prayer and I will update when he gets out of surgery as I can.