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.

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