So How is Jake anyway?

Ok, so I haven't been intentionally avoiding this question, I know many have been asking me about him, but I have been stalling, because I didn't have an answer until now.  He has been doing well from day to day.  We have seen some things that have been concerning, which I will share with you...

First, is that Jacob has been having some episodes of abdominal pain.  They are always relatively brief, but leave me with a very unsettled feeling.  He has had no GI bleeding, just pain and some transient abdominal distention.  We are well aware of all of the GI issues associated with his condition, but are hoping to evade them for as long as possible.  Please continue to pray for no problems in Jacob's intestines.

Secondly, I have been concerned that he has of a lesion that is coming up on his left ankle.  For my friends in medicine, it is right where your saphenous vein runs up the ankle, and at first that is what we thought it was.  But is has continued to get more prominent, and different from the other one.

It's hard to see in the pictures, but when you compare with the other ankle, you can see the blue spot coming up.

As we have seen it getting more and more noticeable for the past year, I pointed out its growth last July in Boston.  But it has never caused him pain or problems...until lately.  I don't know that it is a problem yet, but he has been complaining of his leg hurting for the past 2 months or so.  It is pretty frequent, anywhere from every couple days to a few times a day.  And it is also consistent, the same leg, in the same spot.  I have even tried to divert attention from it and determine if he was just wanting something to complain about, by pointing somewhere else when he says it hurts and asking if it hurt there, and he says "No, mommy, right here!" and points to the same spot.  It looks small, but it is coming from his ankle joint, so could be bigger  than I realize, if I can only see a small portion.  I know how problematic lesions in joints can be to bone growth and function, so I am very nervous about this.  I just called our Boston doctor's attention to it, and they agree that it sounds like a developing lesion, whether it is big enough or problematic enough to chase and treat, we do not know.  They told me that if the pain persists or gets worse, that we need to ultrasound and try to see what we can.  I still feel like it's small, and for the most part does not interfere with his activity, so I feel we need to just keep watching it.

Our last and biggest concern is that we can see and feel his back lesion growing.  It is spreading lower than we have ever seen it go before on his back.  My biggest worry, though is if it is growing on his back, what is it doing inside his chest where we cannot see? We have been in contact often with Jacob's doctors in Boston, and they have been planning since October to repeat his ultrasound of his back in January, to see how things look 6 months after the last treatment.  So we went ahead with the ultrasound, feeling that although it can only show us his back, it can at least give us an idea of how it is looking.  We had the ultrasound 2 weeks ago, and sent the images up to Boston for their review.

I have struggled with this place, not knowing, and guessing what is going on inside my son.  It is a hard place to be in, as we hoped the growth of his back lesion would mirror the growth of his intrathoracic (inside the chest) lesion.  We hope this because the only way to see the intrathoracic lesion is to do an MRI under anesthesia.  Ultrasounding the back is simple and non-invasive.  But these lesions did not mirror in July, his back lesion was tiny where his chest lesion had grown and reinvaded his pleura.  So I have felt at a loss to know what to do.  I don't want to keep putting him under, chasing something that currently could be fine, and not let him be well when he is well.  On the other hand, I don't want to just assume it is fine, when it could be growing and putting him in danger, with its location and proximity to his spine.

I spoke with our doctors in Boston today however, who put my mind at ease.  They were happy with how the ultrasound looked!  Yes, they acknowledged opened channels on his back and a "rediversion" of the lesion, but this was expected as a normal response of a vascular lesion and result of our aggressive treatment.  But the biggest thing they said was that there is no decision to be made.  Nothing to do.  We have gotten him to the best place possible, and now we simply let it be.  Regardless of what surveillance imaging we do from here on, all treatments will be determined by a physical need, i.e. symptoms.  At first I balked at this, but they went on to tell me that the nature of vascular lesions and BRBNS in particular, is varied and unpredictable.  Any routine imaging is pointless as all it tells is that the lesion is essentially dormant, which you know by the absence of pain and symptoms.  They reminded me of our first hand experience of this when Jacob was 1 year old.   We got a routine MRI in mid-September, 2012 (ordered by our doctor here, prior to his diagnosis of BRBNS), to monitor the lesion.  It was unchanged from the year prior, and no problem at all.  But it was only 3 weeks later that he awoke in severe pain with the lesion distending out of his back, a "flare up" that occurred in a matter of hours.  The resulting pain and symptoms from this growth, led us to seek treatment.  They emphasized this experience to show me that even if we routinely ultrasound and MRI, it will only tell me that the lesion is quiet and not a problem, which I already know since he is not hurting.  Routine imaging (in absence of symptoms) is a waste and provides a false sense of security as this vascular condition will clot and "flare up" with no provocation or predictability.  Thus such routine surveillance is no guarantee that the lesion will stay that way, or even still look that way hours, days or weeks later. So all in all, where we go from here is to wait. Wait for a problem.  When Jacob begins having symptoms, pain, a drastic change in the appearance of what we can see on his back, or anything relating to this lesion, we will have our answer.  Time to go.  Time to treat.

I am understanding better and better that for many aspects of this disease, following and treating when pain and symptoms arise can be the main plan of care.  I have always known that it can be a fine balance between treating appropriately and over treating, and I don't want to subject him to unnecessary medical intervention, on virtue of my paranoia.  I have never wanted fear to rule all the decisions we make for him.  I agonized over how I will find that balance and make the right decisions for Jacob.  This new plan, even though there is such a huge unknown element to it, gives me relief.  I will know when it is a problem.  I don't have to decide if anesthesia risks are worth their benefit when no treatment is necessary.  As our Boston doctor said, "This lesion will do whatever it is going to do, whether we looked at it constantly or not.  And if he is doing well and is pain free, it doesn't need treatment, so why look."  He could go weeks, months, even years before this thing rears its head again.  We know it will, because it is the nature of these vascular lesions.  But we will know when it does because he will be symptomatic.  The only reason that we treated this past year (in absence of symptoms) was to get it under control and to a safe place where we could let it be.  So here we are.     

This watch and wait, unknown aspect of his condition however does present a few logistical challenges.  Likely, the next time we travel to Boston, it won't be like the last 3 times.  We won't have months to plan, save, and make arrangements.  He will be symptomatic and need treatment quickly.  We are plugged in with Miracle Flights, so getting our plane travel arranged quickly should not be a problem.  But staying at the Yawkey Family Inn up there is really hard to do last minute.  It is usually booked up, and unavailable for last minute stays.  We will likely have to arrange lodging on our own, which we are trying to save for.  In addition, I will always have to keep available 2-3 weeks of paid leave at work just in case, and David will have to use his sick days judiciously as well, so we are always ready.  We are fortunate enough to have such a supportive family, specifically both sets of our parents, always at the ready to help with the other kids if needed.

But the biggest challenge for me is not these.  Its the personal one.  The fact that I am waiting helplessly, hoping my son stays well.  But I am reminding myself, that this is always where we are.  I have no control over his health the same as I don't have control over my other 3 children's health, or my own for that matter.  I have such arrogance sometimes, albeit unintentional.  I think I can make the right decisions, and advocate enough, and be vigilant enough to keep my son well.   I want so badly to spare him any and all hurt that I can, and I carry such a burden of responsibility (whether well placed or not) to make the right decisions to keep him healthy.  I know I need to be reminded that I do not hold his life in my hands.  As I have said before, it is a daily challenge to trust God with my son's life, and not be consumed by fear and worry.  It is amazing to me when I think about it, as those of you who know me well, know how much I can value control.  I want to know right now, have a plan, be doing something, leading the charge, holding the reins.  What I have always viewed as a strength of my personality, will not serve me well in this.  How ironic life can be.  For Jacob to have something that requires me to watch, wait and see, do nothing for a season, is in stark contrast to every instinct in me. Please pray for me that I will find peace in the waiting.

My feelings aside though, I have stated all of these small vague complaints that Jacob has had the past few months that cause me worry.  But don't read this and think he is doing poorly.  He is actually doing great!  He is doing well at school, so well that we have increased him to 2 days a week, since he was been growing and learning, and enjoying it so much.  At home, he is active as ever, keeping me on my toes, and talking a mile a minute.  He is so full of personality, and is hilarious to talk to.

He is a perfectly normal 3 1/2 year old to those outside looking in.  It's just for those who know him best, we see these small changes and pains.  And for me, I analyze each one and agonize over it.  I want only the best for him, and though all I learn tells me that pain can be a fact of life with this condition, that is a fact I am loathe to accept.  Please pray for David and I to have wisdom in decision making, that we will act in prudence and not fear.  Pray we will keep our eyes open to potential problems, but not to see them where they are not.  Pray for faith to "let him be" when we should.  I know who made my Jacob, and want to entrust his care to Him.

Also one last request.  I don't want to be consumed with the waiting.  In fact, I don't want to wait at all.  I just want us to live.  Live our lives as if there is no shadow of "what if" or "when" looming over.  Because for all I know, maybe there isn't.  I don't want to pray for Today's Miracle, but live in the shadow of fear of not receiving it.  What good is that miracle of Jacob's health if we don't enjoy it?  I want us to live and not exist waiting for a problem.  Pray for us to keep living in faith of good health for Jake and happiness.  I will continue to update as I can on our family and hope to have nothing to report but full, busy lives.  Thank you for continuing to follow our journey and pray for our family.  We have truly enjoyed a season full of "Today's Miracles."  May it continue to be so.