Tis the Season

I apologize that it has been so long since my last update, but we have been very busy in the Casteel household!  It is a good thing, I suppose, that I have had little time to reflect and articulate any of our goings on or my feelings of late, but I feel it is time.  I'm not sure if any are still following, but from the beginning, this blog has been a sort of therapy for me, so I will forge ahead.  

 We have had a great year so far in 2015, albeit an insanely busy one.  I am nearing the end of my schooling, having just finished the bulk of the gateway program to my BSN, but still have 3 prerequisites to go, which I will finish in the spring.  I am still working night shift, which is exhausting, but I am enjoying it and making the most of the new opportunities for growth and building of new relationships with my "dark side" friends.  David has had a successful year thus far in the classroom, and on the field; but his reffing year has come to an early end as he needs another knee surgery which will happen in December. This was disappointing for him, but there is no avoiding it.  Hopefully it will get his knee back in good shape for the officiating seasons next year.  My big kids are good, with Asa excelling in the fourth grade.  He is being placed in the gifted program, and was voted vice president of the fourth grade.  He is starting basketball back this month and is very excited.  He has been working hard and we are very proud of him. 

 Elena is doing great in the third grade, and made the star roll (all A's) for the first time this year.  She is in ballet, and will be performing in the Nutcracker in a couple weeks which we are very excited about.  She will also be playing basketball for the first time, and is taking piano lessons from Nana as well.  I am so proud of my 2 biggest and all of their hard work. 

  

 

Jacob is doing great going 2 days to Mother's Day Out.  He loves it and is really making some strides.  He is so much fun these days, and there is never a dull moment with him around.  He has so much personality, and is hilarious; and thinks himself very grown up to be four.  He adores his siblings, and would rather be with them than anyone.  

 

He is still a very busy little boy and keeps me on my toes; but look out, his brother Luke is giving him a run for his money! Luke is now 2, and is the most high energy little guy I have been around.  He is a tornado of activity, and the combo of him and Jake requires my constant attention to prevent them from tearing the house apart!  They are a lot of fun though, and I am loving their close relationship.  Luke's little personality is out in full force, and he is talking so much, it is fun to watch him grow.  

  

All in all our little family is doing great. We are looking for direction on the home front, as we are feeling led to move (still staying in Chattanooga).  We'd like to get some land for the kids to have more space to play, so are considering school issues and the like in making the decision.  Pray we will know what to do, and that we'll be able to sell our house, and find the right place when we are ready. 

Now for a Jacob update...Many of you may have seen requests for prayer and small updates last month on facebook about him.  Here is a synopsis of what happened.  Jacob began complaining of  a headache in mid-October.  His headache persisted until he began having nausea and vomiting.  The next day his headache got worse until he was literally screaming and crying in pain.  He is a tough little guy so needless to say, I got really concerned.  I took him to the doctor who was further concerned as he didn't seem to have a virus or anything (also unlikely as he had shared his sippy cup with Luke the morning he got sick, and Luke was still fine).  Since his illness presented with a headache, his doctor felt we needed to go to the hospital for a CT to make sure there wasn't something we were missing, especially in light of his condition and its potential.  On the way to the ER, I called our team at Boston Children's Hospital, hoping that they would tell me we really did not need to worry about that.  They did not, though.  They said that we couldn't discount his condition as a possible source of the problem, but really needed a MRI rather than a CT to ensure we would see any potential vascular issues.  They also said that since we were putting him under, we needed to MRI his chest and spine as well, to see that lesion, as growth into the spine could cause a blockage of Cerebrospinal Fluid flow, which would give him a severe headache as well as nausea and vomiting.  So we moved forward.  I will say, that it was our first ER visit since his diagnosis, and it was my first experience having to explain his condition to every physician and nurse (as none had heard of it understandably).  They tried to prepare us for this in Boston, but it is still such an uncomfortable feeling being the authority on his condition in the hospital, and telling them what tests to order, and reviewing the results as well.  I have never been more thankful for my medical background which enables me to communicate on their level, but it would still be nice to simply be the parent and let the doctors do their thing.  They were great in asking questions and learning about this condition they had never heard of, and allowing, no encouraging me to make the treatment decisions.  It was scary though, and never was the burden of responsibility for his care heavier.  

He underwent a MRI of the head, chest, and thoracic spine, which takes much longer than normal, due to the large area being scanned.  He was under for about 4 hours after which we went back to the ER to wait for results.  He did great with the anesthesia, despite the usual difficulty in watching him wake up.  Once the disorientation and anger wore off, he actually felt better than before, with some of his symptoms easing.  We settled in for the wait for results.  The MRI of his head came back with a blow- he has a vascular lesion in his brain, specifically between the cerebellum (brain stem) and the larger portion (grey/white matter) of his brain.  The Chattanooga radiologist or the physician team did not know what to make of it, other than relief to know it wasn't bleeding and didn't seem to be causing any issues right now.  We would refer to our Boston team for further interpretation.  The MRI of his chest and t-spine came back with no read from the radiologist.  We were ready to go, so they allowed me to look at it, as the ER physicians again did not know what to make of it.  Having pored over all of his scans both alone and with the Boston team, I felt comfortable looking at it and making a decision about whether we could go home.  I was thrilled with what I saw.  No lesion showed in his chest that I could see, just some ragged tissue which I presumed to be treatment scarring or the like. The vascular lesions "light up"  with contrast, so I was very confident about seeing none.  There was a good deal on his back, (which we were not surprised as we can feel it), but none near the spine, so I said we're ready to go and will follow up with Boston.  We took our scans and left, and Jacob got better in the following couple of days.

I stayed in constant contact with Boston in the weeks to follow, as they received and reviewed the MRI's.  The process took longer than normal as they brought in a Neuro-Interventional Radiologist (who we have never had to see before), to consult on the findings in his brain.  The news was good as I expected on the chest/back scans.  NO regrowth or rerouting of lesion in the chest at all!  Residual scarring from our treatments in 2014 as to be expected, but absolutely no visible lesion.  This was awesome news, as the lesion is completely involuted after our aggressive treatments.  They said it will likely regrow in the future as such is the nature of his condition, but we potentially could have bought ourselves years of respite from this lesion.  They said they could not have hoped for so successful of results from the treatments, especially considering the large size and difficult location of this lesion.   They did see new growth of the lesion on his back and even new lesion on the other side of his back, but it is still clear of the spine.  They asked if it hurt him or bothered him, or was disfiguring, all of which I answered, no.  I elected not to have this treated right now since it is not hurting him, and they said to let them know if it did and we could arrange treatment.  We are thrilled at the prospect of him not needing treatment for the foreseeable near future, though we know that that could change at any time.  We'll take what we can get though, so this was great news. 

As far as the lesion in his brain goes, it is a bad news-good news-bad news situation.  The bad news (in addition to there being a brain lesion at all), is that the lesion is large, 3.5cm X 1cm X 1cm.  That is pretty big, especially when you consider it is in the brain of a 4 year old.  The good news is that they have never had to treat a brain lesion in a BRBNS patient before.  The other bad news is that that really doesn't mean anything considering there are only a handful of the 150 or so patients with BRBNS, that have brain lesions.  So the sample size is too small to say with any statistical probability that Jacob's will not be a problem or need to be treated.  When you are talking about a handful of people in the world, anything is possible.  They are hopeful that his will follow the trend and never cause problems or need treatment, but cannot tell us that for sure.  It is too large to assume it will be fine, so they want to look at it again in a year to determine a baseline.  Is it growing, changing, etc.  So basically, it is there; we have no idea if it will be a problem, and like everything else, there will likely be no warning or predicting if or when it will be.  It is one more thing (and a huge thing at that) looming over us, that we have to worry about.  One more potential for harm to our Jake.  We desperately ask that you keep Jacob in your prayers that this will never be an issue.  

I read my last post this morning prior to writing this, and really needed to hear it.  My words about not living in fear, or allowing this condition to cloud our days, have never been more relevant than now.  11 months ago when I wrote that post, I had no idea that the ante would be upped even more.  But it has.  The consequences of issues from this brain lesion could have catastrophic effects on our Jake.  But just like the others, we are choosing to hope and believe that he will stay well.  We are praying daily for Today's Miracle, and are ever thankful for it.  Please pray not only for his health, but for peace for our hearts.  We aren't waiting for something bad to happen.  We are just living.  BRBNS is not winning in our home.  We are.

 

 

Happy Holidays to all of you from the Casteel's!

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.

Christmas Update...a little late

Merry Christmas everyone!  I know I'm a little late, but since I neglected my blog the past few months, I'm trying to bring it back up to date.  And since I didn't get Christmas cards out (again), here is our Christmas highlights...I had a glorious stretch off work for Christmas this year (which I will pay for next year), so we had my brother and sister-in-law and their family stay with us for several days, as well as celebrated Christmas with my whole side of the family first in the days preceding Christmas.

It was total chaos, with about 25 people there, so I didn't get many pictures taken, but we had a great time. After several days spent with my family, we had Christmas day at home by ourselves, a rare treat.  Here we are reading the Night before Christmas on Christmas Eve...

 And setting out our milk and cookies...

Elena was so excited to see what filled her stocking in the morning.  They went to bed well, but Asa and Elena woke up at 1am.  We told them to go back to bed.  They tried again at 3:30, and again at 4:30.  But I was not about to wake up the little boys at that hour, a surefire way to ruin their Christmas.  So when they were up at 6am, we agreed it was time!

After a quick weekend of work for me, and playing with the new toys and games for David and the kids, we were off to David's parents to have Christmas with them. Again, with the chaos, I didn't take pictures of the gift extravaganza, but I took some over the week of the kids playing outside.  It was cold, but didn't keep them from spending most of their days outside as usual...

 

We had a great Christmas, I must say, I feel this way every year, but it was the best one yet!  I am so thankful for my family and for all of you who continue to follow and pray for continued miracles for Jacob, and for our whole family.  Love to all.