Rare Disease Day

A disease is defined as RARE in the United States if there are fewer than 200,000 people living with it in our country.  Our 2 year old, Jacob, has Blue Rubber Bleb Nevus Syndrome, a vascular condition that causes overgrowths of blood vessels throughout his body.  It is one of the rarest of the rare conditions in that there are less than 300 cases EVER reported in literature since this condition was identified, and fewer than 150 people living with BRBNS in the world.  We are hopeful that the Global Genes Project and the doctors at Boston Children's Hospital will one day find the cure to his condition, and until then, we will tell our story and raise awareness for his and other rare conditions.

Today is worldwide Rare Disease Day! It is not a day that garners much attention, certainly it never did by me until this year.  The essence of what makes Rare Disease Day is that by being member, you automatically experience isolation in your battle.  In our case, Jacob has one of the rarest of the rare diseases.  After Jacob's diagnosis, we felt very alone in that we knew so little, our doctors had so little information, and most (including us) had previously not even heard of his condition.  In our search for answers and care for our son, we have come across only a few on similar paths, but each has brought us hope. So has the knowledge our family and friends have sought while walking beside us.  We no longer feel alone on our journey and are fortunate to have a wonderful support system of people who love us, as well as an amazing medical team both here at home, and in Boston that understand, and are learning more about Jacob's condition.  I think this day is so important to combat the isolation so many feel in fighting their rare disease, as well as to raise awareness for so many conditions that are otherwise unknown to the most people.  As I said before, learning more about his disease, and finding others who understand it as well gives us
which is what Rare Disease Day is all about.  Hope for a cure. Or at least hope for a little understanding.  By reading his story, you have understanding of the path he walks. As he grows, I hope he continues to find understanding and compassion in those he meets.  We thank everyone who  has followed Jacob's journey, as by doing so, you are showing your support for Jake and people like him living with rare conditions. If you'd further like to support Jacob and others with rare conditions, you can change your profile picture today on facebook to this:

Also, even though we do most days, wear jeans today with the awareness of those struggling with rare diseases and the hope that the answers may lie in their genes.  I love my "rare" boy, and thank all of you for loving him too.  Keep praying for today's miracle!

Boston Roundup

We are settling in to normal life again back home. It is so good to be home and have my family all back together under one roof!  We missed our big kids so much; Jacob was so excited to see Asa & Elena! It was so cute, their little reunion.  Luke was happy to be home, as the last few days of the trip were kinda rough for him, as he is working on cutting his first tooth.  We are all thrilled to be back, and had a great trip. We are still exhausted, but hope to get caught up and back to normal soon.  Jacob is still experiencing a lot of pain at times, which they are not surprised about.  We are continuing to medicate him and keep him as comfortable as possible.  He is getting better each day, and I anxiously look forward to him being back Jake again 100%.

So I promised that I would try to give a better update on the specifics of what we learned in Boston about Jacob's condition. We were so overwhelmed with all we learned that at the time it was too much to process and be able to explain, not to mention the fact that my family needed my full attention and time.  Now that we are back home, to our old nap routine, I have a few minutes.  This is a bit lengthy, so I understand if many aren't interested, but for my medical friends and all who are just curious, read on.  We met with and spoke at length with multiple specialists, and here are some of the highlights of what we learned about Blue Rubber Bleb Nevus Syndrome.

• We can cross off our worry list brain lesions. If they do occur, they do not tend to bleed, so rarely that they do not even screen for these lesions. Though it is listed in literature as a possibility, in their experience, they have not seen this.  We are happy to lay that huge concern to rest.  
• Jacob falls into a smaller group of patients with BRBNS that have what they call a "mother lesion." This is a large, centrally located lesion outside of the GI tract.  The patients with a mother lesion are usually a little more complicated to manage simply because of the size and complexity of these lesions. Jacob's "mother lesion" is even larger than we realized, and surprised the doctors up there with its size when they began his procedure.  Jacob's consists of a large, as well as multiple small lesions on his back extending from scapula down to lower back on the left side with additional smaller lesions up and down his right side (which we did not know).  On the left side, it reaches between 3 of his ribs and expands within the space of his chest (which we knew), but additionally, attaches to and invades his pleura (which we did not know). This is a unique characteristic previously unseen by the Vascular Anomalies Team.  This also explains how he got the hemothorax after the first sclerotherapy treatment in Chattanooga, and because of this, the Boston doctors used a different approach in their sclero treatment. Unfortunately, this attachment to the pleura increases the pain Jacob will experience with flare ups of this lesion, and as well as with treatments.  The pleura is highly innervated, and these nerves when irritated, cause pain with each breath.  Good news is that Jacob will not grow another lesion somewhere else the size of this one.  Mother lesions are present at birth, and continue to grow, but a new one will not grow elsewhere to this size and extent.  It is unfortunate that Jacob's is in such a precarious place with its proximity to his vital organs and spine, but we will simply have to aggressively monitor and treat to prevent problems. Sclerotherapy is the treatment of choice, as surgical removal is not a viable option. We will continue treatment to keep this lesion small and safe for Jacob as he grows. 
• Jacob's skin lesions will continue to grow (and he will likely get more), though usually fairly slowly and should cause minimal problems other than cosmetic. If the location causes them to be painful like on hands or feet, they can be injected or removed if necessary, though may return later and require future treatment. Most patients don't report many problems with skin lesions other than on their feet.  Some of his skin lesions however, as they grow, may punch through the skin, and if on his feet could cause pain and bleeding requiring intervention.  The dermatologist did identify a couple of Jacob's skin lesions as being simple Nevi, and not BRBNS lesions, so unlikely to grow or cause problems. 
• The MRI we got in November, though necessary for his chest lesion, including the abdomen and pelvis was a waste.  We were so excited then to see no intestinal lesions, but only extremely large intestinal lesions would show up on a MRI which he is unlikely to have at this age. MRI's are just not good for diagnosing intestinal involvement; this must be done by endoscopy.  Upon examining Jacob, the GI specialist felt certain that he does have intestinal lesions, as at this age they usually mirror the skin lesions, with the GI lesions increasing with age.  Thus he expects that Jacob probably has a dozen intestinal lesions, give or take, but obviously not large enough yet to cause him to have GI bleeding. Virtually all patients with BRBNS experience GI bleeding, but with varying ages of presentation, most commonly during early childhood.  He expects Jacob to have this eventually, so we will continue to routinely check his blood counts and screen for this.  Most of the time, initial presentation is slow bleeding, not acute massive hemorrhaging. So if and when he presents with GI problems, we will get back with him to decide on management.  He will need scoping at that time, which we will return to Boston for, as they have pioneered new techniques for this for BRBNS patients.  If bleeding stays slow and manageable, they will treat conservatively with Iron replacement and blood transfusions when necessary.  Once this is insufficient and chronic anemia is severe enough, we will explore other options.  They have pioneered certain surgical techniques for removal of lesions when necessary.  However, the surgery to remove the GI lesions is huge, difficult, lengthy, and messy, and with results lasting anywhere between months to many years, depending on the patients disease progression.  It is usually impossible to remove all the lesions, and they will return as well. The good news is that with new medication therapies, they are hoping this surgery will become obsolete. More on those later... Overall, we learned a lot about the GI aspect of this condition, which is the primary problem for most patients, and feel more prepared for the possibility (or probability) of this complication.  We also we told what to do if he has other issues related to intestinal lesions here at home, what to tell our doctors here, and what to ask them to screen for in an emergency.  
• We met with the Hematology/Oncology doctor who works with the VAC team to treat BRBNS patients about the medication options.  He said the advances they have made in the past 2 years have been dramatic.  They are currently seeing great results using the drug Sirolimus (or Rapamycin), which is most commonly used for transplant patients for anti-rejection.  It is eradicating GI bleeding in many patients, which is why they hope to not need to do the intestinal surgery much anymore.  What they do not know is what happens when they take you off this medication.  They have one patient who has been off it for a month, so they are hoping to figure this out.  They recommend staying on it for 2 years, but are unsure if the risks are worth staying on it longer than that.  It is immunosuppresive and has several other side effects, though they are minimizing those by frequent checking of drug levels, and adjustment of dosage. If Jacob requires this therapy, they would work with our Hem/Oc doctor here in Chattanooga to administer and monitor as necessary for the duration of his treatment. One thing they do not know yet, is if this drug has any effect on "mother lesions."  So far they are only using it for patients with persistent, untreatable GI bleeding.  As they learn more they would like to expand the scope of treatment to other patients to see if it would help these large difficult to manage lesions. They are firm though in their approach when deciding whether to use this medication in that their treatment side effects should not be worse than the presenting problem. Currently, Jacob is doing well, healthy, and pain free (other than his recent procedures).  Thus any side effects would be worse than what he is currently experiencing from his condition, so he would not warrant treatment. We are relieved, as that is how we were leaning going into it.  It's good to know we are on the right track in our approach to his condition.  It is also exciting the prospect of medication therapy if he needs it in the future.  The doctor was so knowledgeable and easy to talk to, and he said if at any time we question how Jacob is doing and whether he would benefit from this, to call him, and we would discuss it.  So for the time being, we will keep this option in our back pocket and hope for further advances in this area.  
• We met with the primary surgeon who is the leading expert in the world on this condition (who also performed the lesion removal aspect of Jacob's surgery).  He is still actively with his team researching this condition and its complexity.  We consented to assist in this endeavor, which was easy considering our investment and potential to benefit from this.  We consented for him to take the lesion he removed, 12mls of Jacob's blood, urine sample, and swab of his cheek cells for research.  They have isolated the specific gene mutation that causes BRBNS.  Essentially, this gene controls the development of the blood vessels. This mutation causes a defect in the vessel walls.  The walls of the vessels are supposed to act like rivers, keeping the blood contained within the "river banks." In BRBNS, these walls are weak, allowing at any given time, for a vessel, or river, to weaken and expand, becoming a "lake." There, is will just continue to expand with nothing holding it in, and fill with more blood. These "lakes" are what we see as lesions, expanded blood vessels full of blood. Thankfully, the walls will continue to expand and hold the blood in, except in the GI lesions.  They don't know why these don't hold, and instead break and bleed, possibly because of the constant motility of the GI tract, and food moving through?  Other than those though, the lesions will fill and grow unless disturbed (as in the case of the lesion in Jacob's chest when it bled).  At any rate, in their research, they are trying to create a mouse model, or essentially give BRBNS to a mouse (sorry animal lovers-I want my baby cured), to expand their options for exploring new drug treatments.  It's one thing to guinea pig a child for a new drug therapy, another to try it on an animal.  They are hopeful that if they can create a mouse model, that they will learn a lot about this condition, and if not cure it, at least make living with it much easier. Also, improved drug therapy would decrease the surgical requirement of BRBNS patients, which is huge.  Anything to reduce harm and pain to my child's body I am all for!  It is amazing to me that with their registry of only 60 patients with this condition worldwide, that they are so motivated to finding answers.  I am grateful for the brilliant minds at Harvard University Medical of which our Boston Children's Hospital doctors are a part, and sincerely hope Jacob's contribution helps them find a cure. 

So overall, we learned a lot about Jacob's condition, and how we will work with the team of doctor's in Boston to keep him healthy.  It was an amazing experience.  I was so impressed by the collaboration between different disciplines that is rarely seen in medicine.  These doctors are so comfortable working together, diagnosing together, consulting with each other, communicating treatment plans,  "sharing" anesthesia, even working with another discipline during a procedure.  This is essential with a multisystem condition such as Jacob has, and it is reassuring that they won't be contradicting each other with his treatment plans.  I am also thankful for their desire to work with our doctors here in Chattanooga to ensure continuity of Jacob's care.  We have a great Children's Hospital here and I am thankful for wonderful specialists that are motivated to learn more about BRBNS and give Jacob great care here at home.

The most reassuring thing we experienced in Boston was the comfort with which they approach treatment of BRBNS. They understand this disease and are confident in treating it.  It is such a scary place to be when no one knows your child's condition.  Most of the doctors we have encountered up til now (with the exception of our diagnosing physicians) had never heard of it.  And to suddenly be where doctors specialize in the rarest conditions, and thus they become more commonplace, is such a relief.  They were quick to tell us that by far BRBNS is the rarest condition they encounter, but still their knowledge makes it easy to entrust our child to their care.  Their solidarity in treatment and ultimate goal of nothing less than a full and happy life for Jacob, and united approach to achieving that gives us confidence, and as I previously stated, most importantly, Hope.  We have hope that with the knowledge and skill they have been given, our son will be well cared for. But may I never forget that Jacob's life rests in the hands of the Great Healer, and in Him I try to place my trust.  "Today's miracle" would keep us from needing the help of these doctors, and would spare my son future health problems.  So while I am thankful we have them, I will continue to pray for today's miracle and hope for that above all else.

Thank you as always for your support and prayers. Here's some final pictures of our last day in Boston, and I will try to keep you updated with how our boy is doing.  Thank you for being part of our journey.

Ready to go home...and bringing something back...

Well, we were supposed to have gone home 2 days ago, but because of Jacob's extended post-op hospital stay, and then the weather, we are still here in Boston.  We have pretty much stayed in the Inn all weekend to let Jacob recuperate.  He is doing well, but still requiring regular doses of pain medicine.  He always wakes up hurting as well, which hopefully will get better in the coming days. Though not quite completely himself yet, he is getting there.  We were not prepared for such a difficult recovery, because last time, Jacob was in so much pain, and doing so poorly before the procedure, it wasn't so much of a difference as it is now.  Next time we will know more what to expect and plan our return travel accordingly.

Speaking of next time, Jacob's doctor wants us back in 8 weeks for his next treatment.  With the large size of his lesion and the location of it, he wants to work to aggressively shrink it to a level that is safe for Jacob and that we can maintain with (hopefully) yearly treatments to keep it small. All this will depend on its growth, which is impossible to predict, but we are happy to have a plan in place, although he said it may take several treatments initially to achieve the desired result we can then maintain. So this means we will be back mid April, and then again likely this summer.  At this point, it is exhausting to think about coming back so soon, but at least the weather will be better. We have enjoyed the snow at times, but it does make getting out and about difficult when walking with 2 small children.

Overall, it has been a pretty relaxing weekend, as it goes.  The boys have enjoyed being here at the Inn, as we have been able to keep more to their schedule, and it is much more home like.  Here are some pictures of them here throughout the week. Before surgery...

After surgery...

It has been a great trip overall.  We are still coming to grips with the idea of Boston becoming our second home, but if you needed one, what a great city!  We really have fallen in love with Boston and its people.  For such a big city, everyone is so friendly.  I expected to annoy the locals a bit with our perpetual state of slow moving and being lost.  But everyone was so nice.  We would be standing on a street corner trying to decide which way to go, and more than once someone would stop and ask us what we were looking for and help us find it.  Everyone we asked for directions did not hesitate to help us.  On the subway, I always had at least 2 people offer me their seat when I stepped on with my baby strapped to me.  Bostonians take pride in their city and appear to want visitors to appreciate it as well.  And the hospital, I cannot say enough good things about Boston Children's Hospital. What an amazing place.  It is like being at the United Nations, as there are so many people from all over the world who have brought their children there to be helped by the best in the world.  For many, it is the last chance place.  Where you go when no where else can help your child.  The thing that struck David and I the most was that you would think it would be kind of depressing there with so many severely ill kids. But there is such an air of excitement at Boston Children's Hospital.  Everyone is so happy to be there.  The doctors and nurses are so excited about helping your child.  When we voiced our concerns about Jacob's future, all the doctors were like, "We expect no less."  Their goals weren't to just help him survive, but to live the life he wants. And they are actively looking for a cure.  Imagine, they have a registry of 60 patients in the world with this disease, and they are searching for answers. They are not content with current treatment options, but are continuing to pursue better.  It doesn't matter to them how few will benefit. My son alone is enough. Their motto is "Until Every Child Is Well."  And they mean it.  And all the parents we talked with, regardless of condition, what brought them to Boston Children's Hospital was the same thing.  A chance at a better, fuller life for their child.  Healing.  Answers.  And that's what brought us here. What we are bringing home from Boston was something I didn't expect...what I saw in the other parents here...

Hope

That's what I am bringing home with me.  Hope for a bright future for my Jake.  Thank you as always for supporting us on this journey, and keep praying for today's miracle.

We're home! Sort of...

Sorry I am just now updating, the last couple days have been a little rough.  To start at the beginning, we arrived at the hospital Wednesday at 7am for Jacob's surgery.  We hung out having fun in preop for a bit...

They started the procedures around 8:30, right on time. We got updates throughout the morning as to how they were progressing.  The first part of the procedure was quick and easy, as the surgeon removed a skin lesion that bothers Jacob.  It was simple, requiring just a couple of stitches and some glue. We were excited about this as well, as the doctors wanted to use this tissue sample for research into BRBNS, which we are happy to contribute too.  They also took 12mls of blood for research, and samples of Jacob's urine and cheek cells while he was under. After that, they moved on to the sclerotherapy portion of the procedure.  They worked on him until 12:30 and then the doctor came out and talked to us.  It went well, but was very involved. Even the doctor was surprised and the size and extent of the lesion in his chest.  There are multiple lesions all up and down his back on that left side, and is very extensive through the ribcage and into the chest.  Inside his chest, the largest lesion is attached to and invades the pleura (the lining of his lungs), which explains the bleeding into his lung cavity that he experienced last time.  Because of this, the doctor put a large amount of glue into that side of the lesion to hopefully prevent it from hemorrhaging into his lung.  He then put the sclerosing agent into it to shrink it.  All in all, he injected into several different areas, focusing most of his work inside Jacob's chest, as that was the area he felt needed it most.  He did have a neuroradiologist look at his fluoroscopy imaging during the procedure to see if there was danger to his spinal cord at this time, and he was comfortable leaving that work on his back for the next time. Which by the way, will be in 8 weeks. He was pleased with how it went, but warned of a lot of pain postop as he used the maximum amount of sclerosant he could use and the because of the size of the lesions treated and the density of nerves in these areas. It will hurt with each breath Jacob takes for a while.  He also wanted to keep Jake overnight for pain control and close observation.  A little after 1 we were taken to the interventional radiology PACU. We arrived there to find Jacob still asleep.  This was a relief, as they had already roused him and he was resting comfortably, though still on oxygen.

I had told them that he usually wakes up from anesthesia very disoriented and very angry.  They said because of this, they would use a different combination of anesthetic and see how he did.  I was very happy with the result.  He slept until 2 when they tried to wake him up a bit more.  He was in quite a bit of pain, and hard to handle when he woke this time, but settled when I got in bed with him and held him. And they gave him more pain medicine.  He had a significant amount of blood in his urine, a side effect from the sclerosing agents, which had to be flushed out.  It took most of the afternoon for it to clear, requiring 2 saline boluses, some bicarb, and 2 hits of lasix for my medical friends.  They kept his catheter in until around 10 that night as well to follow it closely. I held him all afternoon in PACU, and he slept off and on.

He was transferred to a room in the post surgical floor around 7pm, but still needed oxygen on and off.  But on this unit, they are still on monitors and are still followed closely, so this was not an issue.  He was still on pretty generous maintenance iv fluids as well. He actually slept pretty well, but they gave him pain meds religiously.  Regardless though, he woke up Thursday am in a lot of pain.  He threw up everywhere and was in rough shape. He had only peed 10mls since 10pm when they took his catheter out so that was a concern as well.  He was really just pretty pitiful the first night.

They had turned off his IV fluids at 6am, but we could not get him to eat or drink all morning, so they restarted them at noon.  They did a bladder scan to see if he was making urine or not, and he was, but for some reason was just not peeing. His pain was pretty out of control at this point as well, they decided to give him a dose of IV valium to try to get him to relax, let his pain med kick in, and hopefully relax enough to pee. It made him happy, but did not succeed at getting him to go pee.  They were threatening to put his catheter back in, which we obviously wanted to avoid. It was becoming clear to us that we weren't going anywhere today. But since he was feeling good after the valium, we took the opportunity to get him up and walk to the playroom. Bless his heart, our walk was really a slow shuffle, but once we got to the playroom just down the hall from our room, he perked up even more. This is all relative of course, as he was still nothing like his normal self.  But he played at the train table, talked a bit, and looked out the window and watched it snow.

We stayed about 30-45 minutes until he couldn't stand up anymore, and I carried him back to the room. We found when we returned that he had a wet diaper!  That was a huge relief. He was exhausted after his outing, and after a dose of pain medicine, he passed out and slept for 5 hours! Upon waking, he still was hurting quite a bit, and would not eat or drink.  We settled in for another night in the hospital.  These were a challenge, as David would have to stay with Luke until his nighttime feeding (around 10) and then go back to the Inn, and return around 7am for Luke's morning feeding.  We were just hoping Luke would sleep all night like usual, and he did. I was not willing to leave Jacob, so David and Luke made the commute.  Luke really was a trooper through the whole thing, as he had less of my attention than normal as Jacob needed me most of the time. Luke is such a good baby though, and just goes with the flow. Love that little guy so much!

Jacob & I slept pretty well overnight, but he woke up in a lot of pain, as they let him sleep longer between doses last night.  It took a bit to get him comfortable, but once he was, he actually ate some breakfast and sipped a little bit of chocolate milk.  He seemed to be feeling some better.

They turned off his IV fluids and said if he could drink enough, and we felt comfortable, we could go home.  We took another outing to the playroom, which he enjoyed, and even decorated a cookie for valentine's day.  He was rocking his superhero cape, and was feeling pretty good.

After an hour though, he wore out and we went back to the room to rest.  He ate lunch well, and drank 2 boxes of apple juice. We quickly told the nurse and got our walking papers.  We left the hospital around 3 after getting his prescriptions filled, and returned to the Inn.  It was amazing after leaving the hospital and getting back to our temporary "home" how much better he was.  It was the first he has seemed at all like himself since the surgeries. He really had a shot of life.  He played for about an hour, and then laid down and we all slept for 3 hours! It was much needed rest for us all. He woke up the best he had yet and we went and made some supper in the kitchen, and he played a little more and got showered and ready for bed.  He is so happy to be out of the hospital, and though still requiring pain medicine, is really doing well.    It took quite a bit of doing as well to get our lodging secured for the weekend, but it seems the weather worked for us on that, but against us for our flights.  Because some families couldn't get in, their cancellations allowed us to stay the weekend at the Inn, but also, there were no available flights leaving Boston until Monday night. So we are resigned to spending another weekend here away from Asa & Elena.  We miss them so much, but am glad the snow down there has provided a nice distraction for them, as well as school being cancelled let them have a sleepover with their cousins. They were so excited about that.

It has been an exhausting 3 days, and we are so thankful for all the kind words, thoughts, and prayers.  Please continue to keep us in your prayers as we are anxious to get our little family back together.  Love to all of you!

2 fun outings for Jacob & Luke

Children's Hospital Boston is so amazing. They gave us free tickets to the New England Aquarium as well as to the Boston Children's Museum.  So after our appointment yesterday, and after nap time, we headed out and took the train to the Aquarium. We were going to walk the harbor but the wind off the water was so frigid, we nixed that idea pretty quickly. The boys loved the Aquarium! The penguin exhibit is right when you walk in, and they have several different kinds of penguins, more than we have in Chattanooga. It was feeding time for them as well, which Jacob was excited about.

I'm not sure what it was, but Jacob was so happy to be there! He has always liked the TN Aquarium, but he was just so excited about it yesterday! He ran from tank to tank exclaiming about all he saw. Luke enjoyed watching the fish as well. We saw some cool stuff that we don't see at ours like Lion fish, and Stone fish, and the biggest sea turtle I have ever seen!

It was a very relaxing afternoon. After that, we went in search of some Italian food in the North End. We managed to stumble into a restaurant that was probably a little fancy for our boys, which was hilarious when it was all said and done. They probably had never had anyone nurse a baby in their establishment before either!  We learned the definition of fear:

We managed to escape without breaking or spilling anything which was a miracle in itself.  We headed home for the night, and today got up and went to the Children's Museum.  It was amazing! We could have spent all day in there if the boys hadn't gotten sleepy.  They had these race tracks for golf balls where Jacob was pretty much in heaven.

He also loved the water sections and the construction zone.

Luke had a great time in the crawler area.  He discovered himself in the mirror, which was pretty much the cutest thing ever.  He stared and smiled at himself, and waved (which he just learned to do yesterday) at himself.

He was adorable. They played and played until both were exhausted, and both fell asleep in the gift shop. That's when you know your kid is whipped-falling asleep in the stroller in a gift shop! We ate a late lunch after that and went in search of the Old North Church (where Paul Revere hung the lantern to warn of the British coming). We found it, but couldn't go in cause it was closed today for some reason.  It was cool though to see a part of history.

Now we are back resting up for a big day tomorrow.  I have been so thankful to the hospital for what they have done for us. With these free tickets, as well as all of you who have helped us, we have been able to sprinkle in enough fun for this to feel like a vacation for Jacob-although that will end abruptly tomorrow.  But my hope is that if he remembers this trip, it will be the fun things we did, and not the time at the hospital.  We have to be there at 7am as Jacob's procedures are scheduled for 8:30.  So glad we are the first case of the day since he cannot eat or drink before.  I am starting to get nervous.  Last time they messed with his chest lesion, it didn't go so smoothly, and he was so sick afterwards, it makes me worry about this time.  I know he is in the best of hands, but it is just scary.  They said he would be in some pain afterwards as well; last time he was in so much pain before hand, we didn't notice much of a difference.  I know this time though we will, as he currently is feeling fine. Please pray we can keep him in as little pain as possible, and as comfortable as we can.  We are hoping they will release him afterwards tomorrow, but they won't say-it just depends on how it goes. It will be difficult though if they do want to keep him, as Luke won't be able to stay, and he needs me but I won't want to leave Jake.  Praying he will do well and there will be no complications so we can all stay together.  Please pray for us tonight and for my baby tomorrow.  Pray for David and I as well, as we are getting anxious, and really missing our big kids too. Love to all of you from the Casteels in Boston!

Fun weekend, then back to the hospital...

We had a great weekend away from the hospital with the boys. We spent Saturday morning resting up after our big day Friday, and spent a bit of time with the Miracle Flights crew. After naptime we headed out to explore the city a bit. We rode the train/subway into downtown which Jacob absolutely loved.

 He was so excited about it! We rode down to the Faneuil Hall area. There is a marketplace there so we walked around and looked a bit and ate dinner down there.

 It was a nice, relaxing evening. Sunday morning we got up and went  back downtown, this time to the Boston Common and the Boston Public Garden.  Though blanketed in snow, it was still very beautiful. I can imagine though, in springtime, it probably looks like a totally different place. (You can see how deep the snow is in this picture, as the sidewalks were cleared.)

 Now though, the pond where they do the swan boat rides is frozen. David and Jacob enjoyed it, as they ventured out on the ice (don't worry, there were many people doing this, as it is well established by the locals as safe to walk on). Jacob thought it was hilarious and had a great time. 

Jacob's favorite thing in the park was the squirrels. They (accustomed to being fed) would just come right up to you. Those of you who know him, know how much he likes giving his squirrel impression, so they were a big hit.

 We walked around the whole park, and then down Beacon street. The houses along there are amazing, and I loved it. We passed the bar from Cheers which was cool (though Jacob wasn't impressed).

 We then took the T a couple stops down and saw Trinity Church.

 It was indescribably beautiful. I really enjoyed seeing it, and felt such peace within its walls. After that, we went back to the house for the boys to nap a bit. After they woke up, we rode back down and went to a Celtics game.

 It was a lot of fun. You all know how much David and I love basketball, so it was a treat for us.  We felt like total degenerates though, when both babies fell asleep during the game.  

Once Luke woke up, he decided this outing was over, so we headed back to the house to put them to bed. It was a great weekend, but this morning, it was back to reality as we went back to the hospital for an 8am appointment with the GI specialist.  Now since Jacob does not yet have any symptoms of GI bleeding, this visit was pretty much intended to just be informative.  And it very much was.  Dr. Fox has a lot of experience treating BRBNS kids and had a lot of information for us, which we are still processing. Though he is fairly certain (as you can be without actually scoping), Jacob likely has GI lesions already, though small enough to not be causing him problems. It was good to get our questions answered and have a plan for if/when that does happen. As with our previous appointments, once we get home and I have some time to think, I will try to summarize all we have learned at Boston Children's with these amazing doctors. But for today, Just know we learned a lot, and feel confident in entrusting Jacob's future care to these specialists. 

After our appointment, we got back to the house around 11am, and went out to play in the snow! It has been out there taunting us since we got here, and Jacob finally got a chance to play in it! We had a great time, but didn't stay out too long as the boys got cold and tired. But here are a few pictures of them loving it! (most of the time)

Thank you all for continuing to follow our journey and pray for our Jake.