Better and Better...

"we'll sing all the songs from way back when
And we'll walk down the avenue again
and the healing has begun"

On September 25th an evil wind blew in and out of our lives that changed our family forever. "What is happening right now is life-threatening." Those were the words of the PICU doctor, who in a moment of stress and panic was fulfilling his obligation to keep us posted on the situation. Jacob needed to transition from the standard ventilator to the oscillator in order to properly feed breaths into his lungs. It wasn't going well, and for the first time since Jacob came into our lives we were confronted with the darkest of variables.

There is something that happens to you when you have to tell your child that it's OK to go. You suspend every selfish instinct, every tendency to ask something of him... Where all the medical details require presence of mind, this moment called upon a presence of soul...you peel away the layers of knowledge, beliefs and programming and you get to the core of your selves. Conversations with God talks about how the source of all of our actions can be reduced to either Love or Fear. But it's this particular moment that requires going even deeper, and tapping into the Everything, into the source of the "why are we here". Somehow you put all emotion aside, because more than any other moment in your life, THIS moment requires you to be a good parent. You need to make sure that he knows that if he needs to take this journey, that he should do it without fear. If he sees Amelia, Elaine or Corduroy, it's OK to go to them. It's an experience that you're never proud to have shared, but one that gives you indescribable strength when you come back from it together. All of this transpires in a quick moment, one that is so overwhelming and so chaotic that you barely recognize when things start to take a turn for the better...

Amidst the madness, the shuffling of 20+ people in our small hospital room and the apparent crowd of both PICU families and staff that were gathering outside the room to observe this tornado, in flew Ron from Respiratory with the tanks of Nitric Oxide that saved our son's life. This was a crazy time...a sad time...but this wasn't Jacob's time...and so began a journey that finds us in a much better, much happier place almost a month later...

Jacob was extubated last Tuesday (i.e. they took the breathing tube out). He's awake and aware. There have been smiles and belly laughs...reacquainting himself with his old pals Monkey, Puff and Elmo. He's recovering well, as are Mom and Dad (though sleep is still quite scarce given our schedule at his bedside). He is breathing mostly on his own, with some minor support from a nasal cannula and a level of oxygen that's just above room air. His respiratory rate is around 40 breaths per minute, which is one third of what it was when he was intubated three weeks ago. They removed his chest tubes on Thursday and Friday, as well as his foley catheter (no more pee bag!). Round one of chemotherapy was successful in getting us closer to containing his disease. Jacob's spleen size was described as "normal" today, and his liver has come down in size even since they first detected its shrinkage. We have a ways to go, but the Encology folks couldn't be happier with Jacob's progress in such a short time. A bunch of things need to go right, but they say we may be able to enjoy a handful of days at home before round two of chemo, which starts on November 5th.

Last week's surgery was postponed because Jacob's platelets were too low for the procedure to safely proceed. This turned out to be a
blessing, as the surgery (currently scheduled for Thursday AM) will now be a 3-in-1 procedure. In addition to the Broviac he will now also be getting a gastrostomy tube installed. This will ensure that Jacob receives maximum nutrition during the chemotherapy, which is a huge part of the recovery process. The G tube will be temporary, but it will give his body a great chance to catch up develomentally to where he should be at 22 months. They will also remove a femoral central line that was placed in his groin while he was unconscious. That will be the last of the tubes/lines/foreign objects that need to come out to restore him back to where he was when he arrived. At one point he resembled a major city highway interchange with the amount of lines going in and coming out of him. Now he's a small hugable package of Love that we can finally hold in our arms again. Ahhhhhhhhhhh...

What a blur this has all been. With the journey far from over we find ourselves at a comfortable plateau where we feel strong and ready to continue our fight onward. Each day has been a positive step forward, and each day has depended on the last for us to determine the course ahead. We feel like we've been through a med school crash course. With Jacob reaching his respiratory goals we move on to conquer his disease, and after that get back to the good ole "Terrible Twos". We're proceeding with the same cautious level of optimism that got us here, bolstered and fueled by the overwhelming power of all your thoughts and prayers. Our appreciation is beyond words. And in the absence of words there will always be a song to help get you through it all...


"Before you go to sleep, say a little prayer...
Every day, in every way, it's getting better and better.
Beautiful beautiful beautiful...
beautiful boy..."

Update from the PICU

Jacob has been more alert and aware every day since we last blogged. He's been surprisingly mellow. He's had lots of secretions (mucous) in his lungs, which the doctors say is a great sign that he is healing as he begins to breathe more on his own. When secretions build up the respiratory folks send a small tube down his larger breathing tube to suction out the mucous. The process is tough to watch, as Jacob gets so uncomfortable during suction. But when it's over he's so much more relaxed, breathing easy, and able to sleep much easier. So as much as it sucks (literally) for about 20 seconds, he gets hours of relief. This process also reduces the amount of pressure needed by the ventilator

Jacob goes into surgery in the morning. The procedure was supposed to originally happen in September, before Jacob's respiratory distress got him intubated. The procedure is very routine, and involves the installation of a device called "Broviac" in his chest. This is a small central line that allows for infusions of meds to enter his bloodstream without having to IV him every time he needs them. Because of frequent visits to clinics and hospitals, children with conditions of all kinds get Broviacs installed. When Jacob was five months old he had a smaller type of device put in called a Port o Cath, which served the exact same purpose. The difference with the Broviac is that it has two points of entry, called "lumens". The multiple lumens allow for two separate streams of meds and/or blood products (red cells, platelets) to be infused simultaneously. So if Jacob is getting an infusion of antibiotics, we don't have to pause that infusion in order to give him a transfusion or another med. This will allow for efficiency while in the hospital, and it will save us hours in the clinic when we visit as an outpatient in the future.

After the surgery is done we might be able to extubate him in the afternoon. If not we will do it on Wednesday, but at this point the ventilator is doing so little the PICU doctors want the tube out of him ASAP. Jacob is breathing 30 breaths per minute, 11 of which are supplied by the vent. The oxygen output on the vent is minimal...so basically with him doing most of the work, the surgery is the only reason he's still on the vent at all. After the breathing tube comes out, we will likely spend another day in the PICU for observation and then we go back up to the 5th floor (Pediatric Encology) for the remainder of our stay. How long will that stay be you ask? GREAT question. We will likely remain here through the next round of chemo, which is loosely scheduled for the first week of November. We say loosely because it will depend on how much his marrow is able to rebuild between now and then. If his body is strong enough to handle another round, they will proceed as scheduled. While it's lofty to think we could be home for Jacob's second birthday (December 7th), we'll just be happy to have him up and around, hospital or not. On that note, we have a bunch of physical therapy scheduled once we get back to the 5th floor. Spending weeks in bed is a developmental setback, but with his young body he'll be back on track in no time. We've been doing gentle massage to stimulate blood flow in his legs, but since we haven't been able to pick him up for weeks, he'll need some major love devoted to his back. We have every confidence the PT staff will have him up and around very soon. Right now all we care about is being able to pick him up and hug him.

Thank you all again so much for the kind words, thoughts and prayers. As much of a rollercoaster as this has been, it has been a lot easier of a ride thanks to all of you. We look forward to relaying more positive news in the weeks to come. Much Love to you all...

Brighter Days Ahead...

Jacob smiled yesterday. He's been reaching out his hand, sometimes for one of our fingers...other times we give him a thermometer or plastic syringe to play with. He kicked back and watched Barney & Blues Clues and we would try to give him his space so he didn't feel we were constantly observing him (which we were). He continues to make progress with the ventilator, and we may be able to extubate him on Sunday or Monday. We understand that we may encounter more bumps in the road, but the doctors are very optimistic.

Jacob has also responded well to the first round of chemo. His liver and spleen are noticeably smaller (based on measurements by the encologists and PICU doctors), which means we could see positive physiological developments in the weeks ahead. These would include an improvement in holding his blood counts (without needing transfusions as frequently) and his ability to retain albumin. These are big steps in controlling (and hopefully eradicating) the LCH.

Last night was tough for both Jacob and the nurses. Around 4:00am it was time to turn him in the bed (this happens every 3-4 hours to stimulate bloodflow and avoid pain of being in bed for weeks). The process isn't fun for Jacob, since he's got multiple nurses manipulating him while making sure none of the tubes and IV lines are out of place. Once he was settled and comfortable, in walked the X-Ray team for their daily chest X-Ray (an hour early). So the nurses had to undo the turning so he was flat on his back. X-Ray was done quickly, but Jacob wasn't happy. One of the few weapons he has left in his arsenal is his perfect timing of the massive poop. So just to make sure the nurses were in on his misery, he opened the floodgates right as the X-Ray people were out the door. New sheets, more turning, the saga continued. Just for kicks he spiked a fever of 102+, and the unrest continued past dawn. By 8:30 his fever broke and he was able to seep into a very deep sleep. His heart rate crept down from the 130s to between 80 & 90. Songs of note during his sleepy journey:

"Promenade" (U2)
"Buckets Of Rain" (Bob Dylan)
"Amazing Grace" (Martin Sexton)
"Dressed In White" (Jonah Smith)
"Orange Sky" (Alexi Murdoch)

Keep the songs coming via email. Thanks to everyone for the continued messages of love and support through email, snail mail and Facebook. It really helps us so much.

We Love You.

Progress...

"Be not afraid of going slowly, be afraid only of standing still."

Jacob switched to a standard ventilator on Friday. This is a huge milestone that we didn't think we would get to remotely this fast. Now that he is off the oscillator some of his swelling has gone down. He finished round one of the chemotherapy, and we've seen typical signs of side effects (low hemoglobin, fevers, low platelets) so he's been getting regular transfusions to offset this. We're unfortunately no stranger to these transfusions, as he would occasionally get them in the clinic this past year. No fever today though, so we're hopeful that those will be less frequent as the month of recovery progresses.

For a couple of days he was off the paralyzing drug (he was on this so he did not try to breathe against the ventilator), which was rough...There are heartbreaking moments when he opens his eyes, sees us and cries. This has been a brutal adjustment for us, but we need to realize that he is not feeling physical pain. We met with a doctor from pain management to discuss this. The amount of Fentanyl (sedative) he is on would sedate any of us, so we have faith that even with these occasional episodes he won't remember what's happening or feel pain. His heart rate and blood pressure have been consistently good. Sometimes when the nurses are suctioning his mouth or nose you can see his heart rate and/or blood pressure increase, which is a normal sign of agitation. He calms down pretty quickly though.

Jacob is progressing well on the ventilator...the progress isn't without some occasional steps backward though. On Sunday we had a couple of episodes where Jacob was "desatting". This means he was not getting sufficient levels of oxygen to his extremities. The oxygen saturation percentage is on his monitor (we watch this screen more often than TV), and as long as the percentage of saturation is between 90 and 100% he's doing fine. Jacob was dropping into the 80s and below during these episodes, so he needed oxygen boosts and respiratory treatments to help get him back on track. At one point the ICU doctor was "bagging" him (using a plastic pump called a "bag" to help him breathe) while they took steps to regulate his oxygen and pressure. It's beyond surreal to contemplate that the only thing keeping your son alive is this extremely mellow (but very deliberate) guy squeezing a plastic bubble. Needless to say we were happy to get past that episode. We realize that the several days of positive developments put us into a bit of a bubble, so when Jacob desatted we got completely sideswiped. And so continues the rollercoaster...

Jacob went back on the paralytic on Sunday, but will come back off it today, so we're bracing for more emotional episodes where he comes in and out of conscious sedation. If the progress continues we may be off the vent as early as next week. Let's all pray that he keeps improving. With any luck round one of chemo might yield some improvement in the liver and spleen, but as his white count is basically nil, it will be a couple of weeks before we can tell if there is any improvement. We will continue to wait and see..

We rigged the iPod to play through the remote that hangs near Jacob's head. We will likely upgrade to decent speakers, but either way if anyone wants to email songs for us to play for him, that would be very cool. Email them to reis.baron@gmail.com, or if you go nuts and send a playlist, just zip it up and send thru www.sendspace.com. Thanks again for all of your emails, Facebook messages, and kind gifts. Your collective Love and prayers are definitely sending Jacob in the right direction. Our gratitude is beyond words.

Much Love to You All...

"A Successful Team Beats With One Heart."

Click to Enlarge Photo


We cannot overstate the magnitude of the team that is working on Jacob. There are multitudes of doctors and support staff who are contributing to Jacob's recovery. Here's a bit of a breakdown:

PICU - The Pediatric Intensive Care Unit is a very special place for children with an array of rare, often life-threatening issues. The doctors have an unbelievable command of each situation, and the nursing staff makes a huge contribution to supporting these children's needs. Jacob's situation requires a massive amount of attention from this staff. Their main purpose is to get Jacob into a stable state so the Hematology/Ocology team can take over and address his deeper disease-related issues. It's truly amazing to watch these folks work. They really make a huge effort to remind us how much we are a part of the team as well. It seems their first priority is making sure that whether it's daily meds or a life-threatening situation, we are aware of what's happening at all times.

Hematology/Oncology - The work of this team (also known as "Hemonc") is mind-blowing. They are well-versed in bone marrow transplants, cancer cases, and the most intense rare disease cases that come into the pediatric hospital. This team is dedicated to treating Jacob's LCH. Last week there was a "tumor board" meeting about Jacob. This summit included the entire Hemonc team, as well as three of the foremost specialists in LCH treatment in North America. They came to a confident consensus about the chemo agents that have the best chance to knock out Jacob's disease. We couldn't have a more accomplished team on the planet to deal with this aspect of Jacob's health. We know that this specific group of people is giving Jacob his best shot to put this disease behind him.

Respiratory - There is a team of folks dedicated strictly to the flow of Oxygen and pressure going into Jacob's lungs. They come in every hour to notate his levels, tweak the oscillator, adjust amounts of Nitric Oxide (helps reduce pressure in the lungs) and make sure he is getting sufficient Oxygen to his extremities. Their efficiency is a large part of why we're still here fighting this fight. The goal of this team is to get Jacob to a point where he can move from the oscillator to a standard ventilator. This will put his lungs back to work part-time, as a step toward eventually waking him up to breathe on his own.

Surgery - Jacob's chest tubes were installed by a surgery team who comes in daily to monitor the amount of air and fluid coming out of Jacob's lungs. They are constantly evaluating the fluid levels, to determine the optimal time to remove the tubes. The removal procedure is apparently as standard as it gets, and will be done at his bedside (likely once Jacob switches to the standard ventilator).

Infectious Disease - Jacob has over a dozen points of entry into his body, including IV lines, chest tubes, feeding and suction tubes, and lines going into his Port O Cath (permanent IV). The Infectious Disease team is dedicated to monitoring his "fever curve" (how high the peaks of his fevers have been over the past few days) to gauge which combination of antibiotics will be best suited to his current state. With each tube and IV line being a separate point of potential infection, they carefully monitor him to guard against these infections, any of which could be bad...given his immuno-suppressive state.

Gastroenterology & Nutrition - While Jacob is sedated he has been placed on IV nutrition, known as TPN. As his condition has improved, they have been giving him a small amount of formula to stimulate the use of his GI tract, to prepare him for solid food consumption once he is awake. The Gastro team is dedicated to Jacob's nutritional health, and will be working closely with us to stimulate his diet properly once he wakes up.

This just scratches the surface of the impact this hospital has had on addressing Jacob's situation. We are overwhelmed and truly thankful that we've ended up in the right place to help Jacob heal.

Treatment Begins

Jacob has improved steadily these past few days. The oscillator settings keep going down, and he's reduced in size with the help of diaretics (i.e. it's been a busy week in the pee bag). We're hoping to transition onto a standard ventilator in the next week. We hope his lungs continue to heal.

Chemo started yesterday. Day 1 was just the Ara-C. Today was both the 2-CDA and the Ara-C. This will be the case for days 2,3,4 & 5. Day 6 will be 2-CDA only and then a month off until round two. During recovery we will expect Jacob to have high fevers and erratic blood counts...both standard side effects of the chemo. He is holding steady at 99ish for his temp so we're encouraged that the fevers haven't started in yet. If there are any positives to draw from his being unconscious, it's mostly the fact that he doesn't have to experience the nausea and pain (joints, headaches) that are also standard with this treatment. His hemoglobin and platelet levels are good today (he got a red cell transfusion last night), which puts his body in a good state to receive the chemo. Now we just sit and wait for the treatment to work.

Over the next couple of weeks the encologists will be looking for reduction in the size of Jacob's liver and spleen. Other than feeling the size difference in his belly, we will notice changes in his body chemistry that help us determine that this is happening. His hemoglobin and albumin levels should be able to hold for a longer period of time before dropping (we are used to these drops because of the increase in the size of his liver & spleen in recent months). Meanwhile the PICU team will transfuse as necessary with blood, platelets, and albumin to correct the effects of the chemo on his body. As marrow begins to rebuild his body chemistry, it will do so without the LCH cells, and in turn will improve his health, moving him further away from the problems brought on by the LCH.

Soon we will update you on this vast team of people working on Jacob. It's an incredible team, and we're blessed to have them in Jacob's life.