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.

Watching the Pee Bag

We get more excitement out of watching our son's hourly level of urine output than basically anything else we've done in the past year. See, when he puts out more than 100 milliliters in an hour, it means he's eliminating more fluid than he's taking in. That's a good thing because between the air pressure from the oscillator and the amount of fluid going into his multiple IV sites, our boy has swollen up a bit...nothing painful, but enough to make watching the pee bag a huge family activity. At one point we had a crowd around it this weekend, with grandparents cheering him on...people fist pumping every time he notched over the 100ML mark. Some people fancy Sunday Night Football. That just isn't how the Barons roll. Give us a 6-pack of OJ from the "patient food" fridge and a decent view of the pee bag, and we'll be cheering Jacob on into the "wee" hours.

Jacob has been improving these past few days. The oxygen output and pressure from the oscillator has been reduced consistently every day. It will get to the point (possibly this week) where we can get him off the oscillator and onto a standard ventilator, with hopes of working toward waking him up. We can't get ahead of ourselves though. We still have a ways to go. But every day he shows small signs of healing, which is good news. Wednesday is definitely the start of five days of Chemo. That will knock us back a few steps. Chemo brings with it a high risk of infection, likelihood of frequent, high fevers, bloodcount rollercoasters that will call for multiple blood & platelette transfusions...not fun at all. Five days on, one month off, then five more days...until we knock this thing out. We're in it to win it at this point. We don't deal well with the "take it day by day" approach. But we're settling in and doing OK given the circumstances.

We will reply to all of your wonderful emails eventually. Thank you so much for sending healing vibes to Jacob. It's definitely been working.

Much Love...

What You Can Do...

Folks:

Because you are are beautiful people we have heard from many of you that you're unsure of what you can do at a time like this...but you want to help. There are two very important organizations directly related to our current situation who would benefit greatly from your generosity:

Histiocytosis Association of America - Jacob's disease is one of many under-researched, under-funded disorders that are affecting hundreds of thousands of families around the globe. Through the generosity of hospitals and medical staff, some projects have gotten off the ground to create a world-wide network around these diseases to keep the community as informed as possible about any positive developments in treatment. The HAA has broadened this community beyond the families and physicians directly dealing with the diseases, to include folks like you who can make a difference with even the smallest of contributions. We encourage you to read more about Jacob's condition, and ones like it that are leaving families with far fewer resources than those who are facing more mainstream illnesses. Check out the HAA Web Site, or go directly to the DONATE PAGE.

Tomorrows Children's Fund - We cannot believe the lengths our hospital has gone to make sure the Baron family is able to focus entirely on Jacob during this difficult time. Living an hour away, it has been very difficult for us to make sure Melissa can be ready for her commute to work after doing the overnight shifts with Jacob in PICU. Also, with Reis doing day shifts in the PICU and then driving home, only to return early enough to hand the car off to Melissa, their schedule has been brutal. Tomorrows Children's Fund has arranged for the Barons to have an apartment 2 blocks from the hospital for the duration of Jacob's stay. They have also committed to covering an enormous amount of our basic living expenses, to the degree that Reis has chosen to leave his position at SCI Fidelity Records. It's the generosity of this fund that is allowing the Barons to focus entirely on Jacob, and in turn bring far less stress into the hospital room with them. This organization completely gets it...that everything they are doing and paying for results in a more comfortable state for the family, and ultimately the child. You can find out more information at the Tomorrows Children's Fund Web Site. You can also go directly to the DONATE PAGE.

This list will surely grow as we continue on this journey, but right now these are great ways for you to show your support in this difficult time.

We Love You

Saturday Update...

OK...so here's what's happening (medical terms and procedures mentioned below...a warning for the squeamish).

Jacob's disease has progressed into his lungs, liver & spleen to a point where it is risky to have him breathe on his own right now. Before he was placed on the ventilator his respiratory rate was almost triple what the doctors would consider normal for someone his age and size. There is only so much time that can pass with him breathing that fast before it has a damaging effect on his lungs. We cannot risk that, so as bizarre as it sounds it is best for him to be on the ventilator right now. His lungs are getting a level of rest that we could not achieve with him breathing on his own. There are three main things that we have to address, the most harmful of which can be reversed by LCH treatment:

1> Because his liver and spleen are enlarged, his diaphragm cannot pass below a certain point, restricting his ability to take deep breaths.

2> The disease in his lungs is compromising his ability to breathe normally.

3> By Friday fluid had built up around and in his lungs, creating an extra level of pressure that contributed to his fast respiratory rate.

The doctors have drained the fluid in and around his lungs, which has reduced the pressure more significantly than we expected. He is now in a stable state, where we have to allow time for his body to heal and for his levels to come up over the next few days. It is ironic that our main goal is to get him well enough so we can make him sicker via chemotheraphy to attack the disease head on...

We need to treat the LCH with several rounds of chemotherapy. On Friday there was a summit between our Hematology/Oncology (also known as "Hemonc") team and the top three LCH specialists in North America. LCH affects around 1000 kids in the US every year, so we do not have the benefit of large studies to determine the best course of treatment for Jacob. However, there are trends in treatment results that these doctors are able to look at in order to determine the best guess. In Jacob's case we are going to administer two Chemo agents, "2-CDA" (Cladribine) and "Ara-C". We recently read about a girl in California who benefitted from these medications. We can only hope that Jacob's reaction will be the same.

We realize that the Chemo increases jacob's risk for infection, and in his unconscious state there are always chances for issues to arise. But we know that without dealing with the disease there is no way out of this. The length of the rounds of chemo treatments will be 21 days (five days on, 16 days off). We may not see results until the second or third round of these upcoming treatments, so we are in this for a potentially very long haul...

Soon we will explain more about this incredible team contributing to Jacob's improvement. You will simply not believe the level of care that we are so honored to receive here. We are amazed at the level of energy and sophistication that is channeling into Jacob.

We are overwhelmed by the amount of loving communication coming from all of you, and we so appreciate your prayers, gifts, and messages of support.

We love you all...more to come...