The journey up north....

As many of you know, Brody fell ill earlier this year and spent a month at Emanuel Children's Hospital. He was diagnosed with EBV induced HLH, a rare blood disorder affecting only 1 in 1.2 million children per year. Very rare. Very scary.

Hemophagocytic Lymphohistiocytosis (HLH) is an infrequent complication of EBV infection and if left untreated, leads to multiorgan system failure and death usually within two months of onset. However, early recognition and therapy results in successful treatment in most cases. Beginning in February of this year, Brody received the 9 week protocol treatment plan consisting of etoposide chemotherapy and dexamethasone steroids. These medications, although life saving, are toxic chemicals pumped in to the body to suppress the immune system and shut down the unregulated activation of lymphohistiocytic cells. Scary stuff, big words, right?!?

Being immune compromised leaves individuals at a far greater risk for infection. In Brody's case, everything that could go wrong, did. The PICC line put in to pump all the toxic medications into his body not only became infected, but also developed blood clots. This little adventure landed Brody in the PICU and on a six month regiment of twice daily blood thinning injections (only 9 weeks to go!!). However, once this secondary infection was cleared up, Brody finished off the 9 week protocol and went in to remission. That lasted about 4 or 5 weeks......

Although Brody's immune system began functioning properly again, his EBV remained active in his system. They aren't sure why Brody's body cannot handle the EBV, there are several theories. It could be an underlying immunological issue that we've been unable to detect or it could be chronic EBV, we continue to search for an answer. At any rate, around Mother's Day Brody began to show signs of illness. For the first week we thought he had a viral infection. However, into the second week, and as he got sicker, I started to worry it was something far worse and I began to hound the doctors nonstop for answers. For the first couple of weeks they ignored me but eventually with much persistence and pushing they started to nose around a bit and do some testing. He was hospitalized twice in May, once for low sodium levels and the second time to have a colonoscopy to see why he'd been having such severe GI issues. The colonoscopy found an ulceration in his colon. Ulcerations are common for EBV. They can be in other organs, the mouth, the skin, etc. In Brody's case, the elevated EBV once again triggered his immune system to overreact and reactive his disease. Unfortunately, Brody had already been quite sick for 4 weeks leading up the colonoscopy. When the results came in on Friday (June 4th) our Emanuel docs continued to put us off and seemed reluctant to answer our questions or start treatment again. In fact, they didnt want us to come in and consult on the pathology results from the colonoscopy until Wednesday, June 9th. After 4 long weeks of Brody steadily deteriorating we were all exhausted, waiting another five days was not an option. We'd also lost confidence in the ability of our Emanuel docs to care for Brody at the level he needed. Are they good doctors who cared for Brody? Yes. But moving forward we felt we needed to get him to a bigger center with more specialists and more resources.

So, pushy and emotional mama bear called a connection at Seattle Children's Hospital. Let's be honest, I called every single doctor I knew. These people are certainly thinking twice about giving me their cell phone numbers :). Anyway, our dear neighbor and friend, Kathleen Dunn, put us in contact with a close friend of hers, Dr. Tom Brogen. An amazing individual. He's a PICU doc at Children's and has been a steadfast support person for us through this entire ordeal. I called Tom on Friday afternoon asking him if he could help me get Brody in to Children's asap. He's a rock star. Friday was an emotional night. Its very hard to know if you're making the right decision to commit to care in a city 3+ hours away and to leave the doctors you've created a 4 month relationship with. I was a mess. Exhaustion was not helping and I was beyond worried about Brody and needed answers sooner than they were coming in.

While Tom was working on getting us in to Seattle, my best girlfriend Melissa was working on getting us contacts with doctors at University of California San Francisco. I was willing to go either direction to get Brody the best, fastest care. I was amazed at how quickly and how hard both Tom and Melissa were working on Brody's behalf. Within a fews hours Tom had it all set up for us to come up to Children's on Saturday (June 5th). By Saturday morning, Melissa had gotten us the home phone number for the HLH specialist at UCSF and she was waiting for us to call so she could weigh in on Brody's case. HOME PHONE NUMBER. How great is that? I couldnt call the clinic and get a phone conversation with this woman if I tried but here I was calling her at home over the weekend! Its not what you know, its who you know :).

Now that it was all set that we'd be leaving for Seattle on Saturday morning the weight of all that had transpired was settling in. I had gotten everything I'd just asked for and yet, I was so nervous about whether I was making the right decision. Enter Carrie Phillipi. Carrie is another dear, sweet friend of mine. Lucky for me, Carrie is also a Physician at Doernbecher and I needed her as a friend and a doctor. I must have talked to Carrie 5 times Friday night and another 5 times Saturday constantly asking for reassurance. She was so great. She told me exactly what I'd find at Children's, the differences we'd experience and so on. Carrie is an amazing friend, a wonderful mother, smart as a whip (even if she writes B papers for her 7th grade son :)) and an exceptional doctor. If your child is ever admitted to Doernbecher and Carrie is your attending, rest assured you are in great hands.

When we walked in to the emergency department at Children's on Saturday afternoon we had a team of docs waiting there for us who had spent the day researching Brody, contacting our Emanuel docs, pouring over volumes of chart notes, they were ready to care for him the minute he walked in the door. What a relief. What we'd been begging for, for weeks in Portland was all coming together in a matter of hours at Children's. They spent Saturday night getting aquatinted with Brody, making sure he was comfortable and safe. They were also devising a game plan that included getting every single diagnostic test under the sun done in as short of time as possible, consulting with Infectious Disease as well as Immunologists. We spent Sunday and Monday having CT scans, bone marrow biopsy and aspirate, lumbar puncture, ultra sound, x-rays, blood draws, stool samples... you name it, they checked it. Coupled with the tests we'd done in Portland and the labs we'd had sent out, by Tuesday, June 8th we had a game plan. One thing they knew right away, the CT scan showed sinusitis, which they are treating with antibiotics. Poor kiddo has been suffering on all ends.

Jeff had to fly home Monday night and so unfortunately had to miss the big meeting on Tuesday afternoon with "the news". I was so anxious all day waiting for the meeting. Wanting and not wanting to know. I thought I knew what was coming and I had prepared myself to hear Brody had relapsed. What I didnt expect to hear is that because of this relapse a bone marrow transplant was now inevitable. The bigger blow was the news that they had to treat this case as a familial (hereditary) condition even though genetic testing couldnt conclusively point toward a genetic defect responsible for triggering his immune system to over activate. All I could think of was the six week old baby in my arms. Had I just committed her to the same fate as Brody because we had possibly passed on a gene mutation? My heart sank. My sentences no longer made sense. I heard what the docs were saying but couldnt focus or pay attention. Was Kade also in danger? Could Jeff or I get sick??? I was trying to play it cool but inside I was reeling in fright. Everyone was contacting me wanting to hear the news but I couldnt say it to myself let alone out loud to someone else. It has taken me a good 24 hours to come more to terms with this news and to realize we just need to take all of this one day at a time. Not only do we have 9 weeks of treatment (which includes starting all over with the many doses of daily medications) but we also have a central line to protect, blood clots to watch over, a weakened immune system, and my all time favorite: roid rage! to get through. Assuming all this goes as planned, we then gear up for a bone marrow transplant, an entirely different beast that we are just beginning to learn about so please, dont ask a million questions because I'll blog here about it as we learn what to expect. As far as Kade and Kaelyn are concerned, we'll all four get genetic testing done to see if Jeff or I (or both) have any gene mutations in the known HLH genes as well as seeing if either Kaelyn or Kade have them.

Brody has one gene associated with familial HLH that has a slight mutation. We knew this the first go round but it was deemed insignificant. Essentially during the genetic recombination of genes, as the sperm meets the egg, Brody gets half of the gene from me and the other half from Jeff. When only half the gene is showing a mutation it is implied that the half that is functioning properly will be enough to make the gene function as it should. In Brody's case, we have to consider that although this gene should function as it should, it may not. The benefit of Jeff and I both getting tested is to see first, if one of us also has this gene mutation. If we dont, there is the chance that this is a spontaneous mutation and only affects Brody. If Jeff has it, it only affects Brody and Kaelyn and Kaelyn still has a 50% chance that Jeff passed on his good half of the gene. If its me, all three kids are at risk and again, both Kade and Kaelyn have a 50% chance that I passed on the functioning gene. Its also good ammo for any future fights... YOU DID THIS!! Kidding. The other part of this is that even if any one of us has the same mutation in this particular gene, it is still presumed that the functioning half of the gene will do its job. So, we breath a little easier for the time being knowing there are many possible outcomes. We wont know the answers to these questions for weeks to come. And even with answers, we still have to watch Kaelyn closely as there are perhaps many unknown genes that could trigger this disease. With it being in the family, she has a risk. At this point, this is all I know on this subject, so again, I hope we dont get bombarded with questions that we cannot answer and that only stress us out further :).

So there you have it. You all know as much as we know. Brody began his treatment yesterday (Tuesday, June 8th) and is already showing signs of improvement. Clinically he looks MUCH better than he did when we walked in here Saturday. We've learned to take this treatment one day at a time and to not look too far ahead because outcomes are unpredictable. The goal is to get him back in to remission and ready for transplant. This consists of 8 weeks of chemo while simultaneously working on finding a bone marrow match. We'll leave the hospital and go home as soon as the doctors feel he's safe enough to do so. This could be tomorrow, this could be next week.

And yes, a successful BMT will 100% cure him of this for life.

The next step... we've had a lengthy conversation with our new Seattle team and we've decided that, once out of the hospital, we'll work with Seattle Children's as well as Doernbecher Children's Hospital moving forward. It will be much easier for us if Brody receives care in Portland close to home. Doernbecher is the obvious choice as they also have a transplant program. We've yet to decide which hospital we'll use for transplant but we've been reassured by Seattle that at any time if we feel we need to be back here, throughout this entire lengthy process, they'll get us here. They will remain a part of Brody's care team and coordinate efforts with both the hemonc and transplant programs in Portland. Carrie has already alerted the head of the transplant program at Doernbecher about Brody and our hemoncs here in Seattle are getting in touch with her tomorrow to begin coordination of care. It is so nice to know we have choices and we can try and make Brody's life as normal as possible as he goes through this next year of intense medical care.

So thank you to all of you who have sent your prayers and positive thoughts our way. Who've checked in, made us meals, sent the kids gifts, given endless amount of support, medical advice, on and on. Brody has a community behind him and we couldnt do this without all of you. We will update this blog sometimes daily, maybe only weekly.. depending on where we are in the process. I hope this lengthy first post answers many questions..... Until tomorrow...

Much love to all of you,

Tracy, Jeff, Brody, Kade, & Kaelyn