Checking in and catching up...

Hi All...

Sorry for the looonnngg delay between posts, I'm finding out more people follow, and rely, on this blog than I thought. Thank you all for caring and worrying about our Brody.

Lots has happened since mid July. The better Brody began to feel, the harder it was to find time to blog about it. The last few weeks of July, as he was winding down his treatment cycle, he really snapped back to his old self. And by old self, I mean OLD SELF... pre illness! It was wonderful to see and Jeff and I have spent every minute since enjoying having him back.

Brody finished the eight week protocol at the beginning of August but Dr. Stork felt it was beneficial to keep him immune suppressed while he received another four week treatment called Rituximab. So, he received three additional doses of chemo and did two 3-day pulses of high dose steroid (that was awful, longest three days ever.. TWICE!) Rituximab is a unique therapy that works by selectively depleting CD20+ B-cells. Ebstein Barr Virus (EBV) lives in B cells. You may remember, EBV is what caused Brody's immune system to go haywire in the first place. So, before taking him off immune suppression it seemed to make the most sense to wipe out the cells where the virus lives. Okay, so it doesn't REALLY make sense to wipe out the cells responsible for fighting infection, but, nothing about immune suppressing and cancer therapy drugs make sense. Rituxan is typically used to treat lymphoma but its often used in post transplant patients who are susceptible to EBV.

He has been off all medication for the better part of September. However, because Rituxan kills B cells, Brody receives monthly IViG replacement therapy. You may remember I blogged about IgG previously. B cells are responsible for making antibodies that help our body fight viruses, so wiping them out makes you vulnerable. IViG therapy is essentially the antibodies from 1,000 healthy blood donors and its infused into Brody to replace what his own body is unable to make. Crazy, right?! IViG therapy is an awesome thing when you think about it. So go donate blood! Lots and lots of kiddos need blood to help their little bodies fight through these illnesses. It takes about 6 months for the B cells to recover, so he'll go in for monthly IViG infusions most likely through March, maybe even April, of next year. I just found out this morning that he will receive 2-4 more weeks of rituxan beginning next week to make sure the EBV is gone, therefore he'll need IViG for the 6 months following rituxan therapy.

It should come as no surprise to those of you who know me well, that I have spent hours pouring over Brody's medical records and seeking out information to educate myself about the immune system, the effects of all these heavy hitting meds on his body, as well as the EBV and HLH diagnoses. I don't have time to go into great detail today but essentially it occurred to me in mid August that I didn't agree that Brody had relapsed. When Brody was taken off immune suppression at the beginning of April, his immune system was still way out of whack. I'd rattle off all the different criteria they use to determine if he's truly in remission, or downward trending toward remission, but many of you wouldn't know what the heck I was talking about! Trust me, its taken me months to learn all of this stuff. At the time, I was told he was in remission but hindsight being 20/20 and all, Brody was trending toward remission but was not there. It would have been in his best interest, looking back, if we had continued therapy and added rituxan at that time. I'm not sure why that group of docs made the decisions they made but I had repeatedly told them the OHSU and Cincinnati docs suggested a drug called Rituximab, but they just continued to dismiss the suggestion. When you go back and look at how active the EBV was in Brody's body, at the time of taking him off immune suppression, it was no wonder he was sick again in a matter of weeks. I guess thats why they call it "practicing" medicine. The only thing I fault the Emanuel docs for, is not contacting the doctors at the other major centers Jeff and I had reached out to so they could all work together on Brody's case. HLH is a very rare disorder. Its not seen nearly as often as other childhood autoimmune disorders and blood cancers. A particular doctor may only see several cases in their lifetime, so reaching out to the experts seems like a no brainer to me. That is exactly what Dr. Stork at OHSU does, that's why we love her so.

I had so much anxiety about the pending bone marrow transplant that we were speeding toward. I still didn't fully understand why Brody needed a transplant. They have never found any genetic links to his HLH and its very difficult to tell the difference between familial (hereditary) and secondary HLH in many cases. The assumption was made that because Brody relapsed it must be a genetic defect causing it. But I don't believe Brody relapsed and his OHSU docs aren't convinced he did either. Transplant is the only sure bet for survival in familial HLH. However, transplant is life threatening and I didn't want to do it unless I was SURE he needed it. At this point, I'm not convinced. After discussing with Dr. Stork we agreed that he is NOW in remission and we will continue to work to get the EBV out of his system, he needs a little help as his immune system has been suppressed since February. So now, its a delicate balance between drugs that both help and harm him. Transplant is not off the table, its just on hold. We don't even have a perfect match yet. We continue to wait on results from potential donors. So, even if we were moving toward transplant, it would not of happened yet unless Brody had deteriorated and needed it asap. He has two potential matches on the table, but the transplant doctor want to look at all viable donors to pick the best one. I should also note that my mothers instinct kept telling me something wasn't right. I didn't know what it was trying to tell me but I had to listen. So I read and read and poured over months and months of blood work and doctors notes only then to have a 5 minute discussion with Brody's doctor to find out she was already thinking what I was thinking :). Boy, she could have just told me and saved me a lot of stress!

This all leads us to our big trip next month. On October 12th we fly to Cincinnati Children's Hospital to meet with Dr. Lisa Filipovich. She heads up the transplant center and is the leading expert in HLH for all of North America. Dr. Filipovich gets the call from centers all over the US and Canada whenever a kiddo comes down with HLH. People fly from everywhere to meet with her and have her treat their little ones. It only makes sense to get Brody in front of the person who has seen the absolute most cases of this. Dr. Filipovich will likely run some more tests in her lab, Cincinnati does all the major testing for the entire country for HLH cases. It likely will take weeks to find out results, but hopefully it will rule out (or rule in--please no, please no, please no) any genetic links to his illness. As mentioned, they've already done every genetic test under the sun and they've all came back normal. But,the question remains.. why couldn't his body handle the EBV? There are several hypotheses for this. One being a genetic defect we (the medical society.. yes, I'm a junior MD as my friend Carrie says:)) aren't smart enough to identify yet. Or, it could be, as I believe, a series of events overloaded Brody's immune system and knocked it out of whack. This is completely plausible and I'll see what Dr. Filipovich has to say. She is on board with us taking Brody off immune suppression and working to rid his body of EBV and waiting to see what happens. The good news is, HLH can't sneak up on us twice and Brody's doctors feel like they could get it under control if he did relapse. But, always a but, if this happened he'd be rushed right in to transplant. All of this feels like such a giant game of chess. I'm truly relaying on the knowledge I've gained, my motherly instincts, input from Jeff (who is also really smart, btw:)) and Brody's amazing team of doctors, who stretch the entire west coast, and now, all the way to Ohio. And, of course, all the wonderful thoughts and prayers from all of you. Keep thinking positively for Brody!

Okay.. exhale. Got it all out, most of it anyway. Its a game of hurry up and wait and see. We try to live in the moment, enjoying the chaos of three kiddos. As mentioned, Brody is an active and normal two year old. Other than his weekly visits to Doernbecher, and a central line protruding from his tiny chest, you wouldn't have any idea he'd been sick. I'm pretty sure all strangers look at me like a complete nut as I'm following him around slathering him with antibacterial wash every 5 seconds. I watch other kids sucking on community toys and licking play structures at the park and I cringe! I'm officially the world's biggest germ-a-phob. And now that Kade is back in school, he's stopped at the front door and hosed down for germs before being allowed to enter! Poor guy!

Keep thinking positive thoughts for Brody and sending him strength. I know he'll have a long and fulfilled life. I just don't, at this point, which path he'll be taking to get there. Maybe he's got this thing beat and maybe he'll still need a transplant. All I know is that I love him with every fiber of my being and I'll do whatever it takes to get him through this. Jeff too.

Here are a few recent pictures of our little fighter :)