Home for the Holidays and So Very Thankful

There was a time, the weekend before Thanksgiving, when I was starting to lose hope. When another fever sent us back to the hospital and his counts were still stagnant and extremely low even two months after his bone marrow transplant and a week after his boost of additional stem cells. That Sunday after our second weekend at home together when we lacked any real progress, he had a fever, the anti viral medication that was making him very sick and refusing food for nearly a month, oh and Jay's car also was hemorrhaging oil that same day (which by comparison was really inconsequential but suffice it to say we decided to buy a new car instead of deal with possible future issues). It was remarkably hard not to sink into a hole of sadness and frustration. My mind went to fears of, what's next? Will we be home for Christmas? Will he need another, even more intense, transplant? Those questions swimming through my head and despite my remarkable ability to keep level headed among even the worst of news, it's hard not to go there, and really it's healthy to go there a little bit and acknowledge reality and consider options even just to know that there are some options. But I should know by now from a lifetime of experience that, just when something seems to be too much and too overwhelming, that's when things change and life brings either redemption or a new challenge. Thankfully, in this case, things changed for the better. Remarkably incredibly better. And a week after that overwhelming day, we were all living at home again after five months apart. Less than two weeks after that day when I thought it might never end, he received his last transfusion of blood an platelets yet I hesitate to say his last ever although every day that is looking more and more probable. It has been 24 days since he had any sort of transfusion, that last one was transfusion number 99, I have to let that sink it because it is most definitely a wow moment for me to realize how far he has come in such a short time. His liver and spleen are completely normal and now he is making his own red cells, white cells and platelets. Not quite enough yet but his body is working hard and her stem cells are working their magic in a huge way. So to be home for Christmas was just overwhelming in the most wonderful sense. I found myself feeling emotional over every little thing, reliving what the past six months has brought us, looking to a bright future, thinking of the people that have been there... tears! Happy awesome tears!

And on that note, I want to express my gratitude to the huge number of people who helped in a multitude of ways over the past six months. Saying thank you seems so insignificant in light of how much you all have meant to us. Every card, every message, every gesture of any and every kind brightened our day in a way that words just cannot express. We have been given a tremendous number of delicious meals, gifts for the kids and for us, financial help, amazing gestures of support, words of love and compassion, cards and letters and countless other wonderful gifts. You have lifted us up in ways that I hope you never have to understand because no family should have to go through what we have gone through. My dear friends Alicia and Julie and their amazing families who took care of Jay and Camryn when they were home and who somehow always knew what we needed and gave to us without asking or needing anything in return; they are my fairy godmothers swooping in and helping out when we are most in need. Seriously, I couldn't have done it without their support. To Elena, Megan, Jen and the many others who rallied our friends and gathered food, donations or organized so many things to help us through the hard times when Nate and I were in the hospital. To Jay's Co-Workers who have been surrounded Jay with incredible support and encouragement and furnished the kids with many happy surprises along the way. To Erin and Jennie who have gotten me through some really dark days with your open ears and tremendous compassion... you ladies always have the right words when I needed them most. To Barb and Lydia at Make A Wish, you have brought fun and hope and a great future vision to our lives which is making this next phase feel even more exciting.... and you don't even mind that the kids are often not on their best behavior. To the amazing staff at the Ronald McDonald House who gave us a much needed home for five months. To the selfless blood donors, without whom, Nathan most definitely would not be here. To every person who sent a meal, a card, a gift, a word of support. And of course to the incredible nurses and doctors and to our families. Thank you! I don't think I can ever convey the depths of our gratitude but it will remain in my heart forever and I will absolutely pay it forward.

Being home for the holidays has been wonderful and we had so many "pinch me" moments because it simply felt that good to be here now. It's strange how quickly it all feels normal again. And now the new year is upon us. New Year's Eve is Nathan's 100 days post transplant and we will celebrate that day with a couple very close friends and a cake from Icing Smiles. I am so excited to turn the page and look forward to an amazing future. I keep saying that 2015 is going to be an epic year of fun for our family. We have a lot of making up to do and we are going to enjoy every moment.

He's Not THAT Sick

Now that we are home, I can't help but look at this little boy with so much energy and his huge smile and think... Wow, he's not that sick anymore.

Never mind the bald head or the bag of fluids that he carries 24/7. Or the fact that he just had blood and platelet transfusions before the weekend and is having a remarkably slow (yet also equally miraculous) recovery post transplant. If I can overlook all of that, and really look at him, with his huge smile and abundant energy, that's the sweet spot. That's where I need to focus. But still, it reminds me of many other times when I've had the same thought.

A couple of weeks after Nathan first entered the hospital, a friend suggested that I sign him up for Make A Wish. I initially shrugged it off and said... No, he's not THAT sick. 

Thankfully, I decided that it wouldn't hurt to apply and my little guy has decided that he wants a relaxing vacation in Hawaii where he can see dolphins and whales when he's all better (sidebar, mommy is very happy with that choice). And the amazing people at Make A Wish are already working hard to help make his wish come true when the time is right. Yes, apparently he is that sick. 

Just the other day, we strung on his latest batch of beads. We love the Beads of Courage program. It is just an amazing way to turn a serious medical situation into a story that you can see and measure and process in a very tangible way. His beads are now long enough to be part of our Christmas tree garland. 99 red beads for blood transfusions, 20 yellow beads for each week in the hospital, two bone marrow transplant beads, too many of those sobering white chemotherapy beads and many many other beads each signifying each and every step in his journey. A few hundred beads measuring well over 10 feet in length. More than any person should have to endure in a lifetime, much less the nearly six month journey of a child who is only five years old. But still, I look at him and compare the person to the "story" and can't help but think... He's not THAT sick. 

His beads reach all the way up to the second floor

I often wonder if I am an optimist, a realist or if I'm just in denial. I think it may actually be a little bit of both. It's a thought I've had many times because his physical appearance and behavior has never matched the clinical picture, not by a long shot.

Hearing the stories of other families, seeing with my own eyes the fragility of life and how quickly things can change or learning the heartache of another child who has earned their wings at far too young an age... He's definitely not that sick.

Looking at that smile and that boundless energy despite hemoglobin levels that would make most adults curl up into a ball in bed all day.... He's really not that sick. 

Then I count those beads, and retell the tremendous story of the past five and a half months of his life. Yeah, maybe he is that sick... but also he's not. 

The good news is that he's on the road to recovery. No, he's not even close to being all better and progress continues at a snail's pace. I think there is a false sense of his progress in the fact that we are home as I get lots of calls and messages from people who are excited that he's better. I shrug, I get it, but if they only knew the story of this boy as deeply as I do. Yes, some things are really really better. We are home, we are together, he can run and jump and play and pretend that life is as it was before. But still, he will never be that carefree little boy that he was on the evening of June 18th and he has a long way to go before he can truly live life again. A life where he's not bound by clinic visits, medication schedules and huge bags of fluid tethered to his picc line. A life where I don't run for cover and slather him in antibacterial spray any time another person so much as sniffles. A life where he can really plan for that amazing wish trip or even go out in public without me having a mini panic attack in the process. But I can see it in our future, maybe just beyond our grasp, and I cannot wait for more of those small milestones that tell me it's coming. The day when I'm no longer comparing the child to the clinical data and reconciling the vast gulf between the two. When I not only know in my heart that he's not that sick, but that he is all better and even better than ever. Because you cannot go through what he has gone through without coming out of it stronger, more joyful and more acutely aware of the beauty that lies in each and every moment on this amazing earth.

Back in the Hospital... Again

The inevitable happened... Nate had a fever that sent us back to the hospital. BMT patients cannot mess around if there are any signs of a virus or infection, especially in those precious early months just post transplant. I figured this was a "cross it off the list" sort of moment because, as much as I didn't want him to be re-admitted, I knew the possibility was there. It seemed that there were a variety of complicating factors that lead to his fever. First off, he started a new anti viral medicine (Foscarnet) last week resulted in some unexpected side effects... complete loss of appetite, vomiting, generally feeling yucky. Add to that a small case of sinusitis and an overgrowth of mucous in his airway and here we are. He had several blood cultures taken and a CT scan and thankfully there is nothing more that is causing his issues, no fungus in his system or bacteria in his blood or unexpected viruses and only a small spot of concern on his lungs that is really not much of a concern at all. He did start two new antibiotics to help clear the infection from his body and he is doing well today. Still vomiting or spitting up mucous (often times with some blood) and some random low grade fevers but very well all things considered. I really dislike this part of his treatment, the having to treat the effects of the treatment. But it's reality and I'm not going to sugar coat it. He is back on IV nutrition (TPN) for the time being because he is not eating. This should resolve once the Foscarnet is gone in a couple of weeks, sooner if he begins to eat again. And of course the IV nutrition adds additional stress to his liver and kidneys, and his kidneys are already being closely monitored due to being on two nephrotoxic medications simultaneously. But, like I said, this is his life right now. Tear him down then we can built him back up later. He has a very rare form of an already rare disease (if aplastic anemia is 1 in a million then auto immune  hepatitis associated aplastic anemia is maybe 1 in 20 million, with only a handful of cases each year) and just to be close to a team of doctors who truly understand it and know how to treat him is hugely reassuring. This is what needs to happen for him to heal, it's unfortunate but very true so to resent the disease or it's treatment is pure futility. I focus on healing instead, being thankful for the antiviral med because, even thought it is making him feel very sick, it also got rid of a virus that had potential to be far more harmful. Being thankful for having access to IV nutrition because he needs some nutrition to heal, even if it is suboptimal and further stresses his body. It's hard to not hate the disease but truly I do not because it serves no purpose. To put energy into hate is still energy and I have none to spare on negativity even if it's very justified. Instead I choose to count our many many blessings and put my energy into love. As with all things in life, this is temporary.

So now we wait... make sure the HHV6 virus is completely gone so we can reduce his anti viral dose... make sure his mucous and sinusitis is improving... and most importantly, get those cells ready to give him a little boost. We should be discharged Monday or Tuesday. Have I mentioned how much I dislike waiting? Ah well, I guess I need to reframe that too, as I am also thankful that we have the luxury of time and that he still remains remarkably healthy despite everything.

While we sit here and do all this waiting, I am spending my time reflecting on what our family has been through, continuing to count our blessings, making future plans and gathering ideas to make 2015 an epic fun year for our family because we all deserve it.

BMT +45

We had thought Nathan would have some real counts by now and hopefully be transfusion independent but it seems that things aren't always quite so simple. After going off the white cell stimulant (G-CFS), his counts plummeted. Yesterday his white count was down to .6 and he still had no red cell or platelet production to speak of. He is still getting platelet transfusions three times a week. He is also getting smaller doses of red blood weekly as they are keeping him quite anemic in hopes that'll send the signal to his body to start producing red cells. And they are giving him that white cell stimulant every so often just to help give his marrow a little bump if/when needed. I keep wondering why things continue to be so unpredictable and it seems there is no concrete answer. The most likely scenario is that his enlarged spleen and liver, the same organs that were chewing up platelets at a rapid pace, also consumed a large portion of his sister's bone marrow. This means that the cells that are left would have to work much harder to repopulate and produce blood cells. The great news is that the cells are there, our official chimerism results are great and show that 95%+ of his white blood cells are being made by her marrow and he is showing no signs of rejecting these cells either. The cells are there, they will grow, but it's just taking a really unusually freakishly long time. Unfortunately, he now has a new virus that we are treating very aggressively. It is the Roseola virus which, as a member of the opportunistic herpes family of viruses, can remain dormant in your body and then reactivate anytime. We never knew he ever had Roseola but I have been told that the vast majority of humans have this virus in their systems even if a large number have never even shown symptoms; and we are at least fortunate that he is still appearing healthy and symptom free even now. But appearing healthy is not enough for a child who recently had a bone marrow transplant as these seemingly benign little viruses can cause some serious complications. So he is being treated with a new medication to suppress the virus and, once the virus is gone, they will rush to give him some of the extra marrow that had been frozen for him. Hopefully waiting for the virus to be gone is a very short delay in the grand scheme of things and these cells should give him the boost he needs to help his marrow to really recover. It feels so reassuring to have this solution waiting for us. Knowing that he doesn't need to be re-conditioned before receiving the cells since his marrow is now essentially made up of her cells, and that he is no longer rapidly consuming platelets so the risk of the marrow cells also getting consumed like they were in the past is very slim. I think I might be a complete mess if it weren't for this. That his doctor took the step to keep these cells "just in case" is a beautiful thing since needing this sort of boost is a very rare thing. Of course, in the back of my mind I also worry that maybe it won't be so simple. But worrying will never solve anything and I have to keep positive for his sake and for my own because all signs point to his full and eventual recovery. It just may take even longer than we had hoped. And I guess that's OK; it's just a monumental task for my (lack of) patience.

Discharged and Engrafted!

Yesterday, Nathan was discharged from the hospital. It also happens to be the day that they officially consider him to be engrafted by all possible definitions. We already knew that his white cells were 98.4% engrafted but now we also have had three straight days where his neutrophil count was above 500. It was very reassuring when we woke yesterday morning and he had the biggest jump in his white blood count thus far, it went from 1.1 to 1.4 and today it jumped again to 1.9. This is real progress. He has been taken off his white cell growth stimulant (G-CSF) for now but will still get it intermittently or as needed. Platelet and red cell engraftment is still a couple weeks in our future but seeing his white count really accelerate is a fantastic sign. We are now 37 days past his transplant and things are going great, still slow but great nonetheless.

However, being discharged from the hospital brought an emotional flood that I hadn't expected or maybe that I simply avoided thinking about. Nathan and I had been there since July 31st, that's 89 days inpatient. So much happened during that long hospital stay that our leaving kind of caused me to look back on it all before we can really turn the page. And turning that page forced me to face many things that I really didn't want to think about. He went through so much that it's nearly impossible to comprehend what this five year-old boy could endure yet still wake up with a smile and keep his spirits so positive through it all. At the time, I felt like my choice to stay with him (rather than switching off with Jay) was a bit of a selfish one but, as time went on, it became clear that having me go through it all with him and also have my life on hold made him feel so much less lonely and more  settled too. It was a lightbulb moment for me over these past few weeks and I am so thankful to have had the opportunity and the support which allowed me to really be there for him through every moment because it made such a huge difference in his ability to deal and cope with it all. And, I will admit, that it helped me tremendously also. Rather than being in my head and worrying about everything, I was always in the moment because I had no other choice. When his labs were getting worse and worse, I could look at him, at his smile, and know that all would be well. And both of our experiences kind of built off one another... my sense of peace helped him to stay positive, and his smile kept me sane. Living so fully in the moment gave me the opportunity to push the big picture of his diagnosis and treatment and all of his challenges aside which was a giant blessing but also a bit of a curse at the same time. I think I will be processing this experience for many years to come. So much sorrow and heartbreak, so much joy and hopeful moments, so many huge lessons that I will hold in my heart forever.

By far the most difficult part of leaving was the people. You build such strong connections during a long hospital stay. We met other families who were able to identify with our experience like nobody in our normal lives could. And those incredible nurses who are our new extended family. I refer to them as the family that nobody ever wants but for whom I am forever thankful. I don't think words could even express my feelings or how much their support meant to us all. They cared for Nathan with such love and attention and provided the sounding board that I sorely needed in my most difficult times. And there was that one nurse who Nathan really adored from the start, and who felt the same way about him. Those relationships meant the world to us both and got us through our long days in the hospital. It's so hard to say goodbye to people who have made such a huge impact on our lives... even when it's such a happy goodbye, even though we know that we will visit again many times in the future.

Now I am excited to start building our lives again. Being together as a family again while living at the Ronald McDonald House, spending some weekends at home and then slowly transitioning to being home full time over the next month or two. Then maybe even putting this whole ordeal completely behind us in another year. I know we will have our challenges and hiccups; the kids actually just had their first real fight since being out of the hospital and I know there are many more to come. Heightened emotions tends to result in sub-optimal behavior in children and even adults too. I get that, but it certainly doesn't make it any easier for this stressed out and very tired mommy. I'd love to pretend that we have this perfect idealized life where nobody ever says an unkind word but that's just unrealistic. We do, however, have a very thankful life and a huge dose of perspective to guide our futures together.

Engrafting!?!

I haven't written in the past few weeks, really it has been a lot of ups and downs and stress and waiting. The good news is that Nathan is so strong. He has been very stable... no infections, blood pressure fluctuations, fevers, nothing. A small bout of rhino virus that he somehow was able to fight off despite not having any white cells at the time. It's incredible really. In the second week following the transplant, he became very very lethargic and stopped eating. This is likely due to the beginning of engraftment and also his rhino virus. Then on day 19 his white count went from 0 to .2 (200 cells) and his energy shot up. Since that day, it has been very slow going. up .1, down .1, holding steady, up a bit more, repeat repeat, repeat. Such is life and I'll take slow, steady improvements. However, my exclamation points in the title to this post very much echo our confusion. He is engrafting... but isn't officially engrafted yet. It is taking so much longer than anyone expected but, at the same time, he is engrafting! We learned the other day that his white cells are 98.4% female meaning that they are derived from his sister's stem cells. They cannot do an official chimerism test just yet, he doesn't have enough cells, but this little peek at the male/female makeup of his white blood cells gave us some relief. Yes, he is 33 days post transplant and still very transfusion dependent. He is getting platelets every other day, red blood every 1-2 weeks and his white count is still only 1.0 (that's 1,000 cells, normal is 4,000+). Baby steps. I can be patient, and I am trusting his body and her cells to do their work. I cannot look at him and think of anything other than his own inner strength and vitality. Through it all, he has trusted us completely and kept in such great spirits (well, most of the time). He takes his meds, listens to his doctors, wakes up every morning telling me how much he loves me and spends his days playing and smiling and laughing and evading my feeble attempts to get him to eat a good meal. This is the life of a very sick (yet also remarkably healthy) five year old little boy. Sure he should be riding his bike and climbing trees but there will be more of that in his future.

So what will the next several weeks bring? Assuming all goes as it has, we are moving out of our hospital room and back into our room at the Ronald McDonald House this coming week. This doesn't mean that he is all better, really he's very far from that. It's just that he is stable, all of his meds are oral now and he can remain on fluids and his anti fungal infusion outside of the hospital so we are basically taking up space here at this point. While he is thrilled at the prospect of getting out of the hospital, he is very attached to many of the nurses and doctors here and becomes sad when he talks of how he will miss them so I've promised that we can visit anytime. He still has a long road ahead of him though and will remain severely immunocompromised for another year or more. We will have daily clinic appointments until all three of his blood lines show signs of recovery. If this takes too long, he may get re-infused with some more of Camryn's frozen marrow. There are risks to this little "boost" but in general the risks are minor in his case and it can even be done in clinic. This helps me rest easy at night. As his clinic appointment space out a bit, then we will begin planning to actually go back home. We hope this won't take more than a month or two but, again, I cannot predict the future and I will stay wherever we need to be to ensure that he makes a full and complete recovery. In the meantime, I am just going to appreciate the present, slow and steady progress, my strong little boy, his generous and loving sister, and the hopes of a full recovery so our family can be together again in the not too distant future.

The Little Things

Sometimes the little tiny things hit me hard when I least expect it... even more than the really big things that I can just kind of hunker down and deal with as it comes.

Nathan was looking at photos and videos on an old iPhone that he uses. I kind of lost it when he started to play a video of him and Camryn playing in his room. It was the sort of random silly stuff that little kids take photos or videos of but that's what hit me. This is how life was; none of the heavy stuff we are dealing with right now and they were both so much more unencumbered by the harsh realities of our current situation.

Or when I glance at a picture of how he used to look, before steroids made his face and body "puffy" and when the twinkle in his eye shone just that much brighter. Or seeing him traverse a creek by walking on a fragile log, or happily scale a fallen tree that was so high that it was out of my reach. I realize I have somehow gotten used to this current version of Nathan and all of the limitations on him, and that's truly ok as living in this moment is all I can do, but it doesn't mean I never wish to go back to the way things were... it's more that I cannot truly long for those days for fear that the longing would hurt too deeply right now. And the reminder that we will never be "that" family again is just overwhelming as I don't yet know in what ways we have change although I know those changes are tremendous and sometimes painful to even begin to consider.

How about the day I noticed his hair falling out? Why does such an expected and inconsequential thing hit me right where it hurts? I know it's just an external sign of what he is going through but I think that's just it! It forces me to really see and realize his illness in a very obvious way. As cute as he is bald, and as much as I adore him this way, it's the circumstances that lead to this point that kind of slapped me in the face as I saw my sweet little boy pull out increasing strands of hair as the day went on.

There was a time when a simple blood draw was a big deal, when the idea of even one blood transfusion was unfathomable, when even a short hospital stay seemed daunting, when a virus was good for the immune system, when prescription medications and chemotherapy weren't even on my radar, when when when... I can go on and on and on but suffice it to say those times are in our past and replaced with a new appreciation for the simple things in life and the people who make every day that much more special.  There is such a huge gulf between "then" and "now" that I just cannot reconcile the two and even just to try feels futile. Hindsight is not my friend right now, I need to focus more on foresight for the time being.

BMT Day +7

We are officially one week post Nathan's BMT. This means that we are done with all chemotherapy medications and just waiting for engraftment to occur. For a bone marrow transplant patient, so much is measured from day zero. Days prior to transplant at "negative" numbered days and days post transplant are "positive" numbered days. I imagine for a good portion of his childhood, we will be counting the days, months and years post transplant as much as his own birthday. The significance of "Day 0" is tremendous to the point that I cannot possibly list all the reasons here but suffice it to say that his transplant will have lasting effects through the course of his life. 

I know lots of other families dealing with bone marrow transplant for aplastic anemia scour the web much like I did in order to kind of know what to expect. I also know that the circumstances are so individual and variable and also depend largely on the conditioning method used. Nathan's conditioning "roadmap" included several chemotherapy medications prior to transplant to clear out his own system and prepare for the new cells, as well as a few very small doses of different chemotherapy meds post transplant to try to reduce the risk of GVHD (graft vs. host disease) and common viral infections like EBV (Epstein Barr Virus). Here is his roadmap in detail, this only includes the major medications used in his conditioning and not the other medications used to mediate side effects or other issues he is having aside from the transplant.

• Days -6, -5, -4, -3: Chemotherapy Pre-Conditioning with Fludarabine, R-ATG and Cyclophosphamide/Cytoxan along with Mesna to protect his bladder from the Cytoxan and Tacrolimus (similar to Cyclosporine) for continued immunosupression for approximately a year post BMT
• Days -2, -1: Rest 
• Day 0: Bone marrow Harvest and Transplant Day
• Day +1: Methotrexate (GVHD prevention)
• Day +3: Methotrexate (GVHD prevention)
• Day +5: Rituximab (EBV prevention)
• Day +6: Methotrexate (GVHD prevention)

As I have said before, I am surprised by how smoothly the pre-conditioning phase went. He was very sleepy on days -5 through -2, likely due to high doses of benadryl as much as chemo effects. He felt sick and had three bouts of vomiting on days -4 and -3. He started to bounce back by around day 0 and has been increasingly active ever since, certainly nowhere near his old self but still we spend a good amount of time in the halls every day walking and playing with his beach ball or in the room playing board games and doing crafts. He loves spending time with the volunteers and the music and art therapists too.

On the evening of day 6, we noticed his hair starting to fall out and will likely shave his head (per his request) sometime over the next few days. Nathan hasn't had any mucusitis or any other major post-chemotherapy effects to date and I am told it is unlikely that he will at this point just due to the time that has passed and the "reduced intensity" regimen that he has had. His liver enzymes have gone up a bit due to the recent methotrexate doses but that is normal and the numbers will go back down as quickly as they went up. The only other not so great effect is that he is consuming platelets rapidly once again likely due to the ATG but that also should be just a temporary issue that will remedy itself just like it did the last time he received this medication. Now we just have to wait for the new cells to grow which can begin to happen as early as day 10 or as late as day 24, and then hope there aren't any major complications following engraftment so we can finally start planning to go home.

Post-Transplant Update

Nathan's bone marrow transplant was yesterday. It was an exhaustingly full day for all of us. Camryn had her marrow harvested at noon and she was so brave and very peaceful but for a few tough moments. We are all incredibly proud of her. She was woozy waking up from anesthesia and a bit sore as well so it took her a while to get back on her feet again. They got back to Nathan's room just moments before her cells began going into Nathan's picc line at 5pm. And of course, by this time, he was crabby and exhausted. I think the events of the day, anxiety, tiredness, hunger, elevated blood pressure and low hemoglobin took their toll on him. It felt like the perfect storm of crabby moods and overwhelm for all of us. Suffice it to say that it wasn't the picturesque rainbows and unicorns sibling transplant that one may envision (albeit unrealistically) but it is done and the cells are in him and both kids are doing great today. And that's what matters most.

Now we get to move forward. We have a good week of waiting for any delayed chemo effects such as mucositis (not very likely) and hair loss (all but inevitable), then another couple weeks waiting and hoping for signs of bone marrow growth. And then the other complications like graft vs. host disease may begin to rear their ugly head. In the meantime, he will continue on whatever medications and transfusions he needs to manage his symptoms and keep him as strong as possible through this. He surprises me with his strength and resilience and his great spirits. Even in his crabbiest moods, which are very rare, he is able to snap out of it and find his own balance remarkably well. He hasn't been home in over three months and I think we are all managing amazingly well considering how intense each day is. We just need to hold on a bit longer and imagine those beautiful stem cells finding their home and growing a perfectly functioning new "blood factory" for Nathan so we can finally begin to plan our journey back home.

And now we rest!

Nathan's four day round of chemotherapy pre-conditioning is over! Now he has two days of rest before his transplant day on Monday. I was really worried about how he would feel on chemo but it really wasn't as bad as I had thought it would be. He had lots of ups and downs for sure but it seemed the first two days were the hardest on him. He mostly slept a lot and had a few intermittent bouts of diarrhea and some vomiting too. On days three and four, he began to perk up and feel a bit better so I hope these two rest days will give him the strength he needs for the next part of his journey. The post-transplant effects are highly variable. He can feel great and have very few of relatively minor issues or he can have a whole host of complications in the forms of serious graft-versus-host disease and delayed chemotherapy side effects. The next month will be critical. I am nervous yet also very excited.

A few people have asked what medications he is on and I will warn that it is a very long and intimidating list, and I am likely forgetting at least a couple medications as it is very difficult to keep track of the daily changes. I am guessing these words won't mean anything to most people except maybe those in the medical field or other BMT patients. His chemotherapy medications over these past four days were Fludarabene, Cytoxan and R-ATG. To support his body through the chemotherapy phase, he also was given Decidron which is a steroid that reduces his risk of reacting to the ATG as well as Benadryl for the same purpose and Mesna to protect his bladder. All of those medications are done now but he is continuing on a high dose of Tacrolimus (similar to Cyclosporine) to suppress his t-cells so they do not attack the new stem cells upon transplant. In addition to these key conditioning medicines, there is a whole host of medicines that he will continue on in order to manage side effects and reduce the risk of infections and viruses while his immune system is suppressed. These include antibiotics (Pipercillin-Zosan), antifungals (Micafungin), antivirals (Acyclovir), antacids (Lansoprazol and Ranitidine), Zofran for nausea, Amlodopine and Enalapril to control his blood pressure, Ursidiol to support his gallbladder, Hydrocortisone to support his adrenals through the transplant, Heparin to protect his liver and a high level of IV fluid maintenance with supplemental magnesium and potassium added. Soon he will begin new medications to address any post-transplant complications and also reduce the risks of various other effects... namely Methotrexate to prevent GVHD, Rituximab to prevent EBV and periodic IVIG transfusions to boost his immune system. Unfortunately both methotrexate and Rituximab are considered chemo drugs, albeit without the traditional chemo effects and in significantly lower doses in this case, but still our time with chemo isn't quite over yet. And then there are all of the unknowns post transplant that will have to be controlled for as issues arise. It's overwhelming to think of this list of complications and medications but it is what it is and all of it is temporary. Suffice it to say, a bone marrow transplant is kind of a big deal.

Three months

Today, September 19th, marks three months since this journey began. 13 weeks plus one day. Nathan and I haven't been home in 92 days total; 71 in the hospital and 21 at the Ronald McDonald House. Many many more days to go still before we will go back home again.

Three months since our world was rocked to its core, since we found out that Nathan was so very sick, since our hearts were broken then immediately expanded exponentially... I guess sometimes things need to break to make room for real growth. Instantly we possessed love and strength that we never thought humanly possible;  love and strength that we would find ourselves clinging to in order to sustain this ordeal.

Three months of waiting and trying to figure out exactly what was truly going on and how to best treat him. Stress, anxiety, a variety of possible diagnoses and finally the official diagnosis presented itself. Thrilled that we have something to treat but terrified that the treatment can be as challenging as the disease.

Three months and we have a whole new vocabulary of terms that would have previously meant nothing to us but now these simple words can make or break our days.

Three months and we are longing to be back home again but knowing that our "home" will never be quite the same, there is no going back to the way things once were. Yet still, we are together, that is what matters most. And we have an extended group of friends and family who has lifted us up and taken such great care of our basic needs; of nurses who have become like family for their love of our sweet little boy, and doctors who have worked tirelessly to make sure that they select the right treatment for him. Home means something very different to us now. Home is where our family is and where love resides so this must be our home for now.

Three months that nearly feel like a lifetime, and I guess it should feel that way, for we are all completely transformed by this experience.

Three months of hope and counting our many many blessings because, despite everything, there is still so very much to be thankful for. But we are also exhausted to the core yet knowing that some of our toughest days are still to come. No five year old should have to fight for his life and endure the many difficult procedures and medications that offer his only really hope of recovery. No seven year old should be asked to step up and save her brother's life. No parents should have to watch a child slowly lose his strength and drift away... a child that was just recently healthy and strong and vibrant. But we must be on this path for some reason and maybe, once we have the luxury of hindsight, that reason will be abundantly clear to us. In the meantime, we have been here for three months... and counting. 

The Transplant Journey Begins

Today was Nathan's first day of chemotherapy. In a previous post, I described his planned conditioning and bone marrow transplants regimen in detail. I refer to it as clearing out the cobwebs of his nearly empty bone marrow to make room for his sister's stem cells to move in. In truth, it is poison. As someone who avoids toxins and generally likes to live a clean life, it is humbling and sobering and sometimes even terrifying. Yesterday he became sick due to an unusual reaction to a blood pressure patch that we started a few hours earlier. I joked that I was getting an early taste of chemo side effects and it seems that this joke was pretty close to reality. Today he seems to be overloaded and, while we didn't expect him to really start feeling really sick for nearly a week, it appears as though his body is feeling some minor effects of the chemotherapy already on day 1. Now we haven't experienced any scary side effects but it is clear that he is very fatigued and definitely not feeling well. I am hoping that this is the extent of it and that he will simply "sleep it off" but it is tough to see my formerly active and exuberant little boy feeling so sleepy and lethargic. I am not sure what to expect for the next three days of conditioning (much less the days beyond his transplant) but I am at least glad that we haven't seen any of the early anaphylactic or otherwise truly life-threatening effects. There are always blessing to be had in every moment, even the really tough moments when you are slapped with some harsh realities. Nathan is sick, very very sick, and the treatment is no walk in the park either. But there is a light at the end of the tunnel. September 22nd is his transplant date and then we can begin his journey towards healing. We are as ready as we can be and hoping to just hold on and keep on swimming. We may be tearing him down now but there is always time to build him back up again after this is all over. And that's what I keep reminding myself of. As with everything, "this too shall pass" and I just need to breathe and envision brighter days ahead.

Planning for a Bone Marrow Transplant

"It's empty!" Those words, the words of our doctor when referring to Nathan's bone marrow, were not at all unexpected. We are five weeks from his ATG treatment and he should be showing some early signs of growth given all of the white cell stimulant (both g-csf and gm-csf) he has been given each day. But thus far nothing has changed in his daily labs, no growth, nothing indicating to us that he is showing even a minor response to the treatments. His bone marrow is just "empty", officially having less than 5% (essentially immeasurable) cellularity, so his lack of progress makes sense given that there is effectively nothing to build from. On the plus side, nothing out of the ordinary was seen on his biopsy, no histiocites, nothing growing that shouldn't be there. And the ATG worked a small miracle on his liver inflammation. And he is still very happy and experiencing minimal complications or discomfort. And these are all really good things. Now it very much feels like the time to plan for the next steps... a bone marrow transplant. I'm going to go into detail regarding some of the preparation that we are looking at in the next couple of days.

Tomorrow Nathan will begin all of the preparation to clear him for transplant. This includes a series of exams to ensure that all of his organs are healthy which includes a CT scan, a GFR test for his kidneys, an echocardiogram, a pulmonary exam, a comprehensive series of blood tests and a dental exam. He will also need another PICC line in his other arm as he will need four access points for all of his conditioning. And of course he will also have a psychological and physical therapy evaluation. And we will switch to a different room that is specially cleaned and prepared for a bone marrow transplant patient. I asked for a room with a sunrise view since Nathan just loves to watch the sun rise; if we are going to be stuck in our room again while waiting for his immune system to grow, we may as well have something beautiful to wake up to!

Camryn is all but cleared to be his donor, and we are just waiting for a few minor pieces of paperwork to be completed. She has had several physicals, two huge blood draws (22 vials in total), and a psychological evaluation. She is of course anxious and wishes he would get better without going through the transplant, but don't we all? She is also very willing to overcome her fears and anxieties to help her brother get better knowing that this is his best chance at recovering and getting back home again. But this is a big deal for her and we are hoping as much as we can to make it a positive experience for her so she remembers her courage first and foremost.

Once both of them are cleared, then the real work will begin. He will start a four-day round of chemotherapy using cytoxin, fludarabine and R-ATG likely before the end of the week. This is considered reduced intensity conditioning for a bone marrow transplant. However, while most regimens for an identical HLA matched sibling would not include fludarabine, Nathan has enough abnormalities in his illness and has had a huge number of transfusions so it makes sense to include this additional medication to ensure that engraftment occurs. After the chemotherapy is complete, we have two rest days and then the transplant occurs. The actual bone marrow harvest from Camryn will essentially be very similar to a bone marrow biopsy except the needle is different and more fluid will be extracted; Camryn will be sedated and then a weight-appropriate amount of her bone marrow aspirate will be removed with the whole process taking less than an hour. After the procedure, her aspirate will be processed and prepared for the transplant to occur most likely the same day. I am told that transplant day will be pretty uneventful for him as he is simply getting a bag of his sister's bone marrow aspirate which will look like blood and it will effectively go into his system through his PICC line just like a blood transfusion. Then we wait... and hope that chemotherapy side effects will be minimal as the doctor suspects given his "reduced intensity" regimen. However, bacterial and fungal infections, viruses and graft vs. host disease are huge risks in the initial weeks following transplant and there seems to be a very rigorous protocol to reduce these risks. He will get a lot of medications to control complications during this time. Then hopefully within a few weeks, we will see his blood counts increase and we can begin planning for the future. But, during this time, he will be very severely immunocompromised and we have made decision to not allow any visitors until he has at least a few white blood cells and neutrophils to protect him. This could take days or weeks to even begin happening... there's no way to know right now exactly how it will go. 

I have felt for a long time that this is where we were headed but I really appreciate the doctors doing their due diligence because it really feels like everything is coming together at just the right time. Getting genetic testing back that rule out major genetic triggers like HLH or a GATA2 defect, our primary and favorite BMT doctor being on staff the majority of the month, Camryn feeling truly ready to do this major thing for her brother, his liver healing, and even small things like getting a visit from our "Make a Wish" wish granters all within the past week... it seems like everything is falling into place to ensure his successful recovery and that is the most that any of us can hope for. 

At the same time, reading through the paperwork outlining his regimen is very sobering. Effectively it states that his disease will result in his death but that the treatment could also result in his death... thats a huge pill for a mom to swallow. But it's not as though we have a real choice in the matter either. And his doctor, if I haven't said so already, is incredible. It is rare to have a doctor who will sit and really talk with you, collaborate, think outside the box, consider protocol but really look at the child in front of him first and foremost. I feel very fortunate that we have one of those doctors and I think I may be his biggest fan right now. I have to continue to trust that Nathan is in great hands and surrounded with the right team of people to help him get well again because he truly is, and the rest is completely out of our hands. We just have to hope that all will be well. 

So I'm going back to a poem that I have read many times through this journey...

"Hope" is the thing with feathers— 
That perches in the soul— 
And sings the tune without the words— 
And never stops—at all— 

And sweetest—in the Gale—is heard— 
And sore must be the storm— 
That could abash the little Bird 
That kept so many warm— 

I've heard it in the chillest land— 
And on the strangest Sea— 
Yet, never, in Extremity, 
It asked a crumb—of Me.

~Emily Dickinson

Riding the Roller Coaster -- Four Weeks Post ATG

This past week has been a roller coaster of sorts. Nathan is increasingly bored from having been in isolation for nearly four weeks and we have been effectively just hanging around and waiting. It has been difficult to keep him busy and happy within the confines of these four walls and, if I'm being honest, I'm pretty bored too. Suffice it to say that life here was quite uneventful... until this past Friday when Nathan spiked a fever and his other vital signs showed signs of stress, plus he stayed in bed sleeping the entire day which is very unusual for him. Of course the doctors were concerned because he has no ability to fight infection so he was given a CT scan to check his sinuses and lungs (all clear) and blood cultures were drawn to see if any bacteria was present (also negative). It seems he was simply having a bit of a reaction to weaning off steroids, which I had initially suspected as the cause of his illness given his lack of any outward signs of infection. A stress dose of hydrocortisone seemed to perk him up quickly but he was also placed on a few broad spectrum antibiotics until the blood cultures were confirmed to be clear as well as a systemic anti fungal, thankfully without any major side effects to date. That was much action for one day and I joked that it was a nine bead day; he was quite excited to add all those new ones to his beads of courage. So my point is that I much prefer being bored. And here we are today, bored again, but I'm sure we will have many ups and downs to come.

Clinical Update

From a clinical perspective there is effectively no change in his aplastic anemia. His body doesn't seem to be showing any signs of responding to the ATG treatment and we are now four weeks since he received his last dose. Seems we are indeed headed down the path of a bone marrow transplant and thankfully Camryn and Nathan are perfect sibling matches which is a huge blessing that I talked about at length here and here. Lots of tests and evaluations will occur in the next 1.5 weeks and I am glad that our primary BMT doctor is the attending physician in the hospital HOT (i.e. hematology oncology & transplant) unit this week as I will feel most confident having him here to make all of the day to day decisions leading up the transplant decision and preparation. We continue to wait for some genetic testing but we did find out that he does not have the GATA2 genetic mutation that they suspected some time ago. I am not sure if I'm relieved or sad at this news, clearly something lead to this illness and it sure would be nice to be able to name it, but I can't dwell on that right now. 

Blood Counts
WBC: 0.3  (4.0-12.0)

Neutrophil Count: 141 (1400-1600)

RBC: 3.41 (4.0-5.3)

Hemoglobin: 9.9 (11.5-14.5)
He received a RBC transfusion three days ago.

Platelets: 22 (150-450)

He is getting platelet transfusions every 1-2 days and will get a transfusion again tomorrow as his counts will definitely be in the single digits once again.

Liver Inflammation

AST: 16 (23-58)

ALT: 98 (6-35)

Total Bili: 1.0 (0-1.1)

We likely won't continue to track these numbers as they are effectively normal now.

Medication Notes

While it looks like we are heading closer to a bone marrow transplant, our doctor wanted to try one more medication to see if he could stimulate white cell growth. Nathan is now on both G-CSF and GM-CSF and still his neutrophil and white cell counts are stagnant and showing no real signs of life. His Tacrolimus level has stayed more consistent now at a dose of 10ML. Given his reaction to the steroid weaning, his steroids have been increased again and we are starting the slow weaning process once more, hoping he doesn't bottom out again. He continues on one antibiotic to inhibit bacterial growth and has added Micafungin to inhibit fungal growth as well as medications for his elevated blood pressure (due to tacro) and acid reflux (due to steroids). I don't love the idea of treating an illness and then treating the side effects of the treatment, but it seems that is where we are right now. Bone marrow failure is serious to say the least and the treatment options seem to match the severity of the illness. I am quite certain that our post transplant regimen (assuming we do in fact go in that direction) will be quite intense. But it is all worth it to heal Nathan and get my sweet little guy back home again. There will be plenty of time to work on building his system back up again when this is all behind us. 

Much love to all of our friends and family!

The Greatest Gifts

Throughout this journey we have been blessed with many gifts. Gifts for Nathan or Camryn, much needed food to nourish us, help with a multitude of tasks that we cannot manage alone, a kind ear, thoughtful words, prayers and love and light, and a tremendous number of things which make our stay here a little more joyful and remind us that we are so very loved... Knowing that our friends, family, and even near strangers are thinking of us and our journey here is a huge help, and I cannot even put into words that anybody who hasn't been in this situation could ever understand. Often times I am left feeling like a simple "thank you" could never be enough to cover the depth of the feelings of gratitude that I hold in my heart each and every time someone reaches out to us. Then there are some gifts which truly leave me grasping for breath at the huge-ness of them, and I want to describe two of those immeasurable gifts...

I wrote at length about Nathan and Camryn, their relationship and their sibling bone marrow match here. She had her blood drawn yesterday as the first step to clearing her as his bone marrow donor. This was a big deal for her as she seems to have a visceral fear of needles, and she had to sit there while they filled 15 tubes of her blood. I am amazed by how well the blood draw went and her willingness to work through this fear to help her brother. And hearing how accepting she is of the possible  future surgery to extract her bone marrow aspirate and how excited she is to watch her cells go into his body, it feels healing on so many levels. It's really hard for me to put into words but I feel like we are finally going down a path to a real solution, and it feels serendipitous to me. That she is a match for him, it just makes sense and I have felt for a long time that this journey is way more about their relationship than anything else. This gift that she is so unconditionally willing to give her brother, for love of him and a deep desire to make him all better, it's truly incredible and unfathomable to me. I have had parents ponder how you ask one child to suffer for the benefit of the other and somehow that hasn't even been an issue; it is so much more about giving and healing that the suffering pales in comparison and isn't even a consideration for HER. How a seven year-old girl is so capable of understanding that on such a deep intuitive level is beyond me and I feel unworthy of being a mother to such a child. While there are so many more steps to go over the coming weeks and months, the biggest most challenging parts still in our future, just seeing these kids welcome each day with their arms wide open and showing such courage and deep love reassures me that everything is going to be alright.

And then my birthday was last week and I began the day thinking that I was just going to get through it, postpone the celebration and just treat it like any other day in the hospital. But that morning I went on my Facebok page and saw post after post and picture after picture of friends donating blood to celebrate my birthday, in Nathan's honor, and sharing information about blood donation. That so many people took the time to think of us and go out of their way to help another, to give this huge and potential life saving gift, it brought me to tears. It seems my dear friends conspired to give me this huge surprise and I could't imagine a better birthday present! Thank you to everyone who helped to give me a truly special birthday. And for those of you who are still thinking of donating blood, it only takes an hour of your time and makes a tremendous difference. I am thankful every day for the countless donors who have taken time to give blood, it is a simple gesture that makes an immediate difference in the life of an individual who is fighting for his or her life. That blood products are so readily available in hospitals at this point in time is nothing short of a miracle, and every person who rolls up their sleeve to donate their blood is a part of this miracle. Here are the links to locate a blood donation center within Illinois or Wisconsin.

So thank you to everyone for all of your gifts, big or small, profound or pragmatic... you are making a difference in these four lives and then some. I absolutely feel the love and, with love, all things are possible!

A New Normal

I try not to look at the big picture, or think about how long we have been here and the fact that an entire summer has been spent in the hospital, or to fully realize how rare and how critical his condition is. It just isn't helpful in any way to our every day lives here. Somehow this has become normal and we have found our own little routine in the confines of this tiny little hospital room and within the space we have at the Ronald McDonald House. This is our new normal for the time being and we have to make the best of it. At the same time, I am also becoming acutely aware of the fact that life is moving on all around us. We miss home, our family and dear friends terribly and we miss our regular old boring life. But we know that our loved ones will be waiting with open arms to welcome us back home when the time comes. It just seems that day is very distant and uncertain still. And I know we will all be changed from this experience, and I wonder how much the world around us will have changed while we were gone. 

Here we are 21 days after his ATG treatment ended, nearly ten weeks away from home, and still no signs of bone marrow growth. This is the week that our doctors said we would start talking about transplant and sure enough our doctor was in our room early this morning. There is still time for his marrow to recover but, at the same time, we have achieved our goals for this treatment since his liver inflammation is all but gone. Nobody really expected this treatment to be enough to salvage his marrow, we have been mentally preparing for a transplant this whole time. In the meantime, Nathan remains isolated to his room since he continues to shead his rhino virus despite having no cold symptoms for about a week now. Suffice it to say that we are increasingly antsy to just get on with it and try a real solution while he is still well appearing and happy and could be expected to handle the transplant and the conditioning well. And, yes, we are also longing to get back home and to unpause our lives and begin really living again only with a new perspective and deep appreciation for our many blessings. 

We are preparing Camryn too. She is incredible, really and I am in awe of her complete openness to give this huge gift to her little brother and her willingness to put aside her very real fears to hopefully help him. We are also not really acknowledging the huge-ness of bone marrow donation when talking to her in case it doesn't actually happen, she doesn't pass the donor process or there are any major complications. She knows it's a pretty big deal and what she is doing is a very good thing, but building it up too much will only cause anxiety or a possible let down in the future. For now, we are living in the moment and trying to be as normal as we can. Today she will have her first (and hopefully only) series of blood draws and then start preparing for the actual marrow extraction that may take place as soon as three weeks from now. She is terrified of needles and has never had a blood draw before but we hope that all the preparation that we have done with her and the work she has done with Jenny her psychologist will make this process go smoothly. Assuming we don't see consistent signs of growth in Nathan's labs, his pre-transplant conditioning could begin in about  2-3 weeks then the extraction and transplant in 3-4. Camryn has requested that she watch her cells go into Nathan (it is essentially done the same as a blood transfusion) and we are going to try to make that happen as I imagine it'll be very therapeutic for her. 

Clinical Update

As I mentioned above, our concerns over his liver are all but gone as the elevated numbers continue to trend down. I am so thankful to not have to worry about the possibility of a liver transplant as well as the bone marrow transplant. Today's lab numbers are below, again the blood counts are not indicative of anything given all the blood products and immunosupression he has had but I wanted to include them here anyway. Normal ranges are in parenthesis after each number for comparison. 

Blood Counts
WBC: 0.3  (4.0-12.0)

Neutrophil Count: 132 (1400-1600)

RBC: 3.26 (4.0-5.3)

Hemoglobin: 9.6 (11.5-14.5)
He received a RBC transfusion two days ago.

Platelets: 14 (150-450)

He is getting platelet transfusions every 1-2 days and will get a transfusion again today.

Liver Inflammation

AST: 74 (23-58)

ALT: 259 (6-35)

Total Bili: 1.3 (0-1.1)

Medication Notes

His tacrolimus medication has been increased again as the doctors attempt to maintain a therapeutic level in his blood stream. I really hope this levels off and we have a more consistent dose before the transplant process begins. He continues to wean off steroids and will likely be completely weaned next week. He remains on GCFS/Neupogen at a double dose to try to stimulate his bone marrow to begin producing neutrophils. He continues on an antibiotic to inhibit bacterial growth as well as other medications to treat any symptoms that he is encountering due to his illness or his medications such as his slightly elevated blood pressure and acid reflux. And we are still awaiting genetic results which will likely still take a long time. We may have some pieces to his genetic puzzle before transplant or we may not, we are preparing for either possibility but also open to the distant chance that his marrow could begin to really rebuild over the next couple of weeks without a transplant. Who knows what the future will hold...

An Ode to Home Education

Like every family who chooses to educate their children away from the confines of a school, we have a whole laundry list of reasons that are meaningful to us... but that's not what this post is about. It's about those little messages we get through our lives that caused us to take a different and unexpected path while trusting that everything is going to work out despite the people who tell us otherwise. The way a variety of choices and circumstances don't make any sense on their own but all together feel like serendipity. So, yes, we "homeschool" and we love this path that we've chosen. Or rather it has chosen us for all we had to do is listen and trust and jump in without a net. Every single day of our lives has been spent together, connecting with one another, exploring our world, learning from everyone and everything around us. It started with Camryn, my vivacious seven year old second grader who has taught me more about love and life than I ever thought possible, and now I look at Nathan who would be starting Kindergarten this year and I'm overwhelmed with gratitude for the life that we lead and the time that we are able to spend together. Having a child with a sudden and life threatening illness provides a giant dose of perspective. I will never for a moment regret the investment that we've made in our children, even those moments of intense frustration and overwhelm, for they are so worth it. On a more pragmatic level, having the flexibility to embrace this difficult journey 100% without concern for school schedules or following someone else's lesson plan is a gift!

Tomorrow marks the beginning of a new school year in our neighborhood and still our lives are on hold. Many people have asked how we will manage to educate the kids given our current circumstances, and I imagine for every person who has asked we have several more who have this same question. The crux is that we are looking at a much larger picture. You see, education isn't something that only happens within the confines of a specific building and I would insist even that our most vital education occurs primarily outside of those big buildings. Then there's the education of our souls, those lessons that we are learning together right now which are far more valuable than anything that we can learn from a book. Sure we will practice reading and math and cover some of the basics as it makes sense but, truly, life is providing us with some of our most important lessons right now which we are  embracing wholeheartedly. The worry that they may fall behind academically is not even a blip on my radar. This is a time for soul growth and character development and learning some harsh realities that kids their age shouldn't have to learn yet we must welcome because, for whatever reason, this is the path we are on. We have to trust in the process and know that there is a time and a place for rigorous academics and inspired lesson plans, but that time is just not right now. Our choice to homeschool gives us infinite flexibility which really comes in handy during times like this. 

So I remain thankful for this twisty turny unconventional life we are living because it is a beautiful life, and so filled with love, and that's what matters most. 

Rejoice with your family in the beautiful land of life! ~Albert Einstein

The Sibling Match

I can only imagine the stress that Camryn must feel right now. Displaced, taken away from everything she knows, her best friend and little brother critically ill, her parents emotionally and physically exhausted... life turned upside down. She and Nathan have a connection that I am just in awe of at times. They fight intensely but love each other even more. We worked very hard as parents to reinforce this connection and strengthen our family unit first and foremost, the downside is that our current turmoil may hurt much more deeply. Camryn has always been a sensitive child in every way. She feels every emotion and all the energy around her yet she is so young, too young to even know what it is that she's feeling and lacking the coping skills that she will need to go through her life being so completely tuned into the world around her. All this means that we have struggled, and she has had such a hard time finding her place among everything else that is going on. The first few days were a blur but she and Nate carried on mostly as normal. Then there were the weeks when Nathan was downright nasty to her no matter what, he even admitted that seeing Camryn was hard for him because he wanted to play but he couldn't and she was able to go home but he couldn't. Simply put, he was taking it out on her. Then there were the weeks when her behavior was just appalling, acting out, attention seeking, sass, and downright rudeness. All normal normal seven year old stuff, amplified during phases of coping, but incredibly hard to parent through when we are at the limit of our own patience and all but emotionally unavailable. And now we seem to have had a breakthrough and are in a more balanced phase, seeing their amazing connection clearly again, and able to more effectively weather the inevitable storms. But I am sure there are more difficult phases to come and lots of emotional healing for all of us once this is behind us.

I've mentioned before that Camryn is a perfect sibling bone marrow match. It's unusual for a sibling to be a match with only a 25% chance but somehow I knew she would be. A friend calls camryn a little healer, for Nathan that may be literally true. When we found out that she was a match, I felt like it was only a matter of time before a bone marrow transplant would become a reality. Maybe I'm wrong but it just felt right that she would be the one to inevitably heal her brother. We have discussed this with them very openly, first talking about their "blood factory" and what is going on with Nathan. Then discussing how they had the same blood factory and how amazing and rare that is, reinforcing their connection with one another. And over time talking about the realities of a bone marrow transplant with them both in an age appropriate way. She seems strangely unfazed by the idea and we talk about what a gift it is that she can be the one to heal her brother which really resonates with her and I think speaks to her need to find a place and a role among this chaos. She is only terrified of the blood draw, and seems to share her father's irrational needle fear. She started seeing a psychologist who works through the bone marrow transplant clinic both to just talk about what she's going through and also to work through some of these fears. The other day she and Jenny (the psychologist) created a visual "toolbox" of things she can do when she's feeling scared or anxious and they also did some role playing with a doll that was sick and needed a blood draw. Such simple exercises but so effective for a child like Camryn and I am thankful that she has someone to turn to who is a neutral ear and able to help her navigate all of her emotions. She also went with us when we had our blood drawn so she could see one in action and know what to expect. The upside of all the time that we have spent waiting is that we have had the ability to work through all of the emotions that she is dealing with and hopefully bring this process about in a way that is healthy and positive and healing for everyone... sometimes things happen just the way and in the time that they are supposed to. I am pretty sure that she may still flip out when the time comes for her to have a blood draw but all of these exercises will hopefully help to give her the skills and courage to get through it. But the donor process is a big deal. Her own unconditional willingness aside, she will have to be physically cleared and also work with a donor advocate to be certain that she can emotionally handle the process and any possible complications that may occur (e.g. what if Nathan has a bad reaction or even dies in the process, etc...). I am very curious to see what the future will hold and I know that this transplant may very well not happen or could even happen years into the future. But the reality is that in less than two weeks we may be traveling this road and all of us need to be ready for the challenge. In the meantime, I am simply enjoying seeing their love and compassion for one another blossom once again. It's a beautiful thing.

Clinical Update - 10 Days Post ATG

It has been ten days since Nathan's last ATG treatment. Unfortunately, his suppressed immune system has succumbed both to a minor cold and an overgrowth of e coli (not food borne, apparently e coli lives in most of our intestines but in some cases can move into our blood stream and cause a systemic infection) which may be further hampering any progress he could be making. Despite being isolated to his room for over a week now, Nathan continues to wear a constant smile. It definitely helps that he has received several packages and tasty food deliveries over the past several days which always boosts his spirits. So thank you again to everyone, you all know who you are, for bringing smiles to our faces in the midst of all this.

Progress?
We continue to wait for his bone marrow to respond to the treatment and specifically hope that his white blood and neutrophil counts begin to increase soon. That would be the first area where we would see progress and also his inability to fight infection is what is currently keeping us in the hospital vs. at the Ronald McDonald House. We will begin preparing for a bone marrow transplant if there aren't any early signs of progress within the next two weeks so we are both keeping our fingers crossed and preparing for the possibility of a transplant. While his blood counts continue to be stagnant at best, his liver inflammation is dramatically reduced which is a huge relief. It will take longer for his liver and spleen size to match the reduced inflammation but in time that will happen as well, it's just a long process. Today's lab numbers are below, again the blood counts are not indicative of anything given all the blood products and immunosupression he has had but I wanted to include them here anyway. Normal ranges are in parenthesis after each number for comparison. 

Blood Counts
WBC: 0.2  (4.0-12.0)

Neutrophil Count: 71 (1400-6600)

RBC: 3.58 (4.0-5.3)

Hemoglobin: 10.3 (11.5-14.5)
He received a RBC transfusion two days ago.

Platelets: 8 (150-450)

He is getting platelet transfusions every 1-2 days. Obviously he received a transfusion after this morning's count, his body continues to consume platelets quite rapidly. I can no longer keep count of the number of transfusions that he has received, maybe 40-something over the past eight weeks, and counting...

Liver Inflammation

AST: 277 (23-58)

ALT: 605 (6-35)

Total Bili: 4.0 (0-1.1)

Conjugated/Direct Bili: 0.7 (0-0.0.3) 

His bilirubin has shown a huge improvement over the past week. His skin and eyes are much more normal to reflect this near normal level of bilirubin so this is progress that we can literally see, which is so reassuring. While the above enzymes and bilirubin are signs of inflammation (aka hepatitis), his liver function continues to be normal despite all this prolonged inflammation. Seems that the possibility of a liver transplant, even though it was only slight, is far less likely now. It's a huge blessing to have one less thing to worry about although we are continuing to track his liver numbers quite closely since we aren't quite out of the woods yet, especially if he does need a bone marrow transplant. 

Medication Notes

We have finally solved the mystery of his strangely low prograf level. He was metabolizing a huge dose so quickly but then one day his metabolism slowed down, his level shot up and his dose was then reduced to a more normal level. I personally feel that his digestion was overstimulated due to his steroid dose and oral magnesium supplementation, resulting in very frequent stooling and an unhappy bottom. His tacrolimus (prograf) dose is down to a third of what it was and is now 5ml twice a day (10ml/day) where he seems to be holding a nice low therapeutic dose to help keep his T-cells from going out of control again. 

The last major change is a decrease in his steroids as they continue to wean him off over the next couple of weeks. He remains on GCFS/Neupogen at a double dose to try to stimulate his bone marrow to begin producing neutrophils. And of course he is on a course of antibiotics until his blood cultures show no signs of e coli for three consecutive days. All of his other medications are mostly treating any symptoms that he is encountering due to his illness or his medications such as his slightly elevated blood pressure and acid reflux. All in all, considering the severity of his illness, he is not as highly medicated as I would expect. 

Genetics
Our doctors feel like there is likely a genetic defect at the root of Nathan's illness. It seems that his body wasn't producing B cells or natural killer cells, essentially meaning he was unable to produce antibodies or mount an appropriate response to a virus, and this complete absence of these cells is highly unusual. There is no way of knowing if this has been the case his entire life and somehow he was managing it just fine in the past, or if this immune system imbalance was triggered by the same thing that caused his liver inflammation and bone marrow failure. So while we continue to wait for the HLH genetic testing (early next week) and NIH testing (mid-September) related to a possible GATA2 core immunodeficiency, the genetics team here is preparing to begin a full genome sequencing to attempt to identify any number of genetic defects that could be contributing to his current illness. Of course this will take up to three months to complete but the information could be vital to any future treatments that he receives.