Eight Months

Another month has passed by and progress continues to be great. Another month of rising counts. Another month at home and without any major hurdles. Another month without any transfusions. Another month of increasing health and vitality and energy.

Here are his counts as of last Monday, which is BMT +147 and boost +91:
White Blood Count: 6.6
Absolute Neutrophil Count: 3.9
Red Blood Count: 3.19
Hemoglobin: 10.9
Platelets: 314

So all of his counts are at a healthy level except for his red count and hemoglobin, but it's slowly getting there. The past month has been pretty quiet. Both kids have been fighting a small string of colds and, thankfully, Nathan seems to be able to handle these minor illnesses remarkably well. He did receive an IVIG transfusion this week just to boost his system a little bit as hisown IGG level, while normal, was still on the low end of normal. Unfortunately, Nathan's kidneys continue to be stressed and, despite his high rate of 24 hour fluids and his abundant oral intake, his creatinine has been steadily increasing  and was 0.81 this past Monday. Dr. Margolis is not terribly concerned which is reassuring but I really do not like to see this number going up especially since his immunosuppressive medication (the one that is causing his kidneys so much stress) will not begin to be weaned for at least a month still, possibly several months. I have hope that this is much like his prior liver inflammation, stressed out organs that are otherwise strong and able to withstand the inflammation... I hope. Suffice it to say that he will continue to have his picc line (peripheral central line in his arm) and Jay and I will be playing nurse for a good long time still. I wish this were not the case but it is what it is and, just like I told myself many many moths ago, I need to continue to be patient and know that all will be well even if it's not on my plan or timeline. And again, if I just look at Nathan I can see that he is doing great. He has such energy and joy and is back to his old playful and silly ways. It's amazing to see Nathan truly being himself again... even if he has a line of fluids and a backpack on his back at all times.

On an emotional level, I am finding myself mourning our former lives much more deeply this past few weeks. The knowledge that, no matter how hard I try, life just isn't and won't be the same ever again. And while I intuitively know that is perfectly alright, it also is a sobering realization and some days this reality hits me to the core. I may have been there every moment but I am simply in awe of it all, it's incredible and amazing and inspiring and heartbreaking all at the same time. I think this mourning, this processing, it's all normal and we need to embrace it and allow it to happen even if it hurts. Somedays I am overwhelmed with joyful tears, and other days its sadness and stress, then there are the days when it's all mixed up in happy/sad ugly cry. But I have somehow never felt anger, I think I am too aware of our blessings and thankful to be where we are to dwell on those destructive thoughts, but still I even have moments when I wonder why I'm not at least a little angry as insane as that may sound. These emotions seem to come in waves and I need to essentially ride those waves as they come.  It's that proverbial peeling of an onion, layer by layer and exposing something different each time. I think it may take nearly a lifetime to truly process the totality of this experience and be able to revisit it without tears and overwhelm.

I have found this experience to be the most intense and constant form of meditation that I could ever imagine. Always keeping a clear head, managing my thought streams every moment of every day; reframing, refocusing, training my mind... It's exhausting but truly the most valuable discipline that I have learned. A friend of mine used to always say that "you can't ride the roller coaster" meaning if I revel too much in the ups then I will be equally saddened by the downs, and that insight has never been more true than over the past eight months. Keeping a level head among such extreme ups and down, among the chaos of a life turned completely upside down, has been an intense practice but such a worthwhile one at the same time. And with this newfound sense of clarity, I began to realize that there are many blessings even in our darkest moments and I would hold onto those blessings for dear life. In our most difficult moments, I would take a breath and look at Nathan, really look at him. Get out of my head, resist the urge to live in denial or escapism or even over dramatization, and live in that moment. To trust his innate strength and in the lessons that we are meant to learn on this path. I cannot predict the future, but I can always remind myself to open my eyes and ears and to really trust the journey. We can make up a whole life in our heads but nothing outside that particular moment is truly relevant. Inhale exhale and let it all go.

Then there is that strength that people often talk of. "You are so strong!" Is a phrase that I hear most every day from well meaning friends, family and even near strangers. It is a great compliment and sometimes this strength feels like a calling, but other times it feels like a burden. Knowing I can go through what every parent would list as one of their worst fears and do it gracefully, while keeping Nathan in great spirits, without breaking down completely... it's so damn empowering but also a monumental challenge. And I am quite certain that every single person who says those words would rise to the occasion in the same way. That's the strength of being a parent, of being a mother, you don't know you have it in you until you desperately need it. But I still brush it off because it just is what it is and I was literally surrounded by strength the whole time. Jay was there every step of the way doing what I thought was the harder job... taking care of Camryn, shuffling between home and the Ronald McDonald House, working full time, making sure we had food to eat and essentially keeping it all together. That man is a saint and I can never wrap my arms around what he was able to do which enabled me to focus so completely on Nathan. And of course we had help, amazing friends and family who filled in all the gaps and made sure we could last the grueling marathon that was our lives. Nurses and doctors who held us together and helped to bring our little boy back. But truly, my strength, Jay's strength, doesn't even come close to Camryn's strength in confronting her biggest fears for love of her brother and a strong desire to help him. And our strength pales in comparison to Nathan's strength in handling everything that he has been faced with and doing it with a smile. To put it simply, we had to be strong because we had these two examples of profound superhuman strength looking right at us and what can we do but empower, encourage and sustain them through it all. Their strength is what is amazing to me, we were just along for the ride. 

Hepatitis?

Sitting at clinic and I figured I'd answer a question that I have gotten quite a bit lately. "How did he get hepatitis?" It's a potentially taboo question so I imagine many people have wanted to ask but didn't. Most people think of hepatitis as a virus cased by drug use or sexual contact (hep b/c) or poor sanitation (hep a). However hepatitis is actually a condition of the liver literally translated from Greek to mean "liver inflammation". Hepatitis is most commonly caused by a virus (aka hepatitis a/b/c/d/e) but it can also be caused by exposure to certain toxins or an autoimmune reaction. In Nathan's case, his hepatitis was caused by an autoimmune attack on his liver, specifically CD-8 T cells (a type of lymphocyte which is a type of white blood cell) went out of control and infiltrated and damaged his liver then subsequently attacked his bone marrow causing his marrow to fail even after his immune system calmed down. This is why I've referred to his liver condition as "autoimmune hepatitis" although I'm sure not everyone truly knows what that means. So there's your fun fact for the day... Hepatitis is not a virus, it is simply inflammation of the liver which could have many potential causes. And I'm very thankful that his particular case of hepatitis was acute and has fully resolved. 

The other question I've been asked is whether this form of hepatitis is vaccine preventable and the answer is no. There are vaccines which cover certain types of viruses that can cause hepatitis (hep A/B) but no vaccine can protect your liver from becoming inflamed or irritated by any number of potential triggers... and I think that is why the word "hepatitis" causes such confusion. Vaccines are meant to protect against specific viruses and that's it. There is no vaccine that could possibly protect against an overgrowth of a specific type of lymphocytes which then decide to attack the liver and bone marrow. Just like there is no vaccine that can protect against environmental exposure to a toxin which also can attack the liver. That simply isn't the purpose of vaccines. 

Seven Months

I had an epiphany last night; it's the 19th! Seven months since the day that our lives were turned upside down. And for once on this monthly anniversary, I am so very happy with Nathan's progress. We are home and together and got through a wonderful holiday season without any issues or admissions to the hospital. He has blood and platelets and white cells, not a ton, but lots and the cells are growing. He's not fighting any active viruses or infections and, despite having been severely neutropenic (i.e. susceptible to infection) for four straight months, he made it through and is gaining strength and energy every single day. He's eating and drinking and keeping it all down and not having any major GI issues. He currently has "angry" kidneys but this problem is well controlled with 24 hour IV fluids. Most importantly, he is himself again; that playful, fun, chatty and silly little five year old boy that he was before this all began (ignore the fact that he was four at the time). In the grand scheme of things, he is doing very very well. I am amazed at his turnaround because this all started with him having a remarkably unusual presentation of a very rare form of a very rare disease. Approximately 900 people are diagnosed with Aplastic Anemia per year, about 45 of which have the form that Nathan had (hepatitis associated aplastic anemia) and he literally wrote his own book given the unprecedented way it all began. He was admitted to the hospital with a increasingly sick liver and a platelet count of ten, requiring near daily platelet transfusions, and bone marrow that could no longer produce enough of the blood that he needed to live... a problem that was only getting worse by the day before his marrow completely shut down. He made some brilliant doctors scratch their heads for two months before they could give us a concrete diagnosis and we truly still do not know what triggered this vicious auto immune attack on his body. But his liver is all better. He is making his own platelets, white cells and red cells and he is finally no longer dependent on blood transfusions to sustain him. I cannot say enough how much joy that brings to my heart... that we made it through some of the worst times and are coming out the other side stronger and profoundly thankful for this gift of life. Here are his counts 119 days post transplant and 63 days post that much needed "boost" of bone marrow from his very generous sister, as usual with the normal ranges in parenthesis:
White blood: 3.2 (4.0 - 12.0)
Absolute neutrophil count: 1,700 (1,400 - 6,600)
Red blood: 2.99 (4.0 -5.3)Hemoglobin: 10 (11.5 - 14.5)
Reticulocyte%: 2.7 (0.5 - 1.1 - high is good in this case, his body is cranking out red cells at a record pace and he was even higher a couple weeks ago)
Absolute retic ~80
Platelets: 297 (150 - 450)
^^^ did you see that? Holy platelets batman! 

Now that we are home after living at or near the hospital for so many months, I find myself more emotional and more anxious about everything. I think the fact that we were getting daily lab results for such a long time caused me to have an irrational attachment to that sort of constant feedback. But I think the bigger factor is the reality check that I had especially during our three months stay at the hospital. Being in the hospital for as long as we have, seeing all the other children fighting their battles, it caused me to really look at Nathan and focus on each day and each moment so intensely. To look at him and see that, no matter what he was fighting, he still had that sparkle in his eyes, that smile on his face, and there was never a moment when the situation became immediately critical... many many children could not say the same thing. And also, sadly, to learn of yet another child who lost his or her battle, it was truly heart breaking. I couldn't mope or focus on the bad stuff, I was far better off looking at the sweet boy in front of me and just being his mommy. Even now, my heart is broken to learn of a third teenaged boy in two months who lost his battle. Three boys who were in the hospital at some point with Nathan; their names are Jake, Nick and Tanner. Three families who I got to know even if just casually while getting coffee or preparing a third breakfast for our boys on steroids. One mother who cried tears of joy with me the morning when Nathan began to engraft and his counts began to come in, but now her boy is gone. Three times I was brought to "ugly" tears upon hearing this tragic news. Three families who are forever left to continue on with their lives with a piece of their hearts forever vacant. That's just a hole that cannot ever be filled. And, while these families touched my heart particularly deeply, unfortunately there are too many more just like them. Now I know many people reading this might feel overwhelmed or want to look away but this is happening, children are suffering, families are losing children to disease at an alarming rate, and many more new families hear the news of a critical and life threatening diagnosis every single day. It's heartbreaking but it IS. I was completely unaware of this stark reality before our family became one of "those families" and I sincerely hope that our story can at least spread a bit of awareness and maybe even inspire others to open their eyes and their hearts to the far too many children who are fighting these huge battles. So what can you do? Truly, every little bit helps tremendously. Give blood at a local blood bank  Join the bone marrow registry at Be The Match; trust me it's easy to join and, if you happen to get called to donate, remind yourself that a seven year old id it and so can you! Give to organizations who are specifically fighting to create new treatments for children because the funding is seriously lacking here. Give time and energy to organizations who support these families; amazing organizations like Make-A-Wish and The Ronald McDonald House Charities among many many others that I will be sure to point out as time goes on. But most of all, open your eyes and your hearts... don't look away. These children deserve your attention. 

So what are we up to now? Mostly we are laying low and enjoying some much needed family time together and attempting to live as normal a life as we can. We don't get out much for fear of the flu which seems rampant right now. We hope that Nathan's kidneys will calm down over time so he can get off fluids and have his picc line removed by summer. I am still not sure how realistic this is but it's a goal. No matter what, we want to make this year, and every year after, special and meaningful and magical and wonderful for my two amazing children. Life is truly a miracle!

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.