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.