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.  

Spinning

Terrifying Loneliness

I'm terrified. There I've said it. As much hope as I have, there is this lingering visceral fear that lives inside me every day. I just want my little boy back. I just want our simple lives back. What? When? Millions of questions gone unanswered. How much longer can we all hang in here? A whole summer almost gone, eight weeks displaced, unknown, slow progress, terrified! I spend so much time focusing on our blessings, the bright side, grace, joy and all the really good moments we have because there is still so much to be thankful for. I guess sometimes I need to also give a bit of attention to the other sometimes unbearable side of this journey. And it's just excrutiating at times for all of us. Especially this uncertainty, that's the hardest part. There have been more than a few moments when I said to Jay "why can't it just be leukemia". It's a concrete diagnosis with a clear cut treatment path, and people know what it is. Now I am not minimizing the journey of a parent with a child suffering from leukemia, not even a little bit, only lamenting the uncertainty and rarity of the syndrome that we are dealing with. Hepatitis associated aplastic anemia is rare to say the least; aplastic anemia alone has a rate around 2 in 1 million, hepatitis associated aplastic anemia is a very rare form of aplastic anemia with only a handful of cases each year. There is likely a genetic trigger but one which may never be identified because it is just so rare. The treatments path is variable and less clear cut but can be very similar to leukemia... Which is why we are in the area of the hospital with leukemia patients which also feels lonely at times if I'm being honest. It seems like nobody else seems to understand what we are dealing with, or has ever heard of it. You have no idea how many times we've been told by well meaning family and friends, "Good thing it's not Leukemia" and it's hard not to feel a sense of dread. My point is, the big scary illness that you don't know is no less difficult than the big scary illness that you do know. In short, they both suck. Having a once vibrantly healthy child become so critically ill that he is dependent on near daily transfusions and there is a reasonable chance that he may not live through the illness (or the increasingly aggressive treatment) is mind boggling, every parents' worse nightmare... No matter the clinical diagnosis. And here we are. Making the most of it like every other family here. Breaking down at times. Falling apart when we can, sometimes even when it's least expected and inconvenient. Then I see that smile, those beautiful blue eyes with their undeniable sparkle, that little boy that brings joy to everyone around him... I can't help but hold on to hope because there it is right in front of me reminding me to hold on and trust just a little longer.