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. 

A Special Note on Gluten Intolerance

I know my audience here is very small but I feel the need to share this important information with those closest to us. Gluten intolerance is a very real thing! I too often see posts and articles shared online calling it nothing but a fad and it is hard for me to restrain my frustration at seeing people who aren't living with this issue pretending that it doesn't exist. Instead I choose to believe that it is a simple lack of understanding. So, for the benefit of my friends and family, I want to share some of our recent experiences with gluten intolerance and navigating food landmines during this intense phase of our lives. As many of you know, Camryn has been strictly gluten free (I.e. avoiding all foods containing wheat, barley and rye or any ingredients derived from those three grains) since she was four and you also know that she have been essentially away from home or traveling back and forth for over seven weeks now while Nathan is critically ill. This means that we don't always have access to our typical foods or have time to make a proper meal happen. As a result, mistakes have been made and she has been exposed to gluten containing foods more than once over the past few weeks, and we are seeing the effects. What do the effects of gluten exposure look like for her? I know this list is different for each person and is even different for a single person depending upon where they are in their healing process. For camryn right now we are seeing frequent dizziness, headaches, stomach aches, night terrors, decreased energy, mood swings, differences in behavior and ability to focus, bacterial build up giving her early signs of urinary tract infections (thankfully we know how to manage to is before it becomes a true infection) and a few additional minor issues. Now I know these may not seem like serious issues when typed out, or when compared to Nathan's life-threatening illness or another child's anaphylactic food allergy, but the effects of gluten exposure are still very real and very frightening to a seven-year old child and her parents. And prolonged exposure results in inflammation and exponentially increases the risk of a whole host of potentially serious future illnesses. I have regrets, we haven't been able to be as careful as we normally would be, and now Camryn is having to deal with these effects on top of all the other stress and anxiety in our lives right now. 

So, what can you do to help accommodate a gluten intolerant person? I want to clarify that we do not ever expect others to cook anything spefically for us although it is always appreciated when people do make special accommodations. Essentially, my wish is that anyone cooking for others in any way simply write down a complete list of ingredients and copy or include the labels of any packaged goods used. This simple step will tremendously help anyone with any dietary need and put the decision as to whether it is safe in our hands. I often feel like eating food in a pot luck setting, or even with family who know about our food restrictions, is akin to navigating landmines. I cannot expect the gereral public to know what gluten is or know the laundry list of foods containing gluten but I do hope that increased awareness will cause fewer people to just assume that our dietary needs aren't serious and try to placate our questions with a, "oh no this doesn't have any gluten", when it clearly does. I will add the caveat that some people are so highly intolerant to a specific food that they also must avoid all forms of cross contamination so their needs would be more complex of course but still a bit of awareness and being proactive in sharing a complete list of ingredients will help a huge number of people avoid the effects of "insert specific food here" exposure. 

I will also admit that, in many ways, "gluten free" does look like a fad on th surface and there are many people eating a gluten free diet by choice (and yes sometimes even without knowing what gluten is) rather than by necessity yet still that choice should also be respected. Truly nobody needs to eat gluten to be healthy and one's personal choice is not a condemnation of another's opposing choice. Plus cooking and eating a stict whole food-based, gluten free diet is a whole lot of work. I wonder why so many choose to belittle or placate this choice (or often necessity) rather than actually understand it and support one another wherever we are in our particular life's journey. I am here and happy to help if anyone would like to learn more about gluten, its effects and gluten free cooking/eating. Any questions?

Post ATG Update

Nathan finished four days of a medication called ATG and now we are waiting to see the effects. It generally takes several weeks, up to three months, for the marrow to rebound after this treatment but his doctors likely won't give him quite that long due to some complicating factors in Nathan's case and the fact that he has a sibling bone marrow match. ATG is a fascinating medicine. In very basic terms, they take human T cells, inject them into an animal (in this case, a horse), let the animal create antibodies against those T cells then inject those antibodies into a person as a form of immunosupression. The objective is to lower his white cell and neutrophil count essentially to zero and then let his marrow regenerate (hopefully) healthy cells and a more balanced immune system that will no longer attack his cells. The other hope was that his liver inflammation would calm down due to this T cell suppression, thus making a possible future bone marrow transplant less complicated, and it seems we have at least partially accomplished that objective but only time will tell... As always. 

While we wait for his marrow to rebuild, he is taking a medication called Neupogen which stimulates production of Neutrophils which are the white cells that fight infection. Once his neutrophil count is at a safe level, above 500, the neupogen will stop and we will see what his body does. This could take a couple days or several weeks, it all depends in how his body responds. Lastly, they are reducing his steroid dose today as it was doubled while he was on ATG. Over the coming weeks they will likely wean him off the steroids completely.  

Today's labs are the first after a full day off ATG and on Neupogen. Most numbers are not terribly indicative of anything given all the blood products and immunosupression he has been given but I wanted to include them here for our own reference. Normal ranges are in parenthesis after each number for comparison. 

WBC: 0.2  (4.0-12.0)

Neutrophil Count: 158 (1400-6600)

RBC: 2.69 (4.0-5.3)

Hemoglobin: 7.1 (11.5-14.5)

He's likely getting a red cell transfusion tomorrow

Platelets: 37 (150-450)

This is two days after his last platelet transfusion, the longest amount of time he's gone without getting platelets since this journey began. Seems like we have at least slowed down whatever process was consuming his platelets so rapidly, likely his liver/spleen. I am guessing he will get a platelet transfusion tomorrow as well, they are trying to keep him above 20 for the time being but that threshold could be reduced to 10 soon as he has gotten into single digits with minimal symptoms in the past. 

Liver enzymes

AST: 372 (23-58)

ALT: 685 (6-35)

Both of these enzymes were above 3000 at one point so today's numbers almost look normal albeit still elevated. 

Bilirubin

Total Bili: 5.9 (0-1.1)

Conjugated/Direct Bili: 2.2 (0-0.0.3) 

His bilirubin will become even more elevated with each red cell transfusion but today is near as low as he's been this whole time and he looks much less yellow the past few days too

While the above liver numbers are signs of inflammation (aka hepatitis), it seems the liver function continues to be normal despite all this prolonged inflammation. 

The last strange mystery is that his tacrolimus/ prograf level is so low despite his extremely high dosage. Our doctors have never even given a dose this high yet he's still just under therapeutic levels. For now they are leaving the dose as is and seeing what happens over the coming days/weeks. 

Tacrolimus Dose: 15ml twice a day (30ml/day)

Tacrolimus Level: hovering in the 4 range, 6-15 is therapeutic, doctor ideally wants him around 8-12. He is metabolizing this medication extremely quickly. 

So basically we are in another holding pattern and Nathan remains a medical mystery with symptoms of various diagnoses yet none that fits exactly. For now they are going with the closest fit which is severe aplastic anemia with CD8 predominant T cell activated acute hepititis. In the meantime, he has handled all of the medications with minimal side effects and a continued happy and easy going attitude. 

Help, Helping and Helplessness

Being in this situation feels so helpless at times. I am a fixer, a Mother... Kisser of booboos and bringer of mommy magic. Knowing that there is nothing that I can do but sit back and trust, and hope and love and just do the best that I can every moment. I can't fix this! That's a big pill for me to swallow. At the same time, we seem to have the most amazing army of helpers. Dear friends, family and even people we barely know lifting us up with their kindness, generosity and amazing energy. Yet we are often faced with the question... How can we help? It's a loaded question for sure. Our needs are both very basic yet profoundly huge. Food, sanity, a listening ear, periodic care for Camryn, random help here at the hospital or the Ronald McDonald house... A magic wand... A crystal ball. And you have come through big time (except those last two of course). I have lost count of the packages and letters and cards and artwork Nathan has received. Or those of you who have visited, brought us food or cared for our basic needs in one way or another, I don't know what I would have done without you. Seriously, it's overwhelming and humbling that so many have reached out with a genuine desire to help, and gone out of their way to brighten our day or lighten our load. From the bottom of my heart, I thank you all! I know words alone are not even close to expressing how I feel and there is no way I can pay it back but I will absolutely pay it forward when this is all over. My heart is very full and that has been the blessing and the lesson through even our darkest moments. 

I am asked daily to provide a few simple ways in which you can help. Usually I brush it off as we are generally managing in the most basic sense, and while I am admittedly not great at this asking for help business, here's my attempt at a general list of helpful things that we would appreciate at any point during this journey:

• If you visit us and you are able, please donate blood. The blood bank is right next to the hospital. Or if you cannot visit us, please find a way to give blood close to home, I'm happy to help link you with a blood bank if you cannot find one. Seeing not only Nathan but so many other children desperately dependent on donor blood for their daily survival is a call to action for me. A big one. It takes minimal time and effort and no risk, yet helps countless critically ill people in their greatest time of need. I have literally lost count of the number of bags of blood products that Nathan has received over the past six weeks (and counting) and I am beyond thankful to each and every person who took the time to give their blood.
• If you are looking to send some food over, we can use all the simple gluten free and nutritious easy foods that you can offer. I think I survived on a very generous friend's delicious kale salad and chicken salad for a good week at one point. Keeping Nathan fed while he is on steroids is no easy task! Just let me know before hand if it's perishable as I don't want to have a huge influx of foods then have no space to store it or have precious food go to waste. Or if you want to send a meal for our freezer, we have a deep freeze in the garage at home and it helps Jay tremendously to have some easy to prepare meals to pull from when he is home, to bring here when we are at the Ronald McDonald House or in preparation for the day when we are all home again.
• If you are thinking of sending us a gift card, here are some of our favorite places that we visit regularly while here: Chipotle, Pizzeria Piccola, Outpost Natural Foods, Five Guys, Trader Joes and of course Amazon and Target continue to get a good chunk of our business as always. Also Nathan has begging me to get some treats from Molly's Gluten Free Bakery so a trip there will be happening very soon. 

I will likely be putting some more specific calls out for help at some point in the future, especially if he does need a bone marrow transplant. Plus I may need a small army to organize a proper welcome back home for this brave little guy when that time comes. 

In the meantime, if you're feeling so compelled, keep sending us your love, prayers, and lending an ear when we need it most. It reminds us that, while life is moving on all around us, Nathan is loved and cared for and thought about. I know that I say this all the time, but he is an extraordinary little boy. Truly. Those of you who know him can see his amazing smile and hear his sweet voice and feel his incredible energy as you read this. Knowing that so many people are missing him and rooting for him to return home quickly is a beautiful thing. This journey is very lonely at times but having all of you to surround us with love and light makes each day brighter. 

Our address at the Ronald McDonald House (we continue to have a room here even when we are at the hospital) if you'd like to send a card, artwork or package to Nathan:

Nathan Kempf

CO Ronald McDonald House

8948 W Watertown Plank Rd

Room 122

Wauwatosa, WI 53226

Back in the Hospital

We were re-admitted to the hospital yesterday to try a new medication. While I feel like a bone marrow transplant is in our future, upon chatting for hours in end with our wonderful doctors (seriously they are fantastic and collaborative and I can't say enough good stuff about their expertise and care), it seems that trying ATG first has a reasonable chance of calming his liver and spleen thus making a future transplant less complicated. There is also a chance that it could fix his bone marrow too but only time will tell. Normally they give this medication three months to have an effect on bone marrow production, in Nathan's case he's giving it four weeks to at least show some initial signs of improvement before switching gears and prepping for the transplant. This four weeks also buys us a little time while we get some genetic testing back as a known genetic diagnosis may necessitate a different preparation method for the bone marrow transplant. So, we wait.

On the more personal side, checking back in here feels very suffocating to me. The flood of emotions and reality of the situation really hit me last night. Aplastic anemia (aka bone marrow failure) is always deadly if left untreated, period. And these treatments are intense, essentially severely suppressing his immune system like an archaic hard reboot of an overloaded computer. Anybody else remember that blue screen on your computer? Yep... force quit, shut down, try to power up again and cross your fingers. That's kinda where we are. It's not chemo but that may also be in our future, who knows, certainly that would be part of the preparation for a bone marrow transplant. But I'm rambling on. This is what it's like to be inside my brain right now. In the meantime, Nate is generally happy and I am not a mess today as I was late last night. Tea and chocolate are awaiting, and I'm running low on both. And planning to add a yoga mat to my nightly ritual here too.