My son stopped walking during Induction. About four days into treatment, he stumbled, fell and wouldn’t walk again for many weeks. We were well into Consolidation before he walked again, and even then he had to hold onto something to remain upright. I recall my two-year old calling out to his father, “Look! Nico walking!” The worst pain he ever experienced was in his legs and occurred during Delayed Intensification, after Vincristine and during the withdrawal of the second steroid pulse. We were giving him Oxycodone around-the-clock then. We got through frontline, but my son still suffered from obvious neuropathy. His left leg seemed to drag a bit as he walked. His left foot flopped about when he climbed stairs. Throughout the years of Maintenance therapy, he required Oxycodone after he received his monthly Vincristine dose. His oncologists discussed adding prophylactic medications to treat his neuropathy or possibly lowering his dose of Vincristine if things worsened, but luckily he eked out of treatment only requiring physical therapy.
 
Now we are more than 17 months off-treatment. He has not told me that his legs hurt in a long time. About 6 months ago, he said it didn’t feel like there was sand in his shoes all the time anymore. And although more than three years of chemo surely stunted his growth, and he is still small, he no longer sticks out when he is among his peers. One of my son’s best friend signed up to run in a racing series, and Nico wanted to join him. Nico has never raced before, and rarely runs except during the course of normal play. But we entered him, and spent race day preparing him to come in last - “This is just for fun” “Winning doesn’t matter” and “It’s your first run, so don’t be disappointed if you’re not as fast this time.” I also prepared myself for his inevitable tears at the finish line, and told myself it was still a good learning experience even if it ended in disappointment.
 
When the first pack of runners rounded the field, and headed towards the finish line, I did not expect to see my boy there. But there he was, running his heart out and keeping up. He came in 5th place out of 20 runners. Winning doesn’t matter. But today, we won.

Here is the gist of what happened from December 2015 through April 2016. Per his protocol, Nico’s blood counts continued to generally run too high. Remember the goal of maintenance therapy was bone marrow suppression - we wanted blood counts low, but not too low. So in response to Nico’s higher-than-desired counts,  his oncologist (per Nico’s protocol) would incrementally increase chemo dosage in hopes of lowering his blood counts to the desired range. Inevitably, however, Nico would catch a virus (because he is five), and this viral exposure in concert with the newly increased chemo dose would obliterate his counts completely. His blood counts went from too high to very dangerously low.  Nico would end up hospitalized, in contact isolation, and required blood transfusions while we waited for his bone marrow to recover. Nico ended up receiving more blood transfusions during maintenance than he did during intensive frontline chemo. It is incredibly anxiety-provoking to know your child has literally no ability to fight off any type of infection at all. I would spend a week feeling bitter, confined to a hospital room, and threaten to pull Nico out of school until the end of treatment. Then his counts would recover, I would soften to his pleas to return to what was his normal life, and realize that all of our sanity depended upon his return to school. And the whole thing would start all over.  


The underlying issue was that Nico was now hypermetabolizing the staple chemotherapeutic agent, a drug called 6MP. This was diagnosed by drawing Nico’s blood levels of 6MP metabolites. Metabolite testing is expensive, but Nico’s stubborn numbers and increasing symptoms of liver toxicity justified the cost. 6MP is metabolized into either 6MMP or 6TGN. The last year of treatment, Nico’s body started to metabolize 6MP into 6MMP preferentially. 6MMP is not only not anti-leukemic, it is very toxic to the liver. So Jeff and I were knowingly administering a daily medication to our child that was not only not benefitting him as it should, it was actually making him measurably sick (raising liver enzymes). Our oncologists would say there was still some benefit of 6MP administration in Nico’s case, just not the desired level of benefit. But based upon the level of 6MMP versus 6TGN in Nico’s bloodstream, that benefit was slight. That was a dark time. I was consumed trying to find a fix (was it the time or the way we were administering the drug?). I also felt like we were up against the clock (another month without adequate suppression might lessen Nico’s ability to stay in remission). 


Jeff and I and our oncologist all agreed that we needed to pull Nico out of his current protocol (the standard high-risk arm of a large clinical trial) in lieu of another pediatric leukemia trial specific to hypermetabolizers. This second, smaller trial was in its nascent stage, still in development by a team at our clinic. The trial-in-design intended to investigate an old medication for a new purpose. This purpose was to correct 6MP hypermetabolism. 6MP is not only a staple in treatment protocols for childhood leukemia, it is also used to treat Crohn’s disease and ulcerative colitis in adults. Adults, just like children, can have issues with drug metabolism. Years ago, studies were done with adult patients with ulcerative colitis who, like Nico, were metabolizing 6MP preferentially to 6MMP (which is a bad thing for those patients too). Scientists with an understanding of the complexities of drug metabolism theorized that a drug that could correct these 6MP metabolism issues already existed and was being used widely for another condition. Allopurinol is an older medication that has been used for many years to treat gouty arthritis. Allopurinol’s mechanism of action made it a potential antidote for 6MP hypermetabolism. So doctors tested this theory in the adult Crohn’s and ulcerative colitis patient populations, and were extremely successful. These adult studies concluded that Allopurinol shunted metabolism of 6MP away from 6MMP towards the desired pathway of the therapeutic 6TGN metabolite. With this data, some pediatric oncologists started prescribing Allopurinol to patients like Nico. Prescribing Allopurinol for pediatric leukemia patients is off-label, meaning there are no large clinical trials showing Allopurinol’s efficacy in children. And children do not necessarily metabolize drugs the same way that adults do. But considering the alternative, which was to do nothing, I think prescribing Allopurinol was a reasonable gamble. Nico's oncologist was one of doctors that was comfortably using Allopurinol in pediatric patients. Coincidentally, the period that Nico became a hypermetabolizer was also when our clinic launched a clinical study specific to Allopurinol in the pediatric leukemia population. The goal is to show that Allopurinol will work in pediatric leukemia patients the same way it did in adult Crohn’s and ulcerative colitis patients. Ultimately, the hope is that Allopurinol will be indicated (labeled) for use in pediatric patients. 


Per the new protocol, which was really still being designed, there were several steps in the ramp up before Nico could sign on. One of these steps included increasing Nico’s dose of 6MP to one that he never tolerated in the past. As his parents, Jeff and I were reticent about this step though we understood the rationale. The doctors needed to demonstate that Nico was a good candidate for the study before pulling him out of what is a tried-and-true treatment protocol for the vast majority of children with leukemia (i.e., prove Nico has toxic levels of 6MMP at X dose). The ramp up seemed to take an excruciatingly long time, again we felt the treatment clock running down. Then just as Nico was slated to start the trial drug, he had another blood count crash that required a chemo-hold and hospitalization. I knew the moment his temperature spiked what it meant - he would not meet the criteria essential to start the second trial. We were so discouraged. Thankfully, our oncologist was as fed up with this cycle as we were, and after Nico recovered we all opted to unofficially start the trial without being technically enrolled. Because Nico did not meet the study parameters when he started, his data will not officially count in the study results. But I know that Nico’s case inspired in a change in the trial’s enrollment criteria; so I like to think that Nico’s case still indirectly furthered this research. 


The new trial required that we spent a lot of time at clinic. Nico has to be closely monitored because the experimental drug was known to shunt, or at least alter, 6MP metabolism. There was a risk of over-correction. The dosages of all of his oral chemo had to be dropped and slowly titrated. We started going to clinic once a week for blood draws and exams. Our oncologists were not just looking at blood counts anymore, they were drawing the actual metabolite levels in Nico’s bloodstream on a weekly basis. Somehow this did not end up being that bad. We have spent so much time in clinic, it became a second home. We know everyone. Everyone knows us. Everyone is really nice. It just became our routine. Tuesday was clinic day. 


Nico could have been the poster child for this drug. His counts immediately shifted. We were able to titrate his doses of both 6MP and Allopurinol very quickly (a matter of a couple of weeks), and Nico remained in a therapeutic range for the duration of treatment. It is important to note that he was not just in the therapeutic range related to the ANC (absolute neutrophil count), which is the indicator used by pediatric oncologists to gauge adequate marrow suppression. Nico’s actual drug metabolites were measured, plotted and graphed. His 6TGN levels were perfect, in the high end of what is considered therapeutic, while his 6MMP levels remained at a safe level (and subsequently his liver enzymes remained in normal levels as well).  The Allopurinol allowed Nico to achieve adequate bone marrow suppression at a much lower dose of both 6MP and the other staple of maintenance therapy, Methotrexate. Nico went from 150% dosing to 75% dosing of both because of the Allopurinol, and he maintained a steady level of suppression within the desired range for months. 


This was a game-changer for us. All of the symptoms that Nico experienced related to liver toxicity diminished. They were not eliminated, but they were not as worrisome. For example, Nico had terrible bouts of hypoglycemia related to the 6MP. He had to drink milk every night around 3:00 a.m., or he would be symptomatic by morning. If he slept in, I was convinced he had slipped into a coma. I was nervous all of the time. On the Allopurinol, Nico still needed to eat ASAP upon rising, but we were able to sleep through the night for the first time in many years. The Allopurinol also eliminated our anxiety about the administration guidelines of 6MP. When we started treatment in 2013, 6MP had to be taken on an empty stomach - 1 hour before or 2 hours after food. It was also suggested that it should be taken in the evening. Try that with a toddler. It meant he either had to finish dinner no later than 5:30 pm everyday or we had to wake him up in the night to take medicine. Combine these rules with the worry over his blood sugar, and this was a real quality of life killer, I promise you. 


So, there is it. That is a wrap-up of the last six moths. There are more updates, but I thought this one was overdue. I wanted to write about our experience because the focus on metabolites is really new. Many clinics do not test metabolites at all. Many clinics that do test metabolites do not consider using Allopurinol. I do not know how difficult it is to get insurance companies to pay for metabolite testing. But it seems likely there might be pushback by some. I want other parents with kids like Nico to know about this possible solution. This is a real thing, and something can be done. 


The last six months were humbling. We became very aware of how lucky we were to both be where we are and have the doctor that we have. Whatever happens, we know that Nico got the best possible care that we were capable of getting for him. We have done everything we can, and that is all we can do. Now we just hope it was enough.

I have intended to update for many months. There have been many ups and downs in the time that I have been away. And I intend to revisit that time. But for today, I want to record that today was Nico's final IV chemotherapy. He started the final 5-day pulse of steroids. He will continue to take oral chemotherapy medication for 26 more days, and then it will all stop. Just like that. Today was a very special day in clinic, and I will post photos soon. But with much gratitude, I can say that we are hopeful. 

On the last clinic visit, after two weeks at 150% of both Methotrexate and 6MP, Nico’s neutrophil count was over 3,000. This is within the normal range, and not what we expected (or want) for a kid at these doses of chemotherapy. We were fairly surprised. Jeff had balked at the idea of increasing his doses based on his last blood counts, and we were very worried that his counts would tank again. Not only did his counts not drop, they were higher. So the plan was to then wait for the results of Nico’s latest metabolite test, and we were pretty certain that his results would be skewed to the liver toxic, non-therapeutic metabolite based on his rising liver function tests and symptoms.

The metabolite results seemed to take forever. This was due, in part, to a computer glitch. While we waited, Jeff and I decided that we want Nico to pull out of his current clinical trial in favor of using Allopurinol in an effort to shunt his metabolism to the desired pathway. It was not an easy decision to reach, but we were both very nervous with his last set of numbers.
 
While the wait continued, Nico started to develop mouth sores. At first they looked like the chronic sores that he gets at least once a month. But the sores started to look more like blisters, and I became worried. I sent a picture of his mouth to his doctors and stressed that we were anxious about his metabolite levels. I also explained Nico’s pattern of developing hypoglycemia at these doses. Nico was waking up two times per night to request milk and acting like he had low blood sugar. We were all exhausted.
 
The metabolite results finally appeared on the same day as these blisters became an obvious issue. Our primary oncologist called me and told me to take Nico off all of his medications. We discussed the direction that we wanted to go, and everyone agreed that Nico’s counts were just too high. Jeff and I felt an urgent need to suppress his counts. So the plan was to let Nico’s liver recover for a week, clear some of these bad metabolites out of his body, and then restart the chemo at a lower dose and in conjunction with this new medication. As we got off the phone, our oncologist said something like: and we’ll just hope he does not get a virus before then.
 
Three days later was Thanksgiving. I thought that Nico was unusually well behaved at dinner, and patted myself on the back. In fact, he was just seriously dehydrated, anemic and neutropenic. His fever was 103 by the time we reached the ER. He needed a blood transfusion, and once again we find ourselves isolated in a hospital room while we wait for his bone marrow to recover.
 
This is our third day in the hospital. Nico seems to feel a lot better since he received blood. But his lips are ripped up. He looks very, very similar to how he looked the last time we tried these doses and then developed hand-foot-and-mouth disease. I am not convinced these symptoms are from viruses anymore. I will not agree to increase his doses to 150% again. I am not sure how we will navigate the road back. We obviously cannot start chemo again until his counts recover. Nico’s neutrophil count is 40 (with one zero). I will update the journal when we know.
 
I guess we knew that after almost three years now, we would be limping to the finish line. These last few months have been rough. At some point Nico decided he was just not going to speak to any of us anymore. He stopped speaking to us, to nurses, to doctors, to anyone. He ignored everything anyone said to him. He has never reacted like this, so I became concerned and mentioned it to my mother. Her explanation made sense to me.  “It’s okay. Helps him cope. I don’t blame him. If he knew how, he would flip a bird.”
 
Signing off with the middle finger salute to leukemia.

A lot has happened and nothing has changed.
 
Since Nico was in the hospital, we have been very cautiously increasing his medications. We go to clinic every two weeks to check blood counts, liver enzymes and the metabolites of 6MP (the staple medication of maintenance chemotherapy).  We went to clinic yesterday, and Nico’s favorite volunteer, Daniel, had returned after a long break. Nico transformed from angry and bitter to completely charming in about two seconds. Daniel stayed with Nico for hours. Daniel will never know what a positive influence he has had in Nico’s life. His time is invaluable to us – it is the difference between a traumatic experience in a scary place and a fun time with someone who cares for you.
 
I am going to backtrack, and this might be a technical post. So if you just want an update on life in general – we are fine, chugging along and doing our best.  Nico continues to have chronic pain, but he has graduated from physical therapy because he has resumed a normal gross motor trajectory. Great news!
 
There was a glitch, as I mentioned before, in Nico’s chemo dosages. In maintenance therapy, two of the main staples are a daily medication called 6MP and a weekly medication called Methotrexate (MTX). These two medications are titrated based on blood work, specifically ANC (neutrophil count) and liver enzymes. Both 6MP and MTX can cause liver inflammation, and this manifests as elevated liver enzymes in blood work. When Nico’s liver enzymes started to climb, we checked 6MP metabolites to determine whether Nico was metabolizing 6MP into the desired anti-leukemic (“good”) metabolite or the liver-toxic-with-no-therapeutic-benefit (“bad”) metabolite. Testing metabolite levels is not commonly done. It depends on your doctor and your hospital. It might even be controversial in some ways. The protocol allows for liver enzymes to reach 40 times the upper limit of normal before considering a chemo hold. So, Nico’s elevated liver enzymes would not concern some doctors because his enzymes were never anywhere near that high. The argument is that bone marrow suppression alone, not metabolite levels, is correlated to treatment success. Whether metabolites really matter, especially if ANC is suppressed, is unknown. Some doctors argue that metabolite levels only matter when the liver is so obviously inflamed as to create a host of secondary problems itself. But there is a caveat. ANC is used to determine proper suppression specifically because if the ANC is too low, a life-threatening infection becomes a very real jeopardy. More children in treatment die from infection than from cancer. So the treatment calls for bone marrow suppression, but only within a range that allows an individual some reserve to fight off potential infections. ANC, however, is not always the best indicator of suppression.
 
The problem is that Nico’s bone marrow preferentially makes neutrophils. He maintains a higher ANC even when all other counts indicate that his marrow is suppressed. He is frequently not in the desired range for ANC, and based on the protocol his chemo dosages should be increased. Increased dosages means increased risk of liver toxicity. The other problem is that Nico’s 6MP metabolites fluctuate. The oncologists like to see the metabolites in a certain range. But generally, they look at the ratio of good versus bad metabolites, and this ratio does not usually change over time (in theory). This is not true in Nico’s case - his ratio has fluctuated wildly. We have tried to ferret out the lynchpin, or even if there is a lynchpin, but it is all a guess. Jeff and I postulated to our doctors that the timing of 6MP administration affects Nico’s metabolite levels. Nico’s 6MP metabolites were always in the desired range when we administered 6MP in the morning. But (and this is a big but) that was also the period of time in which his ANC was way too high. Circadian rhythm effects drug metabolism generally, and this has been studied specifically with 6MP. There is a very slight advantage for most kids if 6MP is given in the evening (but Nico has never been “most kids” during treatment). Nico has big problems with low blood sugar when we give 6MP in the evening. But we switched Nico back to evening administration specifically because we were worried this was contributing to his high ANC. We want any advantage that we can get. We were desperate to get his ANC down, and although it is finally coming down now, his metabolites are terrible again. Yesterday, his ANC was 1780, just barely over the desired range of 500-1500. Two weeks ago, his ANC was over 4,000, so this is a positive direction. We should know his metabolite levels in the next few days, but based on his liver enzymes, they are not good. So what is more important in this situation – ANC or metabolites? No one knows for sure.
 
As stated, 6MP and MTX are titrated to blood counts and are periodically adjusted to height and weight (usually at the beginning of each new 3-month cycle). These dose adjustments are reported as percentages. A child on the standard prescribed dose is said to be at “100% dosing.” Some kids only take 25% of a dose because that is all that is needed to suppress the child’s ANC into the desired range. Some kids require 200% of a dose to get the same amount of suppression. This is because every individual metabolizes medications differently. So this was the other glitch in our treatment course. Apparently, Nico’s doses were not titrated to height or weight for several months. This did not ultimately change the dose that we administered, but it did change the way his dose was reported to Jeff and me. We thought that Nico was taking 175% of both 6MP and MTX and still not getting adequate marrow suppression. In reality, based on his growth, he was just slightly over 100% of MTX and 125% of 6MP. That sounds like a completely different scenario to a parent. In the first scenario we believed that chemo was no longer working, and frankly, we were beyond worried. We essentially stalked our primary oncologist for a private meeting specifically to discuss these doses and that was when all this was uncovered. The next problem occurred when the above situation was “remedied” by applying the higher percentages to the updated height and weight. So basically, Nico’s doses of 6MP and MTX were both increased by 30%.  And then he caught enterovirus. That was my last update. We spent a week in the hospital. Nico required a lot of intervention and was very sick. Between the higher doses of chemo and the viral exposure, he had no reserve left.
 
For the record, I do feel that I bear some responsibility for what happened. I double-check everything. But when I originally asked about when height and weight were calculated into the formula, I was told that during maintenance adjustments are made based on counts alone. The source of this information was not our primary oncologist, and I accepted this answer until Nico’s counts and doses started to look wonky.  Then I kicked myself for failing to continue to calculate doses myself. Should patients have to do this? No. But this is the reality, and I knew this reality from working in healthcare for years. From diagnosis, I have always told our oncologists that I want to see the exact orders so that I can double-check. I should not have stopped questioning.
 
So since then, we have been gradually increasing the medications back to the pre-enterovirus dose. We have done this slowly. We also check blood counts and metabolites every two weeks. The question that has been posed is whether we need to add an additional medication that would shunt 6MP metabolism into the desired pathway. This would mean that after 3 years in a clinical trial, we would be pulled out of our current protocol. Nico’s data would no longer count towards the study’s ultimate conclusion. The additional medication is experimental. It is not a new medication, actually it is medication commonly used for gout, but it is not yet indicated for shunting 6MP metabolism. We would be part of a new study, only being done at a few hospitals, that is looking specifically at the medication’s use for this purpose. Adding this medication would mean adding another drug with a whole new set of side effects. And all of this would happen during the last 4 months of a 3.5-year treatment period. It seems a little late in the ballgame. It would also mean that Nico has to get weekly blood draws. The doctors added that Nico could “just” get an arm stick at a laboratory near our home so that we would not have to travel to the hospital every week. The suggestion that weekly lab draws from his arm (rather than his port) had an upside indicated a real lack of understanding of our situation. Asking a 5-year old to submit to weekly lab draws in some two-bit, drop-in lab after enduring over 3 years of traumatic, sometimes brutal, treatment is too much. If we enroll in this, we will have to figure something else out.
 
Obviously this is weighing on our minds. We are not making any decisions right now. Nico’s doctors want to see his metabolite levels after a couple of weeks of chemo at his pre-enterovirus dose before we decide what to do next. Jeff and I are wondering if we should just switch him back to morning administration. We do not want to make too many changes at once, and again be left wondering what caused what. But we should have the latest set of metabolite levels within a few days. If they are high (as suspected based on Nico’s liver enzymes and abdominal pain), I think we will explore that option. Nico’s doctors are skeptical that timing of 6MP administration will make any significant difference, but Jeff and I are both convinced that timing could shift metabolism. If you look at Nico’s labs, the trend seems obvious. But then we run the risk that we just exchange one problem for another - shifting metabolites but then potentially running the course of treatment with a high ANC. This is all a roll-of-the-dice, and it is scary. Some kids just stay in the desired range through treatment, and do not have to titrate doses very often, if at all. That just has not been our experience.
 
What does this mean? We do not know. Our oncologists say that Nico has been closely monitored, which is true. And his doses have been appropriate, which is true based on the protocol. The oncologists also state that although Nico has been a tricky patient, his bone marrow has generally been very well suppressed. The doctors insist that they still expect the best possible outcome. As parents you wonder if this is just a pat-reassurance because there really are no guarantees. There are so many aspects of care that have no clear answers. We just do the best we can with what we know and hope for both personal success and that our experience aids others in the future.

After months of trying, we finally succeeded in bringing Nico’s counts down!  But we overshot our goal by a whole lot.  For the lab people, WBC 430 (normal 5,500-15,000), ANC 20, (normal 1,500-8,500) Hgb 7.3, Hct 23. And his “bad” metabolites are really bad too, but that is a whole different post unto itself.

Nico developed blisters on his lips last week, which is nothing new. He gets mouth sores often, and that is pretty common side effect of chemo from what I understand. These sores were different though. They kept getting bigger and then they started to form scabs (or something like that). We were just watching him for a couple of days, but things spiraled on Friday night. He stopped eating, stopped drinking, stopped walking, and started to incessantly whine and cry. He had a low-grade fever and complained he was tired. “I want to go to bed” sent us into a tizzy. Coming from Nico, that comment indicates that something is very, very wrong. We took him to the ER and found his blood counts are pretty much as low as they have ever been.

We are staying in the hospital for now, and we could be here for a while. Our doctor said it could be a couple of weeks. They have suggested he might need a blood transfusion. It was offered today since his numbers are low enough to justify it. But we wanted to wait on his next set of labs (tonight). They have also suggested he might require IV narcotics (a pain pump that would either deliver a continuous dose or allow him to dose himself on demand). Currently he is getting Oxycodone every four hours, IV fluids and IV antibiotics every six hours. He has not been able to eat or drink even though he is desperate to do so. His attempts end in sobs. His lips bleed if he opens his mouth or smiles.

Normally Nico just carries on, but yesterday he seemed really down. He had overheard our discussions with the doctors and determined that the “little yellow pills” caused his mouth sores (that is what everyone thought at first, but our primary oncologist later definitively squashed that). Nico told me, “My lips won’t be normal again until I am six.” When I told him the sores were from a virus and would start to clear up in a few days, he seemed very relieved. I think he has started to count down our treatment-sentence in his own way (and without actually knowing when it is supposed to end).

Nico’s ability to fight off any type of infection is virtually nonexistent right now. The doctors suspect the sores span the length of his GI tract. Plus, he cannot hydrate himself. So sending him home is admittedly a bad idea. Jeff and I are probably more calm this hospitalization than any other. It just so happened that our primary attending was on-call this weekend. We had another long, long talk, and I am really glad that he does not bill by the hour. There were many factors that lead to Nico’s crash, but the final straw was a virus (probably coxsackie). I think there will be changes to how we resume chemo once we are able to do so. That is a different post though, so I will follow up on that later.

These medical issues make resuming work especially difficult, and I am actually one week into a new job now. That part is going to be tricky, but I will figure it out. Nico’s birthday is in two weeks, and we have reserved a pirate ship to take him and his classmates around the bay. Our hospital room looks out over the inner harbor, and in the mornings we look at the view and imagine floating away in our boat.  

Pray for smooth sailing.

Last week Nico caught a cold. This led to nighttime coughing and earaches.  Then overnight his eyes and fingers started to ooze pus. These symptoms bought us an extra trip to clinic. Frankly, I was hoping his blood counts would be low since the increasing chemo regimen has been a big worry. Counts were lower, but still not in the target range. We started antibiotics and the NP scheduled another dose of IVIG (antibodies pooled from tens of thousands of donors) with our next IV chemo, which was today. This was to help Nico kick the infections. This seemed like a great idea since he starts school next week. The wrench was that this month also included an LP (lumbar puncture).

I have written endlessly about our struggles with LPs. We thought we found what works best for him, an alternative to the dreaded OR. But honestly the last couple of LPs have been increasingly more difficult. The medication used for sedation in clinic (Versed) just has not worked as well. But what we all seemed to forget on this visit -- in the past all hell broke loose whenever Nico got Versed with any other medication. And the IVIG infusion requires a big dose of Benadryl beforehand.

For Nico, Benadryl and Versed do not mix. We tried this before, but it was almost a year ago. Is this the part where I list all the insanity that happened? I am pretty sure we all cried at some point. On the way home, stuck in rush hour while Nico just screamed, there was a quiet moment in which Nico announced, “Sad things happen in the hospital.” I am so sorry, Nico.

Needless to say, the LP was unsuccessful. Basically we just loaded him with Versed, which upset him so badly he vomited and peed all over both of us. And if that was not traumatic enough, he then got stuck in the back a couple of times with a spinal needle. We get to try all over again next month in the OR, which means dealing with low blood sugar and anesthesiologists again. 

Nico’s counts today were very close to the goal range. The other “good” thing is that we discovered Nico’s chemo doses were not as high as we believed. I have written about how the doses are titrated based on a combination of height/weight and counts. It ends up that the doctors do not recalculate height/weight at the same intervals and subsequently Nico’s doses had not reflected a recent growth spurt. Instead of being on 150% of MTX and 175% of 6MP, he was actually about 110% of MTX and 140% of 6MP. The difference in these numbers is significant to parents who think their child is no longer responding appropriately to chemo. My head might have exploded actually, but after recalculating months and months of past doses myself, I found that ultimately the doses he was taking would have basically been the same, and were just reported differently.

Maintenance therapy sucks. We have been doing this for so long. So many parts of our life have resumed as if our child is not being systematically poisoned everyday. Most of the time, in almost every way, it is easier than front line. But some days, like today, are unpleasant reminders of how this (now 32-month long) nonstop battery of chemotherapy, steroids, anesthesia, poking and prodding is taking a toll on our (not-yet-five-year-old) boy and our family. Some days, like today, we just want to scream enough is enough, even when we know we have many more months of this treatment to go and we will do nothing to stop it. Some days, we just need to erupt on this blog because that is the closest thing to catharsis that we are going to get at this point.

 

Camp Sunrise is a weeklong, overnight camp for Johns Hopkins pediatric oncology patients. Nico is not quite five, and has never before stayed the night with anyone but Jeff and me. Likewise, Jeff and I are the only people that have ever administered his daily chemo. But our child life specialist and clinic nurses thought Nico might be ready for this adventure. The camp includes a 24/7 pediatric oncologist, nurses from our unit and a large number of volunteers including previous patients who now serve as camp counselors. So after much deliberation, we decided to let Nico choose.

There was no hesitation. Nico wanted to spend the night in a cabin with other kids (his cabin contains seventeen 5-7 year-old boys – can you imagine?). Hopkins provided transportation to the camp, and the child life specialist told us that the bus ride is a favorite of the kids. We arrived to the meeting spot at 9:00 a.m. on Sunday. Ages ranged from Nico (the youngest) to teenagers. After an extensive check-in process, in which numerous personnel reviewed individual medication schedules, a huge white tour bus pulled up to the curb. The kids went nuts, oohing and aahing over their ride. I hid my tears, genuinely surprised by how much I hated the idea of separating. Nico was scared, but never waivered in his decision to go.

Nico joined a little wolf pack of older boys that loaded onto the bus first. Jeff and I nervously stood with a group of parents waiting to wave from the proverbial dock. Another couple pushed their own little girl in a wheelchair into the line. About eight years old, she was very thin and mostly bald. She wore a brace on her left foot and I believe her left arm as well. Once to the front of the line, she stood on her own and limped to the open door. I felt the group of parents that lined the curb collectively hold our breath and lean forward. This bus was huge and required a stepstool to even reach the stairs. The little girl swayed as she stood and trekked forward. Her parents stepped towards her ready to swoop. We all wanted to help her, at least prevent her from falling. One of the volunteers, a woman charged with this responsibility for more than twenty years, raised her hand toward the girls’ parents and confidently assured all of the adults in earshot, “She’s got this.” We deferred. With her strong arm, this little girl grabbed the side rail and pulled herself up the steps one leg at a time. She climbed all the stairs alone and became a shadow behind the bus’ tinted windows. Once aboard, she seemed to move quickly and gracefully to a seat. She never looked back.

She wanted to climb by herself. She could do it without us. We just needed to let her try. At least at some point, all of these children become aware of their vulnerability. They find this fragility, and leave it behind. It is not weakness. It becomes the road to their courage. 

Chemo cycles are every 28 days, so we end up at our routine clinic visit more than once some months.

Nico’s bone marrow appears “very robust” and even on the current increased chemo dosage, his blood counts are too high. The white blood cells (neutrophils) that form the basis of determining proper suppression are totally normal – normal for a child that is not on chemo! I know it sounds odd to say that Nico’s normal blood counts are worrisome, but we have been told that bone marrow suppression in the maintenance phase is key to treatment success. Nico’s bone marrow is not suppressed as it should be. So again, doctors are increasing his daily chemo dosage. I am giving the gist here. The above is the distilled version of a very long and involved conversation with our primary oncologist. 

With the increase dose comes more out-of-control behavior. There are extra layers here that make it difficult to navigate. Is he in pain? Is it general malaise? Is the lack of structure and routine of summer an adjustment for him? Is it chemo brain from the neurotoxic drugs? Or is it simply because he has Jeff and me for parents? All are plausible.

Nico’s oncology fellow suggested that in the summer, kids can experience an increase in drug metabolism with the increase in activity. He suggested that in winter Nico’s metabolism may slow down again, and his numbers might fall into place. I know our doctors want to reassure us, and we appreciate that. It feels like we are just riding out a storm.

Nico looks great. You would never know from looking at him what he endures. We incidentally discovered Nico had an ear infection and started antibiotics. A few days later he said, “I feel like someone took off my old ears and gave me brand new ones!” He just accepts discomfort and goes on his merry way. He is a handful, but we are grateful for everyday. We are glad to have our little guy, and if this is the road we have to take to give him a fair shot at life, we will take it.

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