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Wild Type

Jan 26, 2017 (Scott)

During times like these it’s astounding how much one can learn in a day. The daily drug intended to suppress Maya’s marrow and keep her ANC between 750 and 1500, 6-Mercaptopurine (6mp), is metabolized by Thiopurine methyltransferase (TPMT) resulting in two metabolites: 6MMP and 6TG. And that’s more than I’ve ever known about metabolism (you’re lucky Sara isn’t writing this post).

6TG is the good stuff–the product that does the suppressive work; 6MMP is a bi-product, benign in small enough quantity and usually ignored. Rarely, patients taking 6MP (for leukemia treatment or IBD) will show signs of hepatotoxicity (angry liver), and rarely this is caused by unique effects of a patient’s TPMT on 6MP. This can create an imbalance in the breakdown of 6MP resulting in low levels of 6TG and high levels (10 times higher than normal) of 6MMP. This means the ANC is not sufficiently suppressed, and there is a toxic level of 6MMP present in the blood. Last week Maya’s labs included a test for these metabolites, and they were significantly imbalanced.
Now a break from the science lesson… over the past 7 days, Maya has bounced back with authority. My new mindset on the care giver’s role is to recognize that healthy kids will gravitate toward being healthy, and chemo kids will gravitate toward complications–if we’re not extremely attentive. Bowel care, hydration, rest, good nutrition, and love are critical components of a successful therapy; balance and consistency are key. I’m happy to say over the past week as Maya has been off chemo, she has gravitated convincingly toward health and strength. Her energy level, mood, humor, color, appetite, and myriad bodily functions have returned to a welcome balance. She’s been the normal energetic 4 year old and more.  Though, she is unique in many ways… more on that later.
We spent the first part of Sunday shoveling snow and sledding in the side yard. Maya built a snow castle, threw snow balls, ran up the hill carrying her sled over and over, and never showed signs of slowing down until we were eating lunch hours later. Even then she didn’t need a nap and continued on through the day with her natural curiousness and creative energy flowing.
Since last Wednesday she’s steadily improved. It took her a couple days to clear the jaundice; she began sleeping better without nausea or hypoglycemic symptoms. Her constipation seems to be under control, though this requires an unsettling amount of miralax and colace each day… we do what it takes to avoid the recent struggles in that department.
Taking a week off from treatment to recover is a scary thing, but I still think we made the right decision. Maya’s recovery has been substantial, and there were no concerns with respect to her strength beginning phase 2 of maintenance today.
There were, however, still concerns about what exactly got us into trouble in the first place and what to do in order to safely continue treatment. Likely it’s a combination of things, but as I mentioned earlier Maya’s metabolites related to 6MP were way out of balance. We hoped for answers this morning, and I think we’re confident we’re on the right track.
The metabolite imbalance points to a rare genetic anomaly in the way TPMT (the thing that breaks down 6MP into 6MMP and 6TG) is produced or operates in the body. Most people have the TPMT ‘wild-type’ genotype; fewer have the heterozygous genotype, and even fewer have the homozygous genotype. Both the heterozygous and homozygous genotypes lead to increased to extreme sensitivity to 6MP, potentially overproducing metabolites causing hepatotoxcitity (too much 6MMP) or extreme myelosuppression (bone marrow suppression from too much 6TG) which can bottom out immunity and worse. We should know in a few weeks which genotype is Maya’s and take extreme care as we proceed.
The tactic to combat this and get back to a healthy balance is not on the standard protocol, but it is well known among pediatric oncologists. Case studies have been published in recent years indicating efficacy of introducing allopurinol in these rare cases to shift the metabolic pathway with TPMT away from 6MMP and toward 6TG. Since there is the possibility that Maya is extremely sensitive to these metabolites, and the point is to moderately suppress her ANC, we’ll start back with just 20% of the previous dose of 6MP. We’ll also check labs weekly until we find that comfortable balance for our rare, unique, extreme, amazingly strong little dragon.
This continues to be a very stressful time. Uncertainty is the worst. But we have a plan, and we’re as focused as ever. Maya is a unique little creature–more unique all the time it seems. We’ve seen over the past week that her strength remains, and her excitement for life has not been diminished.
Thank you all for your support, love, and understanding. May we all have the energy to strive for and achieve our balance.

Choices and Paths

January 18th, 2017 (Scott)

For the first time in a while Maya had a solid night’s sleep that didn’t end with nausea and vomit at 6 am. She woke up feeling good having missed her first dose of oral chemo in 3 months. Her appetite remains strong, and her desire to get off the couch and back into play and activity grew by the hour. Even her sense of humor and wonderment started to re-emerge.

All of this is what we needed to see–at least it affirmed the decision we’d made to hold chemo therapy for a week. We’ve faced hard decisions all along, but it seems like we’ve always found our comfort zone and moved forward with confidence. In this one we know we’ve split off from the main stream of the treatment protocol and created a new path–probably not far off from the old one, and likely arriving in the same place… Probably…. Likely…. And there’s my problem.

You can only treat so much. Maybe successful chemo therapy is really a partnership between strength and poison. When the balance strays too far from center, there is a choice to reset the system. Dr. Zucker said, “It’s my job to break her down–it’s how we save her life… But I don’t want to actually ‘break’ her.”

This week is our reset–our new path begins with 7 chemo-less days, and it’s our job to ensure every one of them is a effective as it can be–to help restore Maya’s strength, flush out the toxins, and set her up to jump back into the fray with full Dragon-force.

I guess we never know with decisions like this or any of the others along the way… Get her to the doctor, get the blood tested, drive to Oakland, grit your teeth and hang on with every ounce of strength. We trust our team, we trust our instincts, and we trust Maya to show us what she needs. We gathered all the info we could, used care and counsel, and we did not hesitate. I suppose in the absence of immediate results we may rely on our process as evidence that we did the right thing. There is only to go forward on our path and do all we can to make it the right one.

In one week phase 2 of maintenance will start. 5 days of steroids (joy!), infusion of vincristine, oral methotrexate, lumbar puncture, and a return to 6mp. Right back into the eye of the storm and hoping to find that peaceful balance.

Thank you for your love and strength. Together we’ll get the balance back and our little Dragon centered again ready for the next challenge.

Metabolites and tolerance

January 17, 2017  (Sara) 

We went to see Dr. Zucker today and we are grateful we didn’t need to get admitted. Dr. Z could tell Maya’s coloring was off at first sight and he “guessed” we’d be halting the 6MP for a little while. Maya’s labs confirmed his suspicion. It appears she is may not be metabolizing the 6MP (the daily oral chemo). Of course they tell you in the beginning “there is a rare chance of …this or that” and it appears that Maya might be proving her rareness in this not-so-awesome way.

Her liver enzymes are elevated but not near as elevated as I had feared (phew!). This means that while her liver is “angry” as Dr. Zucker puts it….it is still working. I did fear an elevated bilirubin that was confirmed today at 5.5 (normal maxes out at 0.8). Direct Bili is at 3.8 which indicates a halt in the 6MP. He tested for uric acid (normal). Kidney function looks great. Hydration looked good too….we’ll take those small wins! He also is testing for certain “metabolites”. We all have incredible enzymes that help us metabolize everything and sometimes if we are missing a specific enzyme it can get in the way of detoxing. Some of these enzymes could be depleted and we would never know…unless we were on chemo. That is what we might be looking at now. A possible deficiency in a specific enzyme that would normally not be a cause of concern unless heavily medicated with a very specific medication (i.e. 6MP). There is a chance we might need to deviate from 6MP all together but I will wait to hear the doctor’s insight before I delve into that issue too much. I did see that one of the tests looked high on our print out of labs but it might not be “too high”. I don’t know as it is not a typical lab value familiar to a dietitian. I don’t want to freak out over things I don’t know about….. Blah. I wish that was possible.
Even with good hydration, we still got some IV fluids for her while she had her budda button accessed. She slept for most of the appointment and Dr. Zucker noticed she was quite different than when he met the spunky and interactive Maya the first time. We are hoping the hydration will perk her up a bit. Her ANC is 3300…ironically this is pretty scary since this phase is supposed to keep her immunity at a consistent suppression. This is the third month with a high ANC. This only increases the risk of relapse since leukemia can mutate and then take over so quickly. IF she perks up, we can go ahead with her IV vincristine to knock down her ANC as soon as possible. If she doesn’t…we’ll have to wait. And as Dr. Z said “we can’t treat a sick child”. And she is sick. I can tell. I imagine the chemo is solely responsible but there could be a virus at fault too. We don’t know.
Our job is to keep her pooping, eating, and drinking. And we wait. Waiting sucks. But we wait together. I’m so thankful to have such a patient and loving person at my side. Scott makes this so much more easy than it would be without him. His natural patience is inspiring and tangible. I’m not saying he isn’t worried. He is. He is to his core but he has a loving way of dealing with his anxiety. And dealing with me.
We will keep you updated. Thank you again for all the love, prayers, support, light….everything.
Love is Life.

Trials and the unknown

January 16, 2017 (Sara) 

I’m writing tonight with hopes of rallying some extra prayers, healing light, love, and some dragon’s strength for Maya. I continue to try and see…feel… “the easy part” of Maintenance that the doctors and nurses all have alluded to over the past 11 months. Yet here I am writing with fear tonight in the third month of Maintenance; we are due to close the first 84 day cycle of Maintenance tomorrow and start with day 1 of the second cycle Wednesday. This is pending now since we actually need to go see the doctor tomorrow. Though the new hematologist/oncologist, Dr. Zucker, has been kept abreast of Maya’s situation and he likely has been scouring her chart, tomorrow will be our first day as his patient. I wish it was for a less eager appointment.
Scott wrote a necessary post about our trials lately and I (once again) apologize for keeping quiet when I know people want to stay up to date. It is difficult when people ask “how is Maya doing?” and I can’t say “oh you know, she’s doing really well, thank you”. Truth is, Maya has not been great the last couple of weeks. She has been extremely constipated with very light stools that need assistance with passing. The poor child has had poop pulled from her bum too many times and now a fissure is contributing to so much pain…and withholding…cuz she is four. I’m sorry if that is too much information but it had to be said. It was traumatizing for all involved and I am certain I cannot drink enough scotch to make that memory fade. Not just the action of pulling matter from a bum…but the yelling, the coaxing, the begging, the crying, the threatening, the pleading, the whispering, the trauma. I hope she, as well as little Lincoln, don’t remember this part clearly for it is painful physically and emotionally. But we love her with every cell and will do anything to make her feel better.

While her constipation has been her most significant ailment, she has also been quite lethargic, nauseous each morning, some vomiting, belly pain and today… we see yellow. Mostly in her eyes, under the tongue, and even her skin a bit.  I know 6mp and vincristine can deplete liver function but this is really freaking us out. I can handle the laundry every morning from vomit soaked towels. I can handle looking at her on the couch all day. I can give her tylenol for her pain. I cannot handle seeing jaundice. I just can’t because I know the implications too well.
I have heard other cancer families (on Facebook blogs) talk about how the possibility of holding meds/chemo due to liver enzymes being too elevated. I am guessing that might be in our deck of cards. Dr. Salo said that (for whatever reasons) it is very common for the child to “crash” the first three months of Maintenance. Scott and I had no idea it would be in this way since usually her ANC is the most stress ridden lab value. It seems like with each month the symptoms of roid rage and appetite from Decadron come on quicker and linger longer and now with the jaundice creeping in….I am terrified of permanent damage to her organs.  I know the liver is an incredible and resilient organ but…well it’s hard for me to “see clearly” these days.  It is hard to imagine our stress level being higher than it was during induction but both Scott and I are feeling like we are swimming in anxiety. Even DI was more smooth than this and we injected chemo in our own child. Prior to Maintenance she was actually happy most days. Now…I miss her genuine smile with all my heart.

Motherly Assessment: I’m scared. She’s not terribly yellow but the corners of her eyes are no longer the straight white I am used to looking at. She is finally pooping thanks to the aggressive bowel care of Miralax twice a day, colace once a day, 5 oz of prune juice, aloe vera juice, high fiber diet and at least 35 oz of water. She feels pretty crappy. I’m not a doctor. I am also trying not to be a clinician since this scares the shit out of me. But….I’m guessing her liver is “mucked” up and her bile is not excreting enough to help with stooling. Our bile help make our poop brown and softer. Because of the shift between light and brown stool I’m also guessing it isn’t permanent damage yet. Tomorrow we will get to see labs. We will look to see if her liver enzymes are elevated and her bilirubin is high. If they are high enough, a halt on chemo will be initiated for a pulse of time.
I know I am strong but right now I am also a very stressed, fearful, and almost broken mommy. Thank you for the letting me vent and I truly apologize that any anxiety that might seep into other lives. I have such dear, close, and soul connected friends/family that I know you feel it too…

Love is life…


January 14, 2017 (Scott) 

Maintenance is supposed to be the easy part. It’s described that way in comparison to the other phases of treatment. Things are supposed to be as close to normal as can be during these 18 months. But these are the things we are told by the people who haven’t lived the process day by day with a child who may or may not feel crummy and who may or may not have the vocabulary nor the confidence to tell us how things really are.

The past 10 days have been rough, and not for the reasons most people might expect. In fact, I have doubts as I write this that I should even put it out there, since the thing that’s weighing on us so heavily is Maya’s constipation.
Maya didn’t go for 6 days. Each day that passed increased our anxiety and the aggressiveness of the bowel care (laxative) doses we gave. Each day increased Maya’s discomfort and her fear that pushing it out would hurt. By day 6 we pushed each other to the breaking point–she finally produced, and she bled. We all cried and made promises to do all we can to never reach that place again.
We spent a good portion of our Saturday again fighting the same forces–physics and fear. After hours of stubbornness and screaming, and after her strength was worn down even more (and I was questioning my own), our little dragon was strong and calm enough to push. It was very uncomfortable, traumatic in many ways, and it isn’t supposed to be like this. Maintenance is the easy part. No pressure.
We’ve been in contact with our new oncologist with updates and questions. He’s kind and makes himself available to us night and day by email and phone. I’m pretty sure Sara texted him a picture of some poop this evening–I made sure she sent a warning first since it isn’t polite to surprise someone that way.
We have some concerns about the effects of 6-mp and methotrexate on Maya’s liver enzymes (I’ll leave it at that… Sara’s motherly assessment would undoubtedly have more detail). There are worrisome symptoms in the constipation and other details (of which I promise not to show pictures). On the bright side, Maya is not turning yellow, and she obviously feels much better having cleared out her belly tonight. There’s more to do, but we made significant progress. She’s sleeping soundly next to me now, and just before bed she said, ‘You’re the best mommy and daddy ever. Thanks for taking such good care of me.’
These few days have taken us back to the stress of induction. I talked Maya through her fear of needles and procedures then; we had our moments, but we overcame challenges together because she never let fear take control. Surprising that something as simple as constipation has thrown us so far off the routine. As one oncologist I spoke to on the phone mentioned, ‘constipation is no joke.’ When I think back on the sleeplessness, lethargy, vomiting, radiating sciatic pain, fear, and what it took for all of us to get a little forward progress it is clear this is one of the toughest challenges we’ve faced since our time in Oakland. No joke.
Thank you all for being there for us, and for reminding us we’re not alone. Being a parent is hard. It’s also amazing like nothing else in this world that I know of. We’re blessed to have the chance to play this role in a life, and we’re blessed to have the support of so many strong, loving people.
Thank you.