Greasy Expectations

It’s 3 o’ clock in the morning. I’m in a deep sleep, far away. Floating on another planet. Lost, relaxed. My body is heavy and still. Suddenly… something stirs. I hear soft footsteps close by. My mind slowly travels back to earth as I start to hear the familiar sound of the fan in the bedroom. Then I feel a gentle hand on the covers. It’s Theo. He has to go pee. Now that I’m awake, I might as well go, too. We walk to the bathroom. Did he just say something? Is he talking to me? I hear words, but they are not registering. My brain is still half asleep. Again, he’s talking. “Enchiladas. Squash and zucchini. Papa’s marinated chicken.” Food. Is he dreaming? No. He’s wide awake. The steroids make it hard for him to sleep. We finish up and wash our hands. As I tuck Theo back into bed, he looks at me with hungry eyes and says, “Tomorrow, let’s make banana bread.”

This is not our first battle with steroids. The first time Theo was on steroids was in January 2020. Just after his initial leukemia diagnosis. I titled one of my early blog entries, “28 Days Later”, explaining in detail the challenges and hardships steroids caused. Then, in July, during the delayed intensification phase of treatment, Theo was back on steroids for the second time. That blog entry was titled, “Food Fight”. Again, more examples of how awful and destructive steroids are to Theo’s body, mind, and daily life. Theo was back on steroids again earlier this year for four months following his transplant to treat GVHD. Finishing steroids for the third time felt like the end of a world war. Our enemy had finally surrendered, and we claimed victory. We could start cleaning up the mess and destruction and put our lives back in order. It was time for reconstruction, repair, and healing. Looking ahead to a brighter future while putting the pain behind us.

Then GVHD came back with a vengeance. In my last update, Theo was currently taking 30mg of prednisone (1 mg per kg of weight) per day. For the past six weeks (since I last wrote) his liver enzymes (ALT) have been going up and down like a yo-yo. Our weekly visits to Seattle Children’s Hospital turned into twice weekly to check labs and liver inflammation. We were not moving in the direction we wanted. On Thursday, July 20th, Theo’s ALT dropped slightly from the 600s into the 400s after the increase in prednisone to 30mg. With the improvement in inflammation, Theo’s doctor felt it would be safe to start an antifungal medication called, Posaconazole. It has a reputation for causing liver irritation, but with the increased steroid dose, there was a chance Theo’s liver could handle it. On Monday, July 24th, we went back to the clinic for a blood test. It wasn’t good. The ALT was back UP! Theo could not continue taking Posaconazole. The only anti-fungal medication that doesn’t irritate the liver is Micafungin, which is only available in IV form. The following Friday, July 28th, Theo had a peripherally inserted central catheter (PICC) line surgically placed in his right arm. And just like that, we were back to daily flushing of the line, daily infusions of Micafungin at home, no baths – only showers with aqua guard, and carrying medical supplies with us everywhere we go. Not again! I’ll be honest. I was completely agitated and fearful about what was happening but doing my best to not let it show. As usual, Theo kept a positive attitude. He kept reminding us over and over, “this is only temporary”. Surely the liver would recover soon… right?!

To our huge disappointment, the liver was not improving at all! On Monday, July 31st, Theo’s ALTs were back up to 583. Maybe due to steroid refractory GVHD? Theo’s doctor decided to double the steroid dose. That’s 60mg of prednisone a day. HOLY COW that’s a LOT of steroids. It was obvious how serious this was. I could see the concern on her face. Her voice was more pressing. The expression on my face as I listened to her talk must have revealed how worried I was. My nails dug into my thighs, and I was barely breathing. She immediately assured us that what was happening was still completely within their standard of practice. They have treated many kids like Theo with severe GVHD. Some who end up in the ICU with far worse and intense symptoms. Theo was nowhere near that, and she planned to keep it that way. This was going to be resolved. We needed to hear that and BREATHE. Theo’s providers had a plan. They always do. It was finally time for a liver biopsy. Every approach to reduce the liver inflammation had been exhausted and without success. Every card had been played. Time to fold and get out the big guns. We needed to find out if there was some other component contributing to liver inflammation other than GVHD. The liver biopsy was scheduled for the following Friday, August 4th, and fasting labs were done the next day, Tuesday, August 1st, to check cholesterol levels. No more messing around. It was time to go straight to the source to find out what was going on. Hopefully we’d get some answers.

The results from the fasting labs came back right away. We discussed the results with one of Theo’s providers. She told us that Theo’s cholesterol was very high. Not the bad cholesterol, but the good one, which is very unusual. Initially, there was concern for hepatic steatosis. That’s when there is excess build-up of fat in the liver, aka “fatty liver disease”. Was Theo’s liver storing fat? If it was, that is very concerning. I received the following message in MyChart from one of Theo’s providers, “Theo’s fasting cholesterol panel was quite high with a total cholesterol of greater than 300 and LDL cholesterol of 177, so there seems a good/reasonable chance that fatty liver is contributing to the inflammation”. The feeling of angst came flooding over my body again. Like a tide, it never stays low, always rising again. It wasn’t very clear what was causing the cholesterol to be so high. But clearly it needed to be treated regardless. His liver ultrasound did not show anything abnormal. His bilirubin levels were also normal. Hepatology thought it might be a lab error. We were so confused! I had so many questions that I knew had no answer… yet. What is causing the liver inflammation? Why is Theo’s cholesterol so high? Is he storing fat in his liver? Should we be making changes to Theo’s diet? Why am I so tense? There was this burning desire growing inside me to have answers. Like an itch that needed to be scratched. Patience. The answers will come.

Back so soon? The nurses at Interventional Radiology (surgical center at Children’s Hospital) were shocked to see us back just one week after the PICC line placement. On August 4th, the liver biopsy was done, and it was pretty straight forward. Although the procedure was quick, recovery takes much longer. Theo had to stay lying flat in bed for four hours post-op to not disrupt liver healing. After recovery, we went home and eagerly waited for the results… and waited… and waited. We were back the next Monday, August 7th, for another blood draw and provider visit. Still waiting for the biopsy results. Theo’s ALT had dropped, but very little. Not as much as we had hoped since the big increase in prednisone. Why was the liver still so inflamed?? There had to be something else going on. In the meantime, we learned of a new drug called Ruxolitnib. It was approved by the FDA to treat GVHD two years ago. Wait, has this been an option all this time? I thought steroids were the ONLY drug to treat GVHD!! Well, I’ve recently learned that steroids are always the first “go to” drug of choice for treating GVHD. Ruxolitnib has its own list of unwanted side effects as well. It lowers blood counts and puts patients at higher risk for viral and bacterial infections. But it could also be used as a steroid sparing agent. Since the steroids were not working to bring down the liver inflammation, we had to try something else. If it worked to treat the liver GVHD, we could get Theo off the steroids sooner. YES PLEASE.

I finally heard form one of Theo’s providers on Thursday, August 10th about the liver biopsy. Where do I begin? I’ll try to explain this as simply as possible. There was evidence that Theo’s ALTs may be elevated from a combination of two things. First, the biopsy did show changes to the liver that are consistent with GVHD. Ok, that’s good. Just as the doctors suspected. Second, they also saw changes to the liver cells that suggest possible steroid induced effects on the liver. Ah HA!! The steroids are contributing to the liver inflammation! And what about the cholesterol? A message I received from one of the providers states, “On review with hepatology, they do not believe that Theo’s high cholesterol is due to liver dysfunction, however, it very well could be due to the medication effects of the steroids.” Jiminy Cricket. It was those dang steroids all along. The good news is that it doesn’t appear that any issue was arising from Theo’s liver itself. In other words, Theo’s liver function is healthy and normal. ALL GOOD NEWS. So, we are to continue treating the liver GVHD with the steroid sparing agent, Ruxolitnib, as recently planned. And that’s’ exactly what we’ve been doing, since August 15th, when it arrived from the specialty pharmacy. Guess what? IT WORKS!!! Finally, after months of twice weekly labs and a biopsy, and worry beyond belief, Theo’s liver is NOT inflamed anymore. On Monday, August 28th, Theo’s ALT was down below 100, in normal range. Hallelujah. I think I can relax a little now and enjoy the low tide.

To say that we were disappointed when Theo had to go back on steroids is an incredible understatement. It was devastating, crushing, and absolute heartbreak. It’s different this time around. Very different. When Theo was on steroids before, it was during times when he was at risk of being underweight because of chemotherapy. It was almost a relief to see him eat so much because that meant he didn’t need a feeding tube for nutrition. He also had sufficient time for his body to recover between steroid dosing. Not this time. Theo started this round of steroids at a huge disadvantage. He is swimming against the current. His body has barely had enough time to recover since ending steroids in May. So now, instead of starting from a position of health and strength, he is starting from a position of weakness. Everything, from his weight, fluid balance, bones, muscles, and organs, LIVER, have suffered from the long-term steroid use over the years and are therefore compromised and the damage will be greater this time around. Therefore, we have to be extra careful monitoring his organs and managing his diet. We know Theo will gain weight. It’s inevitable! But if he gains too much, too fast, it can cause more problems. After we found out about Theo’s high cholesterol, we started making more strict changes to Theo’s diet. No chips, no red meat, no fried food. No buttery green beans. At first, it felt like taking a pacifier away from a baby. Steroids make him crave salty fatty foods! Theo wants to make healthy choices, but he is not always the one in control. He has been in so much distress over food. Theo is a FOOD LOVER. The problem is not trying to get him to eat healthy food. It’s helping him not eat TOO much food all day long. He eats plates upon plates of vegetables, green smoothies, fresh beet, celery and carrot juice, fish, salmon, squid, sardines, artichoke with olive oil… you name it! His new favorite? Chicken hearts. Healthy protein without the fat. He loves all the organ meat just like his Papa. But his brain is locked. He is completely obsessed with what he is going to eat and when he is going to eat it. We cannot go into the grocery store, or talk about food, or walk by a restaurant, without him breaking down because he wants to eat EVERYTHING!!! We cannot eat food in front of him without him drooling like a starved street dog begging for a bite.

Food, once a topic of joy and love, has turned into stress and frustration. For all of us. After spending all morning cooking then cleaning, then cooking some more and cleaning, and cooking again, Luis was ready to take a break from the kitchen. Theo on the other hand, was still hungry and wanted something else to eat. Luis loves making food for Theo. It was Papa and Theo Day. He was not planning to spend his entire day cooking for Theo but that’s what it had turned into. Although tired of cooking and cleaning, he started listing options and ideas for food he could make. Delicious healthy snacks he knew Theo would enjoy. But Theo could not decide. He didn’t know what he wanted, and it made him beyond frustrated. Theo NEVER raises his voice at Luis. He wouldn’t dare. And that’s just not who Theo is. He does not yell. But in that moment, Theo was fuming. He marched into the kitchen, red in the face, fists clenched tightly, and exploded in anger, “Too many steroids! Too many foods! Too many things in my head!!” The anxiety has become overwhelming for Theo. He understands that the steroids are to blame. That this is only temporary. He is trying SO HARD to not let the steroids take over… but he shouldn’t have to. He shouldn’t have this weight on his shoulders. To deal with this right now, or ever.

So, what do we do? We take it one meal, one moment, one bite at a time. We go outside for a break. Smell the fresh air, listen to the birds, or take a nature walk. There’s always something new to discover and enjoy outside. We talk about our struggles and frustrations openly. We listen. We remind each other to breathe. We do our BEST. And lastly, we remind ourselves that this is temporary!! This is not normal. We will get through this like so many other hard things we’ve been through. And when Theo needs another distraction? Reading and Art. The perfect distraction for him. If Theo is not eating, he is reading or drawing or painting. He reads books like he eats popcorn: daily and fast. He can sit for hours working on a project and not think or talk about food. Theo puts on his headphones, turns up the music, and starts creating. He is in the zone. Ideas flowing, music jamming, and then he is the one floating on another planet, lost and relaxed. No stress, no cravings, no breakdowns. Just a boy in his own world.

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“I think I may paint a family portrait today… that is, if I have enough paint left for all the smiles.” – Paddington Bear