The Bread and Butter

“De el plato a la boca se cae la sopa”

Luis never fails to have a saying to go along with something happening in the world. I have lost count of how many times I have heard him say, “Like we say in Mexico…” He has the ability to sum up the absurdity and complexity of something to then somehow make sense of it all in one simple phrase. He uses metaphors like it’s an art form. Placing them cleverly and casually in his language every day. So, it did not surprise me at all to hear him say the above phrase recently. It translates: from the plate to the mouth the soup falls. In other words, you can plan all you want, but life happens, and plans fall apart. You spend all this time preparing that soup. So much care and planning and just when you are about to taste it… it spills!

Why did Luis say that? One answer: Graft Vs Host Disease, aka GVHD. It’s back and it’s angry and ugly. Theo’s new immune cells are attacking his body again. Just FOUR days away from finishing his immunosuppressant (Tacrolimus). FOUR days away from being able to swim. Days away from adventures, travel, and time with friends. Luis and I had already started planning weekends, get togethers, and some summer fun, knowing Theo would have a stronger immune system. Luis and I even got excited at the thought of the two of us going on a date… finally!! One thing we were extremely optimistic about was the increasing chance of Theo being ready to return to in person school starting in the fall. As long as Theo is off his immunosuppressants he is cleared to go back to school. Just weeks ago, we were planning for vaccinations with his doctor. So yeah, you could say the soup spilled just before we were about to taste it.

Now, Theo will have to go back to the full dose of steroids (Prednisone) AND Tacrolimus and start the process of tapering them down all over again. Why? First, steroids stop Theo’s cells from attacking him. GVHD is like an engine revved up, driving a car towards the edge of a cliff. Steroids are the breaks. Tacrolimus is so that when we are ready to take the foot off the break, the car doesn’t take off at full speed again. Tacrolimus ensures that when the steroids start to taper down, the immune cells do not wake up too quickly or with too much force. Theo’s immune cells need a gentle steady start. They will eventually recognize Theo’s body and stop attacking it… eventually. The doctors say it can take up to one full year. Until then, it’s back to steroids, the frenemy. They’re great, because they treat GVHD. In fact, steroids are the ONLY thing that treat GVHD. But there’s also a downside. I think we all know why steroids are horrible. Weight gain, weakening muscles, water retention, calcium malabsorption in the bones, mood swings, adrenal gland shut down and more. But it’s what we have and what Theo needs. GVHD, I’m told, is the “bread and butter” of bone marrow transplant recovery. Meaning, all the medications and treatment revolve around the presence or absence of GVHD. When you have it, you get ALL the medications for immune support: anti-viral, anti-fungal, antacid, topical steroid ointment and creams, extra calcium, extra vitamins, etc. When you don’t have GVHD, it’s tapering off those meds and then just recovery! It’s all related and dependent on GVHD. Obviously, every patient is different. Different bodies, donors, type of transplant and all. Some patients may get only one mild case of GVHD and nothing more. Others may get it several times at different intensities throughout recovery. Theo’s doctor said, “We expect patients to get GVHD. If they don’t, it’s a pleasant surprise”. So, this is normal, it is expected, and most importantly… the doctors know what to do.

Before I go any further and tell you how it all happened, let me make one thig clear. No one knows how or when it started. GVHD is not at all obvious or easy to identify. It can be very tricky and take weeks or months to reveal itself. There is no swab, blood test, or scan that can find it. Biopsies can be done but are invasive and therefore not recommended unless absolutely necessary. It takes a very experienced BMT doctor to look closely at skin changes, to track the blood trends, and the symptoms and changes of the patient to determine if what is going on is truly GVHD. So there really is no “beginning” to this. Just the beginning of unusual things happening. Are these symptoms or events related? Is there cause and effect? Maybe. But we’ll never know.

Now, let me take you back about one month ago when those “unusual things” started to happen. The week of June 11th (one week before his birthday) Theo started feeling itchy on his arms and face. He developed a couple of hives on his cheek and arm that quickly went away with some topical hydrocortisone ointment. On Monday, June 12th, after taking a warm bath, his skin became red, inflamed and itched all over. He was in tears because he said it felt like his skin burned everywhere. Extremely unusual. On Sunday, June 18th (Father’s Day), we took a morning walk along the beach. The tide was waaaaaaaay out which made it fun to search for sea creatures and star fish. When we got home we all washed the sand and salt water off our feet (Theo was wearing crocs). Theo’s feet immediately started burning. They were red and itchy with white bumps covering the bottom of his toes and feet. We treated his feet with hydrocortisone and sent pictures to his team. Throughout the week, Theo began coughing more often. It originally started off as clearing his throat in the morning and getting out junk that had been determined to be the result of seasonal allergies and post nasal drip. His temperatures, normally around 97.8 degrees Fahrenheit, were reading closer to 99.0, almost in the yellow zone! Something was definitely going on. On June 21st, Theo tested positive for adenovirus. But could that explain everything? The burning feet and rashes? Maybe. His doctor also detected a spotted rash on the hard palate of his mouth. More alarming, however, was the condition of his liver. ALTs are enzymes made by the liver to help it break down proteins. The ALT level is checked with every blood draw. Theo’s ALTs were very high, in the mid 200s, a sign of inflammation. In Theo’s after visit summary notes, his doctor wrote, “we suspect this could be viral but as always, this could be GVHD. To find out, more testing needs to be done.” The next day Theo had a liver ultrasound to check liver function. He also had a chest xray and more blood drawn to check for other viruses. The liver, although inflamed, showed good function from the ultrasound. The chest xray revealed an upper respiratory infection. Nothing else. Theo’s rashes were not looking like “true” GVHD rashes. We hoped that the virus and his symptoms would clear up quickly and that would be the end of it.

By June 26th the rashes were getting worse and his cheeks were tomato red! He needed Benadryl at night because his feet were itching and burning so badly. We were instructed to continue with topical steroid ointment (Triamcinalone or Lidex – a stonger steroid – for the body, hydrocortisone for the face) twice a day and hopefully they would resolve once the virus ran its course. Could this be GVHD? Again, we discussed that possibility with Theo’s team. If it was, we were told it would eventually reveal itself. Unfortunately, without any obvious proof at that point of it being GVHD, the best thing to do was continue as if it was viral and continue applying topical steroids. We were now going on weeks of twice a day ointment. Guess what? We are STILL applying ointment today! You might recall that Theo despises having ointment put on his skin. He loathes it!! Initially it was a struggle. As soon as he heard me say the word, “triamcinalone” he became agitated and in complete dismay. It was the very rare time in his life that I ever saw him this way. He is allowed to be upset, of course! He is allowed to get frustrated and angry. He has more than enough good reasons to be. It’s good for him to be able to release those feelings. And he did. He would huff and grunt, and sometimes cry. And then… he’d be over it and apologize. Now, it doesn’t bother him at all. He got it off his chest and moved on. Wise beyond his years.

The symptoms were not getting better at all… in fact, they were getting much worse! He was red all over. Theo woke up coughing so hard and so long, he often did not have energy to get out of bed until the afternoon. Most mornings he coughed to the point of throwing up. Gagging on sticky mucus that was caught his throat. The first week of July was the worst. Extremely low energy, non-stop coughing, no appetite, labored breathing, and high temperatures into the yellow zone. On the morning of July 5th, we noticed that Theo was having more severe labored breathing. Instead of his chest rising up and down, his sternum was retracting, and his belly was pushing out. He complained of being short of breath and unable to take a full breath in. He described it by saying, “You know how when you yawn, you have to take a deep breath? Well, I cannot do that.” His breathing was very shallow and quick. We rushed to the clinic that afternoon so someone on his team could take a closer look. A new chest xray did not show anything more than the already detected upper respiratory infection. But his oxygen saturation was low at 94%. Anything lower than that and Theo would have to be admitted to the hospital to receive oxygen support. Thankfully, he was ok to go home… my parent’s home that is. His doctors wanted Theo to stay close to the hospital, in Seattle, in case anything changed or got worse. He started using a steroid inhaler twice a day. I was also instructed to give Theo stress doses of hydrocortisone for the next three days. Boy did that do the trick! Like magic, Theo woke up the next day with energy! For the first time in over a week he woke up and did NOT throw up from a violent coughing fit. He hopped out of bed and with bright smiling eyes said, “I’m hungry. What’s for breakfast?” Our boy was back! Just in time to perform in the piano recital that following weekend.

At Theo’s next clinic visit with his BMT doctor, GVHD was no longer in question. It had declared itself. She first looked all over his body. It was clear that the steroid ointments were not enough. Theo’s rash had progressed to the palms of his hands. But it wasn’t until she looked inside Theo’s mouth that she declared it to be GVHD. The rashes on his body, she said were “atypical” but the rash in his mouth was very on point with being GVHD. Theo’s doctor wrote, “the constellation of findings is consistent with chronic GVHD. His eosinophils are mildly elevated which also suggest GVHD”. If left untreated, chronic GVHD can lead to autoimmune disorders and more problems later in life. Even more concerning was Theo’s liver inflammation. Theo’s doctor referred to it as “impressive acute transiminitis”. Meaning, his ALT had quickly gone way up into the 400s!! Just a couple weeks prior it was in the 200s. Normal range is below 100. On that Monday, July 10th, Theo started taking steroids. She decided to start with a lower dose (half) and see if that would be enough to stop GVHD and reduce the liver inflammation. Theo’s Tacrolimus dose also went back up to 1ml twice daily. The news was crushing. Steroids… again!? Okay, at least it was a low dose. Maybe it wouldn’t be that bad. We went back to clinic a few days later on July 14th to check Theo’s progress. The rash had started to show improvement, but Theo’s mouth had developed more hyperkeratotic features at the corners of his lips. Not a good sign. His ALT had barely budged. We left the clinic with our fingers crossed that on the following Monday there would be some significant improvement.

On July 17th, Theo and I walked into the clinic feeling hopeful and ready to hear some good news. I was certain that the recent start of steroids had done the trick and was excited to discuss when we would start tapering him off. Then, two physician assistants, a nurse practitioner, and Theo’s doctor walked into our room. Uh oh, this can’t be good. Why are there so many providers from Theo’s team in the room with us? Usually, it’s just us and the PA or the PA and doctor. I was not prepared to hear the update they were about to give us. Theo’s ALTs were in the high 600s!!!! What?!?! But how? NO ONE was expecting this. Theo’s treatment plan was severely altered. His steroid dose was increased to 30mg a day (one mg per kg of weight, aka FULL DOSE). An antacid was added to help prevent GERD caused by steroids. His tacrolimus dose was also increased. A topical liquid steroid called Beclomethasone was added, to be taken four times a day. A higher dose of steroids and immune suppression also means that Theo needs to be back on an anti-fungal medication. You see? Having GVHD means that ALL the other medications come with it. It felt like we were moving backwards. Are we back where we were six months ago? Do we have to do this all over again? It felt like I had just been mugged in broad daylight! If Theo’s liver did not improve after the increase in steroid dose, it would need a biopsy. Theo was also at risk of needing a PICC line placement. Basically, a permanent IV line placed in his arm to administer IV medications at home. My eyes started swelling up as I listened to the plan and possible outcomes. It was hard to focus and not think about how all OUR family plans were being shattered and Theo’s body would become so fragile again. After making so much progress and looking forward to doing so many things. Theo’s immune system will be severely compromised again. We will need to go back to strict precautions and always be within 30 minutes of an ER. The only thing that kept me strong in that room was Theo sitting on my lap, my arms wrapped around him tightly. His sweet soul reminding me to stay positive and that everything will be ok.

By Thursday, July 20th, the ALT had dropped back down into the 400s. OH PHEW!! What a relief. Theo has been referred to see the hepatologist (liver specialist) in the meantime to have a closer look. For now, he will continue with the 30mg dose of steroids along with the other meds until further notice. Presently, Theo is feeling good. He has lots of that strong steroid energy. His cough is almost gone. The rashes on his body are not as red, more like hyperpigmentation of his skin. Much better. His appetite? You guessed it. Here we go again with the steroid hunger. It always takes over and that makes things extra tough. Combatting the negative effects of the steroids will be even harder this time around. Theo only had about two months of being off steroids to get his weight back down (mostly from water retention) and strength back up. He never got a chance to fully recover and now he’s back on them. Keeping muscle mass and not gaining a lot of weight is very important for his long-term health, but it’s very challenging!! Luis and I have had several conversations with Theo about making healthy food choices even when the steroids don’t want him to. To try to ignore those greasy salty fatty cravings as much as possible. Theo knows it won’t be easy, but he is motivated! He is determined! He is our inspiration!! He has already put together a plan for staying strong and healthy while on steroids. He has a list of healthy foods that he loves and can eat in large portions whenever he wants, like zucchini, fish, quinoa, beans, broccoli, and soups. He even created The Family Strength Challenge: every day we do 20 squats, 20 stand ups (from a stepping stool) with hands tied behind our back, high knees for one minute, mountain climbers for one minute, and plank for one minute. Truly, no one has a better attitude about all of this than Theo. NO ONE. I thought he was going to be crushed after finding out he could not go swimming. Boy was I wrong. He simply shrugged and said, “That’s ok. I’ll just have to wait a little longer.” Luis and I are always so impressed with how hard he works and how positive he is. We both know that feeling down and focusing on the negative will only make things worse… so we decided to follow Theo’s lead. Don’t worry, be happy. Thank you, Theo.

“If you’re in a happy mood, you will pass it on to others.” -Theo Avila

Want to know how you can help millions of kids like Theo? Make a donation to cancer research! Help cure cancer faster by raising funds for Fred Hutchinson Cancer Center. The Fred Hutch Obliteride is happening August 12th and our dear and wonderful friend, Sue, is riding to raise money! We met Sue about two years ago. She is one of Theo’s Make-A-Wish volunteers. Sue instantly became a close friend, like family, and continues to shower Theo with her love, support, and Spider-man gifts. Not only that. Sue supports many kids and is determined to win the fight against cancer. This will be Sue’s 5th Obliteride. Being close to Theo and knowing his battle, Sue wanted her fund-raising money to go directly towards research that Theo could benefit from. This year she has chosen Dr. Paul A Carpenter, MD, MBBS, attending physician of the Pediatric Blood and Marrow Transplant and Cancer and Blood Disorder Center at Seattle Children’s Hospital as the recipient for all the funds she raises. She recently met with Dr. Paul A Carpenter and got to hear about some of the amazing research he is working on. His clinical and research focus is on graft vs host disease. (YES!) His research continues to explore new therapies for the treatment of acute and chronic GVHD. He has also designed studies to evaluate the efficacy of post-transplant therapies for high-risk Philadelphia chromosome positive leukemia (that’s Theo!). Dr Carpenter is researching therapies that directly affect Theo!! Therapies that Theo could benefit from. YOU CAN HELP!! Please make a donation to Sue’s fundraising page: https://tinyurl.com/2p9yf2pb. Your support WILL make a big difference. Thank you, Sue. Thank you to all who donate.