Juliette's Battle Against HLH

My name is Betsi, I am Juliette's mom. Juliette is 9 years old; she will be 10 in October and would have been in 3rd grade at Los Alamitos Elementary this school year. Juliette has always been a healthy little girl up until last summer. We had planned for her to spend some time up in northern California with my sister. On Saturday, July 3rd, 2022. I rushed Juliette to the ER with a fever, cough, sore throat, loss of appetite, fatigue, abdominal pain, nausea, and yellowing in her eyes. Within a few hours, she was transported and admitted to UCSF Benioff Children's Hospital of Oakland where Juliette was diagnosed with Hemophagocytic Lymphohistiocytosis (HLH) driven by EBV. On July 18th, I made a big decision and transferred her back home where she’d be admitted to the Children's Hospital of Orange County.

HLH is a very rare, life-threatening disorder of the immune system and in Juliette's case, it’s further complicated by a persistent infection with Epstein-Barr virus (EBV). Juliette's EBV has been found in her T cells leading to a diagnosis of EBV lymphoproliferative disease/T cell Lymphoma with CNS involvement.

During her first stay at CHOC, her team created an 8-week chemotherapy treatment plan for her. Within a week, Juliette's body began reacting well to the new treatments and medications and was given the fantastic news that she could finally go home. She would be able to finish her 8-week treatment plan from home and visit the outpatient clinic once weekly. She was welcomed home with a beautiful drive-by parade from all of her friends and family. It was an emotional and beautiful moment for her to be able to see everyone again.

On August 19th, she was rushed to the ER with a spiked fever again. After a few days of waiting for blood test results, it was discovered that Juliette’s EBV and HLH had spiked. Chemotherapy wasn't working; her doctors gave us the heartbreaking news that Juliette would need a Bone Marrow Transplant for a full recovery.

On August 30th, Juliette started a new Chemotherapy and Immunosuppressive Treatment. With this new roadmap to recovery, the goal would be to reduce the inflammation and EBV entirely and then take her to transplant. Once again her body was responding well to this new chemo and on September 2 she was able to go home and return to the outpatient clinic with visits 3 times a week. For a month Juliette's EBV maintained itself at a high count, that made us nervous as we didn’t know if this treatment was working or not. Until finally it started going down and got to the lowest she'd ever had. It was a huge achievement and we had high hopes that she was finally getting closer to transplant.

On October 15th, unfortunately, Juliette was rushed to the ER again for abdominal pain. After waiting for test results to come back we were told she had two viruses, pseudomonas and legionella. Her EBV was also starting to spike again. On the morning of October 19th, the unimaginable happened, a code blue and code stroke were called. It was the hardest day of our lifes. Juliette had 2 back to back seizures leaving her unable to breathe and losing all mobility and cognitive state. She was immediately intubated and moved to the ICU, her MRI showed significant inflammation in the brain. She immediately received chemotherapy via a lumbar puncture in her spine and spent 3 long weeks in ICU. During that period, she was given a 21-day CHOP chemotherapy treatment to control her HLH flare. She was unable to fully recover due to other complications such as fevers, shivering, nausea, delirium/hallucination, high blood pressure, high blood sugar, inflammation in the brain, inflammation in the liver, inflammation in the spleen, inflammation in the pancreas, lung failure, mucusitis, muscle deconditioning, a kidney stone, and a rare hematoma. She was fighting her biggest battle yet. But I knew in my heart, that she was strong enough to overcome it all.

On November 9th she was finally moved back to the Oncology floor. Our warrior fought for her life and began to improve day by day. Recovery would be even harder. She would have to relearn everything from breathing on her own, to talking, drinking, eating, sitting, standing, walking and using her hands again. After the 21 days of chemo ended, she was still unable to go to transplant because her body was simply not strong enough nor ready for the difficulties that come with a Bone Marrow Transplant. Physical therapy and occupational therapy worked hard to push her to a speedy recovery. Luckily for us, Juliette's determination has always been beyond what we can imagine, and once again she was proving that. She was desperate to gain her independence back, surpassing goals in record time. Her medical team was shocked to see that she was improving faster than they anticipated. They gave her a month to continue recovering and set a transplant date of December 16th.

The week before December 16th, Juliette’s HLH flared again. I was heartbroken and this feeling of defeat was hard to brush off. It was disappointing to both Juliette and I because she had worked so hard for this day to come. Everyday there was something new she was accomplishing and while it wasn’t always easy, she would still push through the bad days. We thought we almost had it, we were so close to reaching the end of what’s been the longest and toughest journey yet. With tears in my eyes, I accepted the defeat and knew she had to work twice as hard to get even more strong for her next shot at transplant.

On December 12th, her doctors gave her a 2nd round of the CHOP chemotherapy. Day 21 would end on January 2nd. CHOP did as it was supposed to do and lowered her HLH. Although it never took her EBV to zero like the first time, it was less than 500 by the end of the 21 days, enough to move forward. Her transplant was set for January 20th because her team insisted that she go home and see her family and friends before transplant. I was uneasy with this decision, it took me some time to convince myself that it was a good idea. I was afraid she’d get sick while she was at home or that she’d catch a virus or flare again. But the doctors reassured me it was the best thing to do, so on January 9th, we were sent home. We would return and be readmitted January 13th to begin conditioning for transplant.

January 13th finally came, but the very same night we were admitted, Juliette spiked a fever. Cultures were drawn, we would need to wait 48hrs to find out if she had a virus or bacteria. The next day, the doctors made me aware that her EBV test that was drawn on the 9th (the day we left home) came back Wednesday (while we were home) showing her EBV was spiking again. As you can imagine I had a million questions in my head, but the weekend team didn't have answers for me. They did however, at least start her back on Gamifant, an Immunosuppressive, in hopes to control her flare while waiting for cultures and labs to come back.

On Monday, January 16th we were given the crushing news that they would postpone her transplant again. Juliette had developed pneumatosis, which needed to be treated with antibiotics before they can even start any kind of chemotherapy. This time around, however, I was not only heartbroken but I felt angry that once again we missed our chance at transplant. Juliette was the best she had been since ICU, we had worked so hard for this, but our team waited too long. She finished the 21 day chemo on Jan. 2nd, and from the 2nd to Jan. 13th, nothing was done, instead, we were sent home.

After a long discussion, her primary BMT doctor and BMT team didn’t seem ready or willing to work vigorously to push her to transplant. My cry for aggressiveness, confidence and affirmation were not heard. There was no new plan of attack ready, their only solution was giving her a 3rd cycle of CHOP. I was crushed, I felt sick to my stomach, and in that moment I knew in my heart this was no longer the team for her. I truly believed this hospital was fully capable of keeping Juliette alive. But towards the end of the meeting, everyone agreed that she was better off transferring to Cincinnati Children’s Hospital.

On January 27th, we jumped on a plane together with just one piece of luggage on hand and left everything behind because I knew Cincinnati is her best chance. Cincinnati Children's Hospital has a team of world-renowned experts in the field of HLH and bone marrow transplant as well as access to the most up-to-date treatments and research available for these specialties. They are the Center of Excellence for HLH and can offer Juliette the best chance at a positive outcome. It never once crossed my mind that I would so suddenly have to start with a new team someplace else. But what I did know, was that my baby girl, my fighter, my warrior, was deserving of the best medical team and would be just as determined as she is.

I want to thank everyone for taking the time to read the whole page. I know its a lot, but I also know there’s a lot of people who truly care for Juliette and would really like to hear her updates. I want to apologize for not being able to update you sooner. These last few months were the hardest for her, but she continues to fight every day. Her happy, kind, loving and caring personality continues to touch so many people. She left a little mark in the Children’s Hospital of Orange County and I have no doubt she will do that here. In just the few weeks we’ve been here, her nurses and doctors have all grown so fond of her. They say her joy and appreciation to be alive are contagious because even though she’s been through so much and still has a lot to go through, she still hasn’t given up or lost interest as many other kids have. She says yes to everything, she loves physical therapy, occupational therapy, music therapy, art therapy, dog therapy, school, child life, playroom time, the Ryan secrest studio, craft time, etc. Her optimism and eagerness to live give everyone so much hope and I’ve spent every day taking in every second of her good days and laughs and smiles because I’ve seen how fast that can be taken away.

Thank you to those who have donated in the past and will chose to support us again. Thank you to everyone who has prayed for us, sent us your kind words, and continue to keep her in your thoughts. I can’t always reply but I want to assure you that we both read all of your loving messages. Many times those small words of encouragement were the pieces of hope we needed to keep pushing. Your kind words are truly noticed and we will forever cherish them. Thank you to my family and close friends who have been with us throughout this full journey. Thank you to our second families… our Jeep family, our LAE family, our CHOC family. Lastly, thank you in advance to all who will chose to support us.

"Even miracles take a little time." -Fairy Godmother