In May 2016, Danjela was 11 and about to graduate from 5th grade when our world turned upside down in one evening. What we thought was a migraine turned into a 7cm tumor on the left side of her brain, which had burst and was bleeding into her brain. She deteriorated quickly and was airlifted to Children’s Hospital Colorado where she immediately went into surgery with Dr. Todd Hankinson. Dr. Jean Mulcahy-Levy came to see us in the PICU following surgery and told us she was from the Neuro-Oncology team and that she would be Dani’s doctor moving forward.
In June 2016, Dani was diagnosed during a time of rapid change and increasing knowledge about pediatric brain tumors. Had Dani been diagnosed just a year earlier, her tumor would have been called a PNET (primitive neuroectodermal tumor). But due to an increasing understanding of how to genetically classify tumors, the new name for her tumor was CNS neuroblastoma. Pediatric brain tumors are already rare, but this new diagnosis put Dani into an even more rare category and the “new” diagnosis made it difficult to know how to best treat it.
This diagnosis blindsided us because up until the night of her surgery, we had no idea that she even had a tumor. She had no symptoms at all: no vision, hearing, or balance issues, no dizziness and was always a healthy child. Dr. Hankinson told us that her tumor had been growing for years, to the point that it was putting pressure on her skull and thinning the bone structure. We felt like we had been hit by a truck which then went into reverse and hit us again. Danjela’s first round of treatment started with 6 weeks of daily radiation combined with a chemotherapy “boost.” After that there were 6 months of chemo-only treatments. Dani suffered from radiation side effects including sore throat, fatigue, and severe radiation burns on her back, chest, and neck as well as chemo side effects like hair loss, weight loss, neuropathy, to name a few. She tolerated lengthy hospital stays, monthly MRIs, countless blood draws, and NG tube placements. Danjela endured it all with such grace, hope, and fighting spirit. On February 14, 2017, Dr. Mulcahy-Levy shared with us the wonderful news that the treatment worked and Danjela was NED (No Evidence of Disease). We were thrilled knowing that the treatment had worked. We went to MRIs every 3 months and when Danjela started her 7th grade year, we had graduated to every 6 months for MRIs. We were so happy and so thankful!
Danjela celebrated by wanting to give back to the children still in the hospital. She always told us that even though she was so sick, there were always children sicker than her and she wanted to do what she could to help them. With our help, she organized book drives, toy drives, craft kit drives, LEGO drives, nail polish drives, and DVD drives for the kids on the 7th floor at Children’s Colorado. Her love for others was her driving force to keep going through the agonizing treatments week after week. She never stopped smiling.
In August 2018, shortly before she started 8th grade, we noticed that Danjela wasn’t feeling “right.” Dr. Mulcahy-Levy asked us to come in for an unscheduled MRI after which we were told that the tumor had grown back and Danjela had officially relapsed. Two weeks before the first day of school, Danjela underwent another tumor resection with Dr. Hankinson. The pathology officially confirmed a relapse and Dani started a clinical trial with Dr. Lindsey Hoffman. We were so hopeful for this new treatment plan, but unfortunately, Danjela was unable to complete the treatment. In October 2018, only a few weeks after starting the trial, Danjela had a series of seizures that left her partially paralyzed and weak on her right side. We were eventually told that she was diagnosed with SMART Syndrome, a rare late-term side effect of targeted radiation. It mimics a stroke but is self-resolving. She spent 3 months in the hospital and by the time she was discharged, she was able to walk and use her right hand and arm. When Dani relapsed, a deeper genetic analysis of her tumor found a EP300::BCOR fusion, one of the rarest mutations that had been identified in CNS neuroblastoma. Curing Danjela became an even bigger challenge and one that we were not backing down from.
In 2019, once she was strong enough, Dani went through another 6 weeks of targeted radiation to treat the relapsed tumor. Danjela responded beautifully to the radiation and the remaining tumor had started to shrink. But unfortunately, Danjela’s tumor is relentless and continued to spread in small sections.
2020 brought us more tumor spots and more radiation. Throughout 2020, Danjela went through 2 additional rounds of short-term, targeted radiation and one 6-week round of daily, targeted radiation. Unfortunately, the radiation began to take its toll on Danjela’s body. The right-side strength that she regained after her seizure disorder in 2018 was now all but gone. The radiation had resulted in an almost complete loss of the use of her right arm and extreme weakness in her right leg. She has endured some permanent neurological damage as well as memory loss and difficulty with speech. Dr. Mulcahy-Levy told us that we were reaching the maximum tolerance point for radiation and that it might not be an option for future tumor growth, which we knew would appear.
From 2021 until her passing in August 2023, we knew we were running out of time and options. The tumor continued to spread and began to infiltrate the right side of her brain. Danjela endured more daily radiation and a series of alternative chemotherapies that would not completely kill the tumor cells but would hopefully slow down the growth. Unfortunately, these options always brought more problems than hope and we had to halt any new therapies that would give Danjela a chance of survival.
In 2022, Danjela started her senior year of high school with only one goal in mind – to graduate high school with her class. Despite all her difficulties and challenges, she worked very hard and proudly received her high school diploma in May 2023. On August 15, 2023 our beautiful Danjela took her last breath on this earth wrapped in my arms and held by her father and sister. Her fight was over.
Danjela’s Legacy
Dani’s story does not end with her death. When Dani was initially diagnosed, we had donated a sample of her tumor to The Morgan Adams Foundation Pediatric Tumor Bank. Through the support of the MAF and Dr. Jean Mulcahy-Levy, we were able to learn about Dr. David Solomon at UCSF and the work he was doing in the study of tumors with rare genetic changes, like Dani’s. Through generous donors, MAF was able to fund a grant for Dr. Solomon which was used to further his studies. We were able to send Dr. Solomon a sample of Dani’s tumor with the hope he would be able to create a cell line or use her tumor information to better understand tumors with such a rare genetic change. His input played an integral part in helping Dr. Mulcahy-Levy find unique therapies that we were willing to try.
When Dani passed away, we made the final decision to donate her tumor to the lab. This decision has led to one of the most exciting advances we have seen in the potential to develop new therapies for Dani’s tumor. There have been a number of articles published about tumors with Dani’s genetic change, but they have all told what the tumors look like, how they present in the clinic, what the genetic change is and how it looks under a microscope. What the research community had lacked was a way to test new potential treatments because tumors with her genetic change have not been grown in the lab. From Dani’s autopsy sample and continued funding from The Morgan Adams Foundation, the research team at Children’s Hospital Colorado have amazingly been able to grow tumor cells in the lab, creating, as far as we know, the first patient derived tumor cell line with a EP300::BCOR fusion. This will allow them and other labs to begin studying new treatment options for this tumor, hopefully leading to a cure for future patients. It will also help them to compare Dani’s tumor at her original diagnosis and her death, to figure out why her tumor was able to evade everything that we threw at it and she put up with! Dani’s original tumor sample has been shared around the world and her autopsy sample has developed into a potential game changer for research into this tumor type.
While Danjela is no longer with us on this earth, she is continuing to spread joy and happiness….and even more importantly, hope to those still fighting. She has given a great gift to the Neuro-Oncology world, but it can only continue through amazingly generous donations to The Morgan Adams Foundation. Please help our family keep Dani’s legacy alive.
