In the fall of 2020 following an ear infection, Ava began experiencing symptoms that were unusual for an ear infection including double vision. After multiple doctors told us it was just an ear infection, she was finally evaluated by an ophthalmologist at Phoenix Children’s Hospital who immediately referred her to the ER for brain imaging. We were then informed Ava was experiencing a neuro-autoimmune attack and would need to be admitted to the ICU. Initially we were told she had something called ADEM, which is a complex neuro-autoimmune event but that it was acute, not chronic, and she would recover with a week of high immune suppression therapy. And while they were correct in that she did recover, what happened next took all of us by surprise. Her symptoms returned, and she was readmitted to the hospital 10 days later. This time more aggressive therapies were attempted and once again the attack calmed down and she was discharged after a few weeks. This again was not the end of the attack, unfortunately, as she once again relapsed and was back in the hospital a few days later. At this point her symptoms had progressed to the point of her having severe vision issues, tremors, difficulty speaking, walking, and memory issues. Once again, they attempted to control the attack with more immune suppression which was successful, but it came at a cost. We were given no promises her symptoms would ever resolve, as her brain needed to find new pathways to relearn things like walking. She spent an additional 6 weeks in inpatient rehab for speech, occupational and physical therapy where she always showed up with a smile and was determined to get back to her normal self. She eventually was able to do just that, and her life had returned to normal for the past couple of years. 

In 2023 we had thought the worst days were behind her and her routine MRIs continued to paint a picture that things were back to normal for her. In January of 2024, however, at one of her normal appointments, we found out her oxygen level was low and her heart rate was high and they recommend we go to the ER to check things out. What they found was fluid around her lungs, heart, multiple organs inflamed, and her blood counts struggling to keep her from falling anemic. That same night she was once again back in the same ICU she was in a few years prior, this time in respiratory failure again in very serious condition. She spent the next 4 months in the hospital, once again battling back to recovery. 

This time, however, the conversation began to shift from 2020 when the thought was that this was an acute condition to her having a chronic immune dysregulation disorder. Further complicating matters — she was diagnosed with a condition called HLH which is a very rare but serious condition in which white blood cells build up in and damage organs. On top of which she unfortunately had an atypical course of this illness that was resistant to normal treatment options (a similar theme to 2020). Since the end of May, Ava has been at home still getting IV treatments with heavy immune suppression to keep her system under control. 

While we are grateful she’s currently stable, the medications required to keep her stable are not long term options, and we have been informed the best path forward for Ava is a Bone Marrow Transplant. Given this news we opted to take her to Boston Children’s due to the complexity of her case where they are now lining up the transplant. Ava will need to undergo an inpatient admission that will require an intense regimen of chemotherapy prior to transplant with the goal of destroying her current immune system. After transplant she will remain admitted likely for at least a couple months while the new immune system from the donor begins to build in her system. After her cell counts are at levels they are comfortable with, she will be discharged but will need to remain in the Boston area for a few months at least until she is deemed safe enough to travel home. 

We have partnered with the Children’s Organ Transplant Association (COTA) for assistance with transplant-related expenses, which include all the life-saving expenses that result from the diagnosis that would require a patient to be transplanted, such as medical therapies and treatments. Please consider donating to COTA in honor of Ava.

The Children’s Organ Transplant Association (COTA) helps children and young adults who need a life-saving transplant by providing fundraising assistance and family support. COTA is the nation’s only fundraising organization solely dedicated to raising life-saving dollars in honor of transplant-needy children and young adults. 100% of each contribution made to COTA in honor of our patients helps meet transplant-related expenses. COTA’s services are free to our families, and gifts to COTA are tax deductible to the fullest extent of the law.