It all started on September 13th when we found a painless lump on Ava’s left buttock during our bedtime routine. The next day, Ava went to our local pediatric practice where several practitioners examined her and were unsure. We were sent for a stat MRI at our local hospital. We received a call from our doctor and were told that the radiologist was very concerned and we were instructed to go to children’s hospital emergency department. It was there that we were told that Ava had a large tumor that needed to be properly identified to make a diagnosis. We had a biopsy four days later and then the waiting began. It was 6 days until our worst fear became a reality.... Ava had cancer.
Ava was diagnosed with Ewing’s Sarcoma, a rare pediatric cancer affecting approximately 200 kids a year. We were told that to obtain a prognosis, it was necessary to assess if the cancer had spread. The next day Ava was scheduled for an implantation of a mediport to receive her chemo, had bone marrow biopsies, received a PET Scan, and had an ovary removed for preservation.
We received results that her PET scan and bone marrow biopsy were clear and for the first time in two weeks, we were able to breathe. That means her disease is local and not metastatic which gives Ava a 70% chance of beating this horrible disease versus 15%.
We were admitted on September 28th after additional testing to start chemo. Through this all, we have come to learn that Ava was only born with one kidney. Fortunately testing has shown it is functioning to its’ full capacity. We had our chemo for two days and the returned the day after for her neulasta injection to boost her blood counts.
The last week post chemo has been rough. Ava has had severe jaw pain as a side effect from one of the chemotherapeutic agents, along with nausea and poor appetite. Eating has been a real struggle. Ava took a trip to the local ER on Saturday secondary to a fever and has since been admitted to Children’s Hospital for severe neutropenia. So far her blood cultures did not show any bacteria but due to a severely weakened immune system; she is being treated with broad spectrum antibiotics. We will be released when her WBC and more importantly her neutrophils reach a more normal level. We are scheduled for a five day admission in one week for our next chemo.
So chemo is done, what’s next. Four weeks from today, Ava is scheduled for surgery to remove her mediport device. She will remain on antibiotics for the next couple months to decrease her risk of getting pneumocystis pneumonia secondary to her compromised immunity. She has been able to go off two other medications, she liked the least, since chemo has ended. This has made a huge difference in our lives. Her taste for food remains impaired and appetite fluctuates. She is in great spirits and has energy; but by later in the day is wore out more than usual. After her port is removed, we won’t need to go back to Pittsburgh until mid October for her next set of scans. At that time, we will need to return to Cleveland as well to follow up with her orthopedic oncologist. We will follow up every three months this first year and then every four months the second year. She will decrease to every 6 months from year 2 to 5. At these follow ups, she will have either a chest CT or chest X-ray; as well as an MRI of her pelvis. She will also get lab work and an echocardiogram. The main purpose of this is to look for a medication induced cardiomyopathy from the doxorubicin she received. They will also monitor kidney function as the cyclophosphamide she received can be toxic to kidneys and as we found out the day begin chemo begin, Ava was only born with one. From year five through ten, she will be seen once a year. During these years, scans will not be performed. The overall purpose of visits during those five years is to monitor long term side effects of chemo.
The relapse rate for localized Ewing’s is 30%. It most often occurs in the first two years. It can occur later but being declared 24 months NED is a big deal. Ava’s tumor was 100% dead upon removal, and research is showing with complete tissue necrosis, risk for relapse is reduced to around 10%. We are optimistic that this is the end of our treatments. We are hoping scans get easier with time, as we tried doing it last week without sedation and she moved a lot. It took 2 hours to get the images they needed. Hoping that with age and experience, it is effortless. Thank you all so much. We are fortunate to have you all sharing in our journey. We are hoping to enjoy the last few weeks of summer while our blood counts rise and we await surgical removal of her port.