Gagen was diagnosed with diffused leptomeningeal glioneuronal tumors (DLGNT) in December 2018 at the age of 12.
His first symptom was back pain after a wrestling match in November 2017. We went to many specialists with no results until July 2018 when the hydrocephalus appeared.
In December 2018, when checking CSF, Gagen's protein in the spine was >600. It took multiple tests and eventually the third brain biopsy, at the brain stem, showed BRAF KIAA 1549 fusion.
Gagen's first treatment was Carboplatin and Vincristine. He made it to 43 weeks but then had a severe reaction preventing him from breathing. During the 43 weeks the tumors stayed stable.
At this point we reached out to St. Jude Hospital because Children's St. Louis had no other treatments available. The St Jude affiliated hospital in Springfield MO started Gagen on Trametinib. Gagen's tumors began to shrink but his heart function declined to 13% over a short 5 months. He had a stable scan 3 months later but the following scan had significant progression.
We then started Proton Radiation therapy on October 2020 and finished in December 2020. Gagen received 3200 of radiation intracranial. The tumors appeared reduced but Gagen had to have a feeding tube in January 2021 for failure to thrive due, as the fatigue from radiation had him sleeping for 23 hours a day. The feeding tube came out in June 2021.
Scans were stable after radiation until March 2022 when he had significant progression again. These new tumors are new areas that were not there before radiation. Two spots in the brain and on the brian stem are the two that are progressing the most, the spine is stable.
Gagen started Vinblastine for 17 weeks, but his counts and body couldn't handle over 4 weeks. They were trying to get to dose 6 when Gagen started having stroke symptoms frequently. Scans within these 17 weeks showed significant progression.
Gagen has now been diagnosed with SMART Syndrome - stroke-like migraines after radiation therapy. Now they are trying him on Avastin.
Scans will be the first week of October 2022.