Rapamycin

My heart is full this evening as I prepare this blog. I sit here sharing a king bed with my wife and Jared. Poor Jared is snoring loudly and I must apologize for getting personal, but it is a bit heartbreaking considering the news from the CT/PET images. We were informed that the tumor has in fact spread very rapidly into the area just behind the throat all the way down the neck to the C5 vertebrae. Images also revealed that it has recurred in its original location, as well as others in the skull base. What a daunting beast he is dealing with. He somehow remains quiet through most of it. He complains of pain periodically from the surgeries as well as a headache, but overall he just doesn't say much. Oh, what a sweet child, and to have to watch all of this is heartbreaking, but I know there are greater purposes in everything we experience in life.

When Jared was originally diagnosed he was also diagnosed with Tuberous Sclerosis Complex (TSC). This has gone unnoticed throughout the years, and upon being diagnosed, we were told that since he showed no symptoms from this disease it was irrelevant at the moment. The chordoma was first and foremost in this battle and must be dealt with immediately. Following effective chordoma treatments we would deal with the TSC, well, so we thought. It just so happens that Massachusetts General Hospital is home to one of the leading TSC doctors in the world.

Originally, it was planned to treat Jared with chemotherapy drugs that, quite frequently, have shown no effect on chordoma tumors. We were informed that due to the aggressive nature of the tumor we could have a chance, as a handful of other children had shown some response. We were nervous, but hopeful that Jared might be included in that group. It was not until the oncologist became aware that he had TSC that a second option became available.

A third physician was introduced into the picture. This third physician is the world renowned TSC doctor who believes there is a very strong correlation between TSC and chordomas. She believes that TSC tumors and chordomas show metabolic similarities in the mTOR pathway. This is a pathway that apparently regulates cell proliferation. This pathway becomes deregulated in TSC and will present a risk for benign tumors to develop throughout the body. A drug call Rapamycin has been used and has been proven effective at shutting down this pathway and thereby inhibiting and decreasing the growth and size of the existing tumor. She suggested we try a daily dose of Rapamycin on Jared and strongly believes that we might be able to see some of the same results.

Jared took his first dose of Rapamycin last night. We will observe him very closely while he is taking this to make sure he is not growing worse. If his condition does not improve and his symptoms increase, everything is in place to begin chemotherapy immediately. We are excited for the prospects of decreasing the tumor size with a drug that has proven to have far fewer side effects. We are very anxious to see some improvement and be on the cutting edge of chordoma science. What a great thing this could be for others?