Colin is a vivacious, charismatic boy who is both loving and well loved. Leading up to his second birthday, he became more clumsy, tripping when he shouldn’t have been, and complaining frequently of headaches (by pointing at his head). His head also seemed to tilt to one side and, in retrospect, began eating with his left hand and generally favoring that side.
With the sneaking suspicion that something was wrong, we planned on bringing the issue up with his pediatrician at his upcoming well visit. However, we ended up at the doctor’s office on his second birthday, finding out that he had a double ear infection. This could explain the loss of balance at the time but left us still wondering about his behavior over time.
The following day, we returned to the office with a trivial complaint (a bug bite, it turns out, rather than the feared contusion!) because of the neurological concerns. The pediatrician, Dr. Golenbock, heard our concerns and sensed the parental intuition that kept sending us back to the doctor’s office and ordered a non-priority CT scan. His line of questioning helped us understand what sorts of symptoms represented a problem.
On Sunday morning, June 14, 2009, the day we planned to celebrate his birthday with his cousins, Colin couldn’t support his body with his legs and started arching his back, throwing his head back.
Alarmed, we headed straight to the emergency room in Danbury, CT, where the ER doctor, Dr. Michelin, ordered a CT scan. She assured us that we would get an answer that day and suggested that we transfer to the children’s hospital in Westchester for a full neurological workup.
Unfortunately, the CT scan showed a mass in the posterior fossa region of the brain. Growing out of the fourth ventricle, wrapping around the cerebellum and much of the brain stem up to the pons and extending down the top of the spinal cord, it had backed up a large amount of fluid in his brain. The cerebellum controls balance and motor control, explaining much of Colin’s symptoms. Often, the symptoms in these cases are sporadic and appear to improve throughout the course of the day, as we had seen with Colin.
Colin was admitted into the Pediatric ICU (PICU) on Sunday and immediately saw one of the neurosurgeons who treated him there. The MRI the following day made clear the extent of the tumor, which was not fully evident in the CT scan.
The immediate concern was the pressure that caused Colin’s neurological symptoms, and it was also important to diagnose the type and nature of the tumor. Both were impossible to ascertain/confirm without biopsies. The surgeons were reluctant to speculate, as Colin’s case did not fit a common pattern familiar to the team in Westchester. The biopsy and subsequent pathology studies revealed that the tumor is a grade III anaplastic ependynoma, a relatively uncommon cancer that is often restricted to the posterior fossa region (essentially, in the lower back of the head) but in his case migrated much further.
There is some suggestion that the tumor is congenital and it may have become more aggressive only recently. However, this is still not clear. These sorts of tumors typically originate from cancerous stem cells, so it only takes a single errant cell after treatment to serve as the seed for new lesions.
Colin’s first surgery removed the section of tumor on the back side of the cerebellum, freeing up room for the fluid to flow and presumably relieving pressure. Another component of surgery was the installation of a drain that provided an external valve to alleviate pressure. Colin’s body adjusted to the pressure well and the surgeons removed the drain.
Colin’s second surgery was performed by Dr. Jeffrey Wisoff at NYU Medical Center, successfully removing 95 – 98% of the tumor. He reported the tumor remnant as a piece 5 mm x 5 mm x 2 mm. Although the immediately pre-op MRI showed evidence of metastasis in two places in the spine, a later post-op scan found the spine clear of disseminated disease.
The hydrocephalus proved too much for Colin’s system to manage, so Dr. Wisoff also installed a ventriculoperitoneal shunt (VP shunt) that has a valve behind his left ear and wends through his body, letting out into his stomach. The shunt has done it job well (and without complications!), greatly reducing the degree of hydrocephalus over time.
Colin suffered a fair amount of nerve damage as the result of the second surgery, leaving him unable to swallow, with facial paralysis, impairments to the right eye, and deafness in one ear. He received a gastronomy tube in New York to allow feeding. At St. Jude, they recommended that a tracheostomy would keep his lungs safe, given the paralysis of one vocal cord and other deficits that left him unable to swallow properly or protect his airway.
Colin received chemotherapy through a research trial for children under three with certain brain tumors (SJYC07) at St. Jude Children’s Research Hospital. If the chemo sufficiently reduces the tumor, then he will move on to radiation therapy; otherwise, he will have an additional surgery before radiation. Although other children on the protocol are currently getting proton therapy in Jacksonville, FL, Colin will not be traveling there due to his matrix of medical complications.
Colin’s been a model patient, holding still for CT scans and rapid MRIs, tolerating an IV line and other indignities, and flirting shamelessly with the nurses. He has endured so much through the course of his treatment, including a fungal blood infection that later evolved into endocarditis, an infection of the heart.
Colin’s condition has improved considerably, and we look forward to him walking and talking again. The more he improves, the more we see his personality and appreciate his strange combination of a good nature, strong will, and adaptability. His sense of humor is fully intact, as is his ability to charm his many admirers.
We appreciate everybody’s thoughts, wishes and prayers. As for the many offers for help, we expect to be calling them in as we get a better picture of Colin’s treatment plan. This has also been a challenging time for Aidan, who is sensitive and is very concerned about his brother.
The most updated information on Colin’s case is available through the posts on this site. We love to read comments, and you are welcome to contact us directly, though we warn you in advance that we are not very good at keeping up on individual correspondence.
