Date: August, 2006
Mary Pat, mother: Hi, I looked at your website today and felt compelled to share my son Jack’s story of JPA. We have several reasons for sharing this story, one, we believe that every family should have a medical advocate, especially if they have no medical knowledge, to help get second opinions. We found the center where our first surgery was performed to be completely lacking on giving us any advice on where to turn after they had exhausted their resource base of knowledge. They just shrugged and wrote us off.
Second, we believe there should be Brain Tumor Centers of Excellence in the country, at least one on each coast, that handles rarely seen tumor problems such as this. After considerable research, it became evident that one physician in particular had removed more tumors in this location than anyone else we spoke to (and we spoke personally to 6 of the top pediatric neurosurgeons in the country). There has got to be a way to reach families who are dealing with this early on to help them through the discovery process. We found most websites to be unhelpful and many places were reluctant to refer out of their own system. We wanted the BEST outcome for our son, regardless of where it would take us.
Now, we want to help other kids with JPA and find the cause and cure for this problem!
Jack was the picture of a healthy 10 year old boy; he was just finishing a busy summer of swimming, tennis and camp and had returned from vacation to San Francisco to our home in Kansas. The night after the first day of 5th grade, Jack awoke with a low grade fever and extreme dizziness. He almost fell trying to get to the bathroom. The following day, he stayed home, but felt better by afternoon and resumed his normal, busy schedule the next day. Because my husband and I are physicians, we looked Jack over carefully and noted that when he looked to the right, his eyes jiggled, an entity called nystagmus. We thought he must have a virus that affected his inner ear. After the nystagmus did not resolve in a few weeks, an MRI showed that Jack had a brainstem tumor about the size of a plum.
After discussion with our pediatrician and several other prominent physicians, we saw the pediatric neurosurgeon at the regional pediatric medical center about an hour from our home and surgery was performed the following week. Only a minimal amount of tumor was removed, but the diagnosis of JPA was obtained. Jack was also found to have some lower cranial nerve involvement and cerebellar signs, such as deviation of the tongue to the right side, positive Romberg test (poor balance) and past pointing. In retrospect, he had been clearing his throat since age 4 (we had been told this must be a nervous habit), and was afraid of heights in the past 1 – 2 years and was no longer ticklish!
The oncologist at our regional pediatric hospital told us Jack probably had 9 – 18 months to live and said maybe radiation could help. We met to set up RT and were told there is no way they could radiate, that we would do chemo.
At that point, we consulted every possible resource to find a pediatric neurosurgeon for a second opinion. We sent MRI’s and case histories to 6 places most listed on the pediatric brain tumor consortium member list, and visited 4 in person. We had the luxury of time. Jack did not have elevated intracranial pressure and was neurologically stable. The variety of things we were told was incredible and we were very uncertain as to what to do.
After 2 and half months of research, reading articles and consulting, we came to the conclusion that the only chance for reasonable cure was complete surgical resection. We finally found a neurosurgeon who understood every aspect of what was going on. He recommended laryngoscopy, which showed a paralyzed right vocal cord, and a swallowing study. He stated there would be no eating or drinking after surgery, until the airway was proved safe by a post-operative swallowing study. After our first surgery, food and drink was pushed immediately!
Fortunately for Jack, our doctor was successful in removing 100 percent of visible tumor, which has been affirmed by 3 month post-operative MRI. After two months of physical therapy, Jack is basically back to normal. He just attended a week long Boy Scout camp in Colorado and is doing very well.
Editor’s Note: Hooray for Jack and persistent parents! Well known clinicians/scientists worldwide have acknowledged that treatment protocols for JPA are different, depending on where you live. This was part of the problem discussed at the first JPA Professional Workshop with 8 of the world’s scientific leaders, and one of the reasons for specifying a unified, collaborative direction for research. (Read a summary of the workshop.) One of the goals of fightJPA.org, moreover, is to help organize the sea of information available on the internet in published studies, through scientists/and clinician feedback and through parent/family submissions. With more input and a centralized source of information, our hope is that parents/families can turn to fightjpa.org to help identify resources, programs and studies which will enable them to make the BEST choices for their children.