(While much of this document still holds true and useful information, there is an update on Max's medical condition from Nov 23 available here)
Max has neurofibromatosis 1 (NF-1) and has optic gliomas along with other tumors behind the optic chiasm and others in a lower part of his brain. We’re not clear on exactly where the other tumors are as we are still picking up the terminology. In any event, the Neuro-Opthamologist that we saw said that the tumors in the lower part of his brain weren’t going to be an issue and they were very common in people with NF-1. Gliomas are brain tumors that arise from the glial cells or the supporting cells of the nervous system. His tumors are non-cancerous. They are not invading the surrounding tissue or organs but they are occupying the space that is available.
The optic chiasm is where the nerves from both eyes cross on their way to the brain. 50% of the nerves here are shared by both eyes so it is quite remarkable that Max has only been severely affected in his left eye. The gliomas in the chiasm are actually woven between the nerves so they cannot be removed via surgery.
Another option for treatment would normally be radiation but there are two reasons not to do this for Max. First, radiation is risky with children because their brains are still developing and any scarring might cause problems later on that would be worse than the current symptoms. In adults this does not seem to be as large a concern. The other reason that radiation isn’t an option for Max is that people with NF-1 have a modified means of repairing their DNA. This is something that is happening constantly in our bodies. The change is the result of NF-1 and one of the symptoms is that tumors are allowed to grow almost unchecked. Another symptom is that in trying to repair the scarring from radiation, Max would be very likely to develop more tumors as a response.
The only real option for Max is chemotherapy. According to the doctor, the treatment that Max will get is successful for 70% for children with his type of tumor. Her opinion is that success rates are actually higher for children with NF-1 but there is no study currently available to support this. Max will be part of a study to prove this.
Max will be getting a ‘line’ installed on Tuesday, August 24 and The Children’s Hospital of Philadelphia (CHOP). The line is also referred to as a Port-O-Cath. It is a permanent catheter that will provide long term access to his vein. He will end up with a bump just below his collar bone. For this procedure, CHOP is hoping to use anesthesia that will have less of an impact on Max than his previous MRI. We’ve talked about how Max will get the anesthesia and we’ve come to the conclusion that we’ll need to work out the finer details on Tuesday. I suspect he’ll be given the same type of drink that he had before his MRI and then get the IV for anesthesia installed after he is groggy or asleep. Max will have a dressing for a few days after the procedure. We had him wear a gauze patch with tape for a few days on Block Island and he has had one on since Saturday that we’ll keep on until the procedure Tuesday. On the way to his future treatments, we’ll put some skin numbing cream on him and by the time we get there he shouldn’t feel the needle sticks.
Immediately following Max’ procedure to get the line installed, we’ll go to the NF-1/Oncology ward where Max will have a blood test and then start his first treatment. If Bob understood everything, the weekly procedure for Max is:
1) sign in and get weighed, measured, blood pressure and heart rate
2) draw blood for testing
3) I think the first treatment if Vincristine Sulfate (VCR) which only takes a few minutes.
4) Max then has to wait for a while (not sure how long) for the VCR to get through his system
5) Then Max gets the carboplatin which takes about 1 hour.
6) Once the carboplatin is in, he has to wait 10 more minutes to see if he is having any problems and then, if all is well, we get to leave
Max will be going to Philadelphia once a week for 10 weeks. Right now, it looks like this will be every Tuesday. At the end of 10 weeks, Max gets 3 weeks off and then starts a 4 week round of treatment. He repeats the 3 weeks off / 4 weeks on schedule for about a year. The total duration is dependent upon how his gliomas are responding to the treatment.
Max also has an MRI at CHOP scheduled for Thursday, August 26. Unfortunately they cannot use the port for Max because it has to have a week to heal before it can used. Max will have follow up MRIs at least every three months for the duration of his treatment and he will likely need them periodically for the rest of his life. Max has no reactions to the drugs that used for sedation or for enhancement (contrast dye) and the MRI is essentially just high powered magnets with no know side effects. As he gets older and, hopefully, can stay still, he might not need to be sedated.
The goal is keep Max’s gliomas from growing. If they continue growing they will further impair his vision. There is a chance that the gliomas might actually shrink. This will NOT bring back any lost vision, as that is permanent, but it might provide him with some respite from the treatments. For whatever reason, children with NF-1 seem to have their optic gliomas stop growing between the ages of 8 to 10 so the ultimate goal is to get him to the point where nature will start controlling the gliomas. The important things to remember are that the tumors aren’t cancerous and that they are also not life threatening. Hopefully we can limit the impact to Max to just the damage that has already been done.
(While much of this document still holds true and useful information, there is an update on Max's medical condition from Nov 23 available here)