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NF2 Perspective
Bill Macdonald's story

1. Introduction

It was suggested that I contribute an AN story from the perspective of an NF2 patient. We are definitely in the minority, and finding someone who has treated more than a handful of NF2 patients is not easy. NF2 tumors are described as 'different', 'stubborn' and ‘unpredictable’, which ironically are polar opposites of our personalities.

My situation… 42 years old, deaf in left ear (surgical removal '86, no reoccurrence) with a previously hacked at 1.4 cm tumor sitting on the eighth nerve to the right ear. Able to manage with the use of an aid. Hearing diminishing slowly as the tumor continues to progress. Maximum dimension of tumor 1.4 cm with hearing being the major concern.

I recently (1999) did the rounds of several neurosurgeons, Gamma Knifers and FSR treatment providers in the NY-VA area. My best friend (he has perfect hearing and excellent stenographer skills) and I took our questions and notepads to Jefferson University Hospital, University of Virginia Medical Center, University of Pittsburgh Medical Center, The Johns Hopkins Hospital and Staten Island University Hospital. The following is a brief overview of our findings, based upon answers to questions and personal observations.

2. Overview of our findings

Each provider visited felt strongly that his area of expertise was best suited for treatment. Quite frankly, I wouldn't want to be treated by anyone less confident. Asking the question, 'how many NF2 patients have you treated?' reveals some surprisingly small numbers. One center quoted another center's statistics, it's important to find THAT center's statistics. Most everyone was sincere and willing to spend whatever time was needed to discuss the issue and answer questions.

Surgery: There seems to be a direct correlation here with tumor size and the ability to save hearing. Experience of the surgeon is of utmost importance. As I have been privileged to experience two prior surgeries, this option was not considered. As a fellow NF2'er said to me, "any treatment that doesn't involve surgery is the best treatment." That's not to say some neurosurgeons would not have 'attempted' to preserve my hearing. Nor does it say that neurosurgery is not able to preserve hearing or that neurosurgery isn't the best approach for some. Interestingly enough, one surgeon's assistant made a concerted point of indicating that 'no treatment' was a viable option.

Gamma Knife: The tumor control results for NF-2 patients are favorable, but slightly less so than for non-NF2 patients. One provider suggested taking sign language and/or lip reading and then seeking Gamma Knife treatment whenever those skills were accomplished. The nerve of the NF2 patient usually travels through the center of the tumor (the tumor envelops the nerve.) The nerves of non-Nf2 patients may remain along the outside of the tumor. Because Gamma Knife treatment focuses radiation on several isocenters of the tumor, it is possible that the nerve traveling through the tumor will be zapped by the radiation along with the tumor. One center professed a 50-60% chance of preserving hearing. The data used to arrive at this conclusion appeared sparse to me. For example, it was possible for just a few patients with very small tumors and perfect hearing before treatment who still are able to discriminate speech two years later to account for a 50% preserved hearing success rate. Results span the past ten years for GK, but protocols have changed during that time and are evidently going to continue to change.

FSR: There are several different regimens here, depending upon the provider. The two which seem to have the best NF2 tumor control involve five treatments. Their tumor control was 100%, over a 6-7 year period of time. The tumor control rates for NF2 patients are no different than for non-NF2 patients (obviously.) The FSR hearing preservation statistics are also no different than for non-NF2 patients. One provider mentioned that hearing continues to decrease for NF2 patients at a rate 'NOT unlike" non-NF2 patients, the other provider mentioned…'15% increase hearing, 15% decrease hearing, 70% stay the same' and 'what other choice do you have?'  FSR with a LINAC, though less accurate, delivers a homogenous dose of radiation thus avoiding the isocenters or 'hot spots' associated with GK. Little to no other nerve impairment was significant. Both of these providers mentioned that long term there will likely be failures, but they could not obviously say for certain. Results were over a 6-7 year period of time, with protocols still changing. Time in-between individual treatments varied with one center.

3. Conclusion

I seriously considered all options except for surgery. The decision I made was to have FSR with Dr. Lederman at Staten Island University Hospital. There were many reasons for making this decision, some tangible some intuitive. He has the most experience with FSR treatment for NF2 patients, and being a former New Yorker, I appreciated his candor. Besides, there's a great pizza place around the corner.  I would have also been comfortable to have either Dr. Williams at Johns Hopkins or Dr. Steiner at UVA treat me. Both men were exceedingly straightforward and forthcoming with their experience and professional opinions. Having other tumors, I wouldn't be surprised to find myself sitting in any one of these gentlemen's offices again.

Three months after the  treatment I have shown no neurological changes. Hearing, balance and facial nerve have remained constant. I realize it is still early in the game, but to date all is well.

There is absolutely no conceivable way on this earth I could endure this process without the love and support of family, friends and other patients. My prayer is that all of you have someone to encourage and strengthen you to arrive at the best treatment option available for you.  It is important to keep in mind that we are all different. Our circumstances are different and our priorities are different. Each of us must decide what course of treatment to pursue based upon our own experience, our own circumstances and our own intuition. No one choice is the right choice for all.

Please feel free to contact me with any questions or comments.

Bill Macdonald
May 2000


Updates

(November 2000)  It's been approximately 9-10 months since my first and last treatments. Here are a few developments worth noting.

Approximately 3 months after the first treatment I began experiencing tingling, numbness and a lack of sensation on the right side (treated side) of my face and inside of my mouth (forehead, cheek, tongue, chin, lip etc.) This is the trigeminal nerve that was somehow being affected. A call to Dr. Lederman resulted in him thinking the radiation did not play a significant "risk factor". My neurologist suspected swelling. Thrown on Prednisone (steroids) for a week, followed by a maintenance dose of steroids the following month cleared the problem up.

I have never experienced an activity level and craving for food as I did while on the steroids...

To simplify MRI results, they go something like this.  My tumor had been progressing at a rate of .1 cm per year for the past five years. 1.4 cm tumor three months before treatment, 2.0 cm tumor three months after treatment (swelling suspected), onset of trigeminal symptoms, two months of steroid medication, second post treatment scan showing a 1.8 cm. tumor.

Upon sending the films to Dr. Lederman's office, his assistant called to inform me that growth of the tumor had been arrested. I questioned as to how that conclusion can be drawn given the various tumor dimensions following treatment. "Radiological changes are taking place" was given as the explanation. How tumor growth cessation was concluded still escapes me. Swelling seems to have subsided, which does not constitute tumor growth control. However, I do remain optimistic. The real test for tumor control will be the MRIs in the year 2001.

I have experienced no other side effects from the treatment. My hearing continues to decline, more so in discrimination than volume. Shortly before receiving the treatments my hearing discrimination took a dive south. Shortly after the treatments my hearing discrimination improved to "pre-dive" levels. Over the past few months my hearing has returned to the lower discrimination level. What role the radiation has played in all of this is anyone's guess.

(July 2001) My latest MRI was on May 11, 2001. This is approximately 17 months following the radiation treatments and 6 months following the last MRI. The tumor measured 1.8 cm, which is the same measurement taken six months ago. Bottom line: the tumor seems to have settled in at a size greater than when first treated, but less than its greatest dimension due to swelling. No growth, I'll take it. Hearing, facial, balance all have remained constant over the previous six months.

God bless all of you, I will update you with each succeeding MRI and/or development.

(April 2005) 5+ years post FSR...  MRIs indicate that the tumor is still stable and shows no growth.  I show no side effects whatsoever.  My hearing, which was very poor to begin with has declined but seems to have leveled off the past two years.
 
Unrelated to acoustic neuromas but related to NF2, I had gamma knife treatment in August 2004 for a meningioma at Hopkins.  This was an incredibly positive experience.

Bill Macdonald

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Last Edited: Wednesday, April 06, 2005