Back in December 1995, I was diagnosed with a “glomus jugulare” tumor that was behind my right ear. I had surgery in Jan. ’96 to have it removed (all the pics below are clickable to see a larger version).
It was an 18 hour surgery, and a very difficult time. In order to remove the entire tumor, they had to remove my right ear drum, so that left me deaf on that side. And the tumor was wrapped around the facial nerve, so for about 6 months after surgery, I had complete paralysis on the right side, below is a pic of me trying to smile, and the entire right side of my face is “dead”.
They were 99% sure that they removed all of the tumor, so just to be safe, ever since then, we’ve done follow-up MRI scans once every few years, just to keep an eye on things and make sure nothing was coming back. The good news is that things have remained stable in the area behind my right ear, however in the follow-up MRI done in Jan 2012, they found 2 more tumors in my neck. Here’s a picture from that scan:
In the above photo, it’s as if you are looking at me from the front. So the big white blob on the right side is actually the tumor on the left side of my neck.
These are called “carotid body tumors”, and I was diagnosed with one on each side – these are also called “paragangliomas” (hence the name of our blog). On each side of your neck the main carotid artery comes up, and then just under the jawline, they “bifurcate”, or split, into the internal and external carotid arteries. This is where the tumors were sitting, right at the “bifurcation”. The one on the left was the larger one, about 3x3x5 cm, and the one on the right is smaller. The doctor that broke the news to me explained how there is a very good chance that there is a genetic link, and that would be the reason for these current tumors, as well as the glomus from 1995. We started asking around in the family, and it turns out that my Grandma Blake had a tumor removed from her carotid artery way back in the 1950’s.
Also, my sister Amy was concerned as she’s had lumps in her neck for years, so she went and had an ultrasound, and it turns out she has the exact same types of tumors, one on each side like me.
Initially, I saw a couple different surgeons in Denver, and although we liked them a lot, they both told us that they do not treat a lot of these each year – one said about 6 patients a year, while the other one said he had treated about 75 patients over the last 15 years who had these kind of rare tumors – about 5 per year – not a lot!! I did some further research to try and find someone that had more experience with these kinds of tumors, and found a team out at the NIH (National Institutes of Health) in Bethesda, MD, where they have an ongoing clinical research protocol specifically for what I have. I contacted them and got a reply back within hours that they wanted to see me. So we made the arrangements, and Amy and I went together to the NIH in mid-April of 2012. They don’t mess around at the NIH – it was a full 4 days of testing – blood draws (about 15 vials), 24-hour urine test, then the scans:
- MRI of head and neck
- MRI of chest, abdomen and pelvis
- Full body CT scan
- 3 separate full body PET scans – each time they injected us with some kind of crazy radioactive gunk
After all the scans and tests were done, we met with the main doctor and his team. The good news for me:
- There were no other tumors elsewhere in my body (this was a real concern, that there could be more)
- The tumors that I have are “non-functioning”, meaning they are not releasing catecholamines or metanephrines (this would have made things much more complicated and scary)
- There is hope that with continued research, there is the possibility of finding a CURE for this within the next 5 to 10 years!
The bad news, was essentially what we already knew, except for the fact that they were not sure if the larger tumor on my left side was actually one tumor, or possibly 2 or 3 separate tumors all jammed together. They also saw a small “nodule” just behind my thyroid on the left side. I got genetic testing done, and it returned as a positive for the mutation called SDHD (Amy also tested positive). The other huge concern, that weighed on me heavily, was that my 2 sons, Chris and Logan, each had a 50% chance of getting it. We had them both tested, and thankfully, they both came back negative, which was a HUGE RELIEF!! My brother Matt also was tested and came back negative, so we know that the mutation has stopped, and won’t be passed on.
So for me, initially they set a quick surgery date of May 10. But then the surgeons decided they wanted an additional test done before surgery, called an “occlusion test.” So the surgery got postponed, and ended up being pushed back into August. It ended up that Amy and I were both scheduled for surgery on the same date in August, 2012. For me, I went through the following:
- Balloon Occlusion test on Monday, Aug. 6 – this is where they inserted 2 catheters into my femoral artery (by my groin), then snaked them both up into my head – one going to the larger tumor in my neck, on the left side, and one on the right side. Then they inflate a small balloon on the left, and monitor the bloodflow – the purpose of this is to make sure that blood is still flowing adequately up to the brain, in the worst case scenario that they have to remove the entire carotid artery on the left side during surgery. Unfortunately, I had to remain conscious during this test – it was not at all fun, and that afternoon I had the worst headache I’ve ever experienced in my life. Cara had to hussle around Bethesda to get to a pharmacy and get pain meds for me. But thankfully the tests came back as we had hoped, so I was cleared for surgery.
- Surgery happened (for both me and Amy) on Wed, Aug. 8. There are more details and pics in the blog posts, but surgery went well – they were able to get the entire tumor out, without any complications or issues. That afternoon, I was up and about, and on the road to recovery.
- We all (myself, Amy, Cara, and our parents Bob and Di) flew home on Sunday, Aug. 12, and I was able to resume work the following week, pretty amazing!!
I went back to the NIH for follow-up testing in October 2012, and they confirmed that the left tumor was completely gone, and it looked good. For my remaining tumor on the right side, the fortunate thing for me is that we have all of my MRI scans going back over the years, so they are able to compare images going back. From what they see, this tumor has not grown at all in the last 7 years, so for now, the plan is to just leave it alone and monitor it. They made it very clear to us that “once you’re a patient with the NIH, you are a patient for life.” So basically, I was told that I will always have to plan on going out to the NIH about once a year for follow-up tests and scans. Since that time, things have changed a bit, I think primarily because of budget cutbacks, and the NIH will only bring you back for testing if you have “active disease”, which just means that you still have tumors.
Since I still have the one tumor on the right side, I have continued to go back to the NIH about once a year for scans.
Another interesting aspect of the genetic mutation I have (SDHD), is that there is a direct correlation with altitude. The higher the altitude, the worse it’s supposed to be, in terms of tumor growth and the number of tumors. I grew up in the mountains of Evergreen, CO, at about 8000 feet, and had lived in Fort Collins ever since 1991 (at about 5,000 feet). So when the doctors made it clear that I had to get out of Colorado, we had a family discussion. We ended up sticking around in CO to let my older son finish High School. He graduated in the Spring of 2013, and then we sold our house that summer and made the move down to sunny Sarasota, FL! Now I live at an altitude of about 10 FEET.
Will try and keep this site updated with new entries and updates, and pictures! Please be sure to go to the main blog page (https://paragangsters.wordpress.com/) to see updates.
One other note – I decided that it was only fair to give some names to my 2 tumors, so I would like to introduce you to: Mary Kate and Ashley!! It will be sad to see these 2 bitches go…. not..