hi, my name is matthew capogreco.

& this is my story

Most people never forget the first time they were told they had cancer. In April 2001, when I was 25 years old, I had that experience. I was in my office at school and received a call from my doctor personally, apologizing for his doubt. He told me I did have two glomus tumors on either side of my neck. Like everyone, I cried, not knowing what journey lay ahead. This is my story — it is a long and winding road — but I believe it ends up with my being the lucky one …

I had been asking my doctor to do an MRI after learning my father had two glomus tumors on his carotid. At that time, we had never heard of pheochromocytoma, and paraganglioma had been mentioned only a few times. Much like my father, I was asymptomatic; however, I was concerned because I was a vocal music teacher at the time and had been singing as a principal tenor in the Buffalo Philharmonic Church Choir. I was worried about my instrument and the difficulty several family members had had with these mystery tumors. I kept asking for the MRI. My doctor told me on several occasions that the possibility was extremely low that I would have these tumors at such a young age. He eventually ordered the scan after I explained that if he would not, I would find someone who would. I think he might have been just as shocked as I was when the MRI confirmed my fears.

We knew I had other family members with tumors but never really connected the dots that this was a genetic issue. We all approached our care as individuals, and I did the same. With the support of my father, I looked for a surgeon to help remove the tumor. My father urged me to come home to Syracuse to have the procedure performed by his surgeon. My insurance company told me this was not an option, unless I wanted to pay 20 percent out of pocket. Financially this was not possible and, with true youthful stubbornness, I told my father I would find someone in my network who would be able to do the job. I wish now that I had heeded his advice.

I was referred to a vascular surgeon in Buffalo who told me he had removed several of these types of tumors and that I was in good hands. He was very confident and did very little imaging before surgery. I was adamant that we have an ENT present during the surgery to help protect my vocal cords. Once again a doctor was telling me no, and I was saying that I would find someone who would operate with an ENT if he objected. So we went forward with the first removal in June 2001, an ENT on site, with what should have been a routine procedure. The estimated amount of time was 3 to 4 hours for a 4-cm tumor; it ended up taking 13 hours to remove an 8-cm tumor. About 10 hours into the procedure, the surgeon came out and told my father he needed to sign off on doing a full bypass of the carotid. Since bypass had not been discussed, my father told him they would have to do what they could and talk with me after the

procedure about options. The surgeon went back to work and sent the ENT to ask the same of my father, but again the answer was no. After working for several more hours, they were able to remove the tumor but not without damaging the nerve that controlled my right vocal cord. It would never regenerate.

While I waited to see whether my vocal cord nerve would heal, we started to explore options for dealing with the second vascular tumor. This time I did listen to my father and returned home to seek care from his surgeon, Dr. Syed Zaman. Dr. Zaman, head of vascular surgery at St. Joseph’s Hospital in Syracuse, was a savior for my father and eventually others in my family. While looking at my case and original MRIs, Dr. Zaman discovered what the radiologists and surgeons had overlooked in Buffalo, a glomus jugular tumor. This tumor was just behind the ear and was classified as a brain tumor. The plans had once again changed. This new development forced us to deal with this tumor first. I remember crying in the parking lot with my father after we heard the news. There had not been many times in his life that my father had been drawn to tears. When he asked me how I was doing, all I saw was fear in his eyes, and we both just rather lost it. We agreed that we would defeat this new challenge like we had all others, and we did!

The surgery for the brain tumor in September 2002 was successful. The procedure was performed by a local EN, Dr. Charles Woods, another doctor whom my family is proud to call a friend. He was able to remove the majority of the tumor, leaving only a small amount that was involved with specific nerves that would affect major functions. Post-surgery, I had slight loss of hearing, temporary loss of control in my facial muscles, loss of taste on the right side of my tongue, and a lump in my head the size of a football, which was also temporary. After three weeks of recovery, I went back to work part time; in four weeks, I was back on my mountain bike; and after three months of practicing face crunches in the mirror (yes, it was funny), I regained full control of the right side of my face.

Within the next year, we scheduled my left carotid surgery, which was performed masterfully by Dr. Zaman in September 2003. With this “final” surgery, we were hopeful that all was behind us. It was not until April 2007 that the topic of surgery came back up as we discussed what to do about the remaining portion of tumor left from the brain surgery. We were monitoring all of my scans (all of which were head and neck only) and determined the best course of action was to have Gamma Knife radiation. The tumor had grown just a bit, and we wanted to stunt the growth without major surgery again. This was once again a success, and we have seen a 10 percent drop in the tumor year after year. We had all but announced success and told everyone that I was tumor free when the real journey with this disease began.

During the time between my last carotid and Gamma Knife, my father was diagnosed with a heart tumor, classified as a paraganglioma. As the location of the tumor made surgery too complex for local doctors, he had a consultation at Mass General with the head of thoracic surgery. He traveled to Boston in 2004 to have his 12-cm heart tumor partially removed. The surgery was a grueling 15 hours, and the recovery lasted a month in Boston. They were able to take out all but 2 cm of the tumor, but it was positioned in such a way behind the heart that the last piece was deemed unreachable. My father was never the same after this surgery, and to this day, it breaks my heart to recall what he went through; but luckily, for me, he was there to help me through my next battle with tumors.

After my Gamma Knife in 2007, I started to have some side effects that we were hoping were the result of the radiation working. I had bouts of heart palpitations, elevated heart rate while working out, night sweats and an increased intolerance to heat. I had always been a “sweaty guy,” but I always chalked it up to being a hot-blooded Italian — not the case as we found out. At first I saw a cardiologist who was about to prescribe Toprol for the heart issues after doing a clean echo and stress test. It wasn’t until my father asked if he had tested my catecholamines that we found out the true culprit, pheochromocytoma.

I started to see an endocrinologist in the area who was instrumental in the diagnosis of pheochromocytoma and helped me find care at the National Institutes of Health. My catecholamines were way above 5000, and it was evident that the pheos were the cause of my symptoms. My endocrinologist told me there was a study being done at the NIH and that our case would be of great value to them. She contacted the NIH and through Karen Adams and Dr. Pacak, we found a new level of care and attention to our disease. This was also the first time the topic of hereditary gene mutations was introduced. We saw clearly how the tumors that were affecting my father and me were directly related to tumors in my great-uncle, my grandfather, two of my father’s sisters and eventually my brother. My sister has not developed tumors, but she is a genetic carrier. The Capogrecos now knew what plagued them: the SDHD gene. Wow, this was a biggie!

In November 2007, I went for my battery of testing at the NIH and found out that I had masses on my adrenal glands and one on the top of my heart. The dangerous levels of hormones being produced were coming from the pheochromocytomas. In February 2008, the adrenal tumors were removed laparoscopically at the NIH. Within weeks, I was back on my feet. I found over time that the intolerance of heat and sweatiness that I had dealt with for years was actually a result of the pheos. To this day, I get cold like the rest of the world and am happy for it.

We decided to be proactive with the last of my tumors in December 2008. With the help of the NIH and Suburban Hospital, I had a successful removal of my heart tumor. The surgery was open heart and involved a single bypass, but I have to say it was really not all that bad. I am grateful to the surgeons across the board and consider myself to be lucky after five surgeries and one Gamma Knife procedure. Unfortunately, for my father, the outcome was much worse.

My father was a man who was always busy, and for the first time in his life something forced him to stop. After his 2004 surgery, he had a pacemaker implanted to help his heart, and he suffered a stroke. After years of dealing with low energy levels, difficulty with controlling blood pressure and various side effects, my father made the decision to move forward with removing the remaining tumor behind his heart. One major contributor to this was the discovery that, since his last surgery, the tumor had grown from 2 to 6 cm. Very few doctors were comfortable dealing with a second surgery in such a difficult area. He found support at MD Anderson in Houston, Texas. In September 2009, he headed there for surgery. We had been through so many so far that we were almost on autopilot, so we decided to have my father and stepmother go down alone. The doctors were confident, our family was well-seasoned in dealing with difficult surgeries, and my father seemed content with the decision. After a successful removal of the tumor from behind my father’s heart, they spent the next 9 hours working to get it beating again. In the end, it would not sustain on its own, and my father succumbed, far too young. It was then that I realized that nobody should fight as hard as he did and lose the battle.

It was only three weeks after my father’s death that I attended the Pheo Para Alliance Conference in Parsippany. I had previously been asked to host a round-table discussion and was even more determined given the events of that month. The conference was something that would change the course of my participation within the pheo community. The presentations by the doctors were full of information, some of which I am still sifting through. There seemed to be a real identity to our disease for the first time. The truly remarkable moment came in talking with other patients during the conference, especially during the round-table session. It was apparent that there were many commonalities, but one common trait was concerning: the lack of information and support for our disease. With the aid of Karen Adams, Dr. Pacak, Wayne Zandbergen, and several members of the Pheo Para Alliance, four patients were
approached. Allen Wilson, Amy Pitzer, Laura Becktel and I took to the prospect of forming a group that is now known as the Pheo Para Troopers.

As for my part of the story, I want to come back to the first thing I said about being
lucky. I have lived by a phrase for the better part of my adult life that was imparted to me by a teacher of Okinawan karate. Taken from the Japanese phrase “Nana-Korobi, Ya-Oki,” it loosely translates to “Seven Times Down, Eight Times Up.” For every time you get knocked down, you get back up. I consider myself lucky in my disease because I am still standing. Often people mention to me that my journey must have been tough and comment on all that I have gone through. I am forever heartbroken that I can never sing in the way that I used to and more so that I lost my best friend and father in the battle, but in the end I still consider myself lucky. Luck does not come without a fight, and to pick yourself up every time you get knocked down takes courage and determination. I am inspired by those that I have met with our disease because they all have that type of courage. Our pheo community, our Pheo Para Troopers, offer such inspiration to fight harder and do more. For those who have gone before us, for those who are in the fight now, and for those we will save in the future, I pledge to be a Pheo Para Trooper for life.