Patient Stories

Looking back to 1995, it becomes more and more clear that I had been suffering
from pheo symptoms for more than 12 years without knowing it. Knowing what
I know now explains nearly all of the anxiety, stress, depression (you know those
“feelings of doom” they talk about?), and the generally crazy emotions I had
experienced in my 30s and 40s. Who would have guessed, eh?

Having suffered increasingly bad symptoms in late 2006 at the age of 43, I once
again went to my doctor, complaining of a general malaise, anxiety, and night
and day sweats. At this point I asked for a full health check. He told me that I was
a perfectly fit young man and there was nothing wrong with me, even though I
was heavily perspiring on a reasonably cool day in Ireland. My doctor reluctantly
agreed to take my blood pressure and was visibly shocked by the result. He
immediately prescribed beta blockers to suppress the high blood pressure. I,
however, knew there was more to my problems than simple hypertension.
Going against his rather blinkered advice, I sought out a second opinion.

At work I was holding a meeting with staff on a cold October day and was, as
usual, perspiring heavily even though I was only wearing an open necked shirt.
The window was open and a fan was running in the background. My staff’s lips
were almost blue with the cold, and yet I was still overheating. Something was
definitely not right.

A phone call later that day, informing me that I had pheochromocytoma proved
that all was not well. I was directed to go to the hospital immediately. Having
little knowledge of what was happening or why, I was admitted to the Royal
Victoria Hospital on October 18, 2006, for alpha blockade.

Interestingly, having conducted a Google search my symptoms during 2003, I
had asked my doctor if pheochromocytoma might explain my condition. His
response was to laugh and tell me that this disease was so rare that it almost
didn’t exist. In his considered opinion, I was simply overly anxious and suffering
work related stress and anxiety (this was after describing four incidences of
what we now know were profound and life threatening adrenal attacks).

I often wonder if that three year lapse before diagnosis was an opportunity
missed and might have prevented my lung and liver mets, although the folks at
NICHD believe it would have made no notable difference to my disease
progression.

After 2 weeks of learning to cope with the side effects of phenoxybenzamine, we
learned that I had 3 tumors, a primary on my adrenal gland and 2 smaller tumors
on the lower lobe of the left lung. Given the proximity to Christmas and the
National Health Service in the UK being what it is, I was advised the operation
would take place in early 2007 to remove the primary. The operation was
cancelled on 2 occasions due to a lack of intensive care beds, but it finally went
ahead in March.

Once inside my abdomen, the surgical team found that the tumor was not on the
adrenal gland at all but actually located on the paraganglion. It had also attached
to my duodenum and wrapped itself around both my aorta and renal artery. It
took one team of general surgeons and another team of vascular surgeons 11
hours to remove a very aggressive 12 cm diameter tumor. Anecdotally, the
surgery was almost terminated on 3 occasions due to the aggressive adrenal
response from the tumor. Thankfully, they persevered and prevailed.

In May I had a second surgery to remove the 2 smaller lung tumors and this
turned out to be a much easier operation for me to recover from and deal with
than the first. Upon the return of the pathology, however, I was advised that as
the tumor tissue of the primary and secondaries were the same. My “cancer” had
metastasized and would progress rapidly. I would to be dead before Christmas.

Understandably shocked, I spent 24 hours making peace with my maker and
family, only to be told that this intern had been completely incorrect as
pheochromocytoma did not progress in the same way as other cancers. Instead I
was now tumor free and should make a complete recovery.

I find it interesting to note that my local endocrinologist still refers to the
condition as pheochromocytoma, although we now know it was clearly
paraganglioma and highly prone to recurrence. To be fair, paraganglioma only
became a distinct sub category of pheochromocytoma in the medical literature
relatively recently, but the distinction is important, particularly as para
demonstrates significantly higher rates of malignancy.

I finally stopped taking phenoxybenzamine alpha blockade in June after some 9
continual months of the usual unpleasant side effects. I was glad to be free from
its hold. Phenoxybenzamine is a life saver in the fight against pheo, but its side
effects can be debilitating.

I then spent 15 months in phenoxybenzamine free bliss until October 2008,
when my endocrinologist asked me to have an MRI scan. This showed a 3cm liver
tumor right next to my inferior vena cava. I had been blissfully ignorant that my
disease was progressing once again, as my endocrinologist had felt it “better not
to tell me” that my catecholamine levels had been inexorably rising over the
previous 12 months. Alpha blockade began again, and I was scheduled for a
major liver section in late January 2009.

Given the tone of the medical specialists I was referred to, it was clear this
operation would both seriously undermine my health and limit treatment
options going forward. Having done some research on alternatives, I contacted
the NIH in Bethesda to inquire about the possibility of using what was until
recently, experimental RFA, to treat my tumor.

The NICHD (NIH) accepted my case. With my local surgeon agreeing to proceed
should the RFA fail, I postponed the liver section and headed to the States,
entering the whirlwind of diagnosis that is the NICHD. My local endocrinologist
was much less supportive of this choice, however, and strongly recommended
that I proceed with the liver section immediately. I, however, felt the RFA option
had to be explored before I could be at peace with making an informed choice.

To begin with, however, the favored approach at the NIH was also to opt for liver
section due to the close proximity of the tumor to the IVC and no clear margin of
safety to “burn” the tumor without detrimentally affecting the IVC as well. If the
burn margin was not wide enough, the tumor would return. Then too if the RFA
was too aggressive there was a strong chance of damaging the IVC itself which
would ultimately result in death.

With limited options we arranged to head for home until it was suggested that I
try MIBG at UPenn to see if this might encapsulate the tumor and give clearer
margins of safety. Thankfully, it did. In May of 2009, I underwent an
embolization of the artery feeding the tumor prior to the actual RFA. Both of
these procedures were performed by a fantastic team at the NIH and were very
successful, albeit with an extremely aggressive response from the dying tumor
that once again caused my medical team a lot of headaches, resulting in my
spending nearly 2 weeks in intensive care.

In June 2009 the RFA was declared a success, and I am now lucky to have been
tumor free for two and a half years, which is much longer than expected. The one
small downside to the process was that I became anemic from the MIBG infusion
and remain so, with the general impact that has upon my ongoing energy levels.

I am now classified as sporadic metastatic paraganglioma with no history of the
disease within any other members of my family and no known genetic pheo
markers. I attend the NICHD twice yearly for check ups and rotate this with
catecholamine tests back in Belfast to monitor any hormonal developments, with
my main marker being elevated metanephrine levels. Basically, I’m checked
every quarter with the intent of catching any new developments early.

The disease progression has thankfully been slow of late, and I like to hope it will
not return. I am, however, often reminded by my physicians that this disease is
for life and more than likely to come back as strongly as before.

I had expected to marry during my thirties, but unknown to either my girlfriend
or myself, the depressive and psychological impact of the disease had a
profoundly negative effect on my ability to deal with emotions and feelings
properly. Basically, I coped badly and often thought I was going mad. I, therefore,
remain single and unmarried. My now ex-girlfriend understandably moved on
long before I was diagnosed and settled down with someone else. That can be
kind of tough to deal with when I am now, once again, “normal.”

I am still advised not to have children, as the roulette wheel of being sporadic
metastatic means we have no idea what gene mutation or deletion affects me. So
I am deemed likely to pass the disease on to any progeny. Consequently, I choose
not to take such a risk with an unborn child’s life, although I understand that
others might approach this issue with an entirely different view.

Every day is a bonus and I try to make them all count. I am noticeably calmer
now that the tumors have been removed, and I can look back and see how crazy I
was on occasion through no particular fault of my own.

I truly am one of the lucky ones. Through a mixture of making my own luck and
the great skill of some medical professionals, I am fortunate to be as well as I am.
I also know, however, that this disease has had a profound impact on most of the
important elements of my life and continues to do so.

I have only recently become aware of the punishing effects of the 26 PET, MIBG,
MRI, and CT scans that I have had in the last 5 years, which it now seems
substantially raises the risk of blood cancer further down the line. I guess if you
are living with pheo or para, it always seems that if its not one thing that gets
you, it’ll be another, eh? I realize that one does have to learn to live with this
disease and that’s what I continue to try to do, along with a lot of finger crossing
and a little prayer both for myself and for all of you fellow pheos out there.

Keep well and keep fighting!