A PHPT diagnosis at 40

I’m 56 years old — and it seems like another lifetime ago that I was forty, single, working a job I loved, and enjoying good health. While not especially athletic, I still participated in equestrian sports, cycling, and weights. I worked full time as a library reference department head in an academic library. Life was good!

My workplace covered twice-a-year bloodwork which proved valuable to early diagnosis. I got regular bloodwork to monitor mild anemia but otherwise nothing unusual. In the fall of 2002 though, my results came back with a new anomaly. My calcium level was slightly elevated — 10.4 (reference range 10.2). I figured it was a mistake, or a fluke, maybe I was dehydrated? I had no symptoms, so it was easy to rationalize. That spring it was just a tad higher, 10.6, so off to the doctor I went. My GP didn’t seem too concerned but sent me to both an ear/nose/throat (ENT) doctor and an endocrinologist. As you might guess, a battery of tests ensued.  While I don’t recall all of them, who could forget the 24-hour urine collection, and lugging a gallon of pee to the hospital? I’m sure more blood was taken to test parathyroid(PTH) and thyroid (TSH) hormone levels; I had a Dexascan to assess bone density; and an ultrasound of my neck was performed. The main findings were that my PTH was high, by about 30-40 points, and I had low bone density (osteopenia). Not. Good.

Bone density and PTH

You may know this already, but I’ll mention this because for me, it was a big deal. Wonky PTH levels can mess with your bone strength.  PTH does a lot of things, I suspect, but it tells the bones to release calcium, which then goes into the bloodstream. When PTH levels are normal bones maintain an optimal level of calcium. When the PTH level is too high, bones release calcium, which weakens the bones.  The released calcium goes into the bloodstream among other places, and that’s why high blood calcium is a symptom of PHPT. Another symptom which is “silent” is low bone density. There are lots of reasons you might lose bone density, but this is one of them.

So, my parathyroids are running amok

My diagnosis was PHPT and osteopenia (low bone density). Three doctors weighed in, and their advice was all over the map. My GP was skeptical that I had PHPT — it’s not a “young” person’s disease. My ENT said surgery wasn’t needed for levels this low. My endocrinologist, who in retrospect was the guy who knew his stuff, wrote polite but pointed letters to all of us explaining the professional guidelines for PHPT treatment — that at my relatively young age, with calcium likely to increase and side effects likely to worsen, surgery is indicated.

DIY Research

I was confused, if not a little freaked out, by these varied opinions. As a librarian I had research tools at my disposal — time for some DIY medicine! I obtained a book from the UPenn Libraries, entitled something like The Big Book of Parathyroid Disorders. I read it cover to cover. I also did a citation analysis to find out what universities contributed the most to the field of thyroid/parathyroid research. Fortuitously, that institution had a Thyroid Center with a patient hotline. The advice from the surgical nurse who worked the line: “Go to the best. You don’t want a surgeon that does three of these surgeries a year, you want someone who does at least fifty.” And she was quick to point out that one of the best surgeons in the country was in my backyard. At the time, Dr. Douglas Fraker at University of Pennsylvania (UPenn) had a near-100% success rate with first surgeries. He was the go-to guy for second surgeries (when the first surgery fails), and people came to him from all over the country when their first surgeries were botched.

I remember the consultation, Dr. Fraker’s confident and genial manner, and more tests. He ordered some sort of scan (nuclear?)  that localized the “problem gland” among my parathyroid glands. I made the one-hour drive down to UPenn and had the surgery. I remember post-op Dr. Fraker telling me that he had some trouble localizing the problem parathyroid gland tissue, but that the surgery was successful.  I went home the next day with a teensy scar and normal calcium levels. Within a week I was back at the gym, and for fourteen years my calcium and PTH levels remained normal. It was a textbook good outcome.

Fast forward to 2017

I continued to get regular bloodwork. In about 2015, I sort of knew something was percolating with my parathyroids. My blood calcium started to rise, and with each test I saw a slight increase in serum calcium. Finally, in 2017, it hit 10.4.  I went back to my endocrinologist in October of 2017.

It was all too familiar. We did repeats of most of the tests I had in 2002. This time my PTH level was high normal, but the Dexascan was alarming — my T-scores showed a dramatic decrease in bone density — I had osteoporosis, even in the forearm, which usually doesn’t lose density. Apparently bone-thinning in the forearm is a sign of parathyroid issues, so if they suspect you of having PHPT, they may order a Dexascan that includes the forearm in addition to hip, spine, etc.   Based on the overall picture the tests revealed, a recurrence of PHPT was suspected.

But this time it was a bit more complicated. A nuclear scan and an ultrasound failed to reveal an area of concern or localized tumor. Second surgeries are tough, in part because there is scar tissue from the first surgery that obscures the structures of the neck.  Surgeons don’t like to just “go in and explore” the neck, in part because the vocal cords,thyroid, and the jugular vein are delicate and easily damaged.  In these cases, nuclear, ultrasound, and other scans are critical to finding the overproducing tumor’s location to aid the surgery. I was in a bind. Surgery in the absence of a known tumor could be risky, but at the same time with my osteoporosis a “wait and see” stance was risky too.

Back to UPenn

I needed to find a surgeon and as before, I contacted UPenn.  Dr. Fraker had left UPenn just a few months earlier to assume a position at Rutgers U. I immediately started my own research to find a surgeon and ultimately decided to stay locally when I learned that Dr. Rachel Kelz at UPenn was Dr. Fraker’s protege. I scheduled an appointment with her.

The consult
When I met with Dr. Kelz, I was worried — osteoporosis at 55! I had a list of questions. Over the years I’ve learned that you have to do your own homework in making health care decisions — health professionals tend to be good folks, and they want to help, but they’re busy and sometimes they have their own agenda. In my first meeting with Dr. Kelz, I tried to be tactful but asked tough questions. What is your success rate? How are your procedures/techniques different from other hospitals? Why are these procedures better? Dr. Kelz did not bat an eye to any of these questions, and she was actually very jovial. It became clear I wanted her to be my surgeon. After my “tough interview,” it was ironic that she was hesitant to recommend surgery — with repeat surgeries, it is especially important to know where to look, and my scans had turned up nothing. As a last-ditch effort to find a tumor she ordered a 4D CT scan — in a 4D scan, the 4th dimension is time. The scan allows time for the hormone to accumulate and light up the image, I think. While she didn’t refuse to consider surgery, she said “if the 4D scan shows nothing, come back and we’ll talk about the advisability of exploring the neck.”

4D CT Scan

Not many places do the 4D scan, and even with a list of hospitals that do it, calling to schedule the appointment was a challenge. No one in the scheduling pool knew what it was, and even when I arrived for the appointment, the technician seemed to be confused when I tried to get confirmation that she’d be doing a 4D scan. She was on the phone for a half-hour getting directions from someone; it took so long that while sitting on the scan table, I started to make calls to UPenn to make sure the paperwork was right. When they finally did do the scan, it was over so fast I wondered if they had captured that time dimension. I was not expecting a good result.

At last we’ve found you!

The 4D scan did it! The upper right portion of my neck showed a tiny piece of gland/tumor that appeared to be tucked behind the thyroid, presumably overproducing PTH. Dr. Kelz was able to review Dr. Fraker’s notes from over a decade before. He had removed tissue from that area, and in the scan you could even see the sharp edge where the scalpel had been, right next to the tumor. Dr. Kelz posited that there may be a second lobe of the gland that was hidden in the folds of the thyroid and missed in the first surgery. We scheduled the surgery.

A second surgery

On the day of the surgery, I was waiting in pre-op when a familiar face peered into my room. Dr. Fraker! He had returned to UPenn (returned to his former position!) and would observe at my surgery while Dr. Kelz operated. After the surgery, Dr. Kelz told me that they had found a tiny tumor/node very close to the previous surgical site. They were even able to compare the tissue removed in 2018 to the lab slide from 2002 — same tissue — so this was not a new tumor/node, it was a tiny (leftover? microscopic?) piece of the original tumor that was removed in 2002. I guess it grew oh-so-slowly and over time bumped my calcium back up. When I left the hospital, my calcium level was 8.4 and it has hovered around there ever since. My surgical scar is about the same as it was in 2002 — barely noticeable. While I was sore the first day, I recovered quickly. Two weeks after my surgery I ran in a half-marathon!

Long term

I’m taking Fosamax for osteoporosis, a tough decision since new research shows some adverse effects of Fosamax for some folks that take it. In the end, though, my endocrinologist convinced me that the low risks of these problems (which occur mostly in folks taking Fosamax more than three years) is more than offset by the risks of a broken bone. Fosamax reduces the incidence of bone breakage — so I’m on it. With any luck my bones may rebuild what was lost in the years I had a hormone imbalance.

My advice:

• Get informed. Don’t rely solely on your doctor’s knowledge/experience. You can search the Web, but stick to the research. Be wary of anything but clinical research reports, or journals summarizing them.
• Go to the best. This is tough, because some insurance limits where you can go. Consider asking your doctor to write a letter advocating that you go out of network, if necessary. It will be cheaper than a second surgery, and better for your quality of life.
• Speak up! Ask questions, and don’t worry so much about offending your health professional. They’re tough, they can take it.

Thanks for reading my story, and I wish you all the very best.