To round out some of the reader accounts and other reports collected in his thread, occasioned by Garry Shandling’s death, here without further setup are statements from some authorities.
1) The “Parathyroid Peeps.” Barbara Creamer, one of the founders of a patient-advocacy group called Parathyroid Peeps, writes:
We are 3 San Francisco Bay Area women who suffered from primary hyperparathyroid disease. We all had our surgeries performed at the Norman Parathyroid Center in Tampa, Florida - one of us in 2011 another in 2013 and the third most recently in 2014 to remove parathyroid tumors. The surgeries all took less than 21 minutes. You can read our stories here. We didn't know one another prior to meeting in an on-line support group...
There were number of problems we personally experienced that contributed to a delay in diagnosis and surgical treatment. Though cured ourselves, we felt compelled to educate others about the disease to move the larger conversation forward to create change. Thus our education and advocacy group Parathyroid Peeps came into being. You can read about our mission here….
One major issue [in missed diagnoses] is that laboratories do not necessarily take into consideration a person's age when reporting normal calcium values - the first lab test that indicates that one or more of the glands are not function properly. As a result, high values for patients 30+ are often ignored and further testing is delayed.
Another is the "wait and see" model of care that many doctors ascribe to due to the American Association of Endocrine Surgeon's and the Endocrine Society's Positions and Guidelines.
Surgical techniques also vary dramatically and are surgeon specific…. As a result, many patients who are referred to their local surgeons, who are not experts, suffer needlessly. We hear reports of good parathyroid glands being removed inadvertently, 3 1/2 gland being removed unnecessarily causing HYPOparathyroidism ( devastating condition) and/or a second rogue gland being missed. That is why we are also advocating for improvement for surgical treatment.
***
2. From the treatment center. As best I can tell, most of the parathyroid surgeries that are done anywhere in the United States are now done in one place, the aforementioned Norman Parathyroid Center in Tampa. (For instance, check out this startling chart.) As mentioned before, I have no experience them and am not touting this center or anyplace else. But I received this note from Dr. Deva Boone, one of the staff surgeons at Norman:
I am one of the six surgeons at the Norman Parathyroid Center in Tampa, Florida. I wanted to say thank you for highlighting the disease and allowing your readers to share their stories.
Most of our patients have had high calcium levels for years prior to seeing us, due to the "watchful waiting" approach of their physicians. We know from experience (we operate on over 60 patients a week with parathyroid disease) that high calcium always leads to problems over time. Its effects occur throughout the body - on the brain, bones, kidneys, heart, etc. and it can make people miserable from extreme fatigue and body aches. There is no reason to wait for serious complications like kidney failure or heart attacks to pursue a curative operation. We know that the longer you have it, the worse it gets.
Our hope is that your article and others like it will empower those who are currently receiving "watchful waiting" and convince them to pursue the treatment they deserve.
3) Watch those changing age-adjusted levels. A reader makes a point stressed in other messages I’ve received:
One thing I don't think I've seen mentioned is this important fact - as you get older, your [target] blood calcium numbers go down with age. As a result, when the lab says your numbers are in range, this may not be true. It may be in range for a 40 year old, but as an older patient, that number would indicate a parathyroid problem.
That's exactly what happened to my sister. For 5 years, she kept complaining about a myriad of symptoms but because she had other health issues, they were always attributed to side effects. Two years ago, one of her doctors mentioned that her blood calcium number was a tiny bit high, but so slight that it was no big deal.
My sister decided to to check it out herself and quickly found that that a number in that range was not "slightly elevated" for an over fifty woman and that every symptom she had experienced for the past five years was attributable to hyperparathyroidism. She made an appointment with an endocrinologist the next day and a month later had surgery. It was a miracle how much better she immediately felt.
The takeaway here is that once you hit 50, ask for a blood calcium test, especially if you're female (1 in 50 woman over 50 have a parathyroid problem). And ask to see the numbers yourself and know the correct range.
***
4) Other tricks of diagnosis. A doctor in New Zealand on further fine points:
Although not in the US or an endocrinologist I am a physician, have worked in endocrine and have few comments.
As mentioned it is commonly found in elderly women, not middle aged men. In addition, hypercalcaemia is far more common than hyperparathyroidism. [JF: This is, a high blood-calcium level doesn’t automatically mean you have this disease.] That’s (presumably) why many readers have stories of being “ignored”.
OTOH, the key to understanding endocrinology is feedback loops and getting to grips with “inappropriately normal” results. For example, a person with a corrected calcium of 2.9mmol/L (ref range: 2.4-2.6) and PTH of 6 pmol/L(ref range 1.5-6) has an inappropriately normal PTH – it should be fully suppressed by the elevated calcium (in order to return the serum calcium level to normal). This is difficult for anyone to get their head around, especially if it isn’t part of your daily routine workload. [JF: If you’re healthy, you shouldn’t have both a high blood-calcium level, and a high parathyroid hormone level. If the first is high, the second should be low, and vice versa.]
My final piece of wisdom is psuedopseudohyperparathyroidism is a poor word choice in “hangman” games… albeit it does scare everyone at the beginning.
This is part of a brand-new Thread, kicked off for this reason:
As I mentioned in yesterday’s Note, I’m not in a position to do follow-up reporting on a topic I mentioned after Garry Shandling’s death. That topic was the disease Shandling suffered from, hyperparathyroidism, and why it deserves more attention than it gets. Those posts also explained how my own experience with this disease had taught me that it was an exception to the “when in doubt, wait” strategy I generally apply before taking dramatic steps. The longer you wait before getting the operation that is the only known cure for this disease, the more damage it will do.
A lot of people have sent in their positive and negative reports. Notes maestro Chris Bodenner and I will host selections in this thread. You can reach him via hello@theatlantic.com.
“Don’t be bullied into waiting.” A reader in Los Angeles writes:
My sister and I have both had surgery (she twice) to remove a total of five tumors between us … thereby revealing the inaccuracy of the endocrinology community’s assertion that there is no genetic predisposition to the condition. (Our brother is currently suspect and being tested.)
I lost productive years of my life arguing with my [one major LA-area medical center] endocrinologist about whether my calcium levels were “high enough” to warrant action. Finally found a guy at [another center in the area] who believed me when I said I was suicidal from the symptoms. I absolutely would be dead if he hadn't listened to me, and will be forever grateful even though —
— one of my vocal cords was paralyzed while “spelunking” for the second tumor. Sounds much like your experience with your wandering para; you were just a little luckier. I lost my career because my speaking voice is now raspy and annoying, usually failing entirely by mid-day— but I would do it again.
My sister and I are both medically savvy (she is an R.N.) and capable of advocating for ourselves, but we still had trouble getting endocrinologists to take us seriously. We do not understand why that specialty is so reluctant to admit that there are a lot of sick people out there suffering needlessly.
We are heartened by the part you have played in spreading the word, even if you must now bow out of the arena to get back to the rest of your life, and we thank you for joining us in encouraging patients not to be bullied into waiting.
Update from a reader who works as a geneticist (posted by CB):
I just wanted to note one inaccuracy in one of the readers comments on hyperparathyroidism: “My sister and I have both had surgery (she twice) to remove a total of five tumors between us ... thereby revealing the inaccuracy of the endocrinology community’s assertion that there is no genetic predisposition to the condition.”
While I am not an endocrinologist, I am a geneticist, and the fact that this person and his/her sister both had tumors doesn’t prove anything about the genetics of the disease—neither in the population at large nor in the specific case of these siblings. It is impossible and improper to generalize anything about the cause of their tumors or about these tumors in general based on their one specific case.
For example, if I am correct in assuming that these siblings grew up together, then they shared a rather similar environment. According to the reader, the endocrinology community hasn’t found any genetic factors that influence predisposition—that doesn’t mean there aren’t any; it just means that if there are, their effects are not large enough to be able to be detected in the studies, or perhaps that several factors need to be found together in just the right combinations which happened to appear in these siblings but rarely otherwise, or that these combinations of genetic factors aren’t rare but need to be in just the right environment in order to produce tumors, etc.
Biology is messy. If indeed this disease runs in the reader’s family, then I would encourage them to talk to a genetic endocrinologist about studying their family. Targeted family or pedigree studies can be quite powerful in identifying genetic factors and can be an important complement to studies of larger populations.
***
“Feeling like shit.” A less positive experience from a reader in Seattle:
I also had the PT surgery three years ago from the most experienced surgeon in Seattle. Three hours of digging in my neck. They could not find it and told me to wait till it gets bigger and have a better chance of finding it.
Feeling like shit.
Glad yours turned out successful.
***
“Your situation is anomalous.” A doctor writes:
Your situation is unusual, because of such a hard to find tumor. Both you are Mr. Shandling were a bit anomalous because of gender; the disease is most prevalent in older women.
Making an accurate diagnosis for elevated calcium levels is important as medications, cancers and other diseases can cause it.
When I completed training almost 30 years ago the standard was to operate unless there was a very compelling reason not to. That has changed to operating when there is some reason, e.g. bone loss, kidney stones, rising levels….
All should seek a surgeon with experience and where intra-operative PTH testing is done (I think this is universal now). [JF note: This refers to real-time readings of parathyroid hormone (PTH) level while the operation is going on. The PTH level declines very rapidly as soon as a diseased PT gland has been cut out. So if it stays high after one bad gland is removed, that tells the surgeons they have to look for another.]
One of your respondents mentions a place in FL. I have seen their website and despite some distaste for their self-promotion, I bet they do an excellent job. And your readers should know that 5 hours is highly unusual (lucky you!); an hour or less is more typical.
This gets into the tricky terrain of recommending doctors or hospitals, which for obvious reasons I’m not going to do. As with any complex procedure, it makes sense to choose a facility and doctor that perform the operations frequently rather than rarely. When we were asking around, eight years ago, the UCSF hospital and Dr. Quan Duh came highly recommended on those grounds (and I have since heard from other patients grateful for his treatment there).
***
“The place in FL.” I deliberately removed the name of this “place in FL” from a previous reader’s message, because I’ve had no first-hand experience with it. But because it absolutely dominates on-line discussions of parathyroid issues, and will come up at the top of any search you do, and actually runs the site parathyroid.com, there’s no reason to be coy. The place is called the Norman Parathyroid Center, and it is in Tampa. My friend Phil Baker of San Diego, a tech industry veteran and writer, did a long post last week, after Shandling’s death, about his own experience at Norman. You can read it here.
Phil Baker ends with an observation on health care in the internet age:
When [the local surgeon in San Diego] ran his tests and was unable to determine which of the four parathyroid glands needed to be removed, he suggested waiting six months until it might become clearer, as the disease progressed.
So my options came down to relying on my local doctors and living with the disease for another six months or more, or relying on the advice from the [Norman Center] website…. [Which Phil then extensively researched.]
A few weeks later my wife and I flew to Tampa and went to the clinic the next morning at 5 a.m. … I was given one test, received a light anesthesia, and had the short operation. In my case it lasted 19 minutes. About a half hour later when I awoke, the doctor gave me a photo of the diseased parathyroid, and told me I was completely cured, and could leave. We walked out of the hospital at 11 a.m. and went out to lunch.
What I learned is that when it comes to your own health, you need to take charge and do your own research. As good as our doctors may be, they’re not always aware of other options, beyond what they are familiar with in their own community…. Your doctor doesn’t have the time to research every disease and option; therefore, it’s up to you.
Yesterday, in the more formal, non-Notes precincts of the Atlantic’s site, I did a post occasioned by Garry Shandling’s death. It’s about the disorder Shandling said he had been diagnosed with, shortly before he died from a heart attack at the (relatively) young age of 66. The disorder is hyperparathyroidism, which I had never heard of before I learned I had the same condition ten years ago.
The reason for my post was to emphasize a lesson-of-experience I wish I’d known a decade ago, and that might help others now. The lesson is that “watchful waiting,” generally so wise an approach to life, is a mistake in this case. Once you’ve gotten indications that you have a parathyroid gland problem, mainly through a higher-than-normal calcium level in your blood, waiting is the wrong strategy. The odds are that you’re doing yourself real damage with every week you delay before having the bad parathyroid gland surgically removed.
Some details of how and why are in the main piece. (Including why it was a mistake for me to roam around in China for three years, as this condition got worse.) My point in telling the story was to spare others my predicament of (a) never having heard of a certain disorder, and thus (b) thinking, as I did, Aww, what’s the rush? on the question of surgery.
Here are two of the many reader accounts that have arrived overnight on this theme. First, from a woman in Alaska:
I suffered for nine years going to an endocrinologist who every time I saw him said "we'll wait a little longer and repeat the tests again...for 9 years! I got sicker and sicker, and I really think I was dying.
A friend of mine who lives in a small town in Alaska had the same disease, but he researched it on the Internet and ended up going to [a well-known parathyroid center in Florida, which I’m not naming because I don’t want to get into the medical-referral business—JF]. They removed his adenoma and he was immediately well again.
On that strength I self-referred to [the same place] and had surgery on February 2. Many people were skeptical, but I forged ahead. I am a new woman!
And from another woman:
Thank you for bringing this scourge to light, both to patients and to physicians. But so much more needs to be said. So many other people are out there suffering needlessly either due to their own ignorance or their physician's.
Hopefully you will write a follow up article that will get picked up by the broader news service. There needs to be a discussion of the outdated criteria for making the diagnosis. This is a whole lot more common than originally thought.
Doctors also need to get rid of the old idea of watch and wait. In experienced hands the newer procedures are much less traumatic than they used to be so there is no need to wait and hope things get better while risking kidney stones, heart attacks and a dozen other things instead.
Research needs to be done on possible genetic links. There are too many people showing up with this in families that are not part of MENs 1 or 2 etc. [JF: MEN 1 and 2, for Multiple Endocrine Neoplasia, are glandular disorders with a known genetic link.]
And finally, doctors need to entertain the idea that patients with persistent symptoms may have a 2nd bad para that was missed (maybe hiding in the chest like yours) or those with recurring symptoms after initial resolution may have additional adenomas spring forth like mine did.
For busyness-of-life reasons, I’m not likely to write more about this topic. But I’m happy to do what I can to call attention to it, mainly because it’s an exception to the generally sound “when in doubt, wait” approach. Thanks to these readers and others, and thanks to Drs. Brian McBreen, of Washington, and Quan Duh, of San Francisco, for diagnosing and then treating my own parathyroid case.
/media/img/posts/2016/04/PPPeeps/original.png)
/media/img/mt/2020/08/Atlantic_passport_v2/original.png)
/media/img/mt/2020/08/Atlantic_Kamala_wiki_v1/original.png)
