You’ve been diagnosed with primary hyperparathyroidism, perhaps after struggling with various symptoms for many years, with this being the root cause that perplexed your medical professionals. Maybe you joined a social media platform to compare notes with others who have been on a similar journey. After hearing other patient stories, you might be feeling more confused than ever. A few mention having had failed surgeries and are now suffering the consequences. That’s scary and now you have doubts and questions. You want to understand why this happens and want reassurance that this doesn’t happen in your case. Don’t all surgeons perform the surgery in a similar way? What questions should be asked to help ensure a surgery is successful?
To help answer these questions and concerns, we asked parathyroid experts Dr. Jose Lopez and Dr. Doug Politz, who have performed surgeries on thousands of patients and helped cure many whose first surgeries were not successful, for guidance on this topic. We hope this information is helpful to you as you journey onward.
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By Dr. José Lopez and Dr. Doug Politz
Top 3 reasons the first operation fails.
1. Deep upper tumor.
This is definitely the most common scenario we have seen over the years. The upper parathyroid glands can lie farther back in the neck. Surgeons who don’t do this all of the time will get nervous to work back in that area because that’s where the nerve to the voice box runs. It’s a healthy fear! Voice issues can be life-changing for the patient. Having been in that area so many times over the years, we are very comfortable working around this nerve and performing re-operations to fix this problem for patients while preserving their voices.
2. Second adenoma.
Just took out the one on the scan and a second one is still in the neck. As long as the first surgeon didn’t spend hours and hours looking for it, stirring up a lifetime of scar tissue, this is not such an awful thing. It’s not a character flaw to take out what is on the scan and stop there if you don’t have a lot of experience day in and day out in doing parathyroid surgery. You’re going to cure 70-75% of the patients with this disease by doing just that!
3. Looked but didn’t find all four. (Only found 3 and called it hyperplasia).
Surgeons whose experience is limited and is still growing don’t always find all four glands. They may find 2 or 3 and not the fourth and figure it must be hyperplasia. It’s hard to find these tiny little things. The subtle differences between parathyroid and other tissues in the neck can be hard to appreciate. And then the subtle differences between normal and diseased glands can be challenging. It takes thousands of these to notice that a diseased gland is sometimes just a little bit larger than a normal one, but it’s a little bit redder, a little bit firmer, a little bit chunkier, gets angry immediately when you barely touch it with a cotton ball. We’ve seen tens of thousands of these glands. So the first surgeon may have only found 2 or 3 normal glands, and the tumor is still in there!
All 3 reasons above can reflect limited experience, and it is this one factor that accounts for the overwhelming majority of surgical failures!
Some questions you may want to ask your surgeon during your consultation and prior to your procedure:
1. How many of these parathyroid operations have you done in your lifetime or in the last year?
Most surgeons will not have a problem answering this. If they cannot give you an approximate number, it’s probably because they don’t know offhand or they would rather not share. On the contrary, if they have done many parathyroid surgeries, they will be happy to share it with you. In order to have good outcomes doing parathyroid surgery, it is generally accepted that at least 50 PARAthyroid operations should be undertaken per surgeon per year. Although this number is not a rule, it is the consensus of most experts in order to have good outcomes. As you see we didn’t say great outcomes or perfect operations. In our opinion you should be doing at least two parathyroid operations a week in order to consider yourself an expert with high success rates and minimal complications.
2. What is your cure rate?
We all understand that nobody is perfect. We also understand that it is impossible to cure every patient that we touch. But cure rates below 90-95% probably signal that the surgeon has not yet reached the desired level of expertise most patients should seek, that would allow them to feel comfortable going “under the knife.” The best chance to cure somebody is during their first operation.
Any reoperation runs the risk of failing, especially if the surgeon creates a fair amount of scar tissue during the first surgery. Unless the surgeon has the expertise to locate and examine all four glands without exploration as we do, it is best to remove the tumor that can be identified on a scan prior to surgery and avoid an extensive operation, thus avoiding excessive scar tissue.
We do not have a problem with surgeons using this focused approach. It is a lot easier to re-operate if a focused operation was done first. It is also important that the surgeon knows if someone is cured the day of surgery. It is a lot easier to re-operate in the first week after a failed operation than 6 months later. Despite scar tissue we do re-operations every week with excellent results and outcomes.
3. What will you do if you cannot find the tumor? This is the question that determines if we would let somebody operate on us or not. Anybody can find a tumor that shows up on preoperative scans, although their accuracy is not as high as most people think. For example, we know that about 50% of the patients we operate on, have negative sestamibi scans.
So, it is advisable to surgeons that don’t do this operation often, to just remove the tumor found on preoperative imaging and if not, to quit the operation. If a surgeon goes into an operation and he/she is not able to find a tumor, they should be able to find the other parathyroid glands and create a map, but this takes knowledge and experience. That way you know what gland is missing. This ensures that at least you can gather information and center your efforts in finding the missing tumor. The other thing you do not want happening is a 2-hour operation, and no other parathyroid glands identified.
In conclusion, the list of questions above is provided just so you can get a feel for your surgeon and get acquainted with their surgical expertise, it is not meant to be taken as the only source to determine if your surgeon is the right one for you. That is a very personal decision, but our hope is that it helps you start a meaningful conversation during your consultation.
Dr. Jose Lopez
José A. Lopez, MD, FACS, is one of the most experienced parathyroid surgeons in the world today, having performed over 5,000 parathyroidectomies. He has dedicated the last 10 years of his career to the exclusive pursuit of parathyroid disease with particular focus on patients whose disease is complicated by scar tissue from prior surgeries and patients with coexisting thyroid tumors. Known for his upbeat manner and superior surgical skill, Dr. Lopez has helped perfect the advanced techniques of minimally invasive parathyroidectomy used to quickly identify the four parathyroid glands in surgery. He has examined over 20,000 parathyroid glands and has played a pivotal role in training the five most experienced parathyroid surgeons worldwide who have followed him in this field. Dr. Lopez, a native of Puerto Rico who is fluent in Spanish and English, has operated on patients from nearly every US state and multiple continents.
Dr. Jose Lopez together with his partner and friend of over 20 years, Dr. Douglas Politz (below), represent the two most experienced parathyroid surgeons in the world in the last 10 years. In addition to their impressive operative volume as primary surgeons, their expertise has been utilized in a supervisory role for the cure of thousands more patients as these two surgeon-leaders guided the efforts of colleagues they trained. Their combined 25 years of experience in parathyroid surgery have been spent almost exclusively at Tampa General Hospital, a medical center which has seen more parathyroid patients than any other facility in the world by far.
Douglas E. Politz, MD, FACS, FACE, is one of the most experienced parathyroid surgeons in the world, having performed over 8,000 parathyroidectomies. He has dedicated his career to parathyroid disease, combining enormous experience with superior knowledge and skill to compliment his warm, engaging manner. Having seen the simplest and the most challenging scenarios in which parathyroid disease can present, Dr. Politz welcomes those patients who have been diagnosed for the first time and those with prior attempts at surgical cure. Patients whose parathyroid disease is associated with thyroid cancer or kidney disease also benefit from his decades of experience. Dr. Politz has helped refine the advanced techniques of minimally invasive parathyroidectomy used to quickly identify the four parathyroid glands in surgery. He has examined over 25,000 parathyroid glands and has helped to train the most experienced parathyroid surgeons worldwide who have followed him in this field. Dr. Politz has operated on patients from every US state and multiple continents.
The Parathyroid Peeps host a patient support community on Inspireand a question was recently raised regarding the effectiveness of ethanol ablation and when it might be used as an intervention for hyperparathyroidism. We asked parathyroid expert Dr. Deva Boone, our medical advisor on Inspire, to respond and help educate us on this topic.
Dr. Deva Boone founder and surgeon at Southwest Parathyroid Center hosts a Parathyroid Question and Answer website: where you can review answers to many common questions asked about primary hyperparathyroid disease and also have your personal questions answered. We encourage you to take advantage of this excellent source of information!
Deva Boone, MD, FACS, FACE
A few years ago an endocrinologist called me about a very difficult parathyroid case. His patient had recurrent primary hyperparathyroidism and had already had several operations in an attempt to treat it. During his first parathyroid operation, a single large parathyroid tumor was removed, but it was broken apart and a piece was left behind. His second and third operations were aimed at evaluating his other glands and trying to find the problem. Due to thick scar tissue, his surgeons were unable to find the parathyroid tumor remnant, though they believed they could see it on ultrasound. The patient was miserable. His calcium was high, his bones ached, and he was exhausted.
Believing that surgery was no longer an option, his endocrinologist decided to try ethanol ablation. On the ultrasound, he could see what appeared to be a parathyroid tumor. Using a tiny needle to extract a few cells from the mass, he was able to confirm that it was parathyroid. He then injected 96% ethanol into the mass, with the hope of destroying the abnormal parathyroid tissue. The results were immediate. The patient’s calcium normalized and he felt amazing. However, in the end, ethanol ablation was not the solution – let’s learn why.
Ethanol Ablation of Parathyroid Tumors
Many of my patients, upon learning that they have primary hyperparathyroidism (pHPT), ask whether there might be a way to avoid surgery. There are no medications that can cure pHPT, but lately a few people have asked about ethanol ablation. In this procedure, the parathyroid tumor is injected with ethanol in order to damage or kill the gland. It is done under ultrasound guidance, and just involves a needle going into the parathyroid tumor. No skin incision or general anesthesia required. It is also called “chemical parathyroidectomy” because the doctor is using chemicals to essentially remove the parathyroid tumor.
This isn’t a new procedure. Doctors were attempting it in the 1980s, with mixed results. There are still scattered attempts to use it, but in general it is not widely practiced. Standard parathyroidectomy, or surgically removing the diseased parathyroid glands, remains the preferred treatment. It has incredibly high cure rates and is very safe.
But there are people who would prefer a non-surgical approach, so let’s review the medical literature on ethanol ablation.
Medical studies on ethanol ablation of parathyroid adenomas
All of the published studies involve small numbers of patients, usually treated at a single hospital. Most will describe the immediate outcomes of the ethanol injection and then follow the patients for a short period to see whether the calcium level normalized and remained normal.
Overall, the results have been poor. One early study published in 1989[i]looked at 18 patients with primary hyperparathyroidism who received ethanol ablation of their parathyroid tumors. While twelve patients (67%) showed improvement in their calcium and PTH levels, only 8 patients (44%) had normal calcium levels 6 months after injection. In addition, the procedure had a high complication rate: four patients (22%!) had recurrent laryngeal nerve injuries from the injection, causing vocal cord paralysis and difficulty speaking. One of these was permanent! For comparison, the rate of recurrent laryngeal nerve injury for an experienced parathyroid surgeon performing a parathyroidectomy should be well under 1%. While it might seem that injection of the parathyroid would be safer than open surgery, this was not the case.
The same authors published a follow-up study[ii]a few years later. They added another 14 patients, who were given up to three ethanol injections one week apart. They noted lab improvements in 11 (79%). Again, one patient in the group had a permanent vocal cord paralysis. Taken overall, the success rate was still poor, at 59%, and the complication rate was still high, at 6% for permanent nerve injury. In addition, a third of patients still had to have parathyroid surgery, and during surgery it was found that most of them had scarring around the parathyroid. Scarring can make the operation significantly more challenging (I know this from experience) and increases the risks from surgery.
Other studies published in the 1990s had similarly disappointing results. One study[iii]followed 27 patients who received ethanol ablation for parathyroid adenomas. Fifteen patients showed an initial cure after ablation. On following these patients, though, 4 out of those 15 developed recurrent disease within 2 years, for an overall cure rate of 41%. The Mayo Clinic published their results from 36 patients in 1998[iv]. Initial results were satisfactory, but at 16 months out, only a third of patients were still cured. Two thirds still had primary hyperparathyroidism despite ablation.
Better results came from a more recent study released in 2020[v]. The authors looked at tumor size in 45 patients followed for a year after ethanol ablation. After one month, less than half of patients had successful ablations, defined as a decrease in size of the tumor and improvement in calcium and PTH levels. The patients who did not see improvement had subsequent monthly injections (up to 4). By the end of the year they noted a cure rate of 85%, but this excluded the 6 patients were lost to follow up or did not complete the study, suggesting they may have sought care elsewhere. In addition, they did not follow anyone after a year, and prior studies would suggest that a significant number of those patients would develop recurrent disease.
Though this more recent study is somewhat encouraging, the procedure cannot compare to the results of parathyroid surgery. For the majority of patients, a single operation will cure them for life (no monthly repeats necessary). The operation also works for patients who were excluded from the studies of ethanol ablation, such as those with tumors that could not be seen on ultrasound and those suspected of having multigland disease. For ethanol ablation to work, the parathyroid tumor needs to be visible on ultrasound, which is not the case for many patients. Parathyroid surgery is also very safe, with minimal complications when performed by experienced surgeons. For these reasons, few physicians would advocate the routine use of ethanol ablation in primary hyperparathyroidism.
Is ethanol ablation ever useful?
Despite the poor results of “chemical parathyroidectomy” it may still be helpful in select situations. Advocates of the procedure argue that it can be used for patients who are “not surgical candidates,” meaning they are not healthy enough to undergo the operation. In my experience, there are very few patients who are in such bad shape that they cannot have parathyroid surgery. It is a quick (around 30 minutes on average) and safe outpatient procedure that I’ve done on many elderly people with heart disease, lung disease, kidney failure, and other serious medical conditions. I have never had a patient who truly could not get parathyroid surgery because of their other medical conditions, though theoretically it could happen.
Ethanol ablation may be useful in one small group: those rare individuals with multiple endocrine neoplasia (MEN) who have already had a subtotal parathyroidectomy (three of four parathyroids have already been removed) and have recurrent disease in the last parathyroid remnant. Patients with MEN1 develop parathyroid hyperplasia, or overgrowth of all four parathyroids, and this is a lifetime condition. The treatment is surgical; removing three parathyroid glands and part of the fourth, leaving behind a piece of the last gland. The remnant is left there to prevent permanent hypOparathyroidism, characterized by a lack of parathyroid hormone and an inability to keep calcium levels from dropping too low. Recurrent hypERparathyroidism can occur when the remnant grows large again. Treatment for that usually involves another operation, to trim back the gland. Repeat operations are more difficult, since the gland is surrounded by scar tissue, and have higher rates of complications. For patients with MEN1, hyperparathyroidism is usually a chronic disease, which is controlled and managed but never entirely cured.
A study from 2008[vi]looked at this population: 22 patients with MEN1 who had already had a subtotal parathyroidectomy (3.5 glands removed) and had developed recurrent disease. For some of these patients, ethanol ablation was successful, in that it helped to lower their calcium levels. Half of the patients required multiple injections. Unsurprisingly, recurrence was common. Within 3 years of the initial injection, 90% again had high calcium levels. But, the authors noted, repeat injections were usually just as successful as the first. For patients who have a lifelong parathyroid condition, these repeated injections are a viable method of long-term management.
Back to my patient
What about the difficult case I mentioned earlier? The endocrinologist used ethanol ablation, and it worked – for a time. A few months after the injection, the man’s calcium began to rise again, and his symptoms returned. The endocrinologist again injected the tumor with ethanol, and again noted rapid clinical improvement. This time it only lasted a month. After the third injection, which improved matters for just a few weeks, the endocrinologist started looking for other options. Ethanol ablation was not going to be a long-term solution. The man needed another operation, which finally cured him.
Conclusion
Parathyroid ethanol ablation involves chemically damaging a parathyroid tumor with an injection of ethanol. For many patients, it (sort of) works. It can decrease calcium and alleviate symptoms – temporarily. But the gland is rarely destroyed entirely by the injection, so the tumor regrows and the parathyroid disease returns months or years later in up to 70% of patients. And it is not risk-free; patients can have vocal cord paralysis and voice changes after the procedure. For patients with spontaneous primary hyperparathyroidism, who can be cured with an outpatient low-risk operation, ethanol ablation makes little sense. But for those with more complicated disease, such as patients with rare genetic syndromes like MEN1, who have already had one or more operations for their parathyroid condition, ethanol ablation may have a role in managing their disease.
[i]Karstrup S, Transbøl I, Holm HH, Glenthøj A, Hegedüs L. Ultrasound-guided chemical parathyroidectomy in patients with primary hyperparathyroidism: a prospective study. Br J Radiol. 1989;62(744):1037-1042. doi:10.1259/0007-1285-62-744-1037.
[ii]Karstrup S, Hegedüs L, Holm HH. Ultrasonically guided chemical parathyroidectomy in patients with primary hyperparathyroidism: a follow-up study. Clin Endocrinol (Oxf). 1993;38(5):523-530. doi:10.1111/j.1365-2265.1993.tb00349.x
[iii]Cercueil JP, Jacob D, Verges B, Holtzmann P, Lerais JM, Krause D. Percutaneous ethanol injection into parathyroid adenomas: mid- and long-term results. Eur Radiol. 1998;8(9):1565-1569. doi:10.1007/s003300050587
[iv]Harman CR, Grant CS, Hay ID, et al. Indications, technique, and efficacy of alcohol injection of enlarged parathyroid glands in patients with primary hyperparathyroidism. Surgery. 1998;124(6):1011-1020. doi:10.1067/msy.1998.91826
[v]Yazdani AA, Khalili N, Siavash M, et al. Ultrasound-guided ethanol injection for the treatment of parathyroid adenoma: A prospective self-controlled study. J Res Med Sci. 2020;25:93. Published 2020 Oct 28. doi:10.4103/jrms.JRMS_553_19
[vi]Veldman MW, Reading CC, Farrell MA, et al. Percutaneous parathyroid ethanol ablation in patients with multiple endocrine neoplasia type 1. AJR Am J Roentgenol. 2008;191(6):1740-1744. doi:10.2214/AJR.07.3431
ABOUT DR. BOONE – Dr. Boone is the founder of the Southwest Parathyroid Center in Phoenix, Arizona. She has dedicated her career to the understanding and treatment of parathyroid disease, combining compassion, knowledge, and experience to provide world-class care to all patients.
After obtaining her MD at Cornell Medical College, Dr. Boone completed a general surgery residency in New York City and endocrine surgery fellowship in Chicago, IL. She then subspecialized in parathyroid surgery. She joined the Norman Parathyroid Center in 2014, where she performed over 3600 parathyroid operations and consulted with thousands more patients with suspected calcium and parathyroid abnormalities. Very few surgeons worldwide have treated more parathyroid patients than Dr. Boone. At the Norman Center, she also served as the Medical Director from 2017 to 2020, while continuing to operate on 500 to 600 parathyroid patients annually. In 2020 she left to open the Southwest Parathyroid Center.
Dr. Boone is a frequent speaker on parathyroid disease. She enjoys teaching both patients and other physicians about calcium, Vitamin D, and the parathyroids.
Having surgery would hardly have been considered a reason for a road trip a few short years ago. But sometimes different worlds intersect, and the consumer (in this case the patient) benefits. The 21stcentury has seen countless advances across every field of human activity. The internet, along with its cousins in social media, have become digital travel agents in the field of medical tourism, providing easy access to necessary information. Patients can research the most advanced techniques for parathyroidectomy, share their experiences at different centers, and help unite each other with surgeons who have expertise in performing parathyroid surgery.
Dr. Doug Politz and Dr. Jose Lopez, former senior surgeons at the Norman Parathyroid Center in Tampa for 25 combined years, have spent the majority of their careers caring for patients who have traveled long distances to see them. No pair of surgeons in the world has performed more parathyroid operations in the last decade than these two. They have learned what makes it efficient and safe and how to guide and prepare patients for a smooth journey through surgery and the recovery that follows, while avoiding pitfalls with well-researched planning.
Now as Co-Founders of the Tampa General Hospital Parathyroid & Thyroid Institute, Drs. Politz and Lopez enjoy bringing their experience and expertise to patients who seek it. Below these two world leaders answer some of the most common questions patients ask about traveling for parathyroid surgery.
Do I needto stay in the area for a week after surgery in case any problems come up?
This is a perfectly reasonable question. When you plan your surgery, the surgeon (or the office staff) can let you know the follow-up protocols for that particular practice. Some have you stay in town a few days so they can personally see you before you return home, while others may keep you in the hospital overnight only, perhaps to check blood tests again. Some simply contact you by phone or telehealth later that evening or once you have returned home. Any number of factors can influence each surgeon’s routine and comfort with the timing of your departure. These may include: experience with the procedure, findings in your particular operation, length of the procedure, years in practice, call coverage by surgical partners (what the on-call partners are comfortable with), difficulty waking from anesthesia, other medical problems that require additional care, and of course COVID-19 testing (although that is more likely to impact your arrival plans rather than your departure.)
While minimally invasive parathyroidectomy in the hands of expert surgeons is extremely safe, nothing in medicine is perfect. If you have traveled a considerable distance and are being released from the surgery center or hospital the same day as the procedure, staying in the area until at least the next morning is certainly a safe choice. Particular surgeons can let you know what they are comfortable with, and you can plan accordingly.
Our experience has taught us that most anything that happens after surgery that would require our in-person attention is exceedingly rare (well under 1%). Also, anything that requires our bedside attention after parathyroidectomy is almost always apparent in the recovery room. That’s why we have our patients stay for an hour and a half or so afterward–so we can check on them a few times. Our routine is to call and check on them again that evening to review the details of their operation (now that the anesthesia has had more time to wear off) and make sure they are recovering appropriately. We recommend patients stay somewhere in the area if they are flying out the next day. Experience has taught us that the day after surgery is the best day to fly home. We have learned that once a patient gets to the next morning uneventfully, the chance that in-person attention is needed from us falls to near zero. We can troubleshoot virtually anything that comes up from that point forward by phone. Bottom line: safety! The destination is safety, and there are multiple routes to get there. The best route is where the surgeon and the patient are both comfortable and confident with the plan.
I won’t have access to my surgeon after I leave town.
You will want to find out how you will contact your surgeon (or your surgeon’s office) after you leave the hospital in case something comes up. Your surgeon may want you to stay in town for a week and come to the office for an in-person follow-up visit. You might be given a number for the answering service for your surgeon’s group. There are some surgeons who will want to arrange your follow-up care with a local surgeon in your hometown, to whom you would direct any questions. There is no right or wrong answer, just differences in style of practice, experience, and comfort with troubleshooting questions and problems remotely.
Our routine is to provide patients with our personal cell phone numbers to reach us once they leave the hospital. We are the ones most familiar with the details of the case, and we are directing that patient’s care. We prefer to know personally if any issues arise, and patients usually appreciate the access—and not having to tell their story to multiple individuals before speaking to the doctor. We don’t use an answering service. Our patients are able to text message us, call us, call our office to speak with our nurse, or message us through the hospital’s electronic medical record system. We have heard of a number of different arrangements in this regard, all of which are perfectly safe and can be easily learned by asking the staff at the surgeon’s office. This just happens to be a system that has worked well for us and for our patients.
What if I get there, and the scan shows the tumor in my chest?
You will want to find this out since it can affect travel arrangements, extent of surgery, expense, and the medical complexity of your case. Thankfully, this is not very common. Some surgeons may want you to arrive in town a couple of days early to get a scan to rule this out and then meet with them prior to the date of surgery. Still others may want you to have scans done at home and send them the films so they can review them ahead of time before any definitive travel arrangements are made.
If a scan shows a tumor in the chest, some parathyroid surgeons will want you to meet with a chest surgeon (thoracic surgeon) ahead of time or even undergo a chest operation and not a neck operation. This would, of course, depend upon the specific location of the tumor, the coordination of schedules between surgeons, and the experience of the parathyroid surgeon with tumors in this location. There’s no question that a parathyroid tumor seen in the chest adds some challenge and complexity to the situation.
We don’t require a scan ahead of time. After doing thousands and thousands of parathyroid operations, we’ve learned some pretty handy techniques to reach parathyroid tumors in unusual locations….
Our particular approach to this issue has evolved over the years, as we have encountered this time and time again. Our current approach reflects this experience. We don’t require a scan ahead of time. After doing thousands and thousands of parathyroid operations, we’ve learned some pretty handy techniques to reach parathyroid tumors in unusual locations…like the upper chest behind the breastbone. In our experience, over half of parathyroid tumors that are seen in the upper mediastinum (in the chest above the heart) end up being within our reach by using these advanced techniques and specific knowledge of anatomy and embryology (human development before birth). One advantage we have found in approaching these tumors through the neck is that the other three parathyroid glands in the neck can be examined in addition to removing the tumor out of the upper chest. Valuable information is gained by seeing those other three parathyroid glands (about 20-30% of patients have a second abnormal parathyroid gland that is not seen on a scan and needs to be removed.) In other words, the trip is not for nothing! Even if we see a parathyroid tumor in the chest that is out of our reach, this is important information for any thoracic surgeon who will be planning to remove that tumor.
Take-home point: no doctor can hand you a script of exactly how every situation is going to be handled when unexpected findings occur. But sometimes a surgeon has had an experience with a situation like this one and has made a policy for how it will be handled in the future. That’s good information for you to know when you’re trying to plan hotel nights, plane flights, expenses, insurance coverage, time off work, childcare, etc.
I haven’t met my surgeon yet.
Actually, with the technology of telehealth visits, and its proliferation across the healthcare landscape throughout the recent pandemic, physicians are able to carry out “office visits” in exactly the same way as if they were sitting across the table from the patient. Recent experience tells us that most insurance plans cover these virtual visits. They have some important advantages. Patients are in the comfort of their own home, so it’s far more convenient than fighting traffic, finding a parking place, walking a quarter of a mile, sitting in the waiting room with ill patients, etc.…you get the idea. Obviously, while COVID precautions are in place, it minimizes the risk to everyone involved to interact remotely. On a more personal note, we really enjoy visiting on screen because we get to interact without the barrier of a mask. We get to see our patients smile, a most basic human joy that the pandemic has eliminated from face-to-face encounters.
If you really think about the science of parathyroid disease in particular, these remote visits are ideal. We have found that we could sit with a potential parathyroid patient for an hour, or two hours, and still not know whether that patient has hyperparathyroidism until we get a chance to look at blood test results. Some patients really don’t feel comfortable proceeding unless they sit down in a traditional office setting to talk things over with the surgeon, and some surgeons (as mentioned above) will want you to arrive in town a few days early for an office visit anyway, as they are also more comfortable with this approach. So while we have found no particular advantage to an in-person meeting before the day of surgery, we (and just about any surgeon we know of) would gladly arrange for traditional office visits for those patients who desire it. The office staff answering the phone would almost certainly be able to provide this information.
It’s going to be more expensive to travel so far for surgery, isn’tit?
Maybe not! Here’s where you want to check with your local doctors to see what they require. They may require very few tests and visits before having surgery; whereas, some surgeons and endocrinologists may want CT scans, MRIs, ultrasounds, and sestamibi scans. Additional office visits to review these results can add more time (and sometimes anxiety). These can really start to fill up the ledger. Some surgeons will evaluate you remotely for an out-of-pocket fee that your insurance company won’t cover, while others may simply use telehealth and accept your insurance company’s reimbursement. Still others may evaluate your records remotely but ask that you arrive a few days early to meet with them in person and then remain in town for surgery. Hotel nights, meals, and transportation costs then come into play. These are all considerations that can impact whether traveling a long-distance for surgery is cost-effective.
If I need something done to my thyroid gland during my surgery, won’t I have to stay longer?
While most parathyroid operations will not need anything done with the thyroid gland at the same time, some of them do. In fact, up to 20-25% of parathyroid operations in some series can discover an issue with the thyroid that needs to be addressed. This is a straightforward question to ask the surgeon. Removing part of the thyroid gland will prompt some surgeons to keep the patient overnight for observation or ask that they stay in town a few days for a follow-up visit. You may want to plan for this as a contingency by booking an extra night in the hotel or scheduling a flight a day later if this is your surgeon’s routine. Of course, removing part of the thyroid gland can be as simple as taking out a small nodule or as extensive as removing the whole thyroid gland, although unexpected total thyroidectomy in a patient having a parathyroidectomy is indeed a rarity.
Over the years we have run into parathyroid patients needing a thyroid nodule removed fairly commonly, and just about any patient undergoing parathyroidectomy with us who ends up needing a portion of the thyroid gland removed can plan on the same discharge and travel accommodations as someone who needed nothing done with the thyroid.
What if I get symptoms of low calcium and I’ve already left town?
Quite honestly, this was the overarching reason why patients were kept in the hospital for extended periods of time for decades until outpatient parathyroidectomy came along. The safety of having parathyroidectomy and returning home afterward while taking oral calcium supplements has since been shown in tens of thousands of patients. Some surgeons will still keep patients overnight in the hospital with this problem in mind. Others have embraced outpatient parathyroidectomy and have grown comfortable managing this issue outside of the hospital.
An important point to remember is that symptoms of low calcium do not typically occur, even in the subset of patients who experience them, until the second or third day after surgery. This again is why we encourage parathyroid patients who are flying in for surgery to fly out the day after surgery. Even when these symptoms occur, they are mild and easily managed with supplemental oral calcium intake in the overwhelming majority of patients. This is a sign that the problem of hyperparathyroidism has been cured, and it is a development that can be managed quite easily by phone in our experience. If you should be one of those exceptionally rare individuals in whom oral calcium supplements just aren’t enough to control the symptoms, and you need to be seen at a hospital and get the calcium through an IV, we feel that patients are best off near their home for that…in familiar surroundings with family and friends nearby who can help with all kinds of issues like child and pet care, running errands, etc. Everything that is needed to address these issues is available in your hometown. There’s nothing special about the oral calcium pills at a pharmacy near your surgeon (or the bags of IV calcium in your surgeon’s hospital for the exceedingly rare patient that may need them). Trying to rearrange flights, babysitters, and work meetings from a hotel room in another city away from home while recovering from surgery just adds unnecessary stress.
What if I get there, and my COVID test is positive?
Not much anyone can do about this. The good news is: as more people get vaccinated, and more restrictions get lifted, things are drifting back toward normal. How long until we get there? No one knows, but for now we have to follow public policy guidelines.
In many ways, traveling long distances to reach expertise in the field of surgery has become commonplace. Social media and online patient forums put patients in touch with each other, spreading information about centers of expertise worth seeking out. Minimally invasive parathyroidectomy allows small incisions, quick procedures, outpatient discharge the same day, and speedy recovery. This formula has seen tens of thousands of patients undergo parathyroidectomy over the last 20 years, and we have learned how safely it can be done. Not everyone is in a situation to fly across the country for a quick, 20-30 minute procedure, but for those who can, rest assured that it is available, affordable, and safe.
Dr. Douglas Politz
Douglas E. Politz, MD, FACS, FACE, is one of the most experienced parathyroid surgeons in the world, having performed over 8,000 parathyroidectomies. He has dedicated his career to parathyroid disease, combining enormous experience with superior knowledge and skill to compliment his warm, engaging manner. Having seen the simplest and the most challenging scenarios in which parathyroid disease can present, Dr. Politz welcomes those patients who have been diagnosed for the first time and those with prior attempts at surgical cure. Patients whose parathyroid disease is associated with thyroid cancer or kidney disease also benefit from his decades of experience. Dr. Politz has helped refine the advanced techniques of minimally invasive parathyroidectomy used to quickly identify the four parathyroid glands in surgery. He has examined over 25,000 parathyroid glands and has helped to train the most experienced parathyroid surgeons worldwide who have followed him in this field. Dr. Politz has operated on patients from every US state and multiple continents.
Dr. Politz together with his partner and friend of over 20 years, Dr. Jose Lopez, are the two most experienced parathyroid surgeons in the world in the last 10 years. In addition to their impressive operative volume as primary surgeons, their expertise has been utilized in a supervisory role for the cure of thousands more patients as these two surgeon-leaders guided the efforts of colleagues they trained. Their combined 25 years of experience in parathyroid surgery have been spent almost exclusively at Tampa General Hospital, a medical center which has seen more parathyroid patients than any other facility in the world by far.
José A. Lopez, MD, FACS, is one of the most experienced parathyroid surgeons in the world today, having performed over 5,000 parathyroidectomies. He has dedicated the last 10 years of his career to the exclusive pursuit of parathyroid disease with particular focus on patients whose disease is complicated by scar tissue from prior surgeries and patients with coexisting thyroid tumors. Known for his upbeat manner and superior surgical skill, Dr. Lopez has helped perfect the advanced techniques of minimally invasive parathyroidectomy used to quickly identify the four parathyroid glands in surgery. He has examined over 20,000 parathyroid glands and has played a pivotal role in training the five most experienced parathyroid surgeons worldwide who have followed him in this field. Dr. Lopez, a native of Puerto Rico who is fluent in Spanish and English, has operated on patients from nearly every US state and multiple continents.
Dr. Lopez together with his partner and friend of over 20 years, Dr. Douglas Politz, represent the two most experienced parathyroid surgeons in the world in the last 10 years. In addition to their impressive operative volume as primary surgeons, their expertise has been utilized in a supervisory role for the cure of thousands more patients as these two surgeon-leaders guided the efforts of colleagues they trained. Their combined 25 years of experience in parathyroid surgery have been spent almost exclusively at Tampa General Hospital, a medical center which has seen more parathyroid patients than any other facility in the world by far.
Kenzie L. is an entrepreneur and divides her time between running her online business and pursuing a singing career. In Kenzie’s free time she pursues other passions such as painting and writing. Kenzie is a strong advocate for the early detection and treatment of hypercalcemia and hyperparathyroidism. It is Kenzie’s mission to help those afflicted with hyperparathyroidism.
MY TEENAGE YEARS
I had always been a healthy child, but in my teenage years I started to feel like something was off. This feeling was accompanied with a few fainting spells and so my doctor at the time decided to run some blood tests. My blood tests showed I had slightly high calcium for my age, but because I had low blood sugar at the time, I was diagnosed with hypoglycemia without diabetes instead of hyperparathyroidism. It is important to note that hyperparathyroidism is often associated with older women and hypoglycemia occurs more often in young females. According to http://www.parathyroid.com, parathyroid disease is extremely rare for those under twenty and is often only diagnosed after some sort of “event” like kidney stones or a broken bone. (https://www.parathyroid.com/age.htm), Therefore, I went into my early adulthood without a diagnosis or a doctor even mentioning hyperparathyroidism.
INTERSTITIAL CYSTITIS AND KIDNEY STONES
In 2012 I started to have issues with my bladder and for the first time I met with a urologist. The urologist did a scope of my bladder and found damage on my bladder walls. The urologist diagnosed me with Interstitial Cystitis and gave me recommended books to read about changing my diet for this disease. The reading made me believe I had some sort of gluten or wheat allergy that I thought flared up my Interstitial Cystitis. For years I was on a wheat-free diet thinking I was helping my bladder issues, but it was only a couple years after my Interstitial Cystitis diagnosis that I ended up at the hospital for a kidney stone that needed to be broken down.
Sadly, my hospital visit was only the beginning of a gradual decline in my overall health. I had always been a very energetic person, but I progressively started to feel exhausted all the time.
I was seeing a new doctor from the one I saw in my youth and I kept asking the doctor what to do about my severe fatigue. The doctor chalked it up to my being overly stressed about my studies, as I was in graduate school at the time. I would find out later that I did not have Interstitial Cystitis, but that my bladder had been damaged by the kidney stones due to my underlying and undiagnosed hyperparathyroidism.
MISDIAGNOSED AND TREATED FOR LUPUS
My struggles with fatigue persisted. Eventually my doctor ran tests which led the doctor to believe I had Lupus. I was put on Lupus medication. Regrettably, I never met with a rheumatologist to get a second opinion nor did my doctor order further blood panels to check for false positives.
I cannot even begin to describe the level of sick I felt on the prescribed medication. I was so sick that I was unable to hold down food for a couple of months and my professors encouraged me to take a break from school to focus on my health. My weight ballooned and I gained 40 pounds in a short amount of time. This was the first time in my life that I had ever struggled with a weight problem and my self-esteem plummeted to new lows. Along with the weight gain, I started to lose my hair; I lost hair on my head, eyebrows, arms, and on my legs. I was miserable. Eventually the doctor who put me on Lupus medication left the practice. This doctor’s replacement did another blood panel that revealed that I did not have Lupus after all, and I was taken off the Lupus medication.
SYMPTOMS OF HYPERPARATHYROIDISM
In 2017 my blood pressure and heart rate spiked, but my doctor and I thought this was because of my rapid weight gain. I was told to lose the weight and come back. I lost some of the weight, but my blood pressure and heart rate only elevated. Plus, time only made everything worse. I was very irritable and had little to no patience. My memory was suffering – I could not remember things I did 10 minutes ago. I was passing tons of kidney stones a month, I was starting to faint frequently, and my body severely ached. I thought all my symptoms were psychosomatic and I felt crazy. I tried confiding in friends and family but was reduced to “being dramatic” or “wanting attention”. Thus, I started hiding all my symptoms from people out of fear I was crazy, and I was hopeful that I could heal myself privately without anyone knowing or seeing what I believed to be psychosomatic.
REFERRALS AND TESTS
Of course, the symptoms never got better, and someone finally questioned me in 2018. While my husband and I were dating, I passed out a couple times and told him it was just hypoglycemia. (Remember, this was one of the original hypotheses regarding my poor health.) My reasoning for fainting only “bought him off” for a couple of months and it was not long until he had me meet with his general physician. The doctor really listened to me and did another blood panel. The blood panel suggested I had an auto-immune issue, but the doctor eased my ongoing fears about potentially having Lupus, because he said that a full blood panel is needed to get a good read on the issue which I found out my previous doctor did not do.
This GP noticed my calcium being high (see Table #2, 2018) and suggested hyperparathyroidism as a possible reason for my symptoms, but because I was so young, he wanted to test if I had neurocardiogenic syncope first and did not run a parathyroid hormone (PTH) test. I later learned that if a doctor suspects a patient to have hyperparathyroidism, they will test the calcium and PTH levels from the same draw, as it is the relationship of the two values that enables a proper diagnosis. But I assume, because I was young, hyperparathyroidism was deemed unlikely so my PTH was not tested. Instead, I was referred to a cardiologist who had me on a heart monitor for a week to test my heart from day to day. In addition, I had an echocardiogram and a tilt table test. The test revealed that my heart had no homeostasis and would fluctuate regardless of my postural position (like sitting or standing). However, this was not indicative of an underlying heart problem, and so I was referred to a rheumatologist.
ADVOCATING AND FINDING ANSWERS!
In 2019, my husband (who has a PhD in psychology and has a lot of experience in the medical field) did some researching of his own, and was convinced my issues were calcium related.
When we went to the rheumatologist, my husband pushed for the focus to be on the high calcium value of 11.5 mg/dL rather than the auto-immune issue. This led the rheumatologist to make a referral to an endocrinologist.
When we visited the endocrinologist, Dr. Serhal at the Cleveland Clinic (https://my.clevelandclinic.org/staff/4750-dina-serhal), she looked through my charts, instantly noted my high calcium and said there was no doubt that I had hyperparathyroidism.
Table 1 Parathyroid Hormone (PTH) Value 2019
I know this is weird, but I felt such relief to be diagnosed. It had been such a long time to get to that place and I was more than ready to just get better. Dr. Serhal had me look up at the ceiling and swallow and this revealed a golf ball-sized lump in my throat. I had never noticed that before and it felt bizarre to see for the first time, and my husband’s mouth dropped.This was also the first time my PTH was tested. My PTH was incredibly high as shown in Table 1 below and my blood calcium was 12.3 mg/dL (see Table 2, 2019)
Unfortunately, I was not able to obtain my calcium records when my symptoms first started but I was able to acquire my calcium records from 2014 on. The chart below indicates my calcium levels and the standard range of calcium listed on my labs from 2014 to 2021, as provided by my physician.
Table 2. Blood Calcium Levels (Note: Scroll through table to see all values from 2014 – 2021)
2014
2015
2016
2017
2018
2019
2020 (After Surgery)
2021
10.0 mg/dL
10.0mg/dL
10.8mg/dL
10.4mg/dL
11.5mg/dL
12.3mg/dL
10.2mg/dL
9.4mg/dL
Standard Range 8.6 -10.4mg/dL
Standard Range 8.6 -10.4mg/dL
Standard Range 8.6 -10.4mg/dL
Standard Range 8.6 -10.4mg/dL
Standard Range 8.5 -10.2mg/dL
Standard Range 8.5 -10.2mg/dL
Standard Range 8.5 -10.2mg/dL
Standard Range 8.5 -10.2mg/dL
REFERRAL TO AN EXPERT SURGEON
In the same appointment we talked about surgeons and I knew I wanted Dr. Siperstein from the Cleveland Clinic, as he is well known for his expertise in hyperparathyroidism (https://my.clevelandclinic.org/staff/3299-allan-siperstein). Dr. Serhal sent over my information to Dr. Siperstein and, while I was in the appointment with the endocrinologist, his office called to set up an appointment. I first met Dr. Siperstein in late 2019 and had my surgery in early 2020. Dr. Siperstein was absolutely wonderful and answered all my questions! He was very thorough and did a scan of my parathyroid in his office where I was officially diagnosed with primary hyperparathyroidism.
Later I found out I was in the two percentile for bone density. The percentile was based on a z-score, which is an appropriate measure for a female of my age. Z-scores are based on the number of standard deviation units that a value is from the mean (average). For example, if the average number of hours worked in a day is 8 and the standard deviation is 1 hour, then a 7-hour workday would be 1 standard deviation below the mean or a z score of -1, likewise if it was a 9-hour workday then it would be a z-score of +1. So, the z-score will tell you what percentage of values are above the current one, so a z-score of -2.3 (which was the z-score of my left forearm) would be about the 2ndpercentile, meaning that 98 percent of values would be higher than that.
At the time Dr. Siperstein believed one of my four parathyroid glands and thyroid were diseased, but surgery revealed something different. My thyroid was not diseased, but I did have a parathyroid adenoma; a noncancerous (benign) tumor of the parathyroid gland removed.
For context, a typical parathyroid is about the size of a small grain of rice. My parathyroid weighed 3.007 grams and was 5 cm, thus slightly bigger than a golf ball. My PTH and calcium were taken before and after the surgery as shown below in Table 3.
Table 3Calcium and PTH Intact – Before & After Surgery
Before Surgery
After Surgery
Calcium Value
12.3 mg/dl(8.5 -10.2 mg/dl)
11.1 mg/dl (8.5 – 10.2 mg/dl)
PTH Intact Value
562 pg/ml(15 – 65 pg/ml)
11 pg/ml (15 – 65 pg/ml
Some of my earliest calcium values were in normal range for a young adult. What was a possible reason for that? I have learned that water consumption plays a bigger role in calcium values than one would think. My surgeon, Dr. Siperstein, advised me to drink a lot of water as I waited for my scheduled surgery date to arrive, to help mitigate the symptoms of hyperparathyroidism. Harvard Health notes that drinking at least six to eight glasses of water each day lowers the chances of kidney stones in patients with hyperparathyroidism. (https://www.health.harvard.edu/newsletter_article/so_you_have_primary_hyperparathyroidism)
I used to be the kind of person to consume a lot of water, the longer I lived with the undiagnosed hyperparathyroidism, the more water I drank. At one point I was drinking fifteen 16 oz. water bottles a day! Since I was drinking so much water, Dr. Siperstein suggested that my calcium values were most likely higher. However, the increased consumption of water diluted the calcium. In simple, the high calcium made me thirsty, and the thirst made me drink more water which lowered my calcium levels temporarily.
POST SURGERY
After the surgery, I INSTANTLY felt better. If felt like a whole new person and it was as though this fog had been lifted!
I was walking around the same day I had the surgery and my levels started to drop into normal range. The next couple months were amazing because my hair started to grow back, my memory got better, I did not pass out, I did not have kidney stones, I did not feel so irritable, my body did not ache, I lost weight without trying, and I actually felt like myself again.
I met back with my husband’s general practitioner (who is now my general practitioner) and my blood work was the best it had ever been. However, I did still come up positive with an auto-immune disorder and, after a lot of testing, my doctor diagnosed me with Raynaud’s Phenomenon as part of CREST Syndrome. Currently, I am asymptomatic for the other components of CREST Syndrome. A couple months after that I went in for a check-up and my heart rate and blood pressure were normal! My heart rate and high blood pressure were the biggest things to me because they were the source of my fainting spells. When I finally had that under control, I felt like I could finally enjoy my life again!
There has been some time since my surgery in 2020, and I have not had any of the symptoms I had before! I cannot believe it took so long for someone to really help me.
I cannot believe how many times I was written off because I was a young girl. I feel so lucky I was finally put in front of the right people because that has made all the difference.
I know that my story is not unfamiliar to young sufferers. As noted, diagnosing hyperparathyroidism in those under twenty is often done after an “event” and slightly elevated calcium is often left unchecked or in a “let’s see what happens” mentality. With that said, the impact of hypercalcemia does not know age and can be detrimental to any person’s life; young or old. I hope that by sharing my story I can further educate people about the importance of high calciumand illustrate to people suffering from this disease that there is light at the end of this parathyroid fog.
Questions?
If you have questions for Kenzie you may contact her through the following accounts: Personal Instagram: __simply_kenzie___
Our guest blogger, Dr. Kevin Parrack, a surgeon from theNorman Parathyroid Center, educates us about differentiating Primary Hyperparathyroidism (pHPT) from Familial Hypocalciuric Hypercalcemia (FHH) in this two-part series.
Part 2 below focuses on the practical application of differentiating pHPT and FHH though a case study. Did you miss Part 1? Here is the link to Part 1 where Dr. Parrack discusses the important differences between the two conditions.
One of the questions I get asked frequently is whether or not to get urinary studies on patients with primary hyperparathyroidism. These studies have their place, but you have to understand the details to know if they will be useful in a specific situation, and what to do if you have results you didn’t ask for. The following case is an excellent illustration of these issues.
This is one of our patients we operated on who has given our center permission to tell her story. I have changed her name (we’ll call her Bee) and certain details to protect her privacy. She hopes that telling her story can help other people dealing with the same thing.
Patient Profile
Bee is in her 40’s and had been struggling with a number of symptoms that she did not expect at such a young age and in what she thought was good health. Bee was tired most of the time regardless of how much she slept, began having headaches that worsened over time despite treatment, as well as new onset joint pain and palpitations. The patient didn’t have any known medical disease to explain these problems and these symptoms certainly seemed beyond just typical aging so she saw her doctor for help.
Primary Care
Bee’s primary care physician listened to her concerns and checked a number of labs as a first step. Her PCP noticed the high calcium level (10.6 mg/dL) and checked her PTH level (99.8 pg/mL). Bee’s calcium level was high for her age at 10.6 mg/dL, and with this elevated calcium level, the PTH level should have been suppressed, and therefore low, if her parathyroid glands were normal. The fact that the patient’s PTH was high at 99.8 pg/mL with elevated calcium, this corresponds to roughly a 99% chance of having primary hyperparathyroidism.
This PCP realized that Bee had primary hyperparathyroidism and referred her to a local endocrinologist for advice on how to proceed.
Referred to Endocrinologist
The endocrinologist ordered a 24 hour urinary study and the results showed a 24 hour urinary calcium level of 14.9 mg/24 hours and a Calcium : Creatinine ratio of less than 0.01. If youlook these values up you will see that they are consistent with FHH, which stands for Familial Hypocalciuric Hypercalcemia. This is a benign genetic condition for which surgery is not routinely indicated, as surgery will not improve the calcium level and there are no associated symptoms that need to be fixed.
After getting the urinary study results the endocrinologist told her that she had FHH; therefore did not have primary hyperparathyroidism and should not have surgery.
Patient’s Self-Advocacy Pays Off
Bee researched this issue on her own and after reading primary articles in a university library about FHH realized that she probably did not have FHH and that her urinary studies represented what is known as a “false positive.” She contacted our centerand discussed her case with one of our surgeons.
FHH is a rare disorder but it does happen. We speak with so many hypercalcemic patients that we come across it from time to time, but not often. FHH and primary hyperparathyroidism present in different ways and have different characteristics, which I discuss inPart 1 of this blog series.
FHH and primary hyperparathyroidism present in different ways and have different characteristics…
Unfortunately the urinary studies are not perfect, they make mistakes. Some patients who have primary hyperparathyroidism have urinary studies that look like FHH! In other words, if you check urinary studies in every person who has primary hyperparathyroidism, you will find some urinary studies that look like FHH. If you then tell the patient not to have surgery, you are condemning them to keep a tumor that can make them miserable and sick. This is something we take very seriously.
Key to spotting FHH
The key to spotting FHH and avoiding operating on those patients if possible is to understand the difference in how FHH and primary hyperparathyroidism patients present, and to know what to do if you suspect FHH.
FHH is a genetic disorder that will affect about half of the family members without skipping generations. People with this problem will have high blood calcium levels but no symptoms or complications. Their calcium levels may also have been slowly but steadily rising for their entire life. If I have a patient with a family history that makes me suspicious of FHH, there is a protocol in place for how to evaluate the family, not just the individual patients, which includes comprehensive genetic testing. This is not a disorder that you usually find in one person; it is a family diagnosis.
If I have a patient with a family history that makes me suspicious of FHH, there is a protocol in place for how to evaluate the family, not just the individual patient, which includes comprehensive genetic testing. This is not a disorder that you usually find in one person; it is a family diagnosis.
No one in Bee’s family had high calcium levels; she had calcium levels in the 9’s a few years ago, and typical symptoms of primary hyperparathyroidism with no other explanation, so she didn’t think she had FHH. When Bee spoke with us her case sounded like routine primary hyperparathyroidism and we explained that we wouldn’t have obtained urinary studies for her due to the concern of a false positive preventing a curative surgery. With the unwanted urinary study results in hand we explained that we still thought she had primary hyperparathyroidism, but that there was a small chance that she had FHH. However, that would require the genetic mutation happening in her first in the family, which is pretty unlikely. because leaving a parathyroid tumor in is far more dangerous than an expert surgeon examining the parathyroid glands in a patient with FHH, she chose to have parathyroid surgery.
Because leaving a parathyroid tumor in is far more dangerous than an expert surgeon examining the parathyroid glands in a patient with FHH, she chose to have parathyroid surgery.
Surgical Outcome
In her surgery we found a single parathyroid adenoma and three small parathyroid glands. These findings are consistent with long-term primary hyperparathyroidism where one parathyroid tumor is the problem and the other three parathyroid glands have been suppressed for a long period of time. In FHH all of the parathyroid glands are typically enlarged, some more than others. In the recovery room her PTH dropped down to normal, consistent with being cured of primary hyperparathyroidism and not typical with FHH.
Needless to say we are all very happy for Bee and wish her the best. I also appreciate her very much for letting us share her story because of all the types of patients I see, the stories that upset me the most are the people with primary hyperparathyroidism that go undiagnosed for decades and wind up with a complication that could have been avoided. Urinary studies can be very useful when used as part of a workup for a family we suspect has FHH, but in an individual person who has routine primary hyperparathyroidism, these studies can cast doubt on an obvious diagnosis.
Dr. Kevin Parrack M.D., FACS
Dr. Kevin Parrack is a surgeon at the Norman Parathyroid Center, a high volume parathyroid practice that treats around four thousand parathyroid patients per year. In this role Dr. Parrack focuses on teaching patients and physicians about parathyroid disease in hopes that more people will become familiar with this problem and therefore fewer people will suffer from it without appropriate treatment.
Dr. Parrack obtained his undergraduate degree at NYU before graduating from Stony Brook School of Medicine. He completed his residency program at Columbia Presbyterian in Manhattan and the endocrine surgery fellowship at the Cleveland Clinic. His focus on teaching began in college, where his first career was in test prep for the medical school entrance exam. Throughout his training Dr. Parrack worked on admissions and academic committees, designed curriculums and teaching aids, and that interest carried into his role as a surgeon. Before coming to the Norman Parathyroid Center Dr. Parrack was a faculty surgeon at Columbia where he was the director of the Thyroid Biopsy Center and managed outreach to the community to increase awareness regarding endocrine disease.
Our guest blogger, Dr. Kevin Parrack a surgeon from the Norman Parathyroid Center educates us about differentiating Primary Hyperparathyroidism (pHPT) from Familial Hypocalciuric Hypercalcemia (FHH) in a two-part series. Part 1 below discusses important differences between the two conditions. Part 2, scheduled to post later this month, focuses on the practical application of differentiating pHPT and FHH using a case study.
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When it comes to diagnosing primary hyperparathyroidism, there are a number of issues that have been debated over time. One of the tools that can be easily misunderstood is the urinarycalcium test. This is a study that is broadly used but frequently misunderstood. It is used as an attempt to differentiate Primary Hyperparathyroidism (pHPT) from Familial Hypocalciuric Hypercalcemia (FHH, often referred to as FHH1).
WHY IS IT IMPORTANT TO DIFFERENTIATE? Patients with pHPT and FHH both typically have elevated blood calcium levels and parathyroid hormone levels (PTH) that are not normal for the corresponding calcium levels (either high or inappropriately at the higher end of normal). This is a problem because only a select few patients with FHH will benefit from having parathyroid surgery. Most will not have a change in their labs after surgery, nor will they have any clinical benefit. So it is important to differentiate the two in hopes of avoiding an unnecessary surgery on a patient with FHH.
WHY IS A URINARY CALCIUM TEST ORDERED? The idea behind the urinary calcium test is that most patients with primary hyperparathyroidism have a high urinary calcium level, and patients with FHH have a low urinary calcium level. So many doctors will order a 24-hour urinary calcium collection or calcium: creatinine clearance ratio as a standard test when they see blood labs consistent with primary hyperparathyroidism. The assumption is that if the urinary calcium level is below a certain threshold, then the patient likely has FHH and not pHPT. That can lead to a diagnosis of FHH and subsequently a recommendation for no surgery.
WHAT’S THE PROBLEM? As we will see, the problem with this pathway is that some patients with pHPT will be misdiagnosed with FHH, and this can lead to harm to these patients. There are a few factors regarding the urinary calcium collections that lead to this error in diagnosis.
Some patients with pHPT can have very low urinary calcium levels. This is due to problems in vitamin D metabolism in the parathyroid patients.
The error rate for the urinary calcium collections is higher than what would be considered acceptable in the blood labs, so erroneously low urinary calcium levels can be reported.
FHH is extremely rare, so even though the above two issues are not common, the very low incidence of FHH means that the false positive rate of the urinary testing is relatively high.
This is not a minor problem for a patientwho has pHPT.
We know that pHPT decreases a person’s life expectancy and can lead to symptoms that interfere with their quality of life.
Therefore if a patient has a urinary calcium level that is consistent with FHH, is then labeled with this diagnosis despite the fact that they have pHPT and does not have surgery, they may have a shorter, less enjoyable life. This is a problem we should avoid.
WHAT IS FHH? In order to understand what to do about the problem, we firsthave to understand FHH.Understanding certain nuances allows us to develop a plan that helps ensure that FHH is not diagnosed in error. We must balance the desire to avoid an unnecessary surgery in a patient with FHH against the goal of offering an indicated surgery to appropriate patients with pHPT.
FHH is a rare disorder caused by mutations in a gene for the calcium-sensing receptor (CaSR). This receptor is found in both parathyroid and kidney tissue where it is integral to maintaining a balance in the blood and urinary calcium levels. It is also found in many other tissues, including the bone and brain, and responds to more than just calcium, but we will keep our focus narrow.
WHAT DOES THE CALCIUM-SENSING RECEPTOR DO? On the parathyroid gland the CaSR allows the parathyroid gland to sense a low calcium and increase parathyroid hormone (PTH) production, or sense a high calcium and decrease PTH production. In the kidney the CaSR allows the kidney to respond to high blood calcium by excreting more calcium into the urine, or to respond to a low blood calcium by excreting less calcium in the urine.
In FHH the CaSR receptor is not working normally, which reduces the sensitivity of the receptor to calcium levels. Imagine being in a house where the thermostat registersthe temperature lower than it really is. It would keep cranking up the heater even if the temperature in the house were normal or elevated. The results of a poorly functional CaSR is that a higher than normal calcium level is needed to stimulate the parathyroid glands to decrease PTH. So most patients with FHH will have a high calcium level and an inappropriately normal or elevated PTH level in their blood labs.
Similarly the kidneys are confused, they don’t realize the blood calcium is so high, so they keep trying to retain calcium in the blood, by preventing it from being excreted in the urine. The overall result is that the blood calcium goes up, the urine calcium goes down, and the PTH is higher than it should be. The PTH is normal, but not suppressed, in most FHH patients, high in others.
For most people with FHH it is a benign disease, causing no symptoms and no problems. Withcertain forms of FHH, kidney stones, bone problems, pancreatitis or other issues may be more likely than in the general population.
UNDERSTANDING FHH GENETIC MUTATIONS If the cause of this problem is a genetic mutation, understanding some of the details about these particular mutations can help us tell the difference between FHH and pHPT.
Most patients with FHH have one inactivating mutation in the gene that is responsible for CaSR. That means they have one normal CaSR gene, and one mutated CaSR gene that doesn’t work properly. Having just one non-working gene is enough to cause abnormal labs. Another way to say this is that people who are herozygous for CaSR non-functional mutations can develop FHH. This means that FHH is an autosomal dominant disorder.
In autosomal dominant disorders an affected person will have a parent who has the disorder and roughly 50% of the children of an affected individual will have the disorder. In other words in FHH families, about half the family will have FHH.
With all this information in mind we can now try and spot patients who might have FHH, and avoid suspecting it in patients who likely have pHPT, even if their urinary labs are consistent with FHH.
FHH PROFILE FHH patients are usually asymptomatic, with high calcium levels starting in childhood and persisting for life, have multiple hypercalcemic family members, usually with one or more family members who had parathyroid surgery that did not change their labs or symptoms.
Patients who have had blood labs consistent with primary hyperparathyroidism or FHH but do not have a story that matches the FHH profile, usually have pHPT. And avoiding an operation in these patients because urinary labs suggest FHH can hurt the patient.
WHAT ABOUT PEOPLE WHO DON’T KNOW THEIR FAMILY MEDICAL HISTORY? People who do not know their family history should be treated the same as people who know their family history does not include hyperpercalcemia. This is true because genetic disorders that cause hypercalcemia, including FHH, are so rare. Proper counseling is required, but refusing surgery based on urinary calcium testing alone in such patients can be an error. It is true that in this situation some FHH patients will wind up with a surgery that may not clinically benefit them, but if the surgery is done at a center with extremely low surgical risks, this is safer than not doing a surgery in a patient with pHPT that can lead to early demise or serious medical problems.
WHAT ABOUT PEOPLE WHO HAVE A STORY & FAMILY HISTORY THAT FITFHH? What can be done to clarify the diagnosis in hopes of avoiding an unnecessary surgery? The first step is to get parathyroid blood panels and proper urinary studies on all family members with high calcium levels. The calcium : creatinine clearance ratio is better than the 24-hour total calcium level. While it is not perfect we see fewer mistakes with the ratio than the urinary calcium level test results. If all or nearly all hypercalcemic family members have blood and urine labs that are consistent with the biochemical FHH profile, then this is a reasonable diagnosis to consider. Genetic testing and counseling is appropriate, and if the diagnosis of FHH is confidently made, then surgery is unlikely to play a role. There is a subset of FHH patients who may benefit from surgery for whom expert selection and counseling is required before surgery is considered.
WHAT IF UNCERTAINTY PERSISTS? In some cases despite the best workup the diagnosis remains unclear. Doctors know there is an overlap in the urine studies between FHH and pHPT, and the genetic testing available is not infallible, nor are all related mutations characterized. For patients in whom the truth is not clear from the labs, careful counseling is required. If the odds that surgery will help is high enough some patients will choose surgery because they do not want to risk keeping a parathyroid tumor that could hurt them, and they want to know the truth. Others will choose not to have surgery because the risks and costs of surgery are not worth it to them. And for other patients the odds that surgery will help them is so low that surgery should not be offered.
I hope that this has helped answer more questions than it raises. I have operated on multiple patients who were diagnosed with FHH based on urinary studies but turned out to have parathyroid tumors. These patients were relieved to have their symptoms resolved and labs normalized by surgery. Through their own persistence they got the help they needed to be correctly diagnosed and avoid a life with uncured parathyroid disease. I balance this against the very few patients who I operated on that turned out to have FHH.
Dr. Kevin Parrack M.D., FACS
Dr. Kevin Parrack is a surgeon at the Norman Parathyroid Center, a high volume parathyroid practice that treats around four thousand parathyroid patients per year. In this role Dr. Parrack focuses on teaching patients and physicians about parathyroid disease in hopes that more people will become familiar with this problem and therefore fewer people will suffer from it without appropriate treatment.
Dr. Parrack obtained his undergraduate degree at NYU before graduating from Stony Brook School of Medicine. He completed his residency program at Columbia Presbyterian in Manhattan and the endocrine surgery fellowship at the Cleveland Clinic. His focus on teaching began in college, where his first career was in test prep for the medical school entrance exam. Throughout his training Dr. Parrack worked on admissions and academic committees, designed curriculums and teaching aids, and that interest carried into his role as a surgeon. Before coming to the Norman Parathyroid Center Dr. Parrack was a faculty surgeon at Columbia where he was the director of the Thyroid Biopsy Center and managed outreach to the community to increase awareness regarding endocrine disease.
Dr. Courtney Balentine is a fellowship-trained endocrine surgeon with a research program dedicated to improving outcomes for patients with endocrine disease, particularly primary hyperparathyroidism. He serves as a Medical Advisor to the Parathyroid Peeps on Inspire. We are pleased to announce that Dr. Balentine will be available from Monday, November 11 to Friday, November 15 to answer your questions about the diagnosis of primary hyperparathyroid disease.
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Ask your questions in the comment section of the pinned post entitled “Ask the Expert” that will be up beginning Monday, November 11.
Dr. Balentine will post answers to your questions daily. His answers will appear in the comment section as well.
Note: Answers may not immediately follow the question within the thread due to the format. You may find it helpful to refer to (click on) the “In Reply To: post number” at the top of Dr. Balentine’s replies to view the question being answered.
Our topic for this month is Diagnosis of Primary Hyperparathyroid Disease. Please understand if Dr. Balentine is unable to get to your question due to time or topic constraints.
More About Dr. Balentine’s Research…
Dr. Balentine’s work focuses on improving processes for diagnosing patients with hyperparathyroidism to facilitate early detection and treatment. His ultimate goal is to ensure that patients with hyperparathyroidism are diagnosed and treated as early as possible without experiencing unnecessary delays. Dr. Balentine is also interested in reducing disparities among older patients with hyperparathyroidism who are even more likely to experience delays in diagnosis and treatment than younger patients. Dr. Balentine works closely with patients and other stakeholders, including the PARAthyroid Peeps, to design and test interventions that address these gaps in care. His research program has been funded by NIH, the Agency for Healthcare Research and Quality, the Department of Veterans Affairs, and numerous surgical societies. Dr. Balentine is proud to provide endocrine surgical care to our Veterans. His clinical practice is based at the North Texas VA Hospital.
With your continued interest and support our Parathyroid Peeps community is growing! If you haven’t yet joined our parathyroid community on Inspire, we invite you to do so today. As a member you can receive support and also help others by sharing your patient experience.
On September 30th through October 4th we will be hosting our first of many “Ask The Expert” events. Instructions will be prominently posted within the community a few days before the event begins explaining how to participate and where to post your questions.
who serve as Medical Advisors to our Parathyroid Peeps community on Inspire…
Dr. Deva Boone is one of the busiest parathyroid surgeons in the U.S., performing over 600 parathyroid operations annually. She is the Medical Director at the Norman Parathyroid Center.
After obtaining her medical degree from Cornell University, she completed general surgery residency at St. Luke’s Roosevelt Hospital in New York City. While there, she received several awards for research, and during her final year received the highest award for outstanding contributions to surgical education and research. After residency Dr. Boone received additional fellowship training in endocrine surgery (thyroid and parathyroid surgery) at NorthShore University in Chicago. She joined the Norman Parathyroid Center in 2014. She enjoys surgical missions and has provided surgical care to patients in Nigeria, Myanmar, and the Dominican Republic.
Dr. Courtney Balentine is a fellowship-trained endocrine surgeon with a research program dedicated to improving outcomes for patients with endocrine disease, particularly primary hyperparathyroidism.
Dr. Balentine’s work focuses on improving processes for diagnosing patients with hyperparathyroidism to facilitate early detection and treatment. His ultimate goal is to ensure that patients with hyperparathyroidism are diagnosed and treated as early as possible without experiencing unnecessary delays. Dr. Balentine is also interested in reducing disparities among older patients with hyperparathyroidism who are even more likely to experience delays in diagnosis and treatment than younger patients. He works closely with patients and other stakeholders to design and test interventions that address these gaps in care. His research program has been funded by NIH, the Agency for Healthcare Research and Quality, the Department of Veterans Affairs, and numerous surgical societies. Dr. Balentine is proud to provide endocrine surgical care to our Veterans.His clinical practice is based at the North Texas VA Hospital.
The exceptionally talented Mark A. Hicks is sharing his “Color Me” cartoon about hyperparathyroidism with us. Using a medium that is most familiar to him Mark notes, “It’s a lighthearted take on a serious health problem”
The cartoon came about because, while discussing some artwork Mark was doing for a notable parathyroid doctor, the doctor mentioned the general lack of knowledge of parathyroid disease in the medical community. Mark thought that maybe somebody should draw a simple picture that patients could use to help better explain hyperparathyroid disease and its symptoms to doctors. So that’s just what Mark did.
Mark hopes the Color Me activity will help raise awareness of the disease and its symptoms among patients and the healthcare community.
Permission to use and share Color Me image granted by Mark A Hicks 8-13-19
Mark A. Hicks is an award-winning freelance artist and illustrator who has been creating artwork for children’s books, magazines, greeting cards, and other countless publications for the past 30+ years. Please visit his website at: www.MARKiX.net for a few samples of his artwork and more information about his many projects.
Avoid The Pitfalls That Delay Diagnosis & Treatment of Primary Hyperparathyroid Diseaseis a series of blogs that shed light on the most common issues patients face that delay diagnosis and surgical treatment.
#5. Classic symptoms are often treated by specialists who do not necessarily consider primary hyperparathyroid disease as a possible root cause.
Some of the symptoms patients report are more generalized, and can be attributed to any number of health issues. However, there are several classic symptoms associated with primary hyperparathyroid disease. Medical students are often taught a rhyme to recall the effects of excess blood calcium levels. It goes something like this…
“Moans, Stones, Groans and Bones”
Each word in the short rhyme references common ailments typically associated with primary hyperparathyroid disease…
Moans (gastrointestinal conditions):
Constipation
Nausea
Decreased appetite
Abdominal pain
Peptic ulcer disease
Stones (kidney-related conditions):
Kidney stones
Flank pain
Frequent urination
Groans (psychological conditions):
Confusion
Dementia
Memory loss
Depression
Bones (bone pain and bone-related conditions):
Bone aches and pain
Fracture
Curving of the spine and loss of height
As patients, we may present with some, but not necessarily all, of these symptoms over time. You are invited toread Barbara, Sophie and Joyce’s storiesto learn more about the symptoms we suffered from.
Sadly, specialists who are treating some of the classic symptoms of primary hyperparathyroid disease are not necessarily well-versed in primary hyperparathyroid disease.
One might expect the specialist to have a thorough understanding of a related disease that is known to be a possible root cause of a condition they treat on a daily basis. However, for many of us, this has not been our experience.
No correlations were considered and therefore no attempts were made to discover the root cause – only symptoms were treated.
Gastroenterologistsare the medical professionals who treat the “moans”. They may very easily overlook high calcium as a root cause of certain gastrointestinal issues. The story of a fellow advocate, and blogger Lora Park’s comes to mind. In her story she highlights the challenging gastrointestinal issues she faced and laments that she suffered for years without a diagnosis …
Did it start in 1980 when i started getting ‘fainty’ if i didn’t eat every 3 hours? Was it in 1987 when i kept going in to the Dr. complaining that everything was making me ill and having them look at me and say i was young and ‘perfect’ and it COULD be just nervous stomach, anxiety… or was it one of the numerous mysterious trips to ER with vomiting and cramping and being told my appendix needed to come out “STAT” only to find out a few minutes later that everything was ‘fine’.
Eventually, it was an astute RN who alerted Lora to the fact that she had an elevated calcium value.
Urologistsare the ones to treat patients who are suffering from kidneystones. Though there are a variety of reasons people get stones, primary hyperparathyroid disease is one root cause. While we are asked to catch our stones to determine the type, it may end there. It would seem prudent to rule out whether the person has primary hyperparathyroid disease by doing a thorough diagnostic workup.
Psychologistswill likely see patients who present with the“groans”. Patients report having episodes of severe anxiety, depression and/or brain fog. While many of these professionals are now well-versed in understanding the psychological symptoms often associated with thyroid conditions, more education is needed to raise awareness among mental healthcare professionals so they are able to do the same for those potentially suffering from primary hyperparathyroid disease.
General Practitioners, Internists, Rheumatologists, Endocrinologiststreat diseases of thebone.Many PHPT sufferers report being diagnosed with osteoporosis and have shared with us that they were put on bone drugs for osteoporosis without having gone through a thorough diagnostic process. Or if they did, the physician did not necessarily understand the various biochemical presentations of primary hyperparathyroid disease and the diagnosis was overlooked. Sandi, a fellow patient advocate who suffered from osteoporosis for years shares her story in this 28 minute videoand expert parathyroid surgeonDr. Boone, an expert in parathyroid disease, summarizes some of the hurdles in this 6.5 minute video.
Others have reported that they were encouraged to take bone drugs after diagnosis as part of a “wait and see” model of care. This happened in Sophie’s case and it made no sense to her to take a medication that would not address the root cause. After doing her own research, she declined and self-referred to a parathyroid surgical center. Bone drugs will not help improve the bone health of a person if there is an underlying condition of primary hyperparathyroid disease.
Dentistsmay also see rapid changes/deterioration in dental health in patients who have primary hyperparathyroid . Patients would benefit from dentists being familiar with the various symptoms (deterioration of bones and teeth specifically) and biochemical presentations. Dentists could then suggest that these patients follow-up with their personal physician to determine if primary hyperparathyroid disease is possibly the underlying cause.
A glaring group of conditions treated byCardiologiststhat is not addressed in the memorable rhyme, are those that effect theheart and blood vessels, including high blood pressure (hypertension), hardening of the arteries (atherosclerosis), coronary artery disease, an enlarging heart (left ventricular hypertrophy), and abnormalities in the normal electrical activity of the heart.Learn more here.
A patient may report having a fluttering, racing heart and /or it is discovered that they have high blood pressure. When the doctor cannot find anything else particularly wrong with the patient, they may put the patient on blood pressure medications. Yet elevated calcium values may not be considered and/or ignored if mildly elevated. Once diagnosed, patients have realized that their blood calcium were elevated during that time and found that their heart stopped fluttering/racing and blood pressure returned to normal following surgical removal of the diseased parathyroid gland(s).
Conclusion:We understand it is not at all unusual for patients suffering from primary hyperparathyroid disease to go undiagnosed for many years. Parathyroid experts believe the average patients goes undiagnosed at least 8 years, if not longer. Remember that it is the length of time that calcium values are elevated outside of normal range which wreaks havoc in our bodies, rather than the degree to which calcium is elevated. Thus, the condition should not be referred to as “mild” if serum blood calcium is slightly elevated. A “wait and see” approach is actually very harmful to our health. Of course, we cannot attribute the delays in diagnosis entirely to the specialists treating our symptoms. Remember that in our first blog in this series, we discussed that labs often do not report calcium values based on a persons age.However, an excellent understanding of primary hyperparathyroid disease by healthcare professionals across all specializations who treat related symptoms and conditions, would help drastically reduce the delays in diagnosis we experience as patients.
RESOURCES FOR YOU:
Hypercalcemica Calculator: Here is a link to the Norman Parathyroid Center’s hypercalcemia calculator to find the upper limit of blood calcium for your age. Note that when you enter your age in the calculator that your upper limit will appear in the paragraph below in both mg/dl (USA) or mmil/L in most other countries. See the example image below for someone who is 50 years of age.
A new way to help us remember the symptoms of Primary Hyperparathyroid Disease : See our High Calcium Is Bad symptom list.
If you missed the previous blogs in the series, Avoid The Pitfalls That Delay Diagnosis & Treatment of Primary Hyperparathyroid Disease, here are links to catch up:
PARAthyroid PEEPS 