Photo Credit: Natali_Mis

Drawing from personal experience, Patty Keating advocates for greater awareness, improved diagnosis, and treatment among patients with hypoparathyroidism.

Hypoparathyroidism, a rare endocrine disorder affecting 70,000 to 90,000 people in the US, has traditionally been managed with calcium and vitamin supplements rather than addressing its root cause. Often resulting from accidental parathyroid gland removal during thyroid surgery, the condition disrupts calcium and phosphate regulation, leading to serious complications like brain calcium deposits, cataracts, and kidney stones. The recent FDA approval of palopegteriparatide, the first hormone replacement therapy for adults with hypoparathyroidism, marks a breakthrough in treatment.

Physician’s Weekly (PW) spoke with Patty Keating, executive director of the HypoPARAthyroidism Association, where she advocates for greater awareness, improved diagnosis, and treatment—drawing from her personal experience after developing the condition post-surgery in 2015.

PW: How can physicians improve early and accurate diagnosis, especially for post-thyroidectomy patients?

Keating: Hypoparathyroidism is not only underdiagnosed but undertreated. Increasing awareness and understanding of hypoparathyroidism as a potential complication of thyroid or neck surgeries is crucial before surgery. Patients need to understand the symptoms of hypoparathyroidism prior to surgery so they know what to report to their surgeon and clinician. Physicians can improve early and accurate diagnosis, especially in post-thyroidectomy patients, by staying vigilant for hallmark symptoms and routinely monitoring calcium and PTH levels after surgery.

The international guidelines recommend measuring PTH levels 12-24 hours after surgery. A PTH level above 10pg/ml indicates a lower risk of permanent hypoparathyroidism, but levels below 10pg/ml require follow-up due to higher risks of hypoparathyroidism. In addition, it is essential to shift the perception of hypoparathyroidism from being merely a calcium deficiency to recognizing it as a disease caused by insufficient parathyroid hormone (PTH). Fortunately, one new treatment is now available for physicians to address this need, with more treatments on the horizon. These advancements have significantly improved the quality of life for patients.

How have limitations associated with calcium and vitamin supplements affected long-term patient outcomes and QOL?

Patients with hypoparathyroidism not only endure complications from the disease itself daily but also grapple with significant challenges arising from the limitations and risks of the current standard of care. Conventional therapies fail to address the root cause of the condition and often require patients to take excessive amounts of oral calcium and active vitamin D, sometimes exceeding 50 pills daily. Such intensive treatment schedules disrupt daily life, often requiring medications at all hours, including during the middle of the night. Over time, conventional therapies, especially high doses of calcium, can elevate the long-term risk of multiple organs, including the cardiovascular, respiratory, renal, and central nervous system.

This burdensome standard of care compromises physical health and quality of life. Many patients are unable to work, care for their families, or may even become disabled, as they are constantly managing symptoms and living with the fear of further health deterioration.

How do you anticipate palopegteriparatide will change the standard of care for managing hypoparathyroidism?

Palopegteriparatide, a prodrug of parathyroid hormone (PTH), is designed to provide continuous exposure to active PTH over the 24-hour dosing period. Patients who have struggled to care for their families or hold on to a career due to cognitive and physical functions and people who were previously bedridden or dependent on daily IV calcium infusions now have a potential treatment option that could help get them back on their feet. With this recent approval, the hypoparathyroidism community finally has access to an alternative treatment option for managing their disease and hopes for a different outlook in the future.

How important is educating their patients about the risks and signs of hypoparathyroidism following thyroid surgery?

Clear and effective communication from physicians about the risks and warning signs of hypoparathyroidism is crucial, as early diagnosis and treatment are essential for successfully managing the condition. During patient discussions, physicians should emphasize key symptoms such as muscle cramps, tingling, fatigue, and inability to function cognitively (brain fog). The patient must understand these are vital symptoms and report them to their physician.

Unfortunately, many patients, especially those with the genetic form of the condition, are often misdiagnosed and treated for unrelated issues like epilepsy due to symptoms such as tetany and seizures. In some cases, individuals remain undiagnosed for years. For instance, I recently learned about a patient who wasn’t diagnosed until the age of 42 after undergoing genetic testing. They had lived their life with low serum calcium and the symptoms that come with that without PTH testing. Once they were tested for PTH and sent for genetic testing, it all became very clear, and they are now being treated appropriately and living a very different life. Another patient, in their 80s, underwent genetic testing after living their whole life with symptoms but never being diagnosed or treated appropriately. This underscores the critical need for thorough follow-up care with labs and genetic testing, increased awareness, and vigilance in identifying hypoparathyroidism early.

In part 2, Keating reviews the role of advocacy groups, long-term monitoring with hormone replacement therapy, and more.