What is primary hyperparathyroidism (pHPT)?

Hyperparathyroidism is the illness that results from too much parathyroid hormone. There are four parathyroid glands that are each the size of a grain of rice and are usually located behind the thyroid gland. These four glands regulate calcium in the body. Primary hyperparathyroidism results when one or more adenomas (non-cancerous tumors) form in one or more of the parathyroid glands and secrete too much parathyroid hormone (PTH), which disrupts calcium regulation, leading to hypercalcemia, or high calcium in the blood. Hypercalcemia has several bad consequences. As a result of calcium being leached from the bones due to high PTH, primary hyperparathyroid disease can be a root cause of osteoporosis.

How is primary hyperparathyroid disease diagnosed? 

Primary hyperparathyroidism is diagnosed through blood tests. These tests include serum calcium, PTH (parathyroid hormone), and vitamin D-25. Most patients present with:

High serum calcium (normal = 8.7 – 10.1 mg/dl)
High PTH (normal = 15 – 65 pg/ml)
Low vitamin D-25 (normal = 30 – 100 ng/ml).
*Note that normal lab ranges may vary from lab to lab.

According to the experts, persistent calcium levels 10.1 and above (in an adult) is almost always a parathyroid tumor. Approximately 20-25 percent of cases don’t present classically. Normohormonal primary hyperparathyroidism occurs when calcium is high, but PTH is still within the normal range. Normocalcemic primary hyperparathyroidism occurs when calcium is in the normal range, but PTH is elevated. Additional lab tests including ionized calcium and urine calcium may be helpful in making the diagnosis when the presentation is not classic.

Learn more about the function of your parathyroid glands and the classic presentation of the disease by listening to this podcast:

 

Learn more about the normohormonal and normocalcemic presentations by listening to this podcast: 

What are the symptoms? There are many. The mnemonic Stones, Bones, Moans, Groans, and Psychic Overtones is one way the medical community refers to symptoms of pHPT. These include, but are not limited to, bone and joint pain, osteopenia, osteoporosis, kidney stones, heart fluttering or palpitations, heart attack, hair loss, inability to sleep through the night, brain fog, gastrointestinal distress, GERD, anxiety and depression. Symptoms are often vague and erroneously attributed to various conditions such as menopause, osteoarthritis or psychiatric disorders; although a parathyroid adenoma later proves to be the root cause of the patients’ symptoms.

Are there any drugs that can treat this, instead of having surgery?
There are no drugs that can reverse the disease process and make the adenoma shrink or disappear. Surgical removal of the parathyroid adenoma is the only cure. It is our understanding that the drug Cinacalcet is used to treat those with secondary hyperparathyroidism, which is hypercalcemia that is secondary to kidney disease. It is also sometimes prescribed for those who are not eligible for surgery and/or who have had failed surgeries and cannot be cured. Additionally, the osteoporosis drugs (Bisphosphonates) cannot improve bone density when an adenoma is present.

Can I just “wait and see” whether my symptoms persist or worsen? 
Excess calcium in the blood stream can cause serious complications over a period of years. The calcium interferes with digestion and can lead to GERD and ulcer formation. The excess calcium can deposit in the kidneys as kidney stones and ultimately lead to kidney damage. Excess calcium in the blood can cause calcification in the blood vessels and ultimately heart disease and heart attack. Left untreated, calcium can be deposited all over the body and cause calcification of various organs. Depletion of calcium from the bones leads to osteoporosis and fractures.

What should you do if you suspect that you have primary hyperparathyroid disease? 

Become an informed patient: Request copies of your lab work. Understand that the normal ranges for calcium values vary slightly from lab to lab, but more importantly often do not take into consideration variations for a person’s age. Adults over age 30 generally feel best with calcium values in the 9’s with an upper limit of 10.1.

Research: Do not assume that your doctor will know a great deal about this disease, particularly if you present atypically as either normohormonal or normocalcemic.

Advocate: If doctors are dismissive of values you now understand are indicative of the disease, or your doctor proposes the ‘wait and see” model of care, be prepared to educate. There is no such thing as “mild” primary hyperparathyroid disease and it does not get better on its own. Surgery is the cure.

Self-refer if necessary: We all found we needed to self-refer to an expert surgeon. Many patients do not realize that this is indeed possible.

Pick your surgeon carefully: All endocrine surgeons are not created equal. Some surgeons only remove the parathyroid gland with the adenoma that is seen on imaging studies, such as Sestamibi scans or ultrasound scans, and do not look for other bad glands. Others will do exploratory surgery for several hours in the hope they can find the bad glands, as they are very difficult to locate. As a result, excessive scar tissue forms, making it far more difficult and sometimes impossible for a patient to have a successful second surgery if this becomes necessary. It is important to find an expert surgeon that has performed hundreds of parathyroidectomies. Ideally, they should know how to locate and assess the 4 glands in under an hour.

We are NOT medical professionals….we are patients turned advocates! Our mission is to raise awareness and advocate for IMPROVEMENT in diagnosis and standards for surgical treatment of Primary HyperPARAthyroid disease (pHPT). According to the medical community, surgery is the cure and in our cases, it was indeed with the right surgeons! The decision whether to have any type of surgery is a deeply personal one and as always, it is important to weigh the benefits against the risks and carefully select the surgeon.