A Guide To Understanding Hypoparathyroidism In Adults

By Johanna Pallotta, MD and Antonia Stephen, MD

Among the many hormone disorders that fall within the category of endocrine disease, one of the most uncommon is hypoparathyroidism, a rare and complex condition in which the body secretes abnormally low levels or no parathyroid hormone (PTH). And although they have similar names and are situated close to one another, the function of the thyroid and parathyroid (parathyroid means “near the thyroid”) glands are completely separate.

What are the parathyroid glands?

The parathyroid glands are located in the mid-lower neck, usually adjacent to the thyroid gland. In their normal state, the parathyroid glands are quite small, measuring only a few millimeters in diameter and weighing, on average, less than 50 millagrams (around one–thousandth of an ounce). There are typically four parathyroid glands: two on the left and two on the right. Their function is to produce PTH, which is responsible for controlling calcium metabolism in our bodies. PTH works to keep our blood calcium level within a normal range by means of its effect on the bones, which store calcium, the intestines, which absorb calcium, and the kidneys, which excrete calcium.

When parathyroid glands are normal in size and are functioning properly, the PTH level increases slightly when the blood calcium level goes down and decreases slightly when the blood calcium level goes up. When the parathyroid glands and calcium metabolism are working normally, our bones are encouraged to absorb and deposit calcium, keeping them strong and resistant to fractures.

Hypoparathyroidism occurs when the parathyroid gland(s) do not produce adequate levels of PTH, resulting in hypocalcemia (low calcium levels), which can be harmful and life-threatening, especially when severe in the first few days following its appearance.

Having the right amount of calcium in your blood is essential to proper functioning of many organs and systems including the heart, kidneys, nervous system, bones and teeth. Thus, people who lack adequate PTH may experience muscle problems, bone damage, kidney damage, heart problems, cognitive issues and emotional swings.

What are the causes of hypoparathyroidism?

By far, the most common cause of hypoparathyroidism in adults is surgery on or around the thyroid and parathyroid glands. When the thyroid is surgically removed, the tiny parathyroid glands can be mistakenly removed or damaged, resulting in hypoparathyroidism.

Another surgical cause of hypoparathyroidism is deliberate removal of the parathyroid glands themselves. This can be necessary when the parathyroid gland(s) becomes too overactive and increase in size and PTH production (hyperparathyroidism). High levels of PTH can cause hypercalcemia (high calcium levels) and osteoporosis as well as other problems. Hyperparathyroidism usually affects one parathyroid gland, but may affect all four.

Dysfunction of the parathyroid glands after thyroid or parathyroid surgery can be temporary or permanent. Temporary hypoparathyroidism happens quite often after thyroid surgery, but the condition usually subsides within days or weeks. Fortunately, permanent hypoparathyroidism is a rare surgical complication resulting from the removal or damage of too much parathyroid tissue. Fortunately, only one parathyroid gland, or even just part of one, needs to survive and recover for the patient to have normal calcium metabolism. Permanent hypoparathyroidism happens in about 1 or 2 percent (one to two out of 100) of thyroid and parathyroid surgeries when done by a highly experienced thyroid and parathyroid surgeon.

Another cause of hypoparathyroidism is destruction of the parathyroid glands by the immune system in people with autoimmune diseases, where the body mistakenly attacks its own tissues. This condition is usually diagnosed in childhood or adolescence. Antibodies damage the parathyroid glands, and they are not able to produce enough PTH to maintain normal calcium levels.

There are other rare causes of hypoparathyroidism, including genetic disorders resulting in abnormal parathyroid gland development or PTH production; infiltration of the parathyroid glands by iron overload (hemochromatosis), an accumulation of iron in the body from any cause; granulomatous diseases (which occur when the immune system attempts to wall off substances it perceives as foreign); radiation-induced damage; or viral infections. These are all extremely rare.

What are the symptoms of hypoparathyroidism?

The symptoms of hypoparathyroidism can be acute or long-term. Those caused by an acute drop in calcium levels are related to the fact that calcium stabilizes nerves and muscles. When the calcium level in the blood is low, the nerve and muscle endings in the body become irritable. This is called “tetany.” The symptoms of tetany can be mild (numbness around the mouth, tingling in the hands and feet, muscle cramps) or severe (hand, foot and throat spasms, and seizures). The more severe symptoms of low calcium levels, if left untreated, can be life-threatening.

When the hypoparathyroidism continues for a long period of time, other organs can be affected. Some of these problems are a result of the high doses of calcium supplements needed to maintain a safe and normal blood calcium level. Kidney stones can develop if the calcium level in urine is too high. Patients with chronic hypoparathyroidism can have other effects such as basal ganglia (brain) calcifications, cataracts, skeletal changes, dental abnormalities and skin and nail changes. These problems can be minimized or avoided if the calcium level is watched carefully and remains in the recommended range.

How is hypoparathyroidism diagnosed and treated?

The diagnosis of hypoparathyroidism is made based on blood work when an abnormally low calcium level is detected with a low PTH level – usually well below the normal range in this situation. Other laboratory abnormalities include a higher-than-normal phosphate level.

Once hypoparathyroidism is diagnosed, other tests should be done to help manage and treat the patient. These tests include a blood level for vitamin D and magnesium and a collection of urine for 24 hours to measure the urine calcium level. Vitamin D and magnesium stores in the body play an important role in calcium balance, and this becomes even more important in patients with hypoparathyroidism.

At present there is no known cure for hypoparathyroidism. Treatment includes oral calcium and vitamin D. For adults with stable chronic hypoparathyroidism, the dose of oral calcium is typically one to two grams of elemental calcium daily in divided doses. This can be calcium carbonate or calcium citrate. Calcium carbonate is less expensive and works very well for most patients. Calcium citrate may be better absorbed and cause less stomach discomfort, so this may be preferred by some patients. All of these types of calcium are available as over-the-counter supplements.

The vitamin D recommended is called calcitriol or Rocaltrol® and usually requires a prescription. This special form of vitamin D should be used, since regular vitamin D supplements do not work well in patients with primary hypoparathyroidism.

An important aspect of calcium and vitamin D supplementation is that the blood calcium level should be kept in the low-normal range. The magnesium should be kept normal for PTH to be secreted and also for its actions. That is, the patient should take only enough calcium and vitamin D to keep the symptoms of hypocalcemia (low blood calcium level) at a minimum. Without enough parathyroid hormone, too much calcium may be deposited in the kidney and urine, which can lead to kidney stones and kidney damage. Keeping the blood calcium in the low-normal range prevents this. If the calcium level is allowed to get too high, or even in the mid-normal range, calcium supplementation has to be reduced.

If the calcium level cannot be maintained with calcium and vitamin D supplements and the patient is still symptomatic, another option is the addition of a special medication called recombinant PTH 1-84. This is a medication injected under the skin that should only be used with the supervision of an expert in the treatment of hypoparathyroidism. If the patient feels well on calcium and vitamin D, then this medication is not recommended.

Every patient with primary hypoparathyroidism should have an endocrinologist experienced in treating this condition and should visit with them at least every six months.

Dr. Johanna A. Pallotta, FACE, is Associate Professor of Medicine at Harvard Medical School and a Senior Physician in the Department of Medicine Division of Endocrinology and Metabolism with Beth Israel Deaconess Medical Center in Boston. Her areas of clinical interest include thyroid disease, pituitary disorders, osteoporosis and a particular expertise in parathyroid disease. In addition to caring for patients with disorders of the thyroid, pituitary and bone, she established and supervises a referral center for parathyroid problems.

Dr. Antonia Stephen received her medical degree from Harvard Medical School and completed her general surgical residency at the Massachusetts General Hospital and an endocrine surgical fellowship at the Cleveland Clinic. She then joined the surgical staff at Massachusetts General Hospital in the Division of Surgical Oncology. Her clinical practice is focused on the surgical management of patients with endocrine tumors. Her particular areas of clinical expertise are in minimallyinvasive parathyroid surgery, the treatment of thyroid cancer and laparoscopic adrenal surgery. She also performs in-office ultrasound and ultrasound-guided biopsies of thyroid nodules and parathyroid tumors

Hypoglycemia Alert Dogs Saving Lives One Sniff At A Time

Like so many dog owners, Becky Hertz’s bond with her four-and-a-half-year-old Goldcrest Labrador Retriever runs deep. But in her case, it’s a potentially life-saving covenant.

Diagnosed with type 1 diabetes when she was 14 years old, Hertz is the proud (and grateful) owner of Fuji, a hypoglycemia alert dog.

Medical service assistance animals such as guide dogs for the visually impaired, dogs that “hear” for the hearing impaired, or dogs that are trained to retrieve items for those who are wheelchair-bound have been serving their masters for decades, but dogs like Fuji are relatively new to the world of canine service companions. Hypoglycemia alert dogs are trained to sense when blood sugars are reaching an unsafe level in people with insulin-dependent diabetes and respond to their human partner with a specific alerting behavior prior to the onset of a low blood sugar episode.

Although unable to determine exactly how dogs sense low blood sugar, scientists believe they pick up on scents created by the chemical changes that occur in the person’s body using their powerful sense of smell: while humans have five to six million olfactory receptors, a dog has up to 220 million, producing a scenting skill that is a thousand times more sensitive than that of humans.

Becky had served as a puppy raiser for Canine Companions for Independence (CCI) for several Hypoglycemia Alert Dogs Saving Lives One years, so she was aware that many of its dogs and those from Guide Dogs for the Blind were retrained for diabetic alert work by Dogs 4 Diabetics® (D4D) and submitted an application to be put on its waiting list. Once accepted into the program, she says she was unprepared for the intensive, two-week classroom and field training she was required to complete as part of the placement process, despite her experience with medical service dog programs.

“I thought, ‘I’ve puppy raised, so it’s not going to be that hard on me,’ but it was a physical and emotional rollercoaster,” Hertz recalls. “You need to learn the commands that the dogs know, learn about their body language, how to make sure their behavior in public is appropriate and then pass rigorous testing. Gosh, we even had one full day of dog first aid training, because that dog is precious and you have to be able to protect the dog.”

Clients work with a variety of dogs during training to determine the most suitable match, with trainers taking into account the lifestyle, personality and abilities of each client. Matches can occur during or after completion of the training class under a trial placement agreement, and it can take up to a year or more after the dog is placed with a client for the person/dog team to graduate from the program.

Originally paired up in July 2010, Fuji and Becky officially graduated from the program in November 2011. Since then, the partnership has far exceeded expectations.

“She pretty much follows me wherever I go, sleeps next to my bed at night, and alerts me both when I am aware and unaware that my blood sugar has dropped. But what’s really cool is that she will alert me if my blood sugar drops too quickly,” Becky notes.

“Once she alerted me when my blood sugar was a 130, which is a normal range for me,” she adds. “What we’re taught is that if your dog alerts and your blood sugar is in normal or high range, that you need to recheck 10 to 15 minutes later, and if there is a 10 percent or greater drop, then it’s a valid alert. So she can also catch it while it’s dropping and before it gets too low.

” While some alert dogs may be taught to sit and stare at their handler or touch the person with their nose, Becky says D4D trains their dogs to “bringsel.” The bringsel is a small soft tab type of object that hangs off the dog’s collar, and when they alert, they’re supposed to grab their bringsel and hold it in their mouth.

Much to Becky’s amusement, Fuji “doesn’t like her bringsel,” she says. Instead, “she will put her front paws on me when she alerts, which she does at least once a week, if not more.

” Fuji shares the Hertz household with Becky’s husband Reid, son Zach and Cody, her adored eight-year-old pit bull, but it’s evident by talking to Becky that the “constant companion” has carved a special place in her heart.

“The relationship is different that with other pets,” she says. “It’s a symbiotic bond that’s difficult to define. It’s almost like a child in the sense of wanting to protect her and that everything is okay with her, because of the service she provides me. I was very, very fortunate to get her.”

(Editor’s Note: Although Dogs for Diabetics estimates the direct cost associated with breeding, raising and training its service dogs at $25,000, the non-profit organization places its dogs with clients for a minimal cost, currently at $150 for an application fee and training materials. Becky recommends that prospective alert dog owner/handlers exercise caution and do their research when trying to find a legitimate training service. She suggests Assistance Dogs International as a good starting point.) Hypoglycemic alert dogs are trained to sense when blood sugars are reaching an unsafe level in people with insulindependent diabetes.

Your Doctor Thinks You May Need Parathyroid Surgery

Your Doctor Thinks You May Need Parathyroid Surgery

Parathyroid glands are small, pea-shaped, hormone producing glands that reside in your neck in the area of your thyroid gland. They produce a hormone called parathyroid hormone (PTH) that helps control the amount of calcium that circulates in your body.

Why Might I Need Parathyroid Surgery?

The most common reason for needing parathyroid surgery is a diagnosis of primary hyperparathyroidism [hahy-per-par-uh-thahy-roi-diz-uhm]. Primary hyperparathyroidism is usually caused by a benign (non-cancerous) growth of one of four parathyroid glands that everyone has in their neck. This abnormal parathyroid gland overproduces PTH (parathyroid hormone), usually leading to high levels of calcium and PTH in your blood. This can cause symptoms such as those listed below.

Additionally, patients on dialysis because of kidney failure can develop hyperparathyroidism. This is called secondary hyperparathyroidism because the problem does not start in the parathyroid glands, which in this case enlarge due to kidney failure. Secondary hyperparathyroidism due to kidney failure may be referred for parathyroid surgery because of very high levels of PTH in the blood.

What are Some of the Benefits to Having Parathyroid Surgery?

After a successful parathyroid surgery, your blood levels of calcium and PTH will return to within the normal range. This will lead to improved bone density, decreased risk of bone fractures, lower risk of kidney stones and a potentially improved quality of life. Many of your initial symptoms will improve. However, since some of your symptoms such as fatigue are common, they may be caused by other medical conditions and non-condition-related factors. Therefore, they may not go away even after successful surgery.

What to Expect the Day of Surgery

You will be asked to arrive at the hospital about two hours before your scheduled surgery time. You should have stopped taking any aspirin, blood thinners or nonsteroidal anti-inflammatory agents (such as ibuprofen [Advil] or naproxen [Aleve]) one week before your surgery date. The majority of surgeons will perform parathyroid surgery under general anesthesia, though some specialized centers may also offer the surgery under local anesthesia. Surgery can take anywhere from 30 minutes to three hours once you are asleep. It depends on how quickly the surgeon can find and confirm the removal of the abnormal gland(s).

Nervous System Kidney/Urinary Digestive System Musculoskeletal
• Mood swings • Kidney Stones • Stomach or duodenal ulcer • Bone pain
• Depression • Nocturia (going to the bathroom a lot at night) • GERD (acid reflux) • Joint pain
• Forgetfulness • Worsening kidney function • Pancreatitis • Bone fractures
• Irritability • Abdominal pain • Osteoporosis
• Concentration problems • Constipation • Generalized weakness
• Fatigue

Most patients that undergo parathyroid surgery will be able to go home the same day. However, your surgeon may have you stay overnight if you have multiple other medical problems, undergo a more extensive surgery, or live a far distance from the hospital. You will be able to eat, drink, and talk that same day right after surgery and can go back to your normal activities as early as the next day, depending on how you feel.

The size of your incision will likely depend somewhat on if the surgeon is able to localize the hyperfunctioning gland(s) before surgery. However, in 2012, the majority of all parathyroid surgeries were “minimal access,” with most incisions ranging from three-quarters to 1 ½ inches across. There are generally no stitches that need to be removed.

Tools to Assist the Surgeon in Finding or Confirming the Removal of the Abnormal Parathyroid Gland

Before your scheduled surgery date, your surgeon will have you undergo “localization” imaging to help locate the abnormal parathyroid gland. This may be an ultrasound done by either a radiologist or an endocrinologist; a Sestamibi scan, which is a nuclear medicine test where a radiologist injectsa weakly radioactive diagnostic material that will hopefully concentrate in the abnormal gland; or, in special circumstances, a CT scan. The ability of each exam to accurately locate the abnormal gland can vary among radiologists, but in general two-thirds to three-quarters of patients will go into surgery with a localized gland, making it much easier for the surgeon. Your vitamin D level also should be checked before surgery, since a low vitamin D level can delay your recovery.

During surgery, most surgeons will test your PTH levels which will confirm, while you are still asleep, that the correct hyperfunctioning parathyroid gland has been successfully removed. Some surgeons may also utilize intraoperative ultrasound or a radioguided probe (another nuclear medicine technique) during surgery to help locate the abnormal gland.

What to Expect After Surgery, and Are There Any Side Effects?

The most common complaint after surgery is a mild sore throat, which generally doesn’t last more than three to seven days, and mild generalized fatigue. Some people may complain of a non-productive cough or some pain when swallowing, both of which will go away in one to two weeks. Sometimes, hypocalcemia [hahy-poh-kal-see-mee-uh] (low blood calcium levels) can happen in the first few days after surgery, and you would experience numbness or tingling around your mouth or in your fingertips. The treatment is taking more than the usual amount of calcium and vitamin D supplements until the symptoms resolve.

The majority of people who undergo the surgery only need to take off a few days from work. Your surgeon will usually check your blood calcium level the day after surgery as a second confirmation that your calcium level is now in the normal range. In addition, six months after surgery and yearly thereafter, depending on your endocrinologist and surgeon, you will have your blood checked for both calcium and PTH levels.

One to three weeks after surgery you will have a post-op visit with the surgeon to go over your pathology (what your parathyroid gland looked like under the microscope) and to check your wound. Most patients heal very well from surgery and have minimal residual scarring at six months. However, it is important to apply suntan lotion to the surgical site for up to one year after surgery to prevent darkening of any scar.

In the hands of an experienced surgeon, over 95 percent of parathyroid surgeries result in a “cure.” A failure is the result of either the surgeon being unable to find the abnormal parathyroid gland(s) at the time of surgery or your PTH level increasing during the first six months after surgery, which would indicate one or more of the remaining parathyroid glands in your neck are also hyperfunctioning.

One of the unusual complications of parathyroid surgery is injury to the recurrent laryngeal [luh-rin-jee-uhl] nerve. Each person has two of these nerves, a right and a left, that run along the side of your windpipe and control your voice. An injury to one of these nerves results in hoarseness. Many patients that experience hoarseness right after surgery have a temporary nerve injury that will get better over time. However, in about one percent of patients the injury will be permanent. This number is lower in the hands of an experienced surgeon. Another unusual complication of surgery, particularly in those who have more than one parathyroid gland removed or are undergoing repeat surgery for hyperparathyroidism, is a permanently low blood calcium level. This is known as hypoparathyroidism [hahy-po-par-uh-thahy-roidiz-uhm] (too little parathyroid hormone). Treatment consists of calcium supplements and taking more than the usual amount of recommended vitamin D supplements.

Questions to Ask Your Surgeon

You should be comfortable with your surgeon and feel confident that you are in good hands. Studies have shown that experienced surgeons have higher rates of successful surgeries and lower rates of complications, so you should ask your surgeon how many parathyroid operations he/she performs every year, what other surgeries they do in their practice, and what their complication rate and success rate are. You should also ask your surgeon what, if any, testing is performed in the operating room (such as measuring PTH hormone) to confirm that the operation was successful.

Dr. Melanie Goldfarb is a graduate of Brown University. She received her medical degree from the University of Illinois-Chicago and completed her general surgery residency at Beth Israel Deaconess Medical Center/Harvard Medical School. She then completed an endocrine surgery fellowship at University of Miami and is now an Assistant Professor of Surgery at University of Southern California Keck School of Medicine. Her clinical practice is focused on the surgical management of patients with thyroid, parathyroid and adrenal tumors and her research on adolescent and young adult thyroid cancer.

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