Primary Hyperparathyroidism

In Primary Hyperparathyroidism (PHPT or Hyperpara), too much parathyroid hormone is produced by one or more of the parathyroid glands because they have become enlarged or overactive. This in turn causes the body to release calcium from the bones into the blood and results in high calcium levels (hypercalcaemia).

Causes

• The most common cause of PHPT is a parathyroid gland becoming enlarged due to the development of a benign (non-cancerous) tumour called an adenoma. This is more commonly diagnosed in women, particularly postmenopausal women, but can affect both men and women and all ages, including, less commonly, children.
• Sometimes all four parathyroid glands may become enlarged – this is called parathyroid hyperplasia. This may occur sporadically (without a family history) or as part of three familial (inherited) syndromes: multiple endocrine neoplasia 1 (MEN 1) and MEN 2A, and isolated familial hyperparathyroidism. In MEN 1, the problems in the parathyroid glands are associated with other tumours in the pituitary and the pancreas. In MEN 2A, overactivity of the parathyroid glands is associated with tumours in the adrenal gland or thyroid.
• Radiotherapy treatment to your head or neck may increase the risk of developing a parathyroid adenoma or carcinoma (cancer). Only in extremely rare cases indeed will the tumour be due to parathyroid cancer. If you have been diagnosed with parathyroid cancer please go to our Parathyroid Cancer

Symptoms

In Primary Hyperparathyroidism you may not notice any specific symptoms, or you may just feel ‘not quite right’, but for many the symptoms can be severely debilitating. Sometimes the condition is only discovered by chance while investigating something else.

Many of the symptoms of PHPT are vague, and as a result the condition can go undiagnosed for long periods. Tiredness, anxiety, thirst, polyuria (frequent urination), and body aches and pains can all be features of PHPT.

There does not seem to be any correlation between particular symptoms and the level of calcium in the blood. People with a slightly high calcium levels may have very severe symptoms while people with very high calcium levels may not be aware of their symptoms. This may be because the condition can develop over a long period of time and a gradual change is harder to notice.

For some people, symptoms may be subtle and develop gradually, and as a result are sometimes attributed to other causes such as menopause, depression, chronic fatigue, fibromyalgia, or to stress and the general pressures of life.

As calcium is found in every cell in the body, high levels of calcium and parathyroid hormone can affect bone, kidneys, muscles, nerves and the gut, as well as the emotions and cognitive function.

A substantive poll of our hyperpara members at Parathyroid UK members found the most common symptoms, in order of frequency, to be:

• fatigue/ feeling tired and lethargic
• brain fog / loss of concentration/ confusion
• anxiety/depression/low mood/lack of enthusiasm in life
• muscle pain and weakness
• bone pain
• joint pain
• irritability
• frequent urination
• increased thirst
• digestive problems, eg gastroesophageal reflux disease (GERD)
• insomnia

Other warning signs can include:

• loss of appetite
• nausea
• constipation
• upper abdominal pain
• migraine like headaches

If left untreated, symptoms can become much worse.  

In severe cases, extremely high levels of calcium (hypercalcaemia) can cause:

• vomiting
• drowsiness
• dehydration
• confusion – difficulty thinking and speaking clearly
• agitation
• muscle spasms, tremors.
• bone fractures
• irregular heart beat
• high blood pressure
• loss of consciousness
• coma and, very rarely, if not treated, death

Hypercalcaemia can be a life threatening condition if it is not treated.

Long term effects

If Primary Hyperparathyroidism goes undiagnosed, further complications, can develop.

These may affect the following:

Kidneys

The kidneys play an important role in regulating the blood calcium levels and operate to restore the correct levels by removing excess calcium from the blood. Over a prolonged period of time excess calcium can accumulate and form stones within the kidneys. Small stones may be passed in the urine without you noticing but larger stones may get stuck. These can cause pain in your loin area that radiates to your groin. You may also notice blood in your urine. Kidney stones can be very painful. Continual high levels of calcium in your blood can damage your kidneys and eventually can cause kidney failure.

Bones

Increased parathyroid hormone in your blood causes too much calcium to be released from your bones, which can lead to weakness and bone pain. This can eventually cause osteopenia and osteoporosis. It also makes the bones more susceptible to fractures. If bones break after a low impact fall it may be indicative of pHPT.

Eyes

Calcium can be collect in the cornea of your eye (corneal calcifications) but this doesn’t usually cause any symptoms.

Pancreas

Although it is rare, high calcium can cause inflammation of your pancreas and this causes upper abdominal pain (pancreatitis).

Stomach

High calcium levels can stimulate the production of excess acid in your stomach and lead to peptic stomach ulceration.

Brain

Calcium plays an important role in the normal working of the brain and spinal cord. Patients whose PHPT goes undiagnosed for a long time and who therefore suffer from hypercalcaemia over a long period of time, may develop some of the following symptoms: fits, uncoordinated muscles (affecting walking, talking and eating), changes in personality and/or hallucinations.

I asked my GP to refer me for a second opinion, knowing that I needed surgery, but he refused. I left the GP practice that day and joined my partners’ GP surgery. Within a couple of weeks, I had an appointment with my new GP who was very helpful and understanding

Read Al's story

Diagnosis

The subtle nature of the symptoms of PHPT can result in the condition going undiagnosed for some time, although the diagnosis of PHPT is generally clear once appropriate tests are done.

Some people may be diagnosed after a routine calcium blood test shows a high calcium  level. Mostly,  doctors may decide to request a calcium test ( or some patients may request a blood test themselves) based on symptoms which may be caused by high calcium.

The key to diagnosis is to measure the level of calcium in the blood at the same time as the level of parathyroid hormone as it is the relationship between them that is important in reaching a diagnosis.

Vitamin D should also be tested to rule out vitamin D deficiency which may be causing secondary hyperparathyroidism.

These tests can all be done by your GP

Blood tests

• total calcium
• parathyroid hormone (PTH)
• vitamin D (25 OH cholecalciferol)
• serum phosphate

What do my results mean?

In a straightforward case of primary hyperparathyroidism,  blood tests will usually show

• high calcium
• high parathyroid hormone
• low vitamin D
• low phosphate

However, sometimes diagnosis is more difficult. Your tests may be normal (within the reference range) or levels not especially high, but you may still be experiencing symptoms. This can lead to a doctor determining you to be a ‘mild case’ and deciding to monitor you for a while but this practice of ‘watch and wait’ can lead to much distress.

Apart from secondary hyperparathyroidism , there are other conditions that occur which are not yet very well understood. They may be more difficult to diagnose and may require more detailed testing.

These are :

• If your calcium is within the normal range but your PTH is raised you may have normocalcaemic hyperparathyroidism where the glands produce too much PTH but the blood calcium hasn’t yet risen.
• If your calcium is raised but your PTH is low you may have normohormonal hyperparathyroidism.

Other tests

Depending on your results you may be given other tests to check how you are being affected. Some tests can be done at your GP surgery but you may also be referred to hospital for further investigations by a specialist (called an endocrinologist).

Further tests may include:

• a vitamin D blood test, if not done before
• kidney function blood tests to investigate how your kidneys are working
• a kidney scan to find out if you have kidney stones
• 24-hour urine test to measure the calcium in your urine
• a bone DEXA scan to measure the density of your bones
• an x –ray to see whether pHPT has caused any damage
• an imaging scan to locate the malfunctioning gland

It is also important to rule out other possible causes of raised calcium, particularly if the parathyroid hormone levels are not very high.

If you are recommended to have parathyroid surgery you may be referred for the following tests to localise the enlarged parathyroid gland(s):

• ultrasound scan of the neck
• sestamibi parathyroid scan
• CT scan of the neck
• a biopsy is sometimes done to localise the adenoma
• parathyroid venous sampling (usually only carried out after previous surgery has failed, and at specialist centres with experience in this procedure)

Treatment

If there is evidence that your health is affected then treatment will usually be recommended. This will usually be surgery but sometimes you may be monitored or offered medical treatment, depending on your circumstances.

Surgery

The only cure for PHPT is surgery to remove the affected gland(s). In the hands of an experienced surgeon the success rates are high, particularly if the affected gland(s) can be located by preoperative scans. However, very often scans are negative or inconclusive. A skilled surgeon will not necessarily regard this as an obstacle to going ahead with surgery.

Can I go straight to a surgeon?

Your GP may refer you to an endocrinologist to arrange the diagnostic tests, but if the diagnosis is already clear from your blood tests, you can ask to be referred to a surgeon with expertise in parathyroid surgery, who can arrange any further investigations needed. These usually include imaging scans to try and locate the gland(s) affected. By going directly to a surgeon, you will avoid long waits for endocrinology appointments and increase your chance of being listed for surgery without too much delay. However, where diagnosis is uncertain, or where other factors need to be assessed, a referral to an endocrinologist may be necessary.

Finding an experienced surgeon

It is important that parathyroid surgery is carried out by a highly skilled surgeon. Do not hesitate to ask about your surgeon’s experience, the number of operations they perform and their complication rate. You can find out more about your surgeon here

https://www.baets.org.uk/wp-content/uploads/Deanonymised-BAETS-Surgeon-Data-2017.pdf

This information is collected by the British Association of Endocrine and Thyroid Surgeons (BAETS) and is updated annually.

This leaflet from BAETS explains what information is collected for the audit. https://www.baets.org.uk/wp-content/uploads/Patient-Information-Leaflet-%E2%80%93-UKRETS.pdf

Take control!

The operation

The malfunctioning parathyroid gland(s) can be removed by surgery. This operation is called a parathyroidectomy and usually brings about a permanent cure.

Most commonly one, or at most two, parathyroid adenomas are removed. However, all abnormal glands may need to be removed if all four parathyroid glands are overactive, as in the rarer condition of parathyroid hyperplasia. In this case it may be possible to leave half a parathyroid gland in situ to avoid developing hypoparathyroidism, a lifelong condition, but this is not always possible. It is essential to discuss your treatment plan with your surgeon. Please visit our section on hypoparathyroidism to learn more about living with this rare condition.

Parathyroid surgery is normally a straightforward procedure most often requiring an overnight hospital stay for recovery. For removal of a single adenoma, most surgeons are able to perform minimally invasive surgery via a small incision which, after healing, leaves a barely visible scar.

Parathyroid UK has helped BAETS to develop a leaflet about having a parathyroidectomy which you can read and download here: https://www.baets.org.uk/wp-content/uploads/Patient-Information-Leaflet-P1-Parathyroid-Operations-in-Adults.pdf

I wish I  had found this group before my operation as I suffered for a very long time – and as its invisible the lack of understanding can make it a harder road. This group really helps – you have people willing to listen on your bad days and help you with advice.

Catherine

Questions to ask your surgeon prior to the operation:

• Will you look at all four parathyroid glands?
• What checks will you do in the operating theatre to ensure you have removed the affected gland(s)?
• How will you protect my voice?
• Will you prescribe calcium for me to take after the operation?
• What follow-up help will be available if I have any problems?

Possible complications after surgery

Complications can arise, but fortunately they are quite rare.

After surgery, low concentrations of PTH cause a condition called hypoparathyroidism which may be temporary or permanent. If you experience any of these symptoms it is very important that you seek advice from your doctor as soon as possible.

Temporary hypoparathyroidism :  This can cause symptoms of low calcium (hypocalcaemia) such as ‘pins and needles’ or cramps which is not uncommon and will usually settle down in a few weeks. These symptoms are a sign of your body adjusting to new PTH levels and usually pass, though you may need calcium supplements and over- the- counter vitamin D3 during this phase.

Permanent  hypoparathyroidism: If low PTH does not recover due to damage of the glands or if you have had most of your parathyroid glands removed ( eg if you have multigland disease) this condition will be permanent. You may experience severe hypocalcaemia after the operation and need urgent treatment. This is a rare condition and you will need lifelong care and treatment with vitamin D analogues, Vitamin D3, magnesium and calcium supplements. Hypopara UK has been supporting hypopara patients since 2005 – please visit our section on Hypoparathyroidism for more information and to join our support group

Hungry Bone Syndrome is an uncommon but sudden influx of calcium into the bones from the blood which can follow parathyroidectomy in patients with severe primary hyperparathyroidism and preoperative high bone turnover. It requires admission to hospital for several days for intravenous calcium to correct low serum calcium levels.

Vocal Chord damage. Very occasionally, there may be damage to the nerve serving your vocal chords (recurrent laryngeal nerve), which can affect your voice. This usually improves within a few months but if you are still experiencing problems with your voice you may be referred to an ENT specialist for advice. This is a very rare complication and an experienced surgeon will be very careful to isolate and protect the nerve during the operation.

Most people recover gradually over the days and weeks following surgery, both from the anaesthetic and while any residual symptoms resolve. However, if during your immediate post-operation days you experience any unusual or worrying symptoms, do not hesitate to seek advice from your GP, the hospital where you had your surgery, or ask for advice from the NHS 111 helpline.

This is the best UK site for Hyperparathyroidism!

Sharon

Follow up after surgery

Your calcium level will be checked after surgery to check that it returns to normal and does not drop too low. You will also have a follow-up appointment with the surgeon after two or three weeks, where your calcium levels will be checked again and you can discuss any concerns about your recovery.

Follow-up after this varies according to local health authority protocols, but your GP may agree to test your calcium and PTH annually, or to monitor your Vitamin D level and if appropriate correct with supplements, especially if you were deficient before surgery.

Bone health should also be monitored, and treatment discussed if appropriate.

If, after a reasonable recovery period, you continue to experience any unresolved symptoms, or new symptoms that may be related to your calcium levels, such as cramps or tingling, it is important to seek advice from your GP, and if appropriate, an endocrinology referral.

It is important to note that Primary Hyperparathyroidism can cause a multitude of symptoms prior to surgery, but not all symptoms experienced are necessarily caused by hyperparathyroidism – for example, pain could be caused by arthritis and therefore may not improve after parathyroid surgery. If symptoms continue, or new ones appear after your Primary Hyperparathyroidism has been cured, the chances are that you have co-existing or new conditions that are unrelated. Nevertheless, they still should be investigated by primary care practitioners, who will be aware of your medical history, to rule out a recurrent or persistent parathyroid issue.

Medical treatment

If your blood calcium is dangerously high you will be referred immediately to hospital for your calcium concentration to  be reduced and may then be referred for an urgent operation. Calcitonin may be given by injection or intravenously ( by drip)

If your calcium is high but you are unable to have an operation for any reason, you may be offered drug treatment to help control your calcium levels. This is also available for more acute cases in the short term while you are awaiting surgery.

Commonly prescribed drugs include:

Cinacalcet (Mimpara). This can lower the concentration of calcium, reduce symptoms and improve quality of life. It does not seem to improve bone density or reduce the risk of developing kidney stones. Some patients may develop side effects, most commonly, nausea.

Bisphosphonates (Alendronate) This is a drug used to treat osteoporosis. It can help to improve bone density, and they may temporarily lower extremely high calcium levels, though they are not recommended long term as they may increase PTH levels or if you planning to have surgery.

Monitoring

If your calcium is normal or only slightly raised, if you are reluctant to have surgery, or if you have other concurrent conditions which may make surgery more difficult or reduce the likelihood of it improving your overall health, an endocrinologist may decide to monitor you for a period of time ( sometimes called ‘watch and wait’).

You will need to have regular tests to monitor your blood calcium, to check your kidneys are working normally and to check your bone density for results which may indicate the need for an operation. Vitamin D levels should be corrected if necessary but discontinued if symptoms worsen.

You will also be monitored in this way if you decline surgery or if surgery is not an option. If you develop vomiting or diarrhea do not hesitate to contact your doctor.

In discussing your treatment plan, you need to be aware that the only curative treatment is surgery and without it the condition may continue to cause damage to your body, particularly to your bones and kidneys.

What can I do while waiting for treatment?

Your kidneys work hard to try and normalise the level of calcium by removing it from your blood. Drinking plenty of water can help to flush the calcium through while you are waiting for treatment. It may also help to avoid foods high in calcium. Exercise is also known to help to reduce calcium levels. However, these actions may be of limited effectiveness.

Advocate for yourself

Ask questions, inform yourself, keep notes of your symptoms and questions you want to ask at your next appointment. You have a right to your test results from both GP and hospital – ask for copies and keep them so you can monitor how levels change over time. If you are nervous about attending appointments and speaking up for yourself, take a trusted partner, family member or friend with you.

Pregnancy

An excellent article by two of our top surgeons explains what to do about surgery if you are pregnant and have primary hyperparathyroidism.  There is also a section in the NICE primary hyperparathyroidism guidance with recommendations about pregnancy.

Patient stories

Read our patient stories of members’ experience of Primary Hyperpara.