CEO, Hypopara UK
Not many people know about their parathyroid glands until something goes wrong. When the glands fail they stop producing sufficient parathyroid hormone (PTH) to control calcium metabolism, causing hypoparathyroidism (or hypopara), a rare and challenging condition.
Rarely, failure is due to genetic or autoimmune disorders but most commonly it is due to surgical removal of (or damage to) the glands – occasionally necessary but more often accidental. About 22 per 100,000 people in the western world have post- surgical hypopara (PoSH) – and this is rising with increasing rates of thyroid cancer.
Hypopara UK, a patient organisation supporting people with all types of hypopara, says: ‘Around 75% of our membership have post- surgical hypoparathyroidism and most of those had a thyroidectomy. They went into hospital with one condition and came out with another – rare, incurable and life threatening.
Liz Glenister, CEO of Hypopara UK, has brittle post surgical hypopara following a thyroidectomy for thyroid cancer in 1992.
“I was told it was a very straightforward operation. A couple of days post-op I was lying in a total body seizure, completely contorted and unable to move a muscle yet conscious. It took me years to get over the trauma and, like many others, have flashbacks every time I have a calcium hypo.”
In common with many rare diseases, it took her years to get a diagnosis and to find an endocrinologist who understood the condition which requires daily management of calcium levels – ‘basically by guesswork’. Liz had to leave teaching and pull out of her MA due to constant hypos but in 2005 she set up Hypopara UK to provide support and information and to campaign for better treatment. ‘I’d been told by London teaching hospitals that I was a one- off but I was sure there were other patients out there and I couldn’t bear to think that they were going through the same thing.’
Hypoparathyroidism severely impacts quality of life and is associated with renal failure and an increased risk of death. Liz says: ‘We have come a long way but there remains an urgent need to improve surgical outcomes and address the very real risks which patients face.’
The BAETS audit recorded a gradual decrease in the annual incidence of long-term PoSH (now 6.5%) but, as Fausto Palazzo, clinical lead, consultant endocrine surgeon and honorary senior lecturer at Imperial College London, points out, ‘ it remains an under-reported complication’.
Hypopara UK works with top endocrine surgeons to improve this. ‘’ PoSH is a serious condition that is generally not well understood. Our specialist advisors recognize the need for a change in practice and attitudes and are dedicated to furthering education and research.’’
Consultant Endocrine Surgeon, Radu Mihai from Oxford University, found that PoSH had a severe impact on quality of life and led to frequent returns to A&E. This year Consultant Endocrine Surgeon, Saba Balasubramanian from the University of Sheffield will trial an intra-operative imaging device and calls for more research on monitoring and treatment which is currently inadequate.
Tom Kurzawinski, a Consultant Endocrine Surgeon at UCL is currently developing a simple point-of-care, home tester for patients to test their calcium levels. Monitoring is a major problem for hypopara patients who are not unlike diabetics with their fluctuating levels and need for urgent blood tests. ‘Imagine a diabetic without replacement hormone or a home tester to prevent hypos, says Liz, ‘there would be an outrage. But this is what happens when you’re rare.’
‘’Living with PoSH can be very distressing so this research gives us hope. For the newly diagnosed, it is a difficult journey towards acceptance but patients are encouraged to know that they are being heard and keen to get involved in studies that may improve their lives. ’’