Parathyroid UK has nearly 3,500 members, of whom 75% have permanent hypoparathyroidism. When I set up our organisation, this rare condition was largely unrecognised. Today, it is more widely known, but remains quite poorly understood and no less difficult to manage.

MY STORY

In 1992 I went into hospital to have a total thyroidectomy for papillary thyroid cancer. I came out thankfully cured of cancer, but with incurable hypoparathyroidism instead. The hypocalcaemic seizure I had 3 days later was so severe that I needed post-traumatic stress counselling. Unable to stabilise me, my surgeon referred me to an endocrinologist, but my calcium levels were never to settle down. Finally, I was told that I was ‘bringing symptoms on myself ’ and discharged.

For the next few years, I was engaged in a solitary battle for survival and information. I had to fight for the treatment and monitoring I needed. Eventually, I met my seventh consultant, the first with experience of managing hypoparathyroidism. He agreed that I had been severely under-treated, and life began to improve.

LACK OF AWARENESS

Sadly, despite our awareness-raising efforts and the rise of interest in rare disease, this quest for understanding is still part of life for many patients today. We are hugely grateful to our wonderful advisors,¹ who dedicate so much time to education, and to those who learn from them. However, the fact remains that standards of care vary widely, and the reality is a sobering picture indeed. While lack of experience of patients with rare diseases is understandable, it is distressing for patients to encounter a lack of willingness to learn about their condition.

THE NEED FOR SELF-MANAGEMENT

Once diagnosed, the most challenging issue for patients is the daily management of calcium levels. Calcium can fluctuate in response to a myriad of events, yet blood tests are not always accessible. Results take too long when symptomatic, and GPs often have no guidance from consultants. Left to self-manage between appointments, people turn to support groups.

We have found that educating patients on how to self-manage, as far and as safely as they can, is necessary to avoid crises. We follow guidelines and our specialists’ advice, and we also inform patients about the ‘no or low calcium’ protocol,² which has led to great improvements.

Patients are always asked to talk to their doctor, but they are also given appropriate lifestyle advice and can learn about the condition itself: how to recognise symptoms, and how and when to take action if necessary. We direct the urgent cases to A&E; there are too many of these, and we feel anxious and burdened by this responsibility. While some self-management is unavoidable, we need our doctors to have a good understanding of the demands upon us.

UNDERSTANDING MANAGEMENT GOALS

Crucially, the aim is to maintain serum calcium at a high enough level to prevent symptoms, but low enough to protect renal health. And herein lies the hypoparathyroidism Sisyphean task.

Patients can feel very symptomatic within the normal reference range. Most have their own level where they feel comfortable and symptom-free. This level may be above the recommended goal but, as stated at the Society for Endocrinology BES conference last year, if kidney function permits, allowing the patient to maintain calcium at this level can make a huge difference to their quality of life.

Compounding this problem is the lack of understanding around vitamin D. Patients are still frequently told that they don’t need vitamin D3, because they are already on alfacalcidol or calcitriol. We do. The need for us to take vitamin D3 (and magnesium) is enshrined in the European Society of Endocrinology guidelines³ and is reiterated by our advisors, so receiving conflicting advice is very confusing for patients. A patient with normal calcium can feel symptomatic due to low vitamin D or magnesium levels. Sufficient levels of alfacalcidol, vitamin D3 and magnesium allow us to get the calcium we need from our diet, which improves stability and reduces hypercalciuria.

Changes to medication must be made incrementally. In our support groups, we see people suffering the consequences of mismanagement every day. People are regularly being admitted to hospital because their medication has been over- or under-adjusted: a distressing experience that can be avoided. Even tiny changes in medication can cause dramatic changes in symptoms.

Emergency care, too, needs consideration. Patients need access to urgent blood test results, a plan of action and an emergency contact. We have now produced an emergency medical card, but a consultant’s letter would ease A&E visits considerably.

SHARED GOAL SETTING AND DECISION MAKING

After 28 years, my levels have never properly stabilised, yet I feel very fortunate because I have a consultant who listens, reflects, understands my need to feel as good as I can and works with me to find ways to meet that. He recognises that effective management needs to be, as far as possible, a joint endeavour. Mutually respectful, shared, goal setting and decision making can lead to improved outcomes and a better relationship.

However, we are still dependent on our doctors to get things right for us, so I urge you to learn all you can about the condition: read the guidelines,^3–5 attend the Society for Endocrinology Clinical Updates, visit our website and our (virtual) stand at conferences, and work with us to help lessen the daily impact of hypoparathyroidism on our lives. In a shared endeavour we all have an important role to play.

LIZ GLENISTER
Founder and Chief Executive (Volunteer) at Parathyroid UK

REFERENCES

1. Parathyroid UK 2021 Our Medical Advisors www.parathyroiduk.org/about-us/medical-advisors.
2. Al-Sharefi A et al. 2019 Clinical Endocrinology 90 775–780.
3. Bollerslev J et al. 2015 European Journal of Endocrinology 173 G1–G20.
4. Brandi ML et al. 2016 Journal of Clinical Endocrinology & Metabolism 101 2273–2283.
5. Khan AA et al. 2019 European Journal of Endocrinology 180 P1–P22.