How it all started

A few years ago, I came across an article published by Professor Kristian Lovas and his team from Norway, who had been using a hydrocortisone infusion pump since 2007. Their first paper was an open labelled study involving seven patients.

In 2014, I contacted Professor Lovas, following a publication on a larger number of patients with a more refined regimen. I adapted their regimen, asked my colleagues to comment on it, and subsequently adapted it for our patients.

What were the results?

Our first patient had developed Nelson's syndrome, characterised with high levels of ACTH (adrenocorticotropic hormone), following an adrenalectomy for Cushing's Syndrome. We set the hydrocortisone pump to run at a flat rate to suppress and bring down their ACTH levels.

From then on, we gradually built up a group of patients with adrenal insufficiencies. We have found that the first six weeks are the most crucial in starting on a continuous subcutaneous hydrocortisone pump. This is where patients need to learn all the steps for administering cortisol with their pump. They also learn how to apart the pump so they can deal with critical illness or with just being unwell.

Providing support

During the first six weeks, patients are able to contact me at our Endocrinology services department if there are any issues with the pump.

Although the pump company provides the technical support for the patient, I normally address any issues regarding infusion rates or dose adjustment.  This is done following a discussion with the patient on the symptoms they are experiencing, plus a review of their biochemical markers.

Biochemical markers such as blood tests and urinary cortisol levels are collected during the first few weeks or upon dose changes. Normally within the first 2-6 weeks, patients will know if the pump is suitable for them. Patient education and support is provided on a one to one basis for the first six weeks. In my opinion, this is pivotal in ensuring that patients can manage the pump themselves in the long term.

Patients' progress with the pump

The pump is loaned to the patient for a few months and, if it works for them, they are able to purchase it.

This is such a learning curve - both for me and the patients. Every patient responds at a different pace and it is important to support the patient’s journey.

Learning to listen and respond as needed is crucial, as well as using medical evidence and symptoms management to guide clinical practice.

The results

Surprisingly, despite the intensive efforts during the first six weeks, patients tend to get on very well with the pump after just a few weeks.

Most of the patients who have trialled the pumps have already tried oral hydrocortisone, Plenedran, prednisolone and hydrocortisone injections, but have been unable to gain an improvement in their quality of life.

Those who stay on the pump gain significant control over their adrenal insufficiency and are able to greatly improve their quality of life. They report that they are able to reintegrate back into society, get back to their studies, continue with their careers, and importantly, have a better family life.

What is the current status of pump provision in the UK?

Sadly this service is not available on the NHS in the United Kingdom. Over the years, we at The London Clinic have built up a cohort of over 30 patient using the hydrocortisone infusion pump. This makes us the UK leader in providing this service for people with adrenal insufficiency.

Further information

Addison's disease