I’ve been on the path to getting a cortisol pump for about 2.5 years…so far. Why has it taken so long? Basically I’ve been waiting for an endocrinologist to get on board. You see, they hold the gate keys to accessing funding (I’m talking AUD$9k for a new pump), the medical supplies to run the pump, and the testing and monitoring to ensure that the medication levels are right for my body. Unfortunately only a small proportion of worldwide endocrinologists are willing to do cortisol pumping – see a list here.
Endocrinologists in Australia have been very slow to adopt cortisol pumping even though it was first successfully tried in London about 13 years ago by Professor Peter Hindmarsh. One local endocrinologist told me “oh we don’t do that in Australia”. [This was a bit of a white lie; there are endocrinologists who do this in Australia, it’s just rare].
Why exactly “don’t we do that in Australia”? We are talking about pairing a well established technology (a diabetic insulin pump) with an evidence-based medication (hydrocortisone for Addison’s Disease) that has been consistently shown to improve symptoms and well-being.
From my discussions with several endocrinologists and speaking with other cortisol pump seekers, I’ve found that endocrinologists tend to raise similar concerns about cortisol pumping. These concerns are sometimes based on misinformation and resistance to change, and are rarely full explained to patients.
I’m keen to unpack these issues so that patients can make informed treatment choices and more confidently discuss their preferences with their doctors. Below are 5 objections that I’ve faced from my endocrinologists and what I wish I’d know at the time. For contrast, read here about 5 reasons a cortisol pump user gives for not getting a pump.
1. Don’t get sucked in by what you read online from other patients
In a conversation about cortisol pumping, one endocrinologist explained to me how information on online patient support groups can be wildly misleading – promising miracle cures that don’t exist. This comment lead me to wonder if this endocrinologist had read anything about cortisol pumping. Here’s a brief run-down on the science involved:
Cortisol pumping was developed in London by endocrinologist Professor Peter Hindmarsh approximately 13 years ago. It was based on his theory that many patients with Adrenal Insufficiency feel unwell because steroid tablets are a poor substitute for the natural circadian rhythm of cortisol. Addisonian Amber Nichole has developed a free online tool called the theoretical steroid curve plotter to help other patients identify potential deficits in their steroid dosing schedule. It demonstrates how hydrocortisone tablets taken only twice per day (a commonly prescribed schedule) leaves patients with many hours of the day and night with near zero cortisol levels.
Professor Hindmarsh came up with the ingenious idea of hijacking the insulin pump (usually used to treat diabetes) to deliver hydrocortisone in a more natural way (continuously into the bloodstream) for patients with adrenal insufficiency – known as cortisol pumping. Professor Hindmarsh has recently published his work on cortisol pumping in a textbook on the treatment of Congential Adrenal Hyperplasia (a genetic form of adrenal insufficiency).
The following studies document the successful use of the cortisol pump to treat adrenal insufficiency:
- A Phase 2 Study of Continuous Subcutaneous Hydrocortisone Infusion in Adults With Congenital Adrenal Hyperplasia
- Continuous subcutaneous hydrocortisone infusion in Addison’s disease
- Continuous Hydrocortisone Infusion Bests Oral Therapy for Quality of Life in Addison’s Disease: Presented at ENDO
- Continuous subcutaneous hydrocortisone infusion (CSHI) as replacement therapy in Addison’s disease (AD)
- Continuous Subcutaneous Hydrocortisone Infusion versus Oral Hydrocortisone Replacement for Treatment of Addison’s Disease: A Randomized Clinical Trial
- Management of Altered Hydrocortisone Pharmacokinetics in a Boy with Congenital Adrenal Hyperplasia Using a Continuous Subcutaneous Hydrocortisone Infusion
- Results of the Prolonged Use of Subcutaneous Continuous Infusion of Hydrocortisone in a Man with Congenital Adrenal Hyperplasia
- Successful use of subcutaneous infusion of cortisol in an adult case of congentital adrenal hyperplasia
- What is the best long‐term management strategy for patients with primary adrenal insufficiency?
2. It will be expensive for you
First off, this is a decision for the patient, not the doctor. If the patient is paying, it’s their money, their choice whether or not to invest. Personally, I looked at the costs of pumping and weighed them up against all of the potential benefits. Even from a financial perspective, I figured that if the cortisol pump buys me even a day or two of health when I can earn an income, then it’s close to having paid for itself. If it doesn’t help, I discontinue pumping and have to write off the expenses as “at least I know”.
New pumps are expensive- AUD$8,000 to $9000. As are the consumerables (the needle and tubing bits) with the solu-cortef generally being a less expensive component. But there are options for reducing costs.
Pump suppliers, health insurance and government health programs sometimes offer partial or full funding of the costs depending on your country. Because cortisol pumping is rare, you may need to push and dig around a bit. You may need to go back multiple times and ask in different ways. I found it worthwhile to ask other cortisol pumpers what deal they’d gotten and whom they had communicated with at an organisation. It’s easier to say “I want she’s having” than negotiating cold.
Failing receiving funding, pumps can be purchased relatively cheaply second-hand from other users – both with adrenal insufficiency and diabetes. Some pumpers report buying pumps from diabetes associations, pumps that have sometimes been only briefly used. Obviously a second-hand pump will not come with a warranty and may have a greater risk of failure. I’ve also read of people buying discounted or free consumerables from other pumpers who no longer needed them.
Funding Available in Australia for Cortisol Pumping
In Australia, we are lucky. Private health insurance has been known to pay for the full cost of pumps for people with Adrenal Insufficiency – many endocrinologists seem unaware of this. You do need to have coverage under your policy for insulin pumps or else you’ll have to upgrade/switch and wait 12 months to get coverage.
Even when you have coverage under your policy, you’ll need to submit an application for the pump because it’s so expensive. This process varies between insurers. Medibank and Bupa apparently have a fairly straightforward process that’s no different to that of a diabetic. The smaller insurers seem to require a more detailed argument from your endocrinologist and approval from their own medical specialist.
If one insurer knocks you back, apparently you can switch insurers immediately. As long as the new policy covers insulin pumps, you can apply under the new insurer’s process without any penalty.
In Australia, there are also avenues available to receive discounted pump supplies (down from AUD$150-200 per month to $15-$20 per month). This can be negotiated with some pump suppliers. Alternatively it may be possible to obtain a discount via the National Diabetes Services Scheme who do fund diabetes supplies for non-diabetic conditions. Again, there is a special application process.
Solu-cortef can be purchased on “an authority script” (ie buy in bulk and save).
3. It’s an off-label use of an insulin pump
Cortisol pumping is what’s called an “off-label use” in Australia (with equivalence in other countries) but this is not necessarily an impediment.
Off-label prescribing means that the Therapeutic Goods Administration (TGA) has not approved the indication, route of administration or patient group. It does not mean that the TGA has rejected the indication. Commonly the TGA has not been asked to evaluate the indication. – Australian Prescriber
This means that the Therapeutic Goods Administration has not approved (but it hadn’t prohibited) the use of insulin pumps to deliver solucortef for Addison’s Disease. Whilst “off-label” sounds dodgy, there is nothing illegal or even unusual about the off-label use of medical devices.
Off-label use does not necessarily mean there is no evidence for the safety and efficacy of the application. Instead, it usually means that the company selling the medical device cannot justify the cost of applying to the Therapeutic Goods Administration for that particular application or patient group. In the case of cortisol pumps, Addison’s Disease is a rare condition so it’s not worth pump companies investing in getting it to market.
The decision to prescribe a cortisol pump is ultimately that of your doctor. Because it’s an off-label use, they are taking on an increased responsibility to manage the risks associated with this decision and would need to defend their decision if something went wrong.
4. If the pump fails, you’ll go into an adrenal crisis
I believe that this is the meat in the resistance sandwich of endocrinologists – the fear that pump failure will lead to patient deaths.
What causes pumps to fail?
I have not found studies examining pump failure with solu-cortef. However there are studies available about pump malfunction in patients with diabetes. One study found that the rate of pump malfunction over a 5 year period was 68%. This comprised 12% complete failures, 7% alarms, 35% mechanical defects, and 44% minor defects.
Another found that 84% of pump users experienced at least one adverse event over a year – comprising set/site problems (53%), cutaneous (skin) complications (43%) and pump malfunction (38%). Adverse events requiring hospitalisation in 9.8% of cases. I can’t find information on this risk for the cortisol pump – I’m curious to know how adrenal and diabetic crises compare in terms of ease of detection and length of time to act.
So, yes, pumps fail but they fail for diabetics too and some end up in hospital because of it. Yet doctors keep on prescribing pumps for diabetics. Why is adrenal insufficiency any different?
How can we manage cortisol pump failure?
I think that a large part of the problem here is a disconnect between endocrinologist’s knowledge and patient practice. In my experience, many endocrinologist seem to view cortisol levels as being either normal (the patient is well) or critically low (the patient is in adrenal crisis, dying). There is little recognition by most endocrinologists of low cortisol (the patient is experiencing warning signs of impending crisis), a set of symptoms that occur hours or days before a full blown crisis.
I experience clear symptoms of low cortisol well before a full blown adrenal crisis hits. On the standard treatment for Addison’s Disease (hydrocortisone tablets taken twice a day), my body was expected to be without steriod coverage for approximately 10 hours every single night for 6 months (I can’t understand how most endocrinologists see this is an acceptable risk but the cortisol pump is not). As a result, I woke at almost precisely 3am each night shaking, hot, nauseous, flushed, headachey, and dizzy with heightened emotions. This is low cortisol for me.
All Addisonians will have experienced low cortisol symptoms and most manage these by taking extra steroids with or without their doctor’s approval because we are quite keen to stay alive.
When my warning signs occur, I take an extra dose of tablets (patients call this “bump dosing”) and consume extra salt and fluids. If the warning signs continue, I repeat. If the symptoms escalate, I inject myself with solu-cortef using the kit I carry everywhere and I go to hospital.
Most Addisonians will already have a similar risk management plan for reducing the risk of crisis whilst on steroid tablets. This plan needs to be adjusted for the cortisol pump.
My emergency management plan on a cortisol pump would have the additional warning signs of alarms in the pump to tell me something is wrong. It would have several new management options in addition to taking more tablets, injecting or going to hospital – I could try taking a bolus dose with the pump, I could trouble-shoot and fix the pump, and/or I could switch to a back-up pump if I have one.
I believe that patients and doctors need to have an in-depth discussion of what strategies can be used to manage the risk of adrenal crisis on a cortisol pump. I think that many doctors will be surprised to learn of how diligently their patients are already managing their risk of adrenal crisis (particularly given these patients are likely doing poorly on steroid tablets and living with low cortisol symptoms daily).
5. Most patients with Addison’s Disease do really well on tablets
If fear of adrenal crisis is the meat, then this must be the bread in the cortisol pumping resistance sandwich: If everyone with Addison’s Disease is doing great on steroid tablets, why would an endocrinologist go to the hassle and take on the addition risks associated with cortisol pumping?
*Perhaps* most patients with Addison’s do well on tablets but there is also a group of us who do very poorly on tablets. Why not try to help us feel better especially when there is a treatment available that can be life-changing?
From what I’ve learnt on patient support groups, the really-poor-doers are at a high risk of death from adrenal crisis (many are constantly in and out of hospital) and suicide (this occurs far too frequently for my comfort levels).
Every single one of us is disabled Many are unable to leave their houses or beds. To say that our “well-being has been effected” is a vast understatement and a poor descriptor of the experience of chronic, unpredictable and wide-ranging ill-health.
I also believe that the prevalence of really-poor-doers is higher than estimated by doctors. This is partly because they are too sick to see a doctor or have given up entirely on the medical system, and are hidden from the headcount.
It’s partly because doctors rigidly hold the belief that steroid tablets are the perfect treatment for Addison’s Disease. They refuse to accept any evidence to the contrary – that the ill health of these patients is the result of a failure of steroid tablets to provide adequate cortisol coverage. Their ill-health is instead counted as chronic fatigue syndrome or simply something-else-that’s-not-my-field-of-expertise.
Don’t just take my word for it though. These studies provide evidence of impaired health and well-being in patients with adrenal insufficiency treated with steroid tablets (print them out and give them to doubting doctors):
- Impaired subjective health status in chronic adrenal insufficiency: impact of different glucocorticoid replacement regimens
- Impaired Health-related Quality of Life in Addison’s disease – Impact of Replacement Therapy, Co-morbidities and Socioeconomic Factors
- Poor quality of life, depressed mood, and memory impairment may be mediated by sleep disruption in patients with Addison’s disease
- Impaired quality of life in patients with adrenal insufficiency – evidence that improved glucocorticoid replacement strategies are needed
- Subjective health status in Norwegian patients with Addison’s disease
Adrenal Diseases Support Group (a Facebook group with 5000 members) conducted a survey which was completed by 200 of its members with adrenal insufficiency. Only 20% of respondants reported that their adrenal insufficiency was well managed and they functioned normally. Whilst 36% worked full-time, 25% reported being unable to work at all due to adrenal insufficiency. This is a biased sample of participants (mostly those who are unwell enough to have the time to br online rather than at work) but it demonstrates that a group of Addisonians are not doing well.