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 15 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. It will be expensive for you
New pumps are expensive- AUD$8,000 to $9000. As are the consumerables (the needle and tubing bits) with solu-cortef generally being a less expensive component. To minimise these costs, I’d recommend getting in touch with other cortisol pumpers via Facebook groups and word of mouth who live in your country or nearby. Pumps can be often bought secondhand and supplies purchased at a discount from other pumpers, diabetic support groups or pump suppliers.
Pump suppliers and government health programs sometimes offer discounts or funding. Because cortisol pumping is rare, you will probably need to push and dig around a bit. I turned an initial, “no, we can’t help you” into a discount by pointing out that other patients with adrenal insufficiency who had received discounts from them. I found it worthwhile to talk with a manager and/or to ask other cortisol pumpers who they dealt with successfully at an organisation.
Looking at the costs of pumping from another angle, I figure that if the cortisol pump buys me even a day or two of health when I can actually earn an income for the first time in years, and reduces my other medical costs such as pain management treatments, then it’s close to having paid for itself.
Funding Available in Australia for Cortisol Pumping
In Australia, we are lucky. Private health insurance will pay for the full cost of the pump – 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 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.
Solucortef can be purchased on “an authority script” (ie buy in bulk and save).
2. 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. So 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. They need to be prepared to manage the risks associated with this decision and would need to defend their decision if something went wrong.
I don’t believe that being “off-label” is the real impediment for doctors to prescribing cortisol pumps – it’s their reluctance to carry the risks of pumping. This is where the meaty discussion lies.
3. Most patients with Addison’s Disease do really well on tablets
I’m seriously happy for them but that has nothing to do with me. *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 steroids 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. Their ill-health is instead counted as chronic fatigue syndrome or simply something-else-that’s-not-my-field-of-expertise.
For instance, it took me 4 years and 4 endocrinologists to find one who has accepted that at least some of my symptoms (most of which started or worsened after starting steroid tablets) are related to a failure of the tablets for my body. For those 4 years, my endocrinologists counted me amongst those who do well on steroid tablets but have something else going on. This was despite me being so sick that I could only leave the house briefly once maybe twice a day, and the only thing that relieved my symptoms was changes to my steroids and their dosing.
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:
- 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 due to adrenal insufficiency.
These 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?
If you want to understand the science behind why steroid tablets are inferior to the cortisol pump, read the work of Professor Peter Hindmarsh on using the pump method to treat Addison’s and circadian rhythm dosing. I found his textbook on treating Congential Adrenal Hyperplasia (a form of adrenal insufficiency) extremely informative with many of the chapters being applicable to Addison’s Disease – there’s a whole chapter devoted to pump therapy.
You can also play with the theoretical steroid curve plotter. This tool allows you to input your steroid medication schedule and it outputs your theoretical blood cortisol levels. You can use it to identify gaps in coverage and explore alternative schedules.
4. If the pump fails, you’ll go into adrenal crisis
Risks of Disruption to Steroid Supply
Whenever we use an unnatural method of delivering steroids to the body, there is going to be a risk of disruption to supply and therefore of adrenal crisis.
On steroid tablets, the risks of interrupted steroid supply come from patient error (missing doses, mixing up strengths, not cutting tablets evenly), malabsorption (vomiting, diarrhoea, liver problems, gut mobility problems, other medications interferring with steroid tablets) and pharmaceutical issues (tablets degrading in humidity, there have been widespread reported quality problems with the Qualitest brand of hydrocortisone in the US).
With the pump, the risks of interruption to supply include patient error (forgetting to attach or turn on the pump, incorrectly inserting the cannula), pump error (mechanical malfunction, tubing getting kinked, the cannula bending), and pharmaceutical issues (solucortef degrading in heat).
I have not yet come across studies comparing disrupted steroid supply on tablets vs the cortisol pump. There are some 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.
The Management of Risk of Adrenal Crisis
Regardless of the cause of steroid supply interruption, whether or not it progresses to an adrenal crisis is partly (if not largely) dependent on my (and my partner’s) ability to identify early warning signs and to act shiftly.
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 nightly, shaking, hot, nauseous, flushed, headachey, and dizzy with heightened emotions.
These are the same symptoms that I experience whenever my usual dose of steroids is insufficient for the level of stress that I experience during the day. Examples have included when a tooth extraction took longer and was more painful than expected, and when I travelled to India and didn’t take extra steroids to cover the high humidity.
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 signs continue, I repeat. If the symptoms still escalate, I inject myself with a high dose of steroids and go to hospital. I carry extra tablets, an injection kit and salted water with me at all times in case of these emergencies.
My emergency management plan would be very similar on a cortisol pump. The differences being that I would need to trouble-shoot the pump’s functioning, and be prepared to switch to tablets or use an emergency injection should I not be able to fix the problem and/or my symptoms do not improve.
I believe that the pump is potentially less risky than my foggy, sleep-deprived brain and dodgy gut trying to take 6 different doses of tablets per day. I think the degree of risk is also going to depend on how my body tolerates the cannula – kinks and blockages seem to be a large source of error. I’m not going to know the answer to this until I try it.
Again, looking at the other side of this equation, I believe that the potential benefits of the cortisol pump to my health, well-being and functioning outweigh the risks. However everyone needs to weigh up the risks and benefits of the cortisol pump for their situation.
5. Solu-cortef could interact with the plastic components in the pump
I’ve heard this raised a couple of times without an explanation of why they think that solu-cortef (I believe a pH neutral solution) would erode plastic in a medical device designed for soluble medications.
Furthermore, as far as I can figure out, most if not all of the plastic components of the pump (the tubing, cartridge, and cannula) are changed with brand new ones every 2-3 days.
I do know that Professor Peter Hindmarsh has been placing patients on cortisol pumps for 15 years. As far as I’m aware, there have been no reported cases of pumps degrading quickly or of plastic poisoning (?) because of the solu-cortef.