Don’t Take No for an Answer: Common Cortisol Pump Myths Debunked

I’ve been on the path to getting a cortisol pump for over 3 years…so far. Why has it taken so long? Basically I’ve been hunting for an endocrinologist who’s willing and able to get me pumping. 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. One local endocrinologist told me “oh we don’t do that in Australia”. [This was misinformation. I have communicated with several Australian cortisol pumpers. I agree that it’s a uncommon treatment, but that’s because endocrinologists keep saying “we don’t do that”.]

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 life-sustaining 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 often based on a mixture of misinformation and resistance to change. Their reasoning is rarely full explained to patients making meaningful debate or fully informed decision-making challenging.

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 endocrinologists and information I’ve found to debunk each. 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. I’ve heard of mansplaining but is medsplaining a thing? This comment lead me seriously doubt if this doctor had read the literature on cortisol pumping.

Here’s a brief run-down on the science involved in cortisol-pumping:

Cortisol pumping was developed in London by paediatric 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 patients identify potential deficits in their steroid dosing schedule. Based on the average clearance rates and time taken to peak for different types of steroid tablets, the plotter models blood cortisol levels for different steroid dosing schedules. If you input hydrocortisone tablets taken twice per day, you can see how patients are left with many hours of the day and night with near zero cortisol levels – a situation that can be life-threatening but at the least, makes you feel crappy.

Professor Hindmarsh came up with the ingenious idea of using a medical pump (commonly used to treat diabetes, but actually designed to deliver any liquid medication) to deliver hydrocortisone continuously into the bloodstream for patients with adrenal insufficiency. This is 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).

If being published in a textbook isn’t sufficient legitimacy for cortisol pumping, here’s a list of studies documenting the successful use of the cortisol pump to treat adrenal insufficiency (Note that the cortisol pump works in exactly the same way for any type of adrenal insufficiency because although the underlying causes of AI are different, the treatment is the same – we need to replace cortisol. The idea that cortisol pumping cannot be used to treat secondary AI is misinformation.):

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, it’s their choice whether or not to invest. Personally, 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. Plus what price do you put on potential increased quality of life? If it doesn’t help me to feel better, then I discontinue pumping and have to write off the expenses as “at least I won’t die wondering if I could have had better health”.

New pumps are expensive – AUD$8,000 to $9000. As are the consumables (the needle and tubing bits) with the solu-cortef generally being a less expensive component. However 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 pump funding because it’s so expensive. If one insurer knocks you back, you can switch insurers and as long as the new policy covers insulin pumps, you can apply immediately under the new insurer’s process without any penalty or even needing to tell them.

In Australia, there are also avenues available to receive discounted pump supplies, known as consumables. I recently paid AUD$150 for about 3 month’s supply with a discount of over $1000. This can be negotiated directly with certain pump suppliers.

Solu-cortef (essentially liquid hydrocortisone) can be purchased on “an authority script” which is buy in bulk and save. I’m costing this at the moment.

Then you need needles, syringes, alcohol wipes, saline, etc. I’m working on costing these.

I obtained a free sharps disposal bin from my local council.

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).

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 solu-cortef for Addison’s Disease. Whilst “off-label” sounds dodgy, is it not illegal and in some cases such as the prescription of psychiatric medications for children, off-label use is the norm.

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. Hospitals, insure companies and pump suppliers are all going to be more nervous and raise more objections about cortisol-pumping because it’s off-label.

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 the state 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 steroid 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):

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 respondents 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 be online rather than at work) but it demonstrates that a group of Addisonians are not doing well.

Final Thoughts

Because adrenal insufficiency is a rare condition and many patients do well enough on tablets, cortisol pumping remains uncommon and largely unsupported by healthcare systems. For patients with adrenal insufficiency who do not feel well on steroids, it is worth investigating cortisol pumping. This path is often long, frustrating and lonely. You need to be prepared to become an expert in the topic, to sack doctors who are unwilling to get on board, to network with anyone who knows anything about cortisol pumping, to reach out to strangers and ask for their help, to ask a second or third time, to argue your case, to jump through bureaucratic hoops and to address many objections which will be placed in your path.

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