Don’t Take No for an Answer: Cortisol Pump Misinformation Busted

I’ve been on the path to getting a cortisol pump for over 3 years…so far. Why has it taken so long? Unfortunately only a small number of endocrinologists worldwide support cortisol pumping. I’ve seen 5 endocrinologists and travelled interstate in search of treatment. [If you join the Facebook group Adrenal Diseases Support, you can see their list of pump-friendly endos in countries other than US here or in the US here.]

Unfortunately there’s also lots of misinformation being provided to patients. One endocrinologist told me “oh we don’t do that in Australia”. This was inaccurate – I’d spoken with multiple cortisol pumpers in Australia. Plus, update: as of March 2019, I am up and pumping! I can introduce you to my new pump here and I can tell you exactly how I got one here.

Below I address the top 5 objections that I’ve heard about cortisol pumping. I wish to inject more evidence into this discussion so that patients can make an informed choice about the treatment and are better equipped to advocate about pumping should they choose to go ahead. And hey, if any doctors are reading this – welcome aboard!

1. There’s no evidence for cortisol pumping

I had one endo warn me not to get “sucked in” by what I read online about cortisol pumping in patient support groups. He seemed to be of the belief that cortisol pumping was only being driven by a group of patients offering false-hope of a miracle cure without any scientific basis. He thought that cortisol pumping was akin to dust-diets, moon beam-realigning and pixie-sticks.

This doctor clearly had not done his homework on cortisol pumping! He’d not read blogs by highly intelligent and rational women who honestly portray the hardwork it takes to gain modest improvements in health from pumping (see Cortisol Musings and Clearly Alive).

He’d not read the history, theory or science behind this treatment. Here’s a run down:

Paediatric endocrinologist Professor Peter Hindmarsh started cortisol pumping in London approximately 13 years ago. No, this is not a new treatment and it was not started by patients!

It was based on his theory that many patients with Adrenal Insufficiency (AI) feel unwell because steroid tablets are a poor substitute for the natural circadian rhythm of cortisol (you can check out a free online tool called the theoretical steroid curve plotter to see how different medication schedules last throughout the day).

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 in a manner that more closely mimics the work of the adrenal glands than tablets. This is known as cortisol pumping.

Professor Hindmarsh has published his work on cortisol pumping in a textbook on the treatment of Congential Adrenal Hyperplasia, a genetic form of AI (don’t be put off by the name of this book: at least half the chapters, including those on pumping are relevant to all forms of AI). Here’s a list of studies documenting the successful use of the cortisol pump to treat AI:

2. It will be very expensive for you

What I needed from my doctor was detailed information about the costs involved in this treatment so that I could make an informed choice about whether to go ahead. Instead I was simply told that there was no funding available from their hospital and it would be “expensive” for me.

If the intention of this unhelpfully vague statement was to put me off pumping, it had the opposite effect – I am a passionate thrift-shopper afterall. I relished the challenge of finding a good deal. Update: I have written a post detailing my costs of cortisol pumping in Australia. I received full funding from private health insurance for the cost of my pump, and discounted consumables from the pump supplier.

Pumps

Whilst new pumps are expensive – AUD$8,000 to $9000 – some patients can claim the cost under their health insurance, others have received free pumps by participating in research trials, and others get a pump for their diabetes and then decide to use it to treat their AI.

Pumps can also be purchased for a few hundred dollars second-hand through eBay/CraigsList, Facebook groups or diabetes support groups.

A US organisation, CR3 Diabetes sells refurbished secondhand pumps with 2 year warranties. Apparently they are supportive of people with AI buying their pumps.

Consumables

Consumables (the needle, tubing and reservoir bits) can sometimes be purchased with a discount through pump suppliers or bought from other pumpers. CR3 Diabetes sells out of date consumables cheaply.

3. It’s an off-label use of an insulin pump

Yep, this one is true – cortisol pumping is what’s called an “off-label use” in Australia. However, to be told “ooo it’s off-label. Sorry. Full stop. End of Conversation.” is misleading and is not giving the full story.

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

While “off-label” sounds dodgy (particularly when a doctor doesn’t explain the term), is it not illegal. 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 either. 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 with a small market. It’s not worth pump companies investing time or money in the research or applications required to gain official approval. Can you see now why despite being a sound treatment cortisol pumping has been so slow to take off? It’s all about the money, money, money.

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. In my experience, if a doctor is very hesitant or stalling in regards to pumping, it can be quicker to find another doctor who is willing to take on the risk of pumping.

4. If the pump fails, you’ll go into an adrenal crisis

Maybe, but not necessarily. This is an issue of risk management.

What causes pumps to fail?

I have not found studies examining pump failure rates 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%). Hospitalisation was required in 9.8% of these adverse events. 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. Yet doctors prescribe pumps for diabetics. Why is adrenal insufficiency any different? 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.

How can we manage cortisol pump failure?

I experience clear symptoms of low cortisol well before a full blown adrenal crisis hits. 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 have a similar risk management plan for reducing the risk of crisis whilst on steroid tablets.

Update: When I started on a cortisol pump, we slightly adjusted my emergency plan to add the additional step of being able to take a bolus dose with the pump and to be on the look out for an error alarm on the pump. Should I be in any doubt about the functioning of the pump, I switch back to steroid tablets which I carry everywhere and contact the pump supplier. I feel safer on a cortisol pump than I ever did on tablets because my health is more stable and I have more options during an emergency situation.

5. Most patients with Addison’s Disease do really well on tablets

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. Our 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 does exist who 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 steroid tablets, 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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3 thoughts on “Don’t Take No for an Answer: Cortisol Pump Misinformation Busted

  1. Why am I just now finding this post?!! So similar to my story! Oh, my friend we need your skills with the foundation. Your post here is incredible. Kudos to your fight! 🙌 We won!!! And we’re helping other people win too! One day this treatment will be available to ALL adrenal insufficiency patients!

    Like

    1. I thought the same things when reading your blog (and the dragon is really awesome). Yes, it’s becoming clear that most endos are singing the same outdated tunes across the world. “Baby You’ll be Better Now”, “the Drugs do Work” and “Don’t Pump up the Jam” spring to mind 😂😂😂.

      Plus it’s clear to me now that health systems worldwide are founded on the same premise – that the only safe and effective treatments are those that the pharma companies deem economically viable. Treatments with a small customer base like cortisol pumping remain off label and difficult to access.

      Keen to work together to pump out new beats and help others hack-out a pathways to better treatment. Wishing you many spoons and looking forward to connecting Xxx

      Like

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