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 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 by Michelle, Winslow Dixson’s blog and Clearly Alive by Amber).

He seemed unaware that paediatric endocrinologist Professor Peter Hindmarsh started cortisol pumping in London approximately 13 years ago. This is not a new treatment and it was not started by patients!

This medically trained professor has the theory that some patients with Adrenal Insufficiency (AI) feel unwell on conventional treatment because steroid tablets are a poor substitute for the natural circadian rhythm of cortisol. (Clearly Alive has developed a free online tool called the theoretical steroid curve plotter that you can use to see how different steroid 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.

So yes, there is a clear scientific rationale behind cortisol pumping which has been pubished 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).

There is also a steadily growing list of research 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

If you are not well on the standard treatment for Adrenal Insufficiency, know that you are not alone, there is a reason why the standard treatment doesn’t work for everyone and there are treatment alternatives available. I hope that the information above makes it a little easier for you to consider and access cortisol pumping.

Advertisements

6 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

  2. Very well written Charlie. I am also Australian and at the time I wrote below, there were not many doctors putting people onto the pump, but I can see it is slowly changing. Here are my writings on the pump and what I like to call the “Myth Busters’ at the time.

    Continuous Subcutaneous Hydrocortisone Infusion – a patient experience.

    The below information is purely my personal experience and thoughts, as a patient, relating to using Continuous Subcutaneous Hydrocortisone Infusion, for treatment of Adrenal Insufficiency, using an insulin pump. (It is not Medically researched information)

    The insulin pump was originally designed for diabetics, who are able to receive government and insurance rebates. Many arguments for using the insulin pump for off label use in my opinion fall short of good argument against use of the pump for hydrocortisone use. Whether an AI patient uses oral medication, subcutaneous injection or pump therapy, they have to remain just as vigilant to their symptoms for any type of delivery and understand the need for stress dosing.

    As a patient who is both an insulin dependent diabetic and an AI patient, I have first used the pump for delivery of insulin. Therefore I feel I can compare my experiences both from the diabetic and AI point of view. I opted to trial the pump for hydrocortisone delivery as cortisol day curve tests revealed I was a fast metaboliser. My experience resulted in many of my AI symptoms disappearing, eg diarrhoea, tachycardia, morning nausea, and debilitating fatigue, resulting in a better quality of life with more energy enabling me to achieve more than previously. I would say I now feel 80% recovered as opposed to functioning at 30% previously. I have continued to use the pump for hydrocortisone and went back to injecting insulin for treatment of my diabetes.

    PROS
    • Continuous therapy
    • Able to sleep and not worry about missing dose
    • Able to drop to lower doses because body is using medication better
    • The technology allows for setting updosing at higher amounts by the percentage method, and likewise makes weaning easier to work out using the percentage method.
    • While weaning, the dosing can be set to very small amounts if needed. Eg. 1mg, which is difficult to do using oral medication.
    • Convenience
    • Being able to bolus and receive medication more quickly than updosing with tablets
    • If nauseous or having diarrhoea, guarantee of receiving medication is ensured.
    • The pump can more closely match the circadian rhythm than any other current form of treatment
    • Patients are able to receive very small doses overnight while sleeping
    Many patients who do not do well on oral medication, report doing much better on the pump.

    CONS
    • More expensive treatment when compared with medication.
    • Adherence to sterile procedures when changing tubing, cannulas, and reservoirs.
    • Acceptance of wearing an attached device and learning how to use it to ensure proper use.
    • Irritated sites
    • Bent cannula
    • Unable to measure cortisol in the same way a diabetic can measure their blood sugars if feeling unwell.
    • User Error
    • Malfunction
    • Claims made that using the pump is a constant reminder of the illness

    DE-BUNKING CONS
    • More expensive treatment when compared with medication – more expensive also for a diabetic, yet they get rebates.
    • Adherence to sterile procedures when changing tubing, cannulas, and reservoirs. – A diabetic has to adhere to the same sterile procedures.
    • Irritated sites – diabetics get irritation from the sites also.
    • Bent cannula – this would result in non-delivery of medication and the patient would start to feel low cortisol symptoms. The same can happen if a patient forgets to take their medication or sleeps through a dose. The patient would need to be aware of symptoms in both situations and updose accordingly. A bent cannula can also happen with insulin delivered therapy.
    • Unable to measure cortisol in the same way a diabetic can measure their blood sugars if feeling unwell – An AI patient who uses the pump has to be just as vigilant to recognise low cortisol symptoms in the same way as they would have to if they were taking oral medication. With both AI and diabetes, the patient needs to treat low cortisol/diabetic symptoms by adjusting their dosing accordingly.
    • User Error – diabetics can make mistakes with the pump also and yet the pump is used and accepted re insurance/rebate for this condition. Also, it is just as easy to forget to take a tablet as it is to forget to press the correct button on the pump.
    • Malfunction – diabetic use of pump can also result in malfunction in the same way.
    • Claims made that using the pump is a constant reminder of the illness – this is a ridiculous notion, given that a person who has absorption or fast metabolising issues have to dose more frequently with alarms set on their mobile phones. This is just as much a constant reminder of the illness as is the pump. A diabetic patient has a choice between injecting up to several times a day or using the pump. It is in no way any different for an AI patient than it is for a diabetic patient to be reminded of their illness.

    Like

    1. Hi Des,
      This is fantastic, detailed, well-written information. Thank you so much for sharing it here so that others can read more on the issues involved. Have you published this anywhere? Is there anything that I can do to promote it for you?
      Thinking out loud, I’m wondering if you might be interested in being interviewed for Chronically Crappy. I think it could be really helpful for prospective pumpers (and endos) to hear from a range of different pumpers about their experiences.
      What do you think?

      Like

Leave a Reply to Author Winslow E. Dixon Cancel reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s