Perspectives

Perspectives

Postby KitKat » Sat Oct 20, 2018 9:03 am

Hi everyone,

I am a comparatively new member of the Pituitary Foundation although I was diagnosed with adrenal insufficiency 20 years ago, November 1998.

Now that I've effectively taken early retirement I'm starting a PhD looking at patient education programmes and outcomes for people with long-term health conditions. I am thrilled that my co-supervisor will be one of Europe's leading experts in the field of steroid education, Prof Stefanie Hahner in Würzburg. I had the privilege of observing a 2-hour group education session (in German!) earlier this week and it was really exciting to see how they do things in her clinic. I learned some new things about injection method, and got to meet several people very newly diagnosed. Two in-patients still with a cannula in their arm, which was a reminder of just how vulnerable most people feel when first diagnosed.

Even though I was diagnosed 20 years ago, I wanted to say that I am still learning how to juggle this condition, and that I still benefit from hearing about other people's experiences. If only as a reminder of how easy it is to trip up and underestimate when you need take take extra hydrocortisone for physically or emotionally draining events!

Such as massive train delays across Germany, for example, which totally wiped me out on Friday, even though I had been doing 2.5mg top-ups through Thursday evening while Deutsche Bahn rescheduled and then cancelled my connecting train for the Eurostar. Six of us ended up on a 3-hour minibus ride to Brussels, paid for by the railway, and I arrived after midnight at the serviced apartment my husband had booked for me online that evening, when we missed the last Eurostar of the day. How did we manage before mobile phones? I came right with an extra dose and some extra salt, but lost most of Friday morning and had very shaky hands for a while.

Best wishes to all,
KitKat
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Re: Perspectives

Postby Mgh » Thu Oct 25, 2018 9:06 pm

"Even though I was diagnosed 20 years ago, I wanted to say that I am still learning how to juggle this condition, and that I still benefit from hearing about other people's experiences. If only as a reminder of how easy it is to trip up and underestimate when you need take take extra hydrocortisone for physically or emotionally draining events!"

KitKat
I agree wholeheartedly with your statement. What I am noticing more and more is that I can rise to the occasion, sometimes needing a little extra hydrocortisone to cope but not always. However, I often go downhill badly afterwards. Lots of questions come to mind about this but very few answers.
Very interested to hear about the PhD topic you are researching. Would love to hear more about your German clinic experience if you are ok to share it.
Mgh
 
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Re: Perspectives

Postby member_45020 » Wed Nov 14, 2018 10:58 am

Hello Kitkat and Mgh. I have just recently joined, as parent of a teenager with primary adrenal insufficiency.
Noting your 20 years experience with this would be interested to hear learn about the new things you have discovered about the injection meth.d

Best wishes.
Debbie
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Re: Perspectives

Postby Mgh » Thu Nov 15, 2018 11:17 am

Debbie, are you referring to the pump method of administering daily hydrocortisone? I think it is a potentially great way forward. The main advantages for me are that it bypasses the digestive system and it allows administration of hydrocortisone more closely to the circadian rhythm. However, I don't know anything about the logistics of using a pump and how available it is outside of research. Hope others may have more information that they can share with us. Maria
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Re: Perspectives

Postby member_41389 » Thu Nov 15, 2018 9:46 pm

Hi
The following is a bit lies and rumours based but I think the essence is true.
Prof Hindmarsh at GOSH appears to be able to supply pumps for children since their dosing is critical to their development in a remarkable number of ways.
There seem to be a few endocrine departments who will provide them but they are expensive to run. They are creeping into use for insulin delivery where normal routines don't work for some; the numbers are small currently but they are growing. A pump is available privately, cost starts at about £5000 plus diagnostic testing, fitting and training costs followed by ongoing costs for the cannulas and supplies plus annual maintenance etc as is normal in private medicine.
I hope this makes some sense.
Tim
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Re: Perspectives

Postby KitKat » Thu Dec 13, 2018 1:09 pm

Hi Maria and Tim,

Soap box moment: it's important to remember that there are no one-size-fits-all solutions in adrenal replacement and this applies over any one person's lifetime just as much as to the variation in what will work best from one individual to the next. If your current regime feels like it isn't working so well for you as well as it used to, it is okay to raise this in clinic and ask for some labs to help understand why. What is the right adrenal regime for anyone is likely to shift over their lifetime as their residual adrenal capacity, metabolism and oral absorption alter, or as other long-term conditions develop. In general, younger people need more glucocorticoid for replacement than older adults.

There is some brilliant – if dauntingly techie – stuff about glucocorticoids and their diverse, adaptable functions in the new issue of The Endocrinologist. It's a free read on the SfE website, here: https://www.endocrinology.org/endocrino ... ng-stress/ I just found one scientist summing up my thoughts, above, more succinctly than I could:
"glucocorticoids do not play the same role across the life course, but rather serve different functions. This starts with their role in ‘wakening’ of the amygdala in neonatal life to their role in promoting ponderal growth in adolescence and their influence on the vulnerability of the adolescent brain to stressful experiences as well as the age-accelerating role of excess cortisol". https://www.endocrinology.org/endocrino ... e-know-it/

As Tim says, teens and young adults are, speculatively, perhaps more likely to benefit from continuous infusion pump therapy than adults on the clinical parameters, because of their super fast metabolism and the need to ensure steroid therapy doesn't suppress overnight pituitary production of growth hormone. There was also one small-scale UK clinical trial found a real improvement in stability for adults with complex psycho-social needs and a 'frequent flyer' history of multiple A & E admissions, so that there was a net cost saving to the NHS in their overall care, with fewer inpatient stays.

Pump therapy is not for the faint-hearted: you are attached to a machine 24 hours a day. The current models available in the UK are reportedly quite noisy and some people find the night-time whirring and clicking disturbs their sleep, as much as the practicalities of avoiding dislodging the tubing when you turn over in bed.The tubes easily get blocked, because liquid hydrocortisone is either viscous (with allergenic fillers as such formaldehyde) or else hydrophobic (with a tendency to sediment out again). Some people develop a contact allergy to either the fillers or the active ingredient (something which deeper, intramuscular injection seems to avoid). Some people get recurrent skin infections at the injection site because of the concentrated steroid exposure. So there are both safety risks of decompensation and potentially uncomfortable/risky side effects. Note, insulin doesn't gunk up the tubing in the same way, I gather, and doesn't present the same problems with contact dermatitis or infection. That's why there isn't an infusion pump on the market licensed for glucocorticoid use, and getting a maintenance contract for its use 'off licence' to deliver hydrocortisone can be tricky. Speculative thought: if infusion pump therapy were to be adopted more widely, it would be helpful if a pharma company could be persuaded to develop a more dilute form of liquid hydrocortisone that didn't cause all the problems outlined above. However, the limited market size and almost zero predictable profit says this is only likely to be done through a publicly-funded institution.

Bottom line: using a pump for steroid replacement is high maintenance and a lot more faff than oral tablets. It also seems to require some fairly intensive one-to-one coaching and blood monitoring at first to get it optimally configured on dose and timing and ensure individuals are using the bolus function prudently. (It's easy to get heavy handed and I gather, anecdotally, that one or two people trialing it found themselves becoming Cushingoid). Several overseas clinical trials found only limited improvement in clinical parameters or reported quality of life. (Nerdy factette: the biggest pump study, in Scandinavia, also found that it required a slightly higher total daily dose than oral tablets because gastric absorption is more efficient that dermal).

You could argue that these ho-hum outcomes were because the researchers weren't skilled enough to fully optimise the regime so it was tailored to each individual patient. Fair enough, but if several research teams with an interest in adrenal medicine can't match the impressive results achieved by a single clinician with deep expertise in this field, what does this tell us about the practicalities for NHS? Making pump therapy widely available on the NHS would seem to demand a big step-up in endocrine clinical training, on top of a big step-up in implementation costs for equipment and ongoing labs, before it would be viable in the way that Freestyle Libre technology is now being rolled out.

Better news for adults is that advances in the potential for oral therapy to provide that valuable early morning cortisol rise are on the horizon. Chronocort is now available on a named patient basis in the UK for people with CAH, and you can find mention of its wider use for adrenal replacement in some of the north American forums. Within the UK, it should become available for adrenal replacement more generally once it has its full EMA licence. For an update on trial results, https://www.diurnal.co.uk/non-healthcar ... cal-trial/ Good things come to those who wait – we hope.
There are also low-dose enteric coated prednisolone and prednisone tablets which offer a less sophisticated early morning boost when taken close to bed time. Both reportedly have a tendency to accelerate bone loss and metabolic syndrome in those who take high doses, but the jury is out on whether doses <5 mg would have the same side effects.

Okay, I hope I've managed to convey that I think pump therapy has a useful role to play in supporting people with complex/destabilising co-conditions, poor oral absorption, or other factors which mean they do badly on oral therapy. But it is a major commitment, and people contemplating it need to be aware of the potential safety risks and side effects. And I can fully understand why those clinicians who have had success with pump therapy are keen to talk about their achievements, because they have overcome considerable obstacles to do so.

Best
KitKat
Last edited by KitKat on Sat Dec 15, 2018 9:28 am, edited 14 times in total.
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Re: Perspectives

Postby KitKat » Thu Dec 13, 2018 1:37 pm

Hello Debbie,

Bear in mind that in Germany they cannot get the 1ml glass ampoules of liquid hydrocortisone. So this is about powder and water. And the German method for preparing the Solu-Cortef powder and water as an emergency injection was totally different to anything I have seen in the UK.

They have the integrated Act-o-Vial, so it would need tweaking as a method before it could be done here. But the a-ha moment was in using a really long filter needle to draw up the solution without having to turn the fricking bottle upside down with just a tip of the needle through the stopper to get it all up. (Having had to do that once at 1am by a dim bedside light, never again).

This is going to be hard to envisage without a demonstration, but here goes. You need a dirty great filter needle longer than the standard green one we get in the UK. Pull the filter needle back out of the stopper once you have squeezed the water into the powder bottle, then adjust the plunger so it is drawn back about 2ml into the syringe. Poke it through the stopper again and make sure the needle tip is sitting on the base of the bottle, positioned to draw up all the solution. Then push the plunger down to expel the air in the syringe into the bottle. This will create a vacuum, and it will spring back up, this time full of your lovely hydrocortisone solution! Leave the filter needle sheathed in the bottle and carefully remove just the syringe. Have your blue IM needle with its wrapper peeled, back, ready to connect up to the syringe, and you're all set to do the self-stabbing bit.

That's it. It sounds more fiddly, having an outsized filter needle that couldn't be used to give the injection at a pinch and changing needles, but is actually much simpler to do than attempting to use the injection needle to draw up the solution from the Solu-Cortef bottle. And switching needles is so much simpler when you leave the filter needle sitting in the bottle and are only handling the syringe itself while you attach the injection needle. Added bonus, the injection needle is just that bit fresher and less blunted, because it hasn't had to cut through the rubber stopper before puncturing your skin.

Best
KiKat
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