Cushing’s syndrome and adrenal insufficiency – the gifts that keeps on giving!

If I told you there was a rare endocrine illness that makes you fat, you probably wouldn’t believe me. There is never going to be a good time to have a chronic illness, but even so it could have had the decency to not arrive just as I had everything going so well.

Cushing’s doesn’t just make you massively fat, it destroys your muscles, tendons and ligaments too.  It will leave you with diabetes or at the very least in a pre-diabetic state.  It affects your heart either electrically with arrhythmia’s or structurally.  It kills off brain cells in the Hippocampus region of your brain resulting in short term memory problems and also gives you osteoporosis as well as damaging your skin making it as thin as a ninety year old.  The terrible crippling anxiety, depression and insomnia that is a major symptom of Cushing’s, literally feels as though you’re going mad. The vascular damage it leaves you with gives you a massively higher risk of heart attacks and stroke with these risks staying with you even when the source of hypercortisolism (too much steroid) is diagnosed and stopped. This is what Cushing’s does to you and the list could go on and on a lot further but I’m sure you get it – there isn’t much it doesn’t damage!

Left undiagnosed Cushing’s syndrome will kill you.  It might take many years to do so, but as it’s constantly damaging you in many ways you will eventually succumb. This makes it even more shocking that so many Doctors’ can overlook this illness.

There’s different ways to get Cushing’s.  Either from a pituitary tumour that messes with your hormone production and constantly signals the adrenals to pump out toxic excessive amounts of your natural cortisol. With a pituitary tumour it is then called Cushing’s disease. There is also a rarer form where a tumour develops on your adrenals or a hidden tumour that will cause the same havoc. The other way is from excess steroid from inhalers, tablets etc. Mine was from a steroid inhaler that was far too strong that I was left on unchecked for over ten year.  It’s a very rare illness with around fifteen in a million diagnosed yearly and the majority from taking too much prescribed steroid.

What happened to me – after first becoming ill with various symptoms – took a staggering ten years before diagnosis. By the time I was diagnosed the raging levels of steroid in my bloodstream had shut down my hypothalamus, pituitary and adrenals. They were all completely suppressed. At first they thought by changing to a safer steroid inhaler at a much lower dose, that maybe my HPA axis would work again.  Because it was shut down this meant that I wasn’t producing any of my own natural cortisol which you need to stay alive.  What a crazy situation.  You get ill from too much steroid that shuts down your adrenals and then you need to take steroid tablets to stay alive.  Complete madness.

The danger with living with adrenal insufficiency (not to be confused with adrenal fatigue) is the constant risk of going into adrenal crisis.  Something as simple as a stomach bug can turn into a life threatening emergency and it happens fast.  Replacing your natural supply of cortisol is about finding the correct dose to live on but this dose isn’t enough if you suddenly become ill or break a bone or even an emotional upset.  For a healthy person their adrenals will pump out extra cortisol to meet with everything that life throws at you.  It’s your stress response hormone and with it broken it’s then up to you to match an extra amount to different circumstances.  All good and well if you enjoy life on a rollercoaster! I carry an emergency syringe for injecting cortisol in case of sickness.   But ideally – if we could live in a bubble – you just must not get sick.  If you can’t keep your steroid pills down you’re in deep trouble.  In a matter of minutes or even seconds after being sick you can become very ill so fast that it’s doubtful you would have time to mix up a hydrocortisone shot.  If you’re on your own and too weak or collapsed and can’t administer the shot yourself you’d better hope there is someone around who can!

The plan to start with was that I would take a tiny replacement dose of steroid.  Just enough to keep me alive and to stop me plummeting into adrenal crisis.  The trouble with being put on a small dose with the plan of tapering down, is that you cannot function at all.  You exist.  A shower and getting dressed is a major achievement.  I didn’t get dressed for a year unless I had to go out to see a Doctor. Trying to shower was like climbing a mountain and the majority of the time the exhaustion and debilitating weakness made it impossible.  The pain in your arms and shoulders if you held your arms up trying to wash your hair was unbearable.  Your bones hurt all over with a deep aching from within.  You feel as though every cell in your body is screaming for more steroid and it probably is.

Tapering me off replacement steroid failed.  My HPA axis was not waking up and it was not sending signals to my adrenals to make them produce cortisol. I ended up in the emergency room countless times and in ICU twice with my heart going crazy with arrhythmia’s due to not enough steroid for it to function properly.  Not that the Doctor’s in ER knew at the time.  It is amazing how Doctor’s still don’t realise the effects of too little cortisol. It’s well documented but like any rare illness there is hardly any coverage in medical school.  I don’t think there is anything that cortisol isn’t needed for to function properly.  It controls and regulates so much and replacing – even though we take tiny physiological doses – is a nightmare.  Too much or too little cortisol is equally as damaging to your body.  To get the correct dose is trial and error – mostly error!  I had a year of tapering which wasn’t working.  A year of hell where you’re so ill and weak you can’t leave the settee let alone go to work.

After being ill for so long and then to get diagnosed to then find out you will have to put yourself through hell to try to make everything wake up was just the worse experience ever.  The final straw was waking up one morning with a stomach bug and a very high temperature.  I rushed into the bathroom to throw up and don’t remember much about what happened next.  Luckily my husband was there and had quickly given me the hydrocortisone shot.  Apparently, when heading for adrenal crisis, you have 30-60 minutes to get this injection before organs start to shut down.  Without the emergency shot you would end up in a coma and die.  Pretty scary stuff.

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