Living with Chiari

[Blue1998]

I’m 22 and I was diagnosed with congenital chiari malformation a few weeks ago, I’m unsure of what type as I am waiting to see the neurologist for an in-depth talk but from what I’ve been told already it could possibly be type 1

I’ve been taking painkillers & anti sickness to manage the pain but they are slowly not working anymore! I haven’t been to work in over a month now and the only time I get any relief from the pressure in my head & don’t feel nauseous is when I’m lying down

I wondered if there was anyone who has the same condition and how you manage it day to day?

I’m trying not to get worked out about it but I’m un sure if that is because I’m in the dark a little bit about my condition!

[Hidden]

If you’ve developed symptoms in late teens/adulthood, this will almost certainly be a type 1 malformation. Types 2, 3 and 4 are all much more serious, tend to be associated with spina bifida, and symptoms are experienced either from birth or in childhood. The estimate is that 1 in 1000 people actually have a type 1 malformation, but only a minority of those ever develops symptoms, which is why it’s often found as an incidental finding on scans for other things. Whereabouts in the U.K. are you? If you live in England, the best bit of advice I can give you is to request a referral to the Walton Centre in Liverpool rather than get treatment via a local service: it’s the UK’s only neuro specialist hospital, and would be my hospital of choice if being referred now.

I developed symptoms of Chiari at age 26. Initially just the coughing/straining head pain, but it deteriorated quite quickly from there, not helped by the malformation being found on MRI but written off as incidental in spite of the textbook symptoms I had. I only found out about it entirely by chance some 6 months after the scan was done and my mobility was really starting to be effected. Back then (2010), there was a belief that the size of the hernia correlated to severity of symptoms: we now know this isn’t the case, and that people with a small hernia can be far more severely effected than someone with a very large one, but it was problematic to getting taken seriously at the time.

The first thing to say is that it’s a very individual condition. Some people only get relatively minor headaches that never develop further, and a watch and see approach is all that’s required. Some have headaches and more minor symptoms but can manage with painkillers. Some have severe headaches and neck pain, and some of those also go on to develop problems with the nervous system and mobility. For those with severe pain and/or additional symptoms, the only option is generally surgery. I fell into the last group, and the bad news is that I didn’t find anything that helped me at all with pain, or that helped more generally in the interim. Over the course of two years whilst waiting for my first op, I tried everything from prescription codeine and tramadol, through to neuropathic painkillers like amitriptyline and pregabalin with no relief, even when taken in various combinations. It was debilitating, and I was unable to work, in part because I struggled to walk due to the effect the hernia had on my spine and central nervous system. The good news is that, even though they were very dismissive of my symptoms being because of my chiari due to the size of it (even though they were classic for the condition), I had a 95% recovery after my first op. They told me all they thought they could help would be the headaches and neck pain, but I was walking unaided for the first time in 18 months within 3 days of surgery. Prior to then, I’d needed two walking sticks to move. I still had mild neck pain, and I can’t extend my neck back fully without getting pins and needles, so lying on my back for long isn’t a good idea, but I got my life back and returned to full time work in the April having had the op in early Jan.

Unfortunately, I started to relapse after 18 months, and this is one of the reasons I would strongly advise the Walton if you’re based in England. I lived in Wales at the time and couldn’t get the referral to Liverpool purely due to financial politics, but I found out the entire neuro department in Cardiff only did 2 or 3 foramen magnum decompressions a year, maximum. It turns out this is quite common even for big hospitals. When I went back to my surgeon, he said they’d considered doing something additional during the first op but hadn’t: having a second, repeat operation is incredibly rare, but I had to have it done a second time along with the additional bit in 2014. I do wonder if I’d gone somewhere with a lot more experience whether or not that would have happened.

These days, I’m not as good as I was post first op, but I’m a good way off how I was at the height of my symptoms. I can’t ride rollercoasters or take part in contact sports (the price everyone pays for having the op), and I do have some chronic head and neck pain, some of which is due to nerve damage from having the op twice, but my mobility remains unaffected. I can run, swim, do almost everything I could before the chiari ever raised its head. The main sources of information and support for chiari in the U.K. are the brain and spine foundation, and the Ann Conroy Trust. I believe I’m right in saying that the ACT are the only charity dealing specifically with chiari and syringomyelia (a complication that can sometimes occur alongside chiari). It may not be running any more (or any good if it is!), but many moons ago I belonged to a forum that was very helpful in the early days, and I’m pretty sure that if it’s not at the ACT site, I found it via that site. The BSF have a publication specifically on chiari that’s pretty good and can be helpful for explaining to an employer. As an aside, if you have any specific questions about chiari, feel free to message me, but I’m also a qualified HR officer and had responsibility for absence and occupational health at my last role, so if you need any guidance about dealing with work in relation to your health, I’m more than happy to answer those too if it would be helpful.

Here’s the link to the above guide: brainandspine.org.uk/wp-con...

It’s not a fun condition if you’re more significantly effected, but where surgery is required, many people do make full or at least good recoveries. It’s also worth bearing in mind that my story isn’t necessarily the norm, and having been diagnosed, that’s probably the biggest part of the battle. I hope this reply helps a little bit.

[14Blue]

I understand you completely, I am in a similar situation! I got referred straight to a Neurosurgeon after my scans, but I am now waiting on a specialist in Neurology to try and treat medically before re-discussing surgery.

I am starting to manage my pain/nausea more - which painkillers/anti-sickness are you taking?