Hi, my name is Splodge52!

Splodge52

Hi guys .
Im Donna and I have had Chari Malformation for 12 years.

chiarieds

Hi @Splodge52 - & welcome to the community. Myself & family are affected by Chiari 1 Malformation associated with Ehlers-Danlos Syndrome. One of the best (or probably the best) places for information on Chiari 1 Malformation is the American Syringomyelia & Chiari Alliance Project's website. Please see: https://asap.org/

They also have videos of past year's conferences. Here's the one from 2020 which I 'virtually' attended: https://asap.org/conferences/virtual-conference/2020-conference-videos/

Previous year's conferences here: https://asap.org/conferences/past-conference/

These are all for patients, & I have found them so informative.

So how are you?

chiarieds

Hi @Libby_Scope - Chiari 1 Malformation is a disorder in which the normally developed brain is compressed by the lack of space at the back of the skull often resulting in a downward displacement of the lower portion of the cerebellum, i.e. herniation of the cerebellar tonsils into the spinal canal.

In Ehlers-Danlos Syndrome, particularly the hypermobile type, hypermobility at the joints at the base of the skull can result in the lower part of the brain slumping down, resulting also in the descent of the cerebellar tonsils.

Splodge52

Thank you for the welcome.
I would happy to share information about my condition as I also had a syrinx with my chairi and I now suffer from extreme nerve pain 

chiarieds

Hi @Libby_Scope - We haven't pursued a diagnosis of PoTS, but both my son & I definitely have orthostatic hypotension. I've also wondered about MCAS in myself. If you haven't seen it, this video may be of interest. Dr Maitland is great, altho she tends to speak rather fast! Please see: https://www.youtube.com/watch?v=6LBRu0NnEXE
You will see there are other videos by her there, which are well worth watching. She presents for the American Syringomyelia & Chiari Alliance Project's conferences too. See reference above.

chiarieds

Hi @Splodge52 - that's my problem (tho I don't have a syrinx/syringomyelia), the neuropathic pain. Have you had any surgery?

Splodge52

Yes I had foreman Magnum decompression and duraplasty in January 2012. I also had further surgery in November 2012 due to having a leak around the patch which was leaking CSF into the back of my neck but this was still communicating with my brain, so it made me feel very sick whenever i moved my head.

chiarieds

I'm sorry for what you've experienced @Splodge52 - there have been so many advances in the understanding of Chiari 1 Malformation in the past 20 years, & resulting modifications in surgery. With having Ehlers-Danlos Syndrome (EDS), different techniques have evolved when this complicates matters (Complex Chiari). A New York neurosurgeon I know has developed a 'minimally invasive technique' which would potentially benefit not only those with EDS. If you look at the videos of past conferences I mention above you will see some of Dr. Bolognese's.

How are you now? I do hope things have improved for you.

Splodge52

I was good for a few years and even returned to work. But about 4 years ago I started suffering from Peripheral Neuropathy and is affecting my daily life, the brain fog has returned and so has some of the numbness in both my arms and legs .... I feel like I am a walking chemist and if your were to shake me I would probably rattle ..

chiarieds

I'm sorry to read that @Splodge52 - absolutely awful that you now also have Peripheral Neuropathy on top of everything else. You mention that both of your neurological problems are affecting your daily life. I hope you don't mind me asking, but have you considered looking into the Personal Independence Payment (PIP), as this is a benefit to help when certain activities of daily life &/ mobility are affected? Some info here: https://www.cambridgeshire.gov.uk/asset-library/Personal-Independence-Payment-Descriptors-and-Scores-April-2021.pdf

Splodge52

thank you @chiarieds, believe it not my partner has just helped me complete the PIP form, so now it is just a case of wait and see. x

chiarieds

That's good @Splodge52 - may I ask & have you sent your PIP form off?

Splodge52

@chiarieds   Yes I have x

chiarieds

Hoping all goes well @Splodge52 - it does now, due to the pandemic & subsequent backlogs, take on average 10 months from claiming to getting a decision letter (after you get an assessment).

If you haven't given a couple of detailed examples as to the difficulty you face doing/ attempting to do any applicable activity then it may be worth sending these off now (putting your name & National Insurance number on each page & linking your letter to your claim pack) as it will likely be months before you're assessed. Worth getting it right now!

These detailed, fairly recent examples should say, when did it happen, where, what exactly happened & why, did anyone see this, & were there any consequences to attempting/doing an activity?

Say if you can't do an activity 'reliably,' i.e. safely, to an acceptable standard, repeat as often as one would reasonably expect, or if it takes you much longer than someone without a disability. See this mentioned at the end of the link I gave above about PIP.