The Illnesses I Have

I thought it may be helpful to try to explain the illnesses that I have and the treatments that I am hoping to have.  This is in no way intended to be a "woe is me" as I do know there are many people who are far more ill than myself.  However, I thought I would quite like to use my own, tiny corner of the blogsphere to raise awareness and give some information.

This post has taken some time to put together as I wanted it to be medically correct, rather than simply my own experiences.

Chiari Malformation

This is a fairly rare illness, probably the least well known one that I have and what has caused the other two.

Chiari malformations (CMs) are structural defects in the cerebellum, the part of the brain that controls balance. Normally the cerebellum and parts of the brain stem sit in an indented space at the lower rear of the skull, above the foramen magnum (a funnel-like opening to the spinal canal). When part of the cerebellum is located below the foramen magnum, it is called a Chiari malformation.

CMs may develop when the bony space is smaller than normal, causing the cerebellum and brain stem to be pushed downward into the foramen magnum and into the upper spinal canal. The resulting pressure on the cerebellum and brain stem may affect functions controlled by these areas and block the flow of cerebrospinal fluid (CSF)— the clear liquid that surrounds and cushions the brain and spinal cord—to and from the brain.

Chiari picture 2There are several different types of Chiari and I am not entirely sure which one I have.  I was initially told I had Type I because it is the only type where you don't become symptomatic until adolescence or adult-hood, if ever.  My current Neurosurgeon stated in his most recent letter that I have Type II, so I don't know.  Symptoms include dizziness, insomnia, depression, hearing loss, migraines, headaches made worse by coughing, sneezing or straining and balance issues.  I have each of these and have done ever since my early teens, although my hearing loss is from birth.  Whether that is related to Chiari or to my traumatic arrival into this world we don't know and are unlikely to ever know.

There is no cure for Chiari.  The best we can hope for is quite major decompression surgery which involves removing a part of my skull to make more room.  Recovery from this can take anything from a few weeks to several months or even a year or more.  This is not a cure, it is merely to prevent symptoms worsening, possibly ease the current ones and to prevent paralysis.  There is even a risk of paralysis or death in having the surgery.  However, given the rate my symptoms are progressing and the inevitability of ending up in a wheelchair, I feel it is a risk worth taking.  I can hope the surgery will ease my symptoms but this is only a 50/50 chance.

Hydrocephalus

The term hydrocephalus comes from two Greek words.  "Hydro" meaning "water" and "cephalus" which means head.  It used to be referred to as "water on the brain".  My hydrocephalus is thought to have been caused by the Chiari.  This is because that has caused a blockage which prevents the flow of CSF to the spinal cord.  Effectively the vast majority of my CSF is around my brain.  CSF carriesnormal-hydrocephalus-comparison nutrients which are essential for the function of the brain and also carries waste products away from the tissue in the brain.

The brain is usually very good at maintaining the right balance between the CSF that it produces and that which it absorbs.  Hydrocephalus occurs when there is an imbalance between the amount produced and the rate at which the brain absorbs it back.

It is most likely caused by the Chiari which blocks the entrance to my spinal cord.

For hydrocephalus, it is normal to insert a shunt, which drains the fluid away from the brain to another part of the body where it can be re-absorbed.  However, since these are mechanical devices, prone to failure, my own neurosurgeon would like to perform what is called an endoscopic third ventriculostomy (ETV).  Here, he will make a small cut in the base of my third ventricle which should then enable the CSF to flow more freely.  If this doesn't work, I will need a shunt.  All of this is assuming I can have surgery in the first place.  I had my pre-op assessment a number of weeks ago and it has been decided that I need to have a heart scan and see a cardiologist, hopefully I will then get the all clear!

Syringomyelia

The spinal cord is usually solid, passing down the back, inside the spinal canal.  It connects the brain to the rest of the body, passing signals to and fro, enabling an individual to move his or her limbs at will, to feel objects and to control various bodily functions.  Within the spinal canal, the cord is bathed in CSF.

Syringomyelia is a disorder in which a cyst or cavity forms in the spinal cord.  The fluid from which these cysts or cavities are formed  is similar to CSF.  Over time, syringomyelia can lead to paralysis.  Otherwise it can cause pain, weakness and stiffness in the neck, back and shoulders.  A a person with syringomyelia can also lose the ability to feel extremities of hot and cold, particularly in the hands and feet.

My own cavity is pretty much the entire length of my spinal cord.  As such I have very little protection of my spine and have now been advised against white knuckle theme park rides (which I love!), sky diving (on my Bucket List), bungee jump (also on Bucket List) and trampolining.  My NS advised that even a relatively minor shunt in a car accident could cause a huge amount of whiplash, or worse, to me.

Diagnosis is HARD!

I think part of the reason it is extremely difficult to get diagnosed with these conditions is because the symptoms and the impact they cause varies so much.  Not just from one patient to the next but from one day, week to the next for each patient.  From personal experience, I can be totally fine (well fine for me) one day and will spend that day doing normal activities; chores, the nursery run and a trip to the park.  I may also do dinner that night.  Then the next day it will take all of my energy, teeth gnashing against the pain, to do something as simple as go to the toilet.  Having just one good day, wanting to make the most of that, can then mean I am confined to our room for days on end.  Perhaps I shouldn't push myself so much on the good days but it is hard not to when you want to make the most of those good days.

I hope this has given you just a bit more information and knowledge of this thing that is Chiari.  Most of us Chiarians will have  at least one, if not both, of the other disorders too.

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15 thoughts on “The Illnesses I Have

  1. Doug Long

    I was decompressed July 2002 after a few bad years when the doctors thought that I was creating the Chiari related problems in my mind to keep me home from work. They did not know that I had many, many perfect years during my career when I did not have one day absent from work due to illness. Finally a MRI showed that I needed surgery. After a 6 - 7 month recovery I was once again back to work and was so happy to be problem free for 3 - 4 years. Unfortunately, I have low grade asthma attacks a couple of times each year, usually associated with allergies, colds or the flu. I or my family physician wasn't overly concerned with my fits of coughing so badly that I broke tiny blood vessels in my throat. the puffers eventually took away the coughing and asthma after 6 - 10 weeks. I was also having Chiari type problems again, specially nerve pain and numbness in arms , shoulders and neck - chest area, along with the dizziness, weakness, balance problems and headaches. My family doctor told me that it could not have been Chiari as that was "fixed" when I had the decompression surgery. Finally after I was doped up with Demoral to help with the the extreme arm nerve pain, I was referred to the Neuro-surgeon who has now scheduled me an appointment on June 10/13. He tells me that i most likely have pockets of fluid in my spine. since that visit t the surgeon, I have researched more about the pockets of fluid, or Syringomyelia. Apparently often these pockets of fluid can be formed, with straining such as deep coughing, especially for Chiari patients. Neither the surgeon, the family doctor or anyone else told me this. I could have been so much more careful about the coughing and would have purchased oxygen cylinders if that would have helped. I would have done anything to not be where I am today. I am seriously considering suing the doctors at both levels. They either did not know the difference or they did not think that i was destroying my own life. My goal would be to make doctors more aware of the problems associated with Chiari, to make the general public more aware, to help make other Chiarians aware and to be awarded money that will help my family care for me when I require much more care - thanks to these "doctors!"

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    1. Post author

      Part (or dare I say, most) of the issue is that so few people are aware of chiari I think and also the symptoms can be attributed to other things, particularly if you only display a few. Add in the other associated disorders, unless you have a knowledgeable NS or NL you are likely to just be given pain relief as opposed to real help.

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  3. Wow. I have to admit that I had never heard of Chiari. It's obviously hugely debilitating for you. Thank you for sharing and putting us all in the picture. I do hope that the surgery you are about to undertake will give you relief in some way x
    Suzanne recently posted..I Am Going to Britmums Live!My Profile

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  4. Thanks for sharing this. I know you've touched on your illnesses before, but this is a really useful explanation. It must be so frustrating for you going from good days to bad days, but I'm sure you are grateful for the good days. I hope you get your surgery soon. X
    Sarah MumofThree World recently posted..No more bright clothesMy Profile

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    1. Post author

      Thank you, I hope it didn't come across "woe is me". I just want to try to awareness somehow, much like Emma has done. If I could talk today I would be chasing for my cardio appointment! Xx

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  5. Amanda Pritchard

    Hi I have syringomyeua and diastematomyelia spinabifuda curvature vertebrae missing and part missing it has veen very interesting to read this as I have go terrible balance problems from 3 functional strokes that they cannot expkain they are treating it as vertigo I have horrendous migraines which cause pain like nothing like a headache, also unknown unexpkained terrific pain in back now going into hips mibikuty us decreasibg due to intense pain swollen legs like fluid retention mobility is getting worse yearly there is s definet difference for the worse toilet functions are getting worse deafness is worsening too depressions comes upon me for about 8 months which is dark and deep but I am coming out of the kast one thank the Lird I have stiffness in hand legsxwelk all joints and is getting worse I an hopibg ti see a new consultant soon as my oast neurologist jyst keot sayibg uf it getsworse cone and see me but there are onky so many tines you xab hwar that and wonder why nothing is being done I an nit writing for sympathy jyst want to know if anyone can help me with the symptoms also my cord goes all the way to my cocksix which is tethered with a bone going through the middle causing intense pain sensation loss numbness pins and needkes weakness please can you tell me if this is normal and who I shoukd see consultant wise that understands it all becayse I bo nothing at all and I need it explaining and guidance thank you amanda 45 from the uk

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    1. Post author

      Hi Amanda, I will link this up to someone who I think may be able to help, she is certainly been a fountain of information for me, perhaps she can help you, although she is not a doctor but may be able to point you in the right direction. Xx

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  6. I think you're amazing to go through what you're going through in your life and still be trying to help others. This is a really informative post. I'd never heard of Chiari before but I found your post really helped to understand what's going on and if I hear of anyone else who needs information about it I will pass it on.

    Good luck with everything, I really hope the operation provides you with a relief from symptoms.

    x
    Musings from a Mum recently posted..Childcare reform – what’s the solution?My Profile

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    1. Post author

      Attempting to help others I have found is keeping me from wallowing when I am stuck and unable to do anything for ourselves.

      Thank you for your continued support. Xx

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  8. That sounds really really tough. The surgery sounds frightening, but I can see that it may well be worth the risk. I hope it all goes well.

    Hm, just noticed this post is from May, so I imagine there must be more recent updates. Will go in search of them...
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