Health Update- Part 3 (The Finale)









Iron Deficiencies-














Health update- Part 1 (Head and Neck)

Following on from my post yesterday I am going to do a few health updates in various parts to bring everyone up to speed. I have so many ideas of topics bubbling away in this brain of mine I really want to write about but for the purpose of fluidity they won’t really make as much sense without me touching on my current situations. The updates may not be the most riveting of reads however it paints a picture for my future posts. This will be the longest of my updates so line yourself a few expressos up and pop a pro plus whilst I try and articulate what this body of mine is up to.

Those that have followed my journey from the start will be aware one of the most disabling aspects of my health is chronic continual headaches, the term headache simply does not do these beasts justice.  Prior to 3 years ago one thing I never suffered with was headaches then in April 2015 I banged the back of my head and neck quite forcefully and the “headaches” soon commenced. We did not pursue or explore the origin of these at the time as I was also diagnosed with instability in my neck, given neck instability particularly cranio-cervical instability can cause headaches I had pinned ALL my hopes on this symptom amongst many others dissipating after fusion of my skull and neck.  When I realised the headaches were still very much alive and kicking I cannot express the utter sorrow, sadness and loss of hope I felt.  On reflection I should have been more realistic in the outcome that surgery is not a fix for everything and its primary aim was to stabilise my neck and to prevent any further damage which so far it has done.

So what are these headaches like? Here goes…….

Firstly I would like to point out the pains are there constantly ranging from a 5 where I have almost learnt to get by with it and almost call it “irritating background pain” all the way to a 9/10 whereby I am on the floor willing someone to remove my head and ended up in hospital numerous times. I genuinely cannot imagine a worse pain and anything in-between that scale. They can be extremely unpredictable and fluctuate in intensity throughout the day, they can either hit extremely hard from nowhere or build up over the course of the day but they never leave.  We’ve all suffered regular headaches in the past from one too many, too long at the computer etc just imagine that x10 all the time then that x20 numerous times a day.  This is the main symptom stopping progression of other areas of my health as I have to lay flat with ice around my head for large portions of the day. The pains feel like a heaviness at the back of my skull alongside someone taking sharp knives and stabbing it, the back of my skull can throb and often feels like someone has taken a baseball bat to it. Accompanying said knife/baseball bat comes intense throbbing/pulsating of the whole cranium which spreads into my teeth, face and eyes. When I stand or lean over it quite literally feels like my brain is going to pop out of my skull the pressure is unbelievable. Alongside this I get visual auras, blurred vision daily, loss of focus/clarity (all the time), loss of vision, greying of vision, dizziness/vertigo, poor balance, tinnitus, whooshing in my ears, stiff neck and shoulders . I can almost hear my own voice echo loud when I talk  which is beyond irritating, if someone is trying to talk to me and the television is on or its a 3 way conversation its like my brain cannot process it, its too loud for me which in reality I know it isn’t, I will have to either put my fingers in my ears or remove myself from the situation.  At the beginning of these headaches there was a postural element whereby if I lay flat they would ease somewhat but over time the postural element has subsided.  Not only are they impacting on me being able to access other aspects of my healthcare and attend appointments they are also impacting my ability to communicate with people for any period of time or be around noise.

This is where is gets complicated ……

We recently went to see a neurologist who takes a particular interest in headache disorders. He was absolutely lovely asking numerous questions about my other health issues and completely dissecting the various headaches, pains and symptoms. It was kind of one of those appointments where you feel extremely understood and listened too yet come out with more questions than you went in with. He thinks there could be a few possible causes of my headaches which will involve invasive investigations. He also felt one aspect of the headache was secondary migraines which he said can occur when you have head a continual headache from a different origin for an extended period of time other types jump aboard too!!  At present we have been trying to treat the migraine aspect of it, so far to no avail.  I was placed on propranolol which is used to treat chronic migraine it is classed as a preventive medication to take continually. As it is in the beta blocker family we thought it would help my POTS also, my track record with medications is not a good one but I was willing to try anything through utter desperation. The first few days on propranolol were fine other than a bit more tiredness I had no adverse effects, I was extremely happy and hopeful this would at least start to mask some of the pains. Around day 5 I started to feel extremely weak/dizzy more so than usual and noticed my blood pressure starting to drop and heart rate was getting lower. Initially it was a welcome break to be able to stand up and not have your heart rate jump to 130 bpm from pots and continued to take the tablet as I was desperate for an ease of the head pain. Saturday night came and I was lay watching a film and from nowhere my heart started to flutter (I wasn’t watching Orlando Bloom), I went clammy and started with crushing pain in my chest although this didn’t feel like a POTS attack I suspected it was and took my heart rate to find it was 42 bpm, this continued and I tried to walk round to increase it however it would not go higher and I felt extremely faint.  After 30 minutes of this it then started to jump erratically from 42-80, 80-130 etc etc then if I lay down it would got back to the 40’s. I was being quite stubborn and hesitant not wanting to go to A&E as my life is just filled with hospital appointments however it did get a bit much and we decided to ring 111 the operator sent an ambulance which I said I did not need however once you mention chest pain and heart rates I think it is a mandatory protocol they follow.  The ambulance arrived by which time it had levelled out a bit but they said they could tell I’ve had a low heart rate as it was 59/60 when they arrived and they said usually peoples heart rates are sky high when they arrive just by the sheer fact an ambulance is there.  They did a quick ECG, blood sugar, temperature and they also noted my pale paler and clamminess.  After much persuasion I agreed to go with them to get checked out to ensure it wasn’t any kind of heart attack.  Once in A&E they did bloods and another ECG.  When doctors were in the room my heart rate was over 60 which is normal but very jumpy then once they left and I lay down quietly it would drop to the lowest 37 and my partner called them in to see and then they saw it shot within a second to 65.  I felt exhausted. Below is a picture we managed to grab of my heart rate at 39 BPM, Mo Farah has nothing on this….


The bloods and chest x-ray came back clear and I was advised by the doctor to stop the medication immediately, I asked if I could try it one more day to see if my body just needed to adjust she could see I was desperate for my pain to ease from the headaches but she said its far too dangerous to have a heart rate of 37 and it jumping so erratically she felt if I took one the next day it would get even lower and I would be back in. My heart had stabilised somewhat and I was sent home but told to go back if it dropped again. We arrived home around 3 am and I went to lie down and it dropped to 41 I was adamant I was not going back so I walked around my house for an hour trying to get it to rise.  Sadly with it being a bank holiday I could not consult with my neurologist or GP, we have since let them know and are awaiting where we go next in terms of treatment.

The possible causes of the main headaches I will list below and can be seen in Ehlers Danlos syndrome and other connective tissue disorder types. They are notoriously hard to diagnose and many people have been diagnosed with migraine initially which was unresponsive to the treatments and after invasive investigations the source was eventually found after many years of suffering.

Theory 1-

A CSF leak. With connective tissue disorders the dura which is connective tissue that surrounds the brain and spine can be very thin/fragile and can tear either spontaneously, through injury like a whiplash or other defects within the spine. This causes a tear in the dura whereby cerebral spinal fluid which coats the brain and spinal cord which flows constantly leaks out of the tear/defect causing what they call a low pressure headache.  Alongside the headache comes other symptoms and neurological manifestations namely what I am experiencing above.  They can be notoriously hard to find if the defect is small.  They often test the pressure via a lumber puncture to see what your opening pressure is or by a bolt placed in the skull.  They sometimes do a CT myelogram which injects dye into your spinal cord that can show the dye leaking out of the defect. If the source of the leak can’t be identified sometimes they do a blind blood patch where they inject your own blood into suspect areas of the spine in the hope it forms a clot and seals the leak.  They can also move onto fibrin glue or worst case scenario surgical intervention.

Theory 2-

Blood flow issues. With EDS the blood vessels in the head and neck can be sluggish and some patients have had occluded/collapsed vessels/arteries therefore the adjacent vessels/arteries are having to do all the work of pumping the blood around the brain.  A test for this would be an angiogram to see how the vessels and arteries are functioning.

Theory 3-

CSF flow disturbance. Along my spine I have some degeneration, disk disease, congenitally fused parts, a scoliosis and an intra dural spinal cyst with noted CSF disturbance.  We are not yet sure if this cyst is just an incidental finding or if one of two things could be at play.  Either the cyst has caused a csf leak as discussed in theory 1 or if the cyst is acting as a damn and preventing the free flow of cerebral spinal fluid.  On the picture below you can see white space around the spinal cord which is CSF, in the middle is where the cyst is and you can see a marked disruption of fluid.  The picture on the right is a back view more of the bone and my scoliosis.


Theory 4- 

High intracranial pressure.  Many people with EDS, although this condition is by no means exclusive to EDS can have pressure issues within the brain. This theory is that the pressure in my brain is high which causes severe headaches, nausea, visual disturbances, tinnitus and much more.  To diagnose this would be an ICP bolt placed within the head to test the pressures. The bolt tends to be more accurate than a lumber puncture as it will give you continual readings over however many days you are in hospital with it in and the specialist can have you moving, bending and see what happens to the pressure in your brain.  It is also deemed safer for connective tissue disorders as a lumber puncture can infact cause a CSF leak (theory 1) by tearing the dura so you may have had high pressure however from the investigation it has caused a leak and now you’re suffering low pressure.

You can see from the various theories the symptoms are all quite similar which makes it extremely hard to distinguish which course of action is best to take so at present we are at a cross roads.  We have to weigh up risk vs benefits however at the same time I can no longer live with the level of head pain and the symptoms that accompany it.

Now….if you have got this far thank you so much as I know that was a long one.  Id say step it down a notch and make yourself a cuppa for the next part.


Since being fused the muscles in my neck are extremely tight,painful and go into spasms which isn’t easy to live with daily but at the same time this is the nature of the beast of neck fusion with limited neck movement. Biofreeze, heat and ice are my best friends. A few months ago we had a bit of a scare whereby the pain increased and was “different” my neck also started to click and almost catch.  It was frightening. I contacted my surgeon who advised an earlier than expected CT scan to ensure the hardware hadn’t moved and the bone was still where it should be.  We went for a CT  scan, once in receipt of the images we uploaded them onto the computer and panic set in, my fusion appeared bent and warped.  Note to self Ali there is a reason radiologists have years of training you are NOT a radiologist. We sent them to my neurosurgeon in Washington who immediately reviewed them and was happy the hardware was still in place and the rib graft had not slipped.  Then ensued 100,000 questions from me!!  He explained depending on the angle of images taken will depend on how it looks, essentially from certain angles/views it will appear bent and misshapen however it isn’t.  He said there is some element of ensuring the hardware fits your anatomy during surgery so rods may have to be moved so no two fused people will have the same images.  We were so relieved by this. We also got a report from the radiologist who did the scan with words of the same effect. Below are a handful of the hundreds of images taken, to be fair you can see by picture one why we were concerned.



Over the course of the next week I will be doing other health updates as there is an awful lot going on and aim to be all caught up and blogging order to be restored within the week (or so) as I really want to get stuck into writing about some particular subject areas.  I predict around 4 more combined updates still to come.

Thank you for taking the time to read and follow my journey, it means an awful lot to me.

Much love

Ali xxx