What I Need To Accept After Years Of Rare Intracranial Hypertension Treatment

Rare Disease Day 2022 is always on the 28th February, so at this time of year I tend to reflect on the treatment I’ve had for the rare brain disease I have - Idiopathic Intracranial Hypertension (IIH). 

I’ve been treated by a specialist team in Cambridge for the past 7 years. They’ve developed rare treatment plans for patients, who like me, often present with atypical IIH symptoms. Sadly, the treatment options they’ve developed have been exhausted in my case.

I spoke to my neuroradiologist about this last month and he asked about the results following my first procedure, so I re-read my notes and the hopeful blog I’d written back then. I now have to accept that my symptoms haven’t resolved as I’d hoped and this blog could help.

The surgical team has established unorthodox medical care that most neurologists consider controversial. This affected the support I received, so I had to learn to advocate for the care I wanted. Medical journals now publish more on these treatments, giving me hope. 

In this blog I’m going to look at:

• My Background Story Of Idiopathic Intracranial Hypertension 

• What I Wrote About How I Felt After This Procedure 

• The Results Of My Following Treatments 

• What I Am Feeling After Completing My Treatment Plan

My Background Story Of Idiopathic Intracranial Hypertension 

In January 2014 I became ill and was diagnosed with chronic migraine, but after years of migraines I had doubts. I grew aware of a symptom I’d never experienced before, a whooshing in my ears. I researched Pulsatile Tinnitus (PT) and my family doctor confirmed it.

I found an ENT surgeon who specialises in PT and he believed I had IIH. But my symptoms don’t fit typical diagnosis factors, so with the support of my wonderful family doctor, I had to advocate for 18 months to get their Intracranial Hypertension treatment. 

IIH, it’s symptoms and my full story are detailed on this blog. My main symptom is constant head and facial pain that’s mostly right sided and mobility issues due to imbalance. I also have brain fog, nausea, photophobia (light sensitivity) and PT.

In October 2014, my IIH diagnosis was confirmed with a lumbar puncture and a CT venogram (scan with dye). This showed narrowed veins at the skull base and venous sinus stenosis, where the large vein in the brain is narrowed. This causes fluid build up in the head. 

IIH is a rare disease that affects 1-3 in 100,000 people. My scans identified Transverse Venous Stenosis causing constricted outflow in the veins at the back of my skull. This and the lack of papilledema puts me in the group of 6% of all IIH patients with this presentation. 

Their Idiopathic Intracranial Hypertension treatment is controversial with most neurologists. IIH is usually accompanied by Papilledema, which if left untreated can lead to loss of vision. The pressure reading from a lumbar puncture is usually very high, mine wasn’t. 

When I’d mention the Cambridge team or IIH, my local neurologists turned me away. I found one who would see me, but she still didn’t agree with the team’s radical surgical plan to remove some bone to improve venous outflow in my neck (see below for details).

This sounds outrageous but my pain and standard of living was so debilitating that I’d try anything for relief. The plan was brain and spine surgeries and stenting the narrowed vein in my brain. This article explains the link between venous sinus stenosis, PT and IIH.

Reflecting On The First Procedure Of My Treatment Plan

After a very long year of waiting, the team’s neuroradiologist was able to perform my first Intracranial Hypertension treatment. I had a cerebral angiogram and venoplasty as an outpatient procedure to see if surgery to expand a narrowed vein would be beneficial. 

They thread a catheter up to the blood vessels in the neck that supply the brain, imaging and measuring the pressure inside my veins. Then they inflate a balloon in the vein (venoplasty) to monitor how it responds to decompression over a week. 

I noticed a difference immediately and by the time I went back to the ward I was a different person to the one admitted that morning. I had lower pain levels, no nausea and a clearer head. The difference was visible and as I chatted, Joel, my husband, said “you’re Laura again”. 

The improvement continued over the following days. I was able to walk the stairs, which I hadn’t done for 12 months, I didn’t need any oramorph, which I’d been taking 2 or 3 times a day and I reduced my other pain killers temporarily.

My PT and nausea returned first and a week later I woke up in my usual state of brain fog and pain. My surgeon was pleased with the results of the venoplasty. Although temporary they clearly showed I had restricted venous outflow and that surgery should be beneficial.

What I Wrote About How I Felt After This Procedure 

I’d been told not to be disheartened if the symptoms reappeared the following week but it was frustrating to return to debilitating pain. However, I was pleased that this proved I didn’t just have chronic migraine.

At the time I wrote “I am… pretending I’m ok, feeling bad for moaning about hurting, crying and letting people down and not being able to do things with my family… [as] the pressure [builds again]. 

[I’m] missing out on… living life… trying to explain why everything takes so long and feeling worthless… knowing that the freedom of mobility is drifting away. [I’m] crying and waiting for that time bomb of pain to build so much that [I] can’t take anymore.

I have meditated, eaten, napped and had a few pep talks from friends and my husband… There have been tears today but I’ve been reminded that I [will] see my amazing boys grow up… with my best friend [Joel] of 18 years. 

I have hope that there might be light at the end of this very, very long tunnel... I have amazing friends and family that are always there… even though I feel I’ve let [them] down by only being better for a few days… [when] I was so tired I just slept! 

I am back making jewellery… reminding myself that I CAN still do things that matter despite feeling like I’ve been run over by a truck after drinking… whiskey.” My true friends have stuck by me and Joel still gives me pep talks. He and the boys keep me going each day.

The Results Of My Following Treatments 

It wouldn’t be a complete reflection if I didn’t share the Idiopathic Intracranial Hypertension Treatment I’ve had since that first procedure. This blog has been more emotional than I thought because it’s shown me how poorly I was at the start of this journey. 

I’ve had venoplasty procedures and many CT venograms. These show how the jugular vein passes through a small space between two bony structures, the styloid process and lateral mass of the C1 cervical spine. In my case the jugular vein is narrowed and flattened. 

The anatomy of these areas are shown in these images.  

This is my IIH story outline of the surgeries I’ve had: 

1. Styloidectomy - removal of a small bone fragment that sticks out of the skull, behind the ear.

2. Stenting on the right side of the transverse venous sinus.

3. C1 mass removal of the right transverse process.

4. Re-stenting the right transverse sinus due to a compromised stent. 

5. C1 mass removal of the left transverse process.

6. Stenting on the right side of the transverse venous sinus. 

With each surgery I’ve seen some improvement, most notably my cognitive ability. My final surgery, in 2019, has had the most impact. My balance improved significantly and a month on, I managed a day out with my family, using a wheelchair, for the first time since 2014.

What I Am Feeling After Completing My Treatment Plan

It’s been emotionally challenging reliving my IIH patient stories again. There’s been many tears as I think back to the hope I had for this treatment, a normal life off of the merry-go-round of constant pain. I’d naively counted on my surgeons to fix me, so the reality is crushing. 

When the first venoplasty procedure had clear results, confirming I’d benefit from venous stenting surgery, I felt optimistic. Every surgery improved my condition but I’d gradually worsen each time. 

Reading a blog from the start of this journey shows how challenging communication was for me. I’d be stuck there without surgery which would have broken my family. As I complete my treatment, the harsh facts are that I suffer daily but I am able to be a good mum and wife

This 2012 US case study states that “Intracranial venous hypertension may result from… compression of the jugular veins at the skull base. Although rare… [it’s ] important to recognize [this] because stenting…may actually exacerbate the outflow obstruction.“

My surgeon stated this could happen with stenting. He described it as removing a blockage at the top of a hose pipe, when it’s squashed lower down. The water builds up, leading to increased pressure. 

My scans still show narrowed veins at the skull base but I’ve had all bone surgeries recognised for treating IIH. My surgeon can only see one possible surgery to relieve pressure here but it isn’t seen as a viable treatment for IIH. I’m frustrated by having to forget this option. 

All involved are disappointed about completing this treatment plan without the success we’d hoped for. But PT took me down this path, and I’d hoped that this symptom would be resolved. As this is mostly the case, then I can count this part of the treatment a success. 

This has reminded me how my quality of life has improved because of my Idiopathic Intracranial Hypertension treatment plan. The positive outcomes are:

• The cognitive change I’ve experienced is quite dramatic. 

• My mobility has improved so much I no longer fall.

• I can walk on one level of the house, without mobility aids.

• I don’t experience daily dizziness or vertigo 

• My phonosensitivity is less invasive

Sadly, my head pain remains constant, despite initial improvement after surgery. My high pain levels are disruptive so I still spend much time in bed. It still takes days for me to recover from any activity, even going downstairs, which can feel oppressive. However, over time I’ve learnt how to priortise for better life balance.

I’ve created this free wellness planner to help you prioritise for better life balance too. This is also a taster of the the Thrive Not Just Survive Journal, for mums with invisible illnesses coming out soon. Simply click on this link to sign up for your free copy. Please feel free to ask me any questions you have about using this resource.

It’s been an exhausting journey but looking back I take the positives, such as my ability to build connections, from my treatment as I move forwards. My surgeon has suggested a doctor sympathetic to their work, who I’m working with on the next stage of my treatment plan. 

It’s scary saying goodbye to familiar territory as I complete treatment in Cambridge. I’m reassured and hopeful after speaking to my new doctor, but I’m anxious and apprehensive about what I face ahead.

P.S. If you’re a skim reader, catch up here -

I’ve been reflecting on my unusual IIH or Intracranial Hypertension treatment. This rare brain disease causes debilitating pain, mobility problems, brain fog and more. I re-read a hopeful blog I’d written in 2015 after my first procedure, when my husband said “you’re Laura again” as soon as he saw me. As this treatment ends I accept my symptoms haven’t all resolved but there are positives I can take on into the next part of my journey. 

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