Journeying with a CSF Leaker

Having been a neuro rehab physio for over twenty years I felt quite knowledgeable about neurological conditions. In 2018 I was to be confronted with a condition I had never heard of before, Spontaneous Intracranial Hypotension (SIH).

On the morning of December 10th I received a call from my wife Viv asking me to come and pick her up from the GP surgery where she worked as a nurse. She had taken unwell with a sudden head pain that felt better lying down. I found her laying on her own consulting room couch with the lights turned off. From there I drove her straight to A&E where aCT scan revealed nothing unusual and we were sent home.

After two more days of Viv lying in bed due to the unusual upright head pain, along with light sensitivity, I decided it might be meningitis or something more acute and insisted on an emergency GP appointment. From this, Viv was admitted to the Medical Admissions Unit (MAU) at the local district hospital. This was to be the first of six hospital admissions in six months.

The upright head pain was the main issue and so the doctor in MAU contacted our regional hospital neurology department for advice. I believe if a doctor thinks they know one thing about CSF leaks it will be the symptom of orthostatic head pain. And so it was that the diagnosis of SIH was made over the phone by a neurology registrar 30 odd miles away. A head MRI was reported as normal so we were incredibly fortunate to have a diagnosis of SIH so early on. This proved crucial for our journey through the health system as the head pain morphed into head pressure forcing Viv flat & other symptoms became prominent. From that first moment of diagnosis, we never once doubted it.

Within days a blizzard of different symptoms arrived. Sensitivity to sound as well as light; blurred vision; voices sounded like robots; nausea; imbalance; tinnitus; head pressure were just some of the issues that presented. Overwhelmingly though Viv just felt really ill. Life has been derailed and she had to lie down most of the time to relieve the pain and pressure in her head. Family Christmas at our home was cancelled.

Knowing the diagnosis gave the opportunity for research. Viv was already reading up about SIH while still in MAU and I remember her saying "I think I'm in big trouble". Later she came across the most helpful video of the condition we have seen: "The mystery headache" by Dr lan Carroll httP-s://y:outu.be/Q WxobgKrc was so informative and clear, I was able to understand what she was going through. It helped me and our relatives who watched it to just 'get it'. From then we stopped encouraging Viv to try and stay upright for longer. That video gave me the understanding that the condition was "FIXABLE". And this word fixable became the safety net word, the reassurance and hope. At that stage though, I still blindly believed doctors would know what to do. Empowering ourselves with knowledge would provide the drive in making future decisions.

There were two blind blood patches given four & six weeks after onset of symptoms but no improvement. Though bedridden, Viv's failed patching, along with an MRI that we later found out was misread as normal, would now cause the diagnosis to be doubted. However heartbreaking, failed patching early in the leak journey would prove helpful in driving decisions.

At home, between hospital admissions, our two teenage boys and I tried to adapt, took on household chores and became Viv's carers. A day bed downstairs to be among the family, trying generally to be quiet so as not to irritate hypersensitive ears. Viv lost weight as she couldn't be upright long enough to eat a meal and felt nauseous. I kept healthy snacks like nuts and fruit next to her and she would grab a little something to eat and drink whenever she got up to use the bathroom. A leaker's version of multitasking!

Visitors and messages were so helpful for mental well-being. I was coordinator of visitors and updater of news to family and friends. So as not to run out of precious minutes upright, for the morning routine I would even put toothpaste on Viv's toothbrush, lay her clothes out ready to put on & place a towel on the bed so she could quickly lie down to dry her hair.

Being upright a moment too long would result in her crashing down, unable to speak or move. I would leave breakfast beside her before going to work and a hot water bottle to help the nerve pains in her arms. Upright time was too limited to do these things for herself. I was so worried at how ill Viv was and it also had a knock on effect on me. She had told me she couldn't think for herself anymore and asked if I would please be her advocate. I felt the weight of responsibility for getting her decent medical help, was worried, stressed and tired.

In researching CSF leaks we had read a paper by Professor JOrgen Beck. He had an astonishing success rate with bone spur removal and dural repair operations. We had no idea what the cause ofViv's leak was, but we were now aware there was a leak expert in Germany. It felt a long way away. Inspire {a community support group) was a place where fellow leakers shared information and I connected with someone who had made contact with Prof Beck's team in Freiburg. I began to think it a possibility we could travel there. Viv thought I was crazy!

The final three week admission to the regional hospital was chaotic with no treatment plan in place. A previous spinal MRI continued to be misread as normal and when I asked about a CT Myelogram I was told "we don't do them because we don't find leaks". "No more scans" became the mantra. Finally a lumbar puncture, which Viv had long resisted, confirmed a leak. A blood patch was planned, then refused by an anaesthetist who said "we need to locate the leak!". A radionuclide scan was planned but we knew it wasn't the one needed to locate a leak.

Viv had lost all hope in getting the help needed. She was often told she would ••just get better''. I kept saying she was getting worse. The phrase "You might need to live like this" was a hammer blow. While there was definitely reason to be depressed, I believe it to be a physiological cause too. I tried really hard to encourage and lift her spirits with minimal success. I had a vivid dream of me grasping Viv who was in a dark brown swamp, while I was hanging onto her from a steep slope and in turn I was being pulled up by friends. Occasionally Viv would go under the surface. She left hospital that final time in a far worse physical and psychological state than I could have imagined and no treatment had been given.

A Mayo trained Professor of lnterventional Neuroradiology with knowledge of spinal CSF leaks was an unexpected family contact in the USA. His good advice we well understood and that was to "get to the specialist". But where? I had reached out but failed to obtain help via other routes in the UK. I didn't know how we would do it but I felt our only hope was to get to Professor Beck's team in Freiburg, Germany. I sent Viv's scans to them by Dropbox and while Viv was still in hospital I spoke with Dr Fung by phone. He was the first doctor I had spoken to who really understood the condition. He had reviewed Viv's scans, listened to me explain her symptoms and then simply said "if she comes here I think we can help her". There was hope once more! It took me a while to persuade Viv to consent to go, but as soon as she gave her consent I started making plans while Viv started learning some basic German. Funding was now the issue.

Ever sent a "send to all" message a Ia Michael Mcintyre? I did that on the day I set up our just giving page. Our estimate for the trip was based on a quote from Freiburg hospital plus travel costs and accommodation for me. We could not afford to go without raising significant funds.

There was physical and emotional support from friends and family, and now we had to put a begging bowl out too. Our network of contacts, friends, family, church community, even Viv's patients and complete strangers all contributed. Our local paper picked up the story and published a front page article with the link. My band put on a fundraising concert, friends put their creative skills into practice to raise money with coffee mornings, cake sales, selling lights made out of gin bottles and handmade cards. We were astonished and humbled when the money came in and our Freiburg trip became a reality.

Midnight the 15th April 2019 a private ambulance arrived to take us to Freiburg. Viv was able to lay flat for the 16 hour journey and on arrival, exhausted, we could see Dr Fung looking down from a window waiting for us. We were later Viv was quickly whisked away for her MRis. All four tests on the Freiburg leak protocol showed evidence of a CSF leak. Within 24 hours of arrival the exact leak site and the bone spur causing the leak was found using a dynamic CT Myelogram. The 16th April was our eldest son's 18th birthday and this news was the best birthday gift we could give him. The elation of letting friends and family know the leak was found is impossible to describe. So many had contributed in so many ways. We were happy to at last be able to give good news.

It was with relief I received a phone call on the 23rd April to say the surgery had gone well and according to plan. Professor Beck and Dr Fung had performed the minimally invasive microsurgery to remove the bone spur and repair a 4mm tear in the dura, the same surgery we had first heard about from the research paper. The day after surgery, Viv was able to sit inclined in bed. For both of us the recovery could begin.