CSF Leak Story: No doctor could diagnose my strange affliction – so I did it myself


This article featured in Toronto Life on 18 September 2014

By Mireille Silcoff | Illustration by Cat Yelizarov
Memoir: My Strange Affliction

In my late 20s, I became crippled with debilitating dizziness and headaches. I visited eight doctors—and no one could figure out what was wrong with me.

A decade ago, I lived in a beautiful apartment near Avenue Road and Davenport, an elegant one-bedroom that contained most of the frothed-up dramas of my highly decorative 20s. I did up my living room in blue Albrizzi lacquer, with a deep feather loveseat upholstered in fuchsia velvet. It was honeytrap decor: my place wasn’t so much a party apartment as an after-party apartment. I filled my fridge with prosecco and my medicine cabinet with designer drugs and my closet with $1,000 dresses I had no business buying. I spent most nights waiting for a guy who usually called at midnight.

I would lie on my sofa, with my hair done, smoking, tingling. I would sit in front of my computer, drinking, vibrating. If he didn’t call by 12:30, I would fall asleep on my stomach, so as not to squash my hair. In the morning, clumps of hair would come out in the shower, as if shearing the plane for another day of hope surging up and dying on its stem. I was dizzy all the time.memoir-my-strange-affliction

At first, I thought I was just sick with longing. Then one night, sitting by the non-ringing phone, it suddenly felt like my hands and feet were covered in pins and needles. I walked to the emergency ward at Mount Sinai.

In the pantheon of medical symptoms, there are those that doctors will immediately mark as stress-related, and thus lightweight—don’t complain of dizziness, or ringing ears, or headaches, or vertigo. If you are an overworked, nerve-wracked, single Jewish newspaper editor kitted out for a bad affair, walking into emergency at 1 a.m., you’ve got your cultural profile working against you. The ER doctor told me to take a holiday. Try some yoga.

I saw eight doctors in Toronto. One said I might have an inner ear issue. Another thought I was hyperventilating without knowing it. A third put me on steroids. Most agreed my symptoms were “stress-related.” Incredibly, nobody looked at my brain.

Over the next few months, I left my apartment only for medical appointments. I spent the rest of my time helplessly supine, feeling like the top of my head was being torn off with nautical rope. Every waking moment I could keep my head up I spent in front of my iMac, trolling through obscure neurological journals, reading papers I could barely understand. I narrowed it down to the most likely illnesses—multiple ­sclerosis or a brain tumour—but neither seemed right.

One day, I found a syndrome called spontaneous ­cerebrospinal fluid leaks, something so rare most neurologists have never seen a patient with it. I read the symptoms and a huge flashing YES lit up behind my eyes—I knew I had found my illness. It wasn’t anxiety. It was faulty tissue. The sheath of my spinal cord, the dura, was tearing, and my spinal fluid was leaking out, sapping my brain of its natural waterbed, the thing that kept my grey matter from sinking into the occipital bone.

The first doctor to properly check the pressure of my spinal cord found it to be about a third of what it should be. The next few days were blurry and dreamlike. My family came in from Montreal and whisked me back there for treatment. Everything I knew in life blew away like fairy dust. I underwent 14 spinal cord procedures over two years, and they all failed. First, doctors in Montreal sprayed patches of my blood onto parts of my spinal cord, ­hoping the resulting clots would patch the holes. When those didn’t work, doctors at the Mayo Clinic in Minnesota used surgical glue to staunch the leaks. For days at a time, catheters injected saline into my spinal cord, a feeling so ­nightmarish I developed a phobia of needles.

Six months later, I flew to L.A. to see yet another surgeon, who planned to staple my spinal cord back together. The anesthesiologist, a Latino man with a thicket of black curls who had conducted some exploratory tests, called me at my hotel the night before the surgery. “Don’t do it,” he said. “Listen to me. I have felt your spinal cord. Imagine ­stapling wet Kleenex. Your dura is like that. You must simply stop moving. You must see if nature will knit you back together.”

I took his advice, cancelled the surgery and tried to let myself heal. I spent the next 18 months in a declined bed, my head lower than my chest, to give my brain the suspension it no longer had naturally. I wore a corset to keep to keep my torso bound and my spine squeezed tight. For two years after that, I was wheelchair-bound, not knowing whether I would walk again. Death was always near, an aneurysm just a sneeze or a bad pothole away.

By my late 30s, my long-standing leaks began closing up on their own, and life was slowly returned to me. I carefully, incrementally, went back to work, got married and, two years ago, my husband and I had a daughter; I am currently pregnant, at 41, with our second baby. I do have relapses. I disappear into my home, into the pain, and come back to the world a month or two later, with the rawness of a recluse returned to sunlight, but also the strange clarity that deep illness can provide. I now live with new caveats, things I tell myself regularly: don’t get too cocky; illness is always right around the corner for you. Don’t you dare waste your life—that’s not your kind of freedom.

Read the original article here: http://www.torontolife.com/informer/columns/2014/09/18/memoir-mireille-silcoff-my-strange-affliction

New Factsheet: CSF Leak Terminology


We’re pleased to announce the publication our third CSFleak.info factsheet – Quick Reference Guide to CSF Leak Terminology – which can be downloaded via our downloads section or by clicking on the button below.

Our factsheets provide easy access to key facts and information; they are printable and can be taken with you when you meet with your GP. They are provided in good faith and following careful research, however they cannot be guaranteed error-free and are not a substitute for professional healthcare advice.

Living with Intracranial Hypotension: Claire Hubbard’s Story


Before becoming poorly in Dec 2009, I was a happily married, fit and healthy 43 year old with 2 teenage daughters. I collapsed one day for no reason and had not been right since. I would blank out and collapse in a headache attack after being upright too long, resulting in many visits to A&E. After suffering for nearly four years and almost every test you can imagine showing nothing, last July 2013 we decided that enough was enough and to take the bull by the horns and do something once and for all. Final decision. It turned out that was the best decision we had made.

I had been diagnosed with POTs in 2012 but felt it was not quite the right diagnosis for the sort of symptoms I was feeling. I had not got the tachycardia or dizziness many people with POTs have and my heart rate only just increased to the POTs threshold of 30 beats per minute when I stood up. I did have low blood pressure and had spent most of 2010 lying flat, which could have prompted the POTs like symptoms. I also had a daily headache, but mine was ongoing – 24 hours a day, every day. It was lessened in the morning but gradually got worse as the day progressed.

Every day I spasmed or jerked if I was upright too long or did too much. I would have to sleep every day for an hour at lunchtime in a completely flat position just to be able to continue with anything in the afternoon. If I did not, my head pain would simply increase till I had to lie down and spasm or jerk in pain.

My turning point was to pay privately for one consultation with a neurologist at my local hospital. We had to persuade my GP for the referral, as I knew it was my last chance and he said I had seen enough people. But, my new neurologist listened…

He was very interested in my symptoms and sent me for a series of tests – some of which I’d had before. All came back negative for a CSF leak. I would tell anyone who thinks they are leaking to persevere with doctors, as, if they can’t see a leak, they will say you haven’t got one, even if it is a slow leak and not likely to be seen.

I did my research. I read Dr. Schievink’s Question and Answers and highlighted anything that referred to myself. I even went on a POTs medication trial for a fortnight whilst in hospital to see if that would help. Once we had exhausted all the doctors’ ideas, including many of the anti-seizure medications, (all of which made my symptoms worse).

In February 2014, I lifted a heavy weight and carried it some distance, feeling that something had gone wrong as the pressure in my head suddenly grew. I went home and 36 hours later I could not get up at all without severe pain. It took 2 days to get hold of anyone who would help me and I was prescribed phyllocontin as it is a form of theophylline that increases csf. I was up again in 36 hours, but after taking the medication for 3 weeks and got gradually worse and worse until I was having repeated painful non-epileptic seizures – as in 8-11 in a row. I ended up in hospital where they did not know what to do with me. I was seizing at first 3-5 times a day, reducing to three times a day later on. Every day was the same and it was awful. I persuaded the UK doctors to try a scan that I hadn’t had after emailing Dr Linda Gray in North Carolina, USA. I had already had contact with her as I had asked for a quote to see how much money I needed to raise in order to get a second opinion. I asked her what she would do for me that the UK doctors may be able to do.

She said she would scan me and look for any potential leak sites. The scanner she uses is a DSM scanner and my hospital had not got one, but as it is a fairly newly built hospital building, they had the latest MRI scanners up to 3 tesla. Many MRI scanners are 1 – 2 tesla so I knew this scanner would be as good as a DSM scanner (hopefully). The test she recommended was a fat saturated MRI myelogram, using intrathecal gadolinium. My neurologist immediately said it must be done and went to organise it. And this is where it gets tricky. It was proposing to use gadolinium off license and so had to go through 3 weeks of protocol and paper work. I was desperate for help.

Eventually the test came. The way it was organised was to do 3 MRIs, one before the gadolinium was introduced and two after. Each MRI lasted 70 minutes. We split it up so the first one was done on one day and the next two on the next day, as it was a long time to stay absolutely still. This was completed Friday afternoon about 6pm. I would be lying if I said it was not hard work. To stay still in the noisy MRI for that long is difficult, but I knew I must do it if I wanted any chance of any leaks to be found.

The next day I was told the test showed nothing. I was surprised as it was the morning and I was trying to work out who had looked at them in so much detail to ascertain that information in such a short space of time.

So, the next step for me was to get a copy of the scans, to get a second opinion. Again, this isn’t easy. You have to apply to the ministries department at our hospital and pay £50 for your written records and scans. As an inpatient, the scans were not allowed to be released. I had to fight for this by asking the Chief Executive of the hospital to intervene, as my neurologist was more than happy to get the second opinion from the States. This took a while but they did arrive.

I have a friend who was able to take the discs and link them as a hyperlink to his Drop box account. This enabled me to simply email Dr Gray in USA with the link and password. I was the go between for my neurologist and Dr Gray for many days. At first she couldn’t open them, then she could. Then she sent the email that made me take a huge sigh – she could see potential leak sites and could name some of them. She wanted to see the most recent scan too as she couldn’t open them. This took a while with instructions and print screen images of how to find what she wanted. She advised my neurologist to do a full volume patch at the cervico-lumbar and then mid-thoracic region.

When I discussed this with my radiologist, he said the areas were too wide for him to think I could get any gain. I asked Dr Gray for yet more information. I offered to pay for her services all along, yet she would not accept anything. She replied with a comment of doing the same as before but adding a lumbar region patch too, as the UK would probably not be able to do CT fluoroscopic targeted blood patches at any diverticula that could be a problem and that she would look at me when I could get over there.

My radiologist took my scans home for the weekend and then on Monday, he gave me the choice. Three large blood patches or many targeted ones – he said that they could do that but with blood, not fibrin glue. The latter would be more painful and take longer. I decided to go for the targeted ones, as that is what Dr Gray would do in the USA.

It was not an easy procedure. I was asked if I would be able to lie absolutely still for it. As it was in the morning I felt confident I wouldn’t seizure as any seizure activity had started developing in the afternoon. After the lumbar puncture and injection of dye, I was asked to roll 15 times lengthways on the bed to mix the dye with the csf fluid. This is easier said than done. After that I lay on my front on the CT scanner and was asked not to move at all whilst being scanned and whilst the needles and then blood was inserted.

This is really important. If you move, the blood patches will not go to the targeted areas and will be useless. I had numerous needles put all down my back, from my shoulder blades to my lower back. Most of these were in pairs but some were single. The needles were inserted after an initial local anaesthetic on each one. My radiologist was meticulous to keep them in the exact right place. Sometimes a nerve was touched or tickled, but I still was not allowed to move. When all the needles were in place, the blood was syringed out of my arm and into the needles, about 5cc at a time. A lot of that ran out and down my sides! Some of the inserted blood was quite painful.

After each needle was filled, they were all removed and it was over. I lay head down in a gentle tilt for 2-3 hours before I sat up and ate my tea, feeling much better. I did not spasm, jerk or seizure from that moment onwards.

I am now at home, at the moment, 2 weeks in and symptom free. Totally. I’m no longer noise, light and smell hypersensitive. I can stay upright all day if I want to and can drive. I feel free and released. I know I’m not healed yet and so need to take it easy. I am planning to see Dr Gray in North Carolina as my neurologist thinks it would be a good idea to get a second opinion and maybe fibrin glue patching. I’m planning to go out in about a month’s time.

If you want any recent updates, please see my blog on: www.lucyhubblog.wordpress.com

Claire Hubbard

Anonymous donor gives £10k to fund Eccleshall woman Claire Hubbard’s vital brain scan


SERIOUSLY ill Claire Hubbard could soon fly to the U.S. for pioneering treatment on her brain – after receiving a £10,000 anonymous donation.

Mother-of-two Claire, from Eccleshall, has been diagnosed with intracranial hypotension, which causes brain or spinal fluid to leak. It means she suffers from constant headaches as well as daily seizures.

The 47-year-old’s only hope is to travel to America to meet a specialist and undergo a scan to pinpoint the leak.

Her friends and family have been trying to raise at least £13,000 to help meet the costs.

Read the full story here: http://www.stokesentinel.co.uk/Anonymous-donor-gives-pound-10k-fund-Claire-s/story-20963083-detail/story.html#ixzz2zMG8QJaq

CSF Leak Questions Answered by Dr Schievink


We’re pleased to announce that with the kind permission of the CSFleak.org Forum, we are able to publish a recent Questions and Answers session with leading CSF leak expert, Dr Wouter Schievink. The questions were posted by members of the CSFleak.org forum during January 2014 and answers were provided on 4 April 2014. We are deeply grateful to the administrators of the forum for arranging and managing the Q&A session and for making the information available.

Dr Schievink is one of the world’s foremost experts on CSF leaks and Spontaneous Hypertension. Originally from The Netherlands, but now practicing Cedars-Sinai Hospital in California, he has conducted a number of studies into the diagnosis and treatment of CSF leaks and many specialists in the UK base their approach on his work.

This valuable information sheds light onto many of the questions and issues faced by leakers across the UK and Ireland. Dr Schievink’s advice will also be of relevance to GPs and other medical professionals dealing with patients presenting with persistent daily headaches and other symptoms of intracranial hypotension.

You can view the Q&As online by clicking here or download them by clicking here.

Headache Journal: Spontaneous Cerebrospinal Fluid (CSF) Leaks


Spontaneous cerebrospinal fluid (CSF) leaks typically present with orthostatic headaches. Less commonly, spontaneous CSF leaks can present with other headache types. Nausea, vomiting, hearing disturbances, diplopia, back pain, and dizziness are not uncommon associated symptoms. Although the exact cause of CSF leaks often remains uncertain, some patients may be predisposed due to disorders of connective tissue or spinal meningeal anomalies.

When a spontaneous CSF leak is suspected, head MRI with contrast is ordinarily the first study to obtain. Common abnormalities seen include diffuse dural enhancement, subdural fluid collections, engorged cerebral venous sinuses, and cerebral descent. Important to know is the fact that despite typical clinical features, head MRI may occasionally be unremarkable. In such situations, ancillary studies may help answer whether a CSF leak is present. Radioisotope cisternography and spine MRI are helpful tools in such occurrences. Presently, CT-myelography remains the most reliable test to find the exact spinal CSF leak site. Often, however, the exact CSF leak site is not found.

Read the full article here: http://www.headachejournal.org/view/0/CSFLeaks.html

Health: How spinal condition lead to crippling headaches


Struggling off the bus in agony, Deborah Ogg barely knew what was happening.

She had never suffered from headaches, but suddenly she was in such excruciating pain that she had to lie down in the street, utterly overwhelmed.

Her worried partner Peter looked on, helpless, and even the taxi driver he summoned offered to take them straight to hospital. But Deborah, 40, hating to make a fuss, insisted on going home to bed.

“I felt like somebody had hit me over the head with an iron bar,” she recalls, “It was massive pain.”

The pharmacist recommended migraine pills, but they didn’t help. “I tried getting up to go to the toilet but as soon as I got up the pain was even worse and I was on my knees, I just crumpled,” she says.

It was April 1 last year, and the start of a long and painful journey with what she eventually discovered was a rare condition called cerebrospinal fluid (CSF) leak.

Affecting around one person in 50,000, it occurs when the fluid, which cushions the brain and spine, leaks out through a hole in its protective sac. This results in a drop in pressure, so that the brain can no longer float in fluid.

The brain cannot feel pain, but the resulting “brain sag” pulls on tendons and muscles across the head, causing extreme pain, which is slightly relieved when sufferers lie down.

It is most common after epidurals or spinal operations, but in some cases, such as Deborah’s, occurs spontaneously, which makes treatment harder, because the location of the leak is not known.

Read the full article here: http://www.edinburghnews.scotsman.com/news/health/health-how-spinal-condition-lead-to-crippling-headaches-1-2150059