My CSF leak journey began in November of 2013.
Prior to my first cranial leak, life was typical and moving swiftly. I was born and raised in Colorado with a love of the outdoors. I grew up downhill skiing in the winter, playing soccer in the spring and fall and spending the summers at my family’s mountain cabin. As an adult, life still included downhill skiing and summers in the mountains plus working 40, 50 or 60 hours a week. I was also busy with family, friends and typical home life.
That fateful day, in November 2013, I was sitting at my desk and there was this drip. And another. One drip after another followed slowly like a drip from the faucet, clear fluid was dripping from my left nostril. A phone call to my primary physician determined I had “allergies” and I was to start an over-the-counter medication to “clear it up”. The thing is, I never had allergies one day of my 46 years.
My leak started the week of US Thanksgiving. I woke up on Thanksgiving morning, 4 days before the initial start of my CSF leak, feeling just terrible – pounding headache and chills. I was so cold I was wearing sweats, I climbed in my flannel sheets and had four blankets and two comforters on top. I could not for the life of me get warm. Well, being sick today of all days was not acceptable. We were to be at my brother’s house for a huge family dinner. I arrived and immediately took to his couch and laid down. He asked what was wrong, I said I didn’t feel good and had fluid dripping from my nose. And he said, I will never forget this, “are you leaking brain juice?” I know he was only being funny, but I never thought I was leaking “brain juice”. The next day I called my doctor, and since they were closed for the holiday, they sent me to the emergency department to determine what was going on. I arrive at the ER (ED) and they ask what was going on. I said, “I can do this”, as I leaned forward and fluid poured out my nose. Immediately I was rushed back to a room where I was to collect the fluid and wait endlessly. The Beta 2 Transferrin test came back positive for CSF. An MRI and CT scan were ordered. The radiologist could not see the leak on the imaging, and I was to follow up with the neurosurgeon Monday to discuss surgery.
The neurosurgeon believed my leak was a tiny crack in the bone near the left orbital bone and cribriform plate. He said the way to fix the leak was to do a craniotomy. He would cut me ear to ear along my hairline, pull my face down to my eyes, remove the bone over my forehead and fix the leak. I must have looked like a deer in headlights as I sat there thinking “no way are you doing THAT to me!”. As we sat and talked, he said there might be an alternative. His partner had done some work with a local skull base ENT who may be able to get to the leak through my nose. Hallelujah! It was decided Dr. Watts would consult with Dr. Edward Hepworth, an ENT located in Denver who had success performing endoscopic cranial repairs. Repair surgery number one followed a couple of weeks later.
Silly me, I thought life would return to normal once again. Hey, I bounced back from cancer surgery and treatment a year prior. Why not a simple repair up my nose? What followed were months of debilitating high-pressure headaches. The likes of which diamox and methazolamide had no intention of touching. The ensuing months included tests, doctor’s appointments and various studies to decipher my new pressure issues.
In May 2014, I bent forward only to have another gush of fluid pour out my left nostril. I waited patiently for Dr. Hepworth to tell me what I already knew, I had my second cranial leak. Five pin holes above my left eye. Dr. Hepworth presented my case to a group of neurology doctors (neurologists, neurosurgeons, etc.) that I refer to as the “head case clinic”. Those doctors suggested an echocardiogram with bubble test to look for a hole in my heart and an MRV to look for venous stenosis in my head. They wanted to make sure there wasn’t a rational reason for two spontaneous cranial leaks in less than six months.
With nothing noted as suspect, repair number two followed in August of 2014.
After surgery, unrelenting and unforgiving head pain/pressure returned. Again, not to be touched by traditional medications. An MRI in December 2014 showed the beginnings of a third cranial leak. Dr. Hepworth said “I am not patching you every six months, you need a VP shunt”. It was all I could do, to stand up, walk out of the office and get to my car. I sat there in the dark parking lot for ten minutes crying and fully heartbroken while thoughts flooded in – thoughts that this illness was getting the best of me and life would never be the same again.
At the time we decided I needed a shunt, Dr. Watts was moving out of Colorado. So, Dr. Hepworth referred me to a wonderful neurosurgeon. He spent an hour with me explaining the surgery, the purpose of the shunt (not to stop the headaches but lower the pressure in hopes it ends the head pain), and the pros and cons. Then he said to me “Go home and think about it. Call me when you are ready to move forward.” It took about a month for me to decide that a shunt was my best and next logical step. In February 2015, my VP shunt was placed.
I awoke from surgery pain free for the first time in more than a year. I never knew how much pain I was in until the pain was gone. Finally, life could return to normal. Or so I thought.
I eased back into life including a return to yoga and other activities.
In December 2015, I was once again complaining of low-pressure headaches. Based upon my history, Dr. Hepworth decided a 48-hour pledget study with cisternogram images at certain time intervals was necessary to find the cranial leak. Results came in, and surprisingly, no cranial leak. But it didn’t make sense, I was suffering with low pressure headaches. Adjustment of the shunt wasn’t possible as an increase in CSF caused tremendous intracranial pressure. So, a full spinal MRI series was ordered.
The MRI revealed I had 30 perineural cysts (Tarlov Cysts) “up and down my spine”. A referral was made to a neurologist who said “…it would be difficult to determine which, if any, cysts were leaking for a blood patch.” As we know, most spinal leaks are difficult to see on images. We discussed that, although there were local doctors who could do a blood patch, with the extensive number of cysts and no idea of where the leak was located, a referral to Duke would be the best course of action.
It was then I decided I just didn’t have it in me to battle spinal leaks. I had given all I had over the last two plus years fighting cranial leaks. Opening this can of worms was just too much. I decided that I would take time and try to live the best, “normal” life possible.
After a year, I could no longer take the increasing pain, particularly the low-pressure or hypotension headaches, feeling as if my brain was being pulled into my spine. I was finding it way too hard to do anything physical with the hypotension, shoulder and mid-back pain. Work was becoming an issue as I was missing more and more days. Time with extended family and friends was becoming non-existent.
I consulted with a local Interventional Radiologist who didn’t believe that a blood patch was what was needed and maybe “adjusting the shunt” would fix all things low pressure. Well, we knew that I could not go higher on the shunt as too much CSF in my head just put way too much pressure on my thinned forehead and sinuses. Dr. Hepworth and I talked and decided a referral to Dr. Schievink (Dr. S.) and Cedars Sinai was in order. I figured a neurosurgeon would understand the complexities that had become my leak journey, including 2 cranial leaks, a VP shunt more than one interventional radiologist. So, off to Cedars and Dr. S I went.
Treatment at Cedars was scheduled for early May 2017. Although the Cedars imaging didn’t reveal any spinal leaks, Dr. S decided that based upon my symptoms, a double patch in the thoracic and lumbar regions was appropriate. The first patch consisted of 22 cc of blood between the two areas. This patch was successful for roughly 24 hours. Five days later, a second patch with 41 cc of blood between the two regions was completed.
Hallelujah! For the first time in years, I was completely pain free – head and spine. The blood patch had worked.
During my consultation with Dr. S, I mentioned that I had been practising yoga with a private instructor since my shunt had been placed in 2015 and I told him I really wanted to return to yoga after the blood patch. Dr. S said that if the patch didn’t work, I could start yoga in one month and if the patch worked make it two months.
I returned to yoga in July of 2017.
Shortly after my visit to Cedars, I followed up with my neurologist and the interventional radiologist who was against another blood patch. My neurologist had been treating my occipital neuralgia and other issues. It was at this time; he mentioned a possible connective tissue disorder and Ehlers-Danlos Syndrome. A what and what? I had never heard these terms. After much research and discussion, I determined that a suspected EDS diagnosis was realistic.
My appointment with the interventional radiologist was interesting to say the least. He was shocked that not only did Dr. S agree to a bi-level blood patch, but he approved two less than a week apart. I think most shocking to him was that the second blood patch worked and my symptoms resolved. He asked for the notes and a copy of the imaging from Cedars so he could study and learn. I told him that I was only following up with him because he discounted me and my needs. He had treated me as if I didn’t understand what I was feeling and what was going on with my body. I wanted him to know that we are not textbook cases and do not fit into a doctor’s preconceived “box”. We are all different, sure our issues are similar, but what helps me may not work for the next. But that doesn’t give doctors a licence to dismiss the person sitting in front of them. I said “I am only here so that you understand how a leaker presents and I hope you help the next person like me.” In follow up consultations with the interventional radiologist he admitted he didn’t know enough about spinal leaks and blood patches and attended a symposium at Tufts Medical Center. I was thankful for the effort he put forward to learn more about such a debilitating condition.
In late July 2017, I was rear-ended, sending my head, neck and spine forward and then slamming back into the driver’s seat. Post concussive headaches followed. Imaging showed the shunt was intact. But I was back to dealing with frontal forehead pain, neck and back pain. Physical therapy was ordered to help resolve some of the pain. But, in November a sneeze brought fluid dripping out of my nose and my third cranial leak was diagnosed. Surgery in December 2017 revealed and repaired active leaks on both left and right sides of the ethmoid roof of my skull.
I was sealed cranially, but still battling with pressure issues. As a result, I sought another blood patch in August 2017. Fortunately, there was a doctor in Denver to perform the patch and I didn’t have to travel to Cedars Sinai again. The blood patch sent me into unforgiving high pressure for several days until my system figured it out. But it seemed to work and provided the relief I sought.
Update on Denise's experience
Fast forward to December 2018 and I was still not finished with blood patches. The local interventional radiologist agreed to perform a blood patch in December 2018. He was able to push 42 cc of blood into my spine. Once again, I was pretty much functioning with some pressure issues here and there. But I felt really good for once!
In March of 2019, I was sitting in Dr. Hepworth’s office as a massive thunderstorm was pushing its way over the Rocky Mountains. Due to the elevation of the Rockies, with several mountain peaks more than 14,000 feet above sea level and Denver at an elevation of 5,280 feet above sea level, there are tremendous barometric pressure changes anytime a storm blows into Denver. I thought my head would explode.
Dr. Hepworth saw the pain I was in, my eyes bulging slightly. He knew I was struggling. I also told him that my sister had taken me to the local ER one night in February as my left jugular felt as if it would explode and I had to keep my hand on the vein and hold my neck in a certain position to stop the pain. The ER diagnosed it as a “muscle strain”. Sure, I strained my neck and jugular vein!
Based upon that information, Dr. Hepworth ordered a CT scan to look for something called Eagle Syndrome. He believed a jugular compression in my neck was causing the high pressure. The CT scan revealed that my right jugular was “nearly fully occluded” and a system of collateral veins (many new veins) had formed in my skull to take the blood back around to the other jugular. The left jugular was also impacted but not as severely as the right side. It was then, I was diagnosed with bilateral Eagle Syndrome.
A follow up with the vascular surgeon was arranged to get pressure gradients and an idea of the vast collateral network in my head. As I laid there on the table, Dr. Garland injected the dye into the catheter. I watched the dye light up a network of veins that looked like a spiderweb and I thought “hey, that is really cool”. But then, I realized that is my head and it isn’t supposed to be this way. The first surgery to fix this, a styloidectomy, was scheduled for September 2019. The best news ever followed…the jugular had opened right up and the collateral vein network collapsed. But even better, I was able to push my shunt from the low of 100 (10 cc of CSF) to 150 (15 cc of CSF – where the normal population functions). Something finally worked!
Following surgery, I was still dealing with tremendous pain in the left side of my neck. Dr. Hepworth sent me back to the vascular surgeon to determine if the pain was vascular or muscular. Dr. Annest completed an exam and injected nerve blocks into the upper and lower pectorals and into the scalenes. Dr. Annest said that I had Thoracic Outlet Syndrome (TOS) on the left side of my chest. Dr. Annest sent me on to physical therapy to assist with the muscle pain and tightness. The physical therapist was performing my intake exam and said “oh, you have TOS bilaterally”. Great, just what I needed.
Four months of physical therapy followed with the answer that it wasn’t doing any good and I needed to have the left styloid process removed as well.
Opening the neck congestion and raising my shunt setting all contributed to additional low spinal pressure. The interventional radiologist refused to do another patch. He said that four blood patches was “too many” and let's “just adjust the shunt”. My neurosurgeon of five years argued, “it isn’t the shunt!” But to no avail.
Another doctor was sought out to do the blood patch. A fifth and final blood patch was performed in August of 2020. Talking with Dr. Preece, he asked what my last patch consisted of. I said 42 cc of blood. He replied there was no way to get that much volume into the area. So, he pulled the report from my last blood patch. When I arrived in the treatment room, he said, “yes, you did get 42 cc of blood.” I will push as much as I can. He was going to stop at 20 cc and I’m so glad he was able to pull the other report and confirm I needed a higher volume. We stopped at 30 cc of blood.
Right now, I am debating the need for another blood patch. Some days it feels as if it is what is needed and other days are fully productive with gardening, walking the dogs and other activities. It is a very delicate dance we CSF leakers perform – keeping our systems in balance.
After nine long years, I continue to see Dr. Hepworth and a whole host of other doctors. I feel truly blessed to have an amazing group of doctors on my care team. My CSF journey has been comprised of a lot of little pieces, pieces I didn’t even know existed, and Dr. Hepworth has worked diligently helping me gather the pieces.
I tell folks in the CSF groups, both spinal and cranial, to be your own best advocate. No one will fight harder for you, than you. Educate yourself and be ready for a fight. Also, hold on to hope. At the end of the day, all we leakers have is hope. Hope that we find a doctor caring and knowledgeable enough to treat us and that the treatment works.
I am not going to lie, the nine years I have spent dealing with all things leak related has been one heck of a road. But along the way, I have met some of the best doctors and friends, but best of all, my leaker family.