The Mighty Logo

What I Hope Doctors Will Recognize This 'Leak Week'

The most helpful emails in health
Browse our free newsletters

I have a spontaneous cerebrospinal fluid (CSF) leak. Spontaneous CSF leaks have long thought to be extremely rare, but as diagnosis and treatment of these leaks improves we are discovering that these are not as rare as once thought. CSF leaks happen when the dura (the tough layer that holds the CSF around the brain and spine) is punctured or torn. When the dura is torn/punctured CSF fluid leaks out, the brain which is typically suspended/floating in CSF, begins to sag downwards into the spinal column and can cause a wide variety of symptoms. Most commonly (but not always) it causes headaches that are worse when upright. Connective tissue disorders such as hypermobile Ehlers-Danlos syndrome (hEDS) makes the dura more fragile and susceptible to tears and breakage and is likely why I developed this condition.

While spontaneous CSF leaks may not be as rare as once thought, doctors who are able to diagnose and treat them are. Many doctors incorrectly assume that spontaneous CSF leaks present the same and can be treated the same as a post-dural puncture headache (PDPH). PDPH happens then a CSF leak is caused by a medical procedure the results in a puncture to the dura (like a lumbar puncture) that does not heal and results in a leak. There are subtle differences between these two different causes of CSF that many doctors miss. While both typically present with headaches that is worse when upright, a PDPH typically has a headache that begins immediately upon being up upright; whereas the spontaneous CSF leak can take longer to appear after being upright, typically beginning hours after being upright. This leads many doctors to dismiss the idea of a CSF leak in a patient who doesn’t get a headache immediately upon standing. Resolution of the headache upon laying down is also typically instantaneous with a PDPH, but can take longer spontaneous CSF leak. To further complicate matters, the headache in a spontaneous CSF leaker can lose its positional component completely after time. Fortunately, there has been some progress with educating doctors on these differences, but there needs to be more, especially when it comes to the differences in treatment.

While both PDPH and spontaneous CSF leaks can be treated with an epidural blood patch, an injection of the patient’s own blood into the epidural space to help encourage the dura to heal and seal the leak, the approach in the case of PDPH vs spontaneous CSF leak needs to be different. With a PDPH the location of the leak is well known and the blood patch can then be targeted to that area; with a spontaneous CSF leak the location of the leak is often unknown and can be located anywhere along the dura. Finding the location of spontaneous CSF leaks is notoriously difficult and the testing that is needed to find these leaks is invasive, so it’s not uncommon for doctors to empirically treat patients with presumed leaks with a blood patch before doing invasive imaging. This blood patch cannot be targeted to the leak site, so it needs to be done in a way that covers as much dura as possible to increase the odds of covering the leak site. This is where education and information is severely lacking and needs to be addressed.

March 4th-10th is Leak Week, a week aimed at showing just how many people are affected by CSF leaks and educating people on what they are and how they are treated. Doctors today are much more aware of the differences between a PDPH and the headache caused by a spontaneous CSF leak, what they are not aware of is the difference in treatment approaches that should be considered. While I will be joining the ranks of other leakers on Facebook who are donning purple hair to celebrate Leak Week, I am also hoping for something more. I want to see more physicians empowered with the knowledge on how to treat spontaneous CSF leaks. In addition to learning more about leaks ourselves this week and educating family and friends, I’d like us to also reach out to doctors who could be our allies in treating spontaneous CSF leaks and get them engaged and interested in treating leaks.

This became a priority to me after a disastrous blood patching attempt for my spontaneous CSF leak. If there was an established guideline or protocol on blood patching for spontaneous CSF leaks, my experience could have been avoided. I would have had better odds of the blood patch sealing my leak and it could have prevented additional procedures or invasive testing. Sadly, this doesn’t seem to exist.

A quick internet search can provide one with many different guidelines on how a blood patch is performed at various institutions, but there are none addressing the differences that should be considered when patching for a spontaneous leak, when the leak site is unknown. Currently it can be very difficult to get the type of patching needed outside of one of the three CSF Leak Programs in the US located at Duke University Hospital, Cedars-Sinai Medical Center and Stanford University Medical Center. My hope is for other medical centers to learn how to do this. The quickest way to achieve this is to have an established protocol or guideline for other centers to follow.

Currently, those three centers are overwhelmed with the number of patients contacting them, needing treatment and/or diagnosis for a CSF leak. These centers are publishing amazing papers on complex and difficult to treat leak cases, but they need to go back to the basics. Let’s get some basic, but detailed treatment guidelines published with specific protocols for procedures like blood patches to help empower other doctors to treat spontaneous CSF leaks. Doing this would not only help patients everywhere who are struggling to find competent care for their leaks, but it would also allow these leak centers to focus on the truly complex and difficult to treat cases. I strongly feel that this is something that can and should be done and therefore I am not only leaking CSF, but I am also leaking hope.

I hope for guidelines and protocols that my doctors can follow while empirically blood patching me and others to give us the best chance of a cure.

I hope for guidelines and protocols to help save me and many others from invasive imaging to detect the leak location.

I hope for guidelines and protocols that allow me and hundreds of others diagnosed with spontaneous CSF leaks to be treated locally, surrounded by our support systems.

I hope for guidelines and protocols that give all doctors the knowledge and confidence to identify and treat spontaneous CSF leaks.

I hope for guidelines and protocols that allow CSF Leak Programs to focus on the complicated cases, so they can be seen quickly and efficiently. This could help decrease their patient loads as more and more doctors are able to treat the uncomplicated leak cases.

Today, I may still be leaking CSF but I am also leaking hope, hope that change and education will lead to stopping my CSF leak. Hope is power, hope is freedom, hope is strength…. hope is something I am proud to be leaking.

Originally published: March 9, 2018
Want more of The Mighty?
You can find even more stories on our Home page. There, you’ll also find thoughts and questions by our community.
Take Me Home