Tuesday, October 4, 2011

So, What's This CSF Stuff Really All About?

CSF, CSF, CSF...try saying (or typing, for that matter!) this three times in a row, and you'll probably find yourself switching the letters around.  I certainly do.

CSF stands for cerebro-spinal fluid, the liquid that surrounds the brain and spinal cord.  It's some pretty awesome stuff.  When it's appropriately contained by the dura, the special membrane surrounding the brain and spinal cord, it cushions and protects this extremely vital organ.  Jon's CSF, however, isn't staying put.  For reasons unknown to us, a hole developed in his dura somewhere up in his sinuses, thus breaking the needed pressure seal and allowing CSF to leak away from the brain. This creates myriad problems, from something as simple as congested sinuses to as dangerous as having the brain collapse on itself.  Without the steady pressure that a completely sealed dura creates, havoc ensues.

Jon's surgeons believe that he developed his CSF leak around age 15, but he lived with it undiagnosed until age 30.  His symptoms mimicked those of severe allergies, although he'd known for years that allergy medications, even prescription-strength, did nothing to ward off his intense reactions.  He also noticed that there was no pattern for his splitting headaches and and bouts of nasal discomfort.  He wasn't any worse during allergy seasons, and he felt no better once offending pollens diminished.

After L was born and we'd reached our out-of-pocket health insurance maximum, Jon decided to find out what was irritating his sinuses.  After allergy tests proved that he was not allergic to any known substances, the allergist labeled him with "non-allergic rhinitis," meaning his reactions had no known cause.

Dissatisfied with this answer, Jon asked if I could get him in to see an Ear-Nose-Throat doctor.  The Lord completely worked out all the details; usually, a patient has to see a regular doctor and then be referred to an ENT.  Since Jon's doctor had already referred him to the allergist who had given him a diagnosis, the chances of that first doctor choosing to override the allergist's opinion were slim.  However, the phone receptionist with whom I spoke pulled some strings and got Jon the ENT appointment without a referral.  Were we ever grateful for that!

Toward the end of the consultation with the ENT, this new doctor asked Jon if his nose leak tasted salty.  This one question changed Jon's entire course of medical care. 

CSF can have a salty or even metallic taste, we later learned.  Had we known that years earlier, Jon might have gotten help much sooner.  CT scans and MRIs showed that Jon had not only a severe leak but also brain prolapse into his sinuses.  Even with the severity of his case, Jon was very blessed to have had only minor headaches (some CSF patients are completely disabled by pain); to have avoided any bouts of meningitis; and to have experienced no mental distress even with the prolapse.  The Lord was protecting him even in the absence of needed medical care.

One reason that diagnosing Jon was so difficult is that CSF leaks are rare.  An estimated 5 in 100,000 people have them.  Of that number, the vast majority of cases result from trauma, such as a car accident or surgery, and of those that are spontaneous (meaning the cause is unknown), most are middle-to-late aged women.  So when a 30-year-old male with no history of trauma walks in complaining of a runny nose and headaches, CSF isn't the first thing surgeons look for!

As soon as Jon was correctly diagnosed, his doctors treated him as an emergency case even though CSF itself is rarely fatal.  Since it can easily lead to extremely serious situations, such as meningitis, it is nothing to sniff at.

At the time of his surgery in August '09, we thought that would be the end of his treatment.  What we'd not realized is that in cases of spontaneous CSF, the repairs often do not hold.  We heard of one patient who has had 14 repairs to date!

Currently, it is not completely clear whether Jon has another CSF leak or whether he only has blocked sinuses.  No brain prolapse showed up on his most recent MRI.  His upcoming surgery on October 24th will be out-patient surgery to clear up his chronic sinusitis, which is the same as having a continuous sinus infection.  The doctor gives him a 70% chance of staying clear after this surgery, so it's possible he'll need to have this procedure repeated from time to time.  While he is undergoing the sinusitis surgery, the surgeon will also do a visual look for another CSF leak since he has all the symptoms of such.  The difficulty lies in the fact that sinus problems and CSF symptoms are so similar that separating the two is often impossible.

Additionally, CSF leak diagnosis is relatively new.  It wasn't until the 1950s-1960s that doctors discovered cerebro-spinal fluid leaks as the culprit for patients with splitting headaches and allergy-like symptoms.  We're thankful that there is medical help available to Jon; 40 years ago, this would have been unheard of.

Certainly, this is not the most fun experience we've ever dealt with.  Even with the unpleasantness, however, we can clearly see the Lord working.  He helped us to get a diagnosis at the time when we could incur no further medical bills; He provided Jon with excellent surgeons; and He's shown us that even though this could be very dangerous, He's spared Jon from the worst scenarios. 

It would be easy to get caught up in the "what ifs" and unknowns of CSF.  But there is a lot to keep us grounded and thankful.  A friend of ours is going through a kidney transplant process.  Compared to the dangers and worries of organ failure, CSF is much, much less distressing.  And while Jon went through the barrage of CT and MRI tests, he witnessed many gravely ill patients.  He said it was a great reminder to be thankful for the health that he does have.  His symptoms, while irritating, are not consistent, nor do they occur on a daily basis.  It could even be called one of those "unseen" illnesses, since most people would never look at Jon and realize that he is sick.

While it is inconvenient and at times very frustrating, there are many positives - too many to remember or list.  We can witness the graciousness of God, even in something as unpleasing as a CSF leak.  It all depends on our perspective.  We choose to see the glass completely full:  full of God's mercy and blessing.  Life just doesn't get better than living under the protection of the Almighty God!


  1. Very well organized and explained, Julie! I'm sorry you and Jon have to go through this but I am thankful for your perspective, too. It is a good reminder for all of us. Praying for you all!

  2. This was very interesting, and a well explained, easy to understand description of his illness. I hope his next surgery goes well and that he continues to recover from this.

  3. Thank you, Julie and Lisa! It's hard to know how to explain some of this stuff, but wading through what little CSF medical research is out there made me realize that much of it is really hard to understand. His doctor has been really good to help simplify it for us. That's been very helpful, especially for me. :)