My Story of Cranial Neuropathies and Sjogren’s

I have put off writing this blog post a number of times.  To be honest, it's not something that I like to think about, a luxury I sometimes have when my symptoms are not particularly severe. But it is a subject too important for me to continue to put off.  So here goes.

I am one of the unlucky Sjogren’s patients who suffers from significant neuropathies; in my case multiple cranial neuropathies are the particular nemeses. In fact, for me, cranial neuropathies are perhaps the most difficult part of contending with Sjogren's syndrome. They can be exceedingly painful and are one the reasons SS has been so debilitating for me over the last several years.

While I know I am not alone in having these cranial nerve issues, I have also Iearned that, in some respects, I am the exception to the rule. For starters, depending on the study, approximately 30 percent of Sjogren's patients have neurological involvements of some kind. That broad group includes all neuropathies that impact the central, peripheral, and autonomic nervous systems. Multiple cranial neuropathies in particular are a relatively uncommon subset. One estimate places them at just 5% percent of those with neuropathies, which would mean less than 2% of the overall SS population.  (I’m sure others will have seen other numbers, but the point is multiple cranial neuropathies are considered fairly rare with Sjogren’s.)  

However, since at least I know I am not the only one out there with Sjogren's, Lupus, or other autoimmune diseases contending with cranial neuropathies, I thought I would start with some of the basics I have learned over the last few years.  For starters, there are actually 12 cranial nerves. Making things more complicated, some belong to the peripheral nervous system (PNS) and others belong to the central nervous system (CNS). The short version is that these are many of the nerves that are involved in your face, head, eye, and ear. (If you’re interested, greater details on the specific functions of the twelve cranial nerves can be found here.)

With Sjogren’s (and other autoimmune diseases such as MS) the 5^th cranial nerve or the trigeminal nerve is one of the most commonly involved in the disease process.  This nerve has three main branches V1, V2, and V3 impacting the upper, middle, and lower sections of the face as shown below:

Dermatone of Trigeminal Nerve
By Madhero88 via Wikimedia Commons

Patients with trigeminal nerve involvement or trigeminal neuralgia often have severe pain running down the side of their face, lack of feeling, sensations of numbness or general abnormal feeling in their face.  I realize that pain and numbness may sound contradictory, but it will make sense to anyone who has experienced it. Symptoms can be made worse by simple everyday activities that most people don't think about twice such as eating, being outside in the cold or wind, or even just touching the face. Unfortunately, trigeminal neuralgia is an extremely painful condition and can be difficult to control. Doctors recognize it as a condition of its own and often believe it to be untreatable; a terrible thing to hear for those of us who have it.

For me, the trigeminal nerve was the first of my cranial nerves to be affected by the disease.   It started with a change in sensation on the right side of my face such that touching it became uncomfortable. It was as if my face had gone numb and progressively painful at the same time.  Not a good feeling at all.

But that was just the start for me. After the trigeminal nerve, I had other cranial nerves become involved too.  I had problems with the 7^th cranial nerve, which impacted my facial movement and caused hemi-facial spasms.  As a result of its involvement, at times I was unable to move my face or open my eye.  My 8^th cranial nerve, which is responsible for hearing and balance, also joined the dysfunctional nerve party.  I experienced (and at times still do have) extreme ear pain, sensitivity to any sound and vibration, and balance issues.  I was even unable to be in a car or train because the vibrations and bumps of the road caused so much pain.  I was in constant unremitting pain and unable to do basic everyday tasks.  It really left me at my wits end… I could go on, but I am sure you get the idea that my immune system had mounted a full-fledged attack on my cranial nerves.

Now, back to the autoimmune aspect of it, which I hope may help some other patients. All of the nerve/pain issues described occurred before any of the more twenty doctors that I saw during this time diagnosed me with autoimmune disease.  If anything, the pain threw them off. I was told I had a pain syndrome and doctors put me on pain meds. All they did was make me slow-witted and, while they masked the pain at times, they did not make it go away and I kept getting worse. The doctor's response?  You must have a "bad pain syndrome. Take more drugs." This was from some of the most prominent medical centers in the country. 

I know that I am probably not alone in the autoimmune disease community of having had something like this happen.  What I have learned since then is that many doctors, even rheumatologists, are not aware of the possible neurological complications of Sjogren's Syndrome.  And the big and important question of course is why is this the case?  So that is what I’ll try to tackle in a bit more detail in an upcoming post.  Stay tuned.

I realize it's a very tough subject but I would be very interested to hear if any of you have neuropathies related to autoimmune disease. If so, how have they affected your life?

Sources

http://brain.oxfordjournals.org/content/128/11/2518.short

http://www.ncbi.nlm.nih.gov/pubmed/20827117

http://www.hindawi.com/journals/ad/2012/645967/

When a Generic is not Generic

Photo Source

Ah medicines, such a wonderful part of my life.  My latest saga made me find out about the possible hidden problems in switching between generics, of which I was previously unaware.

When I went to the pharmacy last month to pick up a refill of a prescription for oxcarbazepine, I didn’t initially notice that the generic brand the pharmacist gave me was different than the one I usually take.  Oxcarbazepine, or its brand name trileptal, is an anti-seizure medication that I take for autoimmune neuropathies.  (In my case, my cranial nerves or the nerves coming from the brain have been negatively affected by Sjogren’s and this medication provides some incomplete relief.)  Lucky me, I noticed the difference once I started taking this other generic form of the drug.  First, I felt particularly groggy and sluggish on the different generic. Then I noticed that the drug was just not working and my face in particular felt numb, and I was in more pain than usual.  In my opinion, being in more pain than usual for no good reason is unacceptable.

I went to talk to the pharmacist, but that provided little help.  I asked if he could order the brand that I usually take (manufactured by Cadista) and he said that "it was not available" and that the generic manufacturers of drugs change all the time.  He appeared unconcerned by my predicament, and seemed to regard the distinction I was making between generics as irrelevant. This didn’t sound quite right to me since I had been taking this brand of my medication for over a year...so I started making phone calls.  I got in touch with Cadista directly and was told, low and behold, that they were still manufacturing my prescribed dosage of the medication. 

Well, I then knew my neighborhood pharmacist not only didn’t take seriously the problems I was experiencing, but he was also wrong about what was possible.  I went the route of working with my neurologist and my insurance company’s mail order pharmacy.  The mail order pharmacy said that they would be able to order the Cadista brand of the medication and that either I or my physician could request if a specific generic brand was preferred.  Just to make sure that all my bases were covered, I asked my physician write the three month mail order prescription specifying the Cadista brand so that there would be no confusion!  As our own patient advocates, it is often a time consuming and frustrating process to get even what should be small medical details handled.

While sorting through all this to get the medication that I needed, I couldn’t help but think that other patients might have had similar problems switching between name brand and generic drugs or between generic brands. I already knew that generic and brand name drugs have the same active ingredients, but they do not need to have the same inactive ingredients.  My big finding was that the category of drugs, anti-seizure medication that I am on are a particularly tricky problem. The FDA requires concentrations of generic and brand name drugs in the blood stream are within a very close statistical range before they are approved.  However, what the acceptable deviation ought to be for some drug categories is much less clear.

First, it turns out there is a specific open question as to whether switching brands can cause an increase in seizures in some patients and whether the FDA standards for differences in blood level concentrations between brand and generic seizure drugs needs to be narrower for this class of medications (Some generic cardiac, thyroid, and anti-depressant medications have also been of concern).  Then, just to make this issue even more complicated, a recent study from the University of Connecticut and Hartford Hospital indicates that it might not be the difference between brand and generic drugs that can cause problems for patients, but the actual switching itself whether it is between generics or a generic and brand name drug.  Currently, the FDA has three studies in the works to look at these very issues with regard to the efficacy and safety of generic and brand anti-seizure medications.  So, not only was my pharmacist wrong about the differences between generics and the availability of my generic, but he was also woefully uninformed about the particulars of anti-seizure drugs.

I asked a few doctors and started reading online and found out that this was a real problem.  One of the major concerns that physicians have is that, similar to my experience, patients might not even be notified when they are given a new version of a generic anti-seizure medication when refilling their prescriptions.  Pharmacists are not required to give this notice.  Patients also have little control to remain on specific generic medication because pharmacies often change which brand they carry based on issues such as lowest cost and availability of supply. The American Academy of Neurology has put forth two position papers expressing “concerns with generic anti-epileptic medication and that physicians should approve all generic substitutions.”  This would mean that pharmacists could not substitute generic seizure medicines for a brand name without a doctor’s consent.  

Luckily in my case, I was not at risk of a seizure, but I did have adverse side effects from switching between generic anti-seizure drugs.  My take away from this experience like many of the other medical adventures that I have had is that I cannot always rely on medical professionals for help and to provide important information. And I have to trust my instinct when something doesn’t seem right with my health and press forward for answers.  While I do admit it can be tiring and sometimes I want a break, that is what having a chronic illness and being your own advocate requires. I don’t feel that I really have a choice in this one.

Have any of you similar problems with switching between generics or generic and brand versions of the same medication?  Are there other specific conditions that you know patients need to watch out for when switching?













Sources:
Cake Health. Are Brand Name Drugs Better Than Generic?
Epilepsy Foundation of Connecticut. Press Release.
FDA. Facts About Generic Drugs.
MedPage Today. Generic Seizure Drugs Vary Widely in Bioavailability.
MedPage Today. Generic Versus Brands: How it Plays out in Practice. 
New York Times. Not all Drugs are the Same After All.
ScienceDaily. Substitution of Generic Drugs May Cause Problems for Epilepsy Patients.
UConn Today. Study: Switching Anti-epileptic Drugs Could Increase Risk of Seizures.
Wall Street Journal. Inexact Copies: How Generic Copies Differ From Brand Names.