Pediatric TN Issues: Four Key Points

As we have mentioned, this site is administered by four TN Mamas:  Tracy, Meg, Stacie & Ang.  We all came together because we all have kids with this awful condition.  Different pairs of us met just from having kids with TN, but the four of us really came together and bonded and found this purpose because all four of us made it to the same neurosurgeon.  Because of that, the four of us now have kids who are currently TN pain free and medication free.

We don’t want to make this site all about him, and while he knows about the site and ‘follows’ us (Hi!), he doesn’t want it to be about him either.  We all realize that Facial Pain conditions are complex, and that MVD isn’t the end-all be-all answer for everyone.  We also realize there are other neurosurgeons out there.

However, Dr. Mark Linksey, while not a ‘pediatric’ neurosurgeon, has done wonderful things for our kids and many others struggling with TN.   Since first working with TN patients during his residency under the creator of the MVD, Dr. Peter Jeanetta, he has continued to study TN, and is extremely knowledgeable about it and other facial pain conditions.  He is also a member of TNA – The Facial Pain Associatrion Medical Advisory Board.

So, following is a piece written by Dr. Linskey about what he feels are the four key points that need to be ‘out there’ and discussed for pediatric TN patients.

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1)      Traditional US Medical Healthcare training, structure and Culture

In the US Adult Neurologists go through four years (48 months) of training in a neurology residency right out of medical school. However, “pediatric neurologists” do not do a neurology residency.  They do a 3 year general pediatrics residency followed by a 1-2 year “pediatric neurology” fellowship which usually only includes 3-6 months of adult neurology exposure and training, and in some programs includes none at all.  Since less than 1% of TN occurs in patients under the age of 18, most pediatric neurologists do not see a case of TN in their career.

Furthermore there is an assumption inherent in patient referral patterns that children are better cared for by “Pediatric Neurosurgeons” than adult neurosurgeons.  Most pediatric neurologists preferentially refer surgical patients to “pediatric neurosurgeons” despite the fact that even if they were trained in TN surgeries like MVD during their residency (all neurosurgeons go through similar  7 year residency training), given the rarity of the syndrome in pediatric patients, their ongoing surgical experience in TN surgeries may be minimal to none.

What is needed is referral to a neurosurgeon trained and experienced in TN surgery who is comfortable operating on children (whether they are predominantly an adult neurosurgeon, or one of the extremely rare pediatric neurosurgeons who operates on adults with this condition)

Patients and their parents get trapped within this system.

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2)      The problem of out-of-state subspecialty referral for rare or “orphan” pediatric diseases under State Medicaid and perhaps now even under Affordable Care Act individual State Exchange insurance policies as well –

If patients have good primary third party insurance, referral out of state to a neurosurgical TN subspecialist is usually not a problem. However, according to the CMS (Center for Medicare and Medicaid Services) 2012 annual report, in 2011 43.5 million of the 73.9 million children age 0-17 in the US received their healthcare services through MedicaidThis is 59% of all children in the US. These children represent 50% of all patients enrolled in Medicaid across the US (the rest are adults either under a poverty line or permanently disabled).

Unlike Medicare which is federal and is portable state-to-state, Medicaid is individually run by each state and there are significant barriers to approving care outside of the state, as well as barriers to out-of-state hospitals accepting another state’s Medicaid reimbursement rate. This problem appears to also be developing for health insurance plans being offered and sold on individual state health insurance exchanges under the new Affordable Care Act (ACA).

Depending on the size and location of the state in question, there may, or may not, be a very experienced TN subspecialty neurosurgeon who is comfortable operating on children in their own state.  These barriers to out-of-stet referrals then can become significant barriers to access and quality of care.

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3)      The unique problem of high-dose anti-epileptic drug use in children as opposed to adults (this is not “benign” treatment”) as well as TN in children –

TN patients are initially treated with one, or more anti-epileptic drugs (AEDs).  The doses of these AEDs needed in TN is usually orders of magnitude higher than doses usually used to successfully treat epilepsy. Unfortunately these drugs are not selective.  They have general electrical suppressive effects throughout the brain.  In children the brain is still developing and the effect of these drugs at these doses are potentially much more severe than in their adult counterparts.

These drugs at TN doses interfere with learning and memory during the key learning period in life. This interferes with their grades and impairs their competitiveness for higher educational and career opportunities.  They are general suppressants and they interfere with successful peer socialization and interaction at a time when adolescents are developing their social self confidence, and a feel for their place in society.  If the child is young enough, the may even be erroneously diagnosed with ADHD (Attention Deficit Hyperactivity Disorder).

Even the syndrome of TN by itself is potentially devastating for adolescents from a socialization perspective, particularly in adolescents.  Imagine being afraid to have someone touch your face or kiss you if you are an adolescent (especially girls) and the effect that would have on you socially at this vulnerable time. Many even withdraw and become almost social recluses.

Getting them off these meds and pain-free and allowing them to learn, compete, professionally track, socialize and gain personal self-confidence is a crucial need.

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4)      The importance of finding the right surgeon – In my opinion the TN subspecialty surgeon needs to be very well trained and experienced.  For MVD surgery this very specifically means that the surgeon in question:

A)     Has been trained by a well-known and established master in the technique (not picked it up on their own or trained by someone who is not a known MVD expert)

B)      Has overcome their personal learning curve for the procedure which for MVD, in my opinion, is at least 50 cases.

C)      Has an ongoing regular surgical volume to maintain outcome excellence and keep complication rates as low as possible (studies suggest 6-24 MVD’s per year)

D)     Only operates with experienced intra-operative ABR (auditory brainstem evoked response) monitoring to minimize hearing loss risk

E)      Works with an intact experienced team including neuroanesthesia, neurotechnicians experienced with shredded Teflon felt and microsurgery, a dedicated neuro-ICU and neuro nurses

F)      Ideally is analyzing and publishing their own surgical results in the peer-reviewed literature for comparative purposes

That last point, I (Meg) need to emphasize, and I believe the other TN Mamas will agree.  Two of us (Ang & Meg) came to Dr. Linskey for re-dos after our child’s first MVD failed.  MVD, to someone who is an experienced neurosurgeon, is not a super complex surgery, but it is delicate and ‘elegant’.  There is also important information, like the possible location of compressions, that is not widely known by those who do not practice this surgery regularly.  And MVD is something you want to get right the first time.

Stacie has also developed a more in depth questionnaire that she used for neurosurgeons here.

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I hope you all find this helpful.  These are points that have to become part of the discussion for pediatric – and for most of the points all – TN patients.

Pediatric TN Challenges: “Doesn’t Happen In Kids.”

This makes many of us TN parents absolutely insane.  Especially those of us whose children were finally diagnosed correctly and then treated (at least somewhat) successfully.  We know the damage that can be done to our children and families when we keep running up against incorrect diagnoses.

All the literature says that Trigeminal Neuralgia occurs more often in women, but occurs in men also, usually in patients over the age of 50, but can occur at any age.  Even in children.  However in practice, many doctors, including some of the top neurologists and neurosurgeons for Trigeminal Neuralgia, will just say that your child cannot have Trigeminal Neuralgia – because they are children.  One of our TN Mamas was told “I’m not sure what is going on with your child, but it’s not TN, because that doesn’t happen in children.”  Another was told, “If your child has Trigeminal Neuralgia, I’ll eat my belt, because TN doesn’t happen in children.”  (My emphasis added.)  And these docs are at top medical institutions in the country.  And just FYI, both of these children were later diagnosed with TN, and had successful MVDs.

For some doctors it’s just a matter of educating to show that this isn’t true.  Show them this site, show them the Facebook sites “Children with Trigeminal Neuralgia” or “Teens with Trigeminal Neuralgia”.  But for others, it just doesn’t matter.  You can show them children who are text book Trigeminal Neuralgia, and that won’t change their minds.  Show them children who had the pain, were given the anti-seizure meds prescribed for TN, and had their pain stop or suddenly become manageable.  Show them kids who had MVD and have had their pain stop immediately after surgery and not return – and the doctors will still say you cannot have Trigeminal Neuralgia if you are a child.  Because of this, parents will be sent on a wild goose chase of diagnoses that are never quite right, with treatments that do nothing to help.  It also means these doctors eventually conclude nothing can be done for this pain, or it’s just a psychiatric issue, and the child must learn to live with the pain.  Yes, I know it feels like you’re being stabbed in the face with a carving knife non-stop for hours.  I’m sorry, you’ll just have to learn coping skills.

If you have a doctor telling you your child doesn’t, or “can’t” have Trigeminal Neuralgia – maybe they are right.  (Stay with me for a moment…)  There are many facial pain conditions.  If you are new to this journey and/or your child’s symptoms are not quite lining up with Trigeminal Neuralgia, it’s possible you are dealing with something else.  It is important to not get too attached to the “TN” label, or any other label for that matter if it is not quite right, because the treatment will never fully work if the diagnosis is wrong.

It is also possible that your child is dealing with a combination of conditions.  It seems many of the kids we deal with have a combination, like our daughter who had Trigeminal and Geniculate Neuralgia (GN).  Classic (type 1) Trigeminal Neuralgia is caused by compressions on the Trigminal Nerve, while  her Geniculate Neuralgia was caused by compressions on the 7th and 9th cranial nerves. Another of our kids had compressions on cranial nerves 5 through 10 – what a mess!  If you child’s anatomy leans toward having complicated vascular structures, it is easy to see how they could have both TN and GN, or other cranial neuralgias caused by compressions.  However, some doctors will realize some of the symptoms your child is suffering from are not from TN, and just stop there, insisting they therefore do not have Trigeminal Neuralgia.  The doctor will not consider the possibility of a combination of these conditions.

If you believe your child may have Trigeminal Neuralgia, but a medical professional is insisting s/he does not, ask what of your child’s symptoms are indicating a diagnosis besides Trigeminal Neuralgia?  And what would their best guess of a diagnosis for those symptoms be?  If they cannot tell you that, or what they tell you does not line up with current TN literature, get a second or third opinion.  At least.

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Some resources you can arm yourself with for these conversations include…

<CLICK HERE> for information from the Facial Pain Association

<CLICK HERE> for John Hopkins Hospitals information on Trigeminal Neuralgia

<CLICK HERE> for National Institutes of Health information on Trigeminal Neuralgia

This PDF:  Differential Diagnosis Orofacial Pain for a very thorough breakdown of different facial pain conditions.  Thank you Dr. Hegarty & Dr. Zakrzewska!  Sorry for small type, many pages, and big words (honestly, don’t try to read this at 1:00AM) but if you can grab your dictionary and wade through there is some excellent information here.

I would love to include a reliable source of statistics that show the occurrence of TN in children, but honestly, I’ve not yet found any.  (If I do, I’ll edit this!)  There are individual reports of cases of pediatric Trigeminal Neuralgia, and a paper written here and there about a child or a very small sampling, but not much beyond that.  Truth be told, Trigeminal Neuralgia is rare.  And it is said that less than 1% of those diagnosed with Trigeminal Neuralgia are pediatric.  Sadly, I can name over 20+ kids easily because of the work we 4 TNMamas are doing.  But there’s not much literature out there for us.  Yet.

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An accurate diagnosis is crucial for successful treatment, so be open to new information.  However, be sure that the diagnosis you are getting – or ruling out – is being done on the basis of symptoms, not an inaccurate preconception, or dated knowledge.

Wishing you all the best, and pain-free days ahead.

Pediatric TN Challenges: Doctors

I hate to put this challenge so close to the top of our list, but it has to be done.  I know there are some absolutely wonderful doctors out there – with some effort, persistence, and TN Mama networking, we found one we will always be thankful for. Due to the rarity of Trigeminal Neuralgia, many adult patients have a very tough time finding a qualified team of doctors to work with.  Pediatric cases of Trigeminal Neuralgia are < 1% of the diagnosed cases of TN, so you can do the math on how much harder it is for the kids.

Is the very, very, VERY rare pediatric doctor who knows much beyond a quick mention in medical school about Trigeminal Neuralgia.  So, what they know is often restricted to a text book or mention in a lecture.  And who knows how long ago that happened and how dated that information may be.

Let me clarify – I am not saying this to be overly critical of these doctors.  The overwhelming majority of pediatric doctors will never see a case of Trigeminal Neuralgia, so we really can’t have the expectation that they are going to know the most complete and up to date information on symptoms, diagnosis, imaging, and treatment options.

However, there are several areas where this causes real problems:

One, if you have a pediatric doctor that is not working to come up to speed on the current medical information with you.  Some doctors know what they know, will treat based on that, and aren’t going to invest the time to look for other alternatives or better information for you.  Each of us TN Mamas tends to be forced into the position of educating ourselves and coming to the doctor with specific medication questions, options of treatment, etc.  (Which is one reason we started this site!  Check out www.fpa-support.org and www.facingfacialpain.org also.)  If your doctor consistently does not know information you need and isn’t willing to help you find it, or has no interest in research you have done and your questions – find a new doctor.

Two, many of the non-pediatric doctors – who actually know this condition and have experience working with it – just won’t take a pediatric patient on.  This is true for neurosurgery, other specialties, and especially when it comes to neurology.  I’m not sure of the reasons for this, but my guess is it has something to do with medications working differently in pediatric cases than adults, and dosage adjustment concerns.  However, when you are dealing with TN, your neurologist is your partner in keeping your child’s pain managed, which often becomes the center of life.  The frustration level of working with a neuro who isn’t up on the latest medications and medication combinations – which become increasingly important and complicated – is immense.  And it is worse when you know there are doctors who understand this better, but they will not see your child.  While I want a doctor to be honest with me if they do not feel comfortable treating my child, it can be infuriating.

Three, even doctors who have treated a good number of TN patients, may not have treated enough pediatric patients to know about some of the issues specific to them.  Really, finding a medical professional who has treated enough TN patients to be considered somewhat of an expert, and who has treated enough pediatric TN patients to know the issues specific to them is an extremely difficult thing.  They are the magical unicorns of the pediatric TN world.  It is a combination that is nearly impossible to find.

Finally, unfortunately many times now, insurance companies decide whether you can see a certain doctor or not. So, even if you have found a non-pediatric doctor who is willing to take your child on as a patient, and who has treated enough pediatric cases – the approval process with the insurance company just may not come through.  Yes, this makes you want to hit someone, scream until you are blue in the face, and basically throw a temper tantrum until someone listens to you.

Our best advice on this issue, is persistence.  Remember to be someone the doctor you are pursuing would want to work with.  Polite, but firm and persistant.  Tug the heart strings.  Explain your situation. Call.  Call again.  See if you can get an appointment for a consult or a discussion “to learn more about TN” if they are hesitant to take on your child as a patient.  Enlist your pediatrician or whatever doctor will consider it, to write a letter/email or make a call on your behalf.  If it is the insurance company refusing, see if the doctor who is willing to see you and your pediatrician or primary care physician would write letters on your behalf to the insurance company.  Write a letter/email yourself explaining why you want to see a specific doctor and why it is important for your child to have treatment from a medical professional with sufficient knowledge and experience, regardless of the “pediatric” title.  Call whoever says no at the insurance company, and ask (politely but firmly!) who their supervisor is.  Repeat this to the boss.  Never Give Up is the motto of so many of us.

It’s really exhausting, I know.  It seems overwhelming, and sometime, yeah, it is.  When you hit your wall, it’s ok to fall apart, get mad, cry, throw something, nap.  Then get up and keep going.

We’re sorry about this, and we are hoping it starts to change.  But we know you are the best asset your child has right now, and you will leverage that to give them all they need.  You will get there.

Love, Hugs, & Hope, from us to you.

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This photo is of 6 of our TNKids, with our personal favorite team: neurosurgeon Dr. Mark Linskey, and his PA Wendy, of UCI Medical Center.  All 6 kids are post MVD and doing well.

Pediatric TN Challenges: Communication

Having Trigeminal Neuralgia is painful and overwhelming and hard beyond the English language’s ability to describe it.  Someone with TN, in my experience, often does not even try to really communicate the depth of the battle that it is, so I can certainly not explain it sufficiently.

If you child has Trigeminal Neuralgia, they are going through that fight on a daily basis.  On top of that, kids have some unique, and incredibly frustrating challenges when facing this condition that is considered an “Over age 50” affliction.

Here we are going to discuss some of those challenges that need to be taken into consideration, and even expected at some level.  As much as some of them will make you want to scream at someone, or hit something, we need to realize these things happen – and be ready to respond or compensate.

This is the first installment topic, more will follow.

Special Note:  I reserve the right to modify, add to, and update this list going forward.  It seems the longer we think and talk about this topic, the more things pop to mind.

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Communication:

The most immediate and obvious issues will probably be communication.  There is no test or scan that diagnoses Trigeminal Neuralgia, though many – even “top” doctors – will look to the MRI for compressions for diagnosis, MRIs are NOT effective at diagnosing TN.  There are many facial and head pain conditions, so a proper diagnosis depends on an accurate description of the pain and any other symptoms, by a doctor with a large amount of knowledge and experience with Trigeminal Neuralgia.  Depending on the age of your child, this can be anywhere from easily misunderstood to impossible.

Our daughter was 11 years old and highly verbal at the onset of symptoms. (Seriously, when she was very young one of her punishments was “no talking” for a short period of time.  Within a minute she would be moaning in frustration at her lack of expression.)  However, when we made it to the pediatrician, she kept using the word “headache” which threw things off for a short time, giving us a diagnosis of Atypical Migraine.  Once we started keeping a detailed pain journal, and asking her to point to where it hurt, the discussion changed.  But in her defense, she explained, “your face is part of your head, right?”

Very young children may not be verbal enough, or have a sufficient vocabulary to describe what they are feeling beyond the obvious pain.  For the very young it will be important for you to pay careful attention to nonverbal communication. Are they holding a specific spot during pain?  Are they pulling or pressing on a specific spot on their face or head?  Are there things they have stopped doing – like eating, chewing (altogether or on a specific side), drinking cold beverages, brushing teeth.

Older children may still not have the subtle variations in vocabulary to tell you they are feeling “electric shock” pain, or a “boring” pain, subtle differences that are important.  They can answer your questions, so knowing what to ask becomes important.  The 5th neurosurgeon we went to did the absolute best job of this.  He would ask her about an aspect of her pain, letting her describe it.  But then he would also give her options to choose from in describing the location, pain descriptors, any other sensory factors.  Choosing the right word was often much easier than coming up with it out of her head – it’s just important to give a wide enough range of options so they don’t just choose something to give you an answer.  Sometimes these options would also be a starting point for her to say “It’s kind of like that, but it does this, too.”   This is how a good diagnosis happens.

Some pain descriptors you can ask about are:  aching, boring, burning, cutting, dull, electric, freezing, gnawing, inflamed, irritating, numbing, pins and needles, radiating, sharp, shocking, shooting, stabbing, stinging, tender, tight, tingling, throbbing, and vise-like.  This is just a start, feel free to add your own.

Two tools that may help are:

A face front/side image you child can use to specifically point out where pain is located:

Facial Sketch

A photo of the 1-10 pain scale with some descriptors to help understand it:

Facial Pain_Chart Horizontal

It is also good to choose your times to ask about the pain. I would often ask limited questions during the pain when it wasn’t too bad.  As the pain ramps up, there is no sense trying to ask annoying questions.  However, often, an hour or so after the pain stopped, when my girl had rested, she would then be willing and able to answer my questions more thoroughly.  It’s trial and error.  See when it works to talk to your child.  Experiment with different descriptors and see if any of them really connect with your child’s pain.

Your kid may do really well describing what they are going through.  Or they may really struggle. Or, like mine, you may think they are doing great when you are actually missing important pieces.  Know that we all miss things, we all stumble our way along. Especially in the beginning, but even throughout the journey.  It’s ok.  We offer this information to help, not to beat yourself up about – so you are now officially not allowed to do that.

Love, Hugs & Hope, from all of us to you.