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Welcome to the HeadFirst PNP blog

Hi, my name is Vickie and I am a PNP working in pediatric headache/pain medicine for the past 10+ years.  I’ve been a nurse for 37 years and a PNP for 21 years, so I have plenty of experience under my belt.  I’ve started this blog to share some information and insights I have gained over the years, with other providers and colleagues.

Headaches are one of the most common reasons that patients visit their pediatricians. There is a lot of information out there about headaches, most of it geared to adults.  I looked around the blogosphere and only found blogs about headaches/migraines by people who suffer with headaches, particularly migraineurs.  While many of these blogs are interesting, none really looked at the headache world from a provider’s perspective.  So I decided to create my own information stream, sharing helpful guidelines and strategies, up and coming research initiatives, and personal insights.

Headaches in children and teens are different from those in adults. There are many reasons why, such as genetics, biological, psychological and sociological factors, school and environmental factors.  What I do know is that headaches are a source of frequent school absence, family disruption and unnecessary pain.  By sharing my thoughts and ideas, I hope to improve the lives of both our patients/families and those who so diligently care for them.

Join me for a ride on the headache rollercoaster!

 

Migraine and concussion case study

So in the past few posts I have talked about the effect of a concussion can have on a patient with an existing headache diagnosis. Now let’s take a look at a classic headache clinic presentation as case study.  I am using a composite of patients and situations, commonly seen in primary care.

Background: Joe is a 17+ year-old young man with episodic migraine with and without aura for the past few years.  He is not on any preventive medications, and has been having migraine headaches at most once per month. While his migraine episodes are infrequent, they can often last a few days. Joe has an aura of neck pain 50% of the time. His pain score on average is 7/10NRS, pain is located all over his head, and accompanied by abdominal pain, photophobia greater than phonophobia, fatigue and nausea. He usually takes sumatriptan 50-100mg, with naproxen 500mg and ondansetron 4mg, which are effective. At times, he may need a Medrol dose pack, which is effective with prolonged migraine.   Triggers for Joe tend to be seasonal changes, stress, barometric pressure changes, lack of sleep and dehydration.   He did have a mild concussion in 2015 (#1), had a reasonable recovery time.  You have followed Joe for the past 4 years. Joe does a good job with his healthy lifestyle; he sleeps well, stays active playing basketball, drinks well and has a healthy diet. He could do a better job with stress management, mainly using the “Netflix and chilling out” strategy. He is an excellent student, takes his academic achievement seriously. He is also a great kid, polite, respectful and funny.

Situation: In late December 2016, Joe suffered a mild concussion (#2) during basketball tryouts. He was seen by his PCP soon after the incident. You had contact with the family by phone several times, and saw him in the office in February 2018. He did not lose consciousness, did missed 5 days of school, at home on cognitive rest. He had significant headache for 10 days, as well as dizziness, fogginess, irritable, difficulty with screens and school work. He used a Medrol dose pack and took naproxen BID for 5 days right after the incident.  His symptoms slowly regressed and he feels like it took about 1 month for the majority of symptoms to subside.  He still had slower reaction time with basketball and took longer to process information. He had some accommodations at school, and took appropriate breaks and use extra time if needed.

It took another month (2 months total) before Joe felt like he was back to normal and fully functional. His migraine headaches did not get significantly worse during this incident, he did not need to start a daily medication. Joe had a routine follow up appointment during the summer 2017 and continued to do well. He was having more stress heading into his senior year around the college application process.

Fast forward to concussion #3: In early October 2017, Joe was hit in the head with a hard-thrown ball in PE class. He reports no LOC but was immediately unsteady and had headache. Shortly after the blow, he became nauseous and dizzy, no confusion. He was seen in the ED immediately after the incident, had a CT scan which was negative. You have talked to the family on the phone since the incident and decided not to use a Medrol dose pack, as it was not well tolerated the last time (nausea, sleep disruption).

You see him in the office 3 weeks later and he has significantly decreased nausea and dizziness but constant headache, rated 5-6/10NRS, worse with exposures (light, noise, smell, general commotion) and concentration. Along with headache, he has phonophobia, photophobia and osmophobia, difficulty with reading and comprehension, using some computer screens, fatigue, sleep disruption and moody/cranky. Initially he was taking naproxen and Tylenol around the clock for the first week, and then stopped naproxen which improved his nausea. He has since been using Tylenol or Excedrin migraine daily, caffeine being very helpful in the AM. He did try hydroxyzine to help with sleep but had a paradoxical reaction to it (felt wired).

He has some accommodations at school, has access to a supportive learning classroom during study and has been excused from gym. He has had a few absences and early dismissals. He is having lots of stress and anxiety about missing classes and tests, which increases headache. He is currently not doing any physical activity. Joe is struggling with his recovery and feels he is not getting better.  You do remind him that it took a full 2 months after his last head hit to be back to baseline, just to reset expectations. This is a predictably stressful time, with the college application process going on.

Decision point: He was advised to stop daily analgesia, can drink a caffeinated drink in the Am and around lunch, if it is helpful. He may use Tylenol on a limited basis, no more than 3 days/week. You talk again about how even a mild head trauma can cause concussion-like symptoms and recovery should be dealt with as a post-concussion recovery. You talk about concussion accommodations, give written information, and they will discuss together about what he might need going forward. He will continue to take frequent breaks. You encourage starting some physical activity using the home exercise bike, in a graded fashion. You introduce Joe to the Insight Timer meditation app to use when trying to de-stress, sleep and decrease pain. He was encouraged to continue with good hydration and sleep hygiene, and to do what was needed to recover, emphasizing that pushing through no matter what would only prolong the recovery. You ask the family to check in with you in a few weeks or sooner with an update.

Update: You have had several phone calls, once every other week, for support and guidance over the past 6 weeks. Joe is very slowly getting better. His headache has diminished, no longer daily but 2-3 times/week. He is able to tolerate being in school now for a full day, only rarely taking breaks during the day. Sleep is back to normal and Joe is back to his usual mood, no longer irritable. Migraines are more often than baseline still, 2-3 per month. You continue to encourage slow and steady return to full functioning, glad to see he is on a positive trajectory.

Follow up appointment visit: You see him in the office in February 2018, 5 months after his 3rd concussion. He is fully recovered now. Migraine frequency and severity is also back to normal. You talk with Joe and his family about trying to avoid further concussion, but acknowledging that sometimes things happen.  At least doing his best to avoid risky situations would be useful.  They wonder if his concussions are putting his brain at risk in the future.  You don’t know for sure, but know there is considerable research ongoing which should shed more light on the subject.

So this is a fairly typical presentation of a teenaged migraineurs who has had several mild head traumas, leading to progressively more symptomatic and prolonged recovery. The closer in time the traumas occur, the more likely the recovery will take longer than expected.  The key to recovery is recognizing the problem, cognitive rest for a short period of time and then progressive return to functioning.  Joe’s school (and many others) had a supportive option available for these situations which was quite helpful.  And the more information that is disseminated about the difficulty of patients with a headache diagnosis have in recovering from concussion, the better and less stressful the outcome.

Concussion and the headache patient

In my last post I introduced the concept of concussion and the teenager with headaches. I would like to continue this topic by discussing what is helpful for these kids to help avoid disability and too much missed school.

The first step we recommend is for them to see their PCP or even the ED for a basic concussion exam. CT imaging can be useful with a history of more severe trauma, loss of consciousness or amnesia, or general concerns. The mild concussion diagnosis is then made and the usual recommendations of cognitive rest, physical rest, hydration and analgesia can be given.

For the teen with headaches, recent research using fMRI has shown that there is an element of neurogenic inflammation caused by head trauma. Often when the patients call us after concussion has occurred, we have them use a Medrol dose pack (dexamethasone taper), as way to reduce this inflammation. It can be quite helpful, though we really should try to do some research to quantify the results.   It is something to consider for those patients with baseline headache disorders who are very symptomatic.

So time goes by, the usual concussion recovery measures are done, and usually the teens will be feeling much better within 2 weeks. But for my patients with baseline headache disorders, that is not usually how it goes. So often, when one of my patients has a significant (or even not very significant) head bump, the families can go into panic mode. This is because the teen will often have more and prolonged symptoms than would be expected.

The initial symptoms will subside over time, leaving constant headache, and a variety of other complaints consistent with post-concussion syndrome. Besides headache, these symptoms include sleep disruption, moodiness, vision changes, hearing changes, persistent nausea, dizziness, vertigo, and cognitive difficulties, including inability to attend school and learn.  Since every teen and their responses are different, you need to address the issues at hand.  The symptoms that tend to become prolonged are those related to school functioning- trouble with reading, concentration and focus, and trouble related to the school environment –lights, noise, general overstimulation.

The first thing to address is the headache, and to make sure that they are not overusing analgesia. It is OK to use NSAIDS or Tylenol daily for up to 1 week, but much longer than that puts them at risk for developing medication overuse headaches.  So figuring out what they can do to feel better pain-wise is important.  We often talk about strategic use of medications, such as using naproxen during finals or before standardized testing is a smart way to medicate. They need to ensure excellent hydration and could try a little caffeine to help the pain.

For kids with sleep troubles and/or persistent nausea, I have found that a small dose of hydroxyzine (10-25mg) at bedtime can make a big improvement. Melatonin can be helpful for sleep (no more than 5mg), as well as the usual sleep hygiene recommendations (see an earlier post about lifestyle, All about the migraine part 2-3).

School!

There are many challenges for patients with post-concussion syndrome in returning to school. The school environment, with bright fluorescent lights, loud noise, and general commotion and overstimulation (think auditorium assemblies and basketball games in the gym), make school difficult to tolerate. This is true for teens with baseline headache disorders but worse after concussion.

Then there is the challenge of trying to think, focus and learn, especially frustrating for our teens who are natural ‘strivers’; those teens who are place high demands on their achievement. And it is very hard for these students to accept that they need accommodations and that they need to lower the bar of expectations for themselves, at least during recovery. Parents can also have difficulty with this, but they tend to have the maturity to understand what is needed, and can see the big picture. These kids worry me, as they are the ones at risk for becoming disabled, losing hope of ever feeling better, concerned about what the impact of the concussion will have on their future.  They can be unable to come to terms with the need for accommodations and slowing down, start to give up, and begin the slow decline towards disability.

So how can you help?

  • Support and frequent check-ins with the family can be very helpful to keeping them on track.
  • Work with the school on an appropriate re-entry plan, starting with partial days and frequent breaks during the day for cognitive rest. Some schools have special programs to help facilitate this, and can be really helpful. Asking for flexibility with the plan can make it more successful.
  • Enlist your patient to identify the problem areas of his/her day. Is it lunch in the loud cafeteria? Bright lights in English class, flickering lights in the science lab? Loud and hot environment in the gym/PE?   And after identifying the problems spots, work with the student to advocate for what can help them, such as preferential seating, excused from PE class, shutting off the lights, replacing a flickering light bulb, etc. Being able to make these changes can be very empowering and help them stay functional.
  • Cognitive rest is important and there needs to be emphasis on a gradual return to cognitive functioning and demands. They need reinforcement that overtaxing their brain will not help but only hurt them. There are specific accommodations that are helpful and I will attach a link here. Concussion Accommodations
  • For some teens, concentration and focus can be helped by a small dose of stimulant (methylphenidate or amphetamine), being mindful of other issues such as appetite and sleep.
  • Referral to Physical therapy for head and neck stretching. Referral for cognitive rehabilitative therapy (found with occupational or speech language therapy) can help with organization and functional skills post-concussion.  If you have a dedicated concussion center in your area, they will have these resources.
  • Return to physical activity is a slow process and using activity pacing is very useful. It is a difficult subject to explain but there is a series of You Tube videos done by a teenager with chronic pain that helps teens to figure out the concept. She discusses how to assess what is their current activity tolerance, how to set goals and reach them in a sensible measured way.  I put a link to these videos in the resources. YouTube videos on activity pacing
  • Finally, mood issues, irritability, anxiety and depression are a part of post-concussion syndrome. As the headache improves, this can improve also.  But as the duration of the symptoms continues, it is not uncommon for these kids to really get down and feel hopeless, or get very anxious about ever catching up in school and life. The most important thing is to be vigilant and refer to appropriate psychological clinicians as soon as you notice that the teen is having a difficult time. Explaining that feeling down and anxious can be a part of the post-concussion recovery and getting help them keep on the recovery track.

Awareness about the consequences of concussion is becoming much more mainstream. Less familiar is the effect of concussion is for those kids who already have baseline headache disorders. We owe it to our patients to be aware of the impact and support our families through this tough situation.

The Impact of concussion on teens with headache

There is much evidence and information in the literature about the general effect of concussion on kids and teens.  Most pediatric practices and schools use specific protocols for concussion recovery and these plans generally work well. Unfortunately, for patients with headache, the story of recovery can be quite different.  In this post, I would like to discuss the impact of concussion/mild head trauma on patients with a headache history.

A concussion happens when there is an impact injury to the skull that mildly damages the brain tissue, causing bruising and swelling. Immediate symptoms of a concussion can include a loss of consciousness, confusion, amnesia, nausea, vomiting, headache or dizziness. Some signs of a concussion may not appear for several hours after the injury and may persist for several days or weeks.

Post-concussion symptoms are caused by both physiologic and psychological factors from the effect of brain trauma. Physically, symptoms can be caused by structural damage to the brain or disruption of the neurological messaging system, triggered by impact. Psychological factors can be involved, since many of the symptoms, including headache, dizziness and sleep disruption, can also be attributed to depression, anxiety or PTSD.

Healthy teens generally recover from their concussions within a reasonable amount of time, especially if they follow the usual post-concussion guidelines, instituting cognitive and physical rest for a period of time. Depending on the severity of the injury, recovery can be complete within 3-4 weeks, with a gradual return to function.

Unfortunately, teens with a headache history (migraine, tension-type, new daily persistent headache) have a much more difficult time with their recovery. In fact, migraine is a risk factor for prolonged concussion recovery. Even when the head blow is quite mild, kids with headache tend to be much more symptomatic, diagnosed with a concussion. Whether it is a true ‘concussion’ or not, these patients have significant symptoms consistent with concussion. And they are much more at risk for developing post-concussion syndrome.

Post-concussion syndrome (PCS) is a condition in which the symptoms of a concussion persist for weeks, months or years. Symptoms of PCS include:

  • Headache: recurrent or constant mild to severe headache pain
  • Sleep: disruption, insomnia, daytime fatigue.
  • Mood: anxiety, irritability, depression, confusion
  • Sensory Alterations: blurred or double vision, slurred speech, altered sense of smell or taste, tinnitus or hearing loss, photophobia and phonophobia
  • GI complaints: nausea, vomiting, decreased appetite
  • Balance issues: unsteadiness, lightheadedness, dizziness or vertigo
  • Cognition: forgetfulness, misplacing common items, difficulty concentration and focus, comprehension and reading

After concussion, migraineurs tend to have more frequent and severe migraine episodes. The migraines become more difficult to treat with their usual rescue medications. For patients with TTH, there can also be more severe symptoms, often related to muscle spasms and tightness, especially if there was a whiplash injury. For NDPH patients, their overall baseline headache pain is increased with more frequent headache spikes and other symptoms.

Overall, having a mild head trauma can cause considerable symptoms and even the development of disability in your usually well-functioning teenager with headaches. For primary care providers, awareness of the likelihood of prolonged recovery for these patients is key.  Realistic expectations can be set with the patient and family. PCPs can assist in obtaining accommodations for school and academic demands, as well as supportive therapies to foster recovery. Emotional support is very important, as the results of these types of unexpected injuries can really test the resilience of the any family unit.

It’s important to remember that these types of injuries are not just sports-related.  One of the most prolonged and symptomatic PCS patient in my practice was a teenage girl with migraine who slipped and hit her head on the side of a bathroom sink. She was out of school for months and suffered greatly from this injury, requiring a lot of support and intervention to get back to functioning.  And then she fell and hit her head on the ice the following winter! She made a full recovery but….. oh my goodness.

In my next post, I will talk about care and treatment of the patient with headache and concussion.

 

 

The challenge of taking care of patients with NDPH

I have recently reviewed the basics of New Daily Persistent Headache (NDPH) so providers in the community can have a better understanding of this primary headache.  It is often misdiagnosed, mainly due to unfamiliarity.  And now that you have a better understanding, you will be able to recognize it more readily.  One of the most frustrating things for the families is the lack of a firm diagnosis.  Many medications are tried and failed, some appropriate and some inappropriate.  They often express relief after a headache program evaluation, when the providers can explain what is really going on with their child.  The diagnosis is not good news, but uncertainty is often worse.

So what exactly can you do to help a patient with NDPH? Since I know that few medications are useful, I do not routinely recommend one.  I take my cues from the parents and patient, and sometimes they have different opinions.  Oftentimes, the parents are eager to try another med, anything to get this better.  But the teens I deal with really are not interested in doing another medication that ‘will not help me and just make me feel bad’. I generally agree with them.  They are usually interested in participating in research, anything to better understand the NDPH.

In the past 1-2 years, I have been recommending that they try the short-duration lidocaine infusion. The way I look at it, it’s a short intervention that is either going to help or not. But it will not make them worse, which makes it a big plus. And if it is helpful in reducing their symptoms other than headache, that has the potential for improving function and avoiding disability.

Apart from these kinds of initiatives, I just focus on the basics.  That means healthy headache hygiene, which includes adequate hydration, good anti-inflammatory diet, moderate but frequent exercise, and a good night’s sleep.  I reviewed these areas in previous posts (All about the migraine parts 2 & 3). This is even more important for the NDPH patient and most of them are challenged in one or more areas.  They are frequently my patients who don’t drink enough, eat a beige diet, and are persistently nauseous, don’t exercise at all, and have a hard time with sleep. It can be overwhelming to have to tackle many lifestyle issues, so we focus on one at a time, baby steps to improvement.

I like to see them in follow up every 3-4 months to keep them on track. Frequent reminders about any positive progress are also helpful.  When they are discouraged, I refer back to my notes from previous visits, citing an improvement in pain scores or general functioning that I documented 1 year before.  When you are in the weeds with this kind of chronic pain problem, you tend to not notice when things get better incrementally. It can give them encouragement to continue on the path to wellness.

A huge aspect of dealing with teens with NDPH is learning how to cope and manage their chronic pain and stress. I start with recommending counseling with CBT to help them learn pain coping skills. They can be resistant but I can be persistent.   (See previous post: stress and migraine).  There is usually an anxiety overlay in these patients- whether it was there before the headache or developed as a result of having chronic headache.  We can consider starting an SSRI as a way to manage anxiety and also to help with the headache.  I will explain the nervous system pathways for pain and anxiety are right next to each other, and that treating anxiety could very well help the headache.  This is almost always a helpful approach.  It doesn’t ‘cure’ the headache, but promote better coping ability which leads to better functioning, which can lead to NDPH improvement.

I have also tried occipital nerve and trigger point injections in those patients whose headache seems more focal or located in the occipital area. This has the possibility of improving the headache.  I ask that they come for 2 procedures before deciding it does not help. I would say it helps reduce the pain level in about 50% of my patients with NDPH (my guestimate). This is another intervention that could help, and generally does not make the headache worse in the long run.

Understanding and treating NDPH is a work in progress. Redirecting from ineffective and often invasive measures, following patients closely with an open mind and constant support, and emphasizing non-pharmacologic approaches  remain the standard of care until, hopefully, new research brings new insight.

What’s new with NDPH?

I have spent a little time introducing and reviewing New Daily Persistent Headache (NDPH), and what a miserable headache it is. We know that traditional headache medications and treatments are not particularly useful in alleviating NDPH.  But there are some new initiatives available, some research initiatives and novel approaches to NDPH treatment which may bring some positive answers. I happen to be involved in a research project funded by the Migraine Research Foundation and also in a novel treatment approach to treating NDPH at our tertiary pediatric headache clinic.  So while it can be disheartening to care for kids with this diagnosis, it can also be hopeful, if you can identify something that might just help.

Because NDPH often appears after a viral illness, some researchers have posited that the headache is part of a post-inflammatory or autoimmune response. It is also a chronic pain entity, so treatments used for chronic pain might be useful (chronic regional pain syndrome (CRPS) or fibromyalgia). Treatment options now being considered are those that reduce inflammation in the body, such as low-dose naltrexone, anti-inflammatory diets, and short duration lidocaine infusion.

Here are some of the ideas that are being researched concerning NDPH:

Research initiative: Endogenous Modulation and Central Sensitization in New Daily Persistent Headache (NDPH) in Children, Boston Children’s Hospital, Pediatric Headache Program.  This project has started and recruitment is ongoing.  The aims of this research are:

  • Psychophysical Characterization of NDPH: To define differences in altered modulatory systems using offset analgesia in well characterized NDPH pediatric patients in the symptomatic and recovered state.
  • Treatment Effects in NDPH: To define the effects of low-dose naltrexone on offset analgesia.
  • Genetic Markers of Disease Persistence: To define the potential of chronic pain-related gene markers in predicting disease persistence.
  • Brain Markers of Disease Resilience: To evaluate genetic and brain markers of disease state.

The proposed work would be the first comprehensive, study of pediatric NDPH. If successful it will provide insights into altered modulatory systems in the disease and define for the first time potential insights into mechanisms underlying chronicity vs. response.

Research initiative: Analysis of Headache Chronification with Imaging, Deep Phenotyping, and Proteomics, Stanford University School of Medicine

The purpose of this study is to better understand disease processes and risk factors involved in onset of chronic daily headaches/Chronic Migraines. They hope to learn more about the basic pathophysiology and neuromodulation of NDPH to determine effective treatment strategies and prophylactic measures.

Participation in this research study includes online questionnaires, a blood sample, a lumbar puncture, and a magnetic resonance imaging (MRI) scan without contrast to measure brain activity. This study was started in 2014, will continue until 2020, for patients over the age of 18 years.

Research initiative: Plasma Calcitonin Gene-Related Peptide and Nerve Growth Factor levels in New Daily Persistent Headache and Chronic Migraine to identify potential biomarkers and therapeutics targets, Montefiore Headache Center, NYC.

This research is studying whether CGRP or NGF levels are elevated in the plasma of New Daily Persistent Headache patients compared to those with chronic migraine and normal controls. This study is ongoing, for patients over age 18 years.

Treatment: Botox for NDPH: Recent reports from the Cleveland Clinic and Case Western Reserve describe patients with new daily persistent headaches (NDPH) who were treated with Botox injections (standard Botox for chronic migraine protocol). The improvement was modest (30%), with some reduction in headache pain, headache-free days not previously experienced, and positive effects lasting for 8 weeks. Available for patients over 18 years, and pending insurance approval (not FDA approved for this indication).

Treatment: Low dose naltrexone: Naltrexone is an anti-inflammatory agent, similar to the opioid antagonist naloxone, and an effective treatment for opioid addiction. It was recently discovered that when taken in low doses (1/10 of the typical dose), it may reduce the severity of chronic pain symptoms, by acting on the glial cells and other receptors in the nervous system. Because of its analgesic property, low-dose naltrexone may be an effective treatment for the management of several chronic pain conditions, including fibromyalgia and chronic headache. More research needs to be done to evaluate long-term effects of using low-dose naltrexone, and to assess the efficacy and safety of this medication in the treatment of patients with NDPH. If prescribed, this medication has to be prepared at a compounding pharmacy; retail pharmacies carry the standard 50mg tabs, and low-dose naltrexone is dosed at 4.5mg, which requires compounding, usually not covered by insurance.

Treatment: Short-duration lidocaine infusion: Lidocaine has been used as anesthesia and analgesia for more than 50 years, and is very effective at relieving nerve pain, chronic pain syndromes, and pain after surgery.  Lidocaine is an amide local anesthetic agent, which blocks fast voltage-gated sodium channels in the cell membrane of postsynaptic neurons, preventing depolarization and inhibiting the generation and propagation of nerve impulses.  Basically, lidocaine stops pain impulses from going to and from the brain, so pain is blocked.

In my pediatric headache program, we now offer short duration lidocaine infusions to our patients with New Daily Persistent Headache, the first program doing this with pediatric patients. It has been used in adult pain programs for chronic pain for many years. We hoped it would be effective for teens with NDPH, also a chronic pain condition, providing a decrease in pain level and/or reduction in the associated symptoms for these patients.

The protocol for short duration lidocaine infusion for patients over 50kg is 200mg of lidocaine IV over 2 hours , with appropriate monitoring for alterations in cardiac status and signs of lidocaine toxicity. For patients less than 50kg, 4mg/kg is given. At first, we did a bolus dose of 100mg over 30 minutes, followed by an infusion of the remaining 100mg over 1 hour.  Unfortunately, in the 1st 3 months, almost all of the kids had side effects during the bolus.  So we changed to a 2 hour infusion which has been much better tolerated, many fewer reactions.

So what are the results? Well at first we were disappointed because there was really minimal reduction in headache pain. Then, it became clear that the benefits were more significant in reducing the other symptoms. In particular, patients were achieving improvement in their persistent nausea, dizziness, difficulty with concentration, difficulty with physical activity, mental clouding, fatigue and overall functioning. And for the majority of patients who responded positively, they did not return all the way to baseline after several months, with sustained improvement.  We have a number of patients who come in every 3 months and repeat the infusion because it has benefitted them so much.  What the kids tell me is that they can actually tolerate the headache pain, when the other symptoms are reduced, but have a great deal of trouble functioning with both headache and the rest of their symptoms. I think the kids who have many associated symptoms seem to get the most benefit and improvement.  I will put a copy of the poster abstract we did about this- preliminary results over the first year- in resources.

This is an initiative I am quite involved with, screening, monitoring during infusion and follow up with data collection. It is exciting to find something that is showing benefit to my kids with NDPH. Granted, we only have about 60% positive response rate, but for those 60%, it is wonderful.

We will be continuing this initiative and collecting data and at some point be putting together an academic paper to share. I am also hopeful that some of the research work will also yield positive results. At the very least a better understanding of the pathophysiology of NDPH would help guide future therapies.

I will discuss more about NDPH trajectory for resolution and other matters in the next post.

More about NDPH

Let’s talk today about the story of NDPH, a primary headache not well understood or recognized, a zebra among headaches. The more we know about it, the more it is recognized.  But that does not make it any easier to deal with as a provider or a patient.

The first documented description of NDPH was in 1986, by Dr. Walter Vanast, a neurologist in Canada. He described it as a benign syndrome that resolved regardless of treatment in 73-86% in 3-24 months. In a recent interview with Dr. Vanast in Headache journal, he describes his interest and search for the cause of this headache. He thought it was an auto‐immune disorder with a persistent viral trigger, such as EBV. And this description is still in use today, though in my opinion ‘benign’ is not the word I would use.

About half of patients with NDPH have had a febrile viral illness near the onset. Others develop it after a minor head injury, or pseudotumor or idiopathic intracranial hypertension (IIH). Others seem to have no known reason for this headache to develop. There continues to be very little known about the pathophysiology of NDPH.  A recent paper stated that it might be related to persistent central nervous system inflammation or related to cervical hypermobility. We really don’t know.

The incidence in adult of chronic daily headache is 4%; the incidence of these adult patients having NDPH is between 1-10%, so not that many. But in children and teens, the incidence is much higher: 20-40% of pediatric chronic daily headache patients. Way more kids and teens than adults have NDPH. And it is probably underreported and under recognized.

There are several different patterns of headache associated with NDPH.

  • The first type is mild and self-limiting, resolving without therapy.
  • The second type is moderate, with a constant headache that waxes and wanes over time. Good days are more like tension-type headaches and bad days are more like migraine.
  • The third type is a continuous severe headache, with disabling high-level pain all day every day.

We don’t see many patients with the first type, with lower level headaches that just end up going away. Most commonly we see the second type, kids with constant all day every day headache, pain rated 5-6/10NRS on average with a range of 3-9/10NRS. On lower pain days, they might have mild nausea, mild dizziness, mild mental fogginess and light sensitivity along with headache. On higher pain days, all of their symptoms are severe and migrainous; pain is 8-9/10NRS, and they are non-functional. Their pain and symptoms are disabling; change in pain level is influenced by activity, weather patterns, stress and cognitive effort.  Fortunately, while we do see the completely refractory third type, they are not as common– but so difficult.

So it is hard enough to have a constant headache, no headache-free time. But add to that is the realization that most common treatments and even the most aggressive treatments are ineffective against this headache. Current daily medications do not alleviate their pain and the patients just experience all the side effects.

Usually by the time the patient arrives at our specialty headache program, they have already tried at least 1 or more daily medications without success. The families might like to try more medication, so we would pick another of the common ones. The choice is often based on what other symptoms are most bothersome.

  • Unable to sleep? Choose amitriptyline or gabapentin.
  • Worried about weight gain? Choose topiramate.
  • Don’t want any mental clouding? Try propranolol.
  • Super dizzy? Try verapamil.
  • Persistent nausea? Add hydroxyzine at bedtime.
  • Very tight neck and upper back muscle? Choose tizanidine.
  • Don’t want medications? Try a supplement- magnesium or riboflavin.
  • All else fails? Try occipital nerve and trigger point injections.

You might find that something helps other symptoms, even when it does nothing to the headache. I have had great results with using hydroxyzine at bedtime for patients with persistent nausea. Having less nausea may make the difference between going to school or staying home…. again.

It is just a matter of time before the families (really the patients) decide that they do not want to try any further medication, at least daily medication. Most tell me that they feel better off the meds.

Things you might try for a migraineur in status migrainosis such as going to the ED or being admitted for DHE are not usually helpful. Often the patients end up more frustrated, faced with another ineffective intervention.

As far as rescue medications, again you try the basics- NSAIDS, antiemetics, caffeine. Most important is to stress that analgesia should only be used for severe headache and no more than 3 days/week to avoid overuse headache. As you can imagine, overuse is tempting, kids just want to feel better.  But I stress that too much analgesia will just make them feel worse, and in the end, the medications will stop working. I encourage being strategic with analgesia. Save it for when you might really need it, such as before SAT exams or before a big sports game or concert.  This is generally effective in preventing overuse.

What is really important is for the families and patients to know that you are all in this together. Accepting where they are and working with them. Offering hope but being realistic.

I will have more to say about some research initiatives and novel approaches to NDPH in the next post.

Introducing……… New Daily Persistent Headache

In this post, I am going to introduce you to another primary headache, new daily persistent headache (NDPH). This primary headache is not often seen in primary care, but I think our clinic population has about 25% of teens with this diagnosis.  There’s a good reason why a referral to a specialty headache program is important for these patients.

Imagine being a 13 year-old girl, just getting over a viral illness, and suddenly finding yourself with a constant, unremitting headache. At first you and your family think, it will go away soon, probably related to having just been sick.  But it doesn’t go away………….. for months or years.  That’s NDPH.

NDPH is an unrelenting headache that develops rapidly, with the person often knowing the exact day when it began, and having a continuous headache from that day. It often happens in a person with no past history of headaches. It neither a migraine or tension-type headache, but begins as a new headache. This is a very difficult headache for patients and families as it often does not respond to treatment.  It is called Persistent because the average length of this headache can be up to 3 years, or more. Incidence is reported at 2-4% of patients with headache, but this is most likely underreported, as the headache is often unrecognized for what it really is. It is seen in both adults and adolescents, but more frequently in teens.

NDPH is characterized by:

  • appearing without warning;
  • often following a viral illness, several concussions, or for no reason at all;
  • Constant headache that lasts for more than 3 months from onset (within 3 days);
  • At least 2 of these: felt on both sides of the head; feel pressing or squeezing (not pulsing); mild to moderate pain; not worsened by routine physical activity;
  • Both of these: light or sound sensitivity or mild nausea AND NOT moderate or severe nausea/vomiting;
  • All other reasons have been investigated and ruled out.

For some patients, there can be migraine-like symptoms, such as light and sound sensitivity, and nausea. For some, there can be tension-type headache symptoms, such as mild-moderate pain, non-throbbing on both sides of the head. NDPH does not respond to the usual headache prevention medications or the usual rescue treatments and medications.  Unfortunately, many patients can become disabled with NDPH.

So this teenager comes into your office, with the complaint of constant headache for the past few weeks. What do you do? First you rule out a secondary reason for headache, perhaps do some labwork (CBC, chemistry, B12, thyroid, celiac, etc), brain MRI, thorough patient and family history and physical, making sure there’s nothing else going on. The diagnosis of NDPH is a diagnosis of exclusion.

Once the secondary generators have been ruled out, you think about the primary headaches. Migraine and tension-type headaches generally start as episodic, not constant or chronic. So even with a strong family history of migraine, that’s probably not what this is.

So it’s a constant headache, moderate in pain (though with some spikes of more severe pain), probably associated with environmental sensitivities and nausea. It’s a terrible diagnosis. You have to tell the family that the usual headache treatments and medications don’t work well, that there is really no cure except time and adherence to a multidisciplinary approach to care and wellness.

And then you refer them to a specialty pediatric headache program because the family and you will need all the support you can get. In the meantime, you help them work on lifestyle- good sleep, hydration, healthy diet, and staying active.

As you can imagine, this is quite difficult. It is especially hard when there really doesn’t seem to be a reason (virus, too many concussions). In my experience, when there does not seem to be an identifiable cause for the NDPH, these particular families have a VERY hard time coming to acceptance and embracing the multidisciplinary approach. They are always ‘searching for a reason’, which can often send them down some unhealthy and unhelpful rabbit holes. That’s why you send them to a pediatric headache center, where this diagnosis is well known.

The key here is early identification of NDPH, as soon as you suspect it is not a standard primary headache. Delays in diagnosis often lead to unnecessary medication and treatment trials, increased frustration and desperation on the part of the family, and an increased level of disability for the patient.  This is a real problem without an easy answer.

I know from personal experience that partnering with the family, through all the ups and downs, makes a difference in outcomes and level of disability. You stick with them, gain their trust, and offer lots of support.  One thing I will tell my patients in particular is that ‘I believe that their headache will resolve someday. I don’t have a crystal ball to say when, but I believe they will get better. And I have seen many patients who really did get better’.  This can help give them hope.

Hope is so important and powerful. I often refer to my colleagues and myself as chief cheerleaders for these families. They’ll say ‘we know there’s not much out there that can help, but we come in anyway, so you can give us hope’.   Recovery is possible with hope.

I will further review options for dealing with the patient with NDPH in my next post.