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.

Migraine case study, part 3

In this case study we’ll look at a fairly straightforward patient, with some twists and turns and decision points along the way.

Case Study: Patty is currently an 18 year-old teenage girl with a history of migraine headaches since age 9. You have been seeing her for years, and this is her story.  At the time of diagnosis, her migraines were infrequent (monthly at most), easy managed with a dose of ibuprofen and a nap. She was on no daily medications, and did a good job with her healthy headache hygiene. She is otherwise healthy except for exercise-induced asthma, just uses an inhaler as needed. She lives at home with her parents, 2 younger brothers, and several pets.  There is a strong family history of migraine

Around ages 11-12, things started to change, puberty was arriving. During this time her migraines were a bit more frequent, still manageable. Once her menses started, things really took off. Migraine frequency changed from monthly to weekly, and around her menses, they were more intense. She started to notice some aura-like symptoms, such as a black spot in her line of vision 30 minutes before the migraine started (50% of the time). She would also get more moody than usual 1 day before a migraine. She was also more symptomatic, having pain on average at 6-7/10NRS, accompanied by nausea with vomiting and lightheadedness, photophobia and phonophobia, fatigue, and that black visual spot. Ibuprofen was no longer effective and she often threw it up. Sleep was the only thing that helped, and the migraine lasted for several hours.  For lifestyle, she does sleep well at night, and eats a selective diet but no meal skipping.  Hydration is fair; she weighs 45kg and is drinking only 20oz/day and not drinking at school.  She is physically active with soccer and softball.

Decision point: Patty has reached puberty and her migraine headaches have become more frequent and symptomatic.  You decide that it is time to change her rescue plan, and consider a daily medication or at least a supplement.  The family would prefer not to start a medication, so you give them the option of starting daily magnesium 400mg daily as supplement. You also ask that she do a better job with hydration, at least 50 oz/day, and to bring water to school (and drink it).  You also reinforce her already healthy habits.  For rescue, you introduce a triptan, rizatriptan 5mg ODT (dissolving tab) to be taken when she notices her black spot before the migraine hits.  You also provide ondansetron 4mg ODT for her nausea, to be followed by naproxen 375mg.  She may take just the ondansetron and naproxen if no aura or the migraine is already started.  Since her migraines are just weekly, it is unlikely she will overuse medication but you do remind them that migraine analgesia should not be used more then 2-3 days/week. You ask them to return for a follow up appointment in 2 months to see how she is doing.

Follow up appointment visit: Patty is doing much better with her hydration, taking magnesium every day, and her rescue plan is working pretty well.  If she can take the rizatriptan quickly, many times she is able to abort her migraines.  And best of all, her migraines have reduced to 1-2 times/month; usually one of these times is around her menses.

Fast forward 2 years: Patty is here for her yearly check-up, age 14, in the summer before she starts high school. She is doing fairly well though her migraines are more frequent these days, back to once/week.  Her rescue medications are working well.  She has been using the Migraine Buddy app and tracking her headaches, has discovered that stress, caffeine and her menses are the biggest triggers.  She still takes magnesium but as not regularly, has backtracked a bit on hydration and has stopped playing sports.  She is also not sleeping well due to a busy mind, and her diet is slightly more adventurous, no meal skipping. She is particularly worried today about her migraines and starting high school, getting stressed out about it.  The family is thinking it might be a good idea to start a daily medication for migraine prevention.  Her health continues good, no recent illnesses or asthma flares.  Her weight is healthy, at 55kg.

Decision point: You agree with the family about trying a daily medication.  You consider your choices: cyproheptadine is not a good choice as she is beyond puberty and it will most likely cause too much weight gain; amitriptyline might be a good choice, might help with the anxiety  and sleep;  topiramate could also be chosen; Propranolol is not appropriate due to her asthma.  You choose amitriptyline 10mg at bedtime, may increase to 20mg after 1 month, and you check an EKG (normal).  You advise about side effects- sleepiness, mental clouding. She can continue magnesium if desired or try riboflavin B2 instead.  Lifestyle needs to be addressed and again you talk about hydration (60oz/day), sleep hygiene (put away that phone before bed), and getting more physical activity, since she is not doing sports now.  You also explore with the family ways to deal with stress, such as using a meditation app or exercise, seeing a counselor for CBT.  Patty is not really interested in counseling but she might try the Insight Timer app that you showed her how to use today. You ask them to return for a follow up appointment in 3 months to see how she is doing.

Follow up appointment visit: Patty is tolerating the amitriptyline at 20mg, no side effects and sleeping better.  She is having fewer migraine headaches back to twice/month. She did start hydrating better and now that school has restarted, she is in the habit of bringing a water bottle to school, empty when she gets home.  She is still not very active- none of her friends are active and she just wants to hang out with them.  The family does enforce better sleep hygiene with a set bedtime and phone out of her room at night. She is not in counseling and tried to do relaxation exercises but ‘found it a little to woo-woo’ for her liking.  Stress has not really been addressed, though the family is more aware of it now.  They are satisfied with her progress.

Fast forward, age 15, urgent care visit:  It’s winter and there’s a nasty GI virus going through the school, and Patty has gotten it. Patty has had a hard time, has been unable to tolerate fluids, vomiting and having diarrhea for the past 2 days.  And she just got her menses.  A perfect storm.  You see her in urgent care and she’s pale and miserable, and has a migraine on top of it all. She’s had a severe 9/10NRS headache for the past 12 hours, and is unable to take her rescue medications, take her daily amitriptyline or hydrate.

Decision point:  This is clearly not going to get better without intervention.  Fortunately, in your facility’s urgent care, patients can receive IV fluids and medications; otherwise you would need to send her to the local ED. An IV is started and she is given 500cc of saline to rehydrate to start.  Then, she receives a dose of IV ondansetron 4mg, and ketorolac 30mg, and perhaps Compazine 5mg.  Patty falls asleep and awakens in an hour feeling better, though still with a milder headache. You repeat the IV fluids and she is improved.  The family is comfortable taking her home.   You encourage them to continue hydration, electrolyte-rich fluids and antiemetics, and rest. In 1-2 days, all is well.

Fast forward, age 18, routine visit in December: Patty is doing well, in her senior year of high school, has gotten into her #1 choice for college. Her general health has been good, and apart from that 1 episode requiring urgent care, her migraine headaches have been manageable.  She remains on amitriptyline 25 mg nightly, and is having 2 migraines per month.  She does have a healthy lifestyle, doing well with hydration and reports that she has ‘finally learned that I have to drink a lot to prevent migraine’.  She enjoys being outdoors and hiking and kayaking now, and enjoys feeling physically strong and able.  She has a passion for the environment and wants to make a difference in the world through her environmental work in the future. She wants to talk about her menstrual migraines and also about birth control. She has always had bad periods with lots of cramps, and gets a very bad migraine on day #1.  She did some research (Dr. Google) and found out that some people with menstrual migraines and bad periods do better if they are on birth control.  Plus she has a new boyfriend, and has some other concerns as well. She continues to experience her aura of black spots, almost every migraine, and sometimes loses vision in one eye.  Rizatriptan is not as effective as it used to be, especially around her menses.

Decision point: This is a tricky situation, as women with migraine with aura have to be very careful with birth control choices. The general rule is to avoid estrogen-containing OCP, to avoid increased risk of stroke, at least as a start (see article in resources). Patty is correct that being on an OCP can help with dysmenorrhea and menstrual migraine.  You might suggest a progestin-only product, or refer her to a gynecologist.  Progestin-only OCP tend to be a little challenging as they often need to be taken at a consistent time, but there are other options as well such as depo-provera or progestin-only implant. One thing you can do today is switch her from rizatriptan to frovatriptan, the triptan that was specially developed for use with menstrual migraine.  Patty states she would be happy if her menstrual migraines were better; she might even want to stop taking amitriptyline. You and Patty decide that she will see a gynecologist about her birth control.  Once she is on a stable regimen, she can decide about stopping amitriptyline.  You ask her to consider waiting until summer break to do this, as she always has less migraine then and will have less stress.  She agrees and will come for a follow up during the summer.

So there you have it, an example of a typical migraineur from childhood through adolescence. The issues that present themselves here can all be well managed in the primary care setting. Most migraineurs end up in the ED at times, especially during times of illness and it may be unavoidable. These ED visits should not be considered failures. Rather they can be opportunities to fine tune rescue plans or reinforce a healthy lifestyle.   I always tell my patients that they will always be a migraineur, but their migraine headaches will change over their lifespan, for better and worse. Patients with migraine need education and empowerment to keep up with their self-care, and a caring provider to assist them.

 

Migraine case studies, part 2

Here are the case studies with your possible interventions.

Case Study #1:  Sally is an 8 year-old little girl who you have known since birth. She’s happy and friendly, loves to play soccer and school, especially math. She met all of her developmental milestones, though you do remember that she did have colic as an infant.  Over the years her parents have heard her complain at times about having a headache but not often, usually associated with a long busy day or not getting enough sleep. There are many people with headaches in the family, so her complaints did not really concern her parents.  They would occasionally give her a dose of Tylenol with resolution of the headache.  Recently, Sally has been more vocal about her headaches, crying at times, complaining of nausea and light sensitivity with her headaches.  Her mother has brought her in today to talk about this.

Your visit: Sally is her usual happy self, but her mother is a bit concerned.  They report that for the past 3-4 months, Sally is having headaches about 1-2 times/month, with head pain rated 6-8/10 FACES scale, and accompanied by some nausea but no vomiting and light sensitivity.  At times the headaches are preceded by eating a chocolate, occur on a hot day when Sally may not have had enough to drink or did not have enough sleep. She does not have any aura or prodrome symptoms, but her mother notices that she seems droopy and pale before and during the headache. Sally usually receives a dose of Tylenol and a drink, and then takes a nap for a few hours, which resolves the headache. You confirm that Sally has been well in the past few months, no illnesses or head trauma, and confirm the family history of migraine headache.  There is nothing concerning on her physical exam.

Your intervention: You propose that these headaches are most likely migraine, based on the symptoms, history and family history. As her migraines are infrequent, the focus of your intervention is going to be on lifestyle factors and rescue methods and medications.

You talk with the family about the importance of getting a good night’s sleep (10 hours for kids her age), lots of healthy exercise and no meal skipping. Sally weighs 24kg, so her daily hydration goal is 20-25 ounces (at least 1oz/kg/day or half their body weight in pounds), more in the hot weather and with exercise.  You give them information about the migraine elimination diet and any office materials you have about migraine headache. Sadly, she may have to eliminate or at least limit the amount of chocolate she eats, and perhaps make other adjustments, such as keeping to a regular sleep schedule even on weekends and limit sleepovers.

You advise using ibuprofen 200mg (1 adult, 2 chewable tabs or liquid) at the start of the migraine, hydrating with water or electrolyte-rich drink (Gatorade, Smart water, etc.) and then taking a nap for rescue. She can also try using an ice pack on her head, keeping her room dark, quiet and cool.  Encourage them to keep a headache diary so that they can give you good data at their next appointment in several months as follow up. Offer reassurance and support that you will work as a team to manage her migraine headaches.

Case Study #2: Charlie is a 10 year-old boy, known to you for a few years. He is generally healthy, met all his milestones appropriately, and is active playing a variety of sports. He attends school regularly, no learning issues, and has lots of friends.  Lately he has been complaining of abdominal pain, with and without nausea, and not associated with food or diarrhea or constipation.  It has been happening at school and he has had to be dismissed home multiple times in the past several weeks.  The family is puzzled, as the pain is not consistently triggered by anything they can think of, no vomiting or diarrhea.  They are wondering if there is something going on at school, if the pain is real and what is causing it.

Your visit: Charlie is here with his mother and father today, and they all look worried.  Charlie reports that ‘out of the blue’ he starts feeling sick to his stomach, and then he gets really bad belly pain, ‘right in the middle’. This has been happening several times/week now for the past month, usually at school or in the evening.  Sometimes he gets the pain without feeling nauseous.  The pain is so bad that he wants to go home, and he rates it between 8-20/10 on the pain numeric rating scale.  They have noticed that he becomes very pale with dark circles under his eyes with the belly pain, gets clammy and tired, just wants to sleep.  After he naps for 1-2 hours, the pain goes away, but he does feel tired and sleepy afterwards. There does not seem to be anything that triggers the pain and there are no other signs of illness, no diarrhea, or headache. He has regular soft bowel movements every day, no soiling or constipation.  When he does not have pain, he feels just fine, playful and happy.  They do notice that sometimes the pain happens after a very long day of activity, when he has not had enough to drink, or after they eat Chinese food.  They have not tried any OTC medications, thinking it would make him throw up, which he really hates.  There is no history of migraine in the family that they know of. But Charlie’s father remembers that his big brother used to get really bad stomach pain when he was a kid, and he outgrew it as a teenager. There is nothing concerning on his physical exam, his abdomen is soft and non-tender in all quadrants.

Your intervention: You approach this from a GI perspective, first exploring sources of GI pain, checking an XRAY for constipation and other abnormalities, such as intussusception, checking a stool sample, checking labs for celiac, etc.  When it all comes back negative, you propose that these symptoms are consistent with a migraine variant, called abdominal migraine, something particular to children. This diagnosis is supported by his overall wellness, the episodic frequency and his symptoms while having pain, and the family history (uncle with similar presentation).  As this migraine is rather frequent, you consider a daily medication, as well as lifestyle factors and rescue methods and medications. 

As with the previous case study, lifestyle is key in preventing abdominal migraine; sleep, diet, hydration and exercise can help limit the migraine episodes. The family has already noticed some possible triggers, and is advised to avoid Chinese food (or at least MSG) and to start reading processed food labels for it. Information about the migraine elimination diet and office materials about migraine is helpful. There may need to be lifestyle adjustments, enforcing a regular sleep schedule, making sure Charlie brings water to school every day to meet his daily hydration goal (34kg=30-40oz), extra with sports.

Lifestyle measures may be all he needs to decrease the abdominal migraine frequency. But if not, an appropriate daily medication to try would be cyproheptadine 2-4mg at bedtime. This works quite well in pre-pubertal children, though you have to be sure to mention the side effects of increased appetite, sleepiness and at times, irritability.  (If he is grumpy with diphenhydramine, I might not choose it, perhaps choosing amitriptyline instead.)

For rescue, if Charlie is very nauseous, he can take ondansetron 4mg dissolving tabs ODT first. Then he can use ibuprofen 200-300mg (1 1/2 adult or 3 chewable tabs), OTC Aleve 220mg or prescription naproxen 250mg at the start of the migraine. He should hydrate with water or electrolyte-rich drink and then taking a nap for rescue, resting in a dark, quiet and cool room. The family can keep a migraine diary so that they can give you good data at their next appointment in several months as follow up. One thing about abdominal migraine is that they are changeable. As time goes on, he may very well transition from just abdominal pain to a combination of abdominal and head pain. As children proceed through puberty into later teenage years, abdominal migraine either just goes away completely, or transforms into migraine headache. As he is a male, his migraine may just disappear at the end of puberty, similar to his uncle.

Case Study #3: Lucy is a 13 year-old tween, who you have just met this past year.  The family had moved from out of state and Lucy has had a hard time in adjusting to her new school, but she has made a few friends. She is a good student, and is active in drama club and music, not into sports. According to her family, she has always been sensitive to her environment; bright lights, loud noise and certain smells have always bothered her.  She tends to get lightheaded when she stands up quickly, and is very bothered by her sweaty hands and feet.  She is generally healthy, but does have some GERD symptoms and constipation. Her diet could be better, very selective and often complains of mild nausea in the morning.  She has always been a terrible sleeper, since infancy.  She started having her menses 6 months ago. She has started complaining of severe headaches for the past several months and they are here to see you about it.

Your visit: Lucy is here with her mother today. She reports that she has been getting really bad headaches for the past several months. They started out just once in a while and now she has headaches every week, sometimes more than 1, and usually after school.  Pain is rated 6-9/10NRS on average and she gets these weird symptoms right before the headache comes (squiggly black lines in her vision).  The headache is always on the L side- temple and behind her eye, and she feels nauseous, dizzy and really tired.  Lights and noise bother her more than usual, and the smell of some foods makes it worse.  She goes into her room, into the darkness and tries to go to sleep.  She usually takes Tylenol or ibuprofen which helps a bit, but needs to go to sleep for a few hours to feel better. When she wakes up, she might still have a mild headache but feels lousy, back to normal the next day.   She has not been sick lately, and has been feeling well, except for the headaches. The only other thing is that her periods are also really painful, lots of cramps, and this makes her miserable.  In reviewing her family history, her mother does report members of her family who have migraine and thinks Lucy has migraine too.  Lucy is not very physically active, sleeps poorly, and does not like to hydrate, especially at school (the bathroom is gross!).  Her physical exam reveals her to be neurologically intact, though there is evidence of some autonomic symptoms, such as hyperhidrosis of the hands and feet, mild hand tremor, sensitivity in the distribution of the nuclear caudalis (trigeminal sensitivity) and GI issues.

Your interventions: You propose that these headaches are most likely migraine with aura, based on the symptoms, history and family history. She also is neurologically sensitive, with some autonomic nervous system dysfunction. Her case is bit more complex, more moving parts and will require a multidisciplinary approach, including daily and rescue medications, and lifestyle improvement.

Early adolescence is a time of great upheaval, physically, hormonally, socially and emotionally. It is also a time when migraine often appears. Being a sensitive individual since infancy, Lucy is more prone to the ups and downs of this period, and may not have the coping skills she needs to navigate to good health.

Focusing on lifestyle, there are many areas for improvement. But bringing them all up at once is usually overwhelming and unproductive. A step-wise approach will be more helpful and gain her cooperation. Her sleep is not great, she is not active and is not hydrating enough (55kg=50-60 oz/day).  Considering her other symptoms, I would probably first put the most focus on hydration, which would certainly help with her dizziness and nausea. At least until her headaches are better, I would encourage having an electrolyte-rich drink every day, preferably in the AM before school. Lucy should also have a 20-24oz water bottle with her at school every day and bring it home empty.   The parents can check out the issue with the school bathrooms, try to identify the cleanest of them all, perhaps near guidance or the nurse’s office.

The next thing to focus on would be exercise with a goal of 30 minutes 3 times/week, doing whatever she enjoys, such as dancing, swimming or bike riding. Sleep is a longstanding issue, and guidelines for sleep hygiene can be shared and slowly worked on, such as no phone charging in her room overnight for a start.  I would also make sure that there is no meal skipping, especially at school. If there are concerns about anxiety, that should be explored and if appropriate, counseling for coping with pain and anxiety can be helpful (CBT). Learning to turn on the relaxation response (CBT, meditation, yoga) can also help calm down her autonomic nervous system and reduce a lot of her environmental sensitivities.

There are several daily medications which would be appropriate for Lucy. I might choose amitriptyline or gabapentin in particular, as they do cause sleepiness and might help with her poor sleep. If she is a poor eater or there is worry about restrictive eating, I would probably not choose topiramate. Almost all headache medications cause mental clouding so the lowest effective dose is best. Propranolol could be used too, as she is not asthmatic nor a performance athlete, though hydration is really important with this medication. It is important to stress that it takes at least 1 month for any difference to be noticed.

For rescue, since she does have aura, Lucy could try a triptan at the earliest evidence of migraine aura, such as rizatriptan. After a dose and rest, if her migraine is aborted, that’s all she needs. Often a combination of a triptan and analgesia is needed for most effective treatment. The simplest thing is often a combination of a triptan and naproxen, using antiemetic as needed.  You can strongly urge no more than 3 days of analgesia per week.  Again keeping a diary, perhaps using the Migraine Buddy app, to gather good data will be helpful.  

She can use the naproxen for her menstrual cramps too. As she gets older, she may discover that she always gets a migraine around her period. She might want to try an oral contraceptive to help with her dysmenorrhea.  Since she has aura, this needs to be done with caution. We recommend using progestin-only preparations with aura, at least to start, perhaps with the guidance of a GYN.  

It might take a few visits to help Lucy feel better, but I would encourage focusing on all 3 areas from the start to help her. Her daily medication may start to decrease her migraine frequency, a better rescue plan makes her feel more positive and in control, more hydration and a little better sleep will help too.  Then you can move on to adjusting her medications as needed, promoting more positive lifestyle changes, helping her to improve her coping skills and resilience. Frequent office visits can really help families make the necessary changes, with your positive encouragement and obvious caring practices. There is nothing more satisfying than guiding a teen and her family toward having self-agency and competence in dealing with a chronic health issue.

So you can see that these are fairly typical patients seen in primary care. I didn’t go into it, but supplements, and complementary interventions are often a part of treatment, as mentioned in pervious posts. I hope that these case studies are helpful in guiding your clinical decision making.  I will include some patient migraine information in the reference materials.  I think I will do another case study in the next post, this time focusing on someone with increased medical complexity, who needs a team approach.

Chronic migraine, what to do?

So your patient has developed chronic migraine, which is defined as 3-4 migraine per week, 15 migraine days per month, and is predictably miserable. Developing chronic migraine does happen in pediatrics, but it happens much more often in older teens and adults.  You’ve done what you could to prevent it from happening: did a course of steroids, tried increasing their daily medications, worked on lifestyle issues and even sent them to the ED for a cocktail to break the cycle. The ED visit did help but the effect was not sustained, and your patient is back with frequent migraine again. Now what do you do?

I would suggest that this is the time to refer them to a headache specialist, in a clinical setting where there are experienced providers (MD, NP, psychology), skilled at dealing with chronic headache.   Headache programs are usually staffed by a neurologist, or possibly a pain specialist (and rarely by someone who is both) and also a pain psychologist.  Not every neurologist is interested in caring for kids with headaches but pediatric headache programs can be found all across the country. If there are no pediatric headache specialists in your area, there may be an adult provider willing to see an adolescent .  For children, it might be hard to find an alternate provider.  Pediatric headache programs are most often found within a university teaching hospital setting. Some are found within neurology or within a pain (anesthesia) program.

When patients are referred to our headache program, the initial evaluation is done by the pain/neurologist and a pain psychologist.  There are some up front data collection, including psychological measures (FDI, RCMAS-2, CDI-2, fear of pain index, pain catastrophizing scale), done online, prior to the visit. Their medical records are also reviewed. The families see the neurologist first for full history and physical, and then they seen by the pain psychologist- patient and parents, separately and together.  Then a plan is formulated and reviewed with the family by one of our PNPs.  The plan is usually a combination of medications, lifestyle measures, complementary therapies and often a recommendation for psychology for cognitive behavioral therapy (CBT) or physical therapy.

The overriding message to the patients and families is that this is condition that responds best to a multidisciplinary approach, each part of the plan is important. As you all know, each family is different and their expectations and culture vary widely.  Some families are more than happy to change medications, start supplements, even do PT for head and neck stretching and strengthening.  They may rather just focus on lifestyle measures, like working on hydration and sleep.  One of the hardest things to promote is psychology and CBT. Sometimes the family is well aware of mental health concerns, confirmed with the data from the psychological measures and meeting with our psychologist. They are open to the idea and welcome suggestions for where to seek counseling.

But it is often a hard sell to either the parent or patient or both. I encourage that even if there are no serious psychological challenges, having frequent migraine is a source of stress, and can get in the way of participating in life. Learning CBT can provide the teen with better ways to cope with the pain and underlying anxiety and stress. When meeting with particular reluctance/resistance on the part of the teenager, I emphasize that they are not going to ‘talk about your feelings’, but rather to learn concrete skills to use at times of increased pain and stress.  Sometimes the boys are more reluctant (but not always), and the suggestion to see a sports psychologist is better received. There are phone apps that can be used to augment or introduce the CBT exercises. In the end, there is no better way to engage in cognitive behavioral therapy than with a skilled therapist.

Treatments options for chronic migraine:  There are treatments available in a specialty clinic that are not easily obtained in the community. Providers are more familiar with different classes medications used for migraine.  We might be more comfortable in increasing dosing to a more therapeutic level or use medications in combination.  We might also introduce psychopharmacology to the treatment regimen, if indicated. Getting a good medication history is important to decide if the patient actually had an adequate clinical trial of a medication.  Sometimes patient will come in having tried 4-5 daily medications, over the course of 6 months.  Unless there are significant side effects, brief trials are not adequate to determine whether a medication would be helpful. Unfortunately, families and/or patients can have a ‘quick fix’ mentality and inability to tolerate any symptoms or pain, which leads to changing medications before really determining if they work.  Any medication used as a migraine preventive needs to be trialed for at least 2 months, starting low dose and increasing slowly as tolerated. This same approach should be applied to psychopharmacology as well.  This can be hard to communicate to families but necessary.

There are several inpatient options available for chronic or intractable migraine.  Patients can be admitted overnight for the typical migraine cocktail, using ketorolac IV q6h for 24hours, plus adjuncts and steroids. This can help to break a bad cycle, and generally well tolerated.  Also, IV Depakote can be added with mixed effects, followed by 3 days of oral depakote.

Another option is for the patient to be admitted for several days for IV DHE (6 doses, q8h).  This is less well tolerated, with side effects that need to be treated.  Nausea is significant and DHE is generally pretreated with metoclopramide and Benadryl, compazine, ondansetron or even lorazepam.  This can be effective in resetting the migraine pattern back to episodic. Not always pleasant but worth a try.

There are 2 outpatient injection procedures used to decrease the chronicity of migraine.  First there are occipital nerve and trigger point injections, done with local anesthesia (lidocaine and bupivacaine+/- steroids).  The occipital nerve can be inflamed. Trigger points (areas within muscles that are very irritable) will contribute to migraine, other headaches, and myofascial pain.  The area around the greater occipital nerve, as well as any trigger points in the upper cervical, trapezius muscles is infiltrated with local anesthetic.  Initially, patients feel ‘heavy-headed’ or numb, which passes by the next day or so.  The anesthetic medication blocks pain receptors within the nerves surrounding the muscle, thus reducing the pain signals sent to the brain.  Your patient may feel immediate relief of pain, and then (hopefully) a reduction in incidence of migraine.  Some patients have significant improvement with this procedure; some have no benefit at all.  It is generally well tolerated.

Botox© is the only treatment approved by the FDA for chronic migraine for patients over 18 years.  There are strict criteria for its use and generally insurance companies stick to the rules. We have been able to get approval for a few 16 year olds but this is rare. The criteria includes: 15 migraine days/month, migraines lasting up to 4 hours, failure of several preventive and rescue medications. It is not approved for episodic migraine. Another office procedure, the patient will have 155units of Botox© injected via a 30G needle in 31 specific locations on the face, cranium and neck/shoulders, every 3 months. As you can imagine, this is not the easiest thing for a teenager to go through, causing distress and tears. However, in our practice, we have found it to be fairly effective, with decrease in migraine frequency and intensity in many patients, especially after the 2nd procedure.  It is also generally well tolerated.  Most kids tell me that they hate getting the Botox©, but the results make it worth it.  They joke that they know when it’s time to come back when they move their eyebrows again. I was initially skeptical, wondering if it really worked.  But after performing the procedure many times now and seeing the results, almost all positive, I have become a believer.  There is nothing better than getting kids back to functioning.

Your role: So what happens after your patient is seeing a headache specialist? Does this mean you no longer have a role in managing your patient’s migraines? Of course not! As a headache specialist, I enjoy partnering with primary care providers in the overall care of these patients. The families may come from far away, and only come in to see us every 3-6 months. Their community provider is crucial in supporting the families during the difficult time of chronic migraine. We welcome contact, questions and updates.  These families need as much support as they can get, to keep OUR patient functioning, and avoid disability.

So that’s it for now about migraine. I plan to do a case study next to show migraine management in practice.

Daily medications for migraine, part 1

So we have talked about lifestyle habits to prevent migraines, all the things your patient can do to prevent migraines. But your patient is still having frequent migraines or the migraines are significantly impacting the patient’s functioning.  When do you consider adding a daily medication to prevent migraines? The mark I use for deciding when a preventive medication is needed is one migraine/week.  For me, that is when the conversation needs to start.   Any less than that, the kids may be unnecessarily exposed to medication effects and side effects.

And not every kid/teen needs to take a daily preventive medication, even when they have 1 migraine per week. It really depends on how much impact that migraine is having. Are they missing school with every migraine?  Does the migraine last for several days, leading to even more missed school?  Are they missing out on desired activities, like sports, scouts, music or dance? Are they missing out on time with friends and family? Does the patient want to try a daily medication? Is the family in favor or opposed to it? It is a choice that is made after discussion with everyone involved.

However, when the patient is having more than 1 migraine per week, it is a good idea to consider a daily medication, as it is likely that migraine is taking a toll of the patient and family. What you choose depends on the age of the patient and what other issues they are challenged with. Do you start with a prescribed medication or supplements/vitamins?

Supplements:  If you and/or the family are on the fence about starting a medication, a nice intervention to add is a supplement known to help headaches.  We commonly will recommend magnesium, riboflavin or Coenzyme Q10.  I always advocate for using a single product at a time, taking it for at least 3 months and evaluating effectiveness.  Again, you need to keep the good data.   There are many combination products on the market. I have seen one which contains 10 supplements in one tab!  The problem with combination products is that you are unable to figure out what exactly is helping, therefore are ‘married’ to a particular supplement formulation.  And you know that’s going to end up being expensive. A good quality supplement, which can be obtained at the local pharmacy, is totally adequate to try and there are often opportunities to save money on them. These supplements are well tolerated and can be effective.   Here’s a link: Supplements That Help Headaches

One supplement to mention is butterbur, a shrub grown in Europe and Asia, and used as a migraine preventive and for allergies. The concerns with butterbur are that it can be toxic to the liver; the unprocessed butterbur contains chemicals called pyrrolizidine alkaloids (PAs), which can cause liver damage. The only butterbur product you should use is labeled ‘PA- free’, meaning the PA has been processed to remove it. It is unlikely that you would ever find butterbur not labeled this way. Petadolex is the most common butterbur on the market and is PA-free. There are side effects as well such as belching and GI issues, and allergy and asthma, and people allergic to ragweed, chrysanthemums, marigold and daisies should avoid it. Butterbur has fallen out of favor due to report of hepatic issues, though a recent paper has refuted that claim. I have a few patients on it, tolerate it well, and find it helpful, when prescribed medications have not been. I check their LFTs yearly just to be sure.  I mention it here to further your knowledge, as Dr. Google will be sure to mention it, when your families are searching for migraine remedies.

Medications: The aim of daily preventive medication is to decrease the frequency, duration and severity of migraines. Other benefits can include improving responsiveness to the rescue medications and preventing migraines from becoming chronic (vs. episodic). Your choice of medication depends on the patient’s age, medical history/comorbidities, and particular circumstances. Generally all migraine medications for kids and teens are dosed low and titrated up in dose as needed and tolerated.  Groups of medications commonly used for migraine prevention are antihistamines (cyproheptadine), tricyclic antidepressants (amitriptyline, nortriptyline), anticonvulsants (topiramate, zonisamide, gabapentin), beta blockers (propranolol) or calcium channel blockers (verapamil).

Before we talk about the pros and cons of each group of medications, I would like to bring up an interesting research study you may or may not be aware of. A few years ago, the headache program at Cincinnati Children’s Hospital undertook a multi-site double blind medication trial, comparing amitriptyline, topiramate and placebo, for the prevention of migraine in children ages 8-17 years, the CHAMPS study.  The headache program I work in participated in this research project. The study was unusual because it was double blind (no one knew which medication each subject was taking), and it included placebo as one of the treatment arms.  The research study was terminated early because the findings showed no difference between the 3 treatments arms.  The 2 medications both had more side effects than placebo, and there were also serious adverse events in each group. They conclude that “the risk to benefit profile of the two most commonly used preventive medications does not suggest their use as first-line intervention for pediatric migraine.” Basically, they found that placebo was as good as or better than medications and had fewer side effects.  Here’s link to their results: CHAMPS clinical trial publication

This of course presents a dilemma to pediatric providers caring for kids with headaches. You can’t exactly prescribe a placebo and with the family knowing you are doing that, it negates the placebo effect. It is certainly another reason to continue doing research into pediatric headache, and to encourage our families to participate.  We are doing a lot of research at our center, brain imaging, studies looking at the psychological factors impacting episodic and chronic pain, and clinical protocols.  Our families are very interested in participating with research, as they see the value for themselves and for others.

When thinking about daily medications, we all need to be aware that studies are often not done on the pediatric population, just extrapolated from adult data. This is all the more reason to be cautious in prescribing, and encourage appropriate lifestyle measures to decrease the likelihood of migraine.

In my next post, I will review commonly used migraine preventive medications. Some pediatric providers may not feel comfortable starting a daily migraine preventive. But the judicious use of a low dose of medication may really help your patients with their migraines. It may prevent episodic migraine from becoming chronic, and prevent a functioning patient from becoming disabled.  These are all worthy efforts.

Migraine medications to the rescue…..adjunct and combination therapies

There are 3 groups of medications that are used to treat a migraine attack: migraine abortives, analgesia, and adjuncts.  We have covered the migraine abortives (those medications specifically designed to stop the migraine attack) and analgesia (used to reduce pain) in the past 2 posts.   There are several medications that are used either in combination with others to treat the migraine or to treat other symptoms that happen with migraine.  These medications are referred to as adjuncts in migraine therapy, and are often used in combination.  Again, developing an individualized rescue plan is the key to living with migraine, rather than suffering from migraine.  Patients often need several medications for an effective rescue plan.

Adjuncts

Adjunct medications are those that are given with analgesia to complement and enhance the effect and treat other symptoms, such as nausea, dizziness or insomnia.  A classic combination is an NSAID with ondansetron (Zofran), or metoclopramide (Reglan) =/- benadryl, or prochlorperazine (Compazine). I will often encourage taking an adjunct with analgesia at the same time, and if appropriate, with their triptan.

The most common adjuncts are antiemetics, those medications which treat nausea associated with migraine. Antihistamines, such as diphenhydramine (Benadryl), meclizine, and/or hydroxyzine (Atarax) are useful in combination with NSAIDs.  There is the added benefit of drowsiness, to help facilitate restorative sleep.  Ondansetron is also useful for nausea, generally does not cause drowsiness.  Compazine and Reglan are seen as primary agents for treating migraine as well as treating nausea.  The side effects associated with these 2 medications can be avoided by using them with Benadryl.  Dosing is based on weight and age appropriate.

Another option often used in the ED is steroids, such as dexamethasone.  If a patient has had this in the ED, they could use this as an outpatient rescue as well.  Oral magnesium is sometimes used as a rescue medication too.

Treatment Combinations

Many patients develop a migraine rescue strategy, over time and with experience, that involves a combination of medications.  It is always a very individual plan and usually discovered by trial and error.  They might have reasonably good results with a triptan plus antiemetic, or triptan plus analgesia. They may have the best results with an ‘attack pack’ of 3 medications taken together, such as sumatriptan, naproxen and Benadryl.  In cases when a triptan is not effective, NSAID plus antiemetic is a good combination.

The key is to work with the family, figure out what works best, and then BE PREPARED for the migraine to happen.  For younger kids, their parents should always travel with a dose of each of their rescue medications.  They need to have several doses of each medication with the school nurse as well. Older teens can have a prepacked plastic bag with 1 dose of each medication that they can carry with them outside of school.  (Make sure it’s labelled, for obvious reasons).  Most high schools do not allow for self-medication. It is well worth it for the family to have a good relationship and understanding with the school nurse; that when the teen comes to the nurse’s office, they can get their medication promptly and have a chance to lie down and nap for up to 30 minutes at least. Having a clear plan communicated to the school nurse is so helpful.  The workload of our school nurses is tremendous, and having a collaborative relationship with them will make rescue therapy much easier and effective for the kids.

Complementary rescue remedies

In addition to the medications we have talked about, there are a number of complementary interventions that can really help resolve a migraine.

  • Aromatherapy: using an essential oil, such as peppermint, lavender, ginger, or a specially formulated blend (M’Grain from Young Living products) topically, gently massaged on the temples or behind the ears
  • Ice/cold (or heat) applied to the forehead or the neck
  • Gentle massage to the neck and occiput or frontal/temples
  • Anything that activates the relaxation response, such as a CBT or biofeedback exercise, breathing technique, using a relaxation/meditation app, soothing music.  My favorite is the Insight Timer app, so many options and free.

Final rescue thoughts

Having migraine is challenging for the whole family. Migraines often occur at the most inconvenient times, often out of the blue.  It’s important to remember that stress is a trigger for most patients, and stress is not always negative. Positive stress, such as excitement about an upcoming vacation trip, can also trigger migraine.

I also cannot emphasize enough how important it is to treat the migraine promptly- hit it hard and fast- for best results and resolution.  Giving parts of a rescue plan, half-doses spread out over time, usually just prolongs the episode and makes it more difficult to resolve. And there really is no point in prolonging the agony, especially when it is your kid’s pain.

Being prepared, having rescue medications, and extra electrolyte-rich hydration fluids can go a long way in turning a migraine episode from a disaster into a blip on the radar.