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.

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.