Treatment decisions

After a careful clinical assessment, including a full history and tailored examination, decide on the most likely diagnosis and the important differentials. This will usually require no further investigations, and will allow you to give the child and their family reassurance and sensible advice. Occasionally, and especially when faced with an unclassified headache, for example, a non-specific chronic daily headache with no features to further define the diagnosis, investigations for symptomatic headache will be indicated.9 Box 1 summarises the key ‘red flags’, warning symptoms and signs, suggestive of a secondary headache, as does the HeadSmart campaign.10

Advising no medication

The most important consideration is that, very often, it is best to recommend no medication. A mild headache (see box 2)8 may cause concern even when the pain is slight and not interfering with function and participation. In this situation, clinical assessment, diagnosis, explanation and reassurance will usually address the concerns.

Often the child or young person will already have found out that simple analgesics (paracetamol or ibuprofen) have not helped and will be, quite rightly, unwilling to use them. Primary stabbing headache8 is an intense focal head pain lasting 1–3 s and occurring unpredictably without any trigger or autonomic symptoms or signs. It often occurs in people with migraine without aura, at a site within their usual migraine site, typically frontally. It is too short lived and unpredictable to treat, but diagnosis and explanation do help. If frequent, indomethacin, as for paroxysmal hemicrania (see section ‘paroxysmal hemicranias’ within the section ‘trigeminal autonimic cephalalgias’), is worth considering. New daily persistent headache8 is usually refractory to medication. However, in general it is worth checking the doses used in case a sufficiently high dose has not been tried. In patients with primary headache, however, be sure to advise them about medication overuse headache (MOH, see box 3).8 Use the doses in your recommended reference source, for example, in the UK the British National Formulary for Children (BNF C)11 unless otherwise stated.

Preventing medication overuse headache

MOH is common, especially in people with primary headaches, for example, migraine without aura or episodic tension-type headache,8 which becomes transformed by frequent use of any single analgesic into a chronic daily headache, less intense but just as upsetting as the original primary headache. It can be explained as a ‘rebound’ headache, in people where the pathology is in the pain pathways rather than due to tissue damage. All patients with a primary headache should be warned about this and prescriptions for analgesics should state as well as the dose and maximum dose that can be taken in 24 hours, not to use them on more than 2 (or occasionally 3) days a week.

Non-drug treatments and advice

A variety of physical, complementary and lifestyle interventions are available, mostly with little empirical evidence of efficacy. However, acupuncture is included in the NICE guideline for chronic tension-type headache and as a second-line treatment for migraine prophylaxis.7 Low-risk common sense advice has also traditionally been recommended including healthy lifestyle habits (see box 4). We routinely encourage the use of distraction (especially for mild and moderate headaches), cold or warm compresses and balms applied to the forehead and/or temples, for example, ‘Tiger Balm White’, ‘4-Head’, etc. Excess caffeine, aspartame, monosodium glutamate, nitrites, alcohol and chocolate can cause headache (and vomiting); however, the role of exclusion diets per se in childhood migraine is still unproven.

General acute/rescue treatments

Simple ‘over-the-counter’ analgesics, paracetamol and/or ibuprofen are first-line treatments for symptomatic and most primary headaches, especially episodic tension-type headaches.7 Opiate analgesia should be offered for severe symptomatic headaches where there is known tissue damage, for example, for postoperative pain, but is not recommended for primary headaches. It seems less effective and more prone to dose escalation and dependency in patients with primary headaches, with associated incapacitating sedation, constipation, nausea and itching. See tables 1⇓–3 for most useful medicines for migraine with or without aura.

Give the child or young person a headache diary to use.12 This will help establish their headache diagnoses and whether the current acute/rescue and prophylactic treatments are working.

Migraine with or without aura

Triptans are a class of serotonin (5-hydroxytryptamine) 1B/1D/1F receptor agonists, which exert a particular antimigraine attack effect, probably at the level of the trigeminovascular system and its connections.5 Offer a triptan taken together with ibuprofen or paracetamol (or both) for attacks of migraine with or without aura.7 These combinations are more effective and cost-effective than using either in isolation.7 A nasal triptan, for example, sumatriptan or zolmitriptan will have a more rapid onset of action than oral or orodispersible formulations. The triptan can be repeated after 2 hours if required, but give the patient and parent/carer clear instructions on the maximum dose, for example, “two doses in 24 hours and on no more than two days a week”. A nasal triptan may not be tolerated because of the ‘taste’ or, very rarely, it can produce an unpleasant ‘feeling’ or ‘dizziness’. If this happens, it is worth trying an alternative triptan or formulation. If tolerated, a triptan should be tried for three different attacks before giving up for lack of efficacy. Three different triptans should be tried before accepting that this class of medicine is ineffective for a particular patient.

Although sumatriptan and zolmitriptan are the only triptans currently in the BNF C,11 and only sumatriptan has marketing authorisation (a ‘licence’) for young people aged 12–18 years, there are no particular contraindications to triptan use in this population or younger children, for example, of primary school age. We will occasionally use triptans, in the smaller doses available, in younger children ‘off-label’, in accordance with the UK Royal College of Paediatrics and Child Health advice to use the best treatment available irrespective of pharmaceutical company marketing strategies, especially when no ‘licensed’ product of that class is available.13

Many prescribers avoid triptans and similar vasoconstrictor drugs in hemiplegic migraine, a particular type of migraine with aura,8 even though a link to migrainous infarction or ischaemic arterial stroke has not been observed.14

There is no particular contraindication to oral contraceptive use by young people with migraine without aura; however, specialist advice should be sought for those with migraine with aura.7

Antiemetic medication is often needed early on in a migraine attack, together with specific treatment for the headache (as above), for example, cyclizine, domperidone, prochlorperazine. Metoclopramide is particularly associated with acute dystonic reactions including oculogyric crises, and is now only indicated as second-line treatment for postoperative or chemotherapy-related nausea and vomiting. We have therefore omitted metoclopramide and the paracetamol-metoclopramide combination (Paramax) from table 2. However, if a patient has previously benefitted from metoclopramide without adverse effect, it should not be withheld. Another analgesic-antiemetic combination in frequent use (Migraleve) contains codeine, which is not generally recommended for primary headaches. There are a range of formulations for different routes of administration (see box 5).

Occasionally, vomiting and incapacitation are so severe in a migraine attack that the child or young person will need emergency department assessment and treatment with intravenous fluids for dehydration, and a parenteral triptan and a parenteral non-steroidal anti-inflammatory drug (NSAID) or paracetamol, and parenteral antiemetics.

Treating medication overuse headache

MOH (see box 3 for diagnostic criteria) is best treated by abrupt withdrawal of the likely causative analgesic or triptan.7 Warn the patient that headaches may get worse over a couple of weeks before the chronic daily headache improves. Physical treatments and lifestyle advice (see section on non-drug treatments and advice) can help, and sometimes migraine prophylaxis will be appropriate during and following withdrawal (see section prophylactic treatment for migraine).

Trigeminal autonomic cephalalgias

Although comparatively rare in children, the trigeminal autonomic cephalalgias (TACs)8 are important to recognise as they are very painful and incapacitating, and their pathology and treatment is different from migraine. However, effective first-line treatment is available, so if you are unfamiliar with them do consider referring to a paediatric or adult headache clinic, depending on the patient's age and local resources, for diagnostic and treatment advice.

Cluster headache,8 especially when lacking some of the typical features (ie, probable cluster headache), is seen more often in young people aged 12–18 years than in younger children, and may be mistaken for an epilepsy, or psychogenic non-epileptic attacks, or other medically unexplained illness behaviour.15 High flow (12 L/min) 100% oxygen by a non-rebreathing face mask with a reservoir bag is usually very effective acutely. When indicated, home and ambulatory oxygen will need to be provided.7 Triptans, subcutaneously or nasally can also help.7

Paroxysmal hemicranias8 is another TAC experienced rarely in children from preschool to secondary school ages, which is very effectively treated with indomethacin (combined with omeprazole, or ranitidine or similar). Response to indomethacin is so typical that it is formally a diagnostic criterion,8 so children with some but not all the typical features should be tried with indomethacin, for example, for 2–3 weeks.

Prophylactic treatment for migraine

Because episodic tension-type headache, migraine without aura and migraine with aura are on a continuum,16 it is unusual to find someone with episodic tension-type headache requiring prophylactic treatment who does not also have migraine with or without aura. Similarly, chronic tension-type headaches8 would not usually require prophylactic treatment, but if it did the diagnosis should be reviewed, thinking of a symptomatic headache (eg, idiopathic intracranial hypertension, sinusitis, etc), or more likely chronic migraine.8

When incapacitating headaches, particularly migraine with or without aura, occur three or four times a month or more, depending on their severity and impact, offer a daily prophylactic medication. The choice will depend on preferences and comorbidities. Most drugs should be titrated up if starting low doses prove to be ineffective, and the drug should be reduced or abandoned if there are adverse effects or relatively high doses prove to be ineffective. Avoid combinations of prophylactic treatments, except during switching (see table 4).

Topiramate is effective for patients with chronic daily headache, for example, in adults with chronic migraine.19 It is also a first-line prophylactic treatment for migraine with or without aura in younger children,20 as well as in young people and adults.7 Although recommended doses are lower than those often used for epilepsies, these patients are at no more risk of adverse effects than children with epilepsies. The risk of teratogenicity in young women is significantly less than for sodium valproate.

Other drugs with reasonable evidence of efficacy in migraine in children and young people include propranolol and gabapentin.7 Some weaker evidence is also available for valproate,7 flunarizine,17 ,18 amitriptyline7 and riboflavin.7 Unfortunately, there is no good evidence for pizotifen's efficacy7 despite being a ‘licensed’ and popular treatment in the UK and it certainly seems to work well for some children and young people, in the authors' experience. Moreover, as it is now off-patent, it seems unlikely that a properly powered trial will ever be funded, unless a novel formulation is developed and patented.

Menstrual-related migraine

Some young women have predictable menstrual-related migraine.8 If first-line acute/rescue treatments are ineffective and/or not tolerated, consider a triptan twice or three times a day, on the days migraine attacks are expected, even if that is a few consecutive days. Good evidence for this approach exists for zolmitriptan and frovatriptan.7

Prophylactic treatments for TACs

Cluster headache and probable cluster headache in children and young people are generally best diagnosed and treated in specialist paediatric or adult headache clinics. There is good evidence to support verapamil as a prophylactic.7

Paroxysmal hemicrania responds within a few days of reaching an effective dose of indomethacin.8 This is true for some cases not fulfilling all the diagnostic criteria, that is, probable paroxysmal hemicrania. Treatment should be combined with omeprazole or ranitidine or similar drug to reduce the risk of gastrointestinal adverse effects, particularly ulceration and bleeding. It can be continued for 2–3 weeks then stopped, but will need to be reinstated promptly when the paroxysmal hemicrania relapses. The paroxysmal hemicrania may settle in months or continue for years. Relatively low maintenance doses can often work.21 If indomethacin is not tolerated, further expert advice should be sought.