With an unwelcome habit of interrupting life, headaches can appear without warning and follow a path of their own. We've gathered helpful information on this all-too-familiar sensation, reported by 55% of people with Marfan and Loeys–Dietz syndromes in our recent nationwide survey.
A pain anywhere in the head is described as a headache. The location and severity can be clues as to the cause. Causes will be discussed under two headings; general causes, and causes specific to Marfan and Loeys-Dietz syndromes:
WHAT CAN CAUSE HEADACHES
General Causes
Headaches are common, and an important causes of ill health sufficient to warrant absence from work or school. This can be very disruptive.
1. Having a common cold.
2. Stress and fatigue.
3. Drinking too much alcohol.
4. Bad posture.
5. Eyesight problems.
6. Not eating regular meals.
7. Not drinking enough fluid (dehydration).
8. Taking too many painkillers.
9. Occurring during a menstrual period or during menopause (hormone headache).
If headaches are frequent, keeping a headache diary may help you work out what triggers your headache.
How to Ease Headaches Yourself
1. Drink plenty of water.
2. Get plenty of rest.
3. Try to relax.
4. Take over-the-counter medication such as paracetamol or ibuprofen
5. Try to stay at home and avoid contact with other people if you also have a high temperature or do not feel well enough to do normal activities.
See a GP if …
1.Your headache keeps coming back.
2. Painkillers do not help and your headache gets worse.
Migraine
If you have a bad throbbing pain on the side of your head it could be a migraine headache – if you feel sick, vomit, and find light or noise painful.
You regularly get headaches before or during your menstrual period
Get an Urgent GP Appointment or call 111 if …
1. You or your child has a severe headache and jaw pain below the ear when eating.
2. Blurred or double vision.
3. Numbness or weakness in the arms of legs.
4. A headache that gets progressively worse.
Call 999 or go to A&E if you or You or Your Child ...
1. Have a head injury with a headache that comes on suddenly and is extremely painful.
2. Sudden problems speaking or remembering things, loss of vision, feeling drowsy or confused.
Headache Causes Specific to MFS/LDS
1. Temporamandibular Joint Dysfunction which affects up to 52% of MFS patients. This is due to hypermobility of the jaw joint and often occurs in the teenage years. It is a pain which comes with opening or closing your jaw, or when chewing food. The pain is located in the jaw just above the earlobe. Treatment consists of cutting up food into small pieces, or eating soft food temporarily. If the pain persists, the jaw point can be injected for pain relief. See your GP and rheumatologist.
2. Migraine with Aura (warning signs) is increased in Marfan syndrome., The aura is a sign of an incipient headache, and may consist of sights or sounds such as wavy lines, or dots in front of the eyes.
In women these may occur at the time of the monthly menstrual period and are due to decreases in hormone levels which affect the vascular wall contractility. Typically, the arterial wall to the brain constricts producing aura, and then relaxes permitting increased blood flow into a previously deprived part of the brain. This produces the typical throbbing headache which can be very severe and can be accompanied by vomiting. They are treated by resting in a dark room, and taking specific medication such as Imigran. If you find a medication which works, it should be carried with you and taken at the first sign of aura. This may prevent the full-blown headache.
3. Post-Operative Headache or Headache with Severe Exertion. If cardiac surgery normalises the diameter of the aorta, and increases blood delivery through the aortic valve to the head and neck, a headache due to blood pressure may develop. This can be treated post-op and usually improves with time. If headache occurs with severe exertion due to increased blood pressure or heart rate, the amount of exercise should be reduced to moderate level. The headache is due to increased blood pressure and delivery of more blood than usual into a network of arteries with relatively thin walls. The expansion of the walls and delivery of blood into the system causes the headache. Strenuous sports like weight-lifting and long-distance running are not recommended.
4. Sinusitis. This head pain occurs above or below the eyes due to infection of sinuses which are narrower than normal, and also have narrow drainage channels into the nose. This can be treated with nose drops, steam inhalation, rest, antihistamines (such as Piriton or Clarityn) and if the discharge looks infected (yellow or green) antibiotics should be sought from your GP. If your sinusitis is recurrent, referral to an Ear, Nose and Throat Specialist for treatment is recommended.
5. Postural Headache. Spinal scoliosis is common in MFS & LDS (70) and this can cause rounded shoulders which means the head has to be held up to correct posture. This can cause strain on the neck muscles, and pain where they attach to the skull. If the scoliosis extends to involve the neck this can cause pressure on the nerves to the scalp, again producing headache. Suggestions are to wear a soft neck brace with Velcro fastening ((available from your chemist), especially at night, and to adjust pillow height and support for the neck.
6. Intracranial Aneurysms. These are rare in Marfan syndrome, but more frequent in Loeys-Dietz syndrome. An aneurysm may rarely present as headache, but would be picked up on an MRI scan of the head and neck.
Sources:
Headache Management NHS website
The Migraine Trust has advice about what to record in a headache diary
