Headaches in sickle cell anemia patients may
be caused by any of the common etiologies
or may be early symptoms of several life-threatening disorders, which are more
common in sickle cell anemia patients. These
hemorrhage, or osteomyelitis of the jaw or
skull. Patients presenting with headache need
complete and thoughtful evaluation.
Present Illness. The headache must be
as new or recurrent, unusual,
associated with fever, altered mental status,
location, radiation, character (steady,
throbbing, band-like), onset (day, night,
morning, evening), duration, aggravating/alleviating factors, aura, nausea, vomiting,
family history, stress, or trauma.
Review of Symptoms. Allergies, lacrimation,
nasal congestion, earache, toothache,
pharyngitis, neck pain or stiffness,
photophobia, neurologic symptoms (syncope,
ataxia, weakness, paresthesias,
dysesthesias), insomnia, anorexia, anxiety,
Past Medical History. Document hemoglobin
type, general health, past history of stroke,
hemorrhage, meningitis, seizures, recent
hospitalizations, or surgery, present
Vital Signs. Record temperature, blood
pressure, pulse, respiratory rate.
General. Determine mental status and
amount of distress.
HEENT. Note scalp tenderness, masses,
bruits, TMs, sinus tenderness, EOMs, optic
disk, nasal polyps, gums, pharynx, teeth.
Neck. SUPPLE?!, Define muscle spasm,
Chest. Seek signs of pneumonia.
Heart. Define new heart murmur or
change in heart
Abdominal. Record bowel sounds, liver/spleen size, tenderness.
Neurologic. Do a careful mental status
exam, document orientation x 3.
Meningeal signs - Stiff neck, Kernig’s,
CNs - Document II-XII are
DTRs - normal active and asymmetric,
Muscle strength - asymmetric.
Sensation - intact to light touch.
Coordination - Normal gait, finger/nose,
CBC with differential and
Additional Lab. Chemistry pannel if patient is
hypertensive. Skull x-ray for point
tenderness/masses, fever, or trauma. Sinus
films for sinusitis symptoms and fever/tenderness. CT for
severe headache, altered
mental status, or focal neurologic findings.
Lumbar puncture for fever and stiff neck,
severe or unusual headache, altered mental
status, or meningeal signs. MRI-MRA if not an
- Tension/Psychogenic. Bitemporal, base of
skull, band-like, last hours to days, steady,
pressure, tightness, does not disrupt sleep,
negative exam except for scalp and or neck
tenderness and increased tone in neck
- Sinusitis. Frontal and maxillary pain, around
eyes, throbbing, dull aching, positional, hours
in duration, rhinorrhea, itching eyes, nasal
congestion, exam negative except for
tenderness over sinuses, nasal congestion,
grade fever. Fever suggests acute
- Vascular/ Migraine. Headaches are
frequently initially unilateral, throbbing, may
be associated with nausea, vomiting, and
photophobia. Auramay be present and family
often positive. Precipitated by
stress, menstruation, BC pills. Exam usually
- Cluster. Headaches are unilateral, behind
eye, sharppain, of short duration, male
predominance, repetitive. Exam may show
rhinorrheaon side of pain.
- Hypertension. Headaches are diffuse,
throbbing, worse in morning. Exam shows
elevated blood pressure and retinal changes.
- Meningitis/Encephalitis. Diffuse or posterior
with radiation down neck, severe,
recent onset, chills or fever, lethargy,
vomiting, photophobia, irritability, and new
seizures. Exam may show alteredmental
status, fever, rash, or focal neurologic
findings. Laboratory may show leukocytosis
shift, leukopenia, and DIC. LP shows
leukocytosis with increased neutrophilsor
- Subarachnoid Hemorrhage. Anterior or
diffuse, down neck, acute onset, severe,
unremitting, vomiting, confusion, lethargy, or
Exam may reveal meningeal signs,
or focal neurologic findings. Patients are
usually afebrile. Many patients present only
with an unusual headache and no other
findings. CT usually (about 85%) positive. LP
usually positive for RBCs
xanthochromia. MRA or angiography may
demonstrate multiple aneurysms in adults.
- Osteomyelitis. Localized pain over skull or
jaw, fever, mass may be palpable. Lab reveals
leukocytosis with left shift. X-rays, bone scan,
or gallium scan are positive. Bone scan most
sensitive, gallium scan most specific.
- Bone Infarction. Localized pain over jaw or
skull, NO fever and a mass may be palpable.
Lab reveals normal white count. X-rays are
Bone scan shows
decreased uptake for about a week the
normal or increased uptake. Gallium scan
may or may not be positive. Findings improve
Brain Abscess. Diffuse or localized,
intermittent or constant,
history of sinus, ear, or lung infection. Exam
often shows focal neurologic findings and
source of infection. Lab findings include
leukocytosis with left shift. Brain MRI or CT
are positive. LP usually shows increased
protein, and leukocytosis.
Brain Tumor. Diffuse or localized to area of
mass, intermittent or constant, progression in
severity and duration, personality changes, or
new onset seizures. Exam shows no fever.
Focal neurologic findings
Plan - Treatment
- Tension/Psychogenic. Treatment of non-crisis pain in sickle cell patients is
complicated by previous experiences of
complete pain relief with narcotics. Education
is required to assure
patients that all pain
needs not be treated withnarcotics.
Treatment with plain acetaminophen or
aspirin is all that should be given for these
headaches. Relaxation techniques, mild heat
to neck, and massage will all help greatly.
- Sinusitis. Acute sinusitis with fever and
crisis requires admission for parenteral
antibiotics. Acute sinusitis with fever and no
crisis can be managed with ampicillin500
mg. P.O. q6h or bactrim DS i tab p.o. bid for 7
to 14 days
, Actifed i tab p.o. q 6-8 hours PRN
congestion, and plain acetaminophen 900
mg. p.o. q4 h or aspirin 600 mg. p.o. q 4h
PRN pain. Warn patient about operating
machinery and drowsiness. Acute sinusitis
without fever, as above, without
- Vascular. Migraine should not be treated
with ergotamine, Sumatriptan (Imitrex) or
Zolmitriptan (Zomig) in this population
because of vasoconstriction. Tylenol #3 and
nonsteroidal antiinflammatory drugs
may be required to control pain.
Rest and relaxation techniques, propranolol
or Verapamil (Isoptin, Calan), 80 mg tid or qid
.may reduce frequency. Amitriptyline (Elavil)
or imipramine (Tofranil), 50 to 75 mg in
divided doses or at
effective migraine prophylaxis for some
patients. Cluster headaches are treated as
above but oxygen inhalation at a flow rate of
7L/minute for 10 minutes is said to abort
about 80% of cluster headaches.
headaches respond to control
of the blood pressure.
- Meningitis/Encephalitis. Admit for I.V.
antibiotics based on gram stain.
- Subarachnoid Hemorrhage. Needs
emergency admission to a neurosurgery
service. Patient should
have an exchange
transfusion in preparation for immediate
angiography and possible surgery. Exchange
must be done at isovolemia to prevent arterial
spasm making erythropheresis the method of
- Osteomyelitis. Should be
medicine with neurosurgery consult. If
osteomyelitis is suspected, biopsy for culture
should precede antibiotic administration.
- Brain Abscess. Should be admitted to the
neurosurgical or medical service with a
consult for diagnostic and
- Brain Tumor. Should be admitted to a
neurology or neurosurgical service. Exchange
transfusion should be initiated to prepare for
diagnostic procedures and surgery.
Patient/parent must be taught that severe,
uncharacteristic headaches can be early
signs of neurological problems; and that they
need to be evaluated by healthcare providers
for meningitis, encephalitis and subarachnoid
Patients with chronic benign headache
must be reassured about the nature and
proper management of their problems
Encourage patients totake medicine
before pain becomes too intense
Monitor patients for adverse reaction
Encourage rest and provide quiet
Assess history of sinus and ophthalmology
The main prevention strategy is aggressive
work-up of headaches to diagnose potentially
devastating complications early so that
treatment will be of maximum benefit.
Patient and Parent Education
Patients with benign headaches must be
reassured about the nature andproper
management of their problems. This is
in patients with past episodes of
meningitis, encephalitis and subarachnoid
hemorrhage. Parents must be educated about
the need to have children evaluated for fever,
headache, or changes in mental status