Sickle Cell Information Center Guidelines

Edited by James Eckman, M.D. and Allan Platt, PA-C

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 include meningitis, subarachnoid hemorrhage, or osteomyelitis of the jaw or skull. Patients presenting with headache need complete and thoughtful evaluation.

Subjective Data

Present Illness. The headache must be characterized 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, stress.

Past Medical History. Document hemoglobin type, general health, past history of stroke, hemorrhage, meningitis, seizures, recent hospitalizations, or surgery, present medications, drug allergies.

• 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, adenopathy, thyroid.
• Chest. Seek signs of pneumonia.
• Heart. Define new heart murmur or change in heart murmur.
• 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, Brudzinski.
• CNs - Document II-XII are intact.
• DTRs - normal active and asymmetric, negative Babinski.
• Muscle strength - asymmetric.
• Sensation - intact to light touch.
• Coordination - Normal gait, finger/nose, heal/shin, Rhomberg.

Minimum Lab. CBC with differential and reticulocyte count.

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 acute situation.

Differential Diagnosis

- 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 muscles.

- 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, and low grade fever. Fever suggests acute sinusitis.

- Vascular/ Migraine. Headaches are frequently initially unilateral, throbbing, may be associated with nausea, vomiting, and photophobia. Aura may be present and family history is often positive. Precipitated by stress, menstruation, BC pills. Exam usually negative.

- Cluster. Headaches are unilateral, behind eye, sharp pain, of short duration, male predominance, repetitive. Exam may show redness, tearing, rhinorrhea on 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, throbbing, recent onset, chills or fever, lethargy, vomiting, photophobia, irritability, and new seizures. Exam may show altered mental status, fever, rash, or focal neurologic findings. Laboratory may show leukocytosis with left shift, leukopenia, and DIC. LP shows leukocytosis with increased neutrophilsor lymphocytes.

- Subarachnoid Hemorrhage. Anterior or diffuse, down neck, acute onset, severe, unremitting, vomiting, confusion, lethargy, or seizures. 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 and/ or 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 usually negative. Bone scan shows decreased uptake for about a week the normal or increased uptake. Gallium scan may or may not be positive. Findings improve without antibiotics.

Brain Abscess. Diffuse or localized, intermittent or constant, progressive, fever, 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 and papilledema are common.

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 with narcotics. 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 ampicillin 500 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 4 h PRN pain. Warn patient about operating machinery and drowsiness. Acute sinusitis without fever, as above, without antibiotics.

- 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 (NSAIDs) 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 bedtime, provides effective migraine prophylaxis for some patients. Cluster headaches are treated as above but oxygen inhalation at a flow rate of 7 L/minute for 10 minutes is said to abort about 80% of cluster headaches. Hypertensive 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 choice.

- Osteomyelitis. Should be admitted to 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 neurosurgical consult for diagnostic and therapeutic drainage.

- Brain Tumor. Should be admitted to a neurology or neurosurgical service. Exchange transfusion should be initiated to prepare for diagnostic procedures and surgery.

Nursing Considerations:

 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 hemorrhage

 Patients with chronic benign headache must be reassured about the nature and proper management of their problems

 Encourage patients to take medicine before pain becomes too intense

 Monitor patients for adverse reaction to medication

 Encourage rest and provide quiet environment

 Assess history of sinus and ophthalmology symptoms

 

Prevention

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 and proper management of their problems. This is difficult 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.

References

Wertham F, Mitchell N, Angrist A. The brain in sickle cell anemia. Arch. Neuro. Psych. 47:752-767, 1942.

Greer M and Schotland D. Abnormal hemoglobin as a cause of neurologic disease. Neurology 12:114-123, 1962.

Portnoy BA and Heroin JC. Neurologic manifestations in sickle-cell disease. Ann. Intern. Med. 76:643-652, 1972.

Sarnaik SA, Lusher JM. Neurological complications of sickle cell anemia. Amer. J. Pediatr. Hemat./Oncol. 4:386-394, 1982.

Sampson I, Pratila M, Pratilas V. Anesthetic management of cerebral aneurysmectomy in a sickle cell anemia patient. Mount Sinai J. Med. 49:326-328, 1982.

Powars D, Adams RJ, Thomas C. et al. Delayed intracranial hemorrhage following cerebral infarction in sickle cell anemia. J. Assoc. Acad. Minority Phys. 1:79-82, 1990.

Oyesiku NM, Eckman JR, Barrow DL et al. Intercranial aneurysms in sickle cell anemia: Clinical features and pathogenesis. J. Neuro. Surg. in press.

Preul MC, Cendes F, Just N, Mohr G Intracranial aneurysms and sickle cell anemia: multiplicity and propensity for the vertebrobasilar territory Neurosurgery 1998 May;42(5):971-7; discussion 977-8

Klapper JA. Toward a standard drug formulary for the treatment of headache. Headache 1995;35:225.

Silberstein SD. Preventative treatment of migraine. Cephalgia 1997;17:67.

Resar LM, et al. Skull infarction and epidural hematomas in a patient with sickle cell anemia. J Pediatr Hematol Oncol. 1996 Nov;18(4):413-5

Pari G, et al. Headache and scalp edema in sickle cell disease. Can J Neurol Sci. 1996 Aug;23(3):224-6

Send mail to aplatt@emory.edu with questions or comments about this web site.
Copyright © 1997 Sickle Cell Information Center
Last modified: October 08, 2000