Sickle Cell Information Center Protocols

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

by Antonio Guasch, MD

Patients with sickle syndromes have blood pressure lower than normal compared to age and sex matched controls. Those with high values relative to this population had an increased risk of stroke and death. Blood pressure should be monitored but values obtained must be assessed relative to the lower values expected for patients with this disease. Those with blood pressure values above 140/90 mm Hg should be evaluated for secondary causes and considered for treatment. Except in cases of extreme BP elevation (systolic BP greater than 200 mm Hg, diastolic BP greater than 110 mm Hg, or both) or elevated BP with evidence of ongoing target-organ damage, hypertension should not be diagnosed on the basis of measurements made on a single occasion. Hypertension is diagnosed when at least two separate readings obtained at least 1 to 2 weeks apart average 140/90 mm Hg. Patients should abstain from tobacco use and caffeine ingestion for at least 30 minutes before the BP measurement is taken. Use of an appropriately sized cuff, in which the bladder encircles at least 80% of the arm, is essential because a cuff that is too large or too small will result in falsely low or falsely high readings, respectively.

Present Illness. Note onset of symptoms, presence of edema, dyspnea on exertion, PND, orthopnea, chest pain.

Past Medical History. Document history of murmur, rheumatic fever, hypertension, myocardial infarction, cardiac evaluation, kidney disease, diabetes, thyroid disease, cardiovascular risk factors (i.e., hyperlipidemia, alcohol and tobacco use, obesity, sedentary lifestyle, glucose intolerance, and insulin resistance)

Review of Symptoms. diet/salt intake, caffeine intake, smoking, meds: birth control pills, estrogens, decongestants, diet pills, alcohol, hematuria, frequency, nocturia, goiter, weight loss, anxiety, heat intolerant, headaches, palpitations, diaphoresis

Physical Examination

General Inspection: Truncal obesity, moon face, striae, increased facial hair, bufallo hump (R/O Cushings)

Vital Signs. Respiratory and pulse rate, blood pressure sitting and standing, in both arms and pressure in one calf to rule out coactation, temperature, weight.

HEENT. Exophthalmos, lid lag, retinal vascular changes.

Neck. Bruits, thyroid enlargement, venous distension, venous waves, V waves, Kussmaul’s sign.

Chest. Effusion or basilar rales.

Cardiac. PMI, RV lift, ventricular filling wave, thrills, S1, S2 (P2 > A2), S3, S4, murmurs, or rubs.

Abdomen. Listen for renal bruits, hepatomegaly, pulsations, ascites or enlarged kidneys.

Extremities. Edema, peripheral pulses.

Minimum Lab. CBC with reticulocyte count, Chest x-ray, ECG, SMA 18, Urinalysis. 24 hour urine protein,

Additional lab. Kidney ultrasound, thyroid studies, urine vma/metanephrins-(r/o pheochromocytoma) urine 17 - hydroxycorticosteroid - (R/O Cushings)

 

Substances: Estrogens, sympathomimetics, caffine, narcotic and alcohol withdrawal can elevate blood pressure

Renal Disease- Renal Failure can cause hypertension. Proteinuria, hematuria, oligouria, edema, elevated BUN and creatinine may be present.

Myocardial Disease. As patients are living longer, atherosclerotic heart disease and other causes of myocardial dysfunction are more common. Occasionally young patients present with myocardial ischemia or infarction. Findings of diffuse cardiomegaly, ECG changes of damage, poor contractility or hypodynamic areas on ECHO, and thallium exercise scans may be positive.

Essential Hypertension. No other cause is found

Renovascular Hypertension. Renal Bruits may be present on physical exam.A precipitous drop in BP, acute deterioration in renal function in response to ACE inhibitor therapy, or both suggest possible renovascular and warrant further workup.

Hypertensive Heart Disease. Although hypertension is rare in patients with sickle cell anemia, it occurs with normal frequency in patients with other sickle syndromes. Older patients present with heart failure secondary to myocardial disease and renal insufficiency from poorly controlled hypertension. -

Hyperthyroidism. Mild hyperthyroidism can cause decompensation because of the high output state from the anemia.

Error in Cuff size. Using a cuff size that is too small can falsely elevate blood pressure..

Valvular Heart Disease. Flows murmurs from high cardiac output secondary to anemia are difficult to differentiate from murmurs of valvular heart disease. In addition, many patients carry the diagnosis of rheumatic heart disease because flow murmurs with symptoms from sickle cell disease are mistaken for rheumatic fever in childhood. Insufficiency murmurs are usually related to valvular disease. Some reports suggest that mitral valve prolapse may be more frequent, however, this observation may relate more tothe prevalence of this anomaly in the general population. ECHO may help determine the true etiology of the murmur.

Coarctation of the Aorta. Unequal pressure readings in the upper and lower extremities may be caused by coarctation.

Rare Causes- Pheochromocytoma causes pressure elevations, tachycardia, sweating and elevated urine urine vma/metanephrins.Cushing Syndrome causes truncal obesity, moon faces, hypokalemia and elevated urine 17 - hydroxycorticosteroids.

Essential Hypertension. Lifestyle modifications of proven benefit in antihypertensive treatment include weight reduction, moderation of alcohol intake, increased physical activity, maintenance of recommended levels of dietary calcium and potassium, and moderation of dietary sodium. Cessation or avoidance of smoking is also critical because smoking is an important independent risk factor for cardiovascular disease and may interfere with the related benefits of antihypertensive therapy. Treatment hypertension in the sickle cell patient should miniimize water loss and preserve glomerular filtration. ACE inhibitors are the treatment of choice to

Thyroid Disease. Treatment requires admission for careful administration of beta lockers, , and endocrinology evaluation. Because of the potential for severe aggravation of the sickle syndrome, aggressive treatment to control the hyperthyroid state is indicated. Radioiodine administration allows excellent control in most patients.

Hypertension should be treated aggressively in the patients with elevated blood pressure.

Patients and their families sould understand the importance of good blood pressure control in preventing renal damage and stroke. Regular blood pressure checks should be a part of monitoring.

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Denenberg BS, Criner G, Jones R,and Spann JF. Cardiac function in sickle cell anemia. Am. J. Cardiol. 51.1674-1678, 1983.

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The sixth report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure (JNC VI). Arch Intern Med 157:2413,

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Copyright © 1997 Sickle Cell Information Center
Last modified: June 08, 2001