Sickle Cell Information Center Guidelines

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

 Edema by Hiba Tamim, MD

Edema is included as a separate problem not because it is specific to sickle cell syndromes, but because it occurs frequently. If not managed aggressively, edema in the low extremity predisposes the patient to leg ulcers and other complications. Edema is most often benign, however, it can also be the first manifestation of serious complications.

Subjective Data

Present Illness. Note onset of problem, new, recurrent, time of day, symmetric, involvement of hands and feet. erythema, pain, itching, fever/chills. Define present medications, last narcotic treatment, or treatment with chronic oral narcotics.

Past Medical History. Document history of phlebitis, recent hospitalizations, surgery, past abdominal or pelvic surgery. Renal disease, hypertension, myocardial infarctions.

Review of Symptoms. Ask about dyspnea on exertion, PND, orthopnea, angina, chest pain, alcohol abuse, cirrhosis, abdominal swelling, RUQ pain, pharyngitis, frequency, nocturia, diabetes, renal disease or proteinuria.

- General. Distress, palmar erythemia, spiders, nail changes.

- Vital Signs. Respiratory and pulse rate, blood pressure, temperature.

- HEENT. Course hair, loss of lateral 1/3 of eyebrow , periorbital edema, icterus, retinal vascular changes.

- Neck. Increased thyroid, lymphadenopathy.

- Chest. Effusions, rales, rubs.

- Cardiac. Increased neck veins, V waves, PMI, RV lift, increased P2, S3, murmurs.

- Abdomen. Hepatosplenomegaly, ascites, masses.

- Pelvic/Rectal. Masses, guaiac.

- Extremities. Edema symmetric, pitting, tenderness, cords, erythemia, varicosities, leg ulcers, stasis changes, nodes.

- Neurologic. DTR relaxation.

- Minimum Lab. CBC, UA, Chest x-ray, Chemistry pannel.

- Additional Lab. ECG if cardiac disease signs. Consider a 24 hour urine for protein and creatinine clearance and renal biopsy for proteinuria. Do a T4 and TSH if hypothyroid signs. Consider Doppler flow study, nuclide or contrast venogram if phlebitis is suspected.

Differential Diagnosis

- Orthostatic edema. Many patients will develop orthostatic edema which is aggravated by hot weather and prolonged standing or sitting. Patients receiving regular administration of oral narcotics, especially long acting preparations, will develop symmetric pitting edema.

- Congestive Heart Failure or Cardiomyopathy. Right heart failure cause edema. Neck vein distension, hepatomegaly, rales, S3, cardiomegaly are characteristic findings.

- Renal Disease. Edema occurs with fluid retention of renal failure and hypoalbuminemia of nephrotic syndrome. More frequently involves face and upper extremities. UA and SMA 18 may provide diagnostic findings. If there is proteinuria, 24 hour collection is indicated to define the present severity and as a baseline for follow-up.

- Liver Disease. Seen with hypoalbuminemia, portal hypertension, and increased fluid retention of liver disease. Signs of hepatic failure on physical and laboratory examination are often present but may be subtile. Common etiologies include chronic hepatitis, iron overload, and alcohol.

- Hypothyroidism. May manifest pretibial myxedema. Thyroid studies are usually diagnostic.

- Venous Insufficiency/Obstruction. Chronic venous insufficiency, postphlebitic syndrome, or acute phlebitis may cause symmetric or asymmetric edema.

- Lymphatic Obstruction. Brawny symmetric or asymmetric edema may be caused by tumor, lymphoma, infection, or fibrosis causing lymphatic obstruction.

Treatment

- Orthostatic Edema. Reassurance, sodium restriction, elevation, weight loss, and support stockings are the effective treatments. These should be aggressively applied to patients with active or inactive leg ulcers.

- Congestive Heart Failure / Cardiomyopathy. See Congestive Heart Failure.

- Renal Disease. Use control of blood pressure sodium restriction, treat cause of renal disease, diuretics in early disease. Dialysis or renal transplantation may become necessary as the disease progresses. See Hematuria - Nephropathy.

- Liver Disease. Use sodium restriction and treat cause of liver disease if possible.

- Hypothyroidism. Replacement therapy with thyroid hormone is indicated. Start with low dose and increase very slowly in these patients to prevent relative hypermetabolism, thereby, making the sickle cell disease worse.

- Venous Insufficiency/Obstruction. For acute phlebitis, admit for anticoagulation therapy as in any patient. Chronic post-phlebitic or venous insufficiency is managed with leg elevation, support stockings, weight loss, and sodium restriction.

• Lymphatic Obstruction. Determine the cause of the obstruction and treat if possible.

Nursing Considerations:

 Patient must be taught measures of avoiding orthostatic edema

 Teach the patient the value of proper nutrition, sodium restriction, and fresh foods

 Monitor fluid retention, intake, and output, with daily weight

 Instruct patient to rest in supine position with legs elevated for several hours each day

 Remind patients that elastic support stocking should be put on before arising each morning

 Instruct patients with sickle cell syndromes that renal and heart failure become more common with advancing age

Prevention and Patient Education

Edema in the lower extremities seems to be one of the most common antecedents to leg ulcers in our patient population with previous or active leg ulcers. Patients must be educated to prevent orthostatic edema. Renal and heart failure are becoming more common in the older patients with sickle syndromes. Treatment of hypertension, renal infection or other treatable diseases should be vigorous. Transfusions should be used judiciously to prevent iron overload and alcohol should not be used by sickle patients. Vaccination for viral hepatitis often indicated.

Schrier RW The Edematous Patient - Cardiac Failure, Cirrhosis, and Nephrotic Syndrome in Manual of Nephrology Schrier RW editor Lippencott Williams Wilkins, 1995.

Buckalew VM Somern A. Renal manifestations of sickle cell disease. Arch. Intern. Med. 133:660-669, 1974.

Alleyne GAO, Statius Van Eps LW, Addae SK et al. The kidney in sickle cell anemia. Kidney Int. 7:371-379, 1975.

Barakat AY, Noubani H. The kidney in hematologic disease. A Review. Int. J. Pediatr. Nephrol. 67:46-49, 1986.

Allon M. Renal abnormalities in sickle cell disease. Arch. Intern. Med. 150:501-504, 1990.

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