Characteristics of "Older Old" Patients with Sickle Cell Anemia Who Survived for More Than 80 Years

Survival of patients with sickle cell anemia (SS) has increased progressively since the 1990's. Newborn screening, prophylactic antibiotics, newer vaccines, safer blood transfusion, chelation therapy and hydroxyurea are some reasons for this change in longevity. Patients with SS in their sixth or seventh decade have been described previously. This report describes two of our patients with SS who survived beyond the age of 80 years.

The first patient is an 81 year old African American woman with SS that was first diagnosed at the age of 8 years. She used to have frequent vaso-occlusive crises (VOC) during childhood and young adulthood. Crises became less frequent with age and she had one VOC only during the last 15 years. Past medical history was significant for status post (S/P) cholecystectomy & appendectomy, congestive heart failure, avascular necrosis of the right hip, S/P right hip replacement, S/P laser therapy for retinopathy, cataract, glaucoma, history of leg ulcers, history of tuberculosis, hypertension, pneumonia, respiratory failure, pacemaker implantation secondary to complete heart block and iron overload due frequent blood transfusions over the years. At the age of 79 years she had mild Parkinson's disease. On physical exam her vital signs were within normal. Pertinent findings included Grade II/VI systolic heart murmur and scars of previous surgeries and of leg ulcers. Medications included folic acid, antihistamines, furosemide, verapamil, laxatives, multivitamins, baby aspirin, proton pump inhibitor; eye drops for glaucoma, acetaminophen with codeine for pain and Ropinirol for Parkinson's. Iron chelation with desferasirox was not successful due to increase in serum creatinine and was replaced with deferiprone. Her usual hemoglobin (Hb) was 7.0-8.5 g/dl, hematocrit (Hct) 22-25.5%, platelet and white blood cell counts (WBC) within normal and Hb F that varied between 3.1 and 14% over the years.

The second patient was a Caucasian woman of Italian ancestry known to have SS that was first diagnosed at the age of 19 when she presented with VOC. Since then she experienced frequent VOCs that were treated mostly at home or in the Emergency Department and occasionally required hospital admissions. Complications of her disease included generalized avascular necrosis which was most severe in her hips and knees and less severe in her shoulders and wrists. She was S/P bilateral hip replacement, right knee replacement and S/P total right hip revision done about 10 years after the previous hip replacement. Other complications over the years included bilateral sickle retinopathy managed with laser therapy, bilateral cataracts treated surgically, mitral valve prolapse, papillary necrosis, urinary tract infections, irritable bowel syndrome, osteoporosis, and anxiety disorder. In her seventies she had paroxysmal atrial fibrillation treated initially with Coumadin and later prophylactically with amiodarone, mild myocardial infarction and a small ankle ulcer that healed after standard therapy. Physical exam was remarkable for grade II/VI apical murmur and scars of previous surgeries. Medications included a broad spectrum of medications that included folic acid, calcium, vitamin D, opioid and nonopioid analgesics, proton pump inhibitors, antibiotics and anxiolytics. Blood transfusions were given sporadically when needed. Her Hb was 7.5-8.5 g/dL and Hct was 23-27% most of the time, WBC and platelet counts were within normal when in the steady state but the WBC count increased to 14-16,000 /uL during VOCs. Fetal Hb varied from 7 to 12 % over the years. She died recently from cardiac complications at the age of 86 years.

Both patients had excellent family support, were compliant with their medications and kept regular follow-up clinic visits over the year. Their WBC counts were within normal most of the time. These factors may have contributed significantly to their longevity.