The availability of effective virally-attenuated clotting factor concentrates and the increased use of prophylaxis has reduced mortality in individuals with hemophilia. Consequently, the bleeding disorders community must focus on the medical needs of an aging population. Older hemophiliacs may not be receiving appropriate screening and preventive care for the common ailments of aging. Similarly, the older segments of the hemophilia population may be predisposed to obesity and cardiovascular disease because they never received primary prophylaxis to prevent hemarthropathy.
To review the hemophilia-related and non-hemophilia-related medical problems of aging, including bleeding complications of invasive procedures, in the adult hemophiliac population >18 years (yrs).
A retrospective chart review of active adult patients (pts) with hemophilia A and B (HA, HB) receiving care at the Hemophilia Center, was conducted. Medical conditions including Hepatitis C (HCV), HIV, diabetes (DM), hypertension (HTN), hypercholesterolemia (HYC), coronary artery disease (CAD), obesity, arthropathy and psychiatric disorders were identified. Body mass index (BMI) was calculated in kg/m2. Invasive procedures and health maintenance screening were reviewed for bleeding complications.
We identified 58 adult pts with a mean age of 37 yrs (18-81 yrs). 30/58 (52%) are > 30 yrs. 47/58 (81%) have HA and 11/58 (19%) have HB. 30/58 (52%) have severe hemophilia. 32/58 (55%) have HCV. 10/32 (31%) HCV+ pts have received antiviral therapy, and 4/10 (40%) eliminated the virus. 9/58 (16%) have HIV, of whom 4/9 (44%) are currently receiving antiretroviral therapy. 7/58 (12%) have DM, 13/58 (22%) have HTN, 11/58 (19%) have HYC and 1/58 (1.7%) has symptomatic CAD. 5/7 (71%) pts with DM and 9/13 (69%) with HTN have BMI ≥ 25. Psychiatric disorders are present in 18/58 (31%) including anxiety, depression, and attention-deficit disorder. Hemophilia related arthropathy has been diagnosed in 30/58(52%) patients. The mean BMI of pts with arthropathy is 28.9 (19-36) in comparison to 26.1 (20-44) in pts without arthropathy (p= 0.03). The majority of pts with arthropathy are either obese (BMI ≥ 30; 14/30 or 47%), or overweight (BMI 25–29; 10/30 or 33%). Half of the pts ≥ 50 yrs (7/14) have had at least 1 colonoscopy, of whom 3/7 (43%) experienced a periprocedural bleeding complication. These pts have had a total of 23 surgical procedures, consisting of 5 knee/hip replacements, and 12 abdominal, 2 thoracic, 2 vascular and 2 spine surgeries. Bleeding complications were uncommon 4/23 (17%): 2 with abdominal surgery and 1 each with knee replacement and spine surgery.
Patients with hemophilia suffer from common diseases of aging. Those with arthropathy have higher BMIs and may have more complications of aging. HCV remains a major health concern for older patients. Bleeding complications can occur with invasive procedures, so aggressive use of clotting factor concentrates is warranted. Coordination of care between hematologists and primary care physicians is essential in the management of aging patients with hemophilia.
No relevant conflicts of interest to declare.
Asterisk with author names denotes non-ASH members.