Spinal stenosis may be complication of aging in hemophilia patients
Study: Symptoms improved with physiotherapy in some cases
Symptomatic spinal stenosis — the narrowing of spaces within the spinal canal, which can put pressure on the spinal cord and nerves — may be a complication in aging people with hemophilia, a study in Ireland reports.
The risk of developing the condition increased with age, but it was not associated with hemophilia severity or treatment regimen. In some cases, symptoms improved with physiotherapy and pain management.
“Increased awareness [among people with hemophilia] and health care providers of spinal stenosis is directly required; however, optimal management strategies for [people with hemophilia] with established stenosis are yet to be defined,” researchers wrote.
The study, “Spinal Stenosis: An Emerging Complication of Ageing in People With Haemophilia,” was published in Haemophilia.
Treatment advances extend life expectancy for hemophilia patients
Hemophilia is commonly caused by mutations in genes that result in the production of insufficient amounts of functional clotting factors, or proteins needed for the blood to clot. This leads to bleeding episodes that may affect the joints, causing pain and impairing patients’ mobility.
Recent advances in the treatment of hemophilia have extended life expectancy for people with the condition. However, as they age, they may experience complications associated with joint damage.
In this study, researchers analyzed the rates of spinal stenosis in people with hemophilia. A total of 100 men with with a median age of 56 and moderate or severe hemophilia A (71%) or hemophilia B (29%) were included.
Overall, 40% underwent spinal imaging, mainly due to back pain and/or neurological symptoms, and 13% were diagnosed with spinal stenosis. According to the researchers, the rate of symptomatic spinal stenosis in this patient population was higher than expected considering the rate seen in the general population, which is reported to be 4% in people ages 60 or older.
In most cases (69%), spinal stenosis affected the lumbar spine, or lower back. It was also identified in the cervical spine, or neck region (38%), and thoracic spine, or the middle of the spine (8%). In two patients, more than one spinal region was affected.
Participants with spinal stenosis were older than those without it (median of 69 years old vs. 55 years old). The condition was also more frequently seen among those with hemophilia B (20.7%) than in those with hemophilia A (9.8%). Further analysis revealed that age was associated with an increased likelihood of having spinal stenosis.
There were no differences between patients with or without spinal stenosis regarding hemophilia severity, treatment regimen, and development of inhibitors — neutralizing antibodies some patients develop against clotting factors that may render factor replacement therapies less effective.
Obesity rates higher in patients with spinal stenosis
According to the Haemophilia Joint Health Score (HJHS), a standardized tool used to assess the health of joints affected by bleeding in people with hemophilia, there were no differences between patients with and without spinal stenosis. However, patients with stenosis were older at the time of their first joint replacement procedure (55.5 years old vs. 46 years old).
According to the researchers, these results indicate that “current joint assessments fail to capture spinal pathology [disease], highlighting limitations of HJHS in older [people with hemophilia].”
The rates of obesity were slightly higher in people with stenosis (27% vs. 20%), as were those of osteopenia (40% vs. 36%) and osteoporosis (20% vs. 9%). Osteopenia is marked by lower bone density and is considered to be an early stage of osteoporosis, a condition in which bones become brittle and more prone to fractures.
Spinal stenosis was managed with an extensive physiotherapy support program and pain management, including oral medications in all patients and pain injections in more than half. None of the patients underwent spinal surgery, mainly due to improvement with treatments and bleeding concerns.
“As surgical options may be more limited due to bleeding concerns, there is an important need to understand the pathophysiology of spinal stenosis in [people with hemophilia] in order to prevent or mitigate its impact with ageing,” the researchers wrote. “The effectiveness of physiotherapy interventions, exercise therapy, and/or spinal injections … also warrants urgent attention in future research.”
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