Men Talk About Hemophilia’s Toll, Gaps in Care in Canadian Study
A hemophilia diagnosis takes a toll on the social and mental health of men, who often feel misunderstood and excluded from activities such as sports, a Canadian interview-based study reports.
While blood-clotting replacement therapies have improved quality of life, they can also be costly. Study participants highlighted the need for multidisciplinary teams, more disease awareness among doctors in emergency rooms, and better access to hemophilia treatment centers.
The study, “Invisible Bleeds: Lived Experiences and Barriers to Care for Men with Hemophilia,” was published in the Journal of Thrombosis and Haemostasis.
Hemophilia can have a detrimental effect on quality of life. However, few studies have assessed the life experiences of hemophilia patients, or barriers to care they may encounter.
To answer this, researchers at the University of Toronto in Canada conducted interviews with 11 English-speaking male patients (median age of 39 years), recruited via the Canadian Hemophilia Society.
The interviews, conducted by telephone, focused on three categories: impact on identity and daily life, changes in treatment, and care needs.
Of the 11 patients, seven had hemophilia A (five severe and two mild disease) and four had severe hemophilia B. All except for one patient received preventive replacement therapy, which supplies patients the blood clotting factor they are missing — factor VIII (FVIII) in the case of hemophilia A and factor IX (FIX) in the case of hemophilia B. One patient with mild hemophilia received on-demand therapy.
Three patients had a history of inhibitors — neutralizing antibodies made by the immune system that target FVIII, lowering the effectiveness of replacement therapies and, in some cases, rendering them useless. Two of them were still positive for inhibitors.
All patients had regular access to a hematologist, and all but one visited hemophilia clinics every six to 12 months. One of the patients visited the clinic more regularly (four to six times a year) due to bleed frequency.
Five patients lived within half an hour of their care center, while two resided approximately an hour away. Three patients lived one to three hours from their treatment center. Travel distance to a clinic was unavailable for one participant.
All patients described bleeding symptoms occurring at an early age. Ten patients were diagnosed before age 3 and one by age 6. Bleeding symptoms led to chronic pain and limited their activity.
The patients’ pain and limited mobility contributed to increased stress levels and social isolation, and had a negative impact on their mental health. Some patients described being hypervigilant for bleeds, and feeling misunderstood and often “invisible” to their peers.
“It made me a very paranoid person,” one of the patients said. “I was always paranoid. Like every little bump that I would get, I was like, ‘Oh, my goodness. I’m going to die.’ ”
The patients felt excluded from sports activities, and as a result of disease-associated complications, had to miss school and work days.
“I missed an extreme amount of school, which, again, just added complexity to my life, to my family’s life,” said a patient.
A hemophilia diagnosis, the patients said, enhanced their need for resilience and promoted their personal growth. Some patients described hemophilia as their “teacher.”
Before lab-made replacement therapies were available, treatments such as blood transfusions were cumbersome, the patients said. Three patients contracted hepatitis C from blood transfusions, and one of them also contracted HIV.
Clotting factor replacement therapies — available as a freeze-dried powder that can be reconstituted and administered via injection — offered more flexibility as they could be given at home, curbing the need for frequent hospital visits.
In general, the patients shared an interest in clinical research and future therapy development. Some were willing to be personally involved in clinical research to help others with hemophilia. This, they said, would provide them a sense of fulfillment.
Hemlibra (emicizumab) — an approved under-the-skin (subcutaneous) injection for managing the symptoms of hemophilia A in patients with and without inhibitors — was the top treatment choice. However, participants voiced concerns about its funding and availability.
When asked about gaps in current care management, the participants identified the need for multidisciplinary teams of healthcare professionals, particularly physiotherapists, social workers, and chronic pain specialists.
The patients noted the need for readily accessible consultations for pain management. They also said care should include home infusion supplies, in addition to treatment plans.
One of the challenges identified during interviews was the difficulty in finding surgeons willing to work with the patients.
Visits to the emergency room were often described as negative, with physicians lacking familiarity with hemophilia and treatment being delayed. Patients with a pre-existing treatment plan, or who knew what they needed, thought their plan could be disregarded despite their efforts to advocate for themselves.
Participants also highlighted the importance of being heard, following existing treatment plans, and clinic-issued identification documents.
In general, the patients were satisfied with their treatment plans, but acknowledged the importance of being well-connected to a hemophilia treatment center. Some patients said that being close to a such a center influenced their choice of residence.
“Men with hemophilia described significant symptom burden and areas of ongoing need,” the researchers wrote.
“Collaborative efforts between hematologists, emergency room physicians, and surgeons to establish hospital-specific testing, treatment and referral guidelines, and regular [hemophilia treatment centers] audits may help address these care gaps, providing more person-centred, equitable care,” they wrote. “Future work is required to implement these strategies and monitor their effects.”
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