Challenges in the management of haemophilia on transition from adolescence to adulthood

Abstract

Patients with haemophilia undergo many transitions during their lives, but the period between adolescence and adulthood is particularly challenging. During this time, the patient must deal with all of the typical biological, social and emotional changes associated with this phase of life, whilst at the same time, adapting his lifestyle to the needs of his condition, transferring from paediatric to adult services and, most importantly, accepting increasing (and ultimately full) responsibility for managing his condition. Parents may also find their diminishing role equally challenging. Perhaps not surprisingly, given the challenges that adolescents face, treatment adherence during this time is generally low, which can lead to recurrent joint bleeds, chronic pain and reduced quality of life. To address the challenges, it is critical that a transition programme is put in place that meets the needs of not only the patient, but also his parents and healthcare providers. Key elements of the plan are a multidisciplinary approach, early planning, patient education and appropriate follow-up. A successful programme will equip all parties with the skills to deal with the challenges of transition from adolescence to adulthood, ensuring that the benefits of treatment in childhood are maintained, thus optimising health outcomes and quality of life.

Adherence to prophylactic haemophilic treatment in young patients transitioning to adult care: A qualitative review

“…The main clinical (a) barriers to treatment adherence are high perceived burden of treatment and no or low burden of bleeds and symptoms that may reduce patients’ perception of needing PTX . Some patients have venous access difficulties and PTX is often seen as complicated and time‐consuming, with morning infusions being especially inconvenient . Historically, there were concerns about the risk of HIV or HCV infections, yet in later years concerns about adverse events are rarely reported a reason for non‐adherence to PTX.…”

“…Overall, the main patient‐related barriers (b) are lacking knowledge about haemophilia, including low perceived benefit of PTX . Forgetfulness, poor self‐management and self‐efficacy as well as lacking disease acceptance are crucial barriers . Full self‐management includes “the ability to seek, understand and apply health information to participate in health decisions and the daily management of symptoms and treatment as well as the physical and psychosocial consequences and lifestyle changes following from chronic disease” .…”

“…Full self‐management includes “the ability to seek, understand and apply health information to participate in health decisions and the daily management of symptoms and treatment as well as the physical and psychosocial consequences and lifestyle changes following from chronic disease” . These abilities may be extraordinarily challenged by the physical, cognitive and psychosocial changes occurring in YWH …”

“…Reportedly, adolescents frequently lack knowledge, self‐management skills and self‐efficacy, that is, the motivation and belief in their ability to make appropriate treatment choices . YWH often feel PTX interferes with their daily activities, have difficulties remembering infusions, dealing with sports restrictions and telling others about their disease . Their desire to fit in with healthy peers and live “normal” active lives may challenge disease acceptance .…”

“…Among haemophilic patients in the Western world, adolescents (13‐17‐year‐olds) and young adults (18 to 25‐30‐year‐olds depending on the study) reportedly have the lowest adherence levels compared to other age groups . These patients go through major physical and psychosocial changes challenging the transition to self‐care …”