Asunto(s)
Factor VIII/uso terapéutico , Hemofilia A/tratamiento farmacológico , Anciano , Animales , Anticuerpos Neutralizantes/sangre , Factores de Coagulación Sanguínea/uso terapéutico , Coagulantes/uso terapéutico , Pólipos del Colon/cirugía , Transfusión de Eritrocitos , Eritrocitos/citología , Factor VIII/genética , Factor VIII/metabolismo , Hematuria/terapia , Hemorragia/prevención & control , Humanos , Factores Inmunológicos/uso terapéutico , Masculino , Prednisolona/uso terapéutico , Proteínas Recombinantes/biosíntesis , Proteínas Recombinantes/aislamiento & purificación , Proteínas Recombinantes/uso terapéutico , Rituximab/uso terapéutico , Porcinos , Resultado del TratamientoAsunto(s)
Hemofilia A/terapia , Adolescente , Adulto , Ejercicio Físico , Femenino , Humanos , Masculino , Motivación , Proyectos Piloto , Adulto JovenAsunto(s)
Antivirales/uso terapéutico , Coinfección , Hemofilia A/complicaciones , Hepatitis C/complicaciones , Hepatitis C/tratamiento farmacológico , Ribavirina/uso terapéutico , Uridina Monofosfato/análogos & derivados , Adulto , Terapia Antirretroviral Altamente Activa , Carcinoma Hepatocelular/diagnóstico , Carcinoma Hepatocelular/etiología , Carcinoma Hepatocelular/cirugía , Infecciones por VIH/tratamiento farmacológico , Humanos , Fallo Hepático/diagnóstico , Fallo Hepático/etiología , Fallo Hepático/cirugía , Trasplante de Hígado , Masculino , Sofosbuvir , Resultado del Tratamiento , Uridina Monofosfato/uso terapéuticoRESUMEN
UNLABELLED: Climbing has a low risk of injury and strengthens the entire musculature. Due to its benefits in physical and mental health as well as its high fun factor climbing is an established way of therapy. So far, the usefulness of climbing therapy has not been shown for people with haemophilia (PWH). A crucial requirement for physical activity in PWH is regular prophylaxis. As the patient's individual pharmacokinetic (PK) response varies significantly, PK-tailored prophylaxis may decrease bleeding frequency. CASE REPORT: We describe a man (age 25 years) with severe haemophilia A who took part in an 8.5-month weekly climbing program under PK-tailored prophylaxis. Bleeding frequency, factor consumption, joint health (Haemophilia Joint Health Score, HJHS), quality of life (Haemo-QoL-A) and climbing performance (UIAA scale) were assessed before and after the training. Prior to the study, the patient was on demand treatment. The patient was started on standard prophylaxis for a 2 months period and then observed for 6.5 months under PK-tailored prophylaxis. PK-tailored prophylaxis was targeted to a trough level of 1-3%. For high-impact activities a factor activity >15%, for low-impact activities a factor activity >5% was suggested. RESULTS: Climbing therapy was safe. The bleeding rate decreased from 14 (2012) to 1 (during the study period of 8.5 months). The one bleeding event was due to a missed infusion and was not triggered by physical activity. The elimination half-life using Bayesian statistics was determined to be 16h. Using this half-life for PK-tailored prophylaxis reduced the factor VIII consumption in comparison to standard prophylaxis. Joint health was particularly improved in the categories range of motion and swelling. Quality of life scores stayed at a high level. Climbing performance improved by 1 grade. CONCLUSION: The combination of PK-tailored prophylaxis with therapeutic climbing improved clinical outcome in this young adult with severe haemophilia. The tailored concept for high- and low-impact activities appeared to be safe.