Treatment adherence in adolescents with chronic diseases is around 50%, and failure is more common in preventive therapy. In haemophilia, contradictory results are reported by the published studies. The objective of this study was to evaluate adherence with factor VIII (FVIII) prophylaxis in Spanish patients with severe haemophilia A between age 6 and 20 years. Data were collected retrosp-ectively in the previous 2 years. The primary endpoint was the absolute adherence index (AAI), and the endpoints were related to clinical status, age, prophylaxis regimen, responsibility for factor administration and quality of life (QoL), assessed by the Haemo-QoL questionnaires. A total of 78 patients from 14 Spanish hospitals were recruited. Adherence ranged between -64.4 and 66.7 (mean -3.08). No differences were observed between children and adolescents (7.11 vs. 6.39; P = 0.809). A statistically significant association (P < 0.010) between infra adherent group and target joint was found, as was a statistically significant difference (P < 0.010) between the number of bleeding episodes experienced by the adherent group (mean 1.4) and by infra adherents (mean 4.5). There was no significant difference between AAI and prophylactic regimen (6.35 vs. 6.96, P = 0.848), neither between AAI and the person responsible for factor administration (5.57 vs. 8.79, P = 0.326). The Haemo-QoL scores (8-12 years) were related to adherence level (P < 0.05). Adherence was approximately ideal and patients perceived a high QoL. Because of the repercussions for compliance, it is essential to work during puberty on emotional and self-acceptance aspects of the disease, as well as coping, and the patient's family, school and health team relationships.

To determine the prevalence of haemophilia A and B and their complications in Spain, and to characterize the health care network providing support to haemophiliac patients. The study examines clinical and genetic characteristics, treatment options, and complications observed during the course of the disease. Cross-sectional multi-centre study. The study population were patients with HA and HB in active follow-up at any Spanish hospital by December 2006. We studied 2400 haemophiliacs, 2081 (86.7%) HA and 319 (13.3%) HB patients. Illness was severe in 32.3% of patients, moderate in 16.4%, and mild in 51.3%. Genetic screening was carried out in 32.6% of the patients. Treatment administered in 2006 consisted of coagulation factor concentrates in 60% of patients. Until December 2006, 45.8% of severely ill patients were taking prophylaxis. The mean number of bleeding episodes in 2006 was four for patients not receiving primary prophylaxis and 1.3 for those taking primary prophylaxis. Thirty percent of patients had established haemophiliac arthropathy in at least one joint; 16.8% of patients were HIV-infected and 34.8% HCV-infected. Inhibitors were detected in 10% of severe HA patients and in 6.5% of severe HB patients. Immune tolerance induction therapy was started in 34 patients. This is the first comprehensive study on the epidemiology of haemophilia in Spain. It will enable us to draw comparisons with neighbouring countries, to assess the quality of care provided to haemophiliacs in Spain, and to provide evidence-based guidance for the even provision and improvement of such care.

The period between isolation of HIV in the early 1980s and the development of effective viral inactivation procedures able to eradicate the virus from the blood supply was long and unfortunately many recipients of blood-derived products became infected; this translated into a devastating impact on their quality of life, quality of care as well as on their life expectancy. Some years later, hepatitis C virus infection was identified as another known blood-borne disease complicating the treatment of haemophilia. Nowadays, the potential threat of emerging new pathogens has stressed the need to provide effective but primarily safe products with regard to infectious agents, as well as to regularly update therapeutic guidelines for haemophilia. The aim of the present publication was to review some of the crucial aspects related to the choice of haemostatic concentrates for the treatment of haemophilia and other inherited bleeding disorders, to analyse the current situation in the United States, Canada and European Union countries and to report the most relevant aspects of the Spanish consensus opinion of haemophilia-treating doctors for the use of therapeutic products for haemophilia recently issued. Essentially, it suggests that a gradual switch to recombinant concentrates may be a beneficial decision for patients with haemophilia and for the National Health Service.

Purpose/Objective(s): Intracavitary brachytherapy (ICBT) is an essential component of definitive chemoradiation for localized cervical cancer. This procedure is complex, and even in the hands of experts, complications arise. The most notable complication is perforation of the tandem through a friable cervix/vagina or uterine wall. Historical rates of perforation have been reported to be 2 e 15% without routine image guidance, such as intra-operative ultrasound (IOUS). However, with IOUS guidance, perforation rates have been reported to range from 0-6%, although up to 30% has been reported in high risk patients. Notably, IOUS use has also been shown to reduce time required for applicator insertion, reduce complications, and improve overall cost savings. Despite current practice guidelines recommending IOUS, widespread adoption and data regarding the benefit of IOUS remains limited. Here, we examine the impact of IOUS in reducing complications during tandem applicator placement at our urban institution, with a high-risk patient population with more advanced disease than those described in currently published literature. Materials/Methods: With IRB approval, data was collected on patients with cervical cancer treated with EBRT followed by ICBT (1-2 insertions) between 1/1/13 and 2/4/20. All insertions were performed under anesthesia by a gynecologic and radiation oncologist team. Applicators used included tandem & ovoids, tandem & ring, and tandem & split ring. Prior to 2015, IOUS was inconsistently used for ICBT. However, on 1/7/15, based upon root cause analysis, our institution initiated a policy requiring IOUS guidance for tandem insertions to improve applicator positioning and reduce complications, particularly perforations. The rate of intraoperative complication (IOC) was calculated in two ways e 1. the percentage of patients with complications, 2. the percentage of complications per attempted ICBT insertion. Categorical comparisons were completed using a one-tailed Fisher's exact test. Results: 195 tandem insertions were performed for 99 patients treated with ICBT between 1/1/13 and 2/4/20. There was a 70.6% relative risk reduction (95% CI: 0.0989 e 0.8909, p Z 0.03) in the number of IOC for a given patient from 23.1% (6 of 26) to 6.8% (5 of 73) after the 1/7/15 implementation of IOUS. When analyzing attempted insertions, the rate of IOC dropped from 11.5% (6 of 52) of attempted insertions to 3.5% (5 of 143) (95% CI: 0.0966 e 0.9509, p Z 0.04). All patients who had a perforation were treated with prophylactic antibiotics: no patient required hospitalization or had significant complications. Conclusion: Our results demonstrate that consistent IOUS use has the potential to reduce ICBT complications, especially in high-risk, anatomically complicated patients. Additional investigations focused on disease outcomes and cost effectiveness of the procedure will be important to better understand and further advance widespread implementation of IOUS for ICBT.

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