Development of a telematic pharmaceutical care app (Haemoassist) for multidisciplinary follow-up of patients with congenital coagulopathies.

BACKGROUND: Guidelines for congenital coagulopathies recommend that patients record treatment administrations and bleeding episodes to help healthcare professionals monitor the disease. RESEARCH DESIGN AND METHODS: We studied over two years which patient profiles (age, treatment regimen, treatment compliance) were most likely to accept the use of an app to collect this information. We validated the quality of patient-reported data by comparing it with data obtained from hospital electronic records, pharmacy dispensing records and patient interview, collected in an access database used as a reference. Patient and professional opinions were solicited through open-ended interviews. RESULTS: The app was used by 52% of 315 patients studied. Younger patients were the most frequent users. Patients with better treatment compliance used the app more, although data collection was incomplete for most patients. The best rated by patients were the reminders of days of administration and the minimum stock alerts at home. Healthcare professionals rated the app positively. CONCLUSIONS: Healthcare professionals valued the app as useful for managing treatment of congenital coagulopathies. Patients need support and time to use the app and improve the quality of the data entered. Patients who used the app rated it positively. The treatment compliance improved.

Registry of patients with congenital bleeding disorders and COVID-19 in Madrid.

INTRODUCTION: We present the first registry of patients with congenital bleeding disorders and COVID-19. The study has been carried out in the Community of Madrid, which has the highest number of cases in Spain. The objective is to understand the incidence of COVID-19, the course of the disease if it occurs and the psychosocial and occupational impact on this population. METHODS: We included 345 patients (246 of haemophilia, 69 of von Willebrand Disease, two rare bleeding disorders and 28 carriers of haemophilia). A telephone survey was used to collect the data. RESULTS: Forty-two patients presented symptoms suggestive of infection by COVID-19, and in six cases, the disease was confirmed by RT-PCR. The cumulative incidence of our series was 1.73%. It is worth noting the complexity of the management of COVID-19 in two patients on prophylaxis with non-factor replacement therapy. Adherence to the prescribed treatment was maintained by 95.5% of patients. Although 94% were independent for daily living activities, 42.4% had a recognized disability and 58% required assistance, provided by the Madrid Haemophilia Association (Ashemadrid) in 75% of cases. Only 4.4% of consultations were held in person. CONCLUSIONS: Patients with congenital bleeding disorders infected with SARS-CoV-2 presented a mild course of the disease that did not require admission. Their identification and treatment by a specialist team from a Haemophilia Treatment Center are essential to make a correct assessment of the risk of haemorrhage/thrombosis. COVID-19 had a major impact on the psychosocial aspects of these patients which must be remedied with recovery plans.

Accelerating recovery from acute hemarthrosis in patients with hemophilia: the role of joint aspiration.

: Arthrocentesis of an acute hemarthrosis in hemophilia remains a controversial issue. The purpose of this study is to define the role that joint aspiration can play in the recovery from acute hemarthrosis in patients with hemophilia. The study sample included 33 hemophilic patients (55 joints) with acute elbow, knee, and ankle hemarthrosis as confirmed by ultrasonography. Patients were distributed into a treatment group and a control group. Patients in the first group were subjected to joint aspiration, whereas patients in the second were not. Arthrocentesis was carried out immediately after diagnosis of acute hemarthrosis in liquid phase. Patients were infused with the deficient coagulation factor and were instructed to observe relative rest until resolution of hemarthrosis. The following parameters were analyzed: time to full resolution of hemarthrosis (determined by ultrasonography), duration of treatment with the deficient coagulation factor, time to pain relief, time to recovery of prebleed range of motion, and time to resumption of school/work (all of these measured in days). The joints treated with joint aspiration exhibited a significantly faster resolution of bleeding (fewer days). In addition, this group required fewer days of pharmacological treatment, with faster achievement of functional recovery and resumption of school/work activities. No complications were observed. This study shows that joint aspiration under hemostatic cover and in strictly aseptic conditions is a well-tolerated technique that makes the recovery of acute hemarthrosis of hemophilic patients faster.

Objective quantification of adherence to prophylaxis in haemophilia patients aged 12 to 25years and its potential association with bleeding episodes.

BACKGROUND: The treatment of choice for patients with severe haemophilia is prophylaxis with clotting factor. Effective prophylaxis requires optimal adherence. Most published studies are based on surveys and interviews with patients or their parents. However, studies based on objective measurements of adherence are few and inconclusive. OBJECTIVE: The main purpose of this study was to assess adherence to prophylaxis using an objective method in patients with haemophilia aged 12 to 25years as well as to assess its potential association with bleeding episodes. Secondary objectives included comparing objective and subjective adherence (questionnaire) and identifying factors potentially associated with treatment adherence. METHODS: A retrospective observational study was designed to collect data on treatment adherence and clinical course of patients in 2013. The study included haemophilia patients on prophylaxis with clotting factor concentrate; the age range was 12 to 25years. Objective adherence to treatment was measured through pharmacy dispensing records. Dispensing dates within 2013 were used for calculation. Adherence (%) was calculated by dividing the total number of International Units (IU) of factor dispensed by the total estimated number of IU and multiplying by 100. Subjective adherence was measured using an ad hoc questionnaire which was completed by the patient. The number of bleeding episodes (joint, muscle and others) was obtained from interviews with the patient at the consultation with the pharmacist and was subsequently confirmed with the patient's clinical history. Other parameters were also evaluated. RESULTS: We included 52 patients in the study. Average adherence of patients resulting from the analysing of dispensing records was 85.72 (SD=23.76%). The global average of bleeding episodes was 2.2 (SD=2.69). 32.6% (seventeen) patients presented with at least one joint bleeding episode versus 67.3% (thirty five) who showed no joint bleeding episodes. Likewise, only 10% (five) patients presented with one muscle bleeding episode. Even though a significant association between the rate of objective adherence and the number of bleeding episodes was not observed, a higher number of hemarthrosis was observed in less-adherent patients. Subjective adherence perceived by patients as reported by the questionnaire showed that 36 (83.7%) of the 43 patients who completed the questionnaire considered their adherence as good, whereas 7 (16.3%) of the 43 patients considered it as fair. A statistically significant relationship was found between objective adherence expressed as a percentage using dispensing records, and subjective adherence perceived by the patient (p=0.004). The other parameters evaluated were not associated with adherence to prophylaxis. CONCLUSIONS: Measuring objective adherence using pharmacy dispensing records is a simple and useful tool. No significant correlation was found between the rate of objective adherence observed and the number of bleeding episodes; however, a higher number of hemarthrosis was observed in less-adherent patients.

Symptomatic thromboembolic events in patients treated with intravenous-immunoglobulins: results from a retrospective cohort study.

AIMS: To estimate the incidence and predictors of symptomatic arterial and venous thromboembolic events (TEE) from intravenous immunoglobulin (IVIg) therapy according to its indications. METHODS: We performed a retrospective cohort study of patients seen at our institution and treated with IVIg over a 36-month period. Indications, comorbility and comedication associated with TEE were identified by a stepwise logistic regression analysis. RESULTS: Of 303 patients included with at least one infusion of IVIg over three years, TEE were identified in a total of 50 patients treated with IVIg, for an incidence of 16.9% (CI 95%: 13.0-21.6); 27 (54%) arterial (9.1%;CI 95%: 6.3-13.0%) and 23 (46%) venous TEE (7.8%; CI95%: 5.2-11.4%), overall mortality was 32%. Per indication there were more patients with autoimmune conditions, secondary immunodeficiency, dysimmune neuropathies, acute rejection of solid organ transplantation and sepsis. Patients with TEE were significantly older, were more likely to be men, they had more comorbid conditions; the doses of IVIg were high (589.4mg/kg/day vs 387.0mg/kg/day, p<0.001) and differences in comedication were found. The stepwise logistic regression analysis retained high doses of IVIg (OR 3.03; CI 95%: 1.49-5.67) and diuretics therapy (OR 1.69; CI 95%: 1.06-3.97) when combined with the usual comorbid confounders. CONCLUSIONS: The incidence of TEE from IVIg therapy remains high at one in six patients treated. The most remediable factor is a high daily IVIg load. Decreasing the daily IVIg dose together with carefully weighing diuretics therapy and comorbid risk factors may be the keys to saving lives.