Dual enzyme therapy improves adherence to chemotherapy in a patient with gaucher disease and Ewing sarcoma.

This case reports concomitant use of enzyme and substrate reduction therapy to improve chemotherapy adherence in a pediatric patient diagnosed with Ewing sarcoma (ES) and type 1 Gaucher disease (GD). The 17-year-old female presented with 5 months of right knee pain with associated mass on exam. She was diagnosed with ES with pulmonary metastasis. The patient was treated with 17 alternating cycles of vincristine-doxorubicin-cyclophosphamide and ifosfamide and etoposide chemotherapy followed by tumor resection and radiation per standard protocol. As part of her staging work-up, bone marrow biopsy was performed, significant for Gaucher cells. After the second cycle of chemotherapy the patient began to experience severe delays averaging 30 days between cycles compared to 17.29 days observed in Children's Oncology Group data. Given her bone marrow biopsy findings and chemotherapy delays GD screening was obtained and the patient was diagnosed with GD following genetic confirmation. Due to delays in chemotherapy decreasing chance of remission, the patient was referred to Genetics for aggressive management with imiglucerase and eliglustat. After initiation of therapy the period between chemotherapy cycles decreased to 23 days on average, with a 21% increase in platelet count during therapy. The patient was able to complete ES therapy achieving remission. GD is associated with an increased risk of malignancy, as seen in our patient with ES. GD patients experience prolonged hematologic cytopenia during cancer treatment. Combining Enzyme and Substrate Reduction Therapies should be investigated as an option to improve chemotherapy adherence in GD patients.

Acceptability and preferences for long-acting antiretroviral formulations among people with HIV infection.

The study evaluates the acceptability and preferences for long-acting antiretroviral therapy (LA-ART) among a diverse cohort of people with HIV infection (PWH). It consists of a self-administered survey and chart review of PWH presenting to an HIV clinic in Houston, Texas, between February and June 2018; 374 participants were included; 61% indicated that they were likely or very likely to use LA-ART formulations. When asked about preference, 41% preferred pills, 40% preferred injections, and 18% preferred an implant. The most common benefit reported was eliminating the need to remember taking daily HIV pills (74%); 43% were worried that LA-ART will not be as effective as pills. Participants with a college degree, men who have sex with men, and ART-experienced were more willing to use LA-ART. Participants who reported poor or fair health, or who screened positive for depression or anxiety were significantly less willing to use LA-ART. The likelihood of using LA-ART did not correlate with self-reported adherence and HIV suppression. Patients with difficulty scheduling and attending clinic visits preferred injections and implant over pills. Most participants indicated a willingness to use new LA ART formulations. However, 41% still prefers pills, and those more interested in LA-ART were not less adherent.

Exploring the Attitude of Patients with HIV About Using Telehealth for HIV Care.

Telehealth could address many of the factors identified as barriers for retention in HIV care. In this study, we explore people with HIV (PWH)'s attitudes about using telemedicine for HIV care instead of face-to-face clinic visits. We administered a one-time survey to PWH presenting to an outpatient HIV center in Houston, Texas, from February to June 2018. The survey items were used to assess PWH's attitudes toward and concerns for telehealth and explanatory variables; 371 participants completed the survey; median age was 51, 36% and were female, and 63% was African American. Overall 57% of respondents were more likely to use telehealth for their HIV care if available, as compared with one-on-one in-person care, and 37% would use telehealth frequently or always as an alternative to clinic visits. Participants reported many benefits, including ability to fit better their schedule, decreasing travel time, and privacy but expressed concerns about the ability to effective communication and examination and the safety of personal information. Factors associated with likelihood of using telehealth include personal factors (US-born, men who have sex with men, higher educational attainment, higher HIV-related stigma perception), HIV-related factors (long-standing HIV), and structural factors (having difficulty attending clinic visits, not knowing about or not having the necessary technology). There was no association between participants with uncontrolled HIV, medication adherence, and likelihood of using telehealth. Telehealth programs for PWH can improve retention in care. Availability and confidence using various telehealth technologies need to be addressed to increase acceptability and usage of telehealth among PWH.