Telehealth: Navigating the COVID-19 Pandemic and Beyond: The Sickle Cell Unit Experience.

Introduction: To examine the use of telehealth for delivery of health care in persons with sickle cell disease in a resource-constrained country during the COVID-19 pandemic. Methods: This study was a retrospective review of patient encounters at the Sickle Cell Unit (SCU), Jamaica during a 3-year period, March 10, 2019 to March 9, 2022 and a comparison of endpoints between 1 year before and 2 years during the pandemic. Primary endpoints of registration numbers, day-care admissions, and study visits were obtained from logbooks and the electronic medical records. Additional endpoints included well visits, hydroxyurea (HU) visits, and bone pain crisis. Results: Patients registered at the clinic on 17,295 occasions, with 7,820 in the pre-pandemic year decreasing by 43.8% and 35% in the 2 subsequent pandemic years. Overall, study visits increased by 4.9% and 1.3% in the pandemic years. They increased in adults by 13.1% and 8.9% but fell by 3.2% and 6.2% in children. Fewer people were seen in the pandemic years, with children showing a 20.7% decline in numbers. Tele-visits accounted for 31.4% of all study visits during the pandemic years and increased by 23.6% between the pandemic years. There were more well-visits and HU visits, but fewer pain visits and day-care admissions in the pandemic years. Conclusions: The SCU maintained health care delivery for a high-risk population during the pandemic, with tele-visits mitigating the short-fall from in-person visits. Tele-visits may be more acceptable to adults with a chronic illness and may be a suitable alternative for delivering health care.

Interventions for treating neuropathic pain in people with sickle cell disease.

BACKGROUND: Pain is the hallmark of sickle cell disease (SCD) and it can be severe, frequent and unpredictable. Although nociceptive pain is more common, at times, people with SCD may have neuropathic pain. The latter can occur due to peripheral or central nerve injury. This review is focused on identifying treatment of only painful sensory neuropathy in people with SCD. OBJECTIVES: To determine the effectiveness and safety of any pharmacological or non-pharmacological therapies for treating neuropathic pain in people with SCD. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched trial registries, the reference lists of relevant articles and reviews and contacted experts in the field.Date of last search: 31 January 2019. SELECTION CRITERIA: Randomised controlled trials (RCTs) (parallel or cross-over in design), quasi-RCTs of pharmacological or non-pharmacological therapies for treating neuropathic pain in people with SCD compared to placebo or another intervention in any category (i.e. pharmacological or non-pharmacological). DATA COLLECTION AND ANALYSIS: Two review authors independently assessed all trials identified by the searches and extracted relevant data. Two authors independently assessed the risk of bias in the selected trials using the Cochrane risk of bias tool. Two review authors independently rated the quality of the evidence for each outcome using the GRADE guidelines. MAIN RESULTS: One RCT of 22 participants with SCD, conducted in the USA was included in this review. Participants were randomly assigned to either pregabalin (n = 11) or placebo (n = 11). Oral pregabalin was administered at an initial dose of 75 mg twice daily. The drug was titrated at increments of 75 mg to a maximum of 600 mg daily or decreased by 75 mg per day if necessary, based on clinical presentation and pain level. Neuropathic pain was assessed using self-reports on the Leeds Assessment of Neuropathic Symptoms and Signs (S-LANNS) scale and the Neuropathic Pain Symptom Inventory (NPSI), where higher scores were indicative of more pain. Outcomes included self-reported pain, quality of life and withdrawal due to adverse effects measured at baseline and monthly for three months post-intervention. The overall risk of bias was low with a high risk of bias due to attrition.In relation to this reviews primary outcomes, for self-reported neuropathic pain relief, given the paucity of data, we are very uncertain whether there is a difference between the pregabalin and placebo groups at the end of three months as measured by the S-LANSS scale, mean difference (MD) -2.00 (95% confidence interval (CI) -9.18 to 5.18), or the NPSI scale, MD -11.10 (95% CI -33.97 to 11.77) (very low-quality evidence). There was no report of 'Patient Global Impression of Change' in the included trial.Although the mean quality of life scores (Short Form-36) at three months showed small increases in seven of the eight domains post-intervention in the pregabalin group as compared to the placebo group, this was very low-quality evidence and we are very uncertain whether pregabalin increases quality of life. Neither of our pre-defined outcomes of 'time to improvement of symptoms' or 'changes in sleep quality', were measured in the included trial.While treatment-related adverse effects appeared higher in pregabalin group than the placebo group at three months, this was very low-quality evidence and we are very uncertain whether there is a difference, RR 1.33 (95% CI 0.39 to 4.62) (very low-quality evidence). There was one withdrawal for adverse effects in the pregabalin group while three people withdrew or dropped out from the placebo group due to adverse effects and complications and hospitalisation related to SCD. AUTHORS' CONCLUSIONS: The included trial provided very low-quality evidence. Self-reported pain relief was greater in the pregabalin group compared to the placebo control group but only using the S-LANSS scale and we are very unsure whether there is a difference. While the pregabalin group tended to have improved quality of life over the duration of the trial, this was very low-quality evidence and we are uncertain whether there is a difference. Adverse effects and withdrawals were similar across the treatment and placebo control group in trial. There are both insufficient trials addressing this review question and insufficient outcomes addressed in the single included RCT. Therefore, there is still a significant gap in evidence on interventions for neuropathic pain in people with SCD.

Small bowel overgrowth in malnourished children as measured by the breadth hydrogen test - abstract

Overgrowth of the small intestine with bacteria has been implicated in the chronic diarrhoea associated with severe malnutrition. The hydrogen breath test has been introduced as a simple non-invasive technique to assess bacterial fermentation in the bowel. An increase in breath hydrogen in excess of 20 ppm within 60 minutes of the oral presentation of substrate is taken as evidence of small bowel overgrowth (SBO). The hydrogen breath test was used in 30 children admitted with severe protein energy malnutrition. Of the 19 children tested on admission and at intervals during recovery, 5 were positive on admission and a further 3 became positive at varying times after admission. Of these 8 with positive results, 5 were treated with metronidazole and all were negative on subsequent testing. Of the 3 children who were not treated one improved, but 2 had persistently positive breath hydrogen. Eleven (11) children had breath hydrogen measured at some time during recovery. In 6 the test was positive; all received metronidazole and became negative. In 2 children who had received metronidazole and were negative, the test became positive at a later date. Three children had a positive test with lactose specifically, one was treated with metronidazole and became negative. Therefore, of 30 children studied, 14 had a positive test at some time, which became negative in the 11 treated with metronidazole. We conclude that small bowel overgrowth as measured by the breath hydrogen test is a frequent accompaniment of severe malnutrition and responds to treatment with metronidazole (AU)