Comorbidities are risk factors for hospitalization and serious COVID-19 illness in children and adults with sickle cell disease.

Patients with sickle cell disease (SCD) are at high risk of developing serious infections, therefore, understanding the impact that severe acute respiratory syndrome coronavirus 2 infection has on this population is important. We sought to identify factors associated with hospitalization and serious COVID-19 illness in children and adults with SCD.We established the international SECURE-SCD Registry to collect data on patients with SCD and COVID-19 illness. We used multivariable logistic models to estimate the independent effects of age, sex, genotype, hydroxyurea, and SCD-related and -nonrelated comorbidities on hospitalization, serious COVID-19 illness, and pain as a presenting symptom during COVID-19 illness. As of 23 March 2021, 750 COVID-19 illness cases in patients with SCD were reported to the registry. We identified history of pain (relative risk [RR], 2.15; P < .0001) and SCD heart/lung comorbidities (RR, 1.61; P = .0001) as risk factors for hospitalization in children. History of pain (RR, 1.78; P = .002) was also a risk factor for hospitalization in adults. Children with history of pain (RR, 3.09; P = .009), SCD heart/lung comorbidities (RR, 1.76; P = .03), and SCD renal comorbidities (RR, 3.67; P < .0001) and adults with history of pain (RR 1.94, P = .02) were at higher risk of developing serious COVID-19 illness. History of pain and SCD renal comorbidities also increased risk of pain during COVID-19 in children; history of pain, SCD heart/lung comorbidities, and female sex increased risk of pain during COVID-19 in adults. Hydroxyurea showed no effect on hospitalization and COVID-19 severity, but it lowered the risk of presenting with pain in adults during COVID-19.

Coronavirus Disease among Persons with Sickle Cell Disease, United States, March 20-May 21, 2020.

Sickle cell disease (SCD) disproportionately affects Black or African American persons in the United States and can cause multisystem organ damage and reduced lifespan. Among 178 persons with SCD in the United States who were reported to an SCD-coronavirus disease case registry, 122 (69%) were hospitalized and 13 (7%) died.

American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain.

BACKGROUND: The management of acute and chronic pain for individuals living with sickle cell disease (SCD) is a clinical challenge. This reflects the paucity of clinical SCD pain research and limited understanding of the complex biological differences between acute and chronic pain. These issues collectively create barriers to effective, targeted interventions. Optimal pain management requires interdisciplinary care. OBJECTIVE: These evidence-based guidelines developed by the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care professionals in pain management decisions for children and adults with SCD. METHODS: ASH formed a multidisciplinary panel, including 2 patient representatives, that was thoroughly vetted to minimize bias from conflicts of interest. The Mayo Evidence-Based Practice Research Program supported the guideline development process, including updating or performing systematic reviews. Clinical questions and outcomes were prioritized according to importance for clinicians and patients. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations, which were subject to public comment. RESULTS: The panel reached consensus on 18 recommendations specific to acute and chronic pain. The recommendations reflect a broad pain management approach, encompassing pharmacological and nonpharmacological interventions and analgesic delivery. CONCLUSIONS: Because of low-certainty evidence and closely balanced benefits and harms, most recommendations are conditional. Patient preferences should drive clinical decisions. Policymaking, including that by payers, will require substantial debate and input from stakeholders. Randomized controlled trials and comparative-effectiveness studies are needed for chronic opioid therapy, nonopioid therapies, and nonpharmacological interventions.

Perceptions of the term palliative care.

OBJECTIVES: To assess parents' and health care providers' perceptions of the name and description of a pediatric palliative care (PPC) program. METHODS: Survey conducted at three pediatric health care sites; asked respondents (parents and staff) about their likelihood to use a program identified either as palliative care or supportive care, as well as their understanding and feelings about the program before and after reading a program description. RESULTS: Response rate was 89% (195/220); 184 were considered evaluable. Parent respondents in the supportive care group scored significantly higher (Mann-Whitney test, p = 0.003) on "likelihood to use program" (mean score, 4.22, n = 60) than those in the palliative care group (mean score, 3.58, n = 45) before each read the program description. However, this group difference disappeared (p = 0.582) after reading the description (mean scores 4.50, 4.38, respectively; n = 48, n = 40, respectively). The name palliative care evoked more negative emotions compared to the supportive care name in parents, and reading the program description led to more positive feelings. In staff, reading the program description significantly increased likelihood to use the program for those in the Palliative Care group only (4.22 to 4.44; p < 0.05; n = 41). Staff also had more positive feelings about the program called supportive care, and rated this name best most frequently. CONCLUSION: Better definition of and explanation to families and health care providers about what palliative care programs offer may improve perceptions about palliative care and increase program utilization.

Trends in breast cancer presentation and care according to age in a single institution.

BACKGROUND: This study sought to determine the differences in presentation and treatment of young women (< or =40 years of age) with breast cancer. METHODS: A prospective database was analyzed for differences in presentation and care in breast cancer patients < or =40 and >40 years of age. RESULTS: The study group consisted of 1685 women. Younger women were more likely to present with a palpable mass, have estrogen receptor/progesterone receptor (ER/PR)-negative tumors, and have more advanced disease at presentation. Although there was no difference in breast conservation rates, younger women were more likely to have postmastectomy reconstruction. Younger women were more likely to receive chemotherapy, even with node-negative tumors less than 1 cm in diameter (37% vs. 13%, P = 0.01). CONCLUSIONS: The presentation of younger women with breast cancer differs from that of older women. Although the surgical management is similar, adjuvant therapy differs, with younger women more likely to be treated with chemotherapy.