In the United States, sickle cell disease is most commonly found among African Americans or persons of African descent; however, people of all races and ethnicities can have SCD. About 1 in every 365 African-American babies in the U.S. is born with SCD; and worldwide, approximately 300,000 babies are born with SCD each year. As more people move from areas highly affected by SCD to the U.S., schools will become more diverse and there is a higher chance that teachers will encounter a student with SCD in their classrooms. As with any student with a chronic health condition, students with SCD may experience health problems during the school day. To support these students, teachers, nurses and administrators can: Ensure Adequate Access to Water/Hydration Staying well hydrated by drinking plenty of water can help prevent pain episodes and other health problems. Thus, unlimited access to water throughout the school day is essential. Frequent, small amounts of water are better than trying to drink a large amount of fluid at one time. Allowing access to a bottle of water in class is an option. Abstract source

It is time to rethink hydroxyurea for the treatment of sickle cell disease, especially for your children. Hydroxyurea is a critical therapeutic treatment for children with recurring acute pain events. It has shown to deliver long-term benefits such as the prevention of organ damage. Who is the Best Candidate for Hydroxyurea? Children over two years old with sickle cell disease who have recurring and severe pain events (vaso-occlusive crises) are strong candidates for treatment with hydroxyurea. How Old Does My Child Need to Be to Start Taking Hydroxyurea? Hydroxyurea has been approved for use in children as young as 2 years old. Is Hydroxyurea Difficult to Administer? No. For children and adolescents, a pill a day with water is easy. Hydroxyurea comes as a tablet that quickly melts in water. It is important to understand that in order to get maximum benefit, hydroxyurea must be taken every day. As a parent or caregiver, it is important to commit to the recommended daily dosage. Daily dosage is based on your child's body weight. It will target an optimal response with fewer side effects, which will help ease your child’s discomfort.   Abstract source

Hydroxycarbamide is a medicine taken by mouth as a liquid or in a capsule or as a tablet (Siklos®). It causes changes in the blood to reduce the frequency and severity of painful episodes, chest complications and the need for blood transfusions in patients with sickle cell disease. It is also thought to prolong life expectancy if taken over a long period of time. Who Is Hydroxycarbamide Recommended for? Hydroxycarbamide is often recommended if your child is experiencing any of the following: • Frequent painful episodes affecting their quality of life • Has had one or more episodes of acute chest syndrome • They are no longer tolerating blood transfusion programme or they have had no further brain changes on their MRI after being on transfusion for at least a year with normal TCD • Any other complications related to sickle cell disease   Abstract source

Hydroxyurea is a medicine used to reduce the problems caused by sickle cell disease. Other names for this drug are Droxia and Hydrea. People with sickle cell disease have sickle-shaped red blood cells that can block blood flow. Hydroxyurea increases a type of hemoglobin, called fetal hemoglobin that makes the red blood cells bigger and helps them stay rounder and more flexible. Hydroxyurea is also used to treat certain types of cancer. The dose of Hydroxyurea is different based on the health condition it is being used to treat. Why is Hydroxyurea Helpful in Treating Sickle Cell Disease? Research has shown that taking Hydroxyurea for sickle cell disease lowers the number of: • Acute chest syndrome events • Pain crises • Blood transfusions • Trips to the hospital Abstract source

Hydroxyurea is a medicine that doctors have used to treat people with sickle cell disease since the 1980s. The Food and Drug Administration (FDA) approved it for treating adults with sickle cell disease in 1998. In 2017, the FDA approved it to treat children with sickle cell disease. Hydroxyurea is also used to treat cancer. But doctors use a lower dose (amount) to treat sickle cell disease than to treat cancer. How Can Hydroxyurea Help With Sickle Cell Disease? Hydroxyurea reduces the problems that sickle cell disease causes. People with sickle cell disease who take hydroxyurea have fewer: • Pain crises • Episodes of acute chest syndrome • Blood transfusions • Hospital stays Abstract source

Introduction: The main therapeutic intervention for sickle cell disease (SCD) is hydroxyurea (HU). The effect of HU is largely through dose-dependent induction of fetal hemoglobin (HbF). Poor HU adherence is common among adolescents. Methods: Our 6-month, two-site pilot intervention trial, "HABIT," was led by culturally aligned community health workers (CHWs). CHWs performed support primarily through home visits, augmented by tailored text message reminders. Dyads of youth with SCD ages 10-18 years and a parent were enrolled. A customized HbF biomarker, the percentage decrease from each patients' highest historical HU-induced HbF, "Personal best," was used to qualify for enrollment and assess HU adherence. Two primary outcomes were as follows: (1) intervention feasibility and acceptability and (2) HU adherence measured in three ways: monthly percentage improvement toward HbF Personal best, proportion of days covered (PDC) by HU, and self-report. Results: Twenty-eight dyads were enrolled, of which 89% were retained. Feasibility and acceptability were excellent. Controlling for group assignment and month of intervention, the intervention group improved percentage decrease from Personal best by 2.3% per month during months 0-4 (P = 0.30), with similar improvement in adherence demonstrated using pharmacy records. Self-reported adherence did not correlate. Dyads viewed CHWs as supportive for learning about SCD and HU, living with SCD and making progress in coordinated self-management responsibility to support a daily HU habit. Most parents and youth appreciated text message HU reminders. Conclusions: The HABIT pilot intervention demonstrated feasibility and acceptability with promising effect toward improved medication adherence. Testing in a larger multisite intervention trial is warranted. Abstract source Full text

Objectives: To examine the effect of a community health worker (CHW) intervention, augmented by tailored text messages, on adherence to hydroxyurea therapy in youths with sickle cell disease, as well as on generic and disease-specific health-related quality of life (HrQL) and youth-parent self-management responsibility concordance. Study Design: We conducted a 2-site randomized controlled feasibility study (Hydroxyurea Adherence for Personal Best in Sickle Cell Treatment [HABIT]) with 2:1 intervention allocation. Youths and parents participated as dyads. Intervention dyads received CHW visits and text message reminders. Data were analyzed using descriptive statistics, the Wilcoxon signed-rank test, and growth models adjusting for group assignment, time, and multiple comparisons. Changes in outcomes from 0 to 6 months were compared with their respective minimal clinically important differences. Results: A total of 28 dyads (mean age of youths, 14.3 ± 2.6 years; 50% Hispanic) participated (18 in the intervention group, 10 in the control group), with 10.7% attrition. Accounting for group assignment, time, and multiple comparisons, at 6 months intervention youths reported improved generic HrQL total score (9.8 points; 95% CI, 0.4-19.2) and Emotions subscale score (15.0 points; 95% CI, 1.6-28.4); improved disease-specific subscale scores for Worry I (30.0 points; 95% CI, 8.5-51.5), Emotions (37.0 points, 95% CI, 9.4-64.5), and Communication I (17.8 points; 95% CI, 0.5-35.1); and 3-month dyad self-management responsibility concordance (3.5 points; 95% CI, -0.2 to 7.1). There were no differences in parent proxy-reported HrQL measures at 6 months. Conclusions: These findings add to research examining effects of behavioral interventions on HrQL outcomes in youths with sickle cell disease. Abstract source Full text

Background: Despite medical benefits, hydroxyurea adherence in adolescents is often poor. As part of a baseline assessment of 28 youth (10-18 years) parent dyads who participated in a 6-month feasibility trial to improve hydroxyurea adherence, we measured the relationship between greater barriers to adherence and health-related quality of life (HRQL) from youth and parent perspectives. Procedure: Barriers were measured using the Adolescent and Parent Medication Barriers Scales with nine hydroxyurea items added. Barriers reported by >=25% of the sample were considered common. Generic and disease-specific HRQL were measured by PedsQL and PedsQL Sickle Cell Disease modules. Data were analyzed using descriptive statistics, Cronbach alpha, Spearman correlation coefficients, and paired t tests. Results: Fifty-six subjects (28 dyads) participated. Youth reported greater barriers compared with parents (5.0 ± 3.9 and 3.5 ± 3.2; P = 0.03), with >80% of respondents reporting >=1 barriers. Twelve barriers were reported by >=25% of adolescents, whereas six were reported by >=25% of parents. Of these, only two were common to both dyad members. Approximately one-third of youth had generic and disease-specific HRQL scores that fell at or below cutoff scores, suggesting being at risk for impaired HRQL. Greater barriers were inversely associated with poorer generic (parent r = -0.43, P = 0.03; youth r = -0.44, P < 0.001) and disease-specific HRQL (parent r = -0.53, P = 0.005; youth r = -0.53, P < 0.001). Conclusions: Hydroxyurea barriers were frequently reported but differed by dyad members' perspective. Greater barriers were associated with poorer generic and disease-specific HRQL. To reduce barriers to hydroxyurea in youth with sickle cell disease, perspectives of both dyad members should be addressed. Abstract source Full text

Background: Hydroxyurea (HU) is recommended as standard practice for youth with sickle cell disease (SCD). Yet, despite its efficacy, HU adherence in adolescents and young adults is often poor. Poor medication adherence increases disease burden, healthcare cost and widens health disparities. Adolescence is a critical time to improve adherence through improved chronic disease self-management. This study aims to test the efficacy of an intervention delivered to youth/parent dyads by community health workers (CHWs), augmented by tailored text messages on HU adherence (primary outcome). Secondary outcomes are intervention sustainability, youth health-related quality of life, self-management responsibility concordance, acute hospital use and self-reported disease symptoms. Methods: Hydroxyurea Adherence for Personal Best in Sickle Cell Disease, “HABIT,” is a 12 month multi-center randomized controlled trial. One hundred four youth, 10 to 18 years of age prescribed HU who meet eligibility criteria, enrolled with their parent as dyads, will be randomized 1:1 to either the HABIT intervention or to usual clinical care plus education handouts. All subjects will complete clinic visits at months 0, 2, 4, 6 (efficacy component), 9, and 12 (sustainability component) for assessment of HbF biomarker, other hematologic parameters, and to complete questionnaires. In addition, dyads assigned to the HABIT intervention will work with CHWs to identify a daily habit (e.g., brushing teeth) on which to build a HU adherence habit. Tailored daily text message reminders to support the habit will be developed by the dyad in collaboration with the CHWs and sent to parent and youth. At the 6 month visit, the intervention will end and the sustainability portion of the trial will begin. All data analyses will be based on intention to treat with all randomized subjects included in the analyses. Discussion: Prior retrospective studies demonstrate that a majority of adolescents are poorly adherent to HU. If efficacious, the HABIT intervention has the potential to improve the lives of youth with SCD. Abstract source Full text

Background: Hydroxyurea reduces pain crises, acute chest syndrome and blood transfusions in sickle cell disease (SCD), but potential detrimental effects on fertility and birth outcomes are an impediment to its use. Patient data on the effects of hydroxyurea when taken during conception and pregnancy are scarce. The Sickle Cell Disease Implementation Consortium (SCDIC) is a cooperative research program of eight clinical centers, a data coordinating center, and the National Heart, Lung, and Blood Institute (NHLBI). One of the SCDIC programs is an SCD patient registry including at least 300 participants from each center. Methods: The SCDIC Registry retrospectively questioned participants regarding pregnancy history, hydroxyurea use and pregnancy outcomes. Of 2436 subjects 15-45 years of age, 2375 answered at least one pregnancy-related question, including hydroxyurea use during conception, the month and year the pregnancy ended, the outcome (live birth, stillbirth, miscarriage, abortion, currently pregnant), hydroxyurea use during pregnancy and what trimester (females only), and for live births - prematurity, birth weight < 5.5 lbs, and medical complications in the baby. The primary outcome was miscarriage or stillbirth, with separate analyses for males and females. Univariate logistic regression was used to identify significant covariates and predictors, and multivariate logistic regression was used to select the best fitting models. All models included severe sickle cell genotype, pregnancy order (1-3, 4-6, >6) and history of miscarriage or stillbirth at each pregnancy. Results: Among 1,357 women, 1797 pregnancies were reported for a crude rate of 1.32 pregnancies per woman; among 1018 men, 620 pregnancy conceptions were reported for a rate of 0.61 per man. Overall, in 221 (12.3%) of reported pregnancies in women and in 122 (19.7%) of reported pregnancies conceived by men, conception occurred while on hydroxyurea (P<0.001). We further analyzed 2062 pregnancies that did not end in elective abortion as reported by 920 individuals. Pregnancy order, severe sickling genotype of the parent and prior history of still birth or miscarriage were significantly associated with stillbirth or miscarriage in univariate analyses. After adjustment for these covariates, hydroxyurea use at conception was associated with a 2.1-fold increase in the odds of miscarriage or stillbirth in pregnancies conceived by males (P = 0.046) and hydroxyurea use at conception and during the first trimester was associated with a 3.3-fold increase in the odds of miscarriage or stillbirth in pregnancies conceived by women (P<0.001). Hydroxyurea use at conception and during the first trimester was also independently associated with birth weight <5.5 lbs (OR 2.4, P=0.01), but not prematurity or medical problems in the newborn. Discussion: There are important limitations to our study. The pregnancy history is self-reported and therefore complicated by recall bias. The completion of the reproductive history form varied by age. The dates of pregnancies ending in miscarriage were more often missing than other outcomes, so we could not use age at pregnancy in statistical analyses. Males may not have known or remembered all of the pregnancies they conceived that did not end in live birth, and we did not collect information about the female partners of males with SCD. Furthermore, the availability and use of hydroxyurea has increased over time. Nevertheless, our results suggest that the use of hydroxyurea at conception in males and at conception and the first trimester in females may be associated with adverse pregnancy outcomes. Our findings support advising patients who plan a pregnancy to temporarily discontinue hydroxyurea before attempting to conceive and during the first trimester in females, and to provide alternative approaches to prevent SCD complications during this time. Abstract source