Perception to hydroxyurea therapy in patients with sickle cell disease: Report from 3 centers.

Background: Hydroxyurea (HU) is an hemoglobin F inducing agent used in the treatment of sickle cell disease (SCD). Aim: The aim of this study is to determine the perception of HU by people living with SCD. Materials and Methods: A pretested questionnaire was self-administered to known cases of SCD attending pediatrics and adult hematology clinics in three participating centers. Mothers of children <18 years responded on their behalf. Results: There were 101 responders, 49 (48.5%) males and 52 (51.5%) females, of which 24 (23.8%) were children <18 years and 77 (76.2%) were adults. The majority (n = 73, 72.3%) knew their phenotype. Up to 63 (62.4%) had crises in the past 3 months. Only 35 (34.7%) had heard of HU, many through their doctor (n = 16, 45.7%), 8 (22.9%) through online resources, and 7 (20%) from friends. Only 12 (11.9%) had been exposed to HU therapy, of which 5 (41.7%) had discontinued therapy mostly due to side effects (n = 2, 40%). The seven patients (58.3%) on continuous HU therapy for a duration of 6 months to over 5 years, all reported reduced hospital admissions and frequency of crises as benefits of the drug, whereas 4 (57.1%) had stopped requiring blood transfusion since starting therapy. Of those who had never taken HU, 53 (52.5%) believed that HU should be used in treating SCD and majority (n = 32, 60.4%) would want to be commenced on the drug. However, 8 (15.1%) would decline therapy (mostly due to perceived associated side effects; n = 4; 50%). Six (11.3%) were unsure if they would want the drug and 7 (13.2%) would have to discuss the decision first with their family. There were 8 (8.9%) responders who did not think HU will be beneficial in SCD and would decline treatment, while 26 (29.2%) were unsure of both the benefits of the drug or of commencing therapy. Conclusion: The findings from this study suggest that HU is beneficial for patients with SCD; however, the awareness of this medication among SCD patients is still low in our environment. Some SCD patients would decline the use of HU due to perceived side effects. We recommend that more awareness on HU be created and coordinated multi-center studies on the efficacy of HU in the Nigerian population be carried out.

RésuméContexte: L'hydroxyurée (HU) est un agent inducteur de l'hémoglobine F utilisé dans le traitement de la drépanocytose (SCD). Objectif: le but de cette L'étude vise à déterminer la perception de l'HU par les personnes atteintes de SCD. Matériel et Méthodes: un questionnaire pré-testé a été auto-administré aux cas connus de SCD fréquentant des cliniques de pédiatrie et d'hématologie pour adultes dans trois centres participants. Les mères d'enfants de moins de 18 ans ont répondu en leur nom. Résultats: Il y avait 101 répondants, 49 (48,5%) hommes et 52 (51,5%) femmes, dont 24 (23,8%) étaient des enfants de moins de 18 ans et 77 (76,2%) étaient des adultes. La majorité (n = 73, 72,3%) connaissait leur phénotype. Jusqu'à 63 (62,4%) ont eu des crises au cours des 3 derniers mois. Seulement 35 (34,7%) avaient entendu parler de HU, beaucoup par l'intermédiaire de leur médecin (n = 16, 45,7%), 8 (22,9%) par des ressources en ligne et 7 (20%) par des amis. Seulement 12 (11,9%) avaient été exposés à un traitement par HU, dont 5 (41,7%) avaient arrêté le traitement principalement en raison d'effets secondaires (n = 2, 40%). Les sept patients (58,3%) sous traitement HU continu pendant une durée de 6 mois à plus de 5 ans, tous ont signalé une réduction des hospitalisations et de la fréquence des crises comme bienfaits du médicament, alors que 4 (57,1%) avaient cessé de nécessiter une transfusion sanguine depuis le début du traitement. De ceux qui n'avaient jamais prises HU, 53 (52,5%) estimaient que HU devrait être utilisée dans le traitement de la drépanocytose et la majorité (n = 32, 60,4%) souhaiterait commencer le médicament. Cependant, 8 (15,1%) refuseraient le traitement (principalement en raison des effets secondaires associés perçus; n = 4; 50%). Six (11,3%) ne savaient pas siils voudraient le médicament et 7 (13,2%) devraient d'abord discuter de la décision avec leur famille. Il y avait 8 répondants (8,9%) quine pense pas que l'HU sera bénéfique dans la drépanocytose et refuserait le traitement, tandis que 26 (29,2%) n'étaient pas certains des avantages du médicament ou commencer la thérapie. Conclusion: Les résultats de cette étude suggèrent que l'HU est bénéfique pour les patients atteints de SCD; cependant, la conscience de ce médicament chez les patients SCD est encore faible dans notre environnement. Certains patients SCD refuseraient l'utilisation de l'HU en raison du côté perçu effets. Nous recommandons qu'une plus grande sensibilisation à l'HU soit créée et coordonne des études multicentriques sur l'efficacité de l'HU au Nigéria. population être effectuée.

Bortezomib Prescription Pattern for the Treatment of Multiple Myeloma by Hematologists in Nigeria.

PURPOSE: Novel therapy has dramatically changed the outcome of patients with myeloma. Current National Comprehensive Cancer Network guidelines give bortezomib-based combinations a central role in the management of multiple myeloma (MM). The aim of this survey is to assess the use of bortezomib for the treatment of MM by hematologists practicing in Nigeria. MATERIALS AND METHODS: This is a cross-sectional observational survey. A structured, prevalidated questionnaire was self-administered to different cadres of hematologists. Data collected were analyzed using SPSS software version 21 (IBM, Chicago, IL). RESULTS: There were 54 respondents from 24 centers across the country. The most frequently used drugs for first-line therapy were thalidomide (66.7%), dexamethasone (54.2%), and bortezomib (48%), and a combination of bortezomib, thalidomide, and dexamethasone (16.7%) was the most frequently used first-line regimen. Of the 54 hematologists, 39 (72.2%) had prescribed bortezomib previously; no one had used bortezomib as monotherapy. Drug unavailability (86.7%) and cost (46.7%) were the major reasons for those who had not prescribed bortezomib. Approximately 56.4% of responders had patients who had experienced adverse effects, of which neuropathy was the most common (86.3%). CONCLUSION: Bortezomib, thalidomide, and dexamethasone was the most frequently used first-line regimen to treat myelomatosis. Thalidomide and dexamethasone were the most frequently used drugs in myeloma treatment. Despite poor access to health care, coupled with the high cost and poor availability of bortezomib in our low- or middle-income country, those who prescribed bortezomib did so frequently (in more than half of their patients).

Effects of maternal hypertension on the neonatal haemogram in southern Nigeria: A case-control study.

BACKGROUND: Hypertension in pregnancy is a leading cause of maternal and neonatal morbidity and mortality. This study aimed to compare the hematological parameters in neonates of hypertensive mothers with those of normotensive mothers, and also to compare the incidence of polycythaemia, neutropenia and thrombocytopenia in both groups. METHODS: This was a hospital-based case control study. Three milliliters of cord blood from neonates of women with hypertension in pregnancy and those of normotensive pregnant women were sampled for haemogram parameters using a 3-part autoanalyser. Haematocrit and white blood cell differentials were done manually. Data were analysed using SPSS version 16. RESULTS: A total of 200 neonates were recruited, comprising 100 neonates of mothers with hypertensive disorders of pregnancy and 100 neonates of normotensive mothers. The mean haematocrit was significantly higher in neonates of hypertensive mothers than those of normotensive mothers. The neutrophil and platelet counts of neonates of hypertensive mothers were significantly lower than those of normotensive mothers. The incidences of polycythaemia, neutropenia, and thrombocytopenia were found to be 8%, 15%, and 38% among neonates of hypertensive mothers and 0%, 2%, and 8% among neonates of normotensive mothers, respectively. These incidences were significantly different between the groups. CONCLUSIONS: There was a positive association between hypertension in pregnancy and neonatal polycythaemia, neutropenia, and thrombocytopenia. Haematological parameters of neonates of mothers with hypertension in pregnancy should be properly evaluated and monitored to reduce the chances of developing complications associated with these abnormalities.