Maximum tolerated dose" vs "fixed low-dose" hydroxyurea for treatment of adults with sickle cell anemiaTo the Editor:Despite evidence that hydroxyurea is effective for sickle cell anemia (SCA), implementation of the "maximum tolerated dose" regimen in adults is poor. 1 We recently reported a study of "fixed low-dose" hydroxyurea (500 mg/day) for adults with SCA in Ibadan, Nigeria. 2Here we compare the outcomes of "fixed low-dose" hydroxyurea in Nigeria with "maximum tolerated dose" hydroxyurea in the United States and Canada as reported in the Multicenter Study of Hydroxyurea (MSH). 3,4The MSH was the seminal trial conducted in the 1990s that led to FDA approval of hydroxyurea for SCA.Responses to "fixed low-dose" and "maximum tolerated dose" hydroxyurea appear to be similar.We propose that these two regimens be compared in a prospective trial in adults with SCA.Hydroxyurea for SCA increases hemoglobin F and reduces pain crises, acute chest syndrome and blood transfusions. 5The agent is initiated at 15 mg/kg/day in adults followed by dose escalations up to 35 mg/kg/day if tolerated-the "maximum tolerated dose" approach of the MSH. 3,4Doses in this range may enhance survival, 6 however, neutropenia and thrombocytopenia are limitations to achieving maximal dose, frequent blood count monitoring is required, and treatment interruption is usually necessary. 4,5This limits the use of hydroxyurea in parts of Africa where frequent blood monitoring is not feasible. 7In the United States and United Kingdom, the complexity and need for frequent monitoring of the recommended "maximum tolerated dose" regimen cause primary care providers to hesitate to prescribe hydroxyurea 8 and create barriers to compliance. 9In the original MSH study, pain crises in SCA adults decreased during the first 12 weeks of hydroxyurea 15 mg/kg/day before dose escalation and before maximal increase in F-cells.
Background Sickle cell disease (SCD), a disease characterized with abnormal red blood cell morphology and is associated with diverse clinical manifestations and contribute to many psychosocial problems like social stigma, strained relationships and reduced self-esteem. SCD is highly prevalent in Sub-Saharan Africa, with Nigeria having the largest burden. This qualitative study is aimed at exploring possible psychosocial and academic challenges associated with sickle cell disease among undergraduates in Nigeria. Methods From September 2023 to February 2024, the authors conducted an exploratory descriptive study using in-depth interviews among 22 undergraduate students with SCD at Bowen University, Osun State, Nigeria, employing an in-depth interviewer guide. Ethical approval was obtained from Bowen University Ethical Review Board prior to the conduct of the study. The data was analyzed using inductive thematic analysis. Results Six (6) distinct themes emerged from the research data and each of the themes was linked to the study objectives. Many of the participants expressed disruption of academic activities by frequent SCD crisis. This negatively affected their academic performance, and sometimes led to feelings of frustration. Furthermore, the recurrent battles with pain due to vascular occlusion took a toll on their emotional and psychological health. They also experienced stigmatization and strained interpersonal relationships which negatively influenced their mental well-being. Conversely, some SCD patients enjoy some social support from colleagues and family members which gives hope and succor to them during difficult times. Conclusion This study reveals that undergraduates with SCD face various academic and psychosocial challenges that affect their overall performance. The findings underscored the need for increased awareness, support, and understanding to better assist undergraduates with SCD in managing their health and academic responsibilities effectively. Considering the chronic nature of SCD and its myriads of psychosocial and academic challenges, measures should be put in place to mitigate the challenges and enable them to live a fulfilling life.
Poor outcomes for breast cancer in Ghana have been attributed to late presentation of symptoms at biomedical facilities. This study explored factors accounting for delays in initiation of breast cancer treatment at the Korle-Bu Teaching Hospital in Accra. Focus group discussions were conducted with 20 women with breast cancer. A theory-driven thematic analysis identified three multilevel factors influencing treatment seeking delays: (1) patient (e.g. misinterpretation of symptoms, fear), (2) healthcare provider (e.g. negative attitudes) and (3) health systems (e.g. shortage of medicines). Addressing treatment delays will require multilevel interventions, including culturally congruent education, psychosocial counselling/support and strengthening health systems.
Sickle cell anaemia (SCA) is associated with macrovascular complications at relatively normal blood pressures. This has led to the development of the term 'relative systemic hypertension' (RSH). The electrocardiography (ECG) and echocardiography (ECHO) findings in these people has not been well highlighted. Patients with SCA in steady state were consecutively recruited. History, physical examination, ECG and ECHO information were obtained from all participants after informed consent was obtained. Eighty-three people were recruited in all- 15 of which had RSH, giving a prevalence of 18.1%. Those with RSH had higher packed cell volumes (PCV), smaller right atria area, lower tricuspid regurgitant velocities, lower incidence of early satiety, longer QTc and higher frequency of a history of vaso-occlusive crises. The indices of right and left ventricular function were normal in both groups. Right atrial area was the only significant determinant of RSH in this study. RSH is associated with higher PCV, longer QTc and smaller right atrial area in SCA patients. More studies to evaluate sympathetic output in SCA with RSH is required.
Abdominal pain is a relatively frequent occurrence in sickle cell disease. The aetiology of abdominal pain in sickle cell disease is often difficult to diagnose clinically. Despite the frequent occurrence, diagnostic dilemma, and the need for an accurate, early diagnosis, abdominal pain in sickle cell disease has not been rigorously studied.We therefore sought to describe the different presentations and patterns of abdominal pain in persons with sickle cell disease.A prospective case series of 20 patients was done in which data was collected on demographic characteristics, hemoglobin electrophoresis patterns, a description of the abdominal pain including sites, severity, and type of pain, packed cell volume and the provisional and final diagnosis.Haemoglobin S patients were 17 in number constituting eightyfive percent (85%) of our study population whilst the rest 3 were Hb S+C. Most patients (70%) had one site of abdominal pain. The pain was mainly colicky or tightening, moderate to severe in nature and, in some cases, associated with vomiting. We did not find any significant difference between the steady state PCV and the PCV during the acute abdominal pain episodes. The final diagnosis showed that only 38.8% of the patients had vasoocclusive crises and the reliability index between the provisional diagnosis and the final diagnosis was 67%.Abdominal pain in sickle cell disease may present in different ways and it is important to recognize that the possible diagnoses are numerous. Not all cases are due to vasoocclusive crises. Early diagnosis and prompt treatment can be life saving.
Abstract Background/Aims: Sickle cell anemia also known as haemoglobin SS (HbSS) is a genetic disease arising from the replacement of glutamic acid with valine at position 6 of the beta hemoglobin chain. This vaso-occlusive disease affects most of the organs in the body with the spleen commonly affected resulting in recurrent infarction. This study aims to assess the relationship between the ultrasound splenic length (LS) with the steady state packed cell volume (PCV), frequency of blood transfusion, and anthropometric parameters (weight and height) among patients with sickle cell anemia. Materials and Methods: This is an observational cross-sectional study with 128 consenting HbSS patients recruited. Sickle cell anemia (HbSS) patients with no crises/illness within the last 4 weeks prior to the study period were included in the study. Patient’s demographics, steady PCV and ultrasound findings of the spleen were documented into the study pro forma. Data were analyzed using the Statistical package for the Social Sciences software version 21. Mean, median, standard deviation, and Chi-square were used. A P ≤ 0.05 was considered statistically significant and a confidence interval (CI) of 95%. Results: The median age for all the patients was 19.00 years with a CI of 19.06–23.2 years. For children (2–17 years), the median age was 11.00 years with a CI of 9.35–11.36 years while for the adults was 28.00 years with a CI of 27.40–31.77 years. The median steady-PCV obtained in this study was 25.0%. Adult HbSS patients with normal LS had a significant correlation with the steady PCV. Conclusion: Normal-sized spleen on sonography may be a pointer to the steady PCV in sickle cell anemia patients. This study, therefore, suggests the need for sonography of the spleen in HbSS patients as part of their routine follow-up investigations.
Introduction: Mammographic density is a strong predictor of breast cancer. Menopause has a significant effect on breast pattern and has been documented to have more important influence on the decline in mammographic densities than age. The aim of this study was to describe the breast parenchymal density patterns in postmenopausal women in Ibadan and correlate these with their socio-demographic and anthropometric characteristics. Methodology: This is a retrospective cross-sectional study of 196 postmenopausal women who had two screening views done on each breast in the Radiology Department of the University College Hospital Ibadan. Data was pulled from completed questionnaire by patient who had mammogram during the period under review. The report of the mammograms were matched with patients’ socio-demographic characteristics and entered into R statistical package for analysis. Results: The mean age of the women was 55.0±6.8 years while their mean age at menopause was 48.4±4.6 years. The combined BI-RADS 1 and 2 breast parenchymal patterns which are associated with low risk of breast cancer, were found in 82.1% of the women. The mean difference in age at first birth between women with BI-RADS 1 and 2 and BI-RADS 3 and 4 was statistically significant P=0.035. Body mass index, family and personal history of breast cancer also showed correlation with breast parenchymal pattern, though not statistically significant. Conclusion: The low prevalence of dense breast pattern in this study is consistent with previous findings of relatively lower prevalence of breast cancer in African women. The clustering of low mean age at birth of first child, low mean age at menopause and multi-parity, in association with the low mammographic density in majority of the women in this study further corroborates the relatively reduced risk of breast cancer in our studied population.
Background: Sickle cell disease (SCD) is a heterogenous group of inherited haemoglobin disorder characterized by inheritance of β s gene and another β s gene (homozygous sickle cell anaemia -SCA) or haemoglobin S gene (HbSC), haemoglobin D or haemoglobin E gene.. SCA is the commonest type accounting for about 70% of SCD worldwide. Nigeria has the highest burden worldwide with an estimated 150,000 SCD births annually out of the estimated 275,000 annual global births. SCD patients presents with acute painful, haemolytic and sequestration crises, acute chest syndrome, and stroke.
Aims/Objectives: To describe the pattern of presentation and treatment modalities offered SCD patients in the setting of a University health centre.
Materials and method: A descriptive cross sectional study was conducted at the University of Ibadan Health Centre among SCD patients who presented over a five-year period. Data was extracted from the case folders and analyzed using SPSS version 23.
Results: Seventy-eight SCD patients were documented to have utilized the health centre in the period under review. The median age was 24 years (IQR: 16, 52). The male to female ratio was 1.3: 1. Homozygous HbSS constituted 64(82%) while 14(18%) were HbSC. All the patients had presented to the clinic on account of bone pain crisis and a few 12(15%) presented with symptoms of haemolytic crises. The commonest used analgesics were NSAIDS 54(74%), dihydrocodeine 43(54%)) and pentacozine 43 (54%).
Conclusion: Sickle cell disease is a life- long disease where cure is not readily attained hence the recommendation of early intervention of expert to ensure proper management of acute complications of the disease. There is a need to continue to train General practitioners on current management principles and guidelines in care of SCD patients.
Keywords: Sickle cell disease, presentation pattern, analgesic usage, University Health Centre, Ibadan.
ResumeContexte: La drepanocytose (SCD) est un groupe heterogene de trouble hereditaire de l’hemoglobine caracterise par l’heredite du gene âs et d’un autre gene âs (drepanocytose homozygote -SCA) ou du gene de l’hemoglobine C (HbSC), de l’hemoglobine D ou du gene de l’hemoglobine E. Le SCA est le type le plus courant representant environ 70% des SCD dans le monde. Le Nigeria a le fardeau le plus eleve au monde avec environ 150 000 naissances de drepanocytose par an sur les 275 000 naissances annuelles dans le monde. Les patients SCD presentent des crises douloureuses aigues, hemolytiques et de sequestration, un syndrome thoracique aigu et un accident vasculaire cerebral.Buts / Objectifs: Decrire le modele de presentations et les modalites de traitement offertes aux patients atteints de drepanocytose dans le cadre d’un centre de sante universitaire.
Materiel et methode: Une etude transversale descriptive a ete menee au centre de sante de l’Universite d’Ibadan parmi des patients atteints de drepanocytose qui se sont presentes sur une periode de cinq ans. Les donnees ont ete extraites des dossiers de cas et analysees a l’aide de la version 23 de SPSS.
Resultats: Soixante-dix-huit patients atteints de drepanocytose ont ete documentes comme ayant utilise la clinique du centre de sante au cours de la periode consideree. L’âge median etait de 24 ans (IQR: 16, 52). Le ratio homme / femme etait de 1,3: 1. L’HbSS homozygote constituait 64 (82%) tandis que 14 (18%) etaient l’HbSC. Tous les patients s’etaient presentes a la clinique pour crise de douleur osseuse et quelques 12 (15%) presentaient des symptomes de crise hemolytique. Les analgesiques les plus couramment utilises etaient les AINS 54 (74%), la dihydrocodeine 43 (54%)) et la pentacozine 43 (54%).
Conclusion: La drepanocytose est une maladie a vie ou la guerison n’est pas facilement obtenue, d’ou la recommandation d’une intervention precoce d’un expert pour assurer une prise en charge adequate des complications aigues de la maladie. Il est necessaire de continuer a former les medecins generalistes sur les principes de prise en charge actuels et les lignes directrices en matiere de soins des patients atteints de drepanocytose.
Mots cles: drepanocytose, schema de presentation, usage analgesique, centre de sante universitaire, Ibadan.
Corespondence: Dr S. P. Ogundeji, Department of Haematology, College of Medicine, University of Ibadan, Ibadan Nigeria. E-mail: peterogundeji22@gmail.com
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