Screening Newborns for Sickle Cell Disease in Ghana

Abstract

high at the end of therapy. Suppressor cells normalized faster, whereas helper cell levels remained abnormally low for a long period. Most children became nonimmune to polio, whereas the majority had antibodies to measles, mumps, and rubella. Despite depressed immunity, serious infections were not documented. SUPPRESSION OF THE OLIVOCOCHLEAR REFLEX: A NEUROTOXIC ADVERSE EFFECT OF VINCRISTINE Submitted by Helen Kosmidis OBJECTIVE:The purpose of this study was to examine the effects of a known neurotoxic regimen, such as the acute lymphocytic leukemia (ALL) Berlin-Frankfurt-Mü nster 95 (ALL-BFM-95) protocol, on the function of the medial olivocochlear bundle, which was assessed by recording suppression of the amplitudes of distortionproduct otoacoustic emissions (DPOAEs) when white noise was applied simultaneously to the contralateral ear. METHODS: Our population consisted of 3 groups of children with ALL. A baseline examination was performed before the beginning of therapy. DPOAE-suppression measurements were repeated after 4 weekly doses of vincristine in the first group (n ϭ 12), after 8 weekly doses in the second group (n ϭ 12), and 3 years after completion of the protocol in the third group (n ϭ 23). In the third group, a subgroup of 12 children who were exposed to low-dose gentamicin (Ͻ13 days) and another 11 children who were exposed to high gentamicin doses (Ͼ23 days) were evaluated. RESULTS: At baseline examination, all groups presented significant suppression at all frequencies. Efferent mediated DPOAE suppression was still present after 4 sessions of vincristine. However, after 8 vincristine sessions, instead of suppression, an increase of amplitudes was noted at 5 (of 12) frequencies. In the subgroups examined 3 years after ALL-BFM-95, the olivocochlear reflex had recovered. CONCLUSIONS: Enhancement or no significant suppression of OAEs by contralateral noise indicates a probable vincristine-induced insult to the efferent cochlear innervation. This adverse effect seems to take place early in the course of chemotherapy and is slowly reversed a few years after chemotherapy. The clinical implications of these findings may need additional investigation. SCREENING NEWBORNS FOR SICKLE CELL INTRODUCTION:Screening of newborns for sickle cell disease (SCD) allows early initiation of prophylactic therapy, parental education, and comprehensive management, which results in reduced mortality. Since April 1993, a demonstration project to develop and implement a program of newborn screening for SCD has been conducted in Kumasi, Ghana, by the Comprehensive Sickle Cell Center at the Children's Hospital of Philadelphia in collaboration with the Ministry of Health and other institutions in Ghana. OBJECTIVE: Our goal was to assess the program of screening and follow-up of children with SCD in Ghana. METHODS: Infants are screened at birth or at wellinfant visits within days or a few weeks after birth. Mothers are asked to come for results within 4 weeks, and failing that, an extensive tracking system is used to deliver r...

Community engagement to inform the development of a sickle cell counselor training and certification program in Ghana

“…Although national NBS programs for SCD are not established in Africa at present, several pilot schemes in different countries have demonstrated the significance of NBS and early management (Mutesa et al 2007;Ohene-Frempong et al 2008;Kafando et al 2009;Rahimy et al 2009;Tshilolo et al 2009;McGann et al 2013a). Furthermore, one of these pilot NBS and treatment programs implemented in Angola was shown to be cost effective.…”

Sickle cell disease: reappraisal of the role of foetal haemoglobin levels in the frequency of vaso-occlusive crisis

“…3 The deoxygenated Haemoglobin S polymerizes by sticking to each other to form long strands, which stretches the membrane of the red blood cell. 4,5,6 This defect causes polymerization of haemoglobin molecules and erythrocyte sickling under hypoxic conditions 7,8,2 leading to VOC. VOC is caused by sickled red blood cells which obstruct capillaries and restrict blood flow to an organ, resulting in ischemia, pain, and organ damage 9,10 and it is responsible for the high rate of morbidity 3 and mortality 2 in SCD.…”

“…20,21,22 Patients with SCD who have elevated HbF levels have fewer painful crises and improved survival 20 as shown by studies that fetal haemoglobin, which lacks β -globin chains, inhibits sickling in vitro by interfering with the polymerization of haemoglobin S 23,24 . Since polymerization of deoxyHbS is the signal event in the pathogenesis of SCD 4 , HbF could effectively prevent disease manifestation.…”

A Global Perspective on Milestones of Care for Children with Sickle Cell Disease

“…Africa bears the highest burden of SCD. In the past years, several pilot newborn screening programs have been implemented in Central Africa [21], Ghana [20,22], Congo [24], Benin [25], Angola [2 ], Nigeria [23,54,55] and Uganda [5 ] and are underway in Tanzania [1 ]. Some of the most signiicant experiences are described in detail below.…”