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Sickle Cell Service to Humanity Foundation,
Katsina State, Nigeria: Exploratory Visit
Nigerian Sickle Cell Cohort Team, UNDP N-253 project contact (+2348036063948)
Dr Baba Inusa (Chief
Investigator)
Dr Uduak Essen (Project Coordinator)
CarDan Charity Rep
Suleiman Baba Mohammed
Her Excellency,
Dr Fatima I. Shema, Founder Service to Humanity Foundation (SHP)
SHP members
Alh Lawal Yunusa Charanchi (Coordinator-SHP)
Hajiya Halima Idowu (Coordinator
Sickle Cell SHP)
28th June 2010
Sickle Cell Service to Humanity Foundation,
Katsina State, Nigeria: Exploratory Visit
Nigeria Sickle Cell Cohort Study team
Rational For Newborn Screening for Sickle
Cell Disease
The Case For SCD Screening Africa
N-253 UNDP Pilot Project
Developing a Pathway
Partnership with Katsina
28th June 2010
Sickle Cell Service to Humanity Foundation,
Katsina State, Nigeria: Exploratory Visit
Left to right
Dr Funke Lawson,
medical director Zankli and Investigator Nigeria
Dr Yvonne Daniel, Scientfic Advisor
Dr Baba Inusa
Dr Uduak Essen
Prof S Obaro, Michigan State
University and Deputy Chief Investigator
28th June 2010
Sickle Cell Service to Humanity Foundation,
Katsina State, Nigeria: Exploratory Visit
Fantsuam
Foundation Partner Sickle Cell Cohort Study- N-253
From left-
Dr Baba Inusa,
Mrs Bola Ojo,Overall project manager
Dr John Dada, Chief Exceuttive Fantsuam Foundation
Dr Yvonne Daniel
28th June 2010
Newborn Screening for SCD Design Goals
• Public-health based, pilot project for national screening program
• Test ALL babies born in urban and rural communities at birth or at first postnatal visit
• Transport samples to distant screening laboratory
• Track ALL babies with SCD and refer to Sickle Cell Clinic
• Provide longitudinal, comprehensive care through Sickle Cell Clinic and hospital
• Develop and implement database systems for tracking of ALL babies screened and monitor clinical care for children with SCD
• Evaluate screening project on regular basis
28th June 2010
Newborn Screening for Sickle Cell DiseaseJustification
1. Very common, serious condition with clinical manifestations starting in infancy
• Aged 6 - 12mo had highest mortality rate.
2. Simple, accurate diagnostic tests available• Isoelectric focusing, HPLC methods highly accurate and
simple
3. Proven life-saving, inexpensive preventive treatment available
• Penicillin prophylaxis is simple, inexpensive, and effective
28th June 2010
Newborn Screening for Sickle Cell Disease and Other Hemoglobinopathies
National Institutes of Health
Consensus Development Conference Statement
April 6-8, 1987
Conclusions (cont’d)
5. To be effective, neonatal screening must be part of a comprehensive program for the care of sickle cell patients and their families. These services must include a network of providers who ensure optimal medical care, psychosocial support, and genetic counseling. Such follow-up capabilities should be in place before screening is instituted.
6. Further research should focus on the following: improving and evaluating the
technology for screening;
In summary, the panel concludes that every child should be screened for hemoglobinopathies to prevent the potentially fatal complications of sickle cell disease during infancy.
28th June 2010
Newborn Screening for Sickle Cell Disease and Other Hemoglobinopathies
National Institutes of Health
Consensus Development Conference Statement
April 6-8, 1987
Conclusions
1. Effective intervention in children with sickle cell disease provides a major impetus for
neonatal screening. Prophylactic penicillin therapy provided in a setting of
comprehensive care has been found to significantly reduce the morbidity and mortality
of patients with pneumococcal sepsis.
3. The benefits of screening are so compelling that universal screening should be
provided. State law should mandate the availability of these services while permitting
parental refusal.
4. Centralization of laboratory services improves efficiency and decreases the probability
of error.
28th June 2010
Newborn Screening for Sickle Cell Diseasein the U.S.
1. ALL babies screened for “SCD and Other Hemoglobinopathies” as part of state-organized newborn screening programs
2. Good evidence that newborn screening followed by comprehensive medical care including parental health education, penicillin prophylaxis, and anti-pneumococcal vaccination have reduced early mortality in SCD
28th June 2010
Newborn Screening for Sickle Cell Disease
in Africa
• What is the justification in Africa?
• Mortality from pneumococcal sepsis in infancy?
• Mortality from malaria in infancy?
• Is there justification for anti-malarial or anti-
pneumococcal prophylaxis in Africa?
28th June 2010
Sickle Cell Disease in AfricaHigh childhood mortality
Fleming, 1989:
< 2% of expected number of children with SCD
survived beyond 5 years in a rural community.
Athale and Chintu, 1994:
SCD 2.92% of pediatric admissions;
case fatality (per admit), 6.61%, (55% for .5-5yr)
Thuilliez and Vierin, 1997:
8.4% of childhood deaths due to SCD
Koko, et al., 1998:
7.2% of childhood deaths at due to SCD
28th June 2010
Sickle Cell Disease in Africa
Burden of Sickle Cell Disease in
Africa
Total Population
(2000)
855,243,217 >140,000,000
Births/1000 36.8 40
Total Births 29,632,950 5,600,000
Total SCD Births @ 1% 296,330 56,000
@ 1.5% 444,495 84,000
@ 2.0% 592,660 112,00028th June 2010
Newborn Screening for Sickle Cell Disease
in Africa
• No country in Africa has implemented
national, newborn screening program for
sickle cell disease.
• Most children with SCD in Africa are not
diagnosed before clinical presentation,
complications, or death.
28th June 2010
EC-UNDP GRANT- N-253 Brussels
Abuja
18 Month Grant to support the Pilot
1st HPLC machine in Nigeria to support the diagnosis of SCD from birth
Community and Hospital project in Nigeria –FCT and Kaduna State
28th June 2010
Sickle Cell Service to Humanity Foundation,
Katsina State, Nigeria: Exploratory Visit
Laboratory Staff
Training at Zankli
Medical Centre
HPLC machine
14-22 June 2010
28th June 2010
28th June 2010
Newborn Screening for Sickle Cell Disease
Newborn screening is not JUST a blood test!
28th June 2010
Newborn Screening for Sickle Cell DiseaseOrganization of a Newborn Screening System
1. Screening: testing of newborns
2. Follow-up: short-term follow-up and referral of test-positive infants
3. Diagnosis: definitive diagnosis of disease
4. Management: planning and implementation of long-term therapy
5. Evaluation: validation of procedures assessment of interventions and outcomes
28th June 2010
Newborn Screening for SCD in Ghana
Tracking Babies with SCD
Tracking Babies to Homes• 32 Nurses are involved in tracking.
• Nurse Coordinators send screening results to the Public Health and Community Health Nursess in the screening health facilities for follow-up.
• Visits days are at the discretion of the nurses i.e after working hours and weekends.
• Families tracked based on directions given by mother at delivery
28th June 2010
Newborn Screening for Sickle Cell Disease
in Nigeria
Organizational Structure
Nigeria Sickle Cell Cohort
Zankli / Fantsuam F.
Laboratory CounselScreening Education Clinic
Review by NiSCOR board/MSU and GSTT
28th June 2010
Newborn Screening for Sickle Cell Disease
in Africa
WHO:• Has recognized SCD as a major public health problem
• Has recommended member nations to develop comprehensive programs to “control” SCD, including early diagnosis
ALL African and other nations where SCD is common should embark on newborn screening for SCD as a public health program
28th June 2010
Sickle Cell Service to Humanity Foundation,
Katsina State, Nigeria: Outcome of Discussion
Scope for Collaboration with Katsina State Her Excellency support and keenness to engage
international community.
A motivated foundation with over 7000 patients receiving free
medication and Specialist Maternities and Children Hospitals
Conditions for Extension of Project to Katsina Funding to cover additional equipment (HPLC), Vehicle for
community survey and follow up + overall activities
Ethical Clearance from the State
Equipment procurement, Staff Training and Skill transfer
Diaspora experts and technical staff visit to support work
28th June 2010
