Newborn Screening for Sickle Cell Disease in Ghana ... Programme of Newborn Screening for Sickle...

National Programme of Newborn Screening for Sickle Cell Disease

Newborn Screening

for

Sickle Cell Disease in Ghana

----------

Update

Prof. Kwaku Ohene-Frempong, MD

Children‟s Hospital of Philadelphia

University of Pennsylvania

Sickle Cell Foundation of Ghana

Sickle Cell Foundation of Ghana

Sickle Cell Foundation of Ghana

Why screen newborns for

sickle cell disease?

Newborn Screening for SCD

Causes of Death in SCD-SS (0-10 yr)

(Gill FM, et al, CSSCD: 1995) Overall incidence: 1.1/100 person-yrs

Sickle Cell Foundation of Ghana

Newborn Screening for SCD

Age (yr) <3 3-5 6-9 10-19 >20

No. pts. 459 571 630 958 983

Person-yr. 752 1025 1333 2843 3480

No. events 60 26 14 18 30

Incidence/ 7,979 2,536 1,050 633 862

105 Pr-yr

Deaths 11 1 4 0 2

(Zarkowsky,et al. CSSCD; J Pediatr 1986:579-585)

Bacteremia in SCD-SS Disease, CSSCD

Sickle Cell Foundation of Ghana

Newborn Screening for SCD

Age (yr) <1* 1-1.9 2-2.9 3-3.9 4-4.9 5-5.9

No. pts. 291 342 362 393 430 434

Pt.yrs. 188 268 296 334 346 345

No. events 12 17 17 3 5 4

Incidence 6,382 6,343 4,696 898 1,445 1,159

105 Pr-yr

US (98-99)^ 171 214 65 29 16

* 3 patients were younger than 6 months of age

^ MMWR, 2008 :57; 144

(Zarkowsky,et al. CSSCD; J Pediatr 1986:579-585)

Incidence of Strep. pneumoniae

in SCD-SS Disease, CSSCD

Sickle Cell Foundation of Ghana

Newborn Screening for SCD

Sickle Cell Foundation of Ghana

Newborn Screening for SCD

Sickle Cell Foundation of Ghana

Penicillin Placebo

No.of patients 105 110 (Person-yr 131.25 137.5)

Age 3-36m

Pneumovax 67% 71.6%

---------------------------------------------------------------------

Pneumococcal sepsis 2 13

(Incidence/105 Pr-yr 1,524 9,455)

Deaths 0 3

(Youngest septic patient: 6 mo. old)

(Gaston, et al. CSSCD; NEJM 1986:1593-99)

Penicillin Prophylaxis Study (I), CSSCD

Newborn Screening for SCD

Sickle Cell Foundation of Ghana

Newborn Screening for SCD

Pneumococcal Disease in Africa

Williams, et al., Lancet. 2009 Oct 17;374(9698):1364-70.

Interpretation

Introduction of conjugate vaccines against S pneumoniae and

H influenzae into the childhood immunisation schedules of

African countries could substantially affect survival of children

with sickle-cell anaemia

Infection in Sickle Cell Disease Malaria and Bacteremia Risks in SCD – Kenya

7164 hospitalized children, < 5y, living on the coast of Kenya

Age-adjusted odds ratios for disease-specific admission by Hb S genotype

Case category Hb AA Hb AS p* SCA p*

Uncomplicated

malaria 1

0.24 (0.17-

0.36) <0.001

0.43 (0.09-

2.10) 0.30

Severe malaria 1 0.12 (0.08-

0.18) <0.001

0.80 (0.25-

2.51) 0.70

Non-malaria

illnesses 1

0.78 (0.60-

1.01) 0.066

4.17 (1.95-

8.92) <0.001

Bacteremia 1 0.60 (0.48-

0.76) <0.001

8.73 (4.51-

16.89) <0.001

S. pneumoniae 1 15.3 (7.3-

35.1) <0.001

McAuley, et al., Blood. 2010 Sep 9;116(10):1663-8.

Newborn Screening for SCD

Values are ORs (95% CI) compared with the reference group (HbAA) adjusted for age group

(0-23 months, 24-59 months) by logistic regression.

Newborn Screening

for Sickle Cell Disease in Ghana

Kumasi-Tikrom Pilot Project

Sickle Cell Foundation of Ghana

Newborn Screening for SCD in Ghana

Sickle Cell Foundation of Ghana

Kumasi-Tikrom Pilot Project

Project Funding

1. U.S. Government:

National Heart, Lung, and Blood Institute (NHLBI) of

National Institutes of Health (NIH), Comprehensive Sickle

Cell Center Grant: 1993 - 1998; 1998 - 2003; 2003 – 2008

2. Government of Ghana

Ministry of Health and Ghana Health Service

- Staff

3. Children‟s Hospital of Philadelphia (CHOP)

- Staff and donations

4. General public

- donations

Newborn Screening for SCD in Ghana

Results: Feb 15, 1995 – Dec 31, 2011

Babies with SCD totaled 6,025 (1.75%)

Hb Phenotype Number Percentage

F 63 0.01

FA 259449 75.6

FAC 30253 8.9

FAS 45785 13.3

FC 1637 0.5

FS 3186 1.0

FSA 27 0.008

FSC 2812 0.8

OTHER 143 0.04

TOTAL 343,355 100

Babies with Possible SCD 6,025 1.8

Total accounted for 5,637 100.0

Lost to follow-up 784 13.9

Total contacted 4,853 86.1

(19% reported, 81% tracked)

Dead by contact date 43 0.76

Eligible for enrollment 4,810

Known dead after enrollment 4.5

No. (%)

Total screened with results 343,355

Newborn Screening for SCD in Ghana

Tracking Results: Feb 15, 1995 – Dec 31, 2011

Still under active tracking 388 6.4

Clinic Population

SCD Clinic started on 14th December, 1992

with 10 adult patients

Total Number of registered patients in

Clinic by 30th September, 2011:

Newborn screened: 4,028

Non-newborns: 8,168

GRAND TOTAL 12,196

KUMASI CENTER FOR SICKLE CELL DISEASE

Appointments-Routine Clinic Visits

Frequency of well-child visits:

Less than 2 yrs: Every 2 mo

More than 2 yrs: Every 3 mo

Adolescents and adults: every 4-6 mo

• Patients may be seen more frequently

depending on clinical status

Kumasi Centre for Sickle Cell Disease

Antimicrobial Prophylaxis

Penicillin prophylaxis:

o Recommended for the first five years but may be

optional after 5 years

o 125 mg twice daily (< 3 yr old);

o 250 mg twice daily (> 3 yr old).

o Erythromycin ethyl succinate (EES) 10

mg/kg/dose, twice daily for those allergic to

penicillin

Anti-malaria Chemoprophylaxis: ?? Unclear

recommendation: NMCP POLICY for high

risk subjects

Kumasi Centre for Sickle Cell Disease

Newborn Screening for SCD in Ghana

Sickle Cell Foundation of Ghana

Conclusions

Kumasi-Tikrom Pilot Project

1. Newborn screening for SCD makes sense in Africa.

2. Implementation of a Public Health Newborn Screening

Programme is feasible in Ghana in both rural and urban

settings.

3. Ghanaian healthcare systems and staff can support all

phases of comprehensive management of SCD.

4. Children with SCD born in Ghana, diagnosed at birth and

given comprehensive healthcare, have reduced mortality.

Sickle Cell Foundation of Ghana

1. US funding for pilot project ended March 2008

2. Screening has continued with support from MOH,

GHS, Children‟s Hospital of Philadelphia, and Sickle

Cell Foundation of Ghana (SCFG)

3. SCFG proposal for national scale-up submitted to

Ministry of Health 2006 and 2007

4. 2007-2010: Planning meetings among MOH, Ghana

Health Service, SCFG, National Health Insurance

Authority

5. SCFG secures support from Brazil Government

Progress Report (1/2)

Newborn Screening for SCD in Ghana

Sickle Cell Foundation of Ghana

6. Brazil-Ghana Technical Cooperation Agreement in

SCD signed (Sep 2009)

7. National Programme of Newborn Screening for SCD

launched Nov 2010

• Technical Advisory Committee inaugurated

• Screening Plan submitted to MOH, Nov 2011

• Steering Committee being established

8. National scale-up in ALL other Regions, 2012

9. Initial Regional Training Workshops completed Nov,

2011.

Progress Report (2/2)

Newborn Screening for SCD in Ghana

Technical Advisory Committee

DRAFT POLICY FOR NEWBORN SCREENING IN GHANA

AND

TECHNICAL PLAN

FOR

NATIONAL SCALE UP OF NEWBORN SCREENING

FOR SICKLE CELL DISEASE

November 1, 2011

Ghana: National Newborn Screening Program

Sickle Cell Foundation of Ghana

Population: 25.2 million

Birth Rate: 26.99 / 1,000 population

No. of Births: 680,148

SCD Rate: 1.8% (pilot newborn screening data)

No. SCD babies 12,243

SCD Births in Ghana (2012 Estimates)

Newborn Screening for SCD in Ghana

Ghana: National SCD Programme

Sickle Cell Foundation of Ghana

Organization of Centres for Sickle Cell Disease

National Coordinating Centre

Regional Centre for SCD

Sub-Regional Centres for SCD

Initial Education and Training Program

Training Program 1: Public Health Workers

Training Program 2: Clinical Staff of Centers

Training Program 3: “Sample Collectors”

Training Program 4: Data Entry Staff

Counselor Training and Certification Program

Ghana: National Newborn Screening Program

Ghana: National Newborn Screening Program

Training Program 1

Regional Workshops

on

Newborn Screening for Sickle Cell Disease

Ghana: National Newborn Screening Program

Training Program 1:Regional Workshops

H

H

H

H H

H

H

Ghana: National Newborn Screening Program

NNbSP-SCD Training Program 1 (2011)

Trainees by Profession

Community

Health

Nurses

General

Nurses

Public

Health

Nurses

Midwives Doctors Physician

Assists.

Others

TOTAL

60

(10.9)

80

(14.5)

85

(15.4)

127

(23.0)

111

(20.0)

54

(9.8)

34

(6.4)

551

(100)

Ghana: National Newborn Screening Program

NNbSP-SCD Training Program 1 (2011): Trainees

REGIONS %

Midwives

%

Nurses

%

Prescriber

s

% Other

Professions

Total #

Professionals

(%)

Ashanti 25.9 44.4 27.8 1.9 54 (9.8)

Brong Ahafo 29.5 41.0 29.5 0.0 61 (11.1)

Central 28.2 38.0 32.4 1.4 71 (12.9)

Eastern 26.3 36.3 35.0 2.5 80 (14.5)

Great. Accra 6.1 58.5 35.4 0.0 82 (14.9)

Northern 17.0 51.1 29.8 2.1 47 (8.5)

Upper East 16.7 50.0 33.3 0.0 24 (4.4)

Upper West 9.1 81.8 9.1 0.0 11 (2.0)

Volta 23.6 47.3 29.1 0.0 55 (10.9)

Western 32.8 34.3 32.8 0.0 67 (12.2)

ALL Regions 125 (22.7) 247 (44.8) 174 (31.6) 5 (0.9) 551 (100)

Workshop on

Newborn Screening

for Sickle Cell Disease

Pre- and Post-Test

Sickle Cell Foundation of Ghana

Test No. of

Participants

Average

% Correct

Median %

Correct

Pre-Test 412 64 + 28.3 69

Post-Test 397 85 + 14.3 86

Newborn Screening Workshop

Pre- and Post-test

Sickle Cell Foundation of Ghana

List the four most common types of SCD

Question 3

Test % Correct

Pre-Test 1

Post-Test 84

Newborn Screening Workshop

Pre- and Post-test

Sickle Cell Foundation of Ghana

Young children with severe SCD lose

function of their spleen after 5 years of age

Question 17

True / False

Test % Correct

Pre-Test 44

Post-Test 35

Newborn Screening Workshop

Pre- and Post-test

Sickle Cell Foundation of Ghana

What is the most important treatment after

newborns are diagnosed with SCD?

Question 24

Test % Correct

Pre-Test 8

Post-Test 90

National Newborn Screening Program

Training Program 1: Regional Workshop

Takoradi, Western Region, Nov 11-12, 2011

National Newborn Screening Program

Training Program 1: Regional Workshop

Koforidua, Eastern Region, Nov 17-18, 2011

1. October 2008: Application is submitted by Sickle Cell Foundation

of Ghana to Brazilian Ambassador to Ghana

2. February 2009: Prof. Ohene-Frempong meets with President

Mills; and, President Mills meets with Brazilian Ambassador

3. July 2009: 1st Complementary Agreement is signed in Accra

(Complementary to original 1974 Brazil-Ghana Technical

Cooperation Agreement)

4. August-October 2009: Training in Brazil (Lab Techs and Nurses)

5. October 2009: Year 1 Technical Agreement is signed in Brazil

6. February 2011: 2nd Complementary Agreement is signed in Accra

Sickle Cell Foundation of Ghana

International Partnership in SCD: Ghana

Brazil-Ghana Partnership

Program Areas

1. Expansion of Screening Laboratory Capacity

2. Training of Lab Technologists, Nurses

3. Development of Educational Materials and Educational

Program

4. Expansion of IT system and network

5. Construction of Blood and Sickle Cell Center in Kumasi

Sickle Cell Foundation of Ghana

International Partnership in SCD: Ghana

Brazil-Ghana Partnership

Thank you!

Brazil-Ghana Technical Cooperation in SCD Training of Technicians in Brazil, August 2009

Brazil-Ghana Technical Cooperation in SCD2nd Brazilian

Delegation Meets President Mills, Dec „09

Brazil-Ghana Technical Cooperation in SCD Asantehene Fetes 2nd Brazilian Delegation, Dec „09

Brazil-Ghana Technical Cooperation in SCD Asantehene Fetes 2nd Brazilian Delegation, Dec „09

Brazil-Ghana Blood and Sickle Cell Center

Computer Model

Brazil-Ghana Blood and Sickle Cell Center

Computer Model

Brazil-Ghana Technical Cooperation in SCD

Brazil-Ghana Blood and Sickle Cell Center

Survey of Plot at KATH

Brazil-Ghana Technical Cooperation in SCD

Kumasi Blood and Sickle Cell Center

Groundbreaking, Nov 25, 2010