10 Years of Testing, Tracking, and Follow-up
The purpose of diagnosing sickle cell disease (SCD) in newborns is to allow health workers to educate parents about the special needs of the children and also to begin preventive treatment before the children begin the develop complications of the disease. Many of those complications do affect and kill young children before doctors even suspect that they have the disease. Screening programs for SCD, initiated in the United States and Jamaica, have led to sharply reduced mortality and morbidity of the disease in young children.
The research project entitled Newborn Screening for Sickle Cell Disease in Ghana launched in April 1993 began actual testing of newborn babies on February 13th, 1995, 10 years ago. It is the first and only large-scale newborn screening programme for SCD in Africa. The project is funded largely from a grant awarded to Prof. by the National Heart, Lung, and Blood Institute of the National Institutes of Health of the United States as part of the Comprehensive Sickle Cell Center at the Children's Hospital of Philadelphia. Prof. Ohene-Frempong, the Director of the Center, serves also as the Principal Investigator of the project in Ghana. The Ministry of Health and the Ghana Health Service have provided invaluable assistance to the project through organizational, infrastructural, and staff support.
Participating institutions in Ghana include Noguchi Memorial Institute for Medical Research, School of Medical Sciences, Kwame Nkrumah University of Science and Technology (SMS-KNUST), Komfo Anokye Teaching Hospital (KATH), and the Ministry of Health. Prof. Francis K. Nkrumah, former Director of Noguchi, served as Co-Investigator and leader of the project in Ghana for the first 10 years. Prof. Tsiri Agbenyega, Dean of the SMS-KNUST is the local leader of the third phase of this international collaborative project. In addition, the Newborn Screening Advisory Committee created by the investigators has provided overall supervision of the project. The Advisory Committee is chaired by the Chief Executive of KATH and includes officials holding the following positions: Chairman, Board of Trustees, KATH; Director of Health Services, Ashanti Region; Dean, School of Medical Sciences, Kwame Nkrumah University of Science and Technology (SMS-KNUST); Director of Nursing Services KATH; Metropolitan Director of Health Services, Kumasi; and, President, Sickle Cell Disease Association of Ghana (Ashanti Branch).
The operations of the project are effected through five components. The Education and Counseling Component is responsible for public, patient, and health worker education regarding the project and SCD in general. It is currently led by Robert Twene, Manager, National Health Learning Materials Centre (Kumasi Health Education Unit). Its staff includes Andrew Druye, Stella Appiah, and Justice Acheampong. The Screening Component performs the actual drawing of blood samples from newborns, reporting of results to families, and the tracking of babies found to have SCD for referral to the sickle cell clinic. The core staff consists of Hannah Hagan Tetteh and Rose Appiah, the Screening Nurse Coordinators; Owusua Serwaa (Nurse Midwife, Tikrom); Fred Adjei Mensah and Regina Osei the secretarial and data entry staff; plus, several Public and Community Health Nurses and support staff of the Metropolitan Office of Health. The large Screening Component is coordinated by Dr. Joseph K. Oduro who recently succeeded Dr. Agatha Bonney as Kumasi Metropolitan Director of Health Services. The Laboratory Component, based at Noguchi Institute, performs the screening tests to detect SCD and other abnormal hemoglobins on the newborn blood samples. The laboratory is staffed by Ben Asiedu, John Gbenatey, and Tina Ayeh, and supervised by Prof. Nkrumah. The Clinical Component staffs the Sickle Cell Clinic at KATH and provides comprehensive outpatient and inpatient health care services for patients found to have SCD. Led by Dr. Osei Yaw Akoto, the Clinical Component includes Drs. Daniel Ansong, and Emmanuel Asafo-Adjei; nurses Delaena Ocloo and Dora Ntori; and, the data entry clerks Mary Asafo-Adjei and Christie Adade. A special laboratory has been set up at KATH to support the Sickle Cell Clinic and the research programme. This Clinical Laboratory Service is coordinated by Clement Opoku-Okrah and includes two laboratory technologists, Peter K. Appiah and Lawrence Osei-Owusu, and a data entry clerk, Victoria Apemah.
In addition to the present staff, several others in past positions and roles on the project worked to make this screening programme a success. They include: Dr. Joseph Adibo, as Director of Health Services, Dr. George Amofah as Ashanti Regional Director of Health Services, Dr. Mercy Essel-Ahun as Kumasi Metropolitan Director of Health Services and coordinator of the Screening Component of the project, Dr. Benjamin Baffoe-Bonnie as Head, Child Health Department, SMS-KNUST, and coordinator of the Clinical Component of the project, and Mrs. Jemima Dennis-Antwi as Head, National Health Learning Materials Centre and coordinator of the Educational and Counseling Component of the project.
Initially the screening of newborns for SCD was conducted at eight public health facilities in Kumasi including KATH nurseries and KATH Polyclinic, KNUST Hospital, Maternal and Child Health Hospital, Manhyia Hospital, Kumasi South Hospital, Suntreso Hospital, and Tafo Hospital; and, at Tikrom Maternity Home, approximately 15 miles south of Kumasi, a private establishment that served as the rural model of the project. Since 2002, the screening has been extended to a new public health site at Tikrom Government Clinic, and 10 private sites namely: 4MRS, Siaw Larbi Hospital, West–End Hospital, Keffam Hospital, Comfort Maternity Home, Maranatha Maternity Home, Aniwaa Medical Centre, Bomso Clinic, Philippo Maternity Home, and St. Michael's Hospital at Pramso. Babies are screened either at birth or at their initial “weighing-in” or immunization visit to these health facilities. Screening has been open to all babies and free of charge, as part of the research project.
As of, December 31, 2004, a total of 177,283 babies had been screened for SCD in Kumasi and Tikrom; 3,346, or approximately 2% of them had been found to have SCD. In addition, 13.3% of the newborns were found to have sickle cell trait (AS) and 8.5% with hemoglobin C trait (AC), both clinically benign conditions. The most common type of SCD found is sickle cell disease-SS (SCD-SS) followed by SCD-SC. As predicted, the most challenging aspect of the screening programme is finding the families of babies with SCD in order to give them their results and refer them to the clinic. Although families are told to return for their results, only about 20% do so. The Screening Nurse Coordinators and their Public and Community Health colleagues must track the rest of the babies. The tracking depends on information on where the parents live, work or worship, provided at the time of delivery of the babies by their mothers. Babies not found after six months of tracking are declared “lost to follow-up”. Of the 3,346 newborn babies with SCD, 455 (13.6%) had been “lost to follow-up”, 116 were still under tracking, and 39 had died by the time their families were located. The families of the remaining 2,736 (82%) were successfully contacted with results and invited to enroll the baby in the Sickle Cell Clinic at KATH; 2,366 (86.5% of the 2,736) enrolled in the Clinic.
In addition to its primary mission, the project has worked to improve knowledge about SCD in Ghana and Africa. It has produced several educational pamphlets, posters, and radio programs. Annually, it has sent doctors and nurses from the project to present their results at international conferences, mostly in the U.S. It has also organized annual Educational Seminars in Kumasi as well as two International African Symposia on Sickle Cell Disease, the two largest conferences ever held in Africa on SCD, both held at the Accra International Conference Centre (July 1995 and July 2000). The third in this series of international conferences, the 5th International African Symposium on Sickle Cell Disease, will be held July 19th-22nd, 2005 at the Accra International Conference Centre.
By March 2003, the initial aims of the project had been fulfilled. A successful screening program had been designed and implemented within the Ghana Health Service. However, the project has also encountered some significant problems. The Sickle Cell Clinic at KATH opened in December 1992 with 10 patients and now has registered more than 9,700 patients including the newborns. It has had the same number of part-time doctors (four) who have other duties in addition to caring for such a large number of seriously ill patients. The Clinic shares space and rotates its schedule with other clinics at KATH and is therefore able to see patients only on two half-days, Monday and Thursday afternoons. Clinic hours routinely run past 6 pm. Many children are not brought to the clinic regularly as scheduled. The problem of long delays in clinic is the most common reason cited by parents as to why they have defaulted on clinic visits. The clinic needs larger space and more staff and time to function adequately.
The failure to find a significant number of babies through our tracking effort demands intensified parental education. Many of the babies not found through tracking had been taken from the city to rural hometowns by the time their homes were located. Efficiency of tracking would be improved greatly if the tracking nurses had vehicles. Repeated appeals to the Regional Directorate of the Ghana Health Service for vehicles have yielded no positive response so far. The need for new clinic space has also been addressed with the Ministry of Health with similar results. Recognizing the fact that government cannot do everything, the Ashanti Branch of the Sickle Cell Association of Ghana, together with the project staff, approached Otumfuo Osei Tutu II, Asantehene, and appealed for land to build a Centre for Sickle Cell Disease in Kumasi. Otumfuo graciously donated land on the grounds of KATH and agreed to have the Centre honored with his name. The Sickle Cell Foundation of Ghana (SCFG), a registered non-governmental organization, has been formed to support the development of resources and services to improve the health and quality of life of people with sickle cell disease and related conditions in Ghana. The SCFG has joined the SCAG to lead the efforts to raise funds to support the building of the Centre.
Initially the newborn screening project was funded for two 5-year cycles from 1993 to 2003. Currently the screening program continues under a new research study that requires continued testing for the diagnosis of newborns with SCD. The new 5-year research project awarded to Prof. Ohene-Frempong and his collaborators will concentrate on investigating the causes of serious infection in young children with SCD in Ghana. Additional collaborators include Prof. Tsiri Agbenyega, Dean of SMS-KNUST and Dr. Enoch Frimpong, Professor of Medical Microbiology, KNUST and, Director of the Bacteriology Laboratory at KATH. Isaac Boakye has been added as the Project Coordinator.
With successful completion of its initial aims, the newborn screening project made a proposal to the Ghana Health Service for expansion of the newborn screening programme as part of the public health service. A serious disease that continuously affects 2% of the newborn population of any country would be considered a major public health problem. The same rate of the disease exists throughout tropical Africa. It is estimated that more than 400,000 babies are born each year with SCD in Africa. In Ghana, the annual SCD births are approximately 15,000. In the 10 years of screening at Kumasi and Tikrom, more than 150,000 babies with SCD have been born in Ghana. Only 2,366 were properly diagnosed early and enrolled in a clinic for specialized care. Most of the other babies were not properly diagnosed and certainly, many died before anyone suspected that they had SCD. That is not an acceptable situation.
SCD affects people of Africa, the Mediterranean countries, the Middle East, the Americas, the Caribbean, and India. However, Africa bears the greatest share of the burden. In the U.S. where much of SCD research is conducted, only about 1,200 babies with SCD are born each year. The World Health Organization has virtually no programme in SCD. The Government of Ghana needs to commit itself to expand the newborn screening programme throughout the country. The pioneering research project in Kumasi has developed the technical expertise to assist in the expansion effort. However, the limited resources of the project cannot sustain and expand the screening programme throughout Ghana. Newborn screening for SCD has proven to be a major life-saving and health promotional activity in Kumasi as it has in the U.S., Jamaica, and elsewhere. It must be incorporated into the Ghana Health Service. SCD treatment centres need to be established at each of the regional and other referral hospitals and screening programmes should be organized around these centres.
One of the primary objectives of the Sickle Cell Foundation of Ghana is to raise awareness of the need for SCD services and to lead the advocacy for the establishment of such services within both the public and private health institutions in Ghana. In addition, the Foundation is planning to open Sickle Cell Screening and Counseling Centres where individuals concerned about their sickle cell status for purposes of reproductive and other planning can receive modern health education, testing, and genetic counseling.
Kwaku Ohene-Frempong, M.D. Professor of Pediatrics, University of Pennsylvania Director, Sickle Cell Center, The Children's Hospital of Philadelphia Philadelphia, USA.