Reflections on the Current Status of the National Sickle Cell Disease Program in the United States Roland B. Scott, MD Washington, DC
Some clouds of concern now appear on the horizon for the national sickle cell disease program. There is flagging general attention by the black population and a dilution of interest in and visibility of the sickle cell problem brought about by political maneuvering to bring the program under the legislative umbrella of many other genetic diseases (which occur predominantly in Caucasians). In addition, the federal program has recently phased-out six comprehensive sickle cell centers and imposed budgetary cutbacks in the remaining centers. The victims of this disease, the black population in general, and the researchers and investigators who seek ways to bring this disease under control need reassurance from the current national administration that the sickle cell program will not be permitted to die a slow death from financial attrition, attenuation of interest, and skillful neglect leading to the phasing-out of another "minority project." The national sickle cell program, in the relatively short span of six years, has made significant and notable progress not only in research endeavor but also in improved patient care and community-wide education. In this context, certainly, the positive aspects of the national sickle cell disease program continue to far outweigh any negative ones.'
The first slave ships bearing black Africans arrived in the United States in 1619, however, the first recorded case of sickle cell anemia was not described until 1910 by Herrick.2 Following Herrick's report, the disease was widely described in many clinical, pathological, and hematological medical papers. However, the first major scientific breakthrough did not occur until 1949, when Pauling discovered that the sickling phenomenon was dependent upon the presence of an abnormal hemoglobin which had reproducible electrophoretic properties.3 Another
Dr. Scott is a Distinguished Professor of Pediatrics and Child Health, and Director of the Center for Sickle Cell Disease, Howard University College of Medicine, Washington, DC. Requests for reprints should be addressed to the Howard University Center for Sickle Cell Disease, 2121 Georgia Avenue, NW, Washington, DC 20059.
notable advance occurred in the same year when Neel4 and Beet5 independently reported that the disease is transmitted genetically by heterozygouxs carriers as a Mendelian recessive. Despite these and other stimulating biomedical contributions, the disease suffered from relative neglect by the scientific community and was regarded as an unimportant condition occurring predominantly in Black people.
A President Disease
Recognizes the
The low profile of scientific and biomedical interest continued until February 1971, when President Nixon, in his message to Congress, indicated that greater attention and support for sickle cell disease should be made available at the national level. Shortly
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 7, 1979
thereafter, in May 1972, Congress passed the National Sickle Cell Anemia Control Act with the resultant formation of a Sickle Cell Disease Branch in the Heart, Lung, and Blood Institute of the National Institutes of Health (NIH). Under the auspices and funding of the Sickle Cell Disease Branch, ten comprehensive centers* were established in existing medical schools with a mandate to develop programs of research, education, improved patient care, screening, counseling, and community involvement. In addition to mission-oriented research, the federal government supported other projects through the media of contracts, investigator-initiated studies, the development of screening and education clinics, and the inauguration of a hemoglobinopathy detection laboratory in the Center for Disease Control in Atlanta. This federal program stimulated basic and clinical investigation in abnormal hemoglobins and generated more than 200 papers that were presented at the first National Symposium on Sickle Cel Disease held in Washington, DC, June 1974, under the auspices of NIH. The newsmedia responded to this biomedical reawakening of interest in sickle cell disease by an outpouring of reports. At long last, interest in the disease had indeed reached a stage of high visibility!
Research Accomplishments Potassium cyanatef6 and urea7 were heralded in early and preliminary reports as potential antisickling agents that could prevent or control painful sickle cell crises, however, subsequent studies demonstrated the occurrence of
*The number of centers was subsequently increased to 15 but later reduced to ten. 679
toxicity and other untoward effects that essentially eliminated these agents from further consideration.8 The search continues for safer and less toxic compounds for use in the treatment of this disease. It is possible that some of the agents such as cyanate and alkylating compounds (nitrogen mustard), which are too toxic for direct administration to the human, may be employed through the use of an extracorporeal approach. This involves the in vitro mixing of red blood cells from patients who have sickle cell disease with an agent like cyanate and then reintroducing the washed, chemically treated cells to the same donor. This technique may avoid or bypass the toxicity effects but it has the disadvantage of being cumbersome, time consuming, and expensive.9 It is currently available as an experimental procedure in only a few medical centers. New methods are being devised for the detection of sickle and other abnormal hemoglobins. It is now possible to diagnose sickle cell disease in the fetus"s'3 and in the newborn infant. 14 The prenatal diagnostic procedure gives parents at risk an option to have offspring who have normal hemoglobins or the trait, but to avoid the birth of sick children with sickle cell disease. In addition to electrophoresis, other methods such as microcolumn chromatography and immunochemical techniques15 have been developed to diagnose sickle cell disease in the newborn infant. Detection of the disease in early life, before clinical symptoms appear, permits early education and medical surveillance which are helpful in the control of life threatening infection-a chief cause of death in infants and young children.lf Early diagnosis is particularly important in order to identify those children who can benefit from the new vaccine for prophylaxis against pneumococcal in-
fections.17 An animal model has been developed which makes it possible to evaluate the effectiveness of therapeutic agents such as anti-sickling chemical compounds without significant risk to the human subject."8 This involves pretreatment of an animal such as a rat with biologicals so that the rodent is able to accept and tolerate for a limited time transfused sickled cells from human subjects. Then, the living human sickle cells in the rat's circula680
tory system can be "treated" with various anti-sickling agents as a test of their therapeutic efficacy. Newer and different methods of genetic counseling for persons of varied educational and environmental backgrounds are being explored and evaluated in the Medical Genetics Division at Howard University and elsewhere in an effort to improve the effectiveness of this procedure (Murray, R.F., Personal communication, January 1, 1979). A project involving a cooperative study of the natural history or clinical course of patients of all ages with sickle cell disease is now in progress at Howard University and 23 other medical institutions, under the auspices of the Sickle Cell Disease Branch at the National Institutes of HIealth. This project should prove to be beneficial to researchers and patients. The study results will be helpful in making long-range plans for treatment and management. In addition, it will provide information that can serve as a basis for the evaluation of present and future therapeutic regimens.
Education In addition to research accomplishments, there has been the development of an ongoing program for education directed toward both professional and lay groups. The National Institutes of Health and the federally sponsored Sickle Cell Centers and Clinics have all contributed to this program.'9 As an example, the Center for Sickle Cell Disease of Howard University has presented seven postgraduate conferences dealing with various aspects of this disease. More than 2,000 physicians and allied health workers have attended these meetings.20 Moreover, the federally sponsored Sickle Cell Centers and Clinics have produced other educational media such as motion pictures, tapes, exhibits, booklets, pamphlets, and scientific publications.
Patient Care In addition to participation in research and education, a significant responsibility of the NIH-affiliated sickle cell disease centers is to provide and coordinate comprehensive care for victims of this malady. This includes medical care and supportive services such as social work, psychological
evaluation, rehabilitation, and vocational counseling. The Center at Howard University may be used as a prototype. This Center currently provides care for 200 children and 100 adult patients with sickle cell disease and integrates hospital and ambulatory services as well as facilities for accurate screening and counseling for persons with abnormal hemoglobins. A consultative laboratory diagnostic service is also available to physicians in the community. A screening laboratory is located in the Center building and is available to residents of the greater Washington, DC area. The Howard University Center maintains an active Community Outreach Program which is designed to provide current information and professional services to citizens of the metropolitan area of the District of Columbia and adjacent regions of Maryland and Virginia. The program is implemented through the utilization of a mobile health unit, which was specifically designed and customized to provide, under one roof, compartments for laboratory testing for sickle hemoglobin, iron deficiency (nutritional) anemia, blood pressure determination, audiovisual education, counseling, and referral services. The staff of this unit consists of two laboratory technicians, a counselor, a driver/security officer, and a community aide. This group works under the direct supervision of a community coordinator. To date, the mobile health unit has traveled over 5,000 miles and has given service to over 10,000 persons. This unit was donated to Howard University by the United Black Fund of Washington, DC.
Concerns and Unmet Needs Funding The total federal budget for the Comprehensive Sickle Cell Program was only $6.9 million for Fiscal Year 1973 and has not exceeded $20 million in subsequent years. In 1973, the ten existing centers were increased to 15; however, in 1977, six centers were phased out. Subsequently an additional facility was funded thus leaving ten viable centers at present. Two of the discontinued centers were in the state of New York, which has a large black and Hispanic population. The money problem became more acute in 1977, when the budgets of the ten remaining centers
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 7, 1979
were suddenly reduced across the board by ten to 14 percent of the total awards. Some, if not all, of the centers are finding it necessary to seek supplementary financial support from other sources. For example, the Howard University Center is almost totally dependent upon grants, donations, and community contributions for its existence and since it is situated in the District of Columbia, there is no source of state supplementation.
Legislation The initial National Sickle Cell Anemia Control Act (Public Law 92924) was signed by the President in May 1972 and expired June '30, 1975. This Act authorized designated funds to be appropriated for the three year duration of the Act, however, the appropriations were never actually implemented, thereby leaving the funding level of the national sickle cell program to the discretion of the HEW and NIH administrations. The consequences of weak legislation threaten the security of a "minority program" such as that for sickle cell which has to compete for funds with other programs in the Heart, Lung, and Blood Institute. With the expiration of the initial National Sickle Cell Anemia Control Act in 1975, Senator Javits of New York proposed the incorporation of the sickle cell program under the umbrella of an omnibus genetics bill. During the hearing for the new legislation, many of the black researchers and center directors testified in favor of a bill that would maintain the integrity of the program under a specific sickle cell control act. This fight was lost and after the hearings and imposed compromise, the sickle cell program became incorporated with other genetic diseases in a new bill (Public Law 94-278), which included Title IV cited as the National Sickle Cell Anemia, Cooley's Anemia, Tay-Sachs, and Genetic Diseases Act.*
*This title established a national program to provide for basic and applied research, training, testing, counseling, information and education programs with respect to genetic diseases including sickle cell anemia, Cooley's anemia, Tay Sachs disease, cystic fibrosis, dysautonomia, hemophilia, retinitis pigmentosa, Huntington's chorea, and muscular dystrophy.
This bill expired June 30, 1978 and further legislation is pending.
Research There is need for continuous support for research. There is still no cure for this painful, crippling disease which, in the United States, affects predominantly black persons. Research efforts carried out in the USA could also eventually benefit millions of victims of this disease who live in Africa and elsewhere.
drug trials, and better financial support. The effectiveness of the program of this Association is contingent upon the availability of financial support which is solicited from all available sources.
Literature Cited 1. Jackson RE: A perspective of the national sickle cell disease program. Arch Intern Med 133:533-534, 1974 2. Herrick JB: Peculiar elongated and sickle shaped red blood corpuscles in a case of severe anemia. Arch Intern Med 6:517-521,
1910
Patient Care While we await the coming of a "magic bullet" which hopefully will bring a cure, there is need to seek better ways to utilize existing facilities for the comprehensive care of these patients. There is need for more effective analgesics for the control of pain without the hazards of iatrogenic drug addiction. There is need in many areas of the United States for a more effective team approach composed of physicians and other health professionals such as nurses, health educators, clinical psychologists, etc, in order to give greater support to these patients whose quality of life is jeopardized not only by a chronic illness, but also by large medical bills, incomplete insurance coverage, interruption of school attendance by children, and lack of full employment opportunities for adults.
International Consideration This disease has a worldwide distribution and its victims, no doubt, number in the millions. Throughout the world many people who have this disease receive inadequate health care and die prematurely. An International Association for Sickle Cell Disease is now home-based in the Howard University Center. The main goal of this organization is to diminish morbidity and mortality and to improve the well being of the patients wherever they may be found. Achievement of this goal will depend in large measure upon improved communications, education, research, adoption of uniform procedures for treatment, laboratory technique,
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 7, 1979
3. Pauling L, Itano HA, Singer SJ, et al: Sickle cell anemia, molecular disease. Science 110:543-548, 1949 4. Neel JV: The inheritance of sickle cell anemia. Science 110:64-66, 1949 5. Beet EA: The genetics of the sickle cell trait in a Bantu tribe. Ann Eugen 14:279-284, 1949 6. Cerami A, Manning JM: Potassium cyanate as an inhibitor of the sickling of erythrocytes in vitro. Proc Natl Acad Sci 68:11801183, 1971 7. Nalbandian RM, Shulz G, Lusher JM, et al: Sickle cell crisis terminated by intravenous
urea in sugar solutions-a preliminary report. J Med Sci 261:309-324, 1971 8. Brewer GJ: A view of the current status of antisickling therapy. Am J Hematol 1:121, 1926 9. International Meeting on the Development of Therapeutic Agents for Sickle Cell Disease sponsored by L'lnstit National De La Sante Et De La Recherche Medicale (France), The Fogarty International Center, The National Heart, Lung and Blood Institute, and the National Institute of Arthritis, Metabolism and Digestive Diseases of the National Institutes of Health, USPHS, Paris, July 19-21, 1978 10. Nathan DG, Alter BP: Antenatal diagnosis of the haemoglobinopathies. Br J Haematol 31:143-146, 1975 11. Kan YW, Golbus MS, Trecartin R: Prenatal diagnosis of sickle cell anemia. N Engl J Med 294:1039-1040, 1976 12. Symposium on Perinatal Approaches to the Diagnosis of Fetal Hemoglobinopathies, Sponsored by Sickle Cell Disease Branch of National Heart, Lung, and Blood Institute and Martin Luther King Jr. General Hospital and Charles R. Drew Postgraduate Medical School, Los Angeles, February 23-24, 1978 13. Kan YW, Dozy AM: Antenatal diagnosis of sickle-cell anemia by DNA analysis of amniotic fluid cells. Lancet 2:910-912, 1978 14. Pearson HA, O'Brien PT, McIntosh S, et al: Routine screening of umbilical cord blood for sickle cell disease. JAMA 227:420-421, 1974 15. Headings V, Anyaibe S, Bhattacharya S, et al: Early diagnosis of hemoglobinopathies. Pediat Res 12:932-938, 1978 16. Barrett-Connor E: Bacterial infection and sickle cell anemia. Medicine 50:97-112, 1971 17. Ammann AJ, Addiego J, Wara D, et al: Polyvalent pneumococcal-polysaccharide immunization of patients with sickle cell anemia and patients with splenectomy. N Engl J Med 297:897-900, 1977 18. Castro 0, Osbaldiston GW, Aponte L, et al: Oxygen dependent circulation of sickle erythrocytes. J Lab Clin Med 88:732-744, 1976 19. Proceedings of the First National Sickle Cell Educational Symposium sponsored by Sickle Cell Branch, Heart, Lung, and Blood Institute, NIH, Washington, DC, June 1974 20. Scott RB: How a comprehensive center for sickle cell disease can contribute to continued medical education. J Natl Med Assn 69:67-68, 1977 21. Howard University Center for Sickle Cell Disease, Washington, DC, Bull Int Assoc Sickle Cell Dis 1: 14, 1 977
681
Some clouds of concern now appear on the horizon for the national sickle cell disease program. There is flagging general attention by the black population and a dilution of interest in and visibility of the sickle cell problem brought about by political maneuvering to bring the program under the legislative umbrella of many other genetic diseases (which occur predominantly in Caucasians). In addition, the federal program has recently phased-out six comprehensive sickle cell centers and imposed budgetary cutbacks in the remaining centers. The victims of this disease, the black population in general, and the researchers and investigators who seek ways to bring this disease under control need reassurance from the current national administration that the sickle cell program will not be permitted to die a slow death from financial attrition, attenuation of interest, and skillful neglect leading to the phasing-out of another "minority project." The national sickle cell program, in the relatively short span of six years, has made significant and notable progress not only in research endeavor but also in improved patient care and community-wide education. In this context, certainly, the positive aspects of the national sickle cell disease program continue to far outweigh any negative ones.'
The first slave ships bearing black Africans arrived in the United States in 1619, however, the first recorded case of sickle cell anemia was not described until 1910 by Herrick.2 Following Herrick's report, the disease was widely described in many clinical, pathological, and hematological medical papers. However, the first major scientific breakthrough did not occur until 1949, when Pauling discovered that the sickling phenomenon was dependent upon the presence of an abnormal hemoglobin which had reproducible electrophoretic properties.3 Another
Dr. Scott is a Distinguished Professor of Pediatrics and Child Health, and Director of the Center for Sickle Cell Disease, Howard University College of Medicine, Washington, DC. Requests for reprints should be addressed to the Howard University Center for Sickle Cell Disease, 2121 Georgia Avenue, NW, Washington, DC 20059.
notable advance occurred in the same year when Neel4 and Beet5 independently reported that the disease is transmitted genetically by heterozygouxs carriers as a Mendelian recessive. Despite these and other stimulating biomedical contributions, the disease suffered from relative neglect by the scientific community and was regarded as an unimportant condition occurring predominantly in Black people.
A President Disease
Recognizes the
The low profile of scientific and biomedical interest continued until February 1971, when President Nixon, in his message to Congress, indicated that greater attention and support for sickle cell disease should be made available at the national level. Shortly
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 7, 1979
thereafter, in May 1972, Congress passed the National Sickle Cell Anemia Control Act with the resultant formation of a Sickle Cell Disease Branch in the Heart, Lung, and Blood Institute of the National Institutes of Health (NIH). Under the auspices and funding of the Sickle Cell Disease Branch, ten comprehensive centers* were established in existing medical schools with a mandate to develop programs of research, education, improved patient care, screening, counseling, and community involvement. In addition to mission-oriented research, the federal government supported other projects through the media of contracts, investigator-initiated studies, the development of screening and education clinics, and the inauguration of a hemoglobinopathy detection laboratory in the Center for Disease Control in Atlanta. This federal program stimulated basic and clinical investigation in abnormal hemoglobins and generated more than 200 papers that were presented at the first National Symposium on Sickle Cel Disease held in Washington, DC, June 1974, under the auspices of NIH. The newsmedia responded to this biomedical reawakening of interest in sickle cell disease by an outpouring of reports. At long last, interest in the disease had indeed reached a stage of high visibility!
Research Accomplishments Potassium cyanatef6 and urea7 were heralded in early and preliminary reports as potential antisickling agents that could prevent or control painful sickle cell crises, however, subsequent studies demonstrated the occurrence of
*The number of centers was subsequently increased to 15 but later reduced to ten. 679
toxicity and other untoward effects that essentially eliminated these agents from further consideration.8 The search continues for safer and less toxic compounds for use in the treatment of this disease. It is possible that some of the agents such as cyanate and alkylating compounds (nitrogen mustard), which are too toxic for direct administration to the human, may be employed through the use of an extracorporeal approach. This involves the in vitro mixing of red blood cells from patients who have sickle cell disease with an agent like cyanate and then reintroducing the washed, chemically treated cells to the same donor. This technique may avoid or bypass the toxicity effects but it has the disadvantage of being cumbersome, time consuming, and expensive.9 It is currently available as an experimental procedure in only a few medical centers. New methods are being devised for the detection of sickle and other abnormal hemoglobins. It is now possible to diagnose sickle cell disease in the fetus"s'3 and in the newborn infant. 14 The prenatal diagnostic procedure gives parents at risk an option to have offspring who have normal hemoglobins or the trait, but to avoid the birth of sick children with sickle cell disease. In addition to electrophoresis, other methods such as microcolumn chromatography and immunochemical techniques15 have been developed to diagnose sickle cell disease in the newborn infant. Detection of the disease in early life, before clinical symptoms appear, permits early education and medical surveillance which are helpful in the control of life threatening infection-a chief cause of death in infants and young children.lf Early diagnosis is particularly important in order to identify those children who can benefit from the new vaccine for prophylaxis against pneumococcal in-
fections.17 An animal model has been developed which makes it possible to evaluate the effectiveness of therapeutic agents such as anti-sickling chemical compounds without significant risk to the human subject."8 This involves pretreatment of an animal such as a rat with biologicals so that the rodent is able to accept and tolerate for a limited time transfused sickled cells from human subjects. Then, the living human sickle cells in the rat's circula680
tory system can be "treated" with various anti-sickling agents as a test of their therapeutic efficacy. Newer and different methods of genetic counseling for persons of varied educational and environmental backgrounds are being explored and evaluated in the Medical Genetics Division at Howard University and elsewhere in an effort to improve the effectiveness of this procedure (Murray, R.F., Personal communication, January 1, 1979). A project involving a cooperative study of the natural history or clinical course of patients of all ages with sickle cell disease is now in progress at Howard University and 23 other medical institutions, under the auspices of the Sickle Cell Disease Branch at the National Institutes of HIealth. This project should prove to be beneficial to researchers and patients. The study results will be helpful in making long-range plans for treatment and management. In addition, it will provide information that can serve as a basis for the evaluation of present and future therapeutic regimens.
Education In addition to research accomplishments, there has been the development of an ongoing program for education directed toward both professional and lay groups. The National Institutes of Health and the federally sponsored Sickle Cell Centers and Clinics have all contributed to this program.'9 As an example, the Center for Sickle Cell Disease of Howard University has presented seven postgraduate conferences dealing with various aspects of this disease. More than 2,000 physicians and allied health workers have attended these meetings.20 Moreover, the federally sponsored Sickle Cell Centers and Clinics have produced other educational media such as motion pictures, tapes, exhibits, booklets, pamphlets, and scientific publications.
Patient Care In addition to participation in research and education, a significant responsibility of the NIH-affiliated sickle cell disease centers is to provide and coordinate comprehensive care for victims of this malady. This includes medical care and supportive services such as social work, psychological
evaluation, rehabilitation, and vocational counseling. The Center at Howard University may be used as a prototype. This Center currently provides care for 200 children and 100 adult patients with sickle cell disease and integrates hospital and ambulatory services as well as facilities for accurate screening and counseling for persons with abnormal hemoglobins. A consultative laboratory diagnostic service is also available to physicians in the community. A screening laboratory is located in the Center building and is available to residents of the greater Washington, DC area. The Howard University Center maintains an active Community Outreach Program which is designed to provide current information and professional services to citizens of the metropolitan area of the District of Columbia and adjacent regions of Maryland and Virginia. The program is implemented through the utilization of a mobile health unit, which was specifically designed and customized to provide, under one roof, compartments for laboratory testing for sickle hemoglobin, iron deficiency (nutritional) anemia, blood pressure determination, audiovisual education, counseling, and referral services. The staff of this unit consists of two laboratory technicians, a counselor, a driver/security officer, and a community aide. This group works under the direct supervision of a community coordinator. To date, the mobile health unit has traveled over 5,000 miles and has given service to over 10,000 persons. This unit was donated to Howard University by the United Black Fund of Washington, DC.
Concerns and Unmet Needs Funding The total federal budget for the Comprehensive Sickle Cell Program was only $6.9 million for Fiscal Year 1973 and has not exceeded $20 million in subsequent years. In 1973, the ten existing centers were increased to 15; however, in 1977, six centers were phased out. Subsequently an additional facility was funded thus leaving ten viable centers at present. Two of the discontinued centers were in the state of New York, which has a large black and Hispanic population. The money problem became more acute in 1977, when the budgets of the ten remaining centers
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 7, 1979
were suddenly reduced across the board by ten to 14 percent of the total awards. Some, if not all, of the centers are finding it necessary to seek supplementary financial support from other sources. For example, the Howard University Center is almost totally dependent upon grants, donations, and community contributions for its existence and since it is situated in the District of Columbia, there is no source of state supplementation.
Legislation The initial National Sickle Cell Anemia Control Act (Public Law 92924) was signed by the President in May 1972 and expired June '30, 1975. This Act authorized designated funds to be appropriated for the three year duration of the Act, however, the appropriations were never actually implemented, thereby leaving the funding level of the national sickle cell program to the discretion of the HEW and NIH administrations. The consequences of weak legislation threaten the security of a "minority program" such as that for sickle cell which has to compete for funds with other programs in the Heart, Lung, and Blood Institute. With the expiration of the initial National Sickle Cell Anemia Control Act in 1975, Senator Javits of New York proposed the incorporation of the sickle cell program under the umbrella of an omnibus genetics bill. During the hearing for the new legislation, many of the black researchers and center directors testified in favor of a bill that would maintain the integrity of the program under a specific sickle cell control act. This fight was lost and after the hearings and imposed compromise, the sickle cell program became incorporated with other genetic diseases in a new bill (Public Law 94-278), which included Title IV cited as the National Sickle Cell Anemia, Cooley's Anemia, Tay-Sachs, and Genetic Diseases Act.*
*This title established a national program to provide for basic and applied research, training, testing, counseling, information and education programs with respect to genetic diseases including sickle cell anemia, Cooley's anemia, Tay Sachs disease, cystic fibrosis, dysautonomia, hemophilia, retinitis pigmentosa, Huntington's chorea, and muscular dystrophy.
This bill expired June 30, 1978 and further legislation is pending.
Research There is need for continuous support for research. There is still no cure for this painful, crippling disease which, in the United States, affects predominantly black persons. Research efforts carried out in the USA could also eventually benefit millions of victims of this disease who live in Africa and elsewhere.
drug trials, and better financial support. The effectiveness of the program of this Association is contingent upon the availability of financial support which is solicited from all available sources.
Literature Cited 1. Jackson RE: A perspective of the national sickle cell disease program. Arch Intern Med 133:533-534, 1974 2. Herrick JB: Peculiar elongated and sickle shaped red blood corpuscles in a case of severe anemia. Arch Intern Med 6:517-521,
1910
Patient Care While we await the coming of a "magic bullet" which hopefully will bring a cure, there is need to seek better ways to utilize existing facilities for the comprehensive care of these patients. There is need for more effective analgesics for the control of pain without the hazards of iatrogenic drug addiction. There is need in many areas of the United States for a more effective team approach composed of physicians and other health professionals such as nurses, health educators, clinical psychologists, etc, in order to give greater support to these patients whose quality of life is jeopardized not only by a chronic illness, but also by large medical bills, incomplete insurance coverage, interruption of school attendance by children, and lack of full employment opportunities for adults.
International Consideration This disease has a worldwide distribution and its victims, no doubt, number in the millions. Throughout the world many people who have this disease receive inadequate health care and die prematurely. An International Association for Sickle Cell Disease is now home-based in the Howard University Center. The main goal of this organization is to diminish morbidity and mortality and to improve the well being of the patients wherever they may be found. Achievement of this goal will depend in large measure upon improved communications, education, research, adoption of uniform procedures for treatment, laboratory technique,
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 7, 1979
3. Pauling L, Itano HA, Singer SJ, et al: Sickle cell anemia, molecular disease. Science 110:543-548, 1949 4. Neel JV: The inheritance of sickle cell anemia. Science 110:64-66, 1949 5. Beet EA: The genetics of the sickle cell trait in a Bantu tribe. Ann Eugen 14:279-284, 1949 6. Cerami A, Manning JM: Potassium cyanate as an inhibitor of the sickling of erythrocytes in vitro. Proc Natl Acad Sci 68:11801183, 1971 7. Nalbandian RM, Shulz G, Lusher JM, et al: Sickle cell crisis terminated by intravenous
urea in sugar solutions-a preliminary report. J Med Sci 261:309-324, 1971 8. Brewer GJ: A view of the current status of antisickling therapy. Am J Hematol 1:121, 1926 9. International Meeting on the Development of Therapeutic Agents for Sickle Cell Disease sponsored by L'lnstit National De La Sante Et De La Recherche Medicale (France), The Fogarty International Center, The National Heart, Lung and Blood Institute, and the National Institute of Arthritis, Metabolism and Digestive Diseases of the National Institutes of Health, USPHS, Paris, July 19-21, 1978 10. Nathan DG, Alter BP: Antenatal diagnosis of the haemoglobinopathies. Br J Haematol 31:143-146, 1975 11. Kan YW, Golbus MS, Trecartin R: Prenatal diagnosis of sickle cell anemia. N Engl J Med 294:1039-1040, 1976 12. Symposium on Perinatal Approaches to the Diagnosis of Fetal Hemoglobinopathies, Sponsored by Sickle Cell Disease Branch of National Heart, Lung, and Blood Institute and Martin Luther King Jr. General Hospital and Charles R. Drew Postgraduate Medical School, Los Angeles, February 23-24, 1978 13. Kan YW, Dozy AM: Antenatal diagnosis of sickle-cell anemia by DNA analysis of amniotic fluid cells. Lancet 2:910-912, 1978 14. Pearson HA, O'Brien PT, McIntosh S, et al: Routine screening of umbilical cord blood for sickle cell disease. JAMA 227:420-421, 1974 15. Headings V, Anyaibe S, Bhattacharya S, et al: Early diagnosis of hemoglobinopathies. Pediat Res 12:932-938, 1978 16. Barrett-Connor E: Bacterial infection and sickle cell anemia. Medicine 50:97-112, 1971 17. Ammann AJ, Addiego J, Wara D, et al: Polyvalent pneumococcal-polysaccharide immunization of patients with sickle cell anemia and patients with splenectomy. N Engl J Med 297:897-900, 1977 18. Castro 0, Osbaldiston GW, Aponte L, et al: Oxygen dependent circulation of sickle erythrocytes. J Lab Clin Med 88:732-744, 1976 19. Proceedings of the First National Sickle Cell Educational Symposium sponsored by Sickle Cell Branch, Heart, Lung, and Blood Institute, NIH, Washington, DC, June 1974 20. Scott RB: How a comprehensive center for sickle cell disease can contribute to continued medical education. J Natl Med Assn 69:67-68, 1977 21. Howard University Center for Sickle Cell Disease, Washington, DC, Bull Int Assoc Sickle Cell Dis 1: 14, 1 977
681
