December 8, 1971
Page 45341
Mr. MUSKIE. Mr. President, today the Senate will consider S. 2676, the National Sickle Cell Anemia Act which I have cosponsored.
The prevalence of sickle cell anemia in the United States is great. One in every 500 black children has this disease. A total of almost 50,000 children are born in this country every year with this disease. The effect of this disease is catastrophic. Those with the disease will suffer week-long episodes of painful sickle cell crisis on the average of four times per year. Fifty percent of those affected will die before their 20th year, and virtually all will die before their 40th year.
But what is sickle cell anemia? What can we do to alleviate its effect on our children?
Sickle cell anemia is a genetic disease. An individual has two genes which determine the structure of his hemoglobin. If an individual has one gene for hemoglobin S and one normal gene, he is said to have the sickle cell trait and will be normal except for occasional periods of
discomfort. However, if an individual has two genes for hemoglobin S, he will have sickle cell anemia. An individual with both genes for hemoglobin S will occur one time in four if both parents have the sickle cell trait. If an individual has sickle cell anemia, that is, all his hemoglobin is hemoglobin S, his red blood cells will, in times of stress, lose their normal spherical shape and assume a sickle-like form. These abnormally shaped cells will, in turn, mass together, clogging the small blood vessels, inhibiting the flow of normal oxygen-carrying red cells. Thus, the tissues become ischemic – a painful condition caused by a shortage of the oxygen necessary to support the cells' metabolism – and die.
Sickle cell anemia occurs almost exclusively among blacks and other people originating in tropical climates. This is true because the sickle cell trait – the presence of only one hemoglobin S gene – provides protection against malaria. Thus, in a malaria epidemic, those with the sickle cell trait often lived to produce children, while those without the sickle cell trait often died. In turn, we have the survival of the fittest – those with the trait were more fit.
But in the United States today, malaria has been eradicated, so the sickle cell trait serves no useful purpose, while the full anemia causes terrible disability and death.
I would like to commend Senator TUNNEY for introducing and providing the leadership for the desperately needed Sickle Cell Anemia Act. This bill has four central provisions: First, a coordinated Federal grant program of $100 million over 3 years for research, voluntary screening and counseling, and public education, second, a demonstration grant program of $30million over a 3-year period for the development of centers for research and research training in sickle cell anemia; third, a requirement that the Department of Defense implement a policy to provide voluntary screening, counseling, treatment, and education concerning the disease for servicemen, civilian employees, and inductees; and fourth, similar programs by other Federal agencies which provide direct health care for persons eligible in such agencies.
The purpose of this bill is to provide a coordinated and overall effort against sickle cell disease. In addition to research, it provides funds for the creation of community screening and counseling programs. Another provision provides funds for programs to educate the public about the nature of sickle cell anemia and sickle cell trait. This is particularly important in achieving maximization of the counseling and screening services to be offered.
Also the bill provides demonstration funds for the development of centers for research, screening, counseling, or treatment. Funds would be available to public and nonprofit private institutions such as medical schools, hospitals, and foundations.
The bill enables the Armed Forces and other Federal agencies to offer screening, counseling, and treatment. This voluntary program is especially important because it is here that large numbers of
young people can be reached and treated. Since there are conditions under which persons with the trait can be adversely affected, screening would enable these persons to be identified, enabling them to receive treatment and consideration for certain duties which would be more amenable to this health problem.
Finally, other available programs which now exist in the Public Health Service are authorized to screen and counsel for sickle cell anemia. Hopefully, this will permit the many other persons who are not otherwise directly covered to obtain a determination of possession of the trait and such treatment or counseling as is desirable.
Sickle cell anemia annually affects thousands of black children. The tragic consequences of this disease have been neglected too long. I am pleased, therefore, to join Senator TUNNEY and others in support of the Sickle Cell Act.
Mr. BROOKE. Mr. President, I am pleased to have the opportunity to urge my colleagues to support S. 2676, the National Sickle Cell Anemia Act.
As a cosponsor of this measure, I am heartened by the prompt consideration which it received in the Senate Health Subcommittee and full Labor and Public Welfare Committee. I commend the subcommittee and the full committee for the excellent refinements made to this important measure.
I was privileged to have the opportunity to testify before the subcommittee in favor of the measure, and I feel it appropriate to, now repeat part of my urgings:
Sickle Cell Anemia has, for too long, been the forgotten disease. Most Americans do not know that in this country today over two million people – 10 percent of the black population – are carriers of a disease called Sickle Cell Anemia.
Each year over 1,500 new cases are discovered, and more than 5,000 victims require hospitalization.
The facts are grim. The prognosis is uncertain. But today we have a new commitment and I am encouraged that with our concern, energies, and resources we can begin to relieve the suffering of so many. Simply put, Mr. President, too many have suffered too long. Let us get on with the task that has been too long ignored.