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population. It has been shown that not all physicians are familiar with sickle cell disease either because Blacks constitute a minority of their patient population or because emphasis or knowledge of its existence was not widely known during their period of formal education. Training for all levels would be varied from long to short duration.

(3) Initiate and promote an educational program in the sickling diseases for lay persons, college students, medical students and other interested groups, through seminars, conferences, exhibits and printed material.

Past financial support for efforts at Howard University has come from both private and Federal sources. However, that support has been meager in comparison to support for other diseases.

We maintain that Federal and private support have been inadequate to date and vigorously request that additional emphasis be placed on this disease such as recommended in the Bills being discussed today (Bills S. 2676 and S. 2677). This is an encouraging beginning.

We would also respectfully remind the members of this Committee that there remains a great deal to be learned about this serious disease. We feel that Howard University can make a lasting and maximum contribution to the control of this disease through the continued operation and further development of the Center for Sickle Cell Anemia.

Financial support in proportion to the magnitude of this national public health problem must be made available to insure that these efforts can be made.

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With the means provided by S. 2676 and 2677, programs could be

established to effectively deal with Sickle Cell disease on a nationwide

basis.

We agree with and endorse the concept that Centers should be strategically situated in major geographic regions across the country. Such Centers will offer sophisticated diagnostic and treatment facilities to the victims of Sickle Cell Disease within their geographic region. They will also offer training and educational opportunities to personnel from satellite facilities within those regions.

It is proposed that the District of Columbia and Howard University serve as national models of the control and combat of Sickle Cell Disease.

For this we are prepared and eager to do our part. A logical and economical approach to setting into operation a Sickle Cell program is the utilization of existing facilities to the maximum extent possible. Neighborhood clinics, public health clinics, hospitals and private clinics should find little difficulty in including such services as sickle cell screening and limited genetic counseling in their present routines. The Center would be a supportive facility that would perform the services previously described. In summary we support the establishment of regional centers that would direct and lead a national program of prevention and control of sickle cell disease. We recommend that active programs being conducted by local facilities be employed as the vehicles through which sickle cell services are provided to the population at risk.

We unreservedly support Bills S. 2676 and S. 2677 and applaud their

sponsors. We feel that these Bills make a belated but important thrust in the right direction.

Senator KENNEDY. I think we will hear the full panel and then ask some questions. Dr. Rhodes.

STATEMENT OF ROBERT RHODES, M.D., FELLOW IN MEDICINE, VANDERBILT UNIVERSITY

Dr. RHODES. Senator Kennedy, my name is Dr. Robert S. Rhodes. I am an assistant professor of medicine in hematology at Meharry Medical College in Nashville, Tenn. I also hold an appointment on the medical faculty at Vanderbilt University and I am a Josiah Macy Foundation Faculty Fellow. I might mention that Meharry Medical College has graduated over 43 percent of the black physicians practicing in the United States. As a hematologist, I am intimately involved with sickle cell disease from both the research and clinical points of view.

As has been mentioned by others, sickle cell disease is a heritable disorder with red cells containing an abnormal hemoglobin called "Hemoglobin C." Hemoglobin S. when present, is either the symptomatic dreaded homozygous form, which is the usual form in which the disease is symptomatic, or it may be present in the heterogygous form, which is the sickle cell trait.

Hemoglobin S in the United States occurs, in about 8-11 percent of the blacks in the United States. Of those carrying it, about 2 percent have the anemic form of the disease. In other words, approximately one black child in every 400 born in the United States will have sickle cell anemia.

The sickle cell trait, the heterozygous form, can have many of the deadly manifestations of sickle cell anemia as we shall point out. In most instances, however, this is compatible with a normal lifespan and is discovered most often during incidental hemoglobin electrophoresis or family studies.

There are usually no deviations from the normal with respect to physical examination, and excent that an occasional patient's urine specific gravity will not exceed 1.010, the usual laboratory determinations are normal. Occasionally, a patient with sickle cell trait has a vasoocclusive phenomenon, or a hemolytic process when unique anatomic or functional conditions exist.

Recent observations in the clinic and at the postmortem table indicate that extensive in-vivo sickling can be fatal in sickle cell trait if extreme hypoxia or intravascular pooling with stasis takes place. Such conditions have been seen in marked respiratory depression (deep anesthesia, acute alcholoism) or in spinal anesthesia with dilitation of the splanchnic vasculature.

Senator KENNEDY. What does that mean?

Dr. RHODES. This means that the blood in individuals with sickle cell trait under conditions of, say, at high altitudes or during anesthesia may clog up the blood vessels. It will assume the twisted, bent formthe cells of their blood under low oxygen concentration in the airmay assume the bent or twisted character of the sickle cell and thereby clog their blood vessels and prevent blood from reaching certain vital areas of the body.

This has occurred in individuals on many occasions, and recently in the medical literature it was reported-and I do not have the reference in front of me--but several young recruits died in military training and were found as a result-I am sure you are familiar with it— who had sickle cell trait and had not been tested for it.

There are numerous other severe medical problems that can be associated under adverse circumstances with sickle cell trait. These I have outlined in my testimony and they involve virtually every organ system of the body, to include both kidneys, the brain, so that they can possibly suffer from strokes if appropriate conditions are present. They may suffer from adverse conditions of their bones, and a whole gamut of medical problems can also be related to sickle cell trait as well as to sickle cell anemia.

I must emphasize, however, that it is not every day that this occurs, but the danger is ever present and if an individual with sickle cell trait, unknown to have the trait, is exposed to these conditions then certainly he stands to experience the most dreaded complications which could cause him to lose his life as certainly has happened.

Hematuria usually spontaneously but occasionally following trauma, urinary tract infection or hypotensive episodes occurs at a significant frequency. It may occur with or without pain or clots, it may be prolonged and recurrent and is usually from the left kidney. Sickle cell trait, under conditions that bring about low oxygen tension, can result in serious and potentially fatal clinical complications. These complications can be avoided or minimized if the physician is aware of existing latent abnormality.

Nearly the complete spectrum of the major vaso-occlusive complications seen in sickle cell anemia has been described for the trait with no other pathogenic explanation. These include aseptic necrosis of the bone, intestinal infarction, infarctive necrosis of the liver, pulmonary infarction, neurologic disorder, kidney infarcts, leg ulcer, priapsm and increased complications of pregnancy. These findings all remain a constant threat to young blacks with sickle cell disease, particularly in the patient with manifest symptoms.

Due to the high complement of fetal hemoglobin present in the red cells at birth and the consequent low percentage of hemoglobin S, the occurrence of the sickling phenomenon and clinical expression is hindered. Therefore, sickle cell anemia is usually detected only after the first several months of life.

Sickle cell disease has often been fatal before adolescence, but with early detection, medical management, education and counseling, survival to 40 years and longer is now possible. It has been shown that the adolescent ages may show a significant degree of growth failure, delay in appearance of secondary sex characteristics, and delay in the onset of menarche.

Once the disease has been established, it pursues a rather constant course combining the signs, symptoms, and limitations of persistent anemia with the episodic and unpredictable intercurrences of various types of crises.

Early detection, patient education, followup studies, and some preventive measures will lessen the severity of the anemia, painful crises,

and the numerous other complications and morbid features of this disease.

One of the most serious complications is the aplastic aregenerative crisis. It is usually associated with a viral or bacterial infection, the bone marrow is physiologically hypoplastic and ceases to function as an effective blood making organ while the infection persists. The rapid fall in hemoglobin may reach fatal levels in a few days. However, it should be emphasized that the painful crises unless accompanied by a prolonged febrile course are not usually associated with increased severity of the anemia, and that aplastic or aregenerative crisis need not be accompanied by a painful episode.

The laboratory findings are those of severe anemia: hemoglobin content usually 5-9 gm./100 mg., with proportional drop in the hematocrit and red cell count resulting in normocytic and normochromic red cell indices. The stained peripheral blood smear shows variation in size and shape of red cells with 0.5 to more than 20 percent of red cells sickled.

As many of the facts and figures available attest, sickle cell anemia. is a much studied and written about disease in the medical sphere. In spite of this, it continues to be a much neglected disease and a major economic problem in this Nation. This malady afflicts countless black people, most of whom are already of marginal economic means and the occurrence of this disease is sufficient to result in impoverishment by frequent expensive hospitalization. Sickle cell anemia has been estimated by the World Health Organization to kill over 80,000 children annually worldwide.

In the past very little money has been made available for work in sickle cell disease. Almost none of this monev has been available for patient care, screening, education, and counseling.

The development of support programs for sickle cell disease will of natural necessity require adequate expenditures for development of strong victim-oriented clinical programs as well as continued research efforts.

I would remind the committee that much of the effort directed toward the afflicted patient victim of sickle cell disease has been largely experimental. There have been no accolades for the numerous physicians and the several medical centers which have attempted to carry on the clinical efforts against this disease.

Adequate funds must be made available to allow upgrading and expansion of existing programs in patient care, screening, and population education and counseling. Support must be provided for new innovative approaches in these same areas.

Until recent months, grants from the National Institutes of Health have given sickle cell disease a low priority in support, when compared with several exceedingly rare disorders. The attitude in many quarters has been formulated by the basic research accomplishments mentioned earlier. Virtually no concern has been shown for the major problem with this disease: The unfortunate black victim.