5 fortunate areas virtually nothing is known approximating the true incidence of sickle cell disease nor its morbidity or mortality. Funds must be provided to support development of such studies and to provide some care, education and counsel ing of victims so pinpointed. Establish In terms of realistic figures, of funds required to support a farily circumscribed screening and education program the following are illustrative: ment of a proposed pilot project in Davidson County, Tennessee to screen initially 24,000 black school children would total an estimated $57,000 in direct costs. Subsequent years would be at approximately the same level with expansion into other locals and actual number of persons screened increasing. Subsequent costs per screened individual should decrease. This type of program is planned to utilize community organizations and recruit volunteer workers. Using currently accepted incidence figures this program in 5 years should screen approximately 150,000 to 175,000 people. As a result some 2,500 patients with hemoglobin S will be picked up per year. The number of victims afflicted by the dreaded homozygous form would total approximately 57 per year. Should these victims have an average of five vaso-occlusive crisis per year each projected this would require a minimal cost outlay for hospitalization alone of $19,000 - $20,000 (based on hospitalization for 4-5 days per crisis at A.H.A. figure of approximately $78.00 per day for each 57 homozygous patients detected). The above figures are fairly reasonable estimated based on data collected from various reported medical studies and our current hospital figures. No estimate is given for medical center outpatient costs which should be included in legislation such as that proposed. In summary, for all to long sickle cell anemia has been virtually ignored as an important disease among a major segment of this nations population. The victim, malinged and used in many instances as only a "good source of interesting 6 research material". The time certainly has come to give adequate support to such programs as will provide both care for the victim and continue to expand our knowledge of this patient and his disease. It is hoped that the current spurt of interest in sickle cell disease will remain high and provide the victim with support so long denied. Senator KENNEDY. The subcommittee will be in recess for 15 minutes. (Recess.) Senator KENNEDY. We apologize to our witnesses here for these interruptions. They are completely unavoidable. They are an attempt. to extend some unemployment compensation payments to people who are unemployed in many of our communities and States, so it is a good cause. I apologize. STATEMENT OF MAURICE RABB, M.D., CHIEF OF OPHTHALMOLOGY, MERCY HOSPITAL, CHICAGO Dr. RABB. Thank you, Senator Kennedy. I will not read the entire text. Senator KENNEDY. We will include all of your statements in the record as if read at the end of your testimony, and you can highlight it. I have a few questions at the end. Dr. RABB. I am Maurice F. Sabb. I am presently chief of ophthalmology, Mercy Hospital, Chicago: assistant professor of ophthalmology, University of Illinois Eye and Ear Infirmary; medical director, Illinois Eye Bank and Research Laboratory; member of the Ophthalmic Advisory Group to the Food and Drug Administration; ophthalmic consultant, Martin Luther King, Jr. Health Center and Daniel Hale Williams Medical Center. I would like to take this opportunity to express my concern about sickle-cell disease, particularly sickle cell eye disease, as a public health hazard today in the United States. Even though this disease entity was discovered over 50 years ago, most Americans, black and white, are still unaware of its devastating effects upon the black population. In recent months, much has been written in the popular press dealing with the classical clinical manifestations of sickle cell disease, such as joint pains, ulceration of the lower extremities, abdominal pains, fever, jaundice, cardiac, and neurological disturbances, and the frequent fatalities. As more attention is focused upon these crippling effects of the disease, it is astonishing that the majority of the public as well as the medical profession are unaware of the potential danger that exists to the eyes. The eye symptoms are numerous. The classifical picture of sludging of the blood in conjunctival capillaries has been described frequently in the ophthalmic litreaure. However, it is in the funds or retina of the inner eye where the damage produced by the disease manifests itself most dramatically and tragically. This damage often goes un noticed since the so-called proliferative sickle retinopathy or retinal disease originates in the peripheral portion of the retina where both the patient and the doctor find it difficult to be aware of any abnormality in the early stages of the disease. The retinal disease begins initially as the small capillaries in the periphery of the retina become blocked off by the abnormal sickle shaped red blood cells. Next, these tiny capillaries which ordinarily never connect with one another unite, forming abnormal communications. The next stage consists of new blood vessel formation with associated fibrous scar formation. If the disease process is allowed to progress, larger patches of vessels develop, called "sea fans." Should these abnormal vessels break, and they almost invariably do, a serious hemorrhage in the eye may occur with subsequent detachment of the retina and often permanent loss of vision. Eventual loss of the eye is not uncommon. Which individuals are affected? If a person receives the abnormal sickle gene from one parent, he then possesses a sickling trait, and only in exceptional circumstances shows any sign or symptoms of the disease in the eye. However, should two abnormal genes be transmitted, the homozygous individual suffers from sickle cell disease. Another condition persists as sickle cell thalassemia (S-thal hemoglobin) which differs little from sickle cell trait (SA) except for a higher percentage of hemoglobin S, and a higher percentage of patients with new and dangerous vessels in the retina. Patients with S hemoglobin and C hemoglobin show similar retinal pathology. HOMOZYGOUS SICKLE CELL DISEASE (SICKLE CELL ANEMIA) Although this is the most serious form of the disease as far as the general body is concerned, it does not have serious effects on vision in most cases. Characteristic of sickle cell anemia are circular, black chorioretinal scars, "black sunbursts." These lesions represent sequelae of small blood vessel occlusions or hemorrhages but do not ordinarily affect visual acuity. These lesions are found in 43 percent of patients with sickle cell disease. New blood vessel abnormalities are found in 10 percent of patients. In the heterozygous sickle-cell hemoglobin disease, which is called SC hemoglobin disease, dangerous neovascular abnormalities are found in 60 percent to 70 percent with SC disease. Black chorioretinal scars are found in only 18 percent. The early sign of capillary anastomoses has been observed in 91 percent of cases examined which makes hemoglobin SC disease the most prevalent variety to have the type of proliferative retinal disease that leads to bleeding into the inside of the eye. Several recent scientific articles are submitted with this testimony to document what little is known of sickle eye disease. (The information referred to follows:) 428 Reprinted from the Archives of Ophthalmology Copyright 1971, American Medical Association Natural History of Untreated Morton F. Goldberg, MD, Baltimore and Chicago In this clinical study of the natural history of nal" population, from which hypotheses have proliferative retinopathy in sickle cell-hemoglobin C disease, 25 eyes were observed without therapeutic intervention for an average duration of 31 months (range: 6 to 77 months). A new ophthalmoscopic and angiographic classification allowed quantitative assessment and comparison of these eyes at the beginning and at the end of the observation period. Clinically symptomatic and significant stages of proliferative sickle retinopathy (PSR) were frequently observed in patients between the ages of 20 and 30. All stages of PSR appeared to progress. This seemed particularly true of that stage characterized by spontaneous vitreous hemorrhages; ie, 28% of eyes had evidence of vitreous hemorrhage initially, whereas 44% of the same eyes had similar evidence at follow up. No proliferative lesions were seen to regress. Submitted for publication July 29, 1970. From the Department of Ophthalmology and Medicine, Division of Medical Genetics, the Johns Hopkins Medical Institutions, Baltimore, and the Department of Ophthalmology, University of Illinois Eye and Ear Infirmary, Chicago. Read in part before the Section on Ophthalmology at the 119th annual convention of the American Medical Association, Chicago, June 22, 1970. Reprint requests to Department of Ophthalmology, University of Illinois Eye and Ear Infirmary, 1855 W Taylor St, Chicago 60612 (Dr. Goldberg). been drawn concerning the pathogenesis and classificatoin of PSR.1.4 Four eyes presented with retinal detachments which required immediate surgery2 and were thus not included in further studies concerning the untreated course of PSR. Fifteen eyes were treated by photocoagulation for various reasons, including vitreous hemorrhage from neovascular proliferations, and will be included in a separate report concerning therapy of PSR. Three eyes were lost to follow up. Thus, 25 eyes (16 patients) of the "original" 47 eyes were left for observations concerning the untreated course of this retinal disease and will be designated the follow-up population. Because many patients were originally referred only if they had ocular symptoms, possible bias in selection of these eyes for this follow-up study is apparent.1 Ocular fundi of all patients were studied by indirect ophthalmoscopy and fluorescein angiography, the detailed techniques of which have been reported previously4-6 Eyes were observed for a minimum of six months and for a maximum of 77 months (mean: 31 months). The frequency distribution for follow-up durations is shown in Fig 1. Follow up was terminated at varying intervals for arbitrary reasons or when progression of neovascular proliferations or of vitreous hemorrhages was sufficiently rapid or severe that therapeutic intervention appeared warranted. At the initial examination and at the end of the follow-up period, each of the eyes was Arch Ophthal-Vol 85, April 1971 |