Senator KENNEDY. Have you visited the medical schools in trying to get it? Dr. ZAPP. Yes. Senator KENNEDY. What has been their reaction; what has been their response? Dr. ZAPP. I think that the medical schools are probably being extremely responsive. Senator KENNEDY. In what way? Dr. ZAPP. First of all, they probably have a variety of approaches. Senator KENNEDY. Not probably. What approaches, and in which medical schools? Dr. ZAPP. You are talking specifically about sickle-cell disease? Senator KENNEDY. Yes. Dr. ZAPP. I visited many medical schools, obviously. I have not visited one to discuss the aspects of sickle-cell anemia. Senator KENNEDY. Has anybody in the Department, that you know of, gone out to a medical school and said, we are concerned about this, the administration is concerned, our Department is concerned, and we want to know what you are doing, what you can do, and what you are able to do? Have you met with the American Medical Association? What have they told you? All we are trying to get here is a measure of the Department's commitment in terms of trying to do something on this. I do not question yourself or Dr. Beckles. I do not question it one bit, and I have a great respect for you gentlemen, because you have been enormously helpful personally to me in this subcommittee, but you come here in terms of presenting the administration's view, and I am sure you do it in good faith; but we have a right to judge whether the administration is really interested in doing something, and you say the medical schools are preparing to do something. I want to know who has gone down there and visited those schools, and what they have told you. I want to know who has gone to the great research centers of this country and spoken to them on the sickle-cell anemia problem, and what have their responses been, and what they have told you about the kinds of resources that are being used to fight this disease. I want to know if the medical schools are doing any new kinds of training programs. I want to know if any of the medical schools are developing criteria to help assist young physicians, and which medical schools are leading the way. Those particular medical schools which are leading the way ought to be commended, and we ought to know what they are providing in terms of leadership, and I would like to know in terms of whether you have met with neighborhood health centers, and if they have been asked if they could handle this program, and I would like to know their response, and I would like to know whether you have met with the consumer advisory groups and asked whether they are interested and concerned with the problems of sickle-cell anemia and with helping their community. I want to know all of those things in terms of initiatives. Dr. ZAPP. I think you have every right to know that, and we will certainly supply the information you have requested. Dr. Ringler had to leave earlier because of his wife being hospitalized. Senator KENNEDY. Well, you have some idea of what we are looking for. Dr. ZAPP. I think Dr. Beckles could give you a discussion of it on the service side as it relates to the medical schools. Dr. BECKLES. Mr. Chairman, we have worked in some small way with medical institutions, like Howard University, Meharry, and clinics such as those in New York on the private side. It is because of this kind of exchange of information that we have been able to structure the kind of program that we propose to follow. I daresay, as in every other instance, the medical schools that we have worked with have said that they would do more, but they do not have the overall budget with which to follow these kinds of emphasis. Senator KENNEDY. I think it is a very fair response on this. As I said, I believe you gentlemen, if you could spend all of your time doing it, and do a great job at it, but just in terms of trying to evaluate whether we are responsible in allocating this kind of limited resource, $25 million initially in terms of screening programs, $5 additional million that may be used in research, we want to know whether we are in the ball park with these amounts. I think we are in terms of the kind of information we have had here. If you could provide supplemental information on this, we would appreciate it. Dr. ZAPP. We would be pleased to do it. (The information referred to, subsequently supplied, follows:) DISCUSSIONS BETWEEN DHEW AND OUTSIDE ORGANIZATIONS ON SICKLE CELL DISEASE PROGRAM Contact has been made with many types of organizations or groups which might be expected to participate in this effort. The makeup of our Advisory Committee reflects this, representing as it does the large medical schools, city hospitals, neighborhood clinics, major university research centers, social service agencies, neighborhood centers, the black press, and the community itself. Many conversations and visits, to private and public, large and small, institutions spread widely throughout the country have taken place to date. A meeting is planned with representatives of those other government agencies concerned with providing health care in order to determine the willingness and ability of each of these to make a contribution in support of this initiative. Dr. ZAPP. A preliminary study was done for the Advisory Committee, and it is fairly lengthy, but I do not think it is in any way complete. I would like to supply an outline of the survey for the record of the subcommittee. (The information subsequently supplied follows:) SURVEY OF ONGOING PROGRAMS RELATED TO SICKLE CELL DISEASE Information on the magnitude and extent of ongoing work in the area of Sickle Cell Disease was requested from 31 organizations known to be engaged in these activities; 21 responded. A broad spectrum of groups was consulted, from large privately supported medical or academic institutions to voluntary health groups with limited local coverage. A population sample of about 100.000 had been screened for SCD by these groups, and included infants, children, young adults and routine obstetrical patients. About 4 of these were screened as part of SCD programs which include follow-up clinical diagnoses, hemoglobin genotyping, counselling in both the genetic aspects and medical management of the disease, and often active programs of SCD research. Two such comprehensive programs of long standing, those at Howard University and Meharry Medical College, are responsible for screening more than half of the patients enumerated. All of the groups questioned expressed some cautionary feelings regarding what has become a recurring major concern-the need for adequate follow-up to screening and the provision of appropriate educational material. In 70% of the groups, initial screening was followed by confirmatory analysis; 60% of the organizations have incorporated a counselling service into their programs; many provide educational literature and offer medical referral. The operating facilities vary from hospital clinics to schools and mobile units: the staff personnel are equally varied, though most groups include or consult with physicians and nurses in addition to their lay or para-professional workers. Support for these groups generally comes from private donations; departmental funds are usually used for those programs with academic or medical school connections. Senator KENNEDY. Thank you very much. It was nice to have you with us this morning. Our next witnesses this morning are a mother and daughter, Mrs. Dorothy Boyd and her daughter, Mrs. Kaaren Winston. Mrs. Boyd is financial secretary of the National Sickle Cell Disease Foundation and Mrs. Winston is a medical technician in Rochester, N. Y., at Strong Hospital of the University of Rochester. In her work at the hospital she detects victims of sickle cell anemia and counsels with patients. Mrs. Boyd has four children-two have the disease. One has the trait, and one child is not affected. All four of her children were born before she and her husband knew they were passing on this disease. Her daughter, Mrs. Winston, has the disease, but her-Mrs. Winston's daughter does not have it. I want to welcome both of you to the subcommittee this morning. We are looking forward to hearing your testimony. You relax now, just take your time, just tell your story. We get all excited when we sometimes have those administration people up here, but I just want to let you know that you are among friends, and we are friends of the administration too, and you just tell us your story, and we are looking forward to hearing from you. STATEMENT OF MRS. DOROTHY BOYD, SILVER SPRING, MD., AND OF MRS. KAAREN WINSTON, ROCHESTER, N.Y. Mrs. BOYD. I guess the thing to do would be to start at the beginning. I remember, in 1945, my husband came home from the Second World War. We had an infant daughter. My husband entered the seminary and worked part time, leaving me alone with this young baby. We lived with my parents. We were very young. Being alone, I spent a great deal of time with the baby and formed the habit of putting the baby in the bed with me. Kaaren must have been about 8 months old when she had trouble with her elbows swelling. I can remember my mother giving me a long lecture about putting the baby in my bed. She thought I had rolled over on the baby and caused her to have swollen joints. Shortly after that, Kaaren began to have bronchial infections, which would develop into pneumonia. One night she actually went into a coma. We struggled along with this, off and on, for two and a half years, thinking that the child would grow out of it. We had no idea of anything, except that she wa This is what the doctor said, she was just a weak child. In 1947 my husband and I moved to Magnolia, Ark., and he assumed the responsibility of his first charge. He was earning all of $28 per week. We did not know anyone. It was ironic. We moved into this new small community and Kaaren's health began to deteriorate. She was sick most of the time and had to have a blood transfusion about every 6 to 9 weeks. She would perk up immediately after the blood transfusion, and then go back down again, so that in approximately 2 months, she would return to the hospital for another transfusion. At this point no one said anything about sickle cell anemia. The doctor said he thought she had rheumatic fever. He changed it and said it was malaria. He put her on a constant dosage of coco quinine, which really did not help at all. It hindered the whole thing. We found out later it was dehydrating her system. She became thinner and her abdomen was swollen; she did not look well at all. We thought we had a quiet child, but we really had a very sick child. Senator KENNEDY. What did you think was wrong with the child? Mrs. BOYD. What the doctor said. He said it was malaria. Neither one of us could figure out where she got it. We knew vaguely about mosquitoes. Senator KENNEDY. The doctor said malaria? Mrs. BOYD. He said malaria, and we had to accept it as that. Magnolia, Ark., my home is in Chicago, and to move from Chicago to Magnolia, Ark., is like going to a swamp. It is about 18 miles north of the Louisiana border, and I thought maybe that is where the disease came from, the malaria. In 1949, we moved to Tyler, Tex., to a new situation. Senator KENNEDY. What happened, did it have any effect? Mrs. BOYD. It seemed to. We found out later, that she was dehydrated. The doctor who told us this contributed some of it to the coco quinine, which she took daily, every so many hours, night and day, for about a year. Senator KENNEDY. Where were you getting this drug? Mrs. BOYD. We bought it at the drugstore. Senator KENNEDY. You paid for it? Mrs. BOYD. Yes. Senator KENNEDY. For about a year? Mrs. BOYD. Yes. The blood transfusions at that time, you had to pay I think $50. Senator KENNEDY. For a transfusion? Mrs. BOYD. Yes. Senator KENNEDY. Do you remember what your medical bills were generally over that period of time, approximately? Mrs. BOYD. They were out of sight. They were so large, we just lived from day to day, from week to week, and just managed. While we were in Magnolia, we were fortunate, somebody decided that we needed help. The Kiwanis Club there helped us with the bill for the blood. Senator KENNEDY. Which Kiwanis Club? Mrs. BOYD. In Magnolia, Ark. When we moved to Tyler, she became desperately ill, and we knew no doctor. A friend of mine suggested I use her doctor, who incidentally was an internist. I did not know what that meant at the time. It turned out that this man, a Jewish doctor, diagnosed sickle cell anemia. That was the first time we had ever heard of it. Senator KENNEDY. How old was she? Mrs. BOYD. She was 4. He admitted he knew very little about it. He sat down with us in his office with a textbook, and read maybe 10 lines, and that was it. We used this doctor, for approximately 3 years. During this time we had another child, he was a normal child, as far as sickle cell anemia is concerned. He was a very large, active baby. The doctor was afraid that Kaaren's spleen would be ruptured with this rough boy child bounc ing around. Her spleen was absorbing the damaged sickle cells, this is what made her stomach so large. The doctor suggested that we allow her to have a splenectomy. We were given to understand that the spleen should be removed because if she bumped herself at the correct angle, she would bleed to death before he could get to her. So, that this was the primary reason for the splenectomy. The doctor explained Kaaren might experience some relief from the sickle cell anemia incidental to the surgery. The operation was performed: however, Kaaren continued to have her crises, although they did not seem to be as severe. Before surgery all of her limbs ached and were swollen, and she could not walk. After the splenectomy, most of the crisis pain was centered in her abdomen and back. That is all we knew. No one told us that Kaaren's illness was congenital. Senator KENNEDY. Did you have to pay for that splenectomy as well? Mrs. BOYD. We at that time were able to afford hospital insurance, and they did it for whatever was paid by the insurance company. Then, let's see, and we struggled along, and eventually we wound up in Decatur, Ill. Senator KENNEDY. Did anyone counsel you or advise you? Mrs. BOYD. The doctor in Tyler did tell us that Kaaren had a chronic disorder, sickle cell anemia, and the best thing for us to do would be to adjust to it. We were advised to learn to live with it like any other chronic disorder, and to try above all else not to develop hypochondriac. This is what we did. Senator KENNEDY. Did they suggest anything about having the other children tested? Mrs. BOYD. Not at this point. By the time we were living in Decatur. This was 1958, about 13 years later, we had two more sons, the entire family was born. We still did not know very much. No one had counseled us as to whether we should have any children. |