In 1958, the pediatrician we used tested everyone, all six of us. This is when we learned that I have a trait, my husband has a trait, Kaaren, the oldest child, has the disease, the second child has nothing, the third child has the disease, and the youngest child has a trait. This is when it was suggested that Kaaren undergo a tubal ligation.

We were informed she would never live to see womanhood; that if she did, very shortly thereafter she would die, and that there was nothing anyone could do, so, you know, get yourselves together, and get ready to face it.

Senator KENNEDY. Who gave you this marvelous story?

Mrs. BOYD. I cannot remember his name, but he was a pediatrician. We were in Decatur, Ill.

We decided, my husband and I, that the decision was not ours to make concerning whether Kaaren should or should not undergo a tubal ligation.

This was something for her to decide. Consequently we did not accept his advice. Kaaren managed to get along, and eighteen years after her birth she entered Spellman College, Atlanta, Ga., and we moved here into this area.

Now, when Kaaren got to school, she was away from us, at her own choosing, she decided, I guess all children do, she wanted to get as far away from us as she could, and when she made the decision, we were living in Kansas City, Mo., so Spellman was far away, which was Atlanta, Ga.

Well, as luck would have it, we moved right up here to Washington, D.C.

The school doctor and nurse decided Kaaren was, according to Kaaren, a chronic goldbrick, and a hypochondriac; that she had no need for her medication, which she kept always, a little box of Darvon. We had been informed by this time that the pain one suffers could be controlled somewhat with Darvon. A person with sickle cell anemia learns to manage the pains but needs additional help when a crisis strikes.

You do not give into every little pain. This is what Kaaren was doing, in trying to make her classes.

When she was ill, and confined in the school hospital, they took away the Darvon and gave her aspirin. When she was released from the hospital, they refused to let her make up her exams, and so it got to be a very, very tight thing.

Senator KENNEDY. Did they not know she had sickle-cell disease? Mrs. BOYD. They did not believe her.

Senator KENNEDY. They did not test her?

Mrs. BOYD. No, they did not test her. They had her medical charts from our doctors. She took all of this with her when she went away to school, but they just refused to accept it.

I do not know what happened, but that is the way it turned out. Shortly thereafter, Kaaren came home and announced, I am getting married, and, of course, my husband and I just went into orbit.

We talked and talked, and we pleaded and begged, of course, she got married.

There was nothing we could do about that. She stayed with us during her pregnancy.

She had no problems at all, amazingly enough, she was cared for at Georgetown University Hospital, her pregnancy was considered a normal one.

She went into labor and a crisis simultaneously and scared the doctor to death.

He told me later that if he managed to finish his internship, he would never, never take another pregnant sickle-cell patient.

He said that he just knew she had suffered a heart attack, and was going to die. We were very appreciative.

Senator KENNEDY. Please continue.

Mrs. Boyd, you do not look like you had all of that trouble.

Mrs. BOYD. The baby was born, and was fine. Kaaren developed pneumonia after the child's birth, and stayed in the hospital for weeks and weeks. Finally, she did get herself together, she was released and she is doing quite well.

She does have crises from time to time.

What I would like to say before I close, is that down through the years, we have felt that somebody failed because no one was able to tell us anything.

The normal, run of the mill people, do not know about sickle-cell anemia, also a whole lot of professional people, even doctors, do not know. It seems that out of the whole situation, the most important thing would be a program whereby people could be educated. To think that a doctor would examine a child, care for a child over a period of 2 years, and not be able to diagnose the correct disease is frightening.

I understand that medical schools give only a little information about it.

There is a disease called sickle cell anemia. My daughter tells me she has been in classes with medical students and they say there is a disease called sickle cell anemia, flash a big picture of a sickle cell on the screen and say, "This is a sickle cell," and that is it.

It seems that most doctors just do not know enough about the disease and its treatment. It is a frightening thing to consider, that you must depend on somebody to know something, and they really do not know it. I think education is very, very much needed.

I feel that is all I have to say.

Senator KENNEDY. Let me ask you this: If you had known, been tested at the time you were married, and you and your husband had realized that you had the trait, and that he had it, do you think you would have gone on and had children?

Mrs. BOYD. If I were that age, 20, and as knowledgeable then as I am now, I imagine that I might have taken a different approach to having a family, because you can adopt children.

There are all methods of acquiring a family, and I probably would have taken another course. If I had been told a little bit about it, the fact that we carried the trait and that there was a possibility of our children being born ill, I do not think that would have been enough. I would have had to know more.

Senator KENNEDY. Mrs. Winston, what would you like to add to this?

Mrs. WINSTON. Mr. Chairman, I have a statement I would like to enter into the record.

I am Kaaren Boyd Winston, senior counselor in the Department of Preventive Medicine and Community Health, University of Rochester School of Medicine and Dentistry, Rochester, N.Y.

I work primarily in the area of sickle cell education and testing under the supervision of Mrs. Naomi Chamberlain, assistant professor in preventive medicine and community health, and Dr. Robert Berg, chairman of the department.

Before working in this department I was employed for 3 years by the university medical center as a hematology technician.

Sickle cell anemia and sickle cell trait have been described as one of the most neglected health problems in the Nation today.

Virtually all sickle cell anemia victims are of Afro-American heritage, but not exclusively.

It is also prevalent among Latin Americans, Puerto Ricans, Indians, Asians, and people of the Caribbean.

Among blacks, the prevalence of the trait is said to range from 7 percent to 13.4 percent.

The generally accepted estimate for prevalence of the trait is 10 percent.

The anemia occurs in one out of 400 or about 0.2 percent.

The counted 22.5 million blacks and a reported possible 5 million. undercount means there are approximately 3 million persons affected by this inherited condition.

Sickle cell anemia is one of the many hereditary blood conditions known to be caused by an abnormal hemoglobin.

Hemoglobin is the substance in the red blood cells which enables the cells to carry oxygen to the tissues of the body and to transport carbon dioxide away.

It is responsible for the color of the red blood cells. Normal red blood cells are biconcave and disc shaped. When the red blood cells of a person with sickle cell anemia receive a decreased amount of oxygen, the cells assume a sickle or crescent shape. Thus, the name of the condition is derived from the distorted shape.

Red blood cells normally live about 120 days. A sickled red cell may live from 40 to 60 days.

The rapid loss of red blood cells causes the chronic anemia of the condition.

Cells with this abnormal shape also exhibit a tendency to congregate in the small blood vessels and to block the circulation.

These two facts are the causes of the symptoms manifested by those persons with sickle cell anemia, a chronic anemia and various types of crises.

Any organ system can be involved in a (thrombotic) crisis.

Those most often involved are the bones, abdominal organs, lungs, brain, and kidneys.

This serves to explain why a person who is having a sickle cell crisis may be diagnosed as having appendicitis because of the extreme pain and tenderness in the abdomen, or as having rheumatic fever or arthritis due to swelling and involvement of the bones.

Sickel cell was first described in medical literature by Dr. J. B. Herrick in 1910-over 60 years ago.

Subsequently, in 1949, the Nobel Prize winning research of Pauling and his associates cast further light on this condition.

Nevertheless, there continues to be a dearth of programs dealing with sickle cell anemia, reflecting the low priority this serious health condition has held.

A child with sickle cell from earliest infancy is a financial as well as psychological drain on his family.

In school-age children, frequent bouts of pain and fatiuge are serious deterrents to ordinary school achievement.

The child's physical activity must be controlled, competitive sports are not recommended, and the child's entire educational program must be tailored to his physical capabilities.

Excellent academic achievement should be encouraged, as individuals with anemia are limited, generally, to sedentary occupations. There is a mother of a 6-year-old boy with sickle cell involved in our program at the University.

She has told us many times of the numerous problems she encountered when her son first exhibited symptoms.

The doctors had no idea what was wrong with the boy.

She was given several different diagnoses, varying from rheumatic fever to allergies, to viral infection.

This went on for 3 years. She almost lost her home because of excessive medical bills incurred at this time.

The boy still is quite ill and by the end of the first month of school this year had missed 9 days of class.

At the end of his first year in school, he had missed a total of 72 days. Good vocational guidance is of extreme importance. A man with sickle cell anemia will find it difficult to obtain and hold a job to support himself and his family, and a woman will have to superimpose the problems of a chronic illness on the duties of homemaking.

Employers must have an understanding of the illness and be willing to accept an employee who may lose a good deal of time from work. Persons with sickle cell must be most carefully instructed as to what is necessary for their health care.

Proper nutrition, good hygenic and dental care, avoidance of exposure to infections, and prompt medical treatment when a crisis or other complications occur are essential.

For these persons the need for competent education and counseling is obvious.

With this need appearing so obvious, the lack of these services is amazing.

A young mother who has sickle cell disease told us of how she found out what her health problems were arising from.

She was expecting her first child, and was being seen by her obstetrician regularly.

She was given no hint of anything being wrong until she came in at the end of her seventh month for her regular appointment and was told that she would be admitted to the hospital that day and labor would be induced immediately. She has since spent 812 months in a body cast because of involvement of the bones in her hip joints.

The needs of those with the trait are not so obvious. At this point, no one is sure who is affected, or how many of these people there are, or even where they are.

There are very few solid facts concerning how having the trait affects a person's health.

It is only known that there is the possibility that persons with the trait can encounter difficulties. For instance, there were four black servicemen who died while undergoing Army basic combat training at a post at an altitude of 4,060 feet.

It was stated that all four were healthy, had no family history of anemia, and originated from low altitude areas.

Under autopsy, the four had sickle cell trait and evidence was found of sickle crisis.

Distended vessels were found to be packed with sickle cells and there were no other causes of death present.

For 3 years, the Department of Preventive Medicine and Community Health has spearheaded intensive educational efforts in the area

of sickle cell.

These have been directed toward medical students and doctors, regarding the social and family impact, staffs of agencies in the community. Rotary Clubs, Sunday schools, penitentiary inmates, and elementary school children in special projects.

Within the past year, through these efforts, the following agencies in the Rochester area have added sickle cell screening as part of their routine physical examinations:

Planned parenthood; three nursery schools; one State school; one public school; one job training program; one children's center; three industrial firms.

The educational component has been the main thrust, with the Department of Preventive Medicine assuming the responsibility for the followup and counseling of both families and health workers.

Our experience has shown that the often used label of apathy and indifference has proven untrue as it relates to sickle cell anemia. programs.

The demands far exceed the ability of the department in terms of reaching the 55,000 persons of Afro-American heritage and the 22,000 persons of Caribbean origin who live in Rochester, N. Y.

Some of the needs include:

(a) Adequate insurance coverage without prohibitive premiums. (b) Need for research: How much does the disease cost the Nation? The trait? What effects does the trait have during a person's lifetime? What is the expense of misdiagnosis of problems caused by the trait? (c) More intense and improved education for health workers and communities.

(d) The need for development of mandatory child health programs for genetic disorders which threaten health status of children, including sickle cell anemia, should be determined.

(e) There is a need for increased research grants and fellowships for the study of sickle cell anemia and sickle cell trait.

I would like to pass on in the discussion to mention my last crisis and what happened at that time.

It took place about a year ago. The cause never was established, you know, what brought the crisis on was not established.

I had worked at my job, my regular hours that week, and I had come home from work, and prepared the evening meal. I decided that I wanted to go out shopping, so at that time I felt fine.

I went out shopping for perhaps 2 hours, and while I was in the store, one of my legs started to ache, finally it got to the point where