STATEMENT OF ROBERT E. COOKE, M.D., PROFESSOR OF PEDIATRICS, JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE, AND PEDIATRICIAN IN CHIEF OF THE JOHNS HOPKINS HOSPITAL, BALTIMORE, MD.-Resumed

Dr. COOKE. Mr. Roberts and gentlemen, developmental defects are extraordinarily complicated problems which I think many lay people find somewhat difficult to comprehend.

For the purpose of demonstrating the complexity of these problems and the important roles of factors operating early in life, as has been alluded to by the panel that just spoke, I would like to present three patients and their families, who illustrate these complex developmental problems.

I think the purpose of this presentation is in no way to attempt to dramatize this problem, but simply to make clear to the people hearing this bill what kind of problems can develop from defects (of paternal and maternal origin) in the early life of the child.

I would also like to hope that these would emphasize the remarks that have been just made by the panel and also remind you that suggestions for inclusion of "maternal factors" in the body of the bill were made in my testimony yesterday.

I am asking Dr. Frederick Richardson, who is the head of our diagnostic and evaluation center at Johns Hopkins, one of my colleagues and a citizen of the United States but with a British accent, to present this very brief clinical session for you.

This diagnostic and evaluation center is largely supported by the funds of the Department of Health of the State of Maryland, and it does not receive any direct Federal support.

STATEMENT OF FREDERICK RICHARDSON, M.D., M.R.C.P., HEAD OF THE DIAGNOSTIC AND EVALUATION CENTER, JOHNS HOPKINS HOSPITAL, BALTIMORE, MD.

Dr. RICHARDSON. Mr. Chairman and members of the committee; I chose three cases without any difficulty from the thousands or more cases we have seen in the last 31⁄2 years at this Center.

Again, this is not to dramatize the situation, but merely to point. out how easy it is to obtain patients and families who are confronted with this type of problem.

This is David Shelton from Baltimore City. He is aged 2 years. At the time of birth David was noted to have a rudimentary or poorly formed right arm. Also a malformation of the little finger of his left hand. He had imperfect and undescended testicles; a congenital heart lesion which might have put him in the blue baby category, but it was not advanced sufficiently to do this. He had drooping of his upper eyelids, problems of tear duct drainage, which meant that his eyes watered constantly. He had a large port wine stain on his forehead, which has now gradually disappeared.

And all of these lesions which I have mentioned now, which are numerous, have hit at different systems or parts of the body systems. Some involve the cardiovascular system. Others involve the skin, muscles, and skeletal system. His nervous system is also involved.

in that he is a little mentally slow, a little slower than children of his own age.

He was admitted to Hopkins where his heart lesion was diagnosed as a duct running from the left side of the heart to the right side of the heart which should not have been open at the time of birth, and this was closed by surgery and this improved his condition very considerably. Since that time his parents have been pleased with his progress.

He will have orthopedic operations and measures done over the next few years to improve the function of his right upper limb, and he will later on be given help in educational and other school facilities. We attempt to merge together the medical management of the child, at the same time looking out for his educational future and for his future potential as a citizen in that we see patients from birth to the age of 21 years, attempting to see them through the period of development. I do not know whether or not the committee would have any questions at all for Mr. and Mrs. Shelton.

The CHAIRMAN. Except to thank her for coming.

Dr. RICHARDSON. Mr and Mrs. Foster have been kind enough to bring David from the Eastern Shore of Maryland. This is the 61⁄2year-old boy, who has three older sisters.

At birth David was found to have absence of the fingers on his right hand, although he did have a thumb present, and he was found to have webbing or sticking together of the fingers of the left hand, and his tongue was so tiny and small and had a deep cleft in it that there was considerable difficulty with swallowing in infancy, and his swallowing muscles behind the tongue were also involved.

It was noted in the first few months of life that he did not laugh or cry as other children did. Although he cried and the tears ran down his face, his facial expression did not change. It was discovered that the reason that his tongue was small, that he had difficulty in swallowing, the reason that his facial expression did not change, the reason that his eye movements were very poor-he could not gaze to one side or the other without turning his head-were that a developmental abnormality was present which involved the nerves supplying the muscles of the head, face, and neck; and, in addition to this, he had a high arched palate, which meant that, even if his tongue was sufficiently large to speak, there was an additional problem in that the high roof to his mouth made speech extremely difficult as he developed. Unfortunately, children who have this "poker face," which they are born with due to this nerve palsy, are often regarded as being mentally slow. David's parents could tell you that from infancy they had no doubt about his mental abilities, and these were confirmed in that he had a bright, intelligent, high IQ at the center when he was examined.

David has been placed in school and is doing extremely well on the Eastern Shore. We have had surgery done to his left hand to free the fingers, which he is now able to use better, and glasses have improved his facial appearance as well as his vision, and the parents, I think, are very pleased with this very nice little boy's progress.

I do not know whether or not the committee has any questions to

ask.

The CHAIRMAN. No questions, thank you.

Dr. RICHARDSON. Thank you, Mr. and Mrs. Foster.

The CHAIRMAN. We certainly want to thank you for being with the committee and your effort to be here. We appreciate it.

Dr. RICHARDSON. The third little girl is Mary MacIntosh, who comes from high up in western Maryland in the mountain area around Cumberland. She was at the time of birth noted to have a considerable number of developmental anomalies.

At the time of birth she was noticed to have absence of the nostrils of her nose, a lot of skin defects at the top of the head, abnormal genitalia, and she grew very poorly and very slowly. Mary is now aged 10 years, and, as you can see, she is considerably dwarfed in size. Nevertheless, she has near average intelligence and gets along extremely well with people, being a very friendly little girl. Some years ago she was seen at a large clinic outside of Maryland where the parents were told that her dwarfism was due to kidney disease and that she had about a year to live.

When the year elapsed and Mary still seemed to be going strong, they brought her to other doctors, to us at Hopkins, where examination was made and a complete workup was done. Mary has very many problems which are too detailed to go into now. One of her major problems is that she has a neurological deafness. She has deafness involving the brain itself, rather than the nerves going to the brain, so that, even as she receives messages, she is unable to understand them; and she, therefore, ignores all sounds and has never answered to her name.

On the other hand, if you tap your fingers on the desk or tap your foot on the floor, she is as quick as a bird in turning around and picking up signals, and she uses her eyes in much the same way as a blind person might use her ears.

She is extremely perceptive and catches on very quickly to what is required of her, although she has this severe deafness.

Mary has been placed in school and she is doing well there.

We have repaired her nose, as you can see, by giving her a new nose which has now healed satisfactorily, and it looks reasonably good, and we have other plans for other abnormalities of her genitalia and other areas which will require attention over the future years.

She will always be a small child, as the type of dwarfism she has is not due to any glandular deficiency and cannot be improved, although she will grow a little as time goes on. She will always be a very small girl for her age.

Thank you very much, Mr. and Mrs. MacIntosh.

The CHAIRMAN. Thank you, Dr. Richardson.

I appreciate very much the fine presentation you have made, and I want to again express the thanks of the committee to you and Dr. Cooke and to these fine parents of these children for coming here today.

I do not believe we have any questions.

Dr. COOKE. Thank you very much, sir.

The CHAIRMAN. At this time, due to the fact that we are very close to the close of the morning session, I would like to run through the list of witnesses to find out if there are any of you who have pressing engagements who might be willing to file your statements.

The committee will be in session this afternoon, and I am not foreclosing anyone, but I know sometimes there are plane schedules to meet and other engagements so I will just run through the list and,

if you wish to make any disposition of your statement, you can make it to the committee.

Dr. Stewart H. Clifford of Boston Lying-In Hospital. Dr. Clifford?

Dr. CLIFFORD. Mr. Chairman, I could be here this afternoon. I have a written statement I can submit. It does not make the slightest difference.

The CHAIRMAN. I would be glad to hear you this afternoon, Dr. Clifford, if you can stay.

Dr. Gordon M. Heath?

Dr. HEATH. I will be here this afternoon.

The CHAIRMAN. All right, Doctor.

Dr. Joseph C. Denniston, superintendent, Tennessee Glover Bottom Hospital and School, Donelson, Tenn.?

Dr. DENNISTON. I will be here.

The CHAIRMAN. Dr. John W. Riley, Jr., vice president in charge of research of Equitable Life Assurance Co., care of the American Sociological Association of New York University, Washington Square, New York, N.Y.

Is he here?

Dr. Sidney Farber, scientific director, Children's Cancer Research Foundation, Boston?

Dr. Aldrich, chairman, department of pediatrics, University of Washington. You will be here this afternoon, sir?

Dr. ALDRICH. Yes.

The CHAIRMAN. I am not sure about my pronunciation, Dr. Robert Havighurst.

Dr. HAVIGHURST. I will be here this afternoon.

The CHAIRMAN. Dr. E. W. Busse?

Dr. BUSSE. I will be here this afternoon.

The CHAIRMAN. Dr. Wilton Marion Krogman, director of Philadelphia Center for Research in Child Growth?

Dr. KROGMAN. I will be here this afternoon.

The CHAIRMAN. Dr. Julius B. Richmond, College of Medicine, State University of New York?

Dr. RICHMOND. I will be here this afternoon.

The CHAIRMAN. Fine.

The subcommittee will stand in recess until 2 this afternoon, at the same place.

(Whereupon, at 11:55 a.m., the hearing was adjourned, to reconvene at 2 p.m., of the same day.)

AFTERNOON SESSION

The CHAIRMAN. The subcommittee will be in order. We will continue our hearing on the bills before us.

Our first witness is Dr. Stewart H. Clifford, representing the American Academy of Pediatrics, an assistant clinical professor of pediatrics at Harvard Medical School, in the Boston Lying-in Hospital, Boston, Mass.

We are very glad to have you before us, Dr. Clifford. You may proceed in your own way.

STATEMENT OF DR. STEWART H. CLIFFORD, REPRESENTING THE AMERICAN ACADEMY OF PEDIATRICS, BOSTON, MASS.

Dr. CLIFFORD. Mr. Chairman, I am, also, past president of the Academy of Pediatrics. I am speaking both as their representative and as an interested individual.

I have been fascinated by the presentation I have heard this morning. I have a formal statement that is on file, and rather than repeat many of the excellent presentations that have been given I would rather let my detailed statement speak for itself and more or less informally discuss some of the subjects which have been discussed and maybe bring out some refinements as to the statements that have been made.

It is, of course, known, as has been emphasized this morning, that many of the physical, emotional, and behavioral mental handicaps of later life can be traced to the prenatal period of birth. While the actual conditions are extremely rare in terms of pathology per 1,000 births, however, when one thinks in terms of 5 million births in this country, approximately, a year, and forecasts 7 million by 1970, the accumulation of these unfortunate incidents become a tremendous public health problem. And, also, a tremendous source of loss of future manpower, citizenry, and a great tragedy to the families involved. There has been tremendous emphasis this morning on prevention, and I think that this is the goal of the new Institute. I think it has been said that we have had great advances in medical knowledge, and the problem has been to put to work the tools and the knowledge we have. I think it is only fair to say that if we could place in a program and organize and use the material and knowledge that we have at this moment there could be a material reduction in prenatal mortality and morbidity and the tragedies that you have heard of this morning. This is where in programing the new Institute, I think, could make tremendous strides.

There has been a great deal of really very alarming talk about prematures. I would like to put in a plea that all is not as bad with the premature infant as that; that the premature infant who is 5 pounds or 41⁄2 pounds has a good outlook, has as good an outlook as the older and heavier child. I do think it has been emphasized and proven by a number of studies that our real problem is in the small premature. We know that in this period or group, below 41⁄2 pounds at birth, 25 percent will develop serious mental retardation, cerebral palsy, or other neurological disorders. And this will be by the time they are 3 years old. Fortunately, the bulk of the prematures are above this crucial weight. You are only dealing with about 35 percent of the premature population when you concentrate on the 41⁄2 pounds and under. This means in this very small group you are having the bulk of later catastrophes we have heard mentioned. This, to some extent, simplifies our problem.

We have had many programs to try and cut down the overall incidences of prematurity. There has been great activity in the Children's Bureau in planning and assisting programs for premature infant care. These programs have been excellent, but our premature incidence in this country has remained at 7 to 8 percent. It has been steady at that level over the years, in spite of all of the programs. The reason for this is self-evident, because the die is cast by the time

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