Bringing CNS Members Together to Make Children’s Lives Better

CONNECTING TO...

John Mark Freeman, MD

(1933-2014)

Written by: Robert S. Rust MA, MD

John Mark Freeman, MD

John Mark Freeman was born in Brooklyn, New York, on January 11, 1933. He was educated at Deerfield Academy and Amherst College from which he graduated A.M. cum laude in 1954. Deciding to become a physician, he attended medical school (1954-1958) and trained as a pediatrician (1958-1961) at Johns Hopkins. During these years he, as did many Hopkins medical students, fell under the influence of David B. Clark. Clark was not only the key influence on Freeman’s decision to become a child neurologist, he also exerted enduring influence as mentor and primary role model. Freeman’s rather intensely analytical mind was naturally attracted to Clark’s remarkable intellect–his substantial and well organized fund of neurological and neuropathological knowledge and especially his powers of clinical ascertainment and logical ensuing analysis of each case he encountered. He was also influenced by Clark’s desire to considerably refine not only correct ascertainment of diagnosis, but also assessment of the value of treatments, especially as measured by well-designed outcome studies. Clark’s role as advisor to the National Collaborative Perinatal Project provided a model for Freeman’s future, similarly meticulous, studies of neonatal seizures and other neurological disorders of neonates. Indeed, nearly one-quarter of the 158 original papers he would publish were to be primarily concerned with developmental abnormalities associated with pre- or perinatal influences affecting nervous system function. These were to include epilepsy due to developmental disturbances as well as anatomical abnormalities of skull, brain, and spine. Dr. Freeman’s first two papers, published early in his neurological training, concerned management and outcome of craniosynostosis. They established a pattern that would characterize many ensuing papers: gathering together as many meticulously characterized cases as possible for careful retrospective review in order to challenge “accepted wisdom” and habits of practice. His attempts to define improvements in practices would continue throughout his career, including not only assessment of functional outcomes, but examination where needed of the ethical aspects of practice that were associated with seriously disabling diseases.

In 1961 Dr. Freeman was selected for training in neurology and child neurology at Sidney Carter’s program at Columbia Presbyterian one of the earliest-established programs for such combined training. In addition to Carter, Freeman was to be particularly influenced by two individuals who would be among his earliest colleagues in the performance of first class clinical research, Alan Aron and Arnold Gold. In New York, Dr. Freeman also became acquainted with Horace Hodes, whose highly distinguished career at Hopkins as an infectious disease specialist and, incidentally, the describer of the function of Vitamin D, had been followed by appointment as Director of Pediatrics at the Mt. Sinai Hospital. Hodes played a key role, as he had for others, in Freeman’s development as a clinical researcher—exemplifying the skill of selecting topics of importance because they focused on areas of clinical and scientific confusion that merited solutions and insights that could be arrived at on the basis of hard honest work. During Freeman’s third year of neurological training, Aron and Freeman published two important papers that concerned not only the natural history of Sydenham chorea, but also investigated the extent to which emotional and psychiatric consequences may be attributed to an inflammatory illness. The care with which cases were identified, the meticulous manner in which personal and family history and disease manifestations were characterized, and the long follow-up of natural history of these individuals (after as many as 26 years had elapsed) in the challenging context of a restless urban population demonstrated the skills and determination with which Freeman would carry out many ensuing invaluable studies within the context of a large city on a wide variety of topics. Given the care with which the studies were carried out, the impression that persistent psychological and psychiatric disturbances might indeed become apparent and troublesome in the wake of Sydenham chorea was further refined within the context of the premorbid characteristics of these patients and their families. The unusual degree of association of psychological disturbances was thus meticulously shown to have a complicated and perhaps in some degree heritable context within which Sydenham chorea could be seen to play an exacerbating role including post-infectious persistence of some traits. Although the fortunate rarity of Sydenham chorea make it unlikely that a further study of this association will be performed, it is important to consider the value of the approach of Freeman and Aron as a model for current studies attempting to implicate inflammatory processes as treatable causes of psychiatric and behavioral disturbances of children. Freeman wrote another paper at Columbia with another individual who would become celebrated for his concerns for humanity in medicine: Arnold Gold. This paper on early diagnosis of tuberous sclerosis also prefigured Freeman’s ensuing lifelong concern for early diagnosis and excellence in the management of conditions associated with epilepsy. Columbia recognized the abilities that young John Freeman manifested in these papers by awarding him the Lucy Moses Prize for Research in Neurology during his senior year of residency.

Two years of research followed at the Walter Reed Army Institute of Research in Washington, during which Freeman acquired another critical famous mentor: Guy McKhann. Freeman treasured his experience with McKhann not only for his valuable mentorship concerning neurodegenerative diseases but also for another critical contribution: providing, at least as Freeman recollected, a strong sense of the importance of having “the Freedom to do it my way.” At this time Dr. Freeman formed a professional relationship that would last throughout his career with Karin Nelson. His admiration for Dr. Nelson was based on her exceptional critical thinking, her exquisite analytical abilities, and her devotion to finding ways to improve the outcome from all forms of neonatal neurological stress. Both were to spend careers—as would many others in this period of scientific awakening of child neurology–tirelessly demonstrating the inadequacy of many elements of poorly studied and unsystematic “received wisdom” of the subspecialty. Three years on the faculty at Stanford followed for Dr. Freeman.

John Freeman permanently returned to Johns Hopkins in 1969 to found, together with Guy McKhann, the child neurology program, and to serve as Director of the Child Neurology service. As did McKhann, Freeman assumed a vigorous role in reviving the teaching program, the effectiveness of which had been significantly impaired by the departures of a number of remarkable teachers including Frank Ford, David Clark, and John Menkes. As Freeman understood very well, each of these great educators contributed with rather different styles. The Hopkins neurology tradition had been one of quite accomplished bedside teaching in rather various masterful styles. This was well-known to Freeman based on his own experiences and it suited him quite well. In addition to establishment of a formal child neurology training program, Freeman assumed responsibility for the large number of visiting young child neurologists especially from Australia, that had been attracted to Hopkins especially in order to receive some Hopkins bedside training. Throughout his own ensuing career at Hopkins Freeman would continue another Hopkins tradition, that of leaving no doubt in trainees’ minds the degree to which their examination skills and ensuing clinical formulations rested on insufficient foundations. Dr. Freeman would continue the Hopkins tradition of excellence in bedside education, at times perhaps less subtle in his indications of the insufficiencies of those he taught than his predecessors. He likely exceeded all others as well in his devoted teaching omnipresence—presumably the product of his unceasing regard for excellence, as he perceived it, in every detail of the activities of the section. During the twenty-one years that he spent in these leadership roles he would rise to the rank of Professor and in 1991 he was named the Lederer Professor of Pediatric Epilepsy. .

Dr. Freeman’s particular concentration in epilepsy was enriched by interactions with Samuel Livingston. At the time of Dr. Freeman’s arrival, Livingston had directed the epilepsy clinic at Hopkins for 27 years. His remarkable work ethic, and his tenacious desire to accomplish clinical research in order to “get things straight” with regard to the benefits and disadvantages of particular treatments were appreciated by Freeman, as was his willingness to evaluate and employ properly any treatment that might improve the lives of children with epilepsy. Of the 158 original papers published by Freeman and his associates, 58 concern a remarkable number of aspects of the evaluation and treatment of epilepsy. Among these papers, nineteen concern the proper manner and efficacy of treatment of epilepsy with the ketogenic diet, a treatment that Livingston had employed and evaluated in comparison to other treatments for many decades. The studies of the Freeman group demonstrate two differences from those of Livingston: they are far larger and methodologically sophisticated and they are more outspoken about the superiority of the approach to other available therapies especially when employing the cream rather than the MCT oil diet. There is in these various papers a particular sense of both urgency and insistence surrounding treating any neurologically associated problem in the best possible way that has engendered controversy, though the heat of argument seems to have had the valuable effect of usually (though perhaps not always) elevating the playing field upon which such controversies are to be worked out. The papers have proven influential: the first large, well-designed prospective study of the efficacy of the ketogenic diet has been cited nearly 300 times to date. With regard to the efficacy of the cream as compared to the MCT oil forms of ketogenesis, the Freeman group organized a head-to-head trial, published in 1992, that has subsequently been cited 173 times.

Dr. Freeman’s drive to ascertain the truth about treatment efficacy and identification of side effects is shared by many child neurologists—it is a drive that has enriched the field of pediatric epileptology with a valuable sense of intellectual competition. Dr. Freeman’s enjoyment of publicly debating some of the more important issues may have seemed less enjoyable to others than it did for Dr. Freeman. But there was no doubt on anyone’s part that what he really enjoyed was not winning an argument or satisfying himself that he had done so, it was that he was so very earnest about making things better. In addition to medical management of epilepsies, Dr. Freeman, his group, and the strongly committed neurosurgeon, Ben Carson and his team published a series of papers concerning the efficacy of surgical treatments (hemispherectomy, hemidecortication, lesionectomies) for dysplasias, hydrocephalus, and particular treatments for Rasmussen encephalitis and Sturge-Weber. These have demonstrated that remarkably improved seizure control may be achieved in surviving patients while at the same time carefully describing the associated functional gains and losses. These achievements owe something very important to Dr. Freeman: Freeman was the mentor who encouraged the gifted Dr. Carson to take on the challenge of applying his exceptional surgical skills to the care of patients for whom such surgeries were quite often to prove remarkably effective. Dr. Freeman invested a similar degree of time and interest in the comparatively mundane subject of identification and management of febrile seizures. Freeman was among a considerable number of child neurologists who have regarded treatment of one or two febrile seizures with varying degrees of skepticism, given the usual benignity of outcome and the fact that the safest and most effective drug for preventive treatment, phenobarbital, has sedative and sometimes other side-effects. Few, however, have opposed treatment so unequivocally and outspokenly than Dr. Freeman. He demonstrated a similarly unyielding stance against obtaining an EEG in otherwise normal children after a single seizure that has produced no evidence of post-seizure neurological consequences. He turned a fresh and critical eye on the important topics related to the definition and management of status epilepticus in children. He has devoted attention to the ways in which imaging and EEG studies may be used and what he regarded as the too frequent occasions on which these tests were abused or misinterpreted.

With the epilepsies as with all the other diseases upon which he became expert he concerned himself with psychomotor, psychological and psychiatric aspects of these conditions, and on the dangers that such activities as swimming and others posed for persons with epilepsy. These papers are also quite highly cited and influential. The interest of Dr. Freeman and his group have perpetually extended themselves to all virtually aspects of the epilepsy, particularly those aspects that have proven controversial. Additional topics include febrile seizures, intelligent prospective analyses of timing and outcome of discontinuation of antiseizure medications or the ketogenic diet, assessment of the value of cofactor administration, school based assessment and management of learning and other problems, and returning to the interest in the behavioral and psychiatric aspects of disease, the psychiatric and behavioral aspects of the utilization of drugs and other treatments for epilepsy. As if this were not enough, a dozen papers, also with Ben Carson and others, consider all pertinent aspects of the evaluation and treatment of various congenital craniospinal abnormalities, including management of various complications and ethical considerations. True to his early interest in the neonate, seventeen papers consider the pathogenesis, treatment, and outcome of neonatal seizures. Ten consider recognition and management of heritable metabolic diseases. Among these is his fine paper concerning the efficacy of folate treatment for homocystinuria, published in the New England Journal of Medicine and cited 174 times to date. This paper also demonstrates Freeman’s thoroughness in that he was among those who first recognized the autistic features of this and some other metabolic diseases.

A number of papers consider the infectious and inflammatory processes that produce epilepsy, particularly seven important papers concerning SSPE and its relationship to measles virus. Other papers range over a widespread intellectual territory including degenerative neurological illnesses, brain trauma, hemolytic-uremic syndrome, and brain death criteria, whistle-blowing on physician incompetence, informed consent, HIV, end-of life management, and such additional topics as have engaged the active mind of this iconoclastic physician-scientist. Many papers consider or mention clinical signs of importance found on examination of children with neurological diseases. It is not surprising that they reflect Dr. Freeman’s powers of observation and elicitation: he is well known among his trainees and colleagues as a person who, having (as is said to be characteristic of the child neurologist) approached a child on his knees, quickly engages the child’s playful trust and undoubted sense that they are meeting a physician who loves children and cares greatly about their welfare. Dr. Freeman manifested the characteristic abilities of the child neurologist to engage children with his eyes, smile, gradual and non-threatening approach. He has also taught those who have had the privilege to come into contact with him the importance of simple observation of children at play—a favorite technique of predecessors who were similarly keenly observant, such as Frank Ford and David Clark. Freeman also resembled these famous predecessors in his hesitation to tucker out or irritate children with a lengthy formal neurological examination. Generations of neurology residents at Hopkins observed his famous technique of the “three cent neurological exam.” Dr. Freeman received the Johns Hopkins’ Frank Ford Teaching Award in Neurology in 1983. His wealth of knowledge, his insights, and his humane perspective imparted widely in professional meetings, courses, and visiting lectureships and honorary fellowships. Among the most notable of a very long list are those at the Royal Society of Medicine, London, the Montreal Neurologic Institute, Guy’s Hospital, the Peruvian Pediatric Society, the John Stobo Pritchard Lectureship, the McKeith Lectureship, and the Neuropediatric Society of Vienna.

In the entire corpus of his work as physician, scientist, and educator, Dr. Freeman placed considerable emphasis on both the ethics of clinical investigation and of treatment decisions. Fifteen papers are primarily concerned with ethical aspects not only of medical practice, but of the participation of physicians as medical experts in malpractice cases. He enjoyed taking a provocative tack on such subjects, including his published suggestion that courtroom outcomes may be something of a “lottery” rather than a scientific engagement. He painfully recognized that there were errors in the practice of medicine, upon the nature of which from his point of view he was not shy about pointing out. However he was also aware that there were many areas of practice that continued to be governed by opinion and the necessity of making judgments the foundation of which remained uncertain. Thus he was able to find fault with both sides of expert testimony in medicolegal cases. This caused him at times to suggest consideration of a form of “no fault insurance” that would cover such costs as were incurred as the result of medical errors. But this advocacy, perhaps at least in part “tongue-in-cheek,” was not the result of his caring the less about the effects on children and their families of their diseases or of the added difficulties generated by their medical management.

It is quite clear that his worries about such issues—many of which he was more than willing to debate—left him constantly willing to improve with carefully designed and executed studies the foundations upon which judgment could be based. This commitment was joined together with his earnest and unrelenting desire to instill judgment in those he taught together with an appreciation of the limitations of science and practice and the unremitting desire to make both of these foundations for medicine better than he had found them. He has considered with similar sensitivity and practicality ethical issues concerning treatment of the severely ill newborn, difficult questions of right to die and treatment decisions that may alter manner of death, and issues related to the sterilization of women with epilepsy. It is not surprising that Dr. Freeman was the founder and first Chair of the Johns Hopkins Ethics Committee and chair of the bioethics search committee. He was coauthor of the challenging book, Tough Decisions: A Casebook in Medical Ethics, a resource of interest and importance to every practitioner of our complex field of endeavor. Dr. Freeman, throughout his long career, showed particular concern for the dignity and especially the self-esteem of the individual patient. He had consistent commitment to the importance of rendering the life of each individual patient as independent and fulfilling–-as close to “normal” as possible. Among the numerous examples of his attention to the details that were involved in achieving these ideals was the attention he devoted, together with Ben Carson and other surgeons, to improving treatment of fecal incontinence and other management problems of children with myelomeningoceles. His numerous insights into the problems associated with this medically and psychologically complex congenital abnormality were assembled into his valuable book, Practical Management of Myelomeningocele. Dr. Freeman considered from a practical and constructive point of view the vexing questions of etiologies of cerebral palsies and their relationship to birth events, as well as the related questions of medical costs, public policy, and law.

Dr. Freeman remained throughout his career a major collaborator with Karin Nelson and others on various aspects of the NINCD sponsored research of the National Collaborative Perinatal Project. He has participated in the effort to refine the definition of birth asphyxia and to the identification of the role that prenatal or neonatal seizures and their treatment might play in injury to developing brain. He was similarly concerned with other ways in which improvement of the management of epilepsies might be tailored to better suit inner city life, educational adaptation, and successful pregnancy. His concern for education of his patients and the possibility that impaired individuals might attain the dignity of some occupation were not necessarily limited to vocational training, but where possible might extend to the collegiate level. He has played an important role in reviewing and improving approaches to the “insurability” of individuals with neurologic disorders. He has closely investigated the exceedingly important questions of patient perceptions of treatment and outcome, which often remain un-assayed end-points of therapeutic efficacy. The considerable emphasis that he has placed on education of patients and family caused him to write his well-known book, Seizures and Epilepsy in Children: A Guide for Parents, which received a National Book Award.

Dr. Freeman’s service included participation in many local, national and international pediatrics and neurological associations and their committees. Notably, he has served on the executive committees of the CNS, Child Neurology Section of the AAP (of which he was at one time chair), PCN, and ICNA. He has served on the ethics committee of the ANA, as President of PCN, and as Vice President and Honorary Lifetime Director of the Epilepsy Foundation of America. He has served on the Professional Advisory Boards of the Spina Bifida Association and the Sturge-Weber Foundation of America. His numerous responsible positions and committee service at Johns Hopkins have included service as Founder and First Chair of the Johns Hopkins Hospital Ethics Committee and Co-Directorship of an undergraduate course in Bioethics. In honor of his outstanding contributions to many aspects of the epilepsies, Dr. Freeman received many awards, the most prestigious of which include the J. Kiffin Penry Award for Epilepsy Care, the William G. Lennox Award of the American Epilepsy Society, and the Lifetime Achievement Award of the Epilepsy Foundation of America. His readiness to generously volunteer time and resources for the wide range of public causes that he regarded as worthwhile was recognized by his designation as Maryland Health Care Professional of the Year, by his receipt of the JC Penney Golden Rule Award and quite a number of other awards for leadership and commitment in local schools, community, and state. Very appropriately, the Johns Hopkins Pediatric Epilepsy Center founded by Dr. Freeman has been named for Dr. Freeman.

Dr. Freeman has sustained an inimitable career wherein he has played the role of gadfly, critic, and skeptic, the asker of a seemingly endless supply of well-formulated questions. Dr. Freeman recognized his contentiousness as a virtuous attribute, justifying it with a quotation from Gustav Havel’s work, Disturbing the Peace:

The intellectual should constantly disturb, should bear witness to the misery of the world, should be provocative by being independent, should be against all hidden and open pressures and manipulations, should be the chief doubter of systems, of power, and its incantations, and should be a witness to their mendacity. For this very reason, an intellectual cannot fit into any role that might be assigned to him. An intellectual doesn’t belong anywhere; he stands out as an irritant wherever he is: he does not fit into any pigeonhole completely.The intellectual remains, despite all his defeats, undefeated – again, like Sisyphys. He is, in fact, victorious through his defeats.

A number of devoted trainees have spent their ensuing and highly successful careers merely trying to answer the myriad questions posed by this skeptical mentor. A lengthy list of his devoted trainees would considerably enlarge this career summary—though certainly the name of Dr. Patty Vining should be mentioned. She has been Dr. Freeman’s remarkable long-term collaborator in all aspects of patient evaluation, care, analysis and publication of results, as well as in the education of child neurologists. Equally committed to the care and welfare of children, she co-authored, with Dr. Freeman and Diana Pillas in 1990 the excellent book Epilepsy in Childhood: A Guide that, through a number of editions, has proven to be extraordinarily empowering and reassuring to the parents of children with epilepsy.

There are many ways to be a gifted and important physician, medical scientist, a teacher of medicine. A substantial foundation for these roles is certainly helpful. Powers of observation, a sense of timing, excellent judgment, availability, and commitment are also of course important. The carrying out of such roles is often provocative of mixed opinions, some of which are more informed than others. Few individuals garner universal praise in any of these occupations, perhaps especially because medicine is an occupation that is very often suffused with uncertainties, anxieties, controversies, disappointments, surprises, and, even for the best of us, errors. Some of us venture forth more bravely than others in challenging some of the necessary approximations that we employ in trying, as most of us do, to do the right thing. We vary in the degree to which we expose ourselves to situations that involve anxiety or doubt. Most of us are pretty quiet as we diligently get on with doing the right thing. But some of us are so committed to getting things right and doing so upon such a grand scale that we can be unsettling—the sort of person, to use the word Socrates used about himself, that is a “gadfly.” John Freeman was such a person—an extraordinary one whose intellect and values were quite remarkable. He generated controversies, but none so substantial as those that he resolved.

Dr. Freeman is survived by his devoted wife, Elaine Kaplan Freeman, who played a constant and critical role in Dr. Freeman’s life including the many charitable activities and commitments to which both were devoted, including the Freeman Family Fund in Clinical Bioethics. He is also survived by his three children: Andrew D. Freeman, Jennifer Freeman Stevenson, and Joshua L. Freeman.