Unforgettable patients Morris Green, MD
Over the years, clinicians accumulate an extensive memory bank of patients who for one reason or another are unforgettable. Because the care of patients is intensely personal, many remembrances of shared experiences are warm, gratifying, and often inspiring, but occasionally they are saddening and poignant. Some of these experiences have an affective quality that is difficult to communicate; others represent naturalistic observations that, when recorded in the literature, add to the cumulative knowledge base of pediatrics. Although all these experiences are instructive in their own way, the following small sample of my "unforgettable patients" is selected from the latter category--two from the biomedical and two from the developmental and behavioral aspects of child health. PATIENT
1
In 1953 a 2-year-old boy was admitted to the New Haven Hospital because of a swelling of the left cheek that had begun a few hours earlier. On the day before admission, he became irritable and had a temperature of 40.4 ~ C. On admission, he appeared acutely but moderately ill. A hot, tender, dusky, bluish red discoloration, which resembled a fading hematoma, extended superiorly to the ridge of the maxilla, inferiorly to the border of the mandible, posteriorly to the parotid gland, and anteriorly to the corner of the mouth. A 4 cm central area of induration was surrounded by a soft, edematous zone, the border of which was neither elevated nor sharply demarcated. The ipsilateral buccal mucosa was slightly edematous. Laboratory data included a leukocyte count of 25,000 cells/mm3. The initial diagnostic impression was cellulitis of unknown cause, and we were surprised when the blood culture was positive for Haernophilus influenzae type b. During therapy with chloramDr. Green is the Perry W. Lesh Professor of Pediatrics, Department of Pediatrics, Indiana University School of Medicine. From 1967 to 1988, he was Chairman of the Department of Pediatrics and Physician-in-Chief of the James Whitcomb Riley Hospital for Children in Indianapolis. He is a general pediatrician whose chief interests include the developmentaland psychobiologicaspects of child health, and he is the author of Pediatric Diagnosis, now in its fifth edition and coeditor with Robert J. Haggerty of Ambulatory Pediatrics, now in its fourth edition. 9/32/35222
phenicol, the temperature returned to normal within 48 hours, and the swelling diminished rapidly on the third day. About 10 weeks later, a 9-month-old white male infant was admitted with a strikingly similar history and physical examination findings. Later on the day of admission, his right knee became swollen and was held in flexion. Because the purplish red swelling of the cheek reminded us of the child described above, a provisional diagnosis of H. influenzae type b cellulitis was made and therapy with chloramphenicol was begun. Cultures of nasal and blood specimens grew H. influenzae type b. In a review of these clinical and laboratory findings with Mildred Fousek, an experienced bacteriologist in the department of pediatrics at Yale, she was able to identify in her records four other children who had been seen during a 15-year period with similarly associated findings. Three had had cellulitis of the face or eyelid, and one of the left arm. 1 Although I initially thought that the purplish red cellulitis of the cheek or eyelid was specifically pathognomonic of infection with H. influenzae type b, others later reported that Streptococcus pneurnoniae and group B streptococci could cause a similar clinical presentation. PATIENT
2
On ward rounds one morning in 1957 at the Riley Hospital for Children, the residents presented a child who immediately brought to mind a previous experience with two patients seen at the New Haven Hospital with Bob Cooke and Bill Lattanzi. A 27-month-old boy had been admitted because of chronic diarrhea, abdominal distention, and a mass in the chest. The child had passed seven or eight brown, watery, foul-smelling stools daily for the previous 2 months. During a recent hospitalization elsewhere, a chest roentgenogram revealed an abnormal density in the mediastinum. The child's physical appearance suggested the diagnosis of cystic fibrosis or celiac disease. He looked chronically ill, with pallor, loss of subcutaneous fat, extreme irritability, drenching perspiration, a frequent cough, and marked abdominal distention. Height and weight were at the 3rd percentile. The blood pressure was 130/70 mm Hg. Roentgenograms of the chest revealed a triangular posterior medias-
659
660
Green
tinal density that extended from the third to the ninth ribs on the right. Generalized distention of intestinal loops was noted on a plain roentgenogram of the abdomen. Surgical exploration revealed a tumor arising from a pedicle in the region of the fifth interspace. Because the tumor was adherent to the intercostal muscles, periosteum of the ribs, and intercostal bundles, it could not be completely removed. The histopathologic diagnosis was ganglioneuroblastoma. After surgery, the diarrhea, excessive perspiration, and irritability stopped immediately, the abdominal distention diminished strikingly, the blood pressure became normal, and the child's appetite improved dramatically. The two patients who had previously had findings similar to those in this child were seen at the New Haven Hospital in 1952 and 1955. Both had chronic diarrhea characterized by the passage of foul-smelling, frequent, frothy, and loose watery or greasy stools. They had poor appetite, signs of malnutrition, and marked abdominal distention. Profuse sweating and hypertension were present in one child. These symptoms disappeared in each child after resection of a ganglioneuroma. The prompt improvement in each of these three patients after surgical removal of the tumors suggested that their ganglioneuroma or ganglioneuroblastoma functioned to secrete a neurohumoral substance. The secretion of vasoactive intestinal peptide by some neural crest tumors was subsequently reported by other investigators. 2-4 PATIENT
3
In 1957, I had the opportunity to establish a pediatric diagnostic clinic to which family physicians and pediatricians could refer patients for consultation and suggestions for management. After taking the history and performing the physical examination, the residents and medical students would then present their patients to me in a group meeting attended by the other residents and students. I would then interview the parent or child or both, and check physical findings as appropriate. One morning early in this experience, the resident presented the history of a 17-month-old boy referred to us because of severe temper tantrums and "out of control" behavior. The child was reported by his mother to be disobedient, irritable, and uncooperative and to have a sleep problem, awakening several times a night. She also worried about the child's "poor" color and said that he was "sick all the time." The mother came into the conference room, struggling with the child, who was crying and protesting loudly. Taking a deep breath, she dropped into her chair. Although she attempted to hold the child on her lap, he soon began squirming and then kicked the mother in her shin with the
The Journal of Pediatrics October 1992
heel of his shoe. Although embarrassed by this behavior, she was unable to correct the child. I noted that she looked exhausted and despairing. In attempting to understand the genesis of these persistent symptoms, I asked the mother to tell me about her pregnancy and labor. There had been a history of two previous miscarriages, but this pregnancy had been "normal." Shortly after the child's birth, however, respiratory distress developed. When I asked whether he had been seriously ill then, she immediately became tearful as she recounted having been told by the baby's physicians that he would likely die. When I asked her to "tell me about it," she recalled--as if had happened only yesterday--exactly what the physicians had said, the nurses' reports, her deep sense of foreboding, and her symptoms of anticipatory grief. Even recalling this experience, accompanied by considerable feeling, seemed very painful. When I asked whether she still worried that she would lose him, although he had recovered fully from that illness, she seemed surprised that I had guessed her secret worry--one that she had not previously disclosed to anyone. The mother had not previously made an association between this history of a life-threatening illness, her continuing and recurrent fear that her son would die prematurely, and the child's persistent symptoms and behavior. Through the process of assuring the mother that the child was now physically healthy and helping her to understand the etiologic relationship between her natural reaction to the acute, life-threatening illness and the child's current behavior, she was enabled to set limits, lessen her overprotectiveness, and be less concerned about his health. After this experience, A1 Solnit in New Haven and I in Indianapolis were able to identify and characterize the symptoms in a large number of similarly vulnerable children. These initial observations have now been multiplied many times), 6 PATIENT
4
On another morning in the pediatric diagnostic clinic, I was presented with the history of a 10-month-old boy referred to us because of his mother's worry that he was retarded. His development was characterized by delay in sitting up, failure to use his hands to pick up objects, and deficient vocalization. The resident was uncertain of the cause of the baby's obvious developmental delay. When the mother and the baby were brought into the room, I was immediately struck by her depressed affect. In addition to looking very sad and older than her chronologic age, she sighed occasionally, kept her gaze downward, and did not interact playfully with the baby sitting on her lap. The baby kept his upper extremities flexed and his hands held close to his chest at shoulder level. When I offered him
Volume 121 Number 4
a cube from the developmental assessment kit, he did not initially reach out and grab it, as would be expected at his age. With encouragement, however, he finally tentatively reached out and gingerly touched the cube. This "hot cube" behavior, originally described by Gesell and Amatruda, had been reported by Provence and Lipton7 in some institutionalized infants. This infant, however, had been reared in a family, not an institution. My initial clinical impressions were that the mother had a major depressive illness and that the infant's developmental lag was a result of the effects of her illness on the mother-infant interaction. In reviewing the history since the infant's birth, I learned from the mother that 2 or 3 months after the baby's birth, her husband had been killed in an accident at work. As the mother tearfully told of her continuing sadness, fatigue, irritability, loneliness, and insomnia, it was clear that her unresolved grief accounted for her depression. Although she had previously been seen by other physicians, her depression had apparently not been identified, nor had psychiatric treatment been advised. When I commented that mothers of young children need all the energy they can muster and asked whether she had thought about obtaining some help for herself as well as for her baby, she nodded affirmatively. She seemed suddenly relieved that someone recognized what she had been going through, and although she had not been. able to mobilize herself to seek help, there was suddenly some light at the end of the tunnel. An appointment with a psychiatrist was urgently arranged. A few weeks later, she excitedly called to report that the baby was now jabbering more, was smiling, and had actually picked up a piece of toast and put it in his mouth.
Unforgettable patients
66 1
As a result of this experience, I became more alert to the possibility of maternal depression and its effects on the infant and the young child. It was also clear that the diagnosis usually has to be suspected by the pediatrician on the basis of observation of the infant's behavior, because the mother will not spontaneously complain of depression to the pediatrician. Some of these early clinical observations were presented at a 1965 National Institute of Child Health and Human Development conference entitled "Conceptual and Methodological Approaches to Research in Pediatric Outpatient Departments," and a large subsequent experience added to my knowledge of the manifestations in infants and children of depression in their mothers. 8 These instances are not uncommon. REFERENCES
1. Green M, Fousek MD. Hemophilus influenzae type b cellulitis. Pediatrics 1957;19:80-3. 2. Green M, Cooke RE, Lattanzi W. Occurrence of chronic diarrhea in three patients with ganglionearomas. Pediatrics 1959;23:95l-5. 3. Kaplan SJ, Holbrook CT, McDaniel HG, Buntain WL, Crist WM. Vasoactive intestinal peptide secreting tumors of children. Am J Dis Child 1980;134:21-4. 4. Vorhess ML. Functioning tumors. Am J Dis Child 1980; 134-14-5. 5. Green M, Solnit AJ. Reactions to the threatened lossof a child: a vulnerable child syndrome. Pediatrics 1964;34:58-66. 6. Green M. Vulnerable child syndrome and its variants. Pediatr Rev 1986;8:75-80. 7. ProvenceS, Lipton RC. Infants in institutions. New York: International Universities Press, 1964. 8. Green M. Maternal depression. In: Green M. Pediatric diagnosis. 5th ed. Philadelphia: WB Saunders, 1992:440-1.
Over the years, clinicians accumulate an extensive memory bank of patients who for one reason or another are unforgettable. Because the care of patients is intensely personal, many remembrances of shared experiences are warm, gratifying, and often inspiring, but occasionally they are saddening and poignant. Some of these experiences have an affective quality that is difficult to communicate; others represent naturalistic observations that, when recorded in the literature, add to the cumulative knowledge base of pediatrics. Although all these experiences are instructive in their own way, the following small sample of my "unforgettable patients" is selected from the latter category--two from the biomedical and two from the developmental and behavioral aspects of child health. PATIENT
1
In 1953 a 2-year-old boy was admitted to the New Haven Hospital because of a swelling of the left cheek that had begun a few hours earlier. On the day before admission, he became irritable and had a temperature of 40.4 ~ C. On admission, he appeared acutely but moderately ill. A hot, tender, dusky, bluish red discoloration, which resembled a fading hematoma, extended superiorly to the ridge of the maxilla, inferiorly to the border of the mandible, posteriorly to the parotid gland, and anteriorly to the corner of the mouth. A 4 cm central area of induration was surrounded by a soft, edematous zone, the border of which was neither elevated nor sharply demarcated. The ipsilateral buccal mucosa was slightly edematous. Laboratory data included a leukocyte count of 25,000 cells/mm3. The initial diagnostic impression was cellulitis of unknown cause, and we were surprised when the blood culture was positive for Haernophilus influenzae type b. During therapy with chloramDr. Green is the Perry W. Lesh Professor of Pediatrics, Department of Pediatrics, Indiana University School of Medicine. From 1967 to 1988, he was Chairman of the Department of Pediatrics and Physician-in-Chief of the James Whitcomb Riley Hospital for Children in Indianapolis. He is a general pediatrician whose chief interests include the developmentaland psychobiologicaspects of child health, and he is the author of Pediatric Diagnosis, now in its fifth edition and coeditor with Robert J. Haggerty of Ambulatory Pediatrics, now in its fourth edition. 9/32/35222
phenicol, the temperature returned to normal within 48 hours, and the swelling diminished rapidly on the third day. About 10 weeks later, a 9-month-old white male infant was admitted with a strikingly similar history and physical examination findings. Later on the day of admission, his right knee became swollen and was held in flexion. Because the purplish red swelling of the cheek reminded us of the child described above, a provisional diagnosis of H. influenzae type b cellulitis was made and therapy with chloramphenicol was begun. Cultures of nasal and blood specimens grew H. influenzae type b. In a review of these clinical and laboratory findings with Mildred Fousek, an experienced bacteriologist in the department of pediatrics at Yale, she was able to identify in her records four other children who had been seen during a 15-year period with similarly associated findings. Three had had cellulitis of the face or eyelid, and one of the left arm. 1 Although I initially thought that the purplish red cellulitis of the cheek or eyelid was specifically pathognomonic of infection with H. influenzae type b, others later reported that Streptococcus pneurnoniae and group B streptococci could cause a similar clinical presentation. PATIENT
2
On ward rounds one morning in 1957 at the Riley Hospital for Children, the residents presented a child who immediately brought to mind a previous experience with two patients seen at the New Haven Hospital with Bob Cooke and Bill Lattanzi. A 27-month-old boy had been admitted because of chronic diarrhea, abdominal distention, and a mass in the chest. The child had passed seven or eight brown, watery, foul-smelling stools daily for the previous 2 months. During a recent hospitalization elsewhere, a chest roentgenogram revealed an abnormal density in the mediastinum. The child's physical appearance suggested the diagnosis of cystic fibrosis or celiac disease. He looked chronically ill, with pallor, loss of subcutaneous fat, extreme irritability, drenching perspiration, a frequent cough, and marked abdominal distention. Height and weight were at the 3rd percentile. The blood pressure was 130/70 mm Hg. Roentgenograms of the chest revealed a triangular posterior medias-
659
660
Green
tinal density that extended from the third to the ninth ribs on the right. Generalized distention of intestinal loops was noted on a plain roentgenogram of the abdomen. Surgical exploration revealed a tumor arising from a pedicle in the region of the fifth interspace. Because the tumor was adherent to the intercostal muscles, periosteum of the ribs, and intercostal bundles, it could not be completely removed. The histopathologic diagnosis was ganglioneuroblastoma. After surgery, the diarrhea, excessive perspiration, and irritability stopped immediately, the abdominal distention diminished strikingly, the blood pressure became normal, and the child's appetite improved dramatically. The two patients who had previously had findings similar to those in this child were seen at the New Haven Hospital in 1952 and 1955. Both had chronic diarrhea characterized by the passage of foul-smelling, frequent, frothy, and loose watery or greasy stools. They had poor appetite, signs of malnutrition, and marked abdominal distention. Profuse sweating and hypertension were present in one child. These symptoms disappeared in each child after resection of a ganglioneuroma. The prompt improvement in each of these three patients after surgical removal of the tumors suggested that their ganglioneuroma or ganglioneuroblastoma functioned to secrete a neurohumoral substance. The secretion of vasoactive intestinal peptide by some neural crest tumors was subsequently reported by other investigators. 2-4 PATIENT
3
In 1957, I had the opportunity to establish a pediatric diagnostic clinic to which family physicians and pediatricians could refer patients for consultation and suggestions for management. After taking the history and performing the physical examination, the residents and medical students would then present their patients to me in a group meeting attended by the other residents and students. I would then interview the parent or child or both, and check physical findings as appropriate. One morning early in this experience, the resident presented the history of a 17-month-old boy referred to us because of severe temper tantrums and "out of control" behavior. The child was reported by his mother to be disobedient, irritable, and uncooperative and to have a sleep problem, awakening several times a night. She also worried about the child's "poor" color and said that he was "sick all the time." The mother came into the conference room, struggling with the child, who was crying and protesting loudly. Taking a deep breath, she dropped into her chair. Although she attempted to hold the child on her lap, he soon began squirming and then kicked the mother in her shin with the
The Journal of Pediatrics October 1992
heel of his shoe. Although embarrassed by this behavior, she was unable to correct the child. I noted that she looked exhausted and despairing. In attempting to understand the genesis of these persistent symptoms, I asked the mother to tell me about her pregnancy and labor. There had been a history of two previous miscarriages, but this pregnancy had been "normal." Shortly after the child's birth, however, respiratory distress developed. When I asked whether he had been seriously ill then, she immediately became tearful as she recounted having been told by the baby's physicians that he would likely die. When I asked her to "tell me about it," she recalled--as if had happened only yesterday--exactly what the physicians had said, the nurses' reports, her deep sense of foreboding, and her symptoms of anticipatory grief. Even recalling this experience, accompanied by considerable feeling, seemed very painful. When I asked whether she still worried that she would lose him, although he had recovered fully from that illness, she seemed surprised that I had guessed her secret worry--one that she had not previously disclosed to anyone. The mother had not previously made an association between this history of a life-threatening illness, her continuing and recurrent fear that her son would die prematurely, and the child's persistent symptoms and behavior. Through the process of assuring the mother that the child was now physically healthy and helping her to understand the etiologic relationship between her natural reaction to the acute, life-threatening illness and the child's current behavior, she was enabled to set limits, lessen her overprotectiveness, and be less concerned about his health. After this experience, A1 Solnit in New Haven and I in Indianapolis were able to identify and characterize the symptoms in a large number of similarly vulnerable children. These initial observations have now been multiplied many times), 6 PATIENT
4
On another morning in the pediatric diagnostic clinic, I was presented with the history of a 10-month-old boy referred to us because of his mother's worry that he was retarded. His development was characterized by delay in sitting up, failure to use his hands to pick up objects, and deficient vocalization. The resident was uncertain of the cause of the baby's obvious developmental delay. When the mother and the baby were brought into the room, I was immediately struck by her depressed affect. In addition to looking very sad and older than her chronologic age, she sighed occasionally, kept her gaze downward, and did not interact playfully with the baby sitting on her lap. The baby kept his upper extremities flexed and his hands held close to his chest at shoulder level. When I offered him
Volume 121 Number 4
a cube from the developmental assessment kit, he did not initially reach out and grab it, as would be expected at his age. With encouragement, however, he finally tentatively reached out and gingerly touched the cube. This "hot cube" behavior, originally described by Gesell and Amatruda, had been reported by Provence and Lipton7 in some institutionalized infants. This infant, however, had been reared in a family, not an institution. My initial clinical impressions were that the mother had a major depressive illness and that the infant's developmental lag was a result of the effects of her illness on the mother-infant interaction. In reviewing the history since the infant's birth, I learned from the mother that 2 or 3 months after the baby's birth, her husband had been killed in an accident at work. As the mother tearfully told of her continuing sadness, fatigue, irritability, loneliness, and insomnia, it was clear that her unresolved grief accounted for her depression. Although she had previously been seen by other physicians, her depression had apparently not been identified, nor had psychiatric treatment been advised. When I commented that mothers of young children need all the energy they can muster and asked whether she had thought about obtaining some help for herself as well as for her baby, she nodded affirmatively. She seemed suddenly relieved that someone recognized what she had been going through, and although she had not been. able to mobilize herself to seek help, there was suddenly some light at the end of the tunnel. An appointment with a psychiatrist was urgently arranged. A few weeks later, she excitedly called to report that the baby was now jabbering more, was smiling, and had actually picked up a piece of toast and put it in his mouth.
Unforgettable patients
66 1
As a result of this experience, I became more alert to the possibility of maternal depression and its effects on the infant and the young child. It was also clear that the diagnosis usually has to be suspected by the pediatrician on the basis of observation of the infant's behavior, because the mother will not spontaneously complain of depression to the pediatrician. Some of these early clinical observations were presented at a 1965 National Institute of Child Health and Human Development conference entitled "Conceptual and Methodological Approaches to Research in Pediatric Outpatient Departments," and a large subsequent experience added to my knowledge of the manifestations in infants and children of depression in their mothers. 8 These instances are not uncommon. REFERENCES
1. Green M, Fousek MD. Hemophilus influenzae type b cellulitis. Pediatrics 1957;19:80-3. 2. Green M, Cooke RE, Lattanzi W. Occurrence of chronic diarrhea in three patients with ganglionearomas. Pediatrics 1959;23:95l-5. 3. Kaplan SJ, Holbrook CT, McDaniel HG, Buntain WL, Crist WM. Vasoactive intestinal peptide secreting tumors of children. Am J Dis Child 1980;134:21-4. 4. Vorhess ML. Functioning tumors. Am J Dis Child 1980; 134-14-5. 5. Green M, Solnit AJ. Reactions to the threatened lossof a child: a vulnerable child syndrome. Pediatrics 1964;34:58-66. 6. Green M. Vulnerable child syndrome and its variants. Pediatr Rev 1986;8:75-80. 7. ProvenceS, Lipton RC. Infants in institutions. New York: International Universities Press, 1964. 8. Green M. Maternal depression. In: Green M. Pediatric diagnosis. 5th ed. Philadelphia: WB Saunders, 1992:440-1.
