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Hospice care for children
THE LANCET
Hospice care for children SIR—We report two siblings with spinal muscular atrophy type 1 (SMA1). One is a 4-year-old girl now under a respirator management, and the other was her younger brother for whom we decided on a selective use of therapy to deal with his respiratory failure at the age of 4 months. Their parents had been informed about the genetic trait of this disorder before the second pregnancy and decided to proceed after careful consideration. Although the second baby was born with mild asphyxia, he developed well thereafter until the gradual appearance of generalised hypotonia and respiratory insufficiency at around age 3 months. After discussion with the parents about the suitability of management with mechanical ventilation we decided against respiratory care, instead choosing selective use of therapy. The baby died within a week from respiratory failure which was precipitated by acute bronchitis. Through this experience we noted three problems that face paediatricians in treating seriously handicapped children such as our patient. First, there is disagreement among doctors about whether or not to initiate ventilator care for infants with SMA1. In most western countries, vigorous lifesupporting measures are withheld from such babies. On the other hand, many Japanese paediatricians endeavour to resuscitate and prolong the life of such infants. In Japan, many patients with neuromuscular diseases, including SMA1, have been ventilated since infancy, when they were not able to express their own will. In view of the ethical principle of autonomy, this has raised the difficult question of whether or not to ventilate patients who develop respiratory failure early in life.1,2 Sakakihara and colleagues3 pointed out that this disparity between Japan and western countries is related not only to medical systems, but also to the cultural, religious, and historical backgrounds. Second, if we cannot help in deciding on selective non-treatment for these children, it is essential that we physicians should recognise and understand the “death of the second person” principle as defined by Yanagida (ie, the death of a child, parent, sibling, wife, husband, or sweetheart, and the accompanying painful ordeal);4 in paediatrics, we should have deep sympathy for the unbearable emotions that parents have at the death of their children. However, paediatricians in Japan are not used to discussing these kinds of issues. Third, at the moment of a child’s death, enough time and a quiet place for parents to grieve should be provided where such seriously ill children are being cared for. Most of the paediatric departments in Japan do not have such facilities. At present, the requirements of hospice care for aged patients with fatal disorders have been understood and recognised all over the world, including Japan. However, hospice care for children with incurable illnesses has not been generally accepted, let alone properly provided in Japan. Terminally ill children can undergo both extreme mental and physical suffering, which must be eased at their last moments, as too must the parents’. In the neonatal care unit at the Children’s Hospital in Denver in the USA, for example, a nearby hospice care system for severely handicapped newborn babies has been set up.5 Japanese paediatricians want to listen to opinions from doctors in western countries about how they try solve and manage such problems. We would like to solicit opinions and comments on this from your readers. *Shuji Wakai, Nozomi Ito, Noriaki Adachi, Daisuke Ueda, Hiroyuki Tsutsumi, Shunzo Chiba
2
3
4 5
Dysgeusia (“cognate, dis-gusting”) SIR—In January, 1989, I developed a right brachial plexus neuropathy, the cause of which remained obscure until a strongly positive Borrelia burgdorferi blastogenesis stimulation index was found.1 I was given intravenous ceftriaxone and, after recovering from a Herxheimer reaction, resumed my medical duties.2 One morning in October, 1989, I awoke with a foul taste, and assumed that a crowned molar was the source. My dentist assured me that all my teeth were in good condition. The foul taste persisted and eventually I returned to the dentist, insisting that something was “rotting” in my mouth, and with a request that he remove the crown. He was confident the crown was not the source and urged me to delay, which I did. During persistence of the condition the inside of my mouth, including the gingiva and teeth, lacked sensation. The taste was often so bad that I was unable to eat, and avoided standing close to others so as to spare them the offensive odour. In December, 7 months after my treatment for Lyme disease, I returned for neurological consultation. Until the occurrence of dysgeusia, the manifestations had been limited to the brachial neuropathy, with atrophy and weakness of my shoulder-girdle muscles, forearm, and hand.2 I described for the neurologist my terrible taste during the preceding 2 months and he found my gustation to be severely impaired from bilateral dysfunction of the sensory branches of the seventh nerve, a neuropathy presumed caused by borreliosis. I then learned that loss of taste (ageusia) can be associated with acquiring a disgusting taste (dysgeusia). I had been taught that, apart from its motor function, the seventh nerve was responsible for discriminatory taste on the anterior two-thirds of the tongue. Textbooks of neurology did not mention that, along with diminished taste, there may also be a foul taste. A search for case reports was of little help: no one had apparently recorded the symptoms of dysgeusia, other than noting that, along with the sensory loss, there may be a metallic taste. Was it known that dysgeusia can be a distressing complaint, especially when it continues for months? This symptom was far more disturbing than my arm weakness, to which I had adjusted by using other muscles. The perverted taste ranged from awful to repulsive. After 3 years, the vile sensation finally waned (although it has recurred, briefly, several times) but lack of discriminatory taste has persisted. Foregoing the pleasure of lingering over a tasty meal was sad in itself, but after years of dysgeusia it seemed a minor loss. Oh, if only it was merely like the textbooks wrote, “lack of taste”! Christine Lawrence Albert Einstein College of Medicine of Yeshiva University, New York, NY 10461, USA
1
Department of Paediatrics, Sapporo Medical University School of Medicine, Sapporo 060, Japan
1
Lanken P. ATS position statement on withholding and withdrawing life sustaining therapy. Ann Intern Med 1992; 115: 478–85.
1102
Bone RC, Rackow EC, Weg JG, et al. Ethical and moral guidelines for the initiation, continuation, and withdrawal of intensive care. Chest 1990; 97: 949–58. Sakakihara Y, Yamanaka T, Kajii M, et al. Long-term ventilatorassisted children in Japan: a national survey. Acta Paediatr Jap 1996; 38: 137–42. Yanagida K. Sacrifice. Tokyo: Bungei-Shunjyuu, 1995: 203 (in Japanese). Weir RF. Selective nontreatment of handicapped newborns: moral dilemmas in neonatal medicine. New York: Oxford University Press, 1984: 273.
2
Dattwyler RJ, Volkman DJ, Luft BJ, Halperin JJ, Thomas J, Golightly MG. Seronegative Lyme disease: dissociation of the specific T- and B-lymphocyte responses to Borrelia burgdorferi. N Engl J Med 1988; 319: 1441–46. Lawrence C, Lipton RB, Lowy FD, Coyle PK. Seronegative chronic relapsing neuroborreliosis. Eur Neurol 1995; 35: 113–17.
Vol 348 • October 19, 1996
Hospice care for children SIR—We report two siblings with spinal muscular atrophy type 1 (SMA1). One is a 4-year-old girl now under a respirator management, and the other was her younger brother for whom we decided on a selective use of therapy to deal with his respiratory failure at the age of 4 months. Their parents had been informed about the genetic trait of this disorder before the second pregnancy and decided to proceed after careful consideration. Although the second baby was born with mild asphyxia, he developed well thereafter until the gradual appearance of generalised hypotonia and respiratory insufficiency at around age 3 months. After discussion with the parents about the suitability of management with mechanical ventilation we decided against respiratory care, instead choosing selective use of therapy. The baby died within a week from respiratory failure which was precipitated by acute bronchitis. Through this experience we noted three problems that face paediatricians in treating seriously handicapped children such as our patient. First, there is disagreement among doctors about whether or not to initiate ventilator care for infants with SMA1. In most western countries, vigorous lifesupporting measures are withheld from such babies. On the other hand, many Japanese paediatricians endeavour to resuscitate and prolong the life of such infants. In Japan, many patients with neuromuscular diseases, including SMA1, have been ventilated since infancy, when they were not able to express their own will. In view of the ethical principle of autonomy, this has raised the difficult question of whether or not to ventilate patients who develop respiratory failure early in life.1,2 Sakakihara and colleagues3 pointed out that this disparity between Japan and western countries is related not only to medical systems, but also to the cultural, religious, and historical backgrounds. Second, if we cannot help in deciding on selective non-treatment for these children, it is essential that we physicians should recognise and understand the “death of the second person” principle as defined by Yanagida (ie, the death of a child, parent, sibling, wife, husband, or sweetheart, and the accompanying painful ordeal);4 in paediatrics, we should have deep sympathy for the unbearable emotions that parents have at the death of their children. However, paediatricians in Japan are not used to discussing these kinds of issues. Third, at the moment of a child’s death, enough time and a quiet place for parents to grieve should be provided where such seriously ill children are being cared for. Most of the paediatric departments in Japan do not have such facilities. At present, the requirements of hospice care for aged patients with fatal disorders have been understood and recognised all over the world, including Japan. However, hospice care for children with incurable illnesses has not been generally accepted, let alone properly provided in Japan. Terminally ill children can undergo both extreme mental and physical suffering, which must be eased at their last moments, as too must the parents’. In the neonatal care unit at the Children’s Hospital in Denver in the USA, for example, a nearby hospice care system for severely handicapped newborn babies has been set up.5 Japanese paediatricians want to listen to opinions from doctors in western countries about how they try solve and manage such problems. We would like to solicit opinions and comments on this from your readers. *Shuji Wakai, Nozomi Ito, Noriaki Adachi, Daisuke Ueda, Hiroyuki Tsutsumi, Shunzo Chiba
2
3
4 5
Dysgeusia (“cognate, dis-gusting”) SIR—In January, 1989, I developed a right brachial plexus neuropathy, the cause of which remained obscure until a strongly positive Borrelia burgdorferi blastogenesis stimulation index was found.1 I was given intravenous ceftriaxone and, after recovering from a Herxheimer reaction, resumed my medical duties.2 One morning in October, 1989, I awoke with a foul taste, and assumed that a crowned molar was the source. My dentist assured me that all my teeth were in good condition. The foul taste persisted and eventually I returned to the dentist, insisting that something was “rotting” in my mouth, and with a request that he remove the crown. He was confident the crown was not the source and urged me to delay, which I did. During persistence of the condition the inside of my mouth, including the gingiva and teeth, lacked sensation. The taste was often so bad that I was unable to eat, and avoided standing close to others so as to spare them the offensive odour. In December, 7 months after my treatment for Lyme disease, I returned for neurological consultation. Until the occurrence of dysgeusia, the manifestations had been limited to the brachial neuropathy, with atrophy and weakness of my shoulder-girdle muscles, forearm, and hand.2 I described for the neurologist my terrible taste during the preceding 2 months and he found my gustation to be severely impaired from bilateral dysfunction of the sensory branches of the seventh nerve, a neuropathy presumed caused by borreliosis. I then learned that loss of taste (ageusia) can be associated with acquiring a disgusting taste (dysgeusia). I had been taught that, apart from its motor function, the seventh nerve was responsible for discriminatory taste on the anterior two-thirds of the tongue. Textbooks of neurology did not mention that, along with diminished taste, there may also be a foul taste. A search for case reports was of little help: no one had apparently recorded the symptoms of dysgeusia, other than noting that, along with the sensory loss, there may be a metallic taste. Was it known that dysgeusia can be a distressing complaint, especially when it continues for months? This symptom was far more disturbing than my arm weakness, to which I had adjusted by using other muscles. The perverted taste ranged from awful to repulsive. After 3 years, the vile sensation finally waned (although it has recurred, briefly, several times) but lack of discriminatory taste has persisted. Foregoing the pleasure of lingering over a tasty meal was sad in itself, but after years of dysgeusia it seemed a minor loss. Oh, if only it was merely like the textbooks wrote, “lack of taste”! Christine Lawrence Albert Einstein College of Medicine of Yeshiva University, New York, NY 10461, USA
1
Department of Paediatrics, Sapporo Medical University School of Medicine, Sapporo 060, Japan
1
Lanken P. ATS position statement on withholding and withdrawing life sustaining therapy. Ann Intern Med 1992; 115: 478–85.
1102
Bone RC, Rackow EC, Weg JG, et al. Ethical and moral guidelines for the initiation, continuation, and withdrawal of intensive care. Chest 1990; 97: 949–58. Sakakihara Y, Yamanaka T, Kajii M, et al. Long-term ventilatorassisted children in Japan: a national survey. Acta Paediatr Jap 1996; 38: 137–42. Yanagida K. Sacrifice. Tokyo: Bungei-Shunjyuu, 1995: 203 (in Japanese). Weir RF. Selective nontreatment of handicapped newborns: moral dilemmas in neonatal medicine. New York: Oxford University Press, 1984: 273.
2
Dattwyler RJ, Volkman DJ, Luft BJ, Halperin JJ, Thomas J, Golightly MG. Seronegative Lyme disease: dissociation of the specific T- and B-lymphocyte responses to Borrelia burgdorferi. N Engl J Med 1988; 319: 1441–46. Lawrence C, Lipton RB, Lowy FD, Coyle PK. Seronegative chronic relapsing neuroborreliosis. Eur Neurol 1995; 35: 113–17.
Vol 348 • October 19, 1996