Avraham Steinberg

Dissociation between the Wishes of Terminally Ill Parents and Decisions by Their Offspring

Objective: To assess the attitude and factors affecting decision‐making by offspring for life‐sustaining measures toward their elderly terminally ill parents.

The dying patient: new Israeli legislation

The moral, cultural, religious, and legal aspects of treating the dying patient are among the most difficult in modern medicine. Although the dying patient has been one of the most prominent problems within medicine since time immemorial, the dilemma has intensified in the past few decades. This is due to the enormous advances in medicine and technology, the change in patient–physician relationship from a paternalistic to an autonomous approach, the greater involvement of various professionals in treating the dying patient, and the economic and cultural changes. Different cultures have different approaches for the dying patient. Israeli society, like other western societies, has struggled with this issue for many years. Various policies, court cases, and declarations have been enacted. Nevertheless, the situation has been confusing, with physicians not knowing what was allowed or not and acting inconsistently, often not discussing decisions with patients, next-of-kin, or other health-care professionals and not documenting life-and-death decisions. As endof-life practices vary around the world [1, 2] and many countries are battling with these issues, the development process and the current Israeli law may provide useful insights for other countries and various professionals. Israeli legislation: the process

A safety trial of high dose glyceryl triacetate for Canavan disease.

Canavan disease (CD MIM#271900) is a rare autosomal recessive neurodegenerative disorder presenting in early infancy. The course of the disease is variable, but it is always fatal. CD is caused by mutations in the ASPA gene, which codes for the enzyme aspartoacylase (ASPA), which breaks down N-acetylaspartate (NAA) to acetate and aspartic acid. The lack of NAA-degrading enzyme activity leads to excess accumulation of NAA in the brain and deficiency of acetate, which is necessary for myelin lipid synthesis. Glyceryltriacetate (GTA) is a short-chain triglyceride with three acetate moieties on a glycerol backbone and has proven an effective acetate precursor. Intragastric administration of GTA to tremor mice results in greatly increased brain acetate levels, and improved motor functions. GTA given to infants with CD at a low dose (up to 0.25 g/kg/d) resulted in no improvement in their clinical status, but also no detectable toxicity. We present for the first time the safety profile of high dose GTA (4.5 g/kg/d) in 2 patients with CD. We treated 2 infants with CD at ages 8 months and 1 year with high dose GTA, for 4.5 and 6 months respectively. No significant side effects and no toxicity were observed. Although the treatment resulted in no motor improvement, it was well tolerated. The lack of clinical improvement might be explained mainly by the late onset of treatment, when significant brain damage was already present. Further larger studies of CD patients below age 3 months are required in order to test the long-term efficacy of this drug.

Euthanasia: An Overview and the Jewish Perspective

Background: End-of-life care poses fundamental ethical problems to clinicians. Defining euthanasia is a difficult and complex task, which causes confusion in its practical clinical application. Over the course of history, abuse of the term has led to medical atrocities. Familiarity with the relevant bioethical issues and the development of practical guidelines might improve clinical performance. Objective: To define philosophical concepts, to present historical events, to discuss the relevant attitudes in modern bioethics and law that may be helpful in elaborating practical guidelines for clinicians regarding euthanasia and end-of-life care. Concepts found in the classic sources of Jewish tradition might shed additional light on the issue and help clinicians in their decision-making process. Methods: An historical overview defines the concepts of active versus passive euthanasia, physician-assisted suicide and related terms. Positions found in classical Jewish literature are presented and analyzed with their later interpretations. The relevance and application in modern clinical medicine of both the general and Jewish approaches are discussed. Results: The overview of current bioethical concepts demonstrates the variety of approaches in western culture and legal systems. Philosophically and conceptually, there is a crucial distinction between active and passive euthanasia. The legitimacy of active euthanasia has been the subject of major controversy in recent times in various countries and religious traditions. Conclusion: The historical overview and the literature review demonstrate the need to provide clearer definitions of the concepts relating to euthanasia, for in the past the term has led to major confusion and uncontrolled abuse. Bioethical topics should, therefore, be included in medical training and continuing education. There are major debates and controversies regarding the current clinical and legal approaches. We trust that classical Jewish sources might contribute to the establishment of clinical definitions, meaningful approaches and practical guidelines for clinicians.

Neonatal Mydriasis: Intravenous Lidocaine Adverse Reaction

Lidocaine (a sodium channel blocker) serves as an antiepilepsy drug in neonates. We present a neonate with a seizure disorder who acutely developed pupillary mydriasis secondary to lidocaine overdose. We suggest adding this side effect to the list of untoward effects of lidocaine and to the differential diagnosis of fixed dilated pupils in neonates treated with lidocaine. (J Child Neurol 2002; 17:400-401).

The Groningen Protocol – The Jewish Perspective

Despite significant advances in neonatology, there will always be newborns with serious life-threatening conditions creating most difficult bioethical dilemmas. Active euthanasia for adult patients is one of the most controversial bioethical questions; for severely ill neonates, the issue is even more complex, due to their inability to take part in any decision concerning their future. The Groningen Protocol introduced in 2005 by P.J. Sauer proposes criteria allowing active euthanasia for severely ill, not necessarily terminal, newborns with incurable conditions and poor quality of life in order to spare them unbearable suffering. We discuss the ethical dilemma and ideological foundations of the protocol, the opinions of its defenders and critics, and the dangers involved. The Jewish perspective relating to the subject is presented based on classical Jewish sources, which we trust may enrich modern bioethical debates. In Jewish law, the fetus acquires full legal status only after birth. However, while the lives of terminally ill neonates must in no way be actively destroyed or shortened, there is no obligation to make extraordinary efforts to prolong their lives. Accurate preimplantation or prenatal diagnosis might significantly reduce the incidence of nonviable births, but active killing of infants violates the basic foundations of Jewish law, and opens the ‘slippery slope’ for uncontrolled abuse. Therefore, we call upon the international medical and bioethical community to reject the Groningen Protocol that permits euthanization and to develop ethical guidelines for the optimal care of severely compromised neonates.

Disclosure of Information and Informed Consent: Ethical and Practical Considerations

Disclosure of information and informed consent are relatively new concepts in the patient—physician relationship. They are based primarily on the principle of autonomy and they have many favorable practical advantages. However, the practical implementation of these requirements is fraught with difficulties, some of which can cause harm to the patient or be obstacles in fulfilling the moral obligation of beneficence. This is particularly true when disclosure of information and informed consent are done by physicians in a defensive way for fear of malpractice suits. The most ethically defensible approach is to tailor and navigate the information according to the needs and desires of each individual patient in a sensitive and empathic manner. The informed consent should be a process of mutually shared responsibility by the patient and the physician, ensuring adequate and relevant information that is well comprehended by the individual patient, and is used correctly for his or her decision making.

Transverse Myelitis: Unusual Sequelae of Neonatal Group B Streptococcus Disease

Group B streptococcus (GBS) meningitis is one of the most serious infections in the neonatal period. We wish to report a case of transverse myelitis following early onset form of GBS meningitis. The diagnosis and the clinical approach will be discussed.

Efficacy of valproic acid in the treatment of Sydenham's chorea.

following investigations were normal: urinalysis, full blood count, erythrocyte sedimentation rate, liver function tests, calcium, phosphate, uric acid, fasting lactate, plasma amino acids, serum lead, copper and ceruloplasmin, antinuclear antibodies, rheumatoid factor, thyroid function studies, urine organic acids, electroencephalography, computed tomographic scans of the head, chest radiography, electrocardiography, and echocardiography. CSF analysis revealed the following: normal cell count, glucose, and protein, and elevated IgG synthesis index, absent oligoclonal bands, and a normal level of y-aminobutyric acid (GABA). A diagnosis of Sydenham’s chorea was made. The patient was treated with valproic acid in a dose of 25 mg/kg daily for three weeks without improvement. The valproic acid was discontinued. He was commenced on haloperidol and within six days the chorea had lessened. One year after the onset of the involuntary movements he continues to improve. The dosage of haloperidol has been reduced to 0.5 mg twice daily.

The implementation of a protocol promoting the safe practice of brain death determination

PURPOSEnThe purpose of the study is to describe the implementation of measures introduced in Israel in 2009 to promote the safe practice of brain death determination (BDD).nnnMATERIALS AND METHODSnThe measures require (1) physicians to undergo a mandatory training course, (2) the mandatory performance of an ancillary test, and (3) retrospective examination of all BDD forms by an independent committee. Any deviations from practice parameters were noted. Surveys were also undertaken to assess (i) the attitude of local physicians to the measures and (ii) whether similar measures are in place in Europe and whether they were considered necessary.nnnRESULTSnAfter implementation, the measures resulted in the absence of deviations from practice parameters over time. A majority of local physician (n = 64) felt the measures added a sense of security to BDD (73%) and ensured its proper performance (85%). The European survey (n = 20 countries) revealed (1) specialized BDD training is required in 60%, provided in 50%, while felt necessary by 80%; (2) independent supervision of BDD is performed in only one other country; and (3) BDD is performed country-wide using the same criteria in 80% while felt necessary by 95%.nnnCONCLUSIONnThe measures were successfully implemented, reduced diversity in patient testing, and positively accepted by local physicians. Wider application of the measures may be appropriate as suggested by the results of a European survey and the variability of BDD reported in the literature.