Ethical challenges in the care of children and families affected by life-limiting neuromuscular diseases

Journal of Developmental and Behavioral Pediatrics

Volumn 33, Issue 7, 2012, Pages 548-561

  Geller, Gail ^a,b,c   Harrison, Krista L ^a,c   Rushton, Cynda H ^a,b,d  

^a JOHNS HOPKINS UNIVERSITY   (United States)

^b JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^c JOHNS HOPKINS BLOOMBERG SCHOOL OF PUBLIC HEALTH   (United States)

^d JOHNS HOPKINS UNIVERSITY SCHOOL OF NURSING   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ADULT; AGED; ARTICLE; DUCHENNE MUSCULAR DYSTROPHY; ETHICS; FEMALE; HEALTH CARE PERSONNEL; HEALTH SURVEY; HEREDITARY SPINAL MUSCULAR ATROPHY; HUMAN; MALE; MIDDLE AGED; MULTICENTER STUDY; NEUROMUSCULAR DISEASE; PHYSICIAN; PSYCHOLOGICAL ASPECT; QUESTIONNAIRE; UTILIZATION REVIEW;

ADULT; AGED; ETHICS CONSULTATION; FEMALE; HEALTH PERSONNEL; HEALTH SURVEYS; HUMANS; MALE; MIDDLE AGED; MUSCULAR DYSTROPHY, DUCHENNE; NEUROMUSCULAR DISEASES; PHYSICIANS; QUESTIONNAIRES; SPINAL MUSCULAR ATROPHIES OF CHILDHOOD; YOUNG ADULT;

EID: 84865964728     PISSN: 0196206X     EISSN: None     Source Type: Journal    
DOI: 10.1097/DBP.0b013e318267c62d     Document Type: Article

References (71)

1. Crawford TO. Spinal muscular atrophies. In Jones HR, De Vivo DC, Darras BT, eds. Neuromuscular Disorders of Infancy, Childhood and Adolescence: A Clinician's Approach. Philadelphia, PA: Butterworth Heinemann; 2003:145-166.
2. Roland EH. Muscular dystrophy. Pediatr Rev. 2000;21:233-237.
3. Crawford TO, Pardo CA. The neurobiology of childhood spinal muscular atrophy. Neurobiol Dis. 1996;3:97-110. (Pubitemid 26275339)
4. Gibson B. Long-term ventilation for patients with Duchenne muscular dystrophy: physicians' beliefs and practices. Chest. 2001; 119:940-946. (Pubitemid 32201150)
5. Bach JR. The use of mechanical ventilation is appropriate in children with genetically proven spinal muscular atrophy type 1: the motion for. Paediatr Respir Rev. 2008;9:45-50.
6. Hardart MF, Burns JP, Troug RD. Respiratory support in SMA-1: a survey of physician practices and attitudes. Pediatrics. 2002; 110:e24.
7. Ryan MM. The use of invasive ventilation is appropriate in children with genetically proven spinal muscular atrophy type 1: the motion against [review]. Paediatr Respir Rev. 2008;9:51-54; discussion 55-56.
8. Hardart MK, Troug RD. Spinal muscular atrophy type 1. Arch Dis Child. 2003;88:848-850.
9. Birnkrant DJ, Bushby KM, Amin RS, et al. The respiratory management of patients with Duchenne muscular dystrophy: a DMD care considerations working group specialty article. Pediatr Pulmonol. 2010;45:739-748.
10. Jonas M. The baby MB case: medical decision making in the context of uncertain infant suffering. J Med Ethics. 2007;33:541-544.
11. Carnevale FA, Alexander E, Davis M, Rennick J, Troini R. Daily living with distress and enrichment: the moral experience of families with ventilator-assisted children at home. Pediatrics. 2006; 117:e48-e60. (Pubitemid 46061088)
12. Mah JK, Thannhauser JE, Kolski H, Dewey D. Parental stress and quality of life in children with neuromuscular disease. Pediatr Neurol. 2008;39:102-107. (Pubitemid 351970667)
13. Simonds AK. Ethical aspects of home long term ventilation in children with neuromuscular disease. Paediatr Respir Rev. 2005;6: 209-214. (Pubitemid 41258651)
14. Bach JR, Vegas J, Majors J, Friedman A. Spinal muscular atrophy type 1 quality of life. Am J Phys Med Rehab. 2003;82:137-142. (Pubitemid 36120057)
15. Mitchell I. Spinal muscular atrophy type 1: what are the ethics and practicality of respiratory support? Paediatr Respir Rev. 2006;7 (suppl 1):S210-S211. (Pubitemid 43906286)
16. Ryan MM, Kilham H, Jacobe S, Tobin B, Isaacs D. Spinal muscular atrophy type 1: is long-term mechanical ventilation ethical? J Paediatr Child Health. 2007;43:237-242.
17. Kent MA. The ethical arguments concerning the artificial ventilation of patients with motor neurone disease. Nursing Ethics. 1996;3:317-328.
18. Rushton CH, Lynch ME. Dealing with advance directives for critically ill children and adolescents. Crit Care Nurse. 1992;12:31-37.
19. Weir RF, Peters C. Affirming the decisions children and adolescents make about life and death. Hastings Cent Rep. 1997;27:29-40.
20. Wharton RH, Levine KR, Buka S, Emanuel L. Advance care planning for children with special health care needs: a survey of parental attitudes. Pediatrics. 1996;97:682-687. (Pubitemid 26138099)
21. Erby LH, Rushton C, Geller G. "My son is still walking": stages of receptivity to discussions of advance care planning among parents of sons with Duchenne muscular dystrophy. Semin Pediatr Neurol. 2006;13:132-140. (Pubitemid 44486828)
22. Penner L, Cantor RM, Siegel L. Joseph's wishes: ethical decisionmaking in Duchenne muscular dystrophy. Mt Sinai J Med. 2010;77: 394-397.
23. Gitlin JM, Fischbeck K, Crawford TO, et al. Carrier testing for spinal muscular atrophy. Genet Med. 2010;12:621-622.
24. Knebel AR, Hudgings C. End-of-life issues in genetic disorders: summary of workshop held at the National Institutes of Health on September 26, 2001. Genet Med. 2002;4:373-378. (Pubitemid 44698552)
25. Knebel AR, Hudgings C. End-of-life issues in genetic disorders: literature and research directions. Genet Med. 2002;4:366-372. (Pubitemid 44698551)
26. James CA, Hadley DW, Holtzman NA, Winkelstein JA. How does the mode of inheritance of a genetic condition influence families? A study of guilt, blame, stigma and understanding of inheritance and reproductive risks in families with X-linked and autosomal recessive diseases. Genet Med. 2006;8:234-242.
27. Ross LF. Screening for conditions that do not meet the Wilson and Jungner criteria: the case of Duchenne muscular dystrophy. Am J Med Genet. 2006;140A:914-922.
28. Ross LF. Predictive genetic testing for conditions that present in childhood. Kennedy Inst Ethics J. 2002;12:225-244.
29. Borry P, Fryns JP, Schotsmans P, Kierickx K. Attitudes toward carrier testing in minors: a systematic review. Genet Couns. 2005; 16:341-352.
30. Krajewski KM, Shy ME. Genetic testing in neuromuscular disease. Neurol Clin. 2004;22:481-508. (Pubitemid 38797979)
31. Bach JR. Threats to "informed" advance directives for the severely physically challenged? Arch Phys Med Rehabil. 2003;84:S23-S28. (Pubitemid 36417639)
32. Broome ME, Kodish R, Geller G, Siminoff L. Children in research: new perspectives and practices for informed consent. IRB. 2003; 25:S20-S25.
33. Klaus G, Mehls O, Hinderer J. Mode of death in Duchenne muscular dystrophy. Lancet. 1991;337:801-802.
34. Madorsky JG, Radford LM, Neumann EM. Psychosocial aspects of death and dying in Duchenne muscular dystrophy. Arch Phys Med Rehabil. 1984;65:79-82. (Pubitemid 14186750)
35. Hurwitz CA, Duncan J, Wolfe J. Caring for the child with cancer at the close of life: "There are people who make it, and I'm hoping I'm one of them". JAMA. 2004;292:2141-2146.
36. Bluebond-Langner M. The Private Worlds of Dying Children. Princeton, NJ: Princeton University Press; 1978.
37. Sourkes BM. The broken heart: anticipatory grief in the child facing death. J Palliat Care. 1996;12:56-59. (Pubitemid 126673621)
38. Hilton T, Orr RD, Perkin RM, Ashwal S. End of life care in Duchenne muscular dystrophy [review]. Pediatr Neurol. 1993;9: 165-177. (Pubitemid 23199462)
39. Wang CH, Finkel RS, Bertini ES, et al. Participants of the International Conference on SMA Standard of Care. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol. 2007;22:1027-1049.
40. Kristjanson LJ, Aoun SM, Oldham L, et al; American Thoracic Society. Respiratory care of the patient with Duchenne muscular dystrophy: ATS Consensus statement. Am J Respir Crit Care Med. 2004;170:456-465.
41. Bushby K, Finkel R, Birnkrant DJ, et al. DMD Care Considerations Working Group. Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management [review]. Lancet Neurol. 2010;9:77-93.
42. Bushby K, Finkel R, Birnkrant DJ, et al. for the DMD Care Considerations Working Group. Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care. Lancet Neurol. 2010;9:77-93.
43. Solomon MZ, Sellers DF, Heller KS, et al. New and lingering controversies in pediatric end-of-life care. Pediatrics. 2005;116: 872-883. (Pubitemid 44542813)
44. Borry P, Stultiens L, Nys H, Cassiman JJ, Dierickx K. Presymptomatic and predictive genetic testing in minors: a systematic review of guidelines and position papers [review]. Clin Genet. 2006;70:374-381. (Pubitemid 44515636)
45. American Academy of Pediatrics Committee on Bioethics. Guidelines on Forgoing Life-Sustaining Medical Treatment. Pediatrics. 2009;123:1421-1422.
46. American Academy of Pediatrics Committee on Bioethics. Informed consent, parental permission, and assent in pediatric practice. Pediatrics. 1995;96:981-982.
47. Tambor ES, Chase GA, Faden RR, Geller G, Hofman KJ, Holtzman NA. Improving response rates through incentive and follow-up: the effect on a survey of physicians' knowledge of genetics. Am J Public Health. 1993;83:1599-1603. (Pubitemid 23320046)
48. StataCorp. Stata Statistical Software: Release 10. College Station, TX: StataCorp LP; 2007.
49. Badzek L, Kanosky S. Mature minors and end-of-life decisionmaking: a new development in their legal right to participation. J Nurs Law. 2002;8:23-29.
50. Leibowitz D, Dubowitz V. Intellect and behaviour in Duchenne muscular dystrophy. Dev Med Child Neurol. 1981;23: 577-590. (Pubitemid 11054354)
51. Roccella M, Pace R, De Gregorio MT. Psychopathological assessment in children affected by Duchenne de Boulogne muscular dystrophy. Minerva Pediatr. 2003;55:267-273.
52. Wicksell RK, Kihlgren M, Melin L, Eeg-Olofsson O. Specific cognitive deficits are common in children with Duchenne muscular dystrophy. Dev Med Child Neurol. 2004;46:154-159. (Pubitemid 38263450)
53. Diekema DS, Botkin JR; American Academy of Pediatrics Committee on Bioethics. Forgoing medically provided nutrition and hydration in children. Pediatrics. 2009;124:813-822.
54. Kinali M, Manzur AY, Mercuri E, et al. UK physicians' attitudes and practices in long-term non-invasive ventilation of Duchenne muscular dystrophy. Pediatr Rehabil. 2006;9:351-364. (Pubitemid 44581993)
55. Benson RC, Hardy KA, Gildengorin G, Hsia D. International survey of physician recommendation for tracheostomy for spinal muscular atrophy type I. Pediatr Pulmonol. 2012;47:606-611.
56. McMillan HJ, Campbell C, Mah JK. Duchenne muscular dystrophy: Canadian paediatric neuromuscular physicians survey. Can J Neurol Sci. 2010;37:195-205.
57. American Academy of Pediatrics Committee on Bioethics. Ethical issues with genetic testing in pediatrics. Pediatrics. 2001;107: 1451-1455.
58. Bodensteiner JB, Ng YT. Ethical considerations in paediatric neurology: neuromuscular disease and epilepsy. J Paediatr Child Health. 2011;47:599-602.
59. Interprofessional Education Collaborative. Core Competencies for Interprofessional Collaborative Practice. 2011. Available at:http://www.aacn. nche.edu/education-resources/IPECReport.pdf. Accessed November 2, 2011.
60. Institute of Medicine Committee on the Health Professions Education Summit. Health Professions Education: A Bridge to Quality. Greiner AC, Knebel E, eds. Washington, DC: National Academy Press; 2003.
61. Browning DM, Solomon MZ; for the Initiative for Pediatric Palliative Care (IPPC) Investigator Team. The initiative for pediatric palliative care: an interdisciplinary educational approach for healthcare professionals. J Pediatr Nurs. 2005;20:326-334. (Pubitemid 41330920)
62. Solomon MZ, Browning D, Dokken D, et al. The Initiative for Pediatric Palliative Care curriculum: Enhancing Family-Centered Care for Children With Life-Threatening Conditions. Modules 1-5. Newton, MA: Education Development Center, Inc.; 2003. Available at: http://www.ippcweb.org/curriculum.asp.
63. Dyer JA. Multidisciplinary, interdisciplinary, and transdisciplinary educational models and nursing education. Nurs Educ Perspect. 2003.
64. Solomon MZ, Browning DM, Dokken DL, Merriman M, Rushton C. Learning that leads to action: impact and characteristics of a professional education approach to improve the care of critically ill children and their families. Arch Pediatr Adolesc Med. 2010;164: 315-322.
65. DuVal G, Sartorius L, Clarridge B, Gensler G, Danis M. What triggers requests for ethics consultations? J Med Ethics 2001;27(suppl 1): I24-I29. (Pubitemid 32294076)
66. Rushton CH, Erby LH, Cohn RD, Geller G. Integrating palliative care in life-limiting pediatric neuromuscular conditions: the case of SMA-type 1 and Duchene muscular dystrophy. J Pall Care Med. 2012;2:103-109.
67. Parker D, Maddocks I, Stern LM. The role of palliative care in advance muscular dystrophy and spinal muscular atrophy. J Paediatr Child Health. 1999;35:245-250. (Pubitemid 29282271)
68. Weidner NJ. Developing an interdisciplinary palliative care plan for the patient with muscular dystrophy. Pediatr Ann. 2005;34: 546-552. (Pubitemid 41073055)
69. Cohn RD. Best practice in Duchenne dystrophy. Neuromuscul Disord. 2010;20:292.
70. Arias R, Andrews J, Pandya S, et al. Palliative care services in families of males with Duchenne muscular dystrophy. Muscle Nerve. 2011;44:93-101.
71. Rushton CH, Geller G. A vision of hope for children and families living with life-threatening neuromuscular disorder: the expanding role of pediatric palliative care. Medecine Palliative Soins de Support Accompagnement Ethique. 2010;9: 283-290.