1. Ethical issues about children with special needs: Decision-making by parents and providers Benjamin Wilfond MD Social and Behavioral Research Branch National Human Genome Research Institute Department of Clinical Bioethics Warren G Magnuson Clinical Center National Institutes of Health Bethesda, MD 20892

2. Decision making for children with special needs DNR orders –6 yo s/p BMT, candida sepsis Withdrawal/ withholding nutrition and hydration –1 wo, Down syndrome, esophageal atresia Antibiotics –10 yo, severe developmental delay, recurrent pneumonia

3. Decision making for children with special needs Tracheotomy and/or long term mechanical ventilation –2 wo with congenital hypoventilation syndrome –2 wo with Camptomelic dysplasia –8 yo year old with Hurler syndrome –26 yo with hyper IgE recurrent infection syndrome

4. AbsolutismRelativism Relationship between Parents and Providers

5. Surrogate decision making for children with special needs History of pediatric decision making Standards of judgment for treatment Who should decide Deciding not to employ aggressive measures Tolerance of discordant views –Parental refusal of life saving treatments –Parental requests for treatment of lethal conditions The role of language in decision making

6. History of pediatric decision making Decisions to withhold treatment were routinely made by parents and physicians in the 1970s –Private decision vs public standards 1982 - Baby Doe - Down Syndrome and atresia 1984 - Baby Doe Regulations 1985 AAP recommendations

7. Standard of judgment for treatment Sanctity of Life Quality of Life –Independent financial stability Vs permanent coma Best interests –Life is worse than death from the point of view of the infant –Does not address children in permanent coma Relational potential –If interests can not be determined, the potential to form relationships may provide guidance

8. Who should decide Parents Providers Government agencies Ethics committees

9. Tolerance of discordant views Parents views ProvidersViews Treat Treat Dont Treat

10. Agreement- Don ’ t Treat Parents views Providers Views Treat Treat Don’t treat

11. Deciding not to employ aggressive measures Withholding and withdrawing Killing vs letting die Palliative care

13. Disagreement- Treat/Don ’ t Treat Parents views Providers Views Treat Do not treat

14. Can parents refuse life saving treatments Presumption that parents should make medical decisions for children –Parents promotion of child ’ s interests (well being) –Parents self determination Prince v Massachusetts - 1944 –Obligation to protect children may override parents wishes –Freedom of religion does not include exposing child to life threatening situations AAP- (1998) –No religious exemptions for child abuse legislation

15. Considerations for overriding parental requests to refuse medical treatment Harm –Seriousness –Likelihood –Eminency Intervention –Effectiveness –Safety Alternatives –Feasibility

16. Disagreement- Don ’ t Treat/Treat Parents views Providers Views Treat Treat Do not treat

17. Parental request for treatment of lethal condition Which diseases are lethal? –Asthma –Diabetes –Cystic Fibrosis –Down Syndrome –Tay Sachs –Trisomy 18 –Anencephaly What is lethality? –Likelihood –Duration –Impact of treatment –Quality of life –Genetic

18. Familial and social obligations to seriously ill and disabled newborns Financial costs Availability of services Impact on families Family duties Limits of social obligations

19. Social and financial obligations to children with special needs Health care generally costs money, it does not save money –Health care resources are limited Home IV antibiotics Home mechanical ventilation –Rationing of health care already occurs –Bedside rationing does not usually result in reallocation Prioritization of services needs to be made collectively –Special concerns about vulnerable populations Financial concerns are more acceptable than – “ short people got no reason to live ”

20. Provider/parent agreement Provider supports parental decision Provider/parent disagreement Provider supports parental decision Provider/parent disagreement Provider challenges parental decision Provider tolerance for disagreement: expanding the yellow zone

21. How to approach differing views about treatment decisions Some things must be done delicately- Wayne Morgan (citing the wicked witch of the west) Language can be a powerful manipulator Even “ factual information ” may not be neutral

22. Prohibit Don ’ t Discuss Require Alternative approaches to influencing health related behavior ActivelyPromote ActivelyDiscourage ProvidepositiveinformationProvidenegativeinformation FinancialIncentivesFinancialDisincentives

24. The normative component of language Objective and subjective information about having children –Disadvantages of having children: Sleepless nights, toilet training, and less time for a relationship with spouse –Disadvantages of having children in Washington DC, Child who may be exposed to gangs, shootings, drugs, teenage pregnancy and anthrax Information presented prenatally vs postnatally may send different messages –Down Syndrome –Cystic Fibrosis

25. Information always sends a message –Often associated with behavioral problems –May have difficulty relating to other children –Proper treatment is very expensive, time consuming and rarely paid by third parties –May result in marital problems in parents –May cause problems with siblings –However most will become independently functioning adults

26. The way information is presented reflects the message being sent A serious lung disease in children A common cause of hospitalization Some children may die during childhood Most children must take daily medications The disease can limit physical activity and result in frequent school absences Causes emotional and financial stress on the family A mild lung disease in children Most children are not hospitalized Many have few serious symptoms in childhood Children can use medication to control symptoms Most children lead full lives, are physically active, and can do well in school Most families learn self management of problems

27. Mixed Messages Loeben et al AJHG 1998

28. 000 0 1 2 3 4 5 6 7 8 9 All US UK Commercial Non Commerical US Comericial US Non- Commercial Prenatal General Adult Median number of descriptive sentences about CF in carrier screening pamphlets Positive Negative Neutral

29. Message conveyed by statements about life expectancy Optimism/Hope –“ It is impossible to know how long a person with CF will live. Advances in treatment have improved survival so that most people with CF born today will live into their 30's or longer. ” –“ As a result, although some children will die it a young age, it is likely that many children with CF disease who are born today may live into their 40's or longer. ” –“ In the past, people with CF died very young, but now many are living into their late 20's or 30's. The life span of children born with CF today is expected to be even longer ”

30. Message conveyed by statements about life expectancy Pessimism/Caution –“ Even with improved care, only one-half of people with CF survive beyond 30 years of age. ” –“ Lung congestion, pneumonia, diarrhea, and poor growth are all part of CF, and even with modern medical treatment the average life span is 25 years. ” –“ While there has been much improvement in the care of children with CF: many of them still die in early childhood, and about half of all victims of CF die before they reach age 26. ”

31. Conclusion Decision-making in both the pediatric and prenatal setting are challenging when providers and patients have different views Providers should try to be aware of own biases Providers can influence decisions by how they chose to tell the story Providers should participate in broad social discussions to decide –When to support parental views –How strongly to try to persuade parents –When to actively try to prohibit parental actions