Academic journal article Issues in Law & Medicine

Lives Worth Living

Academic journal article Issues in Law & Medicine

Lives Worth Living

Article excerpt

Clinical Issues Related to Children with Trisomy 13 or 18

The clinical picture often painted for parents at the diagnosis of their child with Trisomy 13 or 18 is dismal. Pictures of the most drastic possible facial deformities, conversations about the worthlessness of their child to society, and dismal comments about the "fact" that these disorders are "incompatible with life" are some of the sufferings that parents of children with trisomy 13 or 18 endure. But, what is the reality?

How Long do Children with Trisomy 13 and 18 Actually Live?

The Kaplan-Meier Survival Curve compiles data from Metropolitan Atlanta 19681999, and shows less than 10% survival at 1 year. But contrast that to the 1994 article from Baty et al. showing a 40% survival at 1 year. Which is correct?

Problems with Trisomy Survival Data

* Most center based studies are small and suffer from rare incidence skewing statistics and limiting actual predictive power.

* Large registry studies have more data, but are unable to comment on the the care provided to the infants, a confounding factor which greatly alters survival.

* When interventions are compared, no studies report on "non-feeding" of infants as part of the palliative care "treatment."

* When interventions are described, often there are no details given regarding care after post-natal diagnosis such as withdrawal of previous "aggressive" care.

* Many studies include mosaics (even though these are generally only 2-4% of trisomy cases).

* In older studies: the diagnosis of trisomy was often made later, missing some cases that died prior to diagnosis.

* In new studies: the increased number of terminations prior to birth for more severely affected babies might skew toward higher survival rates for those infants who survive to birth.

Survival of Trisomy 13 and 18 in the Current Era

Overall Survival

The 2015 study by Meyer et al. is the largest population-based study of survival among children with T13 or T18 published to date. Data is derived from 9 US States during the time interval from 1999-2007. The study includes 693 infants with Trisomy 13 and 1113 infants with Trisomy 18. It includes both full trisomies and mosaics.

They found that if the newborn survives to 28 days postpartum, then the subsequent chances of survival to one year is 46% for trisomy 13 and 36% for trisomy 18, and the chances of survival to 5 years is 38% for trisomy 13 and 33% for trisomy 18. These findings were confirmed by Nelson et al. who found similar long term survival rates. They also found large regional variations in survival, illustrating that the care given to the infants is a large determining factor in long term survival.

Congenital Heart Disease

Peterson et al. reviewed cases from the US Pediatric Cardiac Care Consortium (PCCC) from 1982-2008. They found 50 patients with trisomy 13, (of which 29 underwent intervention) and 121 patients with trisomy 18 (of which 69 underwent intervention).

In hospital mortality for patients with trisomy 13 were 27.6% and for patients with trisomy 18 were 13%. This is a lOx higher mortality rate as compared with the general surgical population for all surgical categories. However, post discharge, the survival rate of the patients with trisomy 13 and trisomy 18 was higher than previously reported.

In summai]? care and treatment of patients with Trisomy 13 and 18 clearly increases their long term survival rates, as clearly demonstrated in the literature. However, the attitudes of medical personnel, and their ignorance of clinical data serve to discourage parents from giving this care.

Attitudes of Medical Personnel Toward Patients with Trisomy 13 and 18

Among Canadian cardiologists surveyed by Young, 70% of respondents would recommend comfort care only to Trisomy 18 newborns with a simple cardiac lesion, which increased to 75% for a complex lesion. Surprisingly, if the case were their own child with Trisomy 18, and the child were in-utero, 65% would terminate the child, and 22% would recommend comfort care only. …

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