A Doctor’s Dilemma: Test a Child Up for Adoption for Huntington Disease?

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Dilemma: Test a child up for adoption for Huntington disease?

Several years ago, I met with an infertile couple who had found a beautiful, healthy infant who was up for adoption because the child’s mother was disabled with Huntington disease (HD). This inherited disorder presents with involuntary movements in early middle age and continues with neurologic deterioration and early death (as shown by the folksinger Woody Guthrie). As a prerequisite for adoption, the couple asked our clinical genetics team to coordinate gene testing to ensure the child had inherited the normal HD gene copy (50% chance), being willing to pay for the test (about 1800) regardless of the result. Our genetic counselor, certified by the Huntington Disease Association (www.hdsa.org/) to coordinate HD testing, refused: She cited standard protocols that exclude minors from genetic testing because they cannot give informed consent.

As the supervising physician and medical geneticist, I suggested doing the test as a “coin flip with peek.” I would arrange the test with results sent only to me, telling the adoptive couple that I would flip a coin to determine if the report would be read or destroyed. Privately, I would peek at the result, revealing it if normal, destroying it if positive for future HD and telling the couple that the coin flip prevented disclosure. I felt that this strategy offered a 50% chance of successful adoption without labeling the child. Was it ethical?

Discussion: Should genetic testing be performed on minors and is full disclosure always required of physicians?

The ethical issue of testing minors recognizes the principle of confidentiality of health information plus the potential maleficence of disease labeling with subsequent social, economic, and health insurance discrimination. Minors are tested when good beneficence is thought to outweigh potential harm, as in newborn metabolic screening. In this case, gratifying adoption for infertile parents and a good home for the child is balanced against confidentiality of health information for the child and nondisclosure by the physician. In the instance of nondisclosure, physicians invoke superior medical knowledge to guide decisions rather than educating the patient/parents so they can decide for themselves.

I would suggest that my coin flip with peek approach acknowledged that most medical decisions involve the ethical principal of double effect—choose the option of most good among those where good and bad aspects are inevitable. I think my different perspective from the counselor lies in the fact that physicians take responsibility for the timing and framing of medical information, trying to maximize patient acceptance and minimize suffering. We may be optimistic when families are in shock, gradually filling in the devastating details as they recover and are able to comprehend the facts.

As a pediatric geneticist, I often give bad news in stages—I will tell the family that we must wait for the chromosome test even when Down syndrome is obvious, initially mention learning differences rather than use harsh words like mental retardation. However, I always respond truthfully to direct questions and feel it would be unethical to lie about a test result—hence the “out” of a coin flip.

Outcome—and thoughts on genetic testing

The genetic counselor prevailed, and the parents reluctantly withdrew from adoption because the HD test would not be performed. The child’s fate is not known, although alternative parents were probably found for the child, because there were no health problems aside from the 50% risk of HD developing in early middle age.

Today, the proliferation of genetic-metabolic testing for disorders ranging from cystic fibrosis to breast-ovarian cancer has bypassed ethical recommendations, allowing direct-to-consumer testing that would now be available for this adoptive couple if they could pay out of pocket. Genetic testing has expanded through the use of array technologies that add normal variations or incurable diseases to standard screens (eg, high resolution chromosome/microarray analysis for genetic disorders associated with autism or tandem mass spectroscopy for expanded newborn screening of more than 60 diseases). Many of the findings from these tests, such as the expanded maternal serum/ultrasonographic screening for fetal disorders, offer little benefit to the screened fetus or child.

Many companies promote genetic screening, now offering DNA profiling to define risks for disorders, such as diabetes and coronary artery disease, under the title of “personalized medicine.” On a positive note, the Genetic Information Nondiscrimination Act (GINA) enacted May 21, 2008 is designed to prevent improper use of genetic information in health insurance or employment arenas. While government, industrial, and lay organizations can contribute to health care procedure, I feel the experienced practitioner is best able to coordinate medical testing and to judge the timing and framing of diagnostic results.