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Who has the right to decide how couples at risk for Huntington's should have children? Or more specific to the debate now occurring in Europe: What rights do couples have when requesting reproductive procedures (PGD) for the purpose of having a gene negative child? What are the issues in this debate -- and who has the right to decide?

What is PGD? Medications are given to the woman that will stimulate the development of multiple eggs. When mature, the eggs are retrieved with a needle and are then incubated with the male partner's sperm. On day 3 of embryo development, a small number of cells are tested for the Huntington gene (this is the PGD part) then a few days later the non-HD embryos are placed in the woman's uterus by a very common, low-risk procedure (IVF) frequently performed in fertility clinics. For a more complete discussion of these procedures see Dr. Carne's review for HDDW (linked below).

PGD has been offered to Huntington couples --and to couples at risk for other genetic diseases -- so that they can decrease the risk of passing a disease gene to their child. Up to this point PGD for Huntington’s has been an option for couples with one partner who is known to be gene positive, and for couples with one partner at risk who chooses not to know – or to learn -- personal gene status prior to or during PGD procedures.

What is the debate? The controversy concerns only those at risk couples who choose not to know their own gene status (PGD with non-disclosure). PGD in this situation can result in 3 different possibilities:

There are those who argue, as did members of the committee of the International Huntington's Association (IHA), and groups of medical and legal professionals in the UK and the Netherlands -- that it is not ethical for doctors to perform sham or unnecessary PGD-IVF procedures for couples whose choice is not to know gene status. In fact the government in the Netherlands now requires gene testing in order to qualify for embryo selection by PGD for Huntington's.

What is the counter argument? On the other side, there are those who argue that couples at risk for HD have the right not to know genetic status, and that it is not ethical to restrict their options for PGD. The authors in a recent article in The Journal of Medical Ethics review exactly this question [Asscher E 2010]. The major points:

Therefore they argue that there may be substantial risk for harm in forcing couples to test for HD prior to PGD.

Conclusions: There are two separate but related issues in this PGD debate. The first deals with balancing the risks for harm in this situation: the risks related to unnecessary medical procedures against the risks of forcing gene testing. This sort of balancing of risk and benefit is what happens (or should) in every medical encounter calling for informed consent --whether it is a newly prescribed medicine or a surgical procedure. The second deals with whose right it is to decide: Does it belong to the couple, the government, or a Huntington's organization?

Editors comments: Huntington families know --better than anyone else -- that there are no easy life decisions for those at risk. Do we dare encourage serious relationships, or work toward careers that may be disrupted by HD? What about marriage? And probably the most difficult, what about children? Like every thing else in HD, PGD is a hard decision that does not have a clear right or wrong answer. But I will argue along with the United Nations that the right of decision -- to test or not -- belongs to the couples involved.

It is also legitimate for individual doctors who object to PGD for gene untested couples to refuse to perform the procedure. But in this setting PGD centers have responsibility to identify other doctors who are willing. This type of compromise is nothing new; it already occurs with other difficult medical situations like abortion and death with dignity initiatives; doctors have the right to participate or not.

And finally, it is my opinion that the International Huntington's Association needs to reconsider their proposed (I believe paternalistic) guideline that recommends against PGD for Huntington couples that choose not to gene test. And I would go further and say they may have it backwards: they should be advocating for the rights of Huntington's families -- not proposing they be taken away. And yet further on my wish list: They could be positive international force advocating for insurance coverage for the many Huntington couples who would choose this procedure as an option for having children -- but can't afford it.

References

Asscher E, Koops BJ. The right not to know and preimplantation genetic diagnosis for Huntington's disease. J Med Ethics. 2010 Jan;36(1):30-3. doi: 10.1136/jme.2009.031047. PubMed abstract

Almqvist EW, Bloch M, Brinkman R, Craufurd D, Hayden MR. A worldwide assessment of the frequency of suicide, suicide attempts, or psychiatric hospitalization after predictive testing for Huntington disease. Am J Hum Genet. 1999 May;64(5):1293-304. PubMed abstract