Lillian Hanly’s family has Huntington’s, a fatal neurodegenerative disease. She has a fifty/fifty chance of inheriting it, but is yet to take the blood test to find out. Her short film, Fifty Percent, part of the Loading Docs 2021 series, explores her decision of whether to take this test or not.
By Lillian Hanly
The blood test is a simple one, but the process in order to take the test, to reach a decision, is much more difficult. The disease is a horrible one, for some, a death sentence. Those who inherit the genetic mutation which causes it lose control of themselves physically, mentally and psychologically. It literally kills your brain cells, causing you to ultimately lose all sense of yourself.
You have only been able to test, and therefore know if you will inherit the disease, since 1993, when the gene was isolated. That means it is my generation who is the first to approach major life decisions with the knowledge of the disease in front of them, rather than previous generations who have tested later in life and considered these decisions retrospectively. For us, we have the ability to make much more informed decisions. But with more knowledge comes more difficult discussions and decisions.
As I contemplate whether to get tested or not, I’ve been thinking a lot about those difficult discussions and decisions that come later on. I’m even struggling with the question in the heading of this article. It has the implication, firstly, that one can only have children biologically, and we know there are many ways to become parents. It also implies that one should not have children given the risk you pass on to them.
Malvindar Singh-Bains of the Centre for Brain Research at the University of Auckland, and co-chair of Huntington’s Disease Youth Organisation NZ, understands the consequences of these discussions. “One thing I hate hearing is people saying insensitive things like, we should stop them having children, and that's how we'll eliminate Huntington's disease.”
With each biological offspring having the fifty/fifty chance, there are many variables in this equation. For instance, having children before or after testing, but also choosing how to have those children. Medical developments mean that IVF is an option - ensuring from the outset the child conceived is not at risk at all.
Singh-Bains explains this further. “One of the revolutions of science in terms of fertility has been the introduction of IVF. And in New Zealand, if you test [positive] for Huntington's disease, you are eligible for two fully-funded cycles of IVF. [What it gives you is] an opportunity to have viable, healthy offspring that are free of the disease. And this time 20 years ago, that was virtually unheard of. So there is hope for young families that have Huntington's disease to have a next generation that is free of the condition.”
I’ll let that sink in for a moment, because this is truly fantastic as an option, and that science allows for this. It also offers the possibility of removing Huntington’s entirely from a family line.
But, IVF is a huge undertaking. It can be a very difficult process, and it doesn’t work for everyone. It is also a very long process. After going through what could end up being a year-long process of testing for the gene yourself, there’s then only 40 rounds of publicly funded IVF cycles available per year. You could be on a waitlist for years before you even begin the process.
Another tool being researched is CRISPR, a gene-editing technology that would alter your DNA entirely. Gene editing is itself a Pandora’s Box. There is no knowing yet what the consequences are for future generations if this tool is applied. Furthermore, Huntington’s is caused by a genetic mutation. The chances are slim but, even if we got rid of it, it could appear again, in mine or in anyone else’s family. So, while these options are incredible, and should be considered, for those who cannot access them for whatever reason - should they be told they cannot have babies? What happens if those two IVF attempts don’t work, and you can’t afford to keep going - then what?
Are there other options available that might make having children a less daunting prospect? In the last 30 years there have been significant advancements for possible treatments of Huntington’s Disease. Most recently, there’s been research around treatments focusing on huntingtin-lowering. In Huntington’s-positive individuals, the mutation of the huntingtin gene creates larger amounts of protein than is needed, causing the Huntington’s Disease symptoms that can end up being fatal. The potential treatment would reduce the amount of protein created, thus giving the Huntington’s positive individual more time before symptoms begin, or slow the rate of symptom progression if it had already started. These treatments provide children who might be born with Huntington’s the opportunity to live a normal life.
In presupposing that someone who is at risk of a fatal disease shouldn’t have children, we remove from them the possibilities of whatever good can come from life regardless of such pain. It also dismisses the probability that something else entirely might affect them, from other genetic conditions or from natural events.
It’s important to note that many with this disease cannot and will not have children due to dying from the disease very young - and this is an irreconcilable tragedy, to have the choice removed from you entirely.
For those who do have a choice, there are other considerations to be had, and as I said above with more knowledge comes more difficult discussions and decisions. What the question above doesn’t acknowledge is that having children is and always will be, a risk. With Huntington’s, it’s possible to go down a route that may give that child a significantly different and easier life, but there are equally a number of other genes and also life events that you cannot test for or predict that could completely turn one’s life around, for better or for worse.
Ultimately, I think that having children, and for some - not having children, in whatever way that occurs, and however you may become a parent or caregiver or remain just an aunty, is really about placing hope in a future. It isn’t about the horrible things in the world they might face - otherwise, you probably wouldn’t have children. It is about committing to the things you believe to be good in this world. That you may hope someone else gets to experience those things, or carry on what it is that you hold dear to you in your moment in this universe. And it is also preparing them for the worst that can occur in this universe, and giving them the tools to respond. For each and every one of us, that is going to look very different. The question should then instead be, how might our future generations, in all the ways they will be in this world, face the difficulties that are here now, and that are coming?
Fifty Percent is part of the Loading Docs 2021 collection. The films can viewed online via www.loadingdocs.net