MY grandbabies are more miraculous than YOUR grandbabies! Column by Jim Carroll

posted 07/17/2008
You've seen the bumper stickers. The ones that claim that your child or pet is smarter, cuter, or otherwise more wonderful than mine. Silly. Almost pathetic. It's the kind of meaningless competitive thinking that has led people to shout "USA, USA, USA!" when a lone individual, who has worked incredibly hard for four years in small-town obscurity, wins the pole vault at the Olympics.

Still, if it would fit on a bumper sticker, I might be tempted. But it would miss the point, because the issue isn't the competitive part – it's the miraculous part. Billie Francis and Elijah Oran were born just two years ago. They are normal kids. In fact right now they are normal "terrible twos."They push each other off the couch, and stuff noodles in the toilet, and delight in removing the clothes their mother has wrestled them into only moments before, to burst naked from the bedroom.

They are also incredibly cute and have numerous remarkable abilities including being able to eat those noodles with chopsticks. If Elijah drops some food at the table, Billlie will diligently climb down out of her chair and fetch it for him before she eats her own. They share everything, including a remarkable story that will change the lives of their families for all the generations that follow.

I wrestled with whether to share this story with you. It is very personal, and it carries a huge burden of guilt and pain for me. But it is also important. More important than my fear of reprisal or the perpetuation of my decades of self-doubt. You see, we knew there was a chance that our children would not have normal lives. Huntington's chorea ran in my wife's family. It was the middle 1970s and we were newly married. Cindy and I both wanted kids, even though like most 20-somethings we really didn't know what that truly meant in terms of, well, everything! In our case there was another concern. Cindy's maternal grandmother had Huntington's disease. You would be hard pressed to find a more devastating condition.

Huntington's is a degenerative neuro-muscular condition that affects about 30,000 people in our country. It typically begins to affect the afflicted individual in mid-life, and the normal course of the disease inevitably leads to death in 10-20 years. For reasons that are still somewhat unclear, the mechanism that tells brain cells to die so new brain cells can be generated goes haywire. The brain starts telling cells to die at an abnormal rate. The affected individual begins to deteriorate, both mentally and physically. Emotional and behavioral degeneration is accompanied by physical disability that begins with twitching and spasmodic activity that eventually becomes debilitating and leads to death through any number of complications of swallowing, breathing, or secondary infections. Think of any enemy you detest, and then realize you would not wish this on them.

Huntington's is gene-linked. Everyone in the world has a "Huntington's gene", but very few individuals have the "marker" on this gene which unerringly predicts that this individual will "get" Huntington's. If one of your parents has the marker, you have a 50% chance of having it. So having a child if you have the marker is like playing Russian Roulette with a gun that has only two cylinders and one bullet. These days there is a test for this marker. But back in the 70s no test had yet been developed. There was no way to know. If you knew your family carried the marker, you had a difficult decision to make: Gamble with your children's lives, or play safe and be childless. Most everyone had an opinion about this, but only the potential parent had the burden of the decision. We knew that the marker was there, two generations back. Cindy's mother MIGHT have the marker, but she might not. Even if she did, Cindy still had a 50/50 chance to be disease-free. Once the marker stops in a line, it cannot re-appear. If Cindy's mom had dodged the bullet, we had, too. We also considered that polio, which devastated individuals just a generation before ours, had been literally eradicated by medical advances.

We agonized. We prayed. We had children. You are welcome to your opinion aobut this. I can assure you that you have no thought about it that has not occurred to me. You will NOT lie awake over this, much less for the number of nights that I have. While our children were still young, Cindy's mom began to show evidence of the disease. We had lost the first half of our gamble. Or more correctly, our children had lost that first half. But we still had hope and faith and optimism that Cindy would be the lucky one. At the time we followed a faith that encouraged its members to have large families. By the time we had reached our early 30s and felt we had all we could handle, we had six children. Herein lies the next opportunity for the critic to editorialize. Go ahead. Take your best shot. You cannot think of any derogatory term that I have not applied to myself on any of those previously mentioned sleepless nights.

Now before you give up on this story as just TOO depressing, let me tell you a little something about these "kids", who are now young adults. You would be proud and pleased to call any of them your friend. I could list their accomplishments and just sound like another bragging parent. In one area, I think I will: One is finishing a Masters and will be commencement speaker at WWU. One took his degree and worked for a while, then left it all behind to spend months in sub-Saharan Africa working with orphans of AIDS. Twice. One finished her degree and having been the "timid one" all her life moved to Seattle, bravely lived in the city alone, and began to build a career. Two are still in school and pursuing careers they have long cherished – one as a nurse and one as a firefighter. One is completing his doctorate in Neuroscience and working in a lab in Vancouver, BC. It is the foremost research facility in the world working on Huntington's disease. All of these "brags" are more remarkable because every one of these young people put themselves through college. Two did it by serving in the military. Others worked multiple jobs. They ALL worked hard for everything they have. Still, this is not the remarkable part.

The truly remarkable part is their love and support of one another. I won't make a long story even longer by portraying the myriad ways in which they rally and support and care for each other. Let's just say this: If you mess with ONE, you mess with SIX. One of the events which brought them close was caring for their mother when she began to show evidence of Huntington's symptoms. Facing their own mortality; seeing right before their eyes the same symptoms that now hung over their own heads with 50/50 odds, they managed their fear and anger and concern – mostly for each OTHER – and did what had to be done. Their mom was gone in a relatively short time. Complications and secondary illness hastened her passing, and she "only" battled the disease for a bit over a decade.

Four of the kids have decided to be tested to date. Two of them carry the marker. It is a private decision and a private outcome, except in the case of my older son, who has made his decision public. Jeffrey, 31 in August, lives in Vancouver with his wife Megan. While serving in Kosovo in the Army, Jeff learned that his mother was ill with Huntington's. After his military obligation ended, Jeff came home and moved to British Columbia with his Canadian-born wife. He finished his undergraduate work and began to build a life in Vancouver. Helping to deal with his mother's illness, and wondering what to do with this new set of important circumstances in his life, Jeff learned that right in Vancouver, at the University of British Columbia, was a lab that was doing research on gene-linked diseases. Not only that, they were focusing on Huntington's chorea. Jeff interviewed with Dr. Michael Hayden, and went to work in his lab. While helping with the research that might ultimately save his own life as well as that of his siblings, Jeff could also work on his post-graduate studies and earn his doctorate in Neuroscience. Quite an extraordinary set of circumstances for a kid who had never been challenged by anything academic. I remember going to his second grade conference where his excellent teacher told us, "Take this kid to the library. We don't have any books in this school that can challenge him." So we did.

So wasn't this story supposed to be about my grandkids? Whatever happened to Billie and Elijah? Maybe most of you will have seen this coming. Like his parents, Jeffrey and his wife Megan wanted to have kids. Unlike his parents, Jeff and Megan did not face any "maybes" or 25% odds. Jeff knew he was carrying the marker. He knew that his professional life's work was to try to unlock the key to the disease that was sitting dormant but potentially devastating within his own brain. He also knew that he might be facing unemployment. Dr. Hayden has repeatedly told his staff that he fully expects to close the lab in five years. He doesn't intend to give up. He sincerely believes that they will have found the key to Huntington's chorea and its "cure". They have already learned how to "turn off" the gene in mice. Jeff's doctorate work with Capsase-2 may be part of the solution. Still, with odds of 100% staring one in the face, having children would be considered irresponsible by some.

Enter PGD. Pre-Implantation Genetic Diagnosis. In lay terms, it amounts to samping a small amount of genetic material from embryos at the eight-cell stage of division to look for information about disease. In this case, some of Megan's eggs were harvested and fertilized with Jeff's sperm in a lab. Eight miniscule clumps of cells were then tested. The first two that were determined to be free of the marker for Huntington's disease were implanted in Megan's womb. They hoped that ONE would embed and grow. For once, luck was on their side. BOTH embryos "took", and we now know them as Billie Francis and Elijah Oran. Like I said way back at the start: My grandbabies are more miraculous than yours. Jeffrey and Megan have done something that no one in this particular “line” of Huntington's has been able to do: Stop the disease cold in its tracks. No one in Jeff's lineage will ever face the 50/50 sword of Damocles again. Forever. Not a bad thing to do for your family, eh? Now he continues in the lab, looking for a cure for those like himself and one of his siblings who already carry the marker.

Like all powerful tools, PGD has the potential for miracle or monstrosity. There are those who suggest that it will be abused to selectively raise "super-kids"; That modern "Nazis" will select the child of choice by whatever warped criteria the critic fears most. They insist that the practics should be outlawed. It is a tired argument whose proponents would literally toss out the healthy baby with the bathwater. They would accuse my son and his wife of "playing God" or interfering with some "plan." These people can all go pound sand. In the bible God proclaims that He "works in mysterious ways." I personally believe He can use any tool He chooses, including inundation, evolution, or a selfless, brave genius like my son. If you want to argue, bring it on. But remember, you'll be taking on at least SEVEN of us.

If you would like to know more about Huntington's Disease and the progress being made, check out www.hdsa.org/

If you would like to see a Knowledge Network TV interview with Jeffrey about his work and the PGD decision, go to www.knowledgenetwork.ca/ Scroll down in the menu of videos on the right side to the 9th or 10th one called "Stopping the Genetic Clock." The download will take a while, but I think you will find it's worth it.

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Jim and his wife Hollie Swanson are native northwesterners. They moved here last year from Whidbey Island to take new positions as island caretakers on Brown (Friday) Island. They are enjoying life on a "real" island and have been exploring the San Juans in their little tug "Shulala." Comments can be sent to Jim at shu@sanjuanislander.com.