WORDS HAVE POWER

The following is excerpted from the book, “A Celebration of Family: Stories of Parents with Disabilities.” Published in 2021 by the Advocado Press and the Center for Accessible Living

Chapter 23

Kimberly
Everything in Life
is a Roll of the Dice

Kimberly with her husband, Michael, son, Ian, and daughter, Sayer.

Editor’s Note: The following definition of Von Hippel Disease is taken from the website of the National Institute of Neurological Disorders and Stroke (NINDS). “Von Hippel-Lindau disease (VHL) is a rare, genetic multi-system disorder in which non-cancerous tumors grow in certain parts of the body. Slow-growing hemgioblastomas — benign tumors with many blood vessels — may develop in the brain, spinal cord, the retinas of the eyes, and near the inner ear. Cysts (fluid-filled sacs) may develop around the hemangioblastomas. Other types of tumors develop in the adrenal glands, the kidneys, or the pancreas. Symptoms of VHL vary among individuals and depend on the size and location of the tumors. Symptoms may include headaches, problems with balance and walking, dizziness, weakness of the limbs, vision problems, deafness in one ear, and high blood pressure. Individuals with VHL are also at a higher risk than normal for certain types of cancer, especially kidney cancer.”

I lost my sight when I was 14 to a rare genetic disease. The disease I have is called Von Hippel Lindau (VHL). It can affect many, many areas of the body, I think up to ten organs. It usually starts in the retina. When I was diagnosed in 1996, I showed all the symptoms, but nobody in my family had ever had it. The doctors tested my Mom and Dad and neither had the gene. The doctors didn’t think it was possible for me to have VHL, but it ended up being a new mutation. Now they think as many as 20% of cases are new mutations. It raises the question, what is causing the mutation of this gene? That would be a great thing to know, wouldn’t it?
When I was 10, I had surgery to remove a huge retinal tumor. It was the first of about 30-some operations to try to save my sight. By the time I was 14, the surgeries had caused retinal detachment and other things. I was told that VHL was probably the disease I had, but I was not given any guidelines nor told what it would mean for me. When I was 20 and in college, I started having terrible, terrible headaches and other pain. My mother got connected to the National Organization for Rare Disorders. We found out about research that led us to the National Institutes of Health (NIH) where they did verify that we needed to be watching things a lot closer.
I have had many, many more surgeries since then. The disease started causing tumors in lots of different places. I’ve had eight to ten brain surgeries. In 2016, I had a tumor on my spinal cord at C-2, the top part of the spinal cord. That’s not a place you want them cutting into. That surgery left me without the ability to use my left arm or my hand. Yes, I couldn’t type anymore. I had written a number of books by then, including some fantasy novels. Not being able to type kind of took all the fun out of writing. I think my creativity flows through my fingertips.
My surgery in 2016 was at Vanderbilt University in Nashville. I was in the hospital for a month relearning how to walk. I had to go into physical therapy. I had to relearn how to feed myself. I had to relearn virtually everything all over again. There was no cognitive disability at all. It was all physical and motor. I could still speak, but everything with the hands and the legs was affected. I still walk with a very, very pronounced limp.
My last brain surgery was in 2019. Apparently, the brain has ventricles. I did not know that. The tumor was in one of the ventricles through which spinal fluid flows. You don’t want to monkey with spinal fluid. I had a very large brain tumor and I am still experiencing balance issues. Right now I have a kidney tumor and will be facing surgery for that eventually. This disorder has different levels of severity depending upon the individual. It’s always different. I know people who are blind and I know people who’ve never had any sort of retinal surgery. I know people who’ve hardly had a problem at all. I know people who have battled with kidney issues and I know people who’ve never had a kidney issue.
I had learned how to have one disability, but when you have multiple disabilities, that is a whole other thing. I don’t think I ever realized it, but it’s not uncommon. There are a lot of people who have multiple disabilities.
Growing up, I didn’t want to have kids. It just wasn’t something I saw myself doing. That remained true until my husband, Michael, and I had been married for about eleven years. Up until that time, we did not want children. But our thinking changed and we decided to have kids. I was about 30. We knew that there was a 50% chance any children we had could have VHL.
There is something called pre-implantation genetic diagnosis. Eggs are harvested and the eggs that have the VHL gene are discarded. Only the eggs that are free from the gene are fertilized and implanted. I feel it very important here to say that this is not the route we chose to take. That is not a judgment on anybody else’s decision. It is a very personal decision. There are lots of things, lots of factors that go into that. Just because I made one decision doesn’t mean that I would condemn someone else for making a different decision. I certainly don’t want to come off as saying “My way is the right way.” It was the right way for us. That’s all I can say.
We knew that our children might have VHL. I asked my mother, “What would you have done if you had known this was going to happen to me?” She told me, “I would have had you anyway, even though we have been through so much. I would not have chosen any differently.” Anytime you have a child, there’s a chance that child, either at birth or later, will have an illness, accident or disability. That is part of choosing to be a parent. It is like wearing your heart on the outside of your body. It just is. My husband and I decided that we could handle the VHL possibility. Medical science being what it is, we just decided to hope and pray that there would eventually be an easier treatment than surgery or even a cure.
I had my son when I was almost 33. Before getting pregnant, I talked to my doctor. Not that long ago, we might have been told not to have a child, but he just told me, given my history, I should have a C section. Natural childbirth would be too much. The intracranial pressure during childbirth for someone who’s had a lot of brain tumors was not a good idea. So it was decided I would have a C section. I had one tumor that appeared to be growing that the surgeon wanted to take out before we proceeded with the pregnancy, just because they didn’t want to be in a situation where we couldn’t do surgery because of the pregnancy. Once that was done, I got pregnant, and we had our son, Ian. I had my next brain surgery when my son was 20 months old.
Our daughter, Sayer, followed in 2011. We decided we wanted a second child, mainly because we didn’t want our son to be alone. I was an “only child” and I didn’t want him to be an “only child.” He did not particularly appreciate that decision when his sister came along, believe it or not.
My blindness made me freak out about things when I first got pregnant. For instance, I was convinced that I had to find a talking thermometer because I just knew that my baby was going to have a fever and I wasn’t going to know it because I couldn’t read the thermometer. My baby would die or have brain damage because I couldn’t read a thermometer. Once I had my baby, it was not a problem. I can say right now that, by feeling one of my children, I can tell within an eighth of a degree what their temperature is. I don’t really need a thermometer, but it’s one of those things I just obsessed about.
My daughter did not talk for her first year, did not talk at all. I was concerned and called First Steps (Kentucky’s early intervention program). She was really on the border of being eligible for services given her age and other things, but I think because they knew how important her talking was and how her just pointing at things would cause me problems, they pushed ahead to get services for her. With their help, she was talking in no time.
The simple feat of feeding a baby was wicked hard for me, being blind. It really was hard feeding a baby a spoonful of carrot or whatever. Fortunately, at that time, I still had full use of both hands. I kept one hand on my baby’s face, dipped into the food with the other one, and just tried to get it in there. Sometimes you miss. Bath time always came after feeding. They learned to help Mommy really quick. “Why is Mommy so bad at this? I think I will just take over.”
My daughter, although she was slow at talking, was quick at walking. She started walking at nine months old. I did not promote her early mobility at all. Not at all. And she was a climber, too. That gave me a whole new set of problems, but the problems continually change. For instance, my son, that little baby I fed, is now 12 years old. I’m constantly saying, “Turn it down!”
I couldn’t read to my kids, but we could go to story time at the public library if I could get a ride with other moms. We would go to library story time and Ian got read to that way.
Baby gates, of course, were very important for us. Overall, we did a lot more child-proofing than I think other couples have to do simply because child-proofing is also blind-proofing. We’re not going to have a lot of knickknacks and things sitting around the house for me to knock over anyway. Plug covers, cabinet locks and things like that were important. We were just really, really hyper vigilant about child proofing.
I was never able to take both of my kids anywhere by myself. It was impossible for me. Two kids were very, very hard. When it was just one kid, I had one of those little backpacks with a tether which people say are horrible, “you’re putting your kid on a leash,” but it worked for me. We developed code phrases which I still use. If I say, “here I am” or “Ian, sound off” then he knows he has to say, “here I am.” To this day, they both know when we’re in a store and I say “Sound off”, they have to say, “Here I am.” They know they will get in some serious trouble if they don’t.
Because of my poor balance and lack of stamina, I use a wheelchair when we have extended outings, like the Nashville Zoo, which we recently visited. The kids fight over who gets to push Mommy. People have all the apps now on their phone that have these instructions you need to read. I could use speech on my phone, but I have kids for that now.
My husband has been such a huge help for me, always willing to do anything to assist. And not just as a babysitter. He thoroughly enjoys being involved with his children. Michael had to do some adjusting in terms of his job. Before the children, he had a good career in computer technology. After our son was born, he became a teacher at the school where our kids would eventually go to school. That enabled him to spend more time at home and enabled me to attend more school functions, but it did mean a cut in pay. We had to make that sacrifice; he took a lower paying job so that we could have more freedom. That trade-off is still something that many parents, particularly women, have to make, regardless of a disability. Parenthood can affect your career progress. It did my husband’s because he wanted me to be able to be there for our kids. We could not move further away for his job either, just because I needed to be close to my mother since she helps a lot with the kids, like picking them up from school.
My own expectations of myself and trying to be what is quote, unquote, normal, were the biggest hurdles I had to overcome in parenting. I was so worried about telling my children that I couldn’t see. When they’re babies, they don’t know; they can’t figure it out. When I finally decided to tell my son that Mommy couldn’t see, it was such a big overwhelming deal in my mind that he would view me differently. He absolutely did not care. Mommy was still Mommy. Nothing changed about how he did things. It was no big deal. He probably didn’t even remember it the next day. My daughter, on the other hand, had a different response and this says something about her personality. She said to me one time, “Mommy, I’m very sorry you’re blind.” I said, “That’s nice of you to say.” And she said, “Because you can’t see how beautiful I am.” That is totally my daughter.
Parenting is hard no matter who you are. It is hard every day. It’s emotionally hard. It’s physically hard. It’s taxing. It’s hard for everybody. But it’s very, very worth it. It changes your life; your whole lifestyle is different. But if you’re ready to do that, then I don’t think disability should ever stop someone from parenting.
I guess you could say it’s harder if you are blind. I did have some fear that if I was bad at it, they, whoever “they” are, would come and take my baby away from me. I’ve heard that concern from other women with disabilities. I shared it. I think, as with so many things, the biggest enemy was myself, what I thought, my own fears, what people would think of me. I remember I was all torn up that I couldn”t drive. Public transportation in the small city where we live is virtually non-existent. My husband worked, but I decided to stay home with my son. I was worried I couldn’t drive him to places like to the playground like my Mom did for me. I would stay up feeling guilty about that. He did not care because he did not know any different. It did not bother him. He was just as happy playing in the backyard or playing with Legos or whatever. I am probably one of the few parents who is looking forward to their children reaching driving age. When Ian turns sixteen, I’m going to have a driver at my disposal!
I have never let my disability stop me from doing anything. That includes being a parent. A disability gives you a different worldview and probably greater empathy. We definitely need more people with empathy and understanding. There’s a lived experience that disabled people have, it’s hard fought and it can take a lot of blood, sweat and tears. We’ve earned that lived experience and can pass it on to our children and share it with them. You have to tell your children how important it is to not judge others, to always be there with empathy, love and acceptance for other people.
As I mentioned earlier, I had to have extensive physical therapy after my surgery in 2016. For one thing, I didn’t have any use of my left arm. I couldn’t give my kids a two-arm hug any more. The therapy made it possible for me to do that. It was so wonderful to put both arms around them again. I really missed being able to hug them, to hug them good. I can do that now.
(Editor’s note: One of Kimberly’s two children has Von Hippel-Lindau Disease