29 June 2008

Dave's Insights Into VHL

And now, this is Dave's post. (To learn about basic information regarding VHL see my previous post.):

What’s it like having VHL?

This is probably the question that most of you are asking right now. Well…there’s no one answer. I have been exceedingly blessed with my VHL, I have had two surgeries (both to remove a pheochromocytoma), I currently have a small mass in my left adrenal that has remained stable, and numerous micro-lesions (very, very, very tiny tumors) in my right kidney. Others with the disease have had double digit eye surgeries by the time they are 10. Really, as long as I am spared tumors in my central nervous system, I will consider myself blessed. There are lots of ways having VHL has impacted my life. I take trips to the National Institutes of Health (NIH) on a regular basis, I can’t do Aikido anymore, I’ve known an internationally renowned physician/scientist since I was 11, and, it has always meant that I could pass it on to a child. The last has had the greatest impact on my life, and will continue to do so.

There’s a lot that I’m worried about. Different people manifest the disease in different ways. While our daughter has my exact mutation, it doesn’t necessarily mean that she will manifest the disease in the same way, my mother and I have very different medical histories, and no one is exactly sure why. I am terrified of hearing “I’m sorry, but your daughter has a tumor in her cerebellum.” On a more selfish note, I worry that she will blame me. Valerie and I went ahead with the knowledge that I had this disease and that there was a 50-50 chance that it could affect our daughter. How will she react to hearing, “yeah, we knew there was a 50-50 chance you could get it, but we went ahead anyway.” On the other hand, I know that I’m very glad to be alive, and have never (at least, not that I can remember) blamed my parents for “giving” me this disease.

So, what does the future look like? In many ways, I don’t know, all we can do is take it one day at a time and hope for the best, but how is that any different from what everyone else in the world does? We’ll let the NIH know, and hopefully she can be included in the VHL study. I’ll do my best to encourage her to be curious about what is going on inside herself. I’ve always been extremely thankful that my parents encouraged me to be curious and interested in VHL, rather than to feel helpless and subjugated to it. A desire to know more about something is very powerful, and being curious has turned VHL from something terrifying to something that honestly excites me. Most importantly, I will love her. Valerie and I will both be there for her through everything. We’ll hold her hand when she needs to have an IV put in. We’ll be sitting just outside when she has her first MRI. I’ll be right next to her drinking my awful CT contrast while she is drinking hers. I wish more than anything that she never has to experience any of this, and I feel beyond terrible that I have done this to our daughter, but with no other choice, we’ll just have to make the best of it. In the end, I’ve always thought that VHL has had a positive impact on my life, I just didn’t want to pass it on.

The bottom line? We love our daughter and can't wait to see what the future holds, with or without VHL.


Lindsey said...

Very nicely put, Dave. Your daughter is so blessed to have a father like you.

babystephanie said...

The opportunity to know her will make it all worthwhile.

Ria said...

Dearest David,
Having VHL didn't change your's or your mothers attitude. I had a long phonecall with her on saturdayevening because I know she is worried also. I do have a lot of Dutch frases to say in my mind, but it is hard to express it for me in English. You and your family have been dealing with this disease for so many years and David in all those years I know you, you have been growing up to a strong and marvelous person. Just like your Mom didn't give it on purpose to you, you haven't been giving this on purpose to your daughter. She will be loved so much by you and Valerie. For sure I'll know you will be on her side when things will be difficult or uneasy for her. You will be a great father and Valerie will be a great mother, I hope to see you soon enough again to close both of you and the baby in my arms. I'm looking forwards to be a Dutch Oma for this little girl and I will love her so much. Your whole Dutch family is sad about what is happening right know, but we have faith in future knowing she will have wonderful parents and other family to stand beside her.
Lots of love,
Ria, Willem and Johan

julesc-t2007ju14 said...

Thank you so much for taking the time to share more information about VHL and your feelings about it. You did not do this to your daughter. As parents, we want the best for our children. We give them all that we are, and we do the best that we can. I can remember wishing that my children had pause buttons; I started parenthood at 20 and it was a rough row to hoe for us all. They forgive me for my imperfections and love me in spite of it all. I am so glad you two will know the joy of parenthood! God bless you both! XOXO

Siwan said...

Dearest David and Val;
When Charlie was six months in utero, we found I had protein C activity deficiency. We had a few scary weeks until Tom was tested before we knew Charlie wouldn't be homozygous for it (fatal). I remember that feeling very, very well.

And I remember something I read at that time, too: when you have a child, you give a hostage to Fate.

Yup, you knew about the 50% chance of VHL. Until YOUR child is old enough to want to marry and have children, she won't know what it's like to want your own child. Anybody having a child is taking a risk with known and unknown genetic issues. Your daughter is LUCKY: you live in a society where you chould KNOW what problems she might have and identify them even before birth and have her in a world class hospital. The rest is just about being human. VHL isn't Tay-Sachs - it's a spectrum (like the hospital!)

Tom and I love you both and are praying for you, your daughter, and your families.

Stephanie, Tom and Charlie