Renée's Story

I was diagnosed with VHL during my first pregnancy. The pregnancy itself went without a hitch. Because I have no adrenal glands, I was referred to a high risk pregnancy unit. When I gave my medical history, the astute obstetrician suggested I see the resident geneticist.

During my first appointment with her, at five months pregnant, she told me that she could give me a clinical diagnosis of VHL based on my medical history, and could send my blood to Philadelphia for the genetic test. I agreed, but I was terrified to learn too much about VHL. So when my oldest was six months, I hurriedly became pregnant again, before I could learn anything that might make me change my mind about having more children. I have always wanted to have a big family.

The second pregnancy, from July of 1998 to March of 1999, was a difficult one for me. I had two spinal tumors, both located between L3 to L5, one about 2 cm and one 3 cm. I did not know prior to the pregnancy that I had these tumors, and in fact, did not find out (for sure) until three months after my son was born that they were there. I was only diagnosed with VHL in 1997, and my doctors knew very little about VHL and I was too frightened to do any research on my own.

But as the pregnancy progressed, I was suffering from an unusual amount of back pain, along with some numbness and weakness in my legs. This prompted me to do a little research which led me to suspect I had a tumor on my spinal cord. There was very little to do about it at that point, and I am quite sure my obstetrician thought me a hypochondriac (even though I did give him the literature on VHL), so I just carried on as best I could. I took Tylenol with codeine for the back pain and tried to vary my position as frequently as possible. I spent about three hours of the night in bed, followed by an hour of pacing around my kitchen waiting for the next pain-killer to kick in, and another three hours in my lazy boy chair. I prayed for the pregnancy to end early, and even asked my obstetrician to induce me at 34 weeks. With a one-year-old at home to care for, I truly felt I could not carry on. He refused, but at 35 weeks my water broke and Michael was delivered on March 30, 1999. He spent almost 3 weeks in the neo-natal intensive care unit, and came home on April 17.

I was not checked before and after my first pregnancy, only after my second, and then only at my insistence. My family doctor (also not familiar with VHL) thought it was "overkill" to have an MRI following the pregnancy, even though I gave him the VHLFA Handbook and showed him the part about having an MRI yearly. He told me that I didn’t have "that kind of VHL". Good thing I was tired of keeping my head in the sand, and smart enough to realize that doctors might not always be right. I insisted on the MRI. I had surgery to remove the tumors on May 25 of 2000, after putting it off for as long as possible. I didn’t feel I could have the surgery until Michael was a year old.

The surgeon removed one tumor. When I woke up he told me that there had only been one tumor, and that it was bent over on itself to make it look like two on the MRI films. So I went about my life and assumed that my lingering leg numbness and weakness were residual to the surgery. I paid very little attention when the numbness crept into more areas, assuming that it had always been like that and I just hadn’t noticed. Luckily, in May of 2001, I was invited to the NIH. They, of course, found the remaining tumor and I had surgery to remove it in August of this year. So, although I have been left with numbness, and weakness in my left leg, and bowel and bladder difficulties, I do not like to say that these symptoms were related to or caused by the pregnancy. I think if both tumors had been removed in one fell swoop, I would have had a better outcome. If I do decide to have another child (not very likely at this point) I would certainly be checked before and after the pregnancy.

So what have I learned from all this? Go to the experts, even if I have to travel to do it. Trust myself; I know my body best. And, educate myself and my doctors. It is in my best interest to be an expert in VHL. After all, I am the one with the vested interest. My life, and my sons’ lives, depend on it.

See also the medical article Pregnancy in VHL, with important information for the health care team.

As printed in the VHL Family Forum  9:5, December 2001.  For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.

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