VHL and Pregnancy

I was diagnosed with VHL in January 1990, shortly after I had my right adrenal gland removed due to pheochromocytoma. In July 1991 I had my first brain surgery to remove a VHL hemangioblastoma. About a year later my husband and I were thinking about moving on with our plans for pregnancy, but were concerned about VHL. At this time, I had a 5 mm. cerebellar tumor, and a 1 cm. spinal tumor at the third cervical vertebra (C3) that had been located just after my first brain surgery. My father had VHL, but hadn’t had any problems concerning his brain or spine. We had considered having children, but were uncertain of the effects this illness might have on pregnancy.We tried in vain to find anyone who could tell us anything about it. Even a visit to a medical geneticist from a major university proved disappointing. At this time prenatal testing was unable to tell us whether our child would carry the VHL gene. But even if genetic testing had been available we would have turned it down. The doctor could not do amniocentesis‡ testing until I was four months pregnant, and there was some risk of injury to the fetus. Even if the baby had tested positive for VHL, we would not have consented to an abortion. We decided to move on as planned. Besides, the VHL in my family wasn’t "that bad." We felt even if our child were to carry the gene, by the time he or she were old enough to exhibit signs of the illness, VHL research would have advanced to the point where a drug or therapy would be available to prevent the illness from manifesting itself. In July 1992 I became pregnant. By the middle of February 1993 the 5 mm. tumor was now 4 cm. large. I was immediately put in the hospital. The neurosurgeons decided to place an Omaya reservoir* in my cerebellum at the spot of the tumor so the cystic fluid could be drained until the baby was born, and I could have the surgery to remove the tumor. (The fluid surrounding the tumor had to be drained every 3-4 days). My daughter was born by cesarean section in April 1993. Six weeks later the tumor was removed. My follow up MRI showed another tumor that had been directly behind the second one. In March 1995 the third tumor was resected. Follow up MRI’s showed nothing.

In January 1996 I found myself pregnant again. My husband and I were extremely scared. A MRI in June showed no new developments. I was elated, I knew I was going to make it through! In October of 1996 my son was born. (We decided to go with cesarean due to our worries of intercrainial pressure during childbirth.) At my check-up six months later, my MRI’s showed no changes in my brain tumors.

Today I have no brain tumors, though the spinal tumor has showed some slow swelling. My husband and I are thrilled with our two bouncy little children. I feel it is very important for women with VHL to get all the medical information they can before and after each pregnancy. It was the cooperation of my medical team that helped manage my health and that of my daughter through my first pregnancy.

*Omaya reservoir is a device surgically inserted under the scalp which is used in chemotherapy to deliver drugs directly to the tumor bed or into the spinal fluid. The drugs are then given through the reservoir, rather than through the back during a spinal tap. In Lisa’s case the device was used to draw off fluid from the cyst.