In the month of July, my mother MaryBeth and Aunt Laurie went to the VHL conference in Rochester, Minnesota, where they gained a lot of information about VHL. MaryBeth has VHL, and their mother died of VHL at a young age. All the people in our family who have VHL have had only brain tumors. I had been tested in the past for brain tumors, but I never had DNA testing to look for the gene.

Sitting in the meetings, hearing the many warnings about the importance of checking pregnant women for pheochromocytoma, MaryBeth began to wonder about my pregnancy. I was seven months pregnant. I felt sick every day and had been in bed for the past two months. They said my blood pressure was high, and the medication they gave me did not seem to affect it. We thought it would be a good idea to visit my family physician and talk to him about this.

He thought it would be a good idea to perform an ultrasound since there was unusually little movement from my baby. Also he ordered a 24-hour urine test to check for a pheo. After doing the ultrasound there was concern for my baby’s health. He said that there was slow head growth and a very low level of amniotic fluid surrounding the baby. He referred me to another center for a more extensive ultrasound. The results were the same, and they ordered more tests.

My blood pressure was dangerously high, 199/149. The doctors were unable to stabilize my blood pressure, and increasingly worried about the baby as well. They did an MRI of my chest and abdomen, and found that I had a pheochromocytoma, a tumor on my adrenal gland which secretes adrenaline into my body, causing the elevated blood pressure. This tumor began to explain all my symptoms of illness. The pregnancy might have spurred the growth of this tumor, causing increasing problems. They kept me in the hospital a few days and decided to deliver the baby within the next two weeks. My condition became more dangerous. They transferred me to Intensive Care, did another ultrasound, and decided to deliver the baby as soon as possible for fear of my own and the baby’s health. I was rushed down for emergency surgery to deliver my baby and to remove the tumor.

The surgery went well and I had a 3 lb 15 oz baby girl, whom we named Hanna. The next few days after surgery things were still not looking good. The baby was in good health and was doing well. But blood clots had developed in my lungs, and some additional complications. After another day and many prayers, things began to get better for me.

I truly believe that God helped my baby and me survive through the tough times, and I hope that He can be of help with many others with VHL. -- Emily P., Minnesota

What Laurie learned:

We are so very grateful for the VHL Family Alliance and the meeting in Minnesota, and for Joyce’s "VHL 101" class. At least three times we were reminded that everyone with VHL should be checked for a pheo before any surgery, before or during pregnancy, and before labor and delivery. We finally heard it, and used that information to protect Emily and Hanna.

Our family always felt that we only got brain tumors. We never knew of anyone in the family who had anything else. My mother died of a brain hemorrhage during her fourth pregnancy, and we always thought that too was a brain tumor. Now that we know more about pheos, however, we realize that that was much more likely a stroke brought on by a pheo, so frighteningly like Emily’s experience.

Our family wants to thank everyone for sharing their stories, Dr. Michels and the VHL Board of Directors for providing us with the valuable information that saved Emily’s life, and will help keep her and her sister monitored and safe. Our family has been through a great emotionally stressful ordeal in the past few months, and we sure appreciate the support and help we have received. Thank you so much! — Laurie D., Minnesota

The VHL Family Alliance would like all obstetricians and gynecologists to know the importance of testing for pheochromocytoma in all cases of uncontrolled high blood pressure in pregnancy. Pheos may be rare, but they can be deadly. And among people at risk for VHL, they are not rare. Take the time, get the test, be safe.

For additional information on DNA testing, see Q&A article in this issue.

For additional information on the latest in inexpensive testing for pheochromocytoma, please see "Finding Elusive Pheochromocytomas". The test reported in this article has now been confirmed by a second research team. This test is becoming increasingly available.

As printed in the VHL Family Forum 8:4, December 2000. 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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