Adventures in Pregnancy

He’s ten, our pheo baby. I was happy when he learned to read, happy when he talked, happier still when we learned he does not have an alteration in the VHL gene. He gets math concepts very well, but I can’t help wondering if my pheo contributed to his Attention Deficit Disorder (ADD) and sensory integration problems.

Pheo problems have seemed to run in our family for years. My parents are both hale and healthy, but my brother had pheos on both sides at the age of 11. That was 1962, and at our local hospital they didn’t take children’s blood pressure. "They’re children! Of course it will be high!" But he had bilateral adrenal masses, and he was admitted to the adult unit, so they took his blood pressure before beginning surgery — it was 300. There were no catecholamine blocking drugs in those days. They proceeded with the surgery, and my brother died on the table from a pheo-induced heart attack, one month after his 11th birthday. My parents were told not to worry, that "it never happens twice in the same family."

In 1969, when I was 10, my mother noticed that I was choosing short-sleeved dresses in winter time. This time my parents decided to take me to the University of Michigan in Ann Arbor, the largest teaching hospital three hours from our home. Dr. John Bartlett diagnosed pheos on both sides. But he used a new approach. He used blocking agents to keep my heart rate from soaring. He removed bilateral extra-adrenal pheos at that time — both outside the adrenal glands — and I was able to keep my adrenal glands. I recovered well from the surgery.

In 1975, while in college, I developed some large blind spots in my eye. I went first to the optometrist who suspected an infection and sent me to my regular doctor. He sent me to Ann Arbor, admitting that he had no idea what it was. The doctors looked at my pheo history, and just to rule it out, they sent me off to "do a jug" — a 24-hour urine collection. It came back positive for pheo. They had a hard time locating it, but finally found a pheo on the right ganglia of my spine, again outside the adrenal gland.

While I was in the hospital I met a charming nursing student — not assigned to me, my husband hastens to add! — and we began dating. We married and decided to have only one child because of my health issues. Our David arrived in 1985, following a very uneventful pregnancy. He had a mild abnormality in his heart, pulmonary stenosis, but it was controllable and he is a normal active child, a scholarship student at college with a black belt in Tae Kwan Do.

Eighteen months later, in 1986, our daughter Kate was born. Again it was an uneventful pregnancy, but she too had pulmonary stenosis, this time much more severe, critically ill from birth. She had corrective heart surgery, but died at the age of 18 months in 1987. We went through some genetic counseling then, since we had had two children with pulmonary stenosis, but were not given any helpful information. They suspected that I might have Multiple Endocrine Neoplasia (MEN), but the heart problem didn’t fit the syndrome. My mother had had thyroid cancer, but it was not the type of thyroid cancer typical of MEN, so they were still unsure.

When David was four, my husband was offered a job transfer, and we were pondering the move when David declared that he wanted to move to Grand Rapids, and he wanted a baby brother. Within the next few years we were able to give him both.

When I was 6 weeks pregnant, the normal work-up showed that I had some gestational diabetes, and that my blood pressure was quite high. They "did a jug" and found evidence of a pheo. The first endocrinologist told me that I should abort the child. My obstetrician, who had recently managed a patient through a successful pregnancy with a cancer of the adrenal gland, was concerned but open to discussion: "Clearly we need some advice here in how to manage this tricky situation." So I hunted down Dr. Bartlett, who was then in the navy.

"I’m pregnant," I blurted out. "Congratulations!" he said. "And it looks like I have another pheo." "Oh, dear!" "And I really want to keep the baby."

"Well, of course you can keep the baby!" he said, and proceeded to provide sensible advice for me and my obstetrician.

They blocked me up like crazy, and removed the pheo in the 13th week of my pregnancy. After the pheo was out, the diabetes also went away. Evidently when the catecholamines are high, it can cause the sugar to rise as well.

The remainder of the pregnancy went well, and Brian arrived as scheduled, with a vaginal delivery, weighing ten pounds. We saved amniotic fluid and the umbilical cord for genetic analysis and research.

Later I went to a new endocrinologist, Dr. Tennyson. He read through my history and noted that my brother and I had both had pheos at 10-11 years old. "Your son is now 10 — has he been checked for a pheo?" With all the excitement of Brian’s birth, I had forgotten the promise I had made to myself to have David checked at 10. Dr. Tennyson ordered the tests, and sure enough, David’s catecholamines were 20 times the normal levels. They took him off Ritalin, and the levels dropped to 10 times normal. He had a pheo.

Dr. Tennyson suggested that perhaps NIH would like to review our family’s history. So off we trooped to NIH for analysis. As my husband and I were talking the night before David was scheduled to have bilateral adrenal tumors removed, we decided to make up 3x5 cards of the messages we would like the doctor to tell us. We wrote them out one by one: "He’s fine." "The surgery went well." "Yes, we saved the gland." "I’ve never seen anybody heal like this!" "Gee this boy has nice parents!" "He can go home soon." It helped to keep us focused on a positive outcome. When we handed the cards to his surgeon, Dr. Walther, he laughed. After surgery, however, he opened his folder and began handing the cards to us one by one. All the good messages had come true.

At NIH they did a full work-up on David and me, and for the first time said "VHL". They found that I had a pancreatic islet cell tumor, so David’s surgery was closely followed by my partial pancreatectomy. My parents were also screened for VHL as part of the NIH protocol. My father tested positive for the VHL gene, but still has no tumors, not a single retinal angioma, nothing.

In 1996, the two boys were sleeping together on vacation. When I woke them I noticed that one of the sheets was quite wet. At first I though the younger boy had wet the bed. But instead, David was having a sweating attack -- another pheo at age 13. They immediately put him on blockers, which made him very tired. After the flank-cut surgery, he bounced back quickly and two months later earned his black belt in Tae Kwan Do. Today he is at college, getting A’s in Chinese, working up to a belt in Kung Fu. He is an exceptional boy. He did not read well until second grade, but then went through what the Montessori school calls "a reading explosion" and has doing extremely well since then.

My husband attended a medical conference where they were talking about the learning problems of babies who are born addicted to crack. The problem, they said, is the catecholamine surge that babies get during pregnancy. Catecholamine surge? — sounds like what a pheo generates too. It has made us wonder whether Brian’s ADD and sensory integration problems might be attributable to the catecholamine surges that he underwent during the early stages of my pregnancy.

We’ve gone a couple of years now without surgery. For so long I remember VHL defining my life. Now my second son, Brian will be in sixth grade and won’t get a pheo. He has been tested for VHL and does not carry the mutation. What will that be like? My fears still come up, and I have to remind myself that I don’t have to worry about VHL for this one.

I try not to cripple myself or my son with this condition. I told David when he was 11 that he could go through these VHL episodes and come out the same, or he come out stronger. David still has one adrenal gland. He is very healthy, has a strong spirit, and is in a good spot in his life. He has a good understanding of people and their trials and tribulations, but doesn’t wear it like a cloak or use it like a crutch. We try to make NIH visits a vacation, with trips to the Air & Space Museum.

The doctors and hospitals have learned so much about patient care just during the time we have been observing. In 1986 I had to sleep in a recliner chair near my infant daughter. In June 1987, the University of Michigan had created a "Mom-spot" for every bed — they had learned that the patients did better, and it was easier on the nursing staff. Kate and I worked as a team. I was able to ground her and give her comfort, which in turn comforted me.

I can’t say enough about the wonderful people who have helped us through all this: the many outstanding doctors, and the many wonderful friends. Cia Manolatos and Kathy Hurley at NIH are phenomal people. Cards from classmates, phone calls and offers to cook meals, all are much appreciated. And my friend Rosemary Anderson gave me the best gift of all — she hooked me up with the support group of the VHL Family Alliance. As I have spoken since with people with a variety of genetic diseases, they tell me that when they hooked up with a support network, that’s when things began to get better. We’re really glad we’re here.

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.

mystory