The first time Rita Genesse held her newborn daughter Bryanna in her arms, she says "it was a miracle - a beautiful moment." Genesse and her husband had been trying for six years to have a child. There was a miscarriage. And there were the numerous fears to deal with. These were not only the usual ones most women have, but a host of others because Rita Genesse has hydrocephalus.
It’s only been in recent years that women like Genesse have been having children. The reason being that over 30 years ago, the prognosis for people with hydrocephalus was not good - many didn’t survive. Thanks to advances in medicine, women with hydrocephalus are reaching childbearing age and having children. But there is little published information on the subject. Still, this hasn’t stopped women with hydrocephalus from giving birth. "If you want it bad enough, you’ll do anything to have a child," says Genesse from her home in Cortice, Ontario.
The rewards of having children seem too far outweigh the fear of the unknown. Californian Nancy Bradley, who has been shunt dependent since birth, knows this all too well. Bradley has been working on a computerized database since the summer of 1994 that is designed to collect information about pregnancy and maternal hydrocephalus. She has talked to many women with hydrocephalus who were thrilled to have children despite the unknown. Bradley herself was inspired to have a second child after making contact with other women who had had a second.
But it was Bradley’s need to "find out if there were other people out there who had shunts and children" as well as learn more about the effects of pregnancy on women with shunts that prompted her to start the database. "Six weeks after my son’s birth my shunt malfunctioned and I had to have surgery," she says. My neurosurgeon wasn’t sure if there was a connection, but my husband and I believe that the revision and the delivery were somewhat related."
Well into her second pregnancy, Bradley is still busy working on the database. She’s fatigued at times, but realizes the importance of providing useful information to both women and their doctors. Her database is the only one of its kind in North America, covering everything from the number of pre-delivery shunt malfunctions to types of delivery.
Although Bradley’s study is small, it is encouraging to note that except for one miscarriage, all pregnancies had successful outcomes for both mother and baby. There were, however, a number of shunt revisions required during pregnancy as well as after delivery.
A paper by a group of doctors from both Canada and the United States called "Management for Pregnant Women with Cerebrospinal Fluid Shunts" published similar results. In their review of 17 cases, the doctors found that fetal and maternal outcome was excellent in the majority of cases. They also reported that 25 per cent of the mothers with ventriculoatrial shunts (VA) and 46 per cent of mothers with ventriculoperitoneal shunts (VP), experienced shunt obstructions during their pregnancies.
Still, a more recent paper, "Maternal Neurological Shunts and Pregnancy Outcome" by J.B. Landwehr, Jr. et al offers different results. Of the 25 cases examined, fetal and maternal outcome was not always positive, for example there were five miscarriages. A most interesting note is that there were no related shunt complications.
Because there have been relatively few case studies done to date, it is difficult to determine whether there are any trends emerging. Doctors do know that during pregnancy women with hydrocephalus may experience severe headaches. Shunt malfunctions can occur, and women may have more nausea and vomiting in general. It is up to a doctor to run tests to determine whether problems are the result of a shunt malfunction or simply part of the pregnancy. Doctors like to avoid CAT/CT scans because they can expose a fetus to radiation. Instead, they prefer to conduct less risky checks such as eye examinations.
Doctors involved in this area seem to feel that a woman with hydrocephalus should be treated with special care. This means being monitored closely. But it is up to the woman to ensure her obstetrician has an understanding of hydrocephalus and that both obstetrician and neurosurgeon communicate. When Rita Genesse became pregnant her neurosurgeon, Dr. Fred Gentili of The Toronto Hospital (Western Division), provided her obstetrician with a letter explaining her condition.
Obstetricians should be made aware of the type of shunt a woman has because some procedures are not recommended with certain shunts. Dr. Gentili, for example advised Genesse’s doctor not to give her an epidural because she has an lumboperitoneal (spinal-abdominal) shunt. obstetricians must also know the signs of a malfunctioning shunt.
The more information obstetricians have, the better prepared they are to deal with a situation. Nancy Bradley has heard from women who have been told by their obstetricians to have abortions. "Its irresponsible to say this just because a woman has a shunt," says Bradley.
Rita Genesse has her own horror story about a doctor. When she couldn’t get in to see her own obstetrician while experiencing severe abdominal pains in the initial stages of her pregnancy, she was sent to see another doctor. He was so uninformed about hydrocephalus he suggested she have an amniocentesis to see if he child had cystic fibrosis. "The doctor had no concept of what I had," says Genesse. "I left his office in tears. I had nightmares I was going to die when I had the baby."
Dealing with the misinformed obstetrician made Genesse realize just how crucial it is for women and their husbands to find out as much as they can. "It’s important to be as knowledgeable as possible," says Genesse. "Make sure you have all your questions answered so you’re not afraid."
Bradley suggests that a woman find an obstetrician who works with a team or has a partner. This way, if the regular obstetrician is not on duty when the woman goes into labour, his or her partner who is familiar with the woman’s condition will deliver the baby. If this isn’t possible, the doctor delivering the baby should be made aware of the hydrocephalus and the presence of a shunt.
No matter how much a couple plan for the birth of their child, they may still have their fears. "It takes a lot of thought between you and your husband," says Bradley. "There is a chance that something can happen and it is stressful if something does."
This "something" for women whose hydrocephalus is associated with a neural tube defect, such as spina bifida, is the risk of the infant developing a similar problem. As limited as the studies are, they do show there’s a slim chance that the woman’s offspring will have a NTD. Less-risky tests, such as the alpha-fetoprotein blood test can be done to determine this, however, they are not always 100 per cent accurate. If AFP levels are not normal, though, further tests such as an amniocentesis, can be done. If you are concerned about genetic factors, you may want to contact your doctor or a genetic clinic. Genetic clinics can provide counselling, conduct physical exams, and arrange for labratory tests.
There are uncertainties with almost any pregnancy. As Bradley and Genesse both know, maternal instinct is a powerful thing. And somehow the desire to have a child seems to far outweigh the fear of the unknown.