For years, Wayne Cook III thought he was the only child in his family to escape a bleeding disorder. One of his younger sisters had moderate type 1 von Willebrand disease (VWD). His other younger sister had factor IX deficiency, and so did his father. But just after Wayne turned 18, he too was diagnosed with moderate type 1 VWD.
“All of a sudden I didn’t feel like I was a normal person. That was a tough time because I didn’t know how to react, how to take it and what I would have to do to take care of myself,” recalls Wayne, now 30. He is a client relations executive with CVS Caremark in Sanford, North Carolina. A few months after his diagnosis, at an event for the Bleeding Disorders Association of Northeastern New York, he met 15-year-old Morgan Ryan. She had a bright smile and a bubbly personality. She had also been recently diagnosed with moderate type 1 VWD. “After getting to know her, I was more calm, seeing how she had the same condition and she was able to live a normal life with no problem,” says Wayne.
Wayne and Morgan’s shared experience living with a bleeding disorder made their bond stronger. They were able to skip a lot of the awkward conversations that many people with bleeding disorders feel pressured to have in a new relationship. “There were times when Morgan and I were both sitting with tissues up our noses because we were having nosebleeds.” (This is not the recommended way to treat nosebleeds, however. For more information, see the HANDI brochure “Nosebleeds.”)
In August 2006, Wayne and Morgan got married. Now, after five years of marriage, Wayne and Morgan, 27, are looking forward to starting a family. For some couples in which both partners have a bleeding disorder, the decision to have children is full of excitement. For others, it’s full of angst. Experts agree that the key to deciding whether to have kids lies in making an informed decision and knowing what to expect.
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Making the Right Decision
Children born with a bleeding disorder can usually attribute it to only one parent’s genetic mutation, not both. In the rare cases when both partners have a bleeding disorder, the couple should consider how it might affect their future children.
Wayne and Morgan already know there is a 75% chance their child will have a bleeding disorder. Wayne talks about it with a nonchalant calm: “I think our child will be lucky because he or she will be coming into a house where we already know what to do.”
National Hemophilia Foundation (NHF) Medical Advisor Marion Koerper, MD, never advises such couples to forgo having children. “They have to make their own decisions. I don’t want them 10 or 20 years from now saying, ‘I really wanted to be a father or to have that sensation of a baby growing inside me, and you took that away from me.’” She is also director emerita of the HTC at the University of California, San Francisco, where she practices pediatric hematology and oncology.
Koerper has encountered several couples in which both the man and woman were affected by a bleeding disorder—often, one was a carrier and the other had a bleeding disorder. While some couples met at a bleeding disorders camp or at NHF events, most didn’t realize they both had a bleeding disorder until their children were diagnosed.
That scenario happened to Amy and Bill Selfridge of San Jose, California, a few years after their son, Zach, now 17, was born. Amy knew before she had children that she was a carrier for hemophilia A, but she didn’t know why her 3-year-old son continued to have breakthrough bleeds even though he was on daily factor VIII prophylaxis. Zach had nine joint bleeds before his first birthday. “My brother had severe hemophilia with inhibitors, so I knew the signs. Something wasn’t right,” Amy says. “I wanted the whole family to be tested for VWD.”
Both Zach and his sister, Mollie, who was 5 years old at the time, had dual diagnoses: Mollie, now 19, was diagnosed with mild factor VIII deficiency and mild type 1 VWD, and Zach with severe factor VIII deficiency and mild type 1 VWD. “Bill was shocked when the doctor told him that the VWD came from him,” Amy recalls. “He didn’t really accept it until after his brother was diagnosed with VWD five years later.”
The Puzzle of Possibilities
Koerper advises couples to begin genetic counseling before they decide to start a family or as early as possible in the pregnancy. This allows the couple to know their children’s chances of inheriting a bleeding disorder and decide how to proceed.
When both partners have a bleeding disorder, there are several possibilities in each circumstance. For example:
- If the father has hemophilia and the mother is a carrier for hemophilia, they could have an unaffected son, a son with hemophilia, a daughter who is a carrier or, if she has inherited the disorder from each parent, a daughter with moderate to severe hemophilia. Each possibility has a 25% chance of occurring.
- When the father has hemophilia and the mother has VWD, there is also a 25% chance they could have an unaffected son, a son with VWD, a daughter who is a carrier for hemophilia, or a daughter who is a hemophilia carrier and has VWD.
- When both partners have type 1 VWD, the sex of the child is not relevant, because VWD is not passed through the genes that determine sex. There is a 25% chance they could have an unaffected child, a 50% chance they could have a child with type 1 VWD, or a 25% chance they could have a child with severe type 3 VWD, Koerper says.
Regardless of the scenario, if the couple has a daughter who inherits a bleeding disorder from both parents, they need to be prepared for the severity of her condition. The daughter will most likely have long, heavy menstrual periods and require prophylactic factor infusions, says Koerper. She may also have a higher risk of miscarriages.
“It’s important to know the possibilities before the pregnancy so that they can decide whether they want to go through with the pregnancy, and there are no big surprises when the baby is born,” Koerper explains. “They can get themselves emotionally prepared to accept the child and know what to do when the baby has a bleed.”
The Game Plan
Couples who are expecting an affected child must have an outline of the treatment options and a plan for labor and delivery, says Connie Gibb, MS, CGC, a genetic counselor at Weill Cornell University Medical Center in New York.
Some of Gibb’s patients have said they don’t want to know the child’s potential diagnosis or sex. “They don’t want prenatal testing because they would never terminate and are very comfortable having a child with hemophilia. Some patients want the surprise of having a boy or girl.” While the choices are up to the parents, Gibb makes sure the ob/gyn knows that the baby might have a bleeding disorder.
Gibb devises a written labor and delivery plan that tells the ob/gyn the baby’s confirmed or possible diagnoses. This allows the labor and delivery team to minimize the risk of bleeds during delivery, determine if a cesarean delivery is more appropriate and have factor on hand during the birth.
Treatment for bleeding disorders has come a long way in the past few decades. “I try to tell parents that 40 years ago, kids weren’t treated prophylactically like today,” Gibb says. “Now they can be treated at home in minutes and are able to play noncontact sports.”
That’s how Wayne and Morgan intend to raise their children. “The best thing that has happened for me and my wife is that we had parents who didn’t hold us back,” Wayne says. “They allowed us to just be human, living life and knowing the restrictions. And when we have children, that’s exactly what we plan to do.”