HEMOPHILIA AWARENESS

APRIL 17 TH

Hemophilia Awareness

Nathan receiving a medal at championship finals. Swimming is a fabulous exercise for those with bleeding disorders.

The Snareys persevere against medical challenge together

Family traits are passed down through generations. Sometimes a mom will gush, “My son has his grandmother’s eyes.” Other times a dad will proudly announce, “My girl won state at basketball, just like me!” On April 17, 2022, one inherited trait is recognized globally. World Hemophilia Day is a day to bring awareness to a characteristic passed through families, hemophilia and other bleeding disorders.

A rare genetic disorder, hemophilia occurs in 1 of 5,000 male births. The Centers for Disease Control and Prevention (CDC) states that hemophilia is an “inherited bleeding disorder in which the blood does not clot properly.” Those with hemophilia have low levels of proteins called “clotting factors” in their blood, which can lead to spontaneous bleeding and bleeding after injuries or surgery.

Evan running cross country this fall. Exercise is important to keep strong healthy bones and muscles, which helps reduce bleeding episodes. Little brother Nathan enjoying a shaved ice more than the races.

One family in Monett is very familiar with hemophilia. Mareena Snarey, a teacher at Central Park Elementary, said that her father was born with hemophilia and was very active in the bleeding disorders community. “He was president of the National Hemophilia Foundation around 1980, but like many of his peers, contracted HIV from tainted blood products and died from AIDS in 1986,” Snarey said. “Those were tough years for the hemophilia community and our family.”

After losing her father, Snarey’s family moved from Texas to California and didn’t have much involvement in the bleeding disorders community until her son, Evan, was born in 2008. Evan was diagnosed with hemophilia the same as his grandfather, which brought the Snarey family back into the bleeding disorders community. Going through treatments with Evan prepared the Snarey family for their youngest son to also be diagnosed with hemophilia.

Knowing that she was a carrier of the hemophilia gene, Snarey was aware that her children might inherit hemophilia, but she was surprised to learn a few years ago that she also had a mild form of hemophilia. “Half of our family has hemophilia. Two of our boys have severe hemophilia, less than 1 percent factor 8 in their blood,” Snarey said. “I always knew I was a carrier, or one who could pass hemophilia on to my children, and that I would bleed and bruise easier than the average person, but never considered myself as someone with a bleeding disorder.”

The Snarey family

You can’t tell from looking at them, but half of the family members live with a genetic disorder that requires regular treatments.

After an outpatient procedure, Snarey developed an abnormally large hematoma and decided to have her factor levels tested. “We discovered that my factor levels are only 18 percent of normal, meaning that I too am a mild hemophiliac. Now that I have been properly diagnosed, I have access to medicines that help control bleeding episodes that previously I would just deal with,” Snarey said.

Over the years, this mother of four and her husband, James, have learned to live with hemophilia and the challenges it brings. Two of their sons, Evan (now in 7th grade) and second-grader Nathan were diagnosed with hemophilia at birth. Their daughter, Elizabeth a freshman at Monett High School, and their middle son, Blake in 5th grade at Monett Intermediate School, did not inherit the gene to give them hemophilia.

With their first son, there was a learning curve living with and treating a hemophiliac. “We have a picture of Evan on his first birthday sucking a red lollipop with red drool coming down his face,” Snarey said. “The drool is actually blood though. He had a bleed in his mouth that we were trying to get under control. A nurse came to our house to administer his medicine, which required us to lay him on the kitchen table, with me holding his arms and laying over his chest pinning him down so that the nurse could infuse his medicine into a vein in his foot. We were lucky if the nurse was able to get him infused on the first try, but often we had to retry several times. Squirmy little kids with chubby appendages and nearly invisible veins made it so very difficult to find a vein and infuse medicine.”

Having cared for Evan for years, the Snareys were more prepared when their youngest was diagnosed. “Nathan got a bleed in his thigh from the vitamin K shot he got at birth,” Snarey said. “Without medicine, his little newborn muscle was swollen to roughly the size of an orange by the time we returned to the hospital five days later to try to give him medicine. It took seven attempts to find a vein, and he came home with a needle taped to his head for us to continue giving him medicine daily for the next week. Luckily, he was our second hemophiliac son, so I knew how to infuse his medicine, and immediately we saw improvement in his leg as the bleeding stopped and the swelling went down.”

Living with hemophilia isn’t simple. Having studied the disorder and living with it in Evan, they were prepared when Nathan’s body developed an inhibitor against his medicine. During the

Evan recovering from surgery in 2013 after having a port catheter placed to help infuse his medicine. Accessing a port is much simpler and easier than finding an arm or foot vein to infuse medicine.

Evan Snarey as a toddler holding syringes after receiving an infusion to treat his genetically inherited hemophilia disorder.

“Big, old veins are much easier to find than little kid veins,” Mareena Snarey said as she infused herself with factor 8 at home after a medical procedure. Those living with hemophilia learn at a young age about treatments and how to administer them. Before he was even 10 years old, Evan was self-infusing his medication.

event after his vitamin K shot, Snarey said that they tried to get his bleed under control on their own at first because they knew that introducing the clotting factor medication at such a young age could lead him to developing the inhibitor, which is what happened.

“An inhibitor is where his body created antibodies to the factor medicine, making it useless to the one receiving it,” Snarey explained. “Because he developed an inhibitor, at about a year old we started a regimen to attempt to tolerize him to the medicine by giving him high doses of factor daily for about a year. Eventually, his body learned to stop fighting the medicine, and he has been on an every-other-day regimen since then, for over seven years.”

As time passes, more is learned about hemophilia and new treatments and types of treatments are being developed. “Recently both boys have switched from an intravenous medicine that required finding a vein for each dose, and every-other-day or three days a week dosing, to a new subcutaneous injectable that they can take only once a week,” Snarey said with excitement. “They still don’t like the needle sticks, but they are simpler and less frequent and doing a great job of keeping bleeding episodes under control, so we are sticking with this new medicine for now.”

Looking to the future, Snarey wants people to know that hemophilia is much more manageable than it was when she was a child. “My dad walked with a limp because his joints had been damaged from repeated bleeding episodes,” Snarey said. “But today, thanks to huge improvements in medicines, the boys can lead a mostly normal life. There are some sports they still decline participating in, like football and hockey, because the risks of serious injury are still prevalent. But there is so much they still can do.

“The boys have both played soccer, but only recreationally. They also swim competitively with the Waterthrashers during the summer. Most sports are easy to manage, as long as we stay on top of our treatment regimen and pay attention to any injuries. We treat injuries as quickly as possible to prevent long lasting impairments.”

Snarey and her family have been active in the bleeding disorders community and are advocates for the disorder. While April 17, 2022, is World Hemophilia Day, the month of March is Bleeding Disorders Awareness Month. Organized by the National Hemophilia Foundation, this is a month where those living with bleeding disorders can share about their lives, stories, struggles, and successes. More information about this organization and bleeding disorders can be found at www.hemophilia.org. Also, the Midwest Hemophilia Association organizes a walk each year to raise awareness and funds for research. The next walk is June 11, 2022, in Kansas City.

Snarey encourages anyone who suspects they have abnormal bleeding to get it checked out with their doctor. “Women often deal with abnormal bleeding thinking it’s just part of life. But did you know that about 1 percent of the population, male and female, have bleeding disorders? Many go undiagnosed for years and years like I did, not knowing that there are medicines out there that can substantially help improve their quality of life,” Snarey said. “Whether it’s hemophilia, von Willebrand’s disease, platelet disorders or a different rare disorder, there are so many resources available now that can help improve your quality of life.” n