At a glance
"Every time a bleed happens, I hold my breath to see if Thomas's body is going to respond to treatment." Sonji shares the challenges and concerns she's experienced while raising her son with hemophilia. Thomas developed an inhibitor when he was 8 months old, which makes it more difficult to manage bleeding episodes.
Sonji's story
On August 7, 2003, Sonji Wilkes and her husband, Nathan, welcomed their son Thomas into the world. Thomas has severe hemophilia A. He developed an inhibitor when he was 8 months old, soon after beginning treatment for his hemophilia. He suffered his first joint bleed into his ankle at age 2. He was temporarily confined to a wheelchair, and the bleed took more than 2 months to resolve.
According to Sonji, bleeding management for a person with an inhibitor is a real challenge. At the time Sonji shared her story, there were only two products to choose from to control bleeding, and only one product approved for prophylaxis (preventive treatment). "Every time a bleed happens, I hold my breath to see if Thomas's body is going to respond to treatment, and I never know if it's going to take one treatment or a week of multiple infusions daily to stop a bleed. It's scary. I worry that my child might literally lose life or limb, and I only have a limited range of medical interventions to help him." Few people know the challenges of living with hemophilia and an inhibitor more than Sonji and her family of five.
Venous access (finding a vein in which to infuse the factor) is another challenge for the Wilkes family, because Thomas receives infusions of factor daily. When Thomas bleeds and needs multiple infusions per day, the need for access is even more critical. A venous access device—a long, thin tube called a catheter, which is inserted into a vein—allows a physician to deliver medicine directly into the bloodstream without repeatedly puncturing the blood vessels.
According to Sonji, Thomas's veins are hard to stick on a regular basis, and he has so far had multiple PICC lines (a peripherally inserted central catheter line, which is inserted into a vein in the arm and goes up the vein all the way to a large blood vessel in the chest), as well as five ports (devices that are surgically placed under the skin of the chest and connected directly into a large vein in the upper chest for infusions).
"It is uncertain if he will require another port, or even if his body will hold up to another insertion, not to mention the bleeding and infection risks involved with the surgical placement. It is terrifying to have the very medication that can save his life in your hands, but not know if you'll be able to administer it because of access issues."
Other challenges of living with an inhibitor that Thomas and his family have faced include management of short- and long-term pain from repeat joint bleeds, dependence on and side effects from pain medications, inability to participate fully in sports and activities due to physical limitations, many school absences, and concerns over the costs of medications.
"There's worry over the affordability of the drugs available to him. There's worry over access to his medical team and if my insurance will allow me to utilize their expertise. There's worry over his mental and emotional condition. There's enough worry to fill an ocean."
Despite all of these challenges, the Wilkes family maintains a positive spirit and a focus on the gratifying aspects from their struggles with hemophilia and an inhibitor.
CDC would like to thank Sonji and her family for sharing this personal story.