As crazy as Tyler’s hemophilia journey has been, his compartment syndrome experience was, by far, the worst. Compartment syndrome is a very painful condition that occurs when the pressure within the muscles builds to dangerous levels. This pressure can decrease blood flow, which prevents nourishment and oxygen from reaching nerve and muscle cells.1 In Tyler’s case, he had a bleed in his right forearm. It seemed like just a regular bleed, until it didn’t.
Tyler had gone to a sleepover. I didn’t find out until later that they went to a trampoline place and played dodge ball. At the time, Tyler was not on prophylaxis because his allergic reactions and inhibitor had returned, and he had not been desensitized to Factor 9 (his clotting medicine) again. After the “Extreme Bounce”, he did one big infusion of NovoSeven and fell asleep. By the time he woke up the next day, his forearm was hurting, but he never said a word about it. He came home and slept for seven hours; he was exhausted because they had played video games all night. Sunday evening is when I realized we had a big problem.
It started like any other bleed causing pain and some swelling. I stayed up all night infusing him every two hours which the protocol for Factor 7. I continued for the next day, yet I wasn’t seeing improvement and his pain was escalating. This bleed was different than others because his arm wasn’t as swollen as I would have expected considering the level of pain he was experiencing. I later learned that was because the swelling occurs within the “compartment” deep in the forearm. It was time to be admitted.
The hospital (HTC) continued his every infusions every two hours, but his pain escalated. He was on heavy pain medications and seemed to be getting no relief. By the third night, the hematologist told me they were taking him in for a fasciotomy, a procedure where they make an incision from the elbow to the wrist to relieve the pressure. The fear is that the bulging muscle putting pressure on his nerves was blocking the blood flow to his hand. He was in danger of losing function of his hand if we didn’t act fast. I’ve never seen him in so much pain.
After the surgery, I was relieved to hear that he had no dead muscle tissue in his arm. My joy was short lived, however, because of the impending nightmare of the “wound VAC”. A wound VAC is used to help a wound heal faster by decreasing air pressure to the wound. It also drains blood that is seeping out and keeps the wound clean. Since Tyler was receiving so many infusions of clotting factor, his blood kept clotting in the tubing of this vacuum. He had three more surgeries replacing wound VAC after wound VAC. All the while, he was bleeding like from a sieve. It was terrifying. He had seven blood transfusions in the course of three days. It seemed like as fast as we were pumping it in, he was oozing it out. It was so frightening and frustrating that I completely broke down. Little did I know that my poor boy’s nightmare was far from over.
Coming next week: Part II: Tyler’s “shark bite” recovery.
*NOTE: I’ve posted pics, but they are not for the faint of heart: Compartment Syndrome pics. (Tyler requested the pics to show his friends; we document everything.)
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