Living with an Inhibitor

The big piece of our story is Leland's inhibitor. We discovered he had an inhibitor when he was hospitalized for an intracranial bleed at 15 months. The doctors were able to get the head bleed under control with massive amounts of factor. Despite this, he was not maintaining adequate factor VIII levels. That's when we realized he was developing, or had developed, an inhibitor. Just a month earlier, Leland did not have an inhibitor when he was screened as part of his annual testing.

We're not sure why Leland developed an inhibitor. Often, there is a family history of inhibitors but not in our case. Neither my father nor my two nephews who have hemophilia developed an inhibitor. So, we don't know if Leland was predisposed in some other way or if the onslaught of factor he received to treat the intracranial bleed triggered a reaction by his immune system. That's a question that will never be answered.

After some years of trying to "treat" the inhibitor, we finally started "living" with it. The biggest difference in our situation versus other families I know is that, at this point, we do not have the option of prophylaxis. Kids who are on prophylaxis might get factor three times a week. By maintaining a sufficient level of factor in their blood, these kids have fewer bleeds, and some rarely bleed at all. It still amazes me to hear a mom talk about the last bleed her son had 6 months ago . . . or last year . . . or 3 years ago. I don't mean to say it's easy for these families. Certainly, they face daily challenges, and I don't want to minimize that. However, their experiences managing hemophilia are very different than ours. Leland bleeds all the time; on average he has some sort of active bleed going on 10 to 15 days out of the month. There are times when we're dealing with a bleed for weeks in a row. More often than not, we don't know whether the bleeds are spontaneous or if there has been a trauma of some kind.

Leland has a lot more outward signs that he's different than other kids with hemophilia and that can be a challenge, especially as he gets older and just wants to be part of the crowd. For a lot of the kids on prophylaxis, the only telltale sign of their hemophilia might be a medical alert bracelet and a band-aid on their hand three times a week. Because of Leland's frequent bleeds, he often goes to school with a sling on his arm or a heparin lock in a vein, and he's in and out of a wheelchair because he tends to have a lot of lower body bleeds. For the most part, he seems to deal with it extremely well. His biggest thing is that he gets tired of people asking questions.

Leland's hemophilia has a pretty significant impact on our day-to-day life. Many times we have to cancel planned activities at the last minute because he has a bleed. Sometimes, we treat him, and he can go about most of his regular daily routine despite the bleed. But often, now that he's older, a lot of pain management is associated with his bleeds. It's never a matter of, "Oh, there's a bleed, let's shut everything down." We try to continue with our plans, but when the pain kicks in it changes everything and that's when it's most challenging.

Fortunately, I have a great network of other moms in the community. Through chapter programs and a wonderful e-mail chain, I have people I can talk to and lean on. But, quite honestly, I sometimes feel that other moms I know can empathize to an extent but they really don't understand our daily struggles. Most of their sons are on prophylaxis. They don't have frequent bleeds and the resulting pain issues, missed school, cancelled activities, and general uncertainty that are part of our life. But, that said, we're all moms raising children with an extra challenge thrown in. At the core, I believe that they do know how I feel, and I am so appreciative of their friendship and support.