Twenty years ago, my son, Tyler, was born with severe Hemophilia B. We had no family history so it was quite a shock. I was terrified. The hospital told me he had “Christmas Disease” which didn’t sound so bad; “Christmas” is good, right? We stopped using that term very quickly! At seven months, Tyler had a severe allergic reaction after receiving a dose of clotting factor. It was the scariest day of my life. He had developed an inhibitor. I later learned this was very rare with Hemophilia B patients. Tyler’s rare condition just got rarer. And can you believe, it happened a week before Christmas? What are the odds? #ChristmasDisease
After Tyler’s inhibitor developed, life got much more complicated. No longer could we use Factor 9 to control his bleeding episodes or for prophylaxis. Our only option was to use Factor 7 which had to be given every two hours around the clock until the bleeding was under control. This also meant that we could not use the medicine preventively, only as needed after the bleed had started. Unfortunately, Tyler proved to be the constant bleeder. His early years consisted of bleed after bleed, accompanied with excruciating pain. It was heartbreaking. At the time, we were told trying to desensitize him to the Factor was not an option because it had not been successfully done. Well, that was about to change!
When Tyler was five years old, our doctor decided to attempt to desensitize Tyler to his clotting factor. Desensitization is the process of giving frequent high doses of Factor in order to get the immune system to accept the medicine rather than treat it as a foreign substance that requires an immune system “attack” (allergic reaction/anaphylaxis). This is my laymen explanation. Our desensitization process went on for almost two years. Looking back, I laugh at my extreme optimism. I truly believed failure was NOT an option. I was certain, it was going to work. I’m naively hopeful with every obstacle. Hey, whatever helps you get through it, right?
By the time Tyler was seven, he had been desensitized and tolerized to Factor 9. His inhibitor was gone and he stopped having severe allergic reactions. This did require daily doses of Factor 9 which still continues today. He has had relapses over the years. His inhibitor makes his ugly presence known for time to time. And yes, we’ve named Tyler’s inhibitor. I think giving him a name, makes it seem less scary. I’ll tell more about “Bart” another time…
So this is where we are today, Tyler can use Factor 9 and gets great recovery after infusions. Honestly, he skips a day or two every now and then. For an inhibitor patient, he’s doing great! Don’t get me wrong, we have gone through some crazy stuff, you name it, he’s had it; however, he has definitely embraced the concept of when life hands you lemons, make lemonade. What else can you do?
Well there’s a glimpse of our journey. I made taking care of Tyler my mission for the past 20 years, and now he’s off to a local college and my caregiver role is lightened. Now I’m switching hats to advocate and am looking forward to working with the bleeding disorders community as a Patient Advocate for HF Healthcare. Come back next week blog and I’ll share tales of Tyler’s toddlers years. Think BUBBLEWRAP…
Future topics to come:
-Establishing an 504 & IEP
-Compartment Syndrome
-Blood clots
Blogging about Bleeding Disorders 