June 5, 2018
I know it has been a super long time since I have updated or wrote about Tegan. What is new? Tegan will be 5 in June and is off to Kindergarten next year. We still can’t believe it. He rides a two-wheel bike, thinks he is a ninja/Jedi, and wakes up every morning rushes to greet the dogs and hold his “morning meeting” with them (as he calls it). All around he is doing amazing, thriving and driving us crazy like little boys should.
We all attended our second HPS conference this March and got to meet more amazing people and find ways to get more involved with the HPS network. It was so cool to see Tegan this year at the conference because he was very aware that so many people looked just like him. He would point out a little boy who had white hair just like him, or he would see other adults using a cane to navigate. It was so awesome to see him with his people. Just another reason we believe in this network and their mission. The HPS conference is like nothing I have ever experienced. This disorder is so rare that during the conference researchers fly in from all over to conduct research. It is not often you can gather 100+ people together who have HPS, so they collect blood and stool samples. The researchers also meet all day on Saturday to share their work and what they are finding with each other. It makes me cry every time I see them working so hard to find a cure. They eat with us, talk to us, and party with us Saturday night. The commitment they have to us is profound and humbling and I am so grateful to them.
As you may know the thing that started us on this HPS journey was Tegan’s ear tube surgery that he had 2 years ago. He bled for over 24 hours and we couldn’t get it to stop. We were blessed with a very knowledgeable and amazing ENT surgeon who knew that something was up and got us in to see a hematologist right away. Long story short Tegan was diagnosed with HPS type 1. Well at the end of that 24 hours of bleeding Tegan had a huge blood clot that was sticking out of his right ear and even though the outer parts of the blood clot are gone he still has this in his ear.
For two years a blood clot has filled his ear canal and it is still in there!!! We have tried drops to soften it many times, but the moment the ENT touches it Tegan is in super pain. We keep checking on it every 3 to 6 months hoping it would come out or eventually fall out but it just stays. It seems like it is still attached to the tube which is a problem. They don’t want ear tubes to stay in longer than 2 years because complications develop. The left tube fell out about a year and half ago, but we can’t see anything in his right ear except the blood clot, so unfortunately, we have another surgery. They will be removing the clot June 6th (tomorrow) and if it is attached to the tube and the tube is still in his eardrum they will remove it and patch the eardrum.
Now I am feeling super worried about him bleeding. I talked to his hematologist last night about our tentative action plan and I feel less worried, but we still wish there didn’t have to be a surgery. They will have him take Stimate 15 to 30 minutes before the surgery and potentially Amicar as well. They will also have specifically typed HLA platelets for him just in case the Stimate and Amicar are not working 100%. These HLA typed platelets are specific to his tissue type. We have to do this so that he does not develop an immune response to different tissue types because he will need a lung transplant (at this point) when he is older. The Stimate is kind of a crazy drug too. It will help Tegan develop a blood clot, but it impacts the function of the kidneys temporarily. Due to its impact to the kidneys Tegan can drink no more than 16oz of fluids for 24 hours after the Stimate. We feel super lucky to have such amazing doctors on our team. It always surprises me to see their commitment to him, they genuinely care about him. This will happening June 6th so I will keep you all posted on this.
We feel confident in our doctors and those in the network who have walked this path before us. We are lucky to have such an amazing group of people behind us. Thank you. Thank you for listening to us and allowing us to be sad sometimes and allowing us to not let HPS be the only thing we talk about. We have moments when Tegan’s HPS hits us (like a surgery) and we have to dwell on it, but we have far more moments where it is not even a passing thought for us. It is nice to be in place where we are not just focused on finding a cure, but also on him learning to read, ride a bike, and building the greatest Lego jet. It feels empowering to us to own the HPS diagnosis, but still live the life of adventure we want. It feels like acceptance (with a lot of determination for the cure!!).
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