September 28, 2016
On Monday, Jodi and I had the opportunity to meet with Tegan’s hematologist without Tegan being there. This was our opportunity to ask our endless questions about what this means and not worry about our 3 year old seeing our fears and tears. In reality, we had no tears and walked away feeling very empowered. The meeting really made us feel…equipped. I guess that is the best way to describe it. We were given medications to use when incidents occur’ and an overall plan on how to deal with his bleeding disorder.
Here is a summary about what we learned about his bleeding disorder. Tegan has all his platelets that help create blood clots but his platelets lack the right “ingredients” to create a good blood clot. (For my biologists out there he has a storage pool deficiency. He doesn’t have the dense bodies in his platelets that release ATP, ADP, serotonin, calcium… on and on. So in essence he lacks the items that create a feedback loop to aid in creating a good blood clot.) He described it like Jello. A good Jello jiggler is firm like a good clot, but Tegan is creating a “clot” that is like loose Jello that must be eaten with a spoon and not as effective.
We learned what our fears should be and should not be focused on. Our main concerns are centered around if Tegan has any procedure/surgery or experiences trauma, both of which we have a plan for how to handle. Tegan is not at risk for bleeding without cause. When trauma or a procedure occurs, he will bleed more than the rest of us. We have two ‘as needed’ medications that we walked away with, Amicar and Stimate. Amicar is for the bloody nose that just won’t stop. This drug basically keeps the body from breaking down the clot he does produce. This is useful for the small stuff that doesn’t necessary need medical attention but we need help stopping the bleeding. Amicar is only made by one company in the world resulting in a retail price at a whopping…$2,891!!!!! Stimate is for the more serious injury to use immediately prior to leaving to seek medical attention. If he hits his head and vomits or passes out we give him a squirt of this in his nose and head to the hospital. This drug activates his platelets to release all they got. It also makes his kidneys go on pause. For 24 hours he will not be able to pee, so if we ever have to do this drug he will only be able to have 12 to 15 oz of water for 24 hours. Retail price…$830!!! Jodi has amazing insurance requiring us to only pay $70, but seriously,s how can these cost so much?!?! We learned that hemophilia patients need Factor VIII, a coagulation factor that their bodies don’t produce, which costs half a million dollars causing most to have to save $20,000 for their medications each year. It just made me wonder how people can even do that.
Overall, day to day we can (and should) let Tegan be a 3 year old little boy, without limiting his activity and it is ok for him to fall, skin a knee, or run into stuff (just part of his life). However, he shouldn’t play contact sports (where his vision may have limited him anyway) but now it is a for sure a no-go. Tegan will have a medical bracelet and we will have several bleeding cards (instructions on what to do with information for medical personal) to carry on us and give to everyone he has contact with when he is not with us.
Unfortunately, we already need a surgery to remove the blood clot in his right ear which is blocking his ear drum. Here is where my love for Children’s Hospital continues to grow. We are so used to being Tegan’s secretary, liaison, and advocate that we have come to expect nothing from anyone. But the table has turned as Children’s Hospital expects us to focus on being great moms and let them take care of us?! I love them. After our meeting I asked his hematologist if I should call Tegan’s ENT and inform him of the diagnosis and have him contact the hematologist. He then said he has already spoken to the ENT and are they are working on Tegan’s surgery plan!!! Unbelievable. On top of that, our geneticist already contacted our hematologist to see if we could get a jump on starting Tegan’s genetics tests!! They are so on top of it and just took a huge weight off of us. I know not everyone has the same experience but they have won my heart.
We feel equipped, informed, and prepared to handle his bleeding disorder. Every moment since the day he was born has been a roller coaster of joy, laughter, grieving, accepting, and celebrating that we have this gift named Tegan:)
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