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Wednesday, September 12, 2012

Life with Thalassemia

I've been thinking for awhile, that I should do a post about thalassemia. Actually, I have these ideas all the time, and I  think, "Oh! I should post about that!" but then, I get distracted or busy or both and I forget.

When we started out on our adoption journey the first time, we knew we were called to adopt a special needs child. And we were pretty open to a lot of needs. I wondered if maybe we'd adopt a hearing impaired child. After all, we had lots of experience with speech delays, and Olivia and I had taken two years of sign language already. Or maybe a child with a limb difference. The children with obvious physical differences often wait so long.  But, that isn't what God had planned for us. First, he put a little girl in our path with Thalassemia, and so we started researching, and reading and learning and praying. And we thought that she was the one for our family. She wasn't though, and she was adopted by a wonderful family before we were paper work ready. That sweet little girl set us on our path though. We had learned what thalassemia is, and the lack of treatment options in Ch*na. We learned the heartbreaking statistics that in Guangxi province, where Vivian is from, and thalassemia is pretty common, 80% of children with thalassemia die before the age of 5, and 100% by age 12. We also learned that there were few families willing to adopt these children because it is a chronic medical condition. Meaning it is life long. We started looking for children with thalassemia specifically. And that's how we came to be Vivian's parents. When you fill out the medical checklist for your agency of what needs you are open to....it is long. From really minor things, like eczema or birthmarks, to severe issues, serious heart defects, spina bifida, and all kind of things in between. I hadn't really heard of thalassemia. Maybe in passing in nursing school, but it is pretty rare in this country. It is sometimes called Mediterranean anemia because it is most common around that part of the world. Greek,Italian, Indian, Asian descendents. Here people have the benefit of genetic testing, they often know if they carry the genetic trait to pass it on. Generally, there are three levels of severity in thalassemia. Trait, Intermediate, and Major. This is a great link if you want to understand a bit more about the types and variations of thalassemia.
http://cooleysanemia.com/index.php?option=com_content&view=article&id=19&Itemid=27

E Beta Thalassemia. Hemoglobin E is one of the most common abnormal hemoglobins. It is usually found in people of Southeast Asian ancestry, such as Cambodians, Vietnamese and Thai. When combined with beta thalassemia, hemoglobin E produces E beta thalassemia, a moderately severe anemia which is similar in symptoms to beta thalassemia intermedia.

Vivian's is specifically E Beta Thalassemia. We didn't know for sure that was the case before she came home. We only had a few pages of a medical file and one set of labs, with no gene test. Based on those labs though, we were told by a doctor specializing in thalassemia , that he believed it was E Beta. In her short medical file, it also said she wasn't needing transfusions. We hoped that would be the case, as sometimes intermediate cases will only need blood every few months, or maybe only when they get sick. We were also prepared for her to present like a beta major case, needing blood every 3-4 weeks.  And it turns out that, she is E Beta, but she is more on the severe side and needs blood every 3 weeks.

So what does thalassemia look like everyday? Like normal. Minus a medication first thing each morning. Chronic transfusions lead to high iron levels, which can damage your organs. So, she takes a medication to bind the excess iron and rid the body of it. About two weeks after her last transfusion, we go in and have her blood drawn to check her hemaglobin level. Usually we will go ahead at that point and schedule her transfusion based on that number. Sometimes its in the next day or two, if she's on the low side. Sometimes, it's 4-5 days later. Transfusion day for us, is a long day. We live about 1.5 hrs from our nearest children's hospital and our pediatric hematologist, who oversees her care. If we were local, we would go the afternoon before and they would draw blood and type and cross it and have her transfusion ready the next morning. We'd go in the next morning, and they'd hook her up and it takes about 3 hrs. However, since we are too far to go in for the blood draw the day before(and it can't be done locally) we get get up at 5am, and leave by 6am, so we can be there by 8am when they open. Rush hour traffic and all! The nurse brings her back and starts her IV. Vivian is a rockstar. She knows the routine, and she just deals with it. She rarely cries. She will tell me, "Mommy, I brave." And she is. They draw the blood from the IV for the type and cross. Then, wrap it up and we leave. They usually tell us to come back after lunch, as it takes about 2-3 hrs for them to get the blood ready. We also do direct donor, meaning five of our immediate family take turns donating for her.So, she always gets our blood. I love knowing she has a part of us. When we leave, I try to make it fun. We go have breakfast. Did you know Ikea has free breakfast on the weekdays? ! We go to the park, or mall, or the local Chinatown. We end up there for lunch usually. It's the closest thing to the food I remember from Ch*na. Vivian loves the dumplings and noodles there. After lunch, we head back to the doctor's office, and they hook her up to the blood, and we watch movies, and nap, and play board games, and read books, etc... for three hours. When she is done, they remove the IV, say see you in a few weeks and we head for home. Hopefully, avoiding the five o'clock traffic. Usually, we get home after 7pm. It's a long day for sure, but a fun one. I hope it will always be like that for Vivian. A special day to be together. A positive thing.  The older kids take turns coming with us, and it is a great time for bonding. The rest of the days in between.... are normal.
 Thalassemia IS a chronic medical condition. It does have lifelong implications. It IS so manageable, for us. She has bloomed, and physically come so so far since coming home. She wasn't being transfused in Ch*na, but she needed it. She was tiny, and her spleen was so enlarged from the chronic anemia. She more than doubled her weight in less than a year. Her spleen is normal now. She is smart, and kind, loving, and funny! I can't even bear to think about what would have happened to her if she hadn't been adopted. We are so blessed to be her family. Vivian is so much more than thalassemia. She has taught me that children with special needs are not defined by them. It's just a small part of their life.

Here she is showing off her IV. You should see her drag that IV pole around,lol.

 Here we are napping together during a transfusion.
 Sleepy face, snuggling with Hamilton.
 At the park, not letting a little ole IV slow her down!
 She has her biggest brother, wrapped around her finger.
 Matching boo hands, with Olivia the Pig :)
 Comfort food, Chinese style. Notice her mad chopstick skills even with the IV in.

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