Yesterday was long. We left our house at 9:30 am and got home a little after 10 pm. We spent 7 hours in the Hematology clinic. James and Fiona were so good and patient the whole time. I learned a lot about the direction of her diagnosis and treatment.
In the next month and a half they will be running a lot of tests. First, a genetic test for severe combined immune deficiency (SCID). This test will take 2-4 months to complete. Second they are checking t-cell function again because of her low t-cell counts. Third they will do a head, chest, and pelvic CT scan to check for autoimmune problems and possible infections that don't manifest outwardly. Depending on the results of the CT scan she may need a PET scan to look at specific areas in more detail. Fourth, she will have bronchial and stomach endoscopes to check for infection and autoimmune problems.
It's possible that she may have leaky SCID, she also may have hypomorphic SCID. If you think of SCID as a spectrum, regular SCID is what the bubble boy had, leaky SCID is less severe, and hypomorphic SCID is the least severe. If she is found to have a variation of SCID they will most likely do a bone marrow transplant. If she has hypomorphic SCID the will watch her to see if her t-cell counts and function continue to decrease. Yesterday I learned that t-cell counts decrease as you age, so a low count in a child is more significant than the same count in an adult.
They are also checking for autoimmune responses. An autoimmune response is when your antibodies start attacking your body. They are looking specifically at organ damage. If her body is attacking her organs they will do a bone marrow transplant.
The last thing that they are checking for is lymphocytic gastritis. That's a medical term for losing lymphocites and protein in the gut. Some of her labs suggest that this is a possibility, but some of them rule it out as a possibility. In an effort to make sure we check everything they are still running the tests for this. If this is what she has the treatment is continued IGG infusions.
After our discussion of tests and possible treatment options the doctor ordered labs. They needed 19 mls of blood drawn. For a small child that's a lot of blood. It took 4 sticks to get a good vein. It was horrible! Sometimes she's an easy stick, but if she's not, she's really not. From now on we will ask for the IV team every time.
Once our appointment was over we left the hospital, drove for about half an hour and decided that we should feed the kids. I didn't plan on her appointment going as long as it did, so I didn't bring dinner. We stopped at Cracker Barrel, James' favorite restaurant (he calls it dunkin' barrels). Just after we ordered our food Fiona got sick, all over both of us. I rushed her out to the car, cleaned us up, and waited for Christian and James to finish eating. Christian brought out our food, which we are eating for breakfast.
Because we are pretty new to the immune deficiency thing every illness triggers a call to the doctor. When I called last night the doctor was very thorough, and decided that it was probably a combination of a long day and a stressful blood draw.
Cincinnati called back morning to let us know that her white blood cells just over 10 (the highest we've ever seen them), which sounds great, but it could also signal an infection. Her lymphocytes are a little lower than last week, she's dropped from 2.09 in August to 1.2 yesterday.
Today we are exhausted, and glad to have a day at home to recover from the craziness of yesterday.