Olivia Olive Bottom: Olivia's SJIA Diagnosis (Part 2)

Well if we thought the first week was hard, the second week was even harder.

On Sunday we got a visit from some of her doctors. They informed us that in order to check for some viruses they were concerned about and also some cancers, she would need a bone marrow aspiration. Just hearing those words, especially cancer was the hardest thing for us. Not only that, but Olivia was going to have to be put under. The first thing that went through my mind was her being in pain afterwards. We went over all of the risks and decided it was the best option at that point. We knew that it would help the doctors find a diagnosis and also help them eliminate what they were unsure of. Since they were getting daily labs from her, they had said they would get all the labs they needed for the last of the infectious disease panels as well as any routine daily CBC tests.

(Not related to the paragraph above) We started taking daily pictures of her progress board. Mainly so we could see it once she was discharged. The nurses didn't really bother with filling it out much, but we enjoyed writing in who her visitors were.

They told us they would take her down to pre-op around 7 am. That turned into about 7:30. We were escorted down to the 2nd floor which was pre-op and the OR. She had the sweetest anesthesiologist for that procedure. We really liked him a lot and he he helped calm our nerves by ensuring us she was in good hands. The procedure lasted maybe two hours, I can't remember exactly. During our wait time we went back up to her room and showered and Dannie went and got us breakfast. Once they called us to go meet with her she was awake and fussy. They said they usually aren't in any kind of pain, that she would just be groggy.

They were tracking her daily labs to see if they were trending up or down. From what I can remember at that time, the main concern was her WBC, Ferritin, CRP, and Sed Rate. I'm sure there were others since they were checking to make sure her spleen and other organs were not enlarged.

This was such a rough day for us because the doctor came around in the evening to speak to us about a PICC line for her. Not only that, but her hemoglobin was below a 9 so they recommended a blood transfusion for her. I'm glad my parents were still there for us. Dannie and I had someone to discuss it all with. Not to mention all of the emotions we all had. Olivia had already gone through so much that day, so I felt bad putting her through more, but I knew it would help with blood draws and medications. By the time they came in to take Livi and gave her meds to calm her it was close to 8 PM. It was a LONG night, once that was all done, they had to do an x-ray after to make sure PICC is positioned properly. I think they started the transfusion around midnight and ended around 4 AM. Between the nurse staying in there and Livi waking up every once in awhile, I think I got about two hours of sleep.

Her rash on 11/8/14- Day of Bone Marrow Aspiration

By Wednesday, November 10th they had got some of her labs back as well as the blood smear they needed to look at for any cancer. This is when it gets a little confusing because at some time they spoke to us about Systemic Juvenile Idiopathic Arthritis. They looked over her joints and compared her rash daily. These were all symptoms of SJIA. However, they were also looking at HLH as well. In order for her to be diagnosed with HLH, she would need to meet five of the eight criteria's. Those criteria's are rash, fever, high ferritin, iL2 receptors over 2,400, abnormal nk cell activity, cytopenias, hemophagocytosis in her bone marrow, and an enlarged spleen. She did not meet the criteria's of what is not in bold. Some of the testing they did during her Bone Marrow Aspiration had to be sent off to the Cincinnati Children's Hospital because they are the leading HLH Hospital in the US and they handle the genetic testing for HLH.

They began treating her Wednesday night with Kineret (an injection), Cyclosporin, and Dexamethasone. Her fever actually broke that night and by morning, her rash had started disappearing. Since the rash and fever were more of SJIA symptoms than HLH, they diagnosed her having MAS, a complication of SJIA. If it was full blown-HLH her rash and fever wouldn't have reacted so fast to the medications. The only cure for HLH is a Bone Marrow Transplant. The medications they started her on were the same meds they would have started, with the addition of Chemo, if it was HLH anyway, so they were still going the same route and still waiting for more results.

We were excited about NO RASH!

Since she was responding to her medications and the rash had disappeared and fever was gone, they decided they should move us to a more isolated section on the Pediatric floor. We were moved to 42 Peds. With that came new nurses and a more isolated room. Unfortunately, a few hours after we got over there the doctors noticed her heart rate was dropping some when she would sleep. They monitored her for an hour and felt better about sending her to PICU to be monitored more closely.

She stayed in PICU for three days. I think we got moved back to 42 on Saturday the 13th. We had really got to know the PICU nurse since she was with her all the time. Dannie and I got to share a chair when sleeping in that room. It wasn't very big, but we made it work.

We received more results back from Cincinnati around the 3rd week she was at Shand's. These confirmed the five criteria's for HLH. They needed to make sure that when she was effected by the SJIA it only effected her joints and not her nervous system. At that point, they recommended her having a Lumbar Puncture to check signs of inflammation. Of course that meant being put under again. Again, we were skeptical, but knew it was important to check. She was scheduled for that bright and early again. The procedure took about an hour. She went to recovery and we were able to see and hold her again while she woke up. Results from that didn't take long and they had said her nervous system was not effected. Woo!

A few more days passed and she started eating more and feeling a bit more playful. We could tell she was finally starting to feel better. They were still doing daily lab draws and her labs were trending in the right direction each day. By this time she was getting two injections of Kineret a day (morning and night), Dexamethasone (steroid), and cyclosporine (Immune suppressant), and protonix (helps with upset stomach) They had mentioned a discharge date, but that was only if she continued on the path she was going. They even started doing labs every other day so they wouldn't be drawing so much blood.

She had visits from her Abuela, aunt Kristin, and Jodi the last week she was in the hospital.

She was discharged on the 21st of November. They had to successfully change her IV meds over to oral before she could be discharged. We also decided to change her daily injections to Actemra, which was a bi-weekly IV infusion. She got an infusion before we left the hospital to make sure she didn't have any reaction before we went home.