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.

Rash at its worst.

Still smiling even though she's covered in rash and has a fever.

Decorating her crib. <3

Walking the halls cause she was tired of the room.

Livi's welcome home from The Ekker's & Burgos' <3

Olivia's SJIA Diagnosis (Part 1)

I have been wanting to type this post for many months, but I've been scared. Olivia's diagnosis is still confusing at times to me. I understand everything, it's just putting it into words for others to read.

It all started around the end of October when she developed a rash. It started on her legs and then on her arms. Unsure of what could have caused it, we started thinking about the food we were consuming. Since she was still getting pumped breast milk, I had to think about what I was eating as well. We couldn't ever think of anything that was out of the ordinary that she hadn't had before. After a few days we noticed the rash would be a little worse in the evenings and at night. Dannie and I never thought it was anything serious because she seemed fine the whole time. After a week of having a rash, she began getting temperatures at night. I took her to the pediatrician on Oct. 31st. The pediatrician thought she had a viral infection. Since she had no other symptoms and her temperature was actually back down that day, he wasn't worried. They did pricked her foot to check her blood work though. Her white blood count was a little elevated, but he still wasn't concerned. He wanted to follow-up if her fevers came back.

We returned home and started watching her rash more closely. It began showing all over her body. Her fevers would come back in the evenings and I think the highest was around 102. She hasn't EVER taken oral meds well. Fighting her for tylenol was not easy. She would gag a lot and puke it all up. She had lots of cool baths that helped lower her temp, sometimes. By Tuesday, November 4th she still had a low grade fever. I decided to call the pediatrician back again and they wanted to see her again. Again, they pricked her foot for labs. Her white blood count was very high at that appointment which concerned him. My mom went with us for extra support. He was unsure what it was and referred us to see an infectious disease doctor in the area. We didn't have much luck getting in contact with one locally and the pediatrician didn't want us to continue waiting. He said the best thing would be to go to Shand's Children's Hospital and see what they recommend at that point. Very worried at this point what it was, we headed to Gainesville. I called Dannie at that point to fill him in and sent him home for my breast pump and milk.

We were in the Emergency Room for what seemed like FOREVER. Doctors and nurses would come in every so often and we would have to explain our story every time. They did LOTS of labs and gave Livi oral meds for her fever. (I have pictures of her rash from during this time, but I will have to upload those later) My parents were both there and Dannie as well. They finally decided they were going to admit her to the hospital. At this time, we still had no idea what we were dealing with, all we knew is that her blood levels were very high and that was worrisome.

From what I remember, they poked her a few more times during the night for more labs. Dannie and I were stressed and very on edge at this point because we had no idea what was going on with our baby girl. We didn't sleep well at all. Dannie and I had to get used to nurses coming in every once in awhile to check on her, get vitals, and also give her meds for her fever every 4-6 hours.

The next morning we were up bright and early for more labs. She may have even had an X-Ray and CT Scan. I can't remember when she had all those done because she had so many. Our days usually began around 6:30 AM because the doctors and students made rounds between 7:30-9:00 am. Again, during these times they just asked us lots of questions and we told Livi's story again and again.

 The next couple of days she had lots of tests done. We were still unsure of what was going on which made our situation even more difficult. We were in quarantine, so all medical personal and staff had to wear a mask and gown. This scared Olivia SO much every time someone would enter the room. Poor girl was tired of being touched.

The only thing we could do was try and make her the as comfortable as we could. Without a diagnosis or any end in sight of when we were being discharged, we got daily visitors from friends and family and just tried to enjoy our time with Olivia.

Dannie's work was amazing at letting him be gone during this hard time, can't thank them enough! Livi had daily visits from Granny and G-pa. They were with us every day. The Burgos family came by, the Ekker's, and the Braswell's. Not to mention all the love and support we got through phone calls, texts, and messages from Facebook and Instagram.

The doctors rounded every morning during the week and were still unsure of a diagnosis. They had said she was a mystery for now. That didn't give us much hope that we were getting out of there anytime soon. We still needed a diagnosis and a plan for her care.