Fever

Fevers are no simple matter when it comes to a bone marrow transplant. If a child has a fever, it is taken very, very seriously, because their bodies are extremely fragile. Charlotte was home for 24 hours and got a fever, and so we took her to the Children's Hospital of Philadelphia ER. They drew her blood work, and her white blood cell count is 0.7 which is very, very low. A normal range is between 6-8. They have started her on antibiotics and are admitting us to the bone marrow transplant unit until they draw blood cultures and determine whether or not she has an infection.

Discharge

On day +167, we are going home to live with Laura's aunt and uncle in Delaware! We have to stay within 1 hour of the Children's Hospital in case Charlotte gets a fever and needs to go to the ER.

Dysautonomia

Charlotte has been having very unusual symptoms this month. After having several "episodes", the doctors, nurses, David, and I started to notice that Charlotte's vital signs will change very quickly, for several minutes, and then return back to normal very quickly. For example, Charlotte's body temperature will go very high, her heart rate will go very high, and her blood pressure will go very low. Usually, she will throw up or pass out at the same time. Her entire body goes haywire for several minutes, usually in the morning when she wakes up, and then it returns right back to normal on its own. The doctors and nurses thought Charlotte kept having infections, due to her high temperature. However, after watching her "episodes" closely she only has these events for a few minutes a day, but the rest of the day she will be fine. No more unusual temperatures or abnormal symptoms. She seems very comfortable and happy the rest of the day. Due to the "events" the doctors sent blood work to test for viruses that could be causing a problem. Charlotte tested positive for the Roseolla virus. Usually, roseolla is a very benign virus that children get commonly. However, in a child who has no immune system, and is post transplant, viruses are serious business. They can make these children extremely sick very quickly. That is why children are on such strict precautions after a bone marrow transplant. Their bodies are in a very fragile state for a long time until they build their immune system back up to handle infections. It is basically like having the immune system of a newborn baby again. So, the doctors started Charlotte on an antiviral medicine to treat the roseolla infection. Usually, children are not treated for the roseolla virus because their bodies can get rid of it on their own. Children who have had a bone marrow transplant are treated for everything because the smallest infection is a big deal. Unfortunately, the roseolla virus has started to cause Charlotte's bone marrow blood cell counts to drop. The doctors have reassured us that viruses can commonly cause blood counts to drop, and that with treatment, the virus should go away, and her bone marrow will return to normal. The virus has also caused Charlotte to have symptoms of what is termed "dysautonomia." Dysautonomia is a rare diagnosis that people have, but it can be caused by a virus. There is no way to know if the virus caused a "dysautonomia event" or if part of Charlotte's diagnosis is dysautonomia. We will be watching her closely to see if these events continue or if they go away once the virus is gone. Charlotte has started blossoming with her new experiences that she is trying. She colored for the first time and loved it! She loves to flip the pages and the book to start new drawings. She also enjoys music therapy twice a week with Patrick. In true Papa T fashion, Charlotte loves the guitar. She is not interested in the other instruments. Patrick can hardly play because Charlotte loves to strup his guitar with him while he sings. She is also walking a lot with her walker. OT has encouraged us to try "messy play" since Charlotte has been so isolated in regards to textures that a child would normally experience outside. So, we used shaving cream and Charlotte had a ball! It's been a fun few weeks overall.  

Adrenal insufficiency

The more we learn about MIRAGE syndrome, the more pieces of Charlotte's puzzle seem to be falling into place in regards to healing and recovery from transplant. The most important part of Charlotte's syndrome that we have been discovering is that her body can not produce "cortisol". Her adrenal glands have "insufficiency" and can not produce this hormone when it is needed. Cortisol is an important hormone that our adrenal glands produce to deal with stressful situations. For example, when we have surgery, have an upset stomach, get an illness, our bodies produce cortisol to deal with the stress and keep everything in our body in "homeostasis" so everything doesn't go out of control. Charlotte was started on a small dose of hydrocortisone in July. It was not enough and she continued to get sick. The doctors increased the dose of Charlotte's hydrocortisone, and her body has been doing much better. So, the endocrine doctors have decided that Charlotte will stay on the highest "physiological" dose of the medicine that she can get, which means the highest amount that a typical body would produce at her age. Apparently, Charlotte's body is very insufficient in its ability to produce cortisol. It causes big problems. We are shocked that Charlotte even made it through a bone marrow transplant without it. Unfortunately, this is what caused her to have so many trips to the ICU. The good thing is now we know, and one the correct dose of the medicine was started, her body responded really well.

Gastric emptying

If you want to see evidence of how important our stomach is to our quality of life, look no further than Charlotte Harris. As we all know, Charlotte has had lifelong problems with her stomach that only worsened after transplant. Since her transplant, Charlotte has been having difficulties with her electrolytes. This has caused her to require lots of electrolyte supplements. We had a family meeting with all of Charlotte's specialists to talk about MIRAGE syndrome and how to solve her electrolyte mystery. For some reason, her body is not able to maintain them like they should. GI suggested we do a gastric emptying scan and a GI scope to see how her stomach looks since transplant. So, Charlotte had a scope and another gastric emptying scan to look at how her belly absorbs and empties food. In May, her scan showed that she had severely delayed emptying and her stomach was not moving food at all. Her current scope and scan showed that her stomach....wait for it.....IT IS NORMAL! Laura's jaw nearly hit the ground when she heard it. The doctors laughed too because they couldn't believe it either. This is the first time in Charlotte's life we have ever heard that her stomach is normal. So, the doctors are slowly starting to transition her feeding regemen from J feeds to G feeds. This will allow her food to be absorbed better in the main part of her stomach. Also, they are going to slowly switch her formula from a very complex formula for children with severe GI issues to a NORMAL pediatric formula. This will also allow her to get better nutrition. Charlotte is also on a long term antibiotic for her recurrent infection. The antibiotic dose will be complete next week. This is a good thing for her stomach. However, we hope that the infection is completely gone and will not come back like it has in the past. Since Charlotte has started to get food in her belly, she has been the happiest and most active we have seen her since transplant. Here's to a life a enjoying all the wonderful foods that the world has to offer!