Intubation

Our biggest fear was realized when Charlotte woke up from a restful nights sleep and was unresponsive. She was shivering and unable to open her eyes. Laura was sleeping with Charlotte in her crib, and when they woke up and she immediately knew something was very wrong. Laura yelled for the nurse and the nurse checked Charlotte's oxygen saturation. It was 76%. Normal is 90-100. So, they turned Charlotte's oxygen way up and started fluids immediately. The intensive care team was called and Charlotte was transported to their unit. She started responding after they administered fluid. However, when the IV nurses came to place a line in Charlotte's foot for medications, Charlotte started crying hysterically and could not catch her breath. Her oxygen saturations started dropping rapidly again and the doctors decided they needed to intubate Charlotte and use a ventilator to help her breathe. The doctors have explained that Charlotte has gotten an infection which has caused "septic shock" which means bacteria has entered her blood stream and is causing a severe infection that is causing her organs to shut down. The tremendous stress has rendered Charlotte unable to breathe and she will rest while the ventilator breathes for her as her body heals from the infection.

White blood cells

White blood cells are an important part of our bone marrow. They are the cells that help us fight infections and heal our bodies. A person can not live without white blood cells. After being admitted to the bone marrow transplant unit, Charlotte's white blood cell count has continued to drop. She is now 0. This is an extremely bad situation for Charlotte, because if she gets an infection of any sort, her body can not defend itself and she will get very sick, very fast. The doctors noticed that Charlotte's red blood cell and platelet counts were low prior to discharge, and they also found out that her body was attacking her bone marrow. So, they started her on a steroid to treat the problem. However, as the red blood cells and platelets got better, her white blood cell count continued to plummet to 0. The doctors do not know why. We are, again, in an extremely rare situation.

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.