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May 29, 2017 Latest post:
Sep 13, 2018
You guys are amazing and we want to thank you for all your love and support! Your thoughtful and caring comments in response to journal entries are soooo appreciated! Caring Bridge: While we will try to connect individually, we will try to update this site with information as soon as we can in the "journal" section. With some exception, most journal entries are written by Erin and posted by Meg Turnbull or Maike Johnson. Ways to Help: - You can find ways to help by clicking on the "Ways to Help" link in Caring Bridge. - If you want to share research articles, please make sure they are relevant and current. - Donate blood or platelets, or recruit others to do so –while this does not necessarily benefit Sierra directly, it is a symbolic and also tangible way to help kids like Sierra (and many others). - Participate in road races, triathalons, etc that raise awareness and funds for childhood illnesses with Sierra and other kids in mind. For example, some friends have pledged to run every race this year in her name. - Bringing food when Erin & Sierra are inpatient at CHOP is helpful since we cannot cook here. However, small portions are best since we have very little refrigeration capacity. - As we figure out other ways in which Erin and Sierra can use help, we will add these to the list. You can also donate money using the gofundme link (make sure NOT to confuse the CaringBridge donation link).
Visiting: Children’s Hospital of Philadelphia, contact Erin as to which room. The room we are in will likely change with each readmission. Please be sure to be healthy (i.e. no runny nose or other symptoms), as Sierra' s immune system will frequently be very weak during treatment. Also if you are visiting us at CHOP, flowers are not permitted on the oncology floor. We have set up a calendar where you can pick a day to visit the Johnson ladies or even better where you can choose dates to have food delivered to the hospital (there are options for those out of town, and pls know that Erin eats vegetarian food, and the fridge space we have is only allowed to store food for 48hrs and is not very big)
Background & Diagnosis: - Sierra was born full-term (3 days after her due date), without any complications and was a healthy at birth. Before mid-May (2017), Sierra had never been sick. - Around mid-May Sierra had an ear infection in each ear and was subsequently given a ten day course of antibiotics by her pediatrician. She continued to have frequent low grade fevers throughout the ten days, as well as afterward. - I took her to the pediatrician during those ten days due to concern about continued fevers. They checked her ears to make sure the ear infection was gone (in case there was a resistant strain of bacteria involved). The ear infection had resolved, but the fevers continued. - Sierra contracted a cold at the same time as she had the ear infections, and was also teething. I took her to the pediatrician and/or to urgent care 5 times before we were finally admitted to CHOP for blood work on May 22. - The weekend prior to admission, she had very low energy– she whimpered, was yellowish, and only wanted to sit on my lap or to sleep all weekend. May 21 was Sunday, so I called the nurse on call at the pediatricians office, who said that since Sierra had had fevers 3 days in a row, it was their policy to bring her in for another appointment. I brought her in at 4:15 on May 22 – since the lab they worked with closed at 5, her doctor sent us to CHOP for blood work. - With a sinking feeling, my mom and I got burritos and prepared for a long night in the Emergency Room. - The ER docs and nurse practitioners were amazing, and very quickly focused on the fact that all of Sierra’s blood values were normal except her Absolute Neutrophil Count (I still remember they said it was a scary 30), her Red Blood Cell Count, and her Platelets. They said if these counts had been only a little low, they might suspect a blood borne infection – viral or bacterial – but since the counts were incredibly low, they were more suspicious of Leukemia. - On May 23, and after no sleep but after 2 delicious burritos - we were moved to the PICU (Pediatric Intensive Care Unit) so Sierra could get a blood transfusion. She needed one regardless of the diagnosis. In the mean time, the path lab was testing for a variety of agents of disease in case she did have a bloodborne infection. - There was only one armchair in the PICU, so my mom and I went against safety protocol (I was told) and built a bed of sorts to share out of 2-3 additional normal chairs we found… - We were told that the blood tests came back negative for bacterial or viral infections, and that the likelihood of Leukemia was strong. However, a bone marrow biopsy would have to be done in order to confirm diagnosis. Sierra was sedated and a bone marrow biopsy was done with us in the room. I couldn’t watch. It was very hard to get marrow out because Leukemia cells had taken up so much space in her bone that the spongy, softer marrow was hard to retrieve. - The next day, we had our diagnosis. One more piece of information remained, which was whether or not her Leukemia cells had a chromosomal anomaly: had the cell which had “gone haywire” replicated itself with or without an anomaly? - On May 26, we were finally told that she did in fact have the type of Leukemia where the cell has an anomaly- Acute Lymphoblastic Leukemia (akute lymphatische Leukämie) and KMT2A (MLL) Gene Rearrangement (the last bit describes the particular anomaly). “Acute” describes the rapidity of cell replication- the Leukemia cells take up space in the bones, disabling healthy bone marrow function, and therefore also disable the production of red blood cells (carry oxygen around the body), neutrophils (first line of immune defense), and platelets (patch up wounds). Without treatment, people with Leukemia would die within a few weeks or months. So, upon hearing all of this, we knew we had no choices and that we would have to travel the cancer treatment journey that so many have traveled before us. - Sierra’s treatment for the Induction Phase began on May 27 on a steroid that many of you will find familiar – Prednisone. In oncology, apparently Prednisone is also used as a chemotherapy drug. She was on Prednisone for a week prior to the start of other chemotherapies. - Always thoughtful and generous, my sister flew in from Germany that day to stay with me in the hospital for a week, and to make my birthday on May 28 more bearable. - During Induction, Sierra had 2 spinal taps to prevent Leukemia cells from replicating there. Those areas are called “sanctuary” areas by the oncologist because Leukemia cells can persist there even if the rest of the body is being treated. - June 30 th , Sierra had another bone marrow biopsy - three different methods (2 via microscope, one assay) were performed to determine that she was in remission at that point and that no Leukemia was detectable with modern scientific means. Her remission status determined her course of treatment for the subsequent 4 phases of chemotherapy. - Originally scheduled to conclude in December 2017, the intensive part of chemo will wrap up this next week - the final week of February, 2018. Next, the long-awaited, significantly less intensive "maintenance phase"of treatment will commence. Maintenance is slated to conclude in October of 2019. During this phase, we will give chemotherapy medications at home and will go to the outpatient clinic monthly for check-ups. Sierra will also receive periodic spinal taps under sedation to continue to address any leukemia cells that may take up residence in her spinal fluid. The goal of "Maintenance" is to keep her in remission and to prevent any single cells from replicating rapidly, causing a relapse. In the words of her doctor, she will be able to be "fully integrated," and able to interact much more freely with peers, she will be able to travel, camp, go to petting zoos, etc.