Nightmares…

Scarlett by no means has had an easy recovery since her initial diagnosis. She had multiple pulmonary hemorrhages the first one caused her to go into cardiac arrest on February 21st, the last hemorrhage caused by her lupus was in April. In May, when she was completely off sedation she bled in her lungs again after contracting endocarditis in the PICU. I remember just like it was yesterday. I was sitting on her bed and she started to deteriorate pretty quickly, then blood started coming out of her tracheotomy it was the first critical experience she had when completely off sedation besides her initial code. I was trying not to panic in front of her. She had to be placed back under sedation. I am so thankful to Scarlett’s nurse who voiced the need for me to have a moment to say goodbye to her and in that moment Scarlett was trying to make me laugh and be silly. I cannot imagine how scary it must have been for her to not be able to speak or adequately communicate how she was feeling. Since coming home she has had nightmares. At first they were frequent and I had to sleep in her bed for a long time. Now she might have a bad night once or twice a week and sometimes she shouts out in her sleep. Tonight however, I have already been up with her twice. I ask her what she’s dreaming about. She told me she has dreams about the hospital and that she did not like having the tube in her neck because she couldn’t talk and that it was scary. Tonight her pillow was soaked in sweat. I work in behavioral health and have talked to a few psychiatrists and therapists about her case. The consensus is that she is too young to participate in therapy but hopefully at a year out from the traumatic experience she won’t remember much. Antihistamines can also be helpful for young children with nightmares, but they don’t seem to affect Scarlett, she has a high tolerance for medications. We were so thankful to bring Scarlett home that the aftermath of all the traumatic experiences were not on the forefront of my mind. I can only hope her nightmares get fewer and farther between. I still believe that everything happens for a reason. Scarlett is definitely a little fighter and lupus warrior.

Immunocompromised 1/22/19

I have spent many hours and late nights reading stories and blogs of others’ struggling with lupus, mostly adults and older teens. At times this has become very discouraging to read how so many people are suffering and do not have the best quality of life. Scarlett is currently on mycophenolate which suppresses her immune system and keeps it from overreacting and attacking itself. The mycophenolate replaced her cyclophosphamide chemotherapy. There are many people with lupus that require cyclophosphamide for an even longer period than Scarlett to control their lupus. Scarlett had six rounds. It seems that any and all germs that she is exposed to she will get. She had pneumonia at the end of December which required 10 days of antibiotics then a double ear infection this month which also required another 10 days of antibiotics and now she has another upper respiratory infection which is causing her to have a lot of drainage out of her eyes. One of the biggest threats to lupus sufferers is infection and sadly many with lupus will die from infection complications. With four children it seems almost impossible to keep them from sharing germs even though I regularly use bleach to sanitize surfaces in our home. Our family is affectionate, we hug and kiss each other and I don’t ever want to tell my kids not to. I have started to tell them no kisses on the mouth or face this respiratory season. I just want Scarlett to be healthy. I am already starting to worry about Scarlett starting preschool this fall. Most of the stories I’ve read about younger children with lupus is that a good majority of them are home schooled due to them missing too much school and the exposure to others. I do not want Scarlett to be home schooled. Even before she got sick she always said how she wanted to ride the bus with her big sister Stella and when she was in the intensive care unit I clung to the hope that one day she would get on that bus and ride to school with her sister like any other normal child. I want her to have the same experiences as any other child, but I am currently struggling with the reality of how fragile she is. We are still working on this delicate balance of letting her have normal experiences, but staying away from those that may be sick. I really do hope she can ride the bus this fall to preschool with her big sister. There are so many every day experiences that I have taken for granted in the past. Every healthy day with Scarlett is a gift. She will always be my miracle baby even when she’s sick. She has already proven to us that she’s a determined little fighter.

Rheumatology appointment 1/17/19

Today we met with Scarlett’s rheumatologist for a follow-up appointment. She is currently doing well. Right before Christmas Scarlett developed pneumonia after contracting the respiratory virus our other kids had and had to be on antibiotics and steroids. She is currently on the mend. Her latest lab results are good. She had no protein in her urine for the first time and her complement levels remain stable. Our biggest concern at this point is infection. I constantly worry about germs! In early December we were attending Stella’s (our oldest child) Christmas program. I was sitting with Scarlett on my lap and the man next to me coughed directly on us. I almost lost it on a complete stranger. Cover your mouth! My husband and I are planning to bring Scarlett and Stella to Florida with us next month, as I’m attending a conference for work. I’m already having anxiety over whether people on the plane around us will be sick or coughing and making sure we sanitize her seat and tray. Before Scarlett’s lupus diagnosis I never worried about such things. If a piece of our kid’s food fell on the floor at home it was ok, because that helped build their immune system. It is a balance. We want Scarlett to have normal experiences, but I’m apprehensive of what she’s exposed to. Every week she goes to Awana, which is children’s church and as I drop her off I can’t help but linger in the doorway to make sure I don’t hear any of the kids coughing around her. We are planning on putting Scarlett in preschool this fall when she’s four. For now she’s doing well, we are optimistic. Stella’s 8th birthday was today, which made Scarlett even more excited for her own birthday in a few weeks. Scarlett’s 4th birthday will certainly be special.

Scarlett’s Journey

Scarlett’s lupus journey began when she was just 2 years old. It’s unknown how long she’s actually had lupus as she has had protein in her urine since she was an infant which we contributed to UTIs. Her symptoms of lupus began in November of 2017. She had complaints of pain in her ankles, my husband and I thought she just wanted to be carried. In December of 2017 there were a few days she woke up with periorbital edema but it would resolve a few hours later. Then in late January of 2018 our entire family got sick with a respiratory virus. We got better, but Scarlett was getting worse. A few days before I brought her to the pediatrician’s office I brought her to our local walk-in clinic and we were sent home, that was a Thursday. By Sunday at her 3rd birthday party she was not acting herself, over the weekend she quickly deteriorated, she was pale and lethargic.

On Monday February 19th 2018 I brought her into the pediatrician’s office her oxygen saturation was 76% and she was immediately admitted to our local hospital and treated for pneumonia. But on February 21st 2018, Scarlett experienced her first pulmonary hemorrhage due to lupus and went into cardio-respiratory arrest. I did not learn until months later that the code team could not find a pulse for almost 5 minutes, however they were able to revive her and she was flown to Sanford Children’s in Fargo 4 hours away from our home. I watched the plane fly over our house from our driveway as we were unable to ride with her.

When we first arrived to the Children’s Hospital we were told the most likely diagnosis was leukemia, specifically JMML. Her doctor told us on a scale of 1 to 10 chance of leaving the hospital he gave her a 1 because he did not want to give her a zero. Scarlett began chemotherapy treatment for leukemia. She continued to fight and was intubated on the oscillator (step up from ventilator) for many weeks. In early March her bone marrow biopsy came back with no cancer cells but was abnormal that is when her rheumatologist got involved and a diagnosis of lupus nephritis class five was made and confirmed with more blood tests, genetic testing, and a kidney biopsy.

Scarlett then began aggressive treatment for her lupus which included Cytoxan chemotherapy, high dose steroids, and eventually after her last hemorrhage rituximab. She remained in critical condition for over 3 months in the PICU. She suffered multiple pulmonary hemorrhages her last one being in April and had to have chest tubes inserted multiple times due to bilateral pleural effusions and a rare chylothorax caused by her lupus. She also had several complications from being in an intensive care unit for an extended period of time including a central line infection that caused her to develop endocarditis and shower clots into her already assaulted lungs in May. Due to her critical condition she continued to have a poor chance of survival. After weeks of aggressive treatment for her lupus she started getting better. She spent 43 days on the oscillator (record for the hospital) and 57 days on a conventional ventilator. On her 100th day in the PICU she was taken off ventilator support and on day 108 her tracheotomy was removed.

Genetic testing revealed she has a somatic mutation in her NRAS gene. The geneticist at the hospital feels she has RALD, ras-associated autoimmune leukoproliferative disorder which can contribute to autoimmune phenomena and possibly explain why her lupus is so aggressive. A positive NRAS gene also puts her at higher risk for future malignancy. After 118 days in the PICU she was discharged home with only a g tube for nutrition and giving medications which has now been removed. Four months in an ICU bed left her with severe muscle wasting. A few weeks after she was discharged she took her first steps and now she recently graduated to not needing PT anymore.

She continues to be monitored closely by her team of doctors including a rheumatologist and nephrologist through Sanford. She will need to be on medication to keep her lupus under control for the rest of her life.  Her doctors have found no documented cases like Scarlett. Lupus in a three year old is extremely uncommon and her initial presentation of pulmonary hemorrhage is even rarer. Hopefully Scarlett’s success can help other children with pulmonary hemorrhage due to lupus survive as I am hoping her case gets published.

Scarlett was also nominated by two of her doctors while she was still in the PICU to be the regional Children’s Miracle Network Champion. Being a CMN champion her story will reach more people and hopefully raise money for local kids in our state.