1 Year Diagnosed with Lupus

On March 8, 2018 we received news that Scarlett had Systemic Lupus Erythematosus. After being told there were no cancer cells in Scarlett’s bone marrow we were anxiously waiting for answers and hoping for the best possible outcome. I will never forget Dr. Syverson and Mauriello walking with us to the staff only area where the doctors’ room was to privately talk about her diagnosis. We agreed to have a kidney biopsy which did confirm she had class 5 lupus nephritis. At this point, Scarlett was still on the oscillator and continued to have a poor chance of survival.

I have had several people ask me if I was angry that she was initially misdiagnosed with JMML. I have to definitely say no. Lupus is known as the great mimicker. I have read other lupus blogs that have said it initially did look like cancer for them as well. Scarlett’s oncologists also said to us that her presentation was not typical for JMML because her spleen was not enlarged, and only her lab results met the criteria. Scarlett did have 5 days of chemotherapy for leukemia which she did seem to respond to as the chemotherapy did suppress her immune system and eventually she had cyclophosphamide chemotherapy to treat her lupus.

Scarlett’s lupus diagnosis changed everything. She was still critical with a very guarded prognosis, but it gave us hope. Lupus could be treated and a plan was put in place to treat her with cyclophosphamide chemotherapy and high dose steroids. Eventually she was also treated with rituximab and did respond to treatment. Scarlett made a recovery that many thought would be impossible. Now we are facing the long term aspects of having a child with lupus.

Scarlett is constantly catching every little bug going around. We are hoping that next year when she’s farther out from her chemo that it won’t be so bad, but she will still need to be on mycophenolate which suppresses her immune system. Getting a definitive answer as to what she was up against was a turning point for us. Pulmonary hemorrhage in lupus is very rare and there are no published case studies in this happening in a child as young as Scarlett.

My hope for Scarlett after receiving her diagnosis is that she’s able to live a long fulfilling life. She is already loved more than words could express. Her story has touched so many people. We have to have faith that Scarlett will make it to adulthood and have normal life experiences. The day she graduates high school, college, etc. there will be no one bawling more in the audience than me. Scarlett is a little lupus warrior. She has now been living one year diagnosed with lupus but I just believe she will continue to amaze us for many more to come.

The worst night in the PICU.

Thanks to Facebook and a personal timeline we kept of Scarlett’s hospital stay. I am very aware that one year ago today was probably the worst night Scarlett had in the PICU. Which says a lot as she had many bad days. I can honestly remember like it was yesterday. The crash cart was outside her hospital room 928 for almost 18 hours. We were discussing flying her out for ECMO as a last resort to save her life. It’s never good to have 10 nurses and the PICU Intensivist in your room all night long. Amazingly, in the 24 hours prior to Scarlett having this large pulmonary hemorrhage her physician in the PICU had a very brilliant plan that probably saved her brain function. Her doctor gave Scarlett extra blood than would normally be expected to get her hemoglobin high so that in the event her O2 sats would drop her heart would keep pumping oxygen rich blood to her vital organs and brain. I sat at the edge of her bed surrounded by staff working fiercely on Scarlett. I watched as blood just kept emanating from her ET tube. Her O2 sats dropped twice into the 50s and hung in the 60s for awhile, but her pulse remained strong. I don’t believe in coincidences as only one hour earlier I was reading a scripture my dad had marked in the bible that my husband had brought from home. Luke 8:50 says. “Don’t be afraid, only believe and she shall be made well.” I think I repeated this over 50 times that night. I absolutely believe that prayer helped save Scarlett, but I also know that everyone that took care of her were supposed to. She had the most amazing nurses. There were four PICU Intensivists that rotated with her and I can whole heartedly say that each one had a hand in saving her life. I look at her now and I am completely amazed that she is cognitively intact. I think that is the biggest miracle of all. Not only did she survive being in critical condition for three months, but she remembers her life before, during, and after her hospital stay. She is one intelligent little girl. She wants to be a “baby doctor” when she grows up. I feel guilty that deep down I’m not sure I want her to do anything stressful, but that’s not up to me. Sometimes I don’t think that anything could stop Scarlett. She is the most determined 4 year old I know. I had a moment tonight when I was so frustrated that she was trying to debate me about her bed time, then I became very aware of the fact that she gave me a very intelligent 4 year old reason of why she should stay up late. The “one year ago today” reminds me of how fortunate we are to have our spunky Scarlett still with us today and thriving!

2/21/19 One year from Scarlett’s code blue.

One year ago our lives changed forever. We knew Scarlett was sick , but this was the night we knew how sick she truly was. Around midnight I left Scarlett’s hospital room to go home and sleep. Sheldon said he would stay the night with her in the hospital as I was seven months pregnant at the time and he wanted me to get a good night’s rest. At 3:30am my phone rang and it was Sheldon he told me to get to the hospital right away because Scarlett was crashing. Then he told me do not get into a car accident on the way. As I was looking at my phone he had texted me “RAPID” a few minutes earlier (meaning rapid response). I pulled on my clothes from the bedroom floor I had been wearing earlier and literally ran out the door. My dad was staying at our house to watch our kids and I didn’t even tell him I was leaving.

When I arrived to the pediatric unit the team was no longer doing CPR but the anesthesiologist was placing Scarlett’s central line and the staff were shouting for more epi and atropine to keep her heart pumping. I walked into her hospital room and I’ll never forget how she looked lifeless, laying on the bed with her arms and legs spread out. The ICU doctor continuously monitoring her pulse on her femoral artery. I noticed the blood that had emanated from her endotracheal tube and knew it was not good. Her pediatrician was on the phone I heard him talking that he did not want the helicopter because it would take two hours to get to Fargo and she was too unstable and he was requesting a plane to fly her out. Before the flight team left with Scarlett they gave Sheldon and myself some time to talk to her and say goodbye. I will never forget leaning down to tell Scarlett how much I loved her. Then I turned to her flight team and told them how much we loved her and I knew how we all wanted the same outcome for her.

I can remember 2/21/18 just like it was yesterday and have gone over it a hundred times in my head. I have also read her code blue documentation over and over. I have come to a point however, where I have realized that it is no longer healthy to relive a traumatic moment over and over again. Scarlett survived. Not only did she survive, but she has surpassed all expectations of her recovery. She is alive and doing well. I feel that it is definitely time to move forward from our traumatic experiences and celebrate what we have with her now. Our happy 4 year old spunky daughter who is living her best life. She loves to play with her siblings and always takes her medication without much complaint. One year ago 2/21 was the worst day of my life, but now 2/21 makes me hopeful for Scarlett’s future and the great plan God must have for her.

Happy 4th Birthday, preschool, and nephrology appointment.

Scarlett has had a big week and it’s only Wednesday! Yesterday Scarlett turned 4 years old! To say the day was emotional for us would be an understatement. Her official party is this weekend, but I had to get her a small cake so we could sing to her on her actual birthday. Scarlett got many birthday wishes from the staff at Sanford and even complete strangers on facebook wished her a happy birthday and said they were praying for her. We also registered her for preschool this fall, which she is very excited about. She even had to wear her unicorn backpack to registration which one of her PICU nurses, Hannah gave her. Today she saw her nephrologist at Sanford and got a good report. Her kidney function has remained stable so he is going to wean down her enalapril with a goal of discontinuing it in two months which will be once less medication to take. It’s also good for her long term prognosis. Her doctor told me that by the time most people with class 5 nephritis are diagnosed they often already have significant kidney damage, but thankfully not our Scarlett. When Scarlett was in the PICU I did not take any pictures of her while she was intubated. My logic at the time was that if she did not survive I did not want to remember her like that. Intubated… a tube down her throat, appearing lifeless, hooked up to machines. A few weeks ago my husband, Sheldon revealed to me that he had taken two pictures of her while she was intubated. He said he took the pictures because if Scarlett did not make it he wanted to be reminded of the efforts taken to save her. He thought he had deleted them because of my wish not to have them. Then he found them on a hard drive a few weeks ago and I saw them for the first time. It definitely stirred up feelings in me that we were experiencing at that time, having been told that our precious Scarlett probably would not leave the hospital alive. I want to share this personal photo because this is how our sweet Scarlett started her 3rd year of life. As she enters her 4th year of life, I hope and pray that she is leaving the worst medical crisis that she will ever have behind her. I do realize that she is not going to have an easy road ahead of her, but I feel that if she can survive bleeding in her lungs for almost two months she can conquer anything. It is truly amazing to see where she was almost a year ago to where she is today. She has defied all the odds and surpassed all expectations given to her. Scarlett continues to be our spunky one. Happy Birthday my sweet girl. I hope year 4 brings good things for you.

First trip to the beach.

This past week I had a conference in Florida for work. My husband and I decided to bring Stella (8) and Scarlett to make it a special trip with our girls. Stella has only been to the Oregon coast and Scarlett has never been to the beach at all. Deep down there was no way I could leave Scarlett for almost a week. At the same time, I got heart palpitations thinking about traveling with her, large crowds and enclosed spaces combined with her compromised immune system made me nervous. I got her blood work done a week early before our trip and everything looked good so that gave us some reassurance. Scarlett was the best traveler! She didn’t get scared on the plane. She never complained about wearing her mask, which we only put on her in the large airports and when we were around a lot of people. We went to Sanibel Island and spent a couple hours on the beach under an umbrella. Scarlett is very photosensitive and for families with children who have lupus the sun can definitely hinder the fun. I was actually very worried that the sun would cause a flare that I made sure to google the nearest hospital. However SPF 50 sunblock, long sleeved swim shirts, hats, and making shade was the key. Only once during the trip she got a very faint malar rash. We also noticed that she didn’t once complain of joint pain while we there and her cough was better too. I’m sure the warm humidity really helped. We live in North Dakota. The winters are harsh and dry, we have to keep a humidifier in her room otherwise her reactive airway from her pulmonary trauma causes her to have a chronic cough. The house we stayed at in Florida had a pool and the girls swam for hours. The puddle jumper is the best invention ever. It allowed Scarlett to float in the water and kick her legs which was so good for her. She is still lacking muscle mass. This past week was very encouraging for us. We really want to make memories with Scarlett as well as our other children and have fun life experiences. Hopefully next year we can escape the cold winter with all four of our kids. Our boys are still pretty little, Dean is two and Will is 10 months old today! Scarlett told me how much she missed her little brothers while we were away. I hope we can continue to make fun memories with all of our kids.

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.