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.
The past couple of weeks have been good for Scarlett. She
started preschool, which is a pretty big deal! She gets to ride the bus with
her big sister to school on Mondays, Wednesdays, and every other Friday. It’s
hard to explain how emotional it was to see her step onto the bus for the first
time. When Scarlett was fighting for her life in the PICU, I envisioned her
growing up and riding the bus to school like she always wanted to many times
over in my head. It’s safe to say I pretty much bawled my eyes out all morning,
I was still crying when I got to work that morning. I was extremely apprehensive
about sending her to school this year. She is currently on a 504 plan with her
school. This means that her school must accommodate her medical needs, such as putting
a hat on her at recess, letting us have a hospital grade air purifier in her classroom
so she doesn’t have to wear a mask, and putting covers on the fluorescent lights
in her classroom to protect her from UV lights. So far, she loves being at
school. She has come home with a malar rash more than once now which at times
is discouraging. On Tuesday, which was her day off from school she took a 3
& ½ hour nap. There are times when she feels worn out as tonight she was
complaining of joint pain.
We also made a trip to Fargo during her first week of school
to see her pulmonologist out of Sioux Falls. He prescribed Scarlett an inhaler
twice a day, as she continues to have reactive airway disease from the trauma
of the multiple pulmonary hemorrhages. It already seems to be helping. Scarlett
contracted a cold two weeks into school and I feel that she’s getting better
faster than she normally would. Even though Scarlett starting school and being
exposed to other kids and all their germs at times completely scares me, I
couldn’t be happier for her as she gets to meet this milestone. She loves going
to school. What is supposed to be a rite of passage for most young kids is a privilege
for Scarlett. We hope she will do well in school and will be able to go to
public school and have normal social experiences. There is a fine line between protecting
her, but not ostracizing her from her peers. She just wants to be like
everybody else. I am so happy for Scarlett. I hope and pray she continues to
meet these important childhood milestones and continues making good memories.
It has been a while since my last blog. Partly because I didn’t have a computer but also because Scarlett has been so back and forth these past two months with her lupus. I wasn’t sure what to say. She has her good and bad weeks. One day she is doing well and the next day she is complaining of pain. She had a terrible summer cold in July and her cough lasted for weeks. Now overall, she’s doing well which is what her doctors tell us. I have been thinking about this post for quite some time. Not that long ago a parent posted in our lupus support group that their child had passed away. She was 10 years old. I cried as I read her story and prayed for peace for her parents. Then, I got to the comments. Another parent had commented that this is the fate of all our children with lupus. It’s funny how one comment can affect you. It has bothered me and I have dwelled on this a lot. I refuse to believe that this is Scarlett’s fate. I know the statistics… the younger you are when you get lupus the more aggressive it is and the worse the outcome. Scarlett was two years old when she first had symptoms. Maybe I’m naive but I absolutely refuse to believe that a short life is Scarlett’s fate. Every day I look at Scarlett and I’m reminded that miracles do exist. I remember like it was yesterday, driving to the hospital when Scarlett was coding and prayed to God not to take her. Scarlett survived not having a heart beat for almost 5 minutes and the pulmonary hemorrhages she had for months after. I don’t know of any other person who has survived what she has. She is truly miraculous. I am encouraged that she has had a good year overall with no hospitalizations. I hope that her lupus will continue to be well controlled. Scarlett starts preschool next week which is a pretty big deal. We really want her to have normal social experiences. She told me tonight that she’s “really nervous” to go to school. I whole heartedly believe that she is starting a new trend for childhood lupus. Keep going my sweet girl. I can’t wait to see what new statistics you set for young kids with lupus.
This weekend we are celebrating a milestone. Last year at this time on June 8th 2018 Scarlett’s tracheotomy was removed. It was a very exciting day for our family and everyone that had been taking care of her for the previous 4 months. On February 21st 2018 Scarlett was flown to Sanford Children’s in Fargo after experiencing pulmonary hemorrhage and going into cardiac arrest. She was there almost two months before she was able to get her trach and g tube placed because she was too unstable to go down to the operating room. She spent 100 days on ventilator support, 43 days on the oscillator and 57 days on a conventional ventilator. Finally after 100 days her doctors took her off life support and 8 days later the PICU intensivist removed her trach.
We were told she would most likely be discharged on a home vent. I did her trach cares and changes while she was still in the PICU so that when she came home I’d be comfortable doing them by myself. I didn’t like doing her trach cares, only because I felt like I was giving in to the fact she was going to come home with the tube in her neck that she hated so much. Scarlett’s trach made her even more nauseous when she was being weaned off sedation. She has told me countless times she did not like it and it scared her when she couldn’t talk. Deep down I felt this hope and maybe some defiance she wasn’t coming home with a trach. It served it’s purpose. It helped her get better. It was a tool in her survival, but once she no longer needed it I wanted it gone. At the time I felt that if she was coming home with a trach then her respiratory crisis wasn’t over.
There were four amazing intensivists that rotated in the PICU and three of them told me she would most likely need home support. There was one intensivist who was adamant she would not go home on a vent. He was so sure Scarlett would recover. His words to me were that it was a tool she only temporarily needed to get better and I held on to his words. Needless to say Scarlett’s recovery amazed them all. Dr. Storm got to be the lucky one to remove her trach. He is an older gentleman with white hair and beard at the time. A few weeks after Scarlett was discharged home she looked at me very perplexed one day and asked “Mom, why did Santa Claus take out my trach?” She was serious. It was funny. She has since realized Dr. Storm is not Santa Claus, but in her defense that can be confusing for a 3 year old.
Today I asked her what she remembers from the day she got her trach out and she replied “that all my friends helped me to get better.” That’s right they did! We continue to be thankful for all the milestones Scarlett keeps meeting. One year trach free! (Pic is of the day she got her trach out.)
This past weekend we took our kids camping. My husband went to the lake a few days before me with all four of our kids as I had to work and he wanted to get more days of camping and fishing in with them. Our kids are 8, 4, 2, and 1 year old. Not a small task for one person to tackle. I was a little worried about him handling them all. I know how much work they can be. Our 1 and 2 year old boys have a lot of energy and Scarlett needs her medicine twice a day. We also are vigilant about sun exposure as she’s very photosensitive. Scarlett now knows when she goes outside she needs to put on her hat. Kuddos to my husband for taking them all by himself. Everyone survived, no serious injuries, and our kids had the best time. Scarlett loved spending time with her siblings and she was the first one to catch a fish!
The first night our oldest child had a cough and of course Scarlett caught it the next day. Our other kids got sick as well. This has been a very long respiratory season. Currently she is on day nine of having this cough. Initially her cough sounded wet, she had wheezing, but no fevers. Her appetite was poor and she wasn’t sleeping very well. I started her on guaifenesin three times a day with her albuterol neb treatments. I was worried she was going to develop pneumonia, but she began to turn the corner a few days ago and seems to be getting better. It is stressful when Scarlett gets sick which she easily does. She has scar tissue on her lungs from her pulmonary hemorrhage. When she contracts a respiratory illness, she is obviously affected more not only because of her lupus but also the scarring and reactive airway she now has. She has been sick enough now for us to know when to take to her in to the doctor and when to treat her at home. Thankfully she seems to be on the mend. Her cough is improving, she slept well last night, and her appetite is coming back at full force.
All our kids may have left this camping trip with a cold, but they also left with a lot of fun memories. They are already planning the next trip where they want to catch more fish. Even though Scarlett had a cold she wasn’t letting it keep her from running around and having fun with her brothers and sister. We cherish all the good moments spent with our kids. Hopefully the next camping trip we come home with just the fun memories and leave the sickness behind.
Update: A few days after this post Scarlett’s cough started to get worse. I took her to see her primary physician and he diagnosed her with bronchitis. She was prescribed 3 days of steroids and 10 days of antibiotics. She seems to be getting better. It just goes to show we think she’s on the mend then she can easily go the other way.
In April of 2018 Scarlett was still in the PICU at Sanford when she was nominated by Dr. Mauriello for Make A Wish. She remained in critical condition, but was improving. I remember him saying something about submitting an application for Make A Wish during morning rounds one day, but didn’t think much about it as Scarlett’s condition was still guarded.
We were very excited when Scarlett’s Wish Granter, Dawn called to let us know that Make A Wish ND was going to grant a wish for Scarlett. We met with them in early October. We decided not to talk to Scarlett or our other kids about what was going on. After all, it was Scarlett’s only wish and we wanted it to be her’s. Her wish granters came to our house and asked Scarlett questions about what she liked to do, then they talked to Sheldon and myself about our family and Scarlett’s needs. Her lupus makes her extremely sensitive to heat and sun. She cannot be around large crowds. We have to be careful with what she eats as preservatives and gluten tend to contribute to flares. Scarlett had not been home very long when we met the Make A Wish volunteers for the first time. Questions about Scarlett’s prognosis and what was important to our family really stirred up emotions in me.
The most precious thing you have with your children is time. You can never get back today or relive yesterday. You never know when your family might receive a life changing or absolutely devastating diagnosis and all you want is more time. You think that something like this could never happen to you or your family. I felt the same way, until it did. I know what it feels like to be told that your child will not survive. I give credit to God and Scarlett’s amazing team of doctors and nurses that she did. I also remain positive for her future even though her doctors have a hard time giving us a long term prognosis, but they have been very encouraged by her progress and remain positive as well.
The best part about being gifted a camper from Make A Wish is all the memories we will make for hopefully a long time to come with Scarlett and all our kids. Scarlett is looking forward to camping with her sister and little brothers.
We are so grateful to Make A Wish ND and everyone that supports them. They are changing lives for the critically sick and terminally ill children. Giving the most important gift. Time for families to be together and memories that last forever.
Last Wednesday Scarlett had her adenoids removed and PE tubes placed. She has had many infections these last several months and we are hoping this helps keep her off so many antibiotics. She was intubated for two months in the PICU so there was concern over how much stenosis she would have from this and how well she would tolerate anesthesia for her surgery. She did very well and as far as it could be seen she hardly has any stenosis which is quite remarkable. She also had several of my former co-workers taking care of her which was special and they did an excellent job.
Two days after her surgery she developed a fever and it continues today. Last night I brought her to the ER for the first time since she came home from the PICU. I’m pretty sure the ER doctor thinks I’m slightly nuts because I was demanding (very nicely) multiple tests to rule out infection. Of course with Scarlett’s history the ER doctor completed understood my concerns. I have to say Scarlett had very good care in our local ER. I really felt the provider listened to me. Scarlett is not the typical case. Her labs that measure inflammation were 4x higher than they were 5 days ago and her wbc is up. Which could be because she’s either having a flare, an infection is brewing, or stress from her surgery.
At this point I highly suspect a lupus flare. Usually her flares last 2 days and she’s better, but this flare is going on 4+days. But it’s still important to rule out any possible infections. Scarlett definitely keeps us on our toes. It’s hard to relax when she’s having a flare especially with her history of pulmonary hemorrhage. I have all the tools to monitor her vital signs at home. Her O2 sats have been good. She seems to be getting a little better. She finally does not have a fever tonight and she is in better spirits. Her lupus is definitely not keeping her from being a kid.
Scarlett knows she has lupus and in her own words tonight she said “my lupus is not keeping me from getting big.” That’s right my sweet girl. Keep the positive attitude and hopefully your flares get fewer. Scarlett’s lupus is definitely not keeping her down.
We were recently in Fargo and saw Scarlett’s rheumatologist. I have to take a minute and rave about her doctors, especially her two main doctors that rotate seeing her. I used to work in surgery and now behavioral health. I feel comfortable interpreting most of her lab work as they come in, however I have to say immunology is not my specialty. Scarlett’s rheumatologist and nephrologist are her main doctors and has been absolutely amazing answering my questions and making sure I understand her disease process completely. I am also aware that I can sometimes become obsessive over every test that comes back. Her latest lab work has been reassuring. Her hemoglobin is up, which is very good as many with lupus suffer from anemia. Her platelets were low which could be the disease process or her medication. She also had her B and T cell counts done which were abnormal. One of the medications from the PICU that was crucial in saving her life, rituximab also robbed her from all her B cells. Her doctor has explained to me that these cells are responsible for making memory in her immune system. The good news is that they are higher than when she was in the PICU which hopefully means her immune system is trying to make memories. She is still very immunocompromised.
This winter Scarlett was sick a lot. In December she had a round of antibiotics and steroids for pneumonia. In January she had a double ear infection which would not go away. Over the next few months she required three different antibiotics to cure it. Every time she was placed back on an antibiotic I literally got heart palpitations. As we already know she is developing antibiotic resistance. My husband who usually gives Scarlett her morning medications missed one dose of her clarithromycin due to a miscommunication about how many days she had to be on it and I literally lost it. I yelled at him. I felt like a terrible parent and wife over ONE missed dose of an antibiotic. I cried as I felt like we were contributing to her resistance.
Scarlett is now scheduled to get PE tubes placed and her adenoids out soon. Her audiogram showed her ear drum movement is minimal meaning she probably still has fluid even after her infection has cleared. Hopefully this procedure will keep Scarlett from having to be on so many antibiotics in the future. We have to save her antibiotic susceptibility for when she really needs it. She has a large team of doctors including her rheumatologist, nephrologist, pulmonologist, primary, and now an ENT doctor. They all agree it’s the best for her with her compromised immune system.
We still plan on putting Scarlett in preschool next year where she will be exposed to more germs and viruses. Our kids go to a country school and right now there are only 13 kids in each preschool class which really is pretty minimal. Next year will be quite the test run, but I really do not want her pulled from school. Several other lupus families I now know of have had to home school their child due to them catching too many bugs at school. I really want Scarlett to have normal social experiences. I think her being able to go to a small school and having her big sister Stella there to help her clean her hands will be a big help. Hopefully her upcoming surgery will help eliminate her having to be on so many antibiotics and help keep her healthy. After all that is the main goal of all her doctors and us.