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 3^rd birthday party she was not acting herself, over the weekend she quickly deteriorated.

On Monday February 19^th 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.

Scarlett was also nominated by two of her doctors while she was still in the PICU to be the Children’s Miracle Network North Dakota Champion. This past year she has served as the North Dakota Champion, helping to raise money for local kids and raising awareness for lupus.

2 years since coming home…

I haven’t posted since February. I must admit that it has been a stressful time with covid, which I’m sure any parent with an immunocompromised child understands. I am really hating covid right now, besides Scarlett being a vulnerable child, myself working in healthcare and taking every precaution possible, it is also delaying her trip to the NIH to see the RALD specialist. Hopefully we will be in touch with him soon. Scarlett’s trip to Washington D.C. to advocate for lupus research was also cancelled because of covid. We were still able to meet with our state’s representatives over the telephone, which were positive discussions and the Lupus Foundation of America is honoring her scholarship and she will be able to attend next year’s summit. Which is great!

I always try to look on the bright side and since Scarlett has been home from school, she has hardly been sick. I added up all the days she missed at school this past year and it was half of her school days due to being sick and one day I kept her home because a classmate was diagnosed with influenza A. In January she had a chest xray and was given antibiotics for pneumonia. In February Scarlett celebrated her 5th birthday, but also had another round of antibiotics for pneumonia. In March, she was given steroids and antibiotics again for pneumonitits. Then covid happened and she had to stay home from school with her sister and she’s been doing well since.

On June 18, 2018 Scarlett was discharged home from the Sanford PICU. She has now made it two years without being readmitted to the hospital. She’s had her setbacks when she’s been sick, but she has not experienced an illness that is life threatening. That in itself is worth celebrating. She keeps defying the odds as most children with her complications have more admissions to the hospital. I will never forget the day she was finally able to come home. She may have had an IV pole next to her bed, but she was so happy to be back in her room. The road to recovery has not been easy. It is a strange coincidence that she had an appointment with her primary rheumatologist today, on the anniversary of her coming home. Scarlett has never had a normal chest xray, and since she is healthy he ordered one today to have a comparison. She also had blood work drawn. Her doctor told me today that he believes she will be able to attend school with her classmates and there was even discussion of lowering the dose of one her medications that suppresses her immune system.

I am so proud of our girl. Not only is she doing well health wise right now, but she has the best attitude. During her chest xray she followed all the radiology tech’s instructions precisely without any complaint. She also had to have a blood draw, which is the hardest for her. Scarlett always gets scared when she has to have her blood taken, especially after everything she’s been through. When Scarlett has to have her blood drawn she sits in my lap with her favorite teddy, which she got in the PICU and I always whisper to her that she is the bravest and strongest girl I know and that she can do it and I put Peppa Pig on my phone to try and distract her. I want to cry with her every time she’s in pain, but I’m so proud of her willingness, and she knows it’s to help keep her healthy. Her lab work already came back this afternoon and her inflammation markers are lowest they have been in a long time, which is the best news!

I am so happy that Scarlett is still doing well overall. The fact that she has made it two years since her discharge without any life threatening complications is amazing and gives me so much hope. When she left the hospital I was told by one of her doctors that if she could go two years without any serious complications it would be a good indicator of her long term survival. Way to go Scarlett! I couldn’t be more proud of you.

Watch out Washington, here comes Scarlett!

On February 21st 2018, Scarlett went into full cardiac arrest around 3:30 in the morning. I remember having a hard time last year at this time, but I think this year has been even more difficult. We still have a lot of unanswered questions about Scarlett and her diagnosis. Scarlett has RALD. Ras-associated leukoproliferative disease. When she was first diagnosed and I was told she had RALD. I thought it was just a genetic mutation making her lupus more aggressive. It is actually it’s own disease process. It is extremely rare. Less than one in a million children will develop it. Through the whim of fate (by that I mean God) I met another parent online in our lupus support group whose child also has RALD. She directed me to a doctor at the National Institute of Health who specializes in RALD research. Needless to say a few days later and I was directly speaking to Dr. Rao on the phone with plans to have all of Scarlett’s medical records sent to him so the NIH can follow her long term and in a few months we will be making the journey to the NIH outside of Washington D.C. for her first appointment. This is extremely incredible to have the TOP RESEARCHERS in the country studying Scarlett and following her long term. Scarlett’s rheumatologist at Sanford is also excited for her and they will collaborate with him as well. I hope that not only can Scarlett help other children with RALD and autoimmune phenomena, but I also want answers. Her long term prognosis is still really unknown although I’m optimistic most of the time. I read one article that stated any child with RALD will likely develop cancer at a young age. Hopefully collaborating with the NIH will give Scarlett the best possible outcomes and I’m excited for her case to help other children with the disease.

A few months ago I applied Scarlett for a scholarship to attend the 2020 Lupus Advocacy Summit in Washington D.C. in March. Her inspiring story must have won a few hearts as she was chosen from over 200 applicants to attend the summit and share her story with Congress helping to raise more funds for lupus research. Raising awareness for lupus and sharing Scarlett’s story has been my personal mission since she came home from the hospital.

When Scarlett first got sick I was devastated and angry. I could not accept the fact that I would have to bury my baby even when we were told she was not going to survive. Why did Scarlett draw the short straw? She didn’t deserve the crappy diagnosis she was handed, but neither did any of the cancer kids I watched walking the hallways at Sanford pushing their IV poles or any child for that matter. At times when she was on the brink of death the only comfort I could muster is that Scarlett had the perfect life. She had never known anything but love. My hope is that Scarlett’s life is going to make a positive difference in other children’s lives. I hope her medical course becomes the blueprint for which other doctors use to treat patients successfully. Watch out D.C. because Scarlett and this momma are coming and we’re on a mission with a vision to improve Scarlett’s outcomes and help other kids like her and a passion that is not slowing down.

Respiratory Season

There are many things that cause me apprehension when it comes to Scarlett’s weakened immune system. Respiratory season is one of my top three concerns and it can sometimes send me into panic mode. Scarlett’s initial lupus flare was triggered by a respiratory virus, which we later found out to be the Coronavirus. Many people with lupus experience their first big flare after being exposed to a bacterial infection or virus. Scarlett’s respiratory virus triggered her first serious flare which caused her to have diffuse alveolar hemorrhage over a two month span. Over two months of bleeding in her lungs… that is so crazy!

Scarlett is currently on mycophenolate, which surpresses her immune system and keeps it from overreacting and attacking itself. Mycophenolate replaced her chemotherapy. This medication is just one of the many reasons why she is so immunocompromised. Her nephrologist told me that if she can make it two years without being hospitalized he would consider taking her off it. I recently read a case study, although it was published in 2003, that correlated the use of mycophenolate and pulmonary hemorrhage in children. Research is limited, but the case concluded that mycophenolate improved the chance of a re-occurrence of pulmonary hemorrhage. However, the medication can ultimately lead to life threatening infection. She’s already at high risk of developing cancer because of her positive NRAS gene and mycophenolate increases that risk as well. I’m terrified of her getting an infection or cancer that she cannot recover from, but I’m also terrified she could have another pulmonary hemorrhage.

These past two months Scarlett has been sick off and on. Of course it is the season for germs. She has been on antibiotics multiple times and a round of steroids. She is now recovering from pneumonia. This last bout of pneumonia left me the most worried I have been in a long time. She lost too much weight and got down to 31lbs. She is already very petite and doesn’t have much reserve even though she usually has a good appetite, unless she is sick. This last week and a half she has definitely made a comeback. She has gained 3lbs in less than two weeks!

Scarlett has also missed a lot of preschool due to being sick. This week we kept her home one day because there was another child in the other preschool class that tested positive for influenza. We just can’t take that chance. We plan on sending Scarlett back to school next week. We are very relieved she’s getting better, but we still have a few more months of respiratory season to go. Not to mention we plan on travelling with her in March. Hopefully Scarlett will continue to bounce back from any infection that comes her way!

(Picture taken on 1/9/20. When you need to gain weight you can have ice cream anytime!)

Feeling Blessed this Holiday Season.

Scarlett has been doing well overall since starting preschool this fall. She is currently on a 504 plan at school, there are UV filters on the lights in her classroom, she has a hospital grade air purifier in her room as well, and her teachers are taking extra precautions to avoid germs. Everyone is Scarlett’s life has been doing everything possible to keep her from getting sick. However, this past November she was on steroids again and antibiotics for 20 days to treat an ear infection that wouldn’t go away and pneumonia.

Last week we were in Fargo to see Scarlett’s rheumatologist and pulmonologist. She was still a little sick at that time, but her lab numbers for lupus were good. She has continued to maintain good lab results, which is very encouraging. She is over a year out from her last rituximab so she had more specialized blood work done. Rituximab was a medication she was given in the PICU that helped save her life, but it completely wiped away her immune system. Her recent labs show us that she is still very immunocompromised, but her body is making immune memories, which is a pretty big deal. Her biggest threat at this time is infection.

Scarlett’s lupus is unpredictable, she has days when she doesn’t feel well, which usually means a low grade temperature, joint pain, and complaints of a tummy ache. The days she doesn’t feel well can be discouraging. When she has a flare it usually lasts a couple of days. We constantly worry about her. However, I am really encouraged about her latest blood work, her lupus seems to be under control overall and her kidney function is good. Her pulmonary function is also much improved since staring an inhaler back in August.

I don’t think I’ll ever stop worrying about Scarlett. Her recent blood work has her doctors and myself feeling very encouraged. She just keeps amazing everyone around her with her positive attitude and miraculous recovery. This past week when I asked her what she wanted from Santa. She told me that she already told Santa and God that she wanted all the kids at her hospital (meaning Sanford Children’s) to be able to go home. She has expressed several times now over these last few weeks how she’s worried about the kids in the hospital over Christmas. It’s interesting, since she was diagnosed, she has hardly expressed she is worried about herself. I think she knows she’s going to be ok, even when she is sick like this past November.

Our family wishes everyone a healthy and happy holiday season. We appreciate everyone that has been thinking and praying for Scarlett. Merry Christmas from our family to yours.

Scarlett’s First Day of Preschool

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.

Not Scarlett’s fate.

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.

“Mom, why did Santa Claus take out my trach?”

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.)

Camping and Coughing…

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.

Make A Wish

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.

Surgery, ER Visit, and Most Recent Flare.

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.