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.
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!)
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.
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.