This is the story of Kimberlee's journey with Toxic Epidermal Necrolysis. We hope our experience will help all those with children affected by SJS/TEN
Faith: Seek Without Seeing
Hope: Do Without Doubting
Charity: Love Without Lacking
Kindness For Kimberlee wants to spread our message of hope and compassion. We believe that a single action can make a difference in the lives of those affected by Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.
Kimberlee's Experience with Toxic Epidermal Necrolysis (TEN) - Written by her father, Dustin Waters
The weekend of the 22-23nd of September 2018, Kimberlee who was 13 years old at the time, came down with a fever. All day Saturday the 22nd, she just laid on the couch having a low-grade fever that never got much above 101 degrees Fahrenheit. She had no other symptoms, other than a headache and a mild fever.
The next morning, Monday, September 24, 2018, Kimberlee woke up feeling well enough to go to school. I distinctly remember her coming home and saying she was somewhat tired. I began to be a little worried, because this was unlike her. I worried a little that she maybe had strep throat as she had had vague symptoms before with an episode of strep throat. The next morning, Tuesday, September 25, we were even more concerned with the possibility of strep throat, although we would be proved wrong as the day went on. Kimberlee woke up that morning with a rash all over her face and trunk. We took her to her pediatrician, again concerned for strep throat. The rapid test was negative, making strep less likely. She also had some other concerning symptoms. She had a single swollen lymph node in her neck, and had conjunctivitis as well. She also complained of a sore throat that morning. Her throat looked a little red and blotchy, but nothing out of the ordinary to me. After she got home from the doctor, and I got home from work, her rash was continually getting worse. By 4 or 5 o’clock in the afternoon that Tuesday, she started getting blisters on both her skin and her lips. By now, I was very nervous. I was starting to get concerned for Kawasaki syndrome with the rash, conjunctivitis and lymphadenitis. I remember texting a resident that we were going to go to Kenya with in a few weeks, both about Kenya, and randomly about Kimberlee. I was secretly concerned about how serious this could be. I can’t remember if it was at this point that I was concerned about Steven’s-Johnson syndrome (SJS) or if it was later, but I’m sure it crossed my mind at this point, because of her mucosal involvement. It just didn’t make sense, though because she hadn’t been on any medications that were likely to be associated with SJS. We (Kimberlee and I) FaceTimed with pediatric hospitalist on call, and he raised the possibility of SJS as well, but some random viral or bacterial infection was on his radar as well, and also Kawasaki’s was in there as well. He wasn’t super concerned about admitting her that night, saying that she didn’t look sick enough at the time, and recommended following up in the morning.
The morning of Wednesday, September 26, Kimberlee was much worse. Her rash had spread to her extremities and was starting to blister. I called Dr. Beverley back, and he said we could admit her to McKay, or just go straight to Primary Children’s. I called Cindy and let her know that this is what we should do, head to Primary Children’s. Cindy left with Kimberlee, and I left as soon as I could get things straightened up at work. Cindy and Kimberlee arrived about 5 minutes before I did. By the time I arrived, there were probably 10-15 healthcare workers in her room. Dermatology was also rather emergently consulted while she was in still in the emergency room. They were somewhat hesitant to confirm a suspected diagnosis of Steven’s Johnson Syndrome, or to talk about a prognosis, but I could tell they were pretty sure that SJS was what we were dealing with. I was not prepared for how bad this would turn out to be. The case or two of Steven’s Johnson Syndrome that I had seen were not that severe, with the exception of one case of Toxic Epidermal Necrolysis that I saw in my first year at McKay-Dee Hospital, and that patient ended up in the burn unit at the University of Utah, and I am unsure of how he actually did.
It was decided that Kimberlee would need to go to the intensive care unit, so she was taken up there. She, Cindy, and I went up there and got her all settled in. We again told her story to the attending PICU physician, the critical care fellow, the resident, nurses, students, and others who were in the room. They all did think it was ironic that this was likely infectious-related, especially considering this was an infectious diseases pharmacist daughter. She was getting increasingly more uncomfortable and was having difficulty swallowing her secretions as her mouth and throat were getting much more sore and inflamed. One discussion that was had after seeing how severe her case was, was about using cyclosporine to treat her SJS/TEN. I hadn’t heard of that, but I knew I would research it when I got home. We decided that Cindy would stay the night with her that night, and I would stay the next night.
The next morning on my way to the hospital I called Cindy on the way to see how Kimberlee was doing and she just said “You will see when you get here.” She did not sound encouraged, which made me nervous. I was not prepared for how bad Kimberlee would be when I got there. When I walked in to her room I was shocked. She was being examined by the dermatologist, and had a huge blister under her chin (she literally looked like a pelican) and large blisters all the way down both sides of her back. The dermatologist, talked with us about cyclosporine, as she had more than 40% involvement of her BSA and so did the intensivists. After my research, I definitely wanted Kimberlee to get this medication as the data looked promising.
During rounds in the PICU, it was again brought up about using cyclosporine. I indicated to the team (and when discussed with the dermatologist I indicated the same thing) that I felt that using cyclosporine in her case was the right thing to do. At this point, they felt like she had about 42% of her body surface area covered, which being greater than 30%, would put her in the category of toxic epidermal necrolysis (or TEN for short). This diagnosis made me extremely nervous, because what I knew of TEN was that the mortality was extremely high, and that it was more like a burn than any other disease. Also at this time we talked about the possibility of intubating Kimberlee. I told Cindy that I thought that although I didn’t like the prospect of Kimberlee being intubated, I felt like in this situation it was the most humane way to help her heal. Cindy readily agreed. Ultimately at this point we decided not to intubate her, but she was getting increasingly miserable. Her eyes and throat were extremely dry and irritated, and she kept asking for ice chips. By about 11 o’clock that morning, which was September 27th, it was evident that Kimberlee’s disease was going to continue to progress over several weeks. The PICU attending and the critical care fellow came to us and told us they thought she would be best served in the burn unit at the University of Utah Hospital. As Cindy and I talked, I knew that it was the right place for us to go. They were very adept at dealing with pain management, wound care, and fluid and electrolyte balance. It would take several hours for them to get us transferred over there, so Cindy and I just tried to keep her as comfortable as possible. The ophthalmology resident came back in and checked her eyes, and said that her corneas looked good still. About an hour before they transferred us over there, we finally got started on the cyclosporine. I knew the dose was 3 mg/kg, but most of the studies had used oral cyclosporine down the feeding tube. Hers came up as the IV formulation, because she couldn’t swallow anything more than just ice chips. I remember having the distinct thought, “Hmmmm, I didn’t know that the biovavailability of cyclosporine was 100%.” I had checked pretty much every medication that she had been given, but on this one, as it was kind of out of my league, I just checked to see what the dose was and left it at that. Later, me not checking on this, would prove to be about the biggest blessing of this whole ordeal.
Shortly after the cyclosporine was started, Cindy decided that she would go home, as she was very tired from the previous night of being up all night with Kimberlee and helping her. So soon after the cyclosporine was started, the LifeFlight nurses came to package Kimberlee up and take her across the walkway from Primary Children’s to the burn unit at the University of Utah Hospital.
After we got to the burn unit, the staff all asked a million questions again. Had she taken any medication? What was her health history like? When did her symptoms start? What had happened that led up to this process? These and a myriad of other questions were going strong. After she got somewhat settled, I went out to the parking garage at Primary Children’s to move the truck over to the parking garage at the University of Utah Hospital.
Shortly after I returned, the burn doctor, came to talk to us. He said that she did have extensive disease and had approximately 40-50% body surface area involvement. He may have said 60%, I can’t really remember. I only remember thinking of the mortality rates with TEN and that much body involvement and I felt numb. Was Kimberlee really going to die? That was all I could think. We (Amberlee and I) went back in to see Kimberlee. She was still pretty out of it at that point. We waited a little bit and talked.
After all of the procedures around trying to get her stabilized were finished, I tried to lay down and get some rest, because I knew it would be a long night. I had to help suction her, because her throat hurt to bad for her to swallow her secretions, and she was complaining about her eyes still. I tried to lay down on the couch that folded into a bed, but I couldn’t lay down without having heartburn problems. So, I sat up in the chair next to her bed and tried to sleep. I knew that things were not going great for her overall because her urine output was starting to dwindle. After a couple of hours of urine outputs of 10-13 ml/hr, she finally started making more urine. 25 ml/hr, 30 ml/hr, and that is where she stayed for awhile. It wasn’t great, but I was glad for at least that much. In between dozing and watching her urine output, I was awakened by her vitals alarms going off, and wondering if her blood pressure was going to hang in there. Her blood pressure wasn’t the best either. That night, I never thought morning would come, but finally it did.
Cindy arrived back at the hospital around 9 or so that next morning, Friday, the 28th of September. About the time she got there, they were ready to do wound care, and I can’t remember if we just met downstairs for breakfast, or if she came to the room first, and we went down there, but while they were doing wound care, we went and got something to eat, I think maybe over at Primary Children’s. Cindy and I got back to the room and they were done with wound care, and the nurse told us that Kimberlee had had a fairly funny moment during wound care. While trying to keep her mind off of the wound care, the nurse talked to her about her friends, about school, about what she liked to do, etc. In the middle of all of this, Kimberlee raised up and yelled “Slackers!!!” to which the whole team laughed and got a kick out of. This day, she showed a little more of her Kimberlee self, although she was still in a great deal of pain, and was overall pretty miserable.
That afternoon, a new ophthalmologist came in with a resident to examine Kimberlee’s eyes. After putting the dye in, it was clear that she was starting to have corneal involvement. The ophthalmologist said that the standard of care for SJS/TEN patients with corneal involvement was to have an amniotic membrane placed over the corneas to help protect them and to help them heal. I didn’t even think twice about giving the ok for them to do this. I knew it was the right thing immediately. . They said that the surgery would be first thing Saturday morning. The next morning, surgery to place the amniotic membrane went well.
I returned to the hospital and visited with Cindy for awhile. Kimberlee was still on the ventilator, so there was no visiting to be done with her. Cindy was pretty spent from her two nights in a row at the hospital, so she left to come home, and I stayed with Kimberlee. From here on, everything that happened and on what specific day is a bit of a blur, but I will do my best to get everything straight with the help of Facebook.
The rest of what I remember about Sunday night is not pleasant. I remember that night about 9 pm Kimberlee’s urine output started dropping again. I had visions in my head of her having to go on CRRT, or Continuous Renal Replacement Therapy, which I knew was not a really good sign. I finally broke down to the nurse, who was wonderful. Through tears I asked her, “Am I just torturing my daughter to death?” She hugged me and said, “ No, we will try every intervention” at which point I felt a little better. Her urine output still struggled that night. While she was out of the room, I believe it was that night, I gave Kimberlee a blessing. That was a special experience for me as a dad, to have it just be me and her in the room as I blessed her that she would heal, that her kidneys would work properly, and that she would be strengthened to make it through the difficult circumstances that she was and would continue to face. I didn’t sleep much again that night, as Kimberlee needed to be calmed down at times. I kept having to remind her she was in the hospital with a tube in her throat to help her breathe. This, combined with the propofol and fentanyl, oxycodone, and gabapentin helped to calm her. She had had problems earlier in the day with her blood pressures, likely from needing furosemide to help pull water off her lungs. They did have to turn down her sedation some, which made her more fidgety. On this day too, her Mycoplasma IgG was slightly positive, giving us some hope for what was the cause of the SJS/TEN, although this would prove to not be the case.
The next day, Monday October 1st, was a day of little miracles. In the night, her kidneys really started mobilizing fluid and her urine output was great. I was very encouraged by this, and was very happy that her kidneys had finally started figuring out what to do with all that fluid. Also, the night before, a terrific respiratory therapist asked Kimberlee if she was bored. As she was on the ventilator she couldn’t answer verbally, but she nodded a vigorous yes!!! This respiratory therapist then asked her if she would like to listen to a book. The only one I could find on my phone was a Harry Potter book. Kimberlee wasn’t thrilled with that, but it was the best I had. She was ok with it, she guessed. The burn doctor on the morning of the 1st (Monday), said during rounds that he was optimistic that she could be extubated as early as Wednesday. I was excited at that prospect, but also wanted her to be as comfortable as possible.
Tuesday, October 2nd, was an exciting day. About 2pm that afternoon she was extubated. I was worried about how well she would be able to control her secretions. That morning when the dermatologist came to see how she was doing, she said that she was way ahead of schedule, in part due to the whopping dose of cyclosporine she got to start off her treatment. They had not been able to give her the first dose enterally, which the correct dose was 3 mg/kg, but instead had done it IV. The correct IV dose would likely have been 1 mg/kg, instead of the 3 mg/kg dose that she was given. I joked that I would someday write a book called, “How a medication error saved my child.” One thing we were grateful for today was that Kimberlee smiled at a joke that her friend sent on a postcard to Kimberlee. She sent postcards most every day with a joke on them, and they helped keep her spirits up. I was always concerned and asking what each intervention was, whether it was a medication, or whatever. The nursing staff swore they weren’t getting mad at me, but I was pestering them pretty good, but I didn’t care much, because Kimberlee was my daughter and I was determined that an error was not going to hurt her.
Wednesday, October 3rd was an okay day. Kimberlee continued to struggle with wound care. After every wound care her heart rate and respirations would skyrocket and we would have a hard time getting her under control. Her temperature would go up, she would rigor, and overall just be plain miserable. After I left, Kimberlee was able to sit up at the bedside with Cindy for a short few minutes. This felt like some progress, so that was encouraging. Additionally, the ophthalmologists were pleased with the progress of her eyes. She was still not able to open them, but the progress was good.
I don’t remember much about the day to day activities after this time, but I did keep track of everything that happened on Facebook, so will have to refer to my posts for more detail. I don’t remember much about that night or afternoon, other than that every day when I went home, I slept for an hour or so.
Thursday October 4th was an extremely frustrating day. Kimberlee had not tolerated wound care very well at all every time they did it. She would get tachycardic, tachypneic, hypotensive, and febrile every single time. Cindy and I were present for wound care this day, as the child life specialist asked us if we would be there to support her. Neither of us wanted to be there, but at the child life specialist’s insistence, we stayed. It was horrible. They scrubbed and scrubbed her, and she was in horrible amounts of pain, despite being sedated some, and given fairly reasonable doses of fentanyl
Given the fact that Kimberlee was not tolerating wound care, a burn doctor who was new on that day suggested that she go to the OR the next morning for comprehensive wound care/cleaning and placement of a special bandage, called Suprathel, that would hopefully help with healing and decrease the need for daily wound care. This prospect seemed like a step in the wrong direction for us, but what choice did we have? Kimberlee was not tolerating daily wound care without some medication to help, and the treatment teams were loathe to give her any, worried that they might give her a medication that had brought on the SJS/TEN. She would develop a fever and high heart rate every time wound care was done, and her respiratory rate would get into the high 30s. It was hard to get her to calm down after any wound care session. So, we thought it would be best to put the Suprathel dressing on. It seemed like the lesser of two evils.
The next couple of days were relatively non-descript. On October 7th, the highlight of the day was that Cindy got to go wash her hair for the first time. It was pretty gross since it had been 10 days plus some since she had washed her hair. My parents went to say hi to her on their way home, as did Cindy’s brother Sam, and his son Braden, who was just ready to leave for a mission to Liberia, West Africa. This is the first real day that Kimberlee remembered being in the hospital. On October 8th Kimberlee was doing well enough to play several rounds of “Who Wants to Be A Millionaire” as I asked her questions and she answered. She did really well and had a fun time doing it
October 9th, 2018 was a scary day for us. Kimberlee underwent wound cleaning under deep conscious sedation. The staff were amazed at her healing, and were saying that she was a week or two ahead of schedule. The scary part of today was that just before I left to come home for the day, her foley catheter quit working. The nurse thought that it was likely just clogged so she flushed it which helped a little, and it started working again. However, after I left it clogged again. Additionally, she developed a fever of 104 degrees, was much more somnolent, and less interactive than she had been the night before. At this point Cindy called me and I was very worried. I was afraid she was getting septic, which appeared to be true. I called and talked to the PA and voiced my concern. It wasn’t until 1:15 the next morning before they started antibiotics and replaced her foley (her bladder had 800 ml of urine in it, more than twice what is normal). Luckily with the initiation of antibiotics she quickly turned around, and this is when she really started getting better.
The next day, October 10th, 2018 she started working more with physical therapy, and sat up a little on her own, which was a huge accomplishment, considering she had been in bed for two weeks straight. She was also doing well by taking clear liquids, another great accomplishment. She worked with PT today, and sat up a little on her own, which was great.
October 11th, 2018 was a wonderful day. Kimberlee was joking around, and told all the PT folks when they put her on the tilt table, “I’m not going anywhere” when asked if she was ready to get on. It was wonderful to see her bright shining self showing through. After wound care today, they left all the dressings off because her skin was starting to look so good
From this point on, the improvement came increasingly quickly. On October 12th, 2018, we had another great day. This morning, she had the ring holding the amniotic membrane in place removed, and as soon as she opened her eyes she yelled, “I CAN SEEEEEEEE!!!!!!!!” It was terrific. Right after that we were able to go outside and take Kimberlee in a wheelchair. She loved the opportunity to do that. That night, she hit another milestone as she got up and went to the bathroom on her own. She did have quite a bit of nausea and vomiting over these days, likely due to the buildup of gunk on her feeding tube and losing so much tissue in her mouth. The next day, October 13th, 2018, Kimberlee was able to get up to the bathroom several times with assistance, and pulled herself up on her own several times. She also got to shower for the first time which she said felt wonderful. She was also able to walk for a few laps around the burn unit. Also, on this day, I reflected on the many phone calls, texts, messages, gifts, acts of service, and love that were shown to our family. It was beyond overwhelming.
Day 19 of Kimberlee’s illness was October 14th, 2018. At that time, 3 weeks seemed like an eternity to Cindy and I. This day was again another day of vast improvement for her. She got another shower, and her diet was advanced to a soft pureed food diet. She also made 2 sets of 5 laps around the burn unit. We were so grateful for the progress that she was making, and in such a timely manner too. It really was like a miracle.
The next day, October 15th, 2018 was another day full of progress. Kimberlee made it 20 laps around the burn center. In addition, she ate about 700 calories that day, which was on her way to the goal of 3000 calories she had to help her skin heal. She also had a good time beating Cindy in Clue, and got another shower, which was about the highlight of her day.
On October 16th, 2018, we went with her nurse, and went on a walk around several other areas of the hospital. We walked up and down some stairs as well. I was amazed at how quickly she had been able to get up and move, and how quickly she had got back to normal. It was truly amazing. Cindy played “Food Sorry” with her today, where if she rolled certain numbers she had to take a bite of food to try and get up to the caloric intake required to help her skin fully heal.
October 17th was an amazing day. We found out on that day, that Kimberlee would be going home the next morning. She got her feeding tube out, her midline catheter out, and was eating pretty well. Kimberlee was so thankful for all those who fasted, prayed, offered service, visited, and supported our family in any way during her hospitalization.
October 18th, was the most glorious day. Kimberlee got to come home!!! We left the hospital about 11 am, after getting everything packed up from her room and ready to go. A friend had told us that they wanted all the girls to be there to meet her when she got home, and as we pulled into the subdivision there they all were! It was the best welcome home ever. All of them were wearing their “Kindness for Kimberlee” shirts, and let teal colored balloons go, right as we pulled in. It was so wonderful.
I have reflected over and over on this experience. It was difficult, to say the least. Steven’s Johnson Syndrome/Toxic Epidermal Necrolysis, to the extent which Kimberlee had it (70% BSA involvement) carries a mortality rate of anywhere between 20-80% depending on which article you read. I knew these statistics going into her illness. I was an emotional, physical, mental, and spiritual wreck. The most amazing part of the whole experience was how much love, support, and strength we felt from all those around us. I cannot begin to count the calls, texts, Facebook messages, comments, prayers, and hugs that we felt and were blessed with during her illness. Still to this day (nearly a year later), I get teary eyed just reading all the comments on Facebook and remembering the love I felt from friends, our ward, family members, and most especially the Lord Jesus Christ and our Father in Heaven. We had many visitors at the hospital when it was feasible. Justin and Jan Oliver came, Presidents’ Jackson, Barnes, and Charlesworth all came and visited. My cousin Michelle came, and just visited with me in the lobby. She had coordinated with all of my Hope family to provide some financial support for us, for which I was tremendously grateful. Also, Heather Hinckley, a childhood friend, whose husband is a dermatologist, came to say hi at one point too. This visit was near the end of Kimberlee’s hospitalization, and at that point, I remember not having anymore tears to cry. My parents, Carol Anne, Cheryl and Allen and Owen all came, and all of the Young Women from her class, which made her very happy. I have detailed many of the service activities that we received, many of which I still don’t know about. The hospitalists at the hospital had all gone in on a gift basket for us, and Brandon Zaugg had given her a tablet and their family had given her a gift basket as well. We were overwhelmed from all the support.