The Danger of Not Knowing: A Race Against Kawasaki Disease
You could know someone it's happened to, or maybe, it's happened to you. If not, you might have seen a show about it on Netflix.
Stories of people having an illness or ailment, and doctors not having a diagnosis for it.
Practitioners will do the best they can, but it is impossible for them to have every sickness, disease, and ailment memorized. They, like us, are only human, and can miss the mark on making a correct diagnosis. Unless someone else recognizes the signs and symptoms of a rare case in the sea of ailments, diseases, and illnesses, it can remain incorrectly diagnosed or not diagnosed at all.
I thought these cases were so rare... I never imagined that my baby would be one of them.
We were there at the hospital. My daughter, who had just turned four months old, was being admitted for a strange rash; not eating; vomiting; a mild cough; and a high fever.
Her rash, which first appeared two days before, was the first cause for alarm. I took her into the urgent care on a Saturday evening and while we waited she vomited in her car seat. I took her to the changing room and put on her spare clothes. We were called in to be seen right after midnight.
A general practitioner examined her, but didn't have the proper pediatric equipment to finish the assessment fully. She then explained that the rash was probably just an allergic reaction but there was no way for her to really know for sure. She prescribed her an antihistamine and we went home.
On Sunday, her fever stayed high. She vomited again, was irritable, lethargic, and had stopped eating. Her rash had spread around her neck, back, and torso.
On Monday, I took her to the doctor and he referred her to the pediatric hospital. When we arrived, she was assessed again. Everything then changed gears and went into a panic type mode.
They started talking about the possibilities of measles, meningitis, scarlet fever and fifths disease. Then every preparation to treat and test my little baby was carried out.
Blood tests, swabs, and a lumbar tap were done, then she was put on an iv drip and antibiotics. We were quarantined because there was the risk of an infectious disease; doctors coming in and out with masks, gloves, and aprons.
My husband and I were anxious, frustrated, worried and we felt so helpless. How did our little girl get this? What is it? We decided to put out a Facebook post to our friends and family, explaining what had been happening and asking for prayer for our baby girl, and for answers.
The response we got was overwhelming. So many commented, messaged, texted, and called to give us kind words, prayers, and offered to help with watch our other girls at home, make us meals, bring us essentials and come to encourage us. Many offered their thoughts on possible viruses, diseases, and infections that it might be.
One comment read, "ask them to check for kawasaki disease." I furrowed my eye brows and thought, What is that? It sounds like something you'd get if you were traveling through a 3rd world country. I knew nothing about it, I never heard of it before. How would they even check for that? Wouldn't the doctors already know to check for every possibility? They had already done so many blood tests and assessments. I felt pesky asking them to look for another ailment a friend suggested on Facebook. I didn't feel qualified to ask them to check for other things. I could see they were doing their best to rule out any life threatening possibilities.
We waited for two days before knew anything about what was happening to our little girl. The second morning we were there, the doctor explained that her rash was indeed strange and had all the other doctors baffled trying to figure out what it was. The way it was moving around her body, presenting in different locations each day. He assured me that I'd be the first to know, after him, what the test results were.
I waited. It was 1am the next morning and another doctor came in to apologize that we hadn't been told yet what was happening.
The results had come back, she was RSV and RV positive and the measles and meningitis tests had come back as negative. This was good news, but it felt like bad news all at the same time. She had two viruses at once but neither of those viruses were known to be associated with rashes.
The doctor went on to explain that she would be taken off the antibiotics as it'd have no effect on a virus. I asked why she had the rash and she said it was probably just her body's way of fighting two viruses at once.
The next few days she'd started to eat again, and her oxygen levels were at 100%.
In the evening on the fourth day in the hospital, a nurse came with discharge papers to tell us that we could go home. She informed me that all they could do for the viruses was to offer assistance in oxygen or to give iv fluids if she was to get dehydrated. Since she didn't need those things she could recover at home.
My heart sank. Something still didn't seem right. Her fever was still constant and high. Nights seemed to be the worst, fever spiking and her waking up in pain. Her hands and feet looked like big puffy marshmallows. She'd been sleeping all day except to wake to cry and for feedings. She hadn't smiled since the fever started a week earlier, and she cried every time I lifted her up or moved her.
I asked the doctor if she could stay another night because I wasn't comfortable taking her home with her still being so ill. The doctor agreed and allowed us to stay to monitor her through the night. Another rough night went by with a spiking fever that wouldn't go down, even with medicine.
She had a diagnosis but things just weren't adding up. The doctors came in the next morning to assess her again and then to say that we could go home that day. I expressed my concerns again about the constant high fevers, the swelling and the rash. They said it was probably just a reaction to the two viruses and we'd just have to wait it out.
To be honest, the only words I kept hearing were, "probably." I knew something else was going on but I didn't know what to do. I didn't like hearing them say that they weren't sure why she was reacting the way she was but I felt so helpless to do anything about it.
Since she started eating again and we had the positive test results for RSV and RV, we were discharged and given a 48 hr open access back into the assessment unit.
It was the weekend and our 48 hr open access came and went. I asked a Facebook group of moms if they had ever heard of someone getting a rash from RSV. I got many different comments suggesting possible allergic reactions, viral hives, adnoevirus, vaccines, and Kawasaki disease. I looked up and considered each of their suggestions but still couldn't determine what it was.
On Sunday, her cough became more persistent. She still had a rash, fever, and was lethargic. If we moved her or even looked at her she would just look back at us and cry. We missed her smiling face and knew she still wasn't herself.
Monday morning came and she had been coughing all through the night. Two hours went by and she hadn't stopped coughing so I tried to phone the doctor. I couldn't get through on the line, so I quickly packed my bag and drove her back to the hospital.
As soon as I pulled into the hospital she fell asleep. I was already there so I went in and registered with the reception. A nurse called us in and she'd recognized us from the first time we had been admitted. " She's looking a lot better than the last time I saw her. Why did you bring her in today?"
I responded, "She was having a coughing fit and I didn't know what to do."
I felt embarrassed. She was peacefully sleeping in her car seat. I knew that she was much better than she had been the week previous as far as eating. Her fevers hadn't been as high. Her rash was in another part of her body but it didn't look any worse than it had before. Her hands and feet were still puffy, but why was I there?
I think it was because I knew that something was still wrong, and I wanted them to tell me what it was. But instead, I got a mild lecture of coming to the wrong department, and told again that it was just a virus and there was nothing they could do to help. I was told by the nurse that unless her lips or toes were turning a paleish blue, she was unconscious, or unless she stopped eating or was gasping for air, that I shouldn't worry. She assured me, "We don't normally do this, but I'll ask one of the pediatricians to come speak to you to let you know that everything is okay."
I felt like a shamed fool; like I had jumped the line to be seen before others who were needing care. Like an overprotective mother. I felt so stupid. She showed me to a room, and said the doctor will be with you shortly. I sat with my baby. Tears rolled down my face, in frustration. I wasn't sorry that I'd brought her in, because I wanted to make sure she was okay.
I regained my courage, gathered my thoughts and wiped the tears from my eyes and waited for the doctor.
The doctor came in and assessed her. He observed her saying that she still did not look well and reassured me that I could come back to the assessment unit if she stopped eating or started to get worse. I thanked him and we went back home.
A day passed and her fever finally broke. Her rash only remained on her legs and a little spot on her cheek. Her swelling went down and she had been staying awake a little bit longer during the day. On the same day, her lips formed a white film and crusted over. I thought it was odd, but I was hopeful that she was starting to improve.
The week went by and she seemed better but still not herself. She still hadn't smiled, and her lips and the top of her nose were cracked and constantly bleeding.
On Friday evening, I decided to google what the best remedies for severely chapped lips were. I'd been putting some thick natural baby moisturizer on her lips every hour but each day they looked worse than before. I searched images and couldn't find anything that looked even close to what hers looked like. That's when I knew, this was something else. It was coming into the weekend, so I decided I'd take her to the pharmacist in the morning to see what they suggested. Then I remembered something I'd read about red cracked lips as a symptom. I then recalled what it was. It was one of the symptoms from the Kawasaki disease page I'd researched a week earlier.
I looked it up again and I read the symptoms.
"There's no single test to diagnose Kawasaki disease, but there are some key signs that suggest a child may have this condition. Your child may have Kawasaki disease if they have:
High temperature (fever) of 100.4 F (38C) or above for longer than 5 days and at least 4 key symptoms. These symptoms include:
Red eyes – where the whites of your child's eyes are red and swollen
Changes to the mouth or throat – such as dry, cracked lips or a red, swollen tongue
Changes to the hands and feet – such as swollen or painful hands or feet, or red or
Peeling skin on the palms of the hands or the soles of the feet
A rash
Swollen lymph nodes in the neck
The skin on your child's fingers or toes may become red or hard, and their hands and feet may swell up. Your child's hands and feet may also be tender and painful to touch or put weight on, so they may be reluctant to walk or crawl.
https://www.nhs.uk/conditions/kawasaki-disease/diagnosis/
I couldn't believe it. This sounded so similar to what had been going on. But the article said it was a rare disease and I knew she didn't have the peeling skin nor the strawberry tongue, so I started to read more.
Kawasaki Disease can affect anyone but it usually occurs in ages 6 months to 5 year olds. It is predominantly found in those of Asian descent and found more often in boys.
I thought to myself, she is none of those predominant defining criteria.
I read another article stating the rise of the disease, or rather the awareness of it. It is unknown what exactly triggers the disease but it usually presents as a virus, reaction, or bacteria before it is correctly diagnosed. It was named after a man in Japan who first recognized and described it in 1967, Dr. Tomisaku Kawasaki, but the disease has been around for far longer.
I started to feel sick as I read on, realizing the dangers of this acquired heart disease. KD is when the immune system starts to attack itself, and the vessels in the body become inflamed. In some cases the inflammation, especially if left untreated, causes permanent damage to the hearts vessels and in some cases causes aneurysms, and in some cases being fatal. The younger the child is the more of a serious risk it can be.
For the best chance of making a full recovery, treatment should be given by day 10 of when the fever first started.
As I read this, I knew that this was day 15 from when her fever first started.
My thoughts were being torn in two completely different directions. I thought that either I was being overly paranoid or I was being neglectful for not rushing her into the ER right away. I didn't know who to call, or what to do. It was the weekend again and our "open access" was over the time allotted. She didn't look like an emergency and since this disease was so "rare" I didn't know how to ask them to assess for it.
I wasn't even sure if it was what she had. I didn't want her to get poked again with needles and put through anymore tests unnecessarily just because I wanted peace of mind.
I searched images and public Facebook posts that had anything about Kawasaki Disease. I compared their rashes with my daughter's and sent messages to a few Kawasaki pages asking if they could give me an online consultation for my daughter who I thought might possibly have this disease.
It was late and I somehow managed to convince myself on the side of that she was looking better and I was probably just worked up and overthinking everything. She didn’t have the peeling skin nor the strawberry tongue, so I thought she probably didn’t have this disease. But, if it was- if she did have this illness, I prayed to God, asking Him to give me a sign.
The next day we went to church. My baby just slept the whole service and afterwards woke and started to cry. She still looked miserable so I excused myself early and went home with my girls. As soon as I got home I looked at my email and the heading read:
This is very suspicious for KD. This is urgent! Go to medical provider and demand blood work to include: CBC with manual differential ESR CRP ALT GGT I am on call and happy to speak to a physician on call why this is an emergency. If ESR is elevated she needs to be treated with IVIG even if fever gone. Needs echocardiogram ASAP with sedation so that they can see the arteries. This is urgent!!
The email read on to give me details of who to contact for help.
This was the sign that I had prayed for. It couldn't have been more clear what we needed to do next.
The email confirmed my worst fears but also calmed me in the same instant. I now knew that I wasn't paranoid. I had a referral and specific instructions from a completely qualified medical professional and professor.
From there I took her into the A&E and she was admitted that evening.I showed them the email and told them the history of what had been going on, and showed them the pictures of her rash progression.
She was seen by a doctor who assessed her for Kawasaki symptoms. Her lips and the top of her nose was cracked and bleeding. Her heart rate was high and kept getting higher each time they checked it. A different looking rash started to come around her body. The palm of her hands were red and flushed, and the inside of her ears were inflamed.
After combining all the history and current symptoms the doctor confirmed the Kawasaki diagnosis and advised to begin treatment as soon as possible. She was then seen by the main cardiologist of the hospital and by more doctors and specialists.
They then explained to me the dangers and risks of the IVIG treatment, and said they'd be moving her to the high dependency unit.
In Kawasaki disease, the longer the inflammation and fever persist the more risk of there being damage done inside the bodies vessels. Immunoglobulins (IVIG) therapy is made up of donated plasma and super antibodies that help the body overcome the illness more quickly.
It was day 17 from when her fever first appeared and she was finally getting the treatment she needed.
The next day after the ivig treatment, she was transferred to another hospital where she was observed and treated. She had an ekg, a cardiac CT scan and an echocardiagram (ultrasound of the vessels in the heart) among other tests to determine if any damage from the KD had been done to her vessels.
The echo results showed that three of her heart's arteries were slightly enlarged. This was devastating news and good news all at the same time. Her coronary arteries were inflamed, but since she got the treatment needed the inflammation had been greatly reduced.
The following weeks she was able to recover at home. She was on four different medications and went back into the hospital every few days for blood tests until her inflammation levels returned back to normal. They repeated two more echocardiagrams and a month later she was able to come off of all her medications.
Not all cases are as lucky as my daughters. Those who are found to have larger dilation or aneurysms have to remain on aspirin and other medications to keep the heart from getting any worse. In some cases the hearts vessels heal, but in other cases inflammation can continue for years. Echocardiagrams are scheduled yearly and sometimes more often if more risk is suspected.
If she had been left untreated, her story could have ended very tragically.
I am thankful that God was looking over us, and leading us to the right people at the right time. I know that He didn't give me peace about her first diagnosis and that I needed to keep searching for answers.
As bizarre as this story may sound, it happens more often then we realize. Many KD stories are very similar. It is initially diagnosed as a virus or as an allergic reaction. Unless someone recognizes the signs, it can go undiagnosed and can lead to permanent heart problems and in some cases can become fatal.
Kawasaki disease is not contagious. It is a rare illness that presents specific symptoms of what reactions are going on inside of the body. It is more common then meningitis, but it is not more known. It is the leading cause of acquired heart disease in children in many modern countries today. It can be easily overlooked, as the symptoms can come and go. Most people know the signs of a heart attack in adults. Kawasaki is a type of heart disease known to happen in children, teenagers, and less frequently seen in adults as well.
If you believe your child has KD symptoms, keeping good records and pictures of the progression of the illness can help diagnose your child sooner.
Since they don't yet understand what exactly triggers, nor who exactly is susceptible to get Kawasaki Disease, I believe that it is not only pertinent that doctors and pediatricians recognize and know the signs of Kawasaki Disease, but also every parent should be aware and know the signs of Kawasaki Disease.
I hope some will read this and learn from my daughter's story so that they are able to get the treatment needed as soon as possible.
Some useful links to learn more about Kawasaki Disease.
https://kdfoundation.org/aboutkd/
https://www.societi.org.uk