My name is Dr. Edmond Sarraf, better known as Dr. Ed, and I am a practicing integrative pediatrician in the LA/Beverly Hills area. I have been asked by several of my patients to comment on what may be this Kawasaki Disease (KD) phenomenon that is happening to some kids around the world after getting Covid infection. In the past 19 years of practice, which includes residency, I have hospitalized what I recall 8 kids with Kawasaki Disease (KD). If I recall correctly, six kids were typical KD and 2 were Atypical KD.
There is currently a lot of worldwide reports of this rare, but real inflammatory condition in children in some parts of the world, so lets take this opportunity to discuss some of the classic findings, compare it to Covid and perhaps put some perspective to all this.
The more we pay attention to something, such as the possibility of KD, the better it is to get the right diagnosis, KD can be tricky and can mimic other illnesses, but if we pay attention, and even bring it to the attention of our doctors, then we will likely get the diagnosis earlier and for proper treatment, enhance avoiding the complications of KD!
What is Kawasaki Disease
KD is widespread, systemic, INFLAMMATORY (“fire”) REACTION that primarily affects the medium-sized muscular arteries – primary the coronary arteries of the heart.
What Causes it?
NO ONE KNOWS FOR SURE! A number of theories link the disease to bacteria, viruses or other environmental factors, but none has been proved. One reason, viruses are suspected, are times like this, where we see increased clusters of KD with widespread infections, suck as Covid. Certain genes may make your child more likely to be more susceptible.
A variation in the ITPKC gene has been associated with an increased risk of KD. The ITPKC gene provides instructions for making an enzyme called inositol 1,4,5-trisphosphate 3-kinase C. This enzyme helps limit the activity of immune system cells called T cells. Other genes have also been implicated.
ghr.nlm.nih.gov/condition/kawasaki-diseasehttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC6224133/
I pray that one day we would make medicine more personalized and investigate people who get certain less common diseases or have a family history of certain disorders/reactions so that we can make medicine more personalized in the future. We can then apply that knowledge so that we reduce reactions to various hard to avoid circumstances in our lives, reducing unfortunate reactions to certain foods, vaccines, medications, and infections.
Diagnostic Criteria for Kawasaki Disease
Main sign: FIVE days of FEVER without other known causes with 4 of 5 of following criteria
- Red eyes without pus (conjunctival injection)
- Dry cracking lips, red throat and tongue where you can see the taste buds look like strawberry (picture included) (oral membrane changes)
- Swollen or red hands and feet (early) or loosing finger nails (late)
- Red, Tiny or slightly raised patches of skin
- Large lymph nodes (greater than 1.5 cm) of the neck (cervical lymphadenopathy)
Diagnosis sometimes can be difficult since this is NOT a common disorder.
Sometimes there are some symptoms that develop before 7-10 days before the main criteria:
- Diarrhea, vomiting, or abdominal pain – 61 percent
- Irritability – 50 percent (older children with KD more commonly present with lethargy than irritability)
- Vomiting alone – 44 percent
- Cough or rhinorrhea – 35 percent
- Decreased oral intake – 37 percent
- Joint pain – 15 percent
- Irritability – 50 percent (older children with KD more commonly present with lethargy than irritability)
- Vomiting alone – 44 percent
- Cough or rhinorrhea – 35 percent
- Decreased oral intake – 37 percent
- Joint pain – 15 percent
Atypical Kawasaki
This is a tough diagnosis because the only presenting symptoms may be prolonged fever. One may or may not present with any of the other criteria. So this can easily be an easily missed diagnosis if one does not think of it. So far, I have had 2 patients that have presented in this manner and after hospitalization and a work-up, we found out that they likely had Atypical KD. After treatment with IVIG, they also rapidly recovered.
“It is typically a self-limited condition, with fever and manifestations of acute inflammation lasting for an average of 12 days without therapy”. The problem is that the longer a patient has fever without treatment, the higher the risk of aortic aneurysms.
How Common Does This Happen?
Kawasaki disease is listed as a “rare disease” by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Kawasaki disease, or a subtype of Kawasaki disease, affects less than 200,000 people in the US population.
Ophanet, who are a consortium of European partners, currently defines a condition rare when if affects 1 person per 2,000. They list Kawasaki disease as a “rare disease”.
The incidence of this disease, or rate that this disease seem to occur at any particular time period, is approximately 1 in 271,440 or 0.00% or 1,002 people in USA [Source statistic for calculation: “30 cases in NJ 1998 (NJ DHSS)”
https://www.rightdiagnosis.com/k/kawasaki_disease/prevalence.htm
Work-Up
There are no simple “smoking gun” tests for KD. We check various inflammatory markers and other blood tests to rule out other infections.
Typically, the following blood tests are typically obtained on children in whom a diagnosis of KD is being considered:
- Complete blood counts with differential white blood cell (WBC) counts
- Liver function tests including aspartate transaminase (AST), alanine transaminase (ALT), and albumin
- C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR)
- Urinalysis
Elevated WBC and platelet counts, transaminases, and acute-phase reactants, as well as anemia and pyuria, are suggestive of KD. Almost all my patients interestingly, including my last patient in 2018 had pyruria (which are white blood cells in the urine without infection, such as bacteria/virus that grows out in culture). Now this is especially important finding in males who over the age of one, are very unlikely compared to females to get Urinary Tract Infections (UTIs).
The most important diagnostic workup in someone suspected to have KD or Atypical KD is an Echocardiogram. This is an ultrasound of the heart and needs to be done with an experienced technician who will measure to see if there is any signs of dilation of the coronary arteries or complications of aortic aneurysms formation.
Treatment
The American Heart Association (AHA) and American Academy of Pediatrics (AAP) guidelines. The recommended initial therapy includes intravenous immune globulin (IVIG). All published guidelines also include aspirin (30 to 50 mg/kg daily divided into four doses) with IVIG as initial treatment of KD unless it is contraindicated. Patients who are at high risk for IVIG resistance are additionally treated with systemic glucocorticoids.
COVID and Kawasaki Disease Association?
Is there a clear association? If so or if not, what does this mean?
In April, there was an observation and possible association, published by a small Italian town, suggesting that there was a “30 fold increase” of KD-like signs and symptoms in children. This got unrelentless widespread attention throughout the world from our news media outlets.
https://www.ncbi.nlm.nih.gov/pubmed/32410760
That 30 fold increase was reported in 10 children there….7 boys/3 girls. Out of the 10 kids, 5 had “classic criteria” and the other 5 kids, the harder to diagnose and confirm, atypical criteria. Since then, another 9 kids had this KD like syndrome – something they are currently provisionally coining as “Pediatric Inflammatory Multisystem Syndrome (PIMS)”. Currently, there are a couple studies being set up to look at this phenomenon so we can better understand how Covid can cause this hyper immune response which can lead to information to create therapeutics, etc. Please note this important point as well – there apparently were no fatalities among these kids and no long-term coronary aneurysms. The article suggested, that along with the traditional KD treatment and other medications, all the children recovered!
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7220168/
As of this morning, before I had a chance to send this blog, I have learned that there are likely 82 kids with NYC thought to have this phenomenon. That just confirms the possible association between a virus and KD/PIMS. Nothing changes in principle when you add the total number of cases with this treatable condition.
What inspired me to review and write this was the fear this created within my patient population and the perspective they were seeking with this information.
The take home points here are the following — because Kawasaki is such a rare disease, when we have a small cluster, in this case (10 kids presenting with this where you normally would not see anything), indeed, that is a 30 fold increase which sound astounding on television! But if we put perspective to this information, out of the millions of kids who are likely infected, the numbers for KD, Atypical KD, or PIMS is still very uncommon/rare complication. This information indeed supports that KD may have a viral etiology and may even help with understanding our immune system better and lead to better therapeutic treatments in the future as the Lancet article suggests. To all the families reading this, don’t forget, that the ultimate outcome for all these kids have been good, which ultimately is the most important information one can read. Please put things in perspective!!
A few more things to think about when it comes to Covid and children. By no means, is Covid safe for every child. There are many hospitalized children and some may have long term lung sequela. Approximately 6% of children that are hospitalized are in critical condition based on a study published in Pediatrics.
Pediatrics. March 16, 2020,
But as far as I could find, there were only 3 children that have been reported to have died thus far from this infection/secondary infection and or associated diseases (such as the one we are discussing in this blog). In addition, this year alone, a total of 144 deaths have been reported in children due to INFLUENZA infections. I calculated the average number of deaths from Influenza the past 10 years and found out that the average is 138 deaths per year. I mention all this because the fear associated with Covid and infections is greater than anything I have witnessed in my lifetime, but we forget that there are much more common diseases we face every year that has as astronomical high level of morbidity and mortality than this virus and how its effecting our children. Perspective allows us to make more rational decisions without allowing fear to control us. We can be rightly concerned, but fear is debilitation and can have a detrimental effect on our health.
https://publications.aap.org/aapnews/news/8095?autologincheck=redirected
Parting Thoughts and Future Investigations
As I was thinking about writing this blog or whatever you want to call it, it occurred to me that I have read some articles on blood clots and pulmonary embolisms as one of the many theories and possible causes of death in many people. Treatment of many individuals remain unproven with mix results on benefits.
https://www.medicalnewstoday.com/articles/are-blood-clots-to-blame-for-covid-19-deaths
https://www.statnews.com/2020/04/16/blood-clots-coronavirus-tpa/
But I have also heard that aspirin therapy can also help many people especially in the early stages of the illness, perhaps in before the actual clots occur. Doing a quick search I am happy to say that there is currently a trial going on this.
https://clinicaltrials.gov/ct2/show/NCT04365309
Both Kawasaki Disorder in children and blood clot formation in adults have a massive inflammatory response and both may need aspirin to prevent complications. Can these two phenomenon be related by some shared mechanism? I find this an interesting question to pose and I will follow up and ask some researchers soon.
Final Words
1. Please put everything you read or hear in PERSPECTIVE. Yes, KD is real but it is super RARE and all the children have recovered.
2. Chronic FEAR is terrible for the immune system and our mental state. If you look for the top causes of death in adolescent and young adults, it is mental illness by a landslide. Suicide and Homicide rank in the top 4 causes of death so PLEASE don’t ignore the importance this issue. LEARN to deal with age appropriate stress management techniques. I have listed a few things on Instagram, but will add suggestion of books, apps, and great authors soon. I not only suggest traditional therapist to my patients, but alternative therapists such as Chiropractors, Muscle Workers, Hypnotherapist, Reiki therapist, Chanting, Mindfulness, Yoga, NLP, etc. Please SHARE the amazing resources you know with your friends and colleagues so we can adequately deal with this important issue TOGETHER.
3. COVID has a very LOW MORTALITY in children, much lower than influenza and its complications (as shown above). When we have a new disease, that “uncertainty” creates massive anxiety. Now that time has passed, we are more aware of how this virus generally acts and who it targets so we must ADJUST that anxiety (flight or fight sympathetic response).
4. While the news focuses 99% of the time on the negative, lets not forget the positive. I will mention one here that is so important that it may in fact save HUMANITY one day!! I don’t think thee will be anyone here that will dispute that our hospitals, doctors offices, supply centers and our mayors/governors and government is not much more prepared than ever before when it comes to a pandemic. Yes, they are still learning. Yes, we are still in a bit of a chaos. But, and this is a big but, when this is over, and we will survive this, never again will our system be so ill prepared!! If this was Ebola, the majority of the health care workers would have died first since we did not have any protective gear. Good luck finding anyone to run any ventilators!! At this point, likely billions of people would have succumbed to a virus with an over 50% mortality rate. Yes, I am speculating, but look at our world wide response. This will forever, change how we cooperate and coordinate and will set up centers and procedures that may save us all from a very deadly virus. There is value in seeing both sides and discussing it with your friends and families everyday. This was only one example. I’m sure you can find 100 others!
5. We are in a “NEW NORMAL” – I hope and pray that people wake up and look at life differently. In traditional medicine, we are simply popping a pill to suppress a symptom, but hardly ever dealing with the underlying condition. Whether you want to survive Covid, or any other pandemic, we much focus on HEALTH and VITALITY – not just surviving disease. Even though you read that a healthy person died here and there, the majority of people dying have different chronic morbidities. So use your NEW NORMAL to challenge the way you think about health and demand more from yourself and your doctor. Start thinking outside the box and find the RIGHT COACHES to really help you live your life fully – BALANCED (mind-body-spirit)!!
6. Lots of people will lose their LIVELIHOODS from this pandemic and become desperate. Lets look out for eachother , by finding way to see how we can give. I tell my patients, especially if they have kids over 5, to find projects to help people. It has a very strong impression on kids growing up being INVOLVED with their parents in helping people. These types of experiences are imprinted on them many times and are deep QUALITY TIME. There will be many opportunities to help available in the coming months and probably years. Pull up your sleeves and TEACH your kids how to help and solve problems! I promise you, you will not forget it and you will make cherished memories.
I hope you have learned a little about KD, Atypical KD and PIMS. More importantly, I hope you embrace perspective, love, growth and contribution. WE WILL SURVIVE THIS so lets EMBRACE this NEW NORMAL and work TOGETHER to make this world a more BEAUTIFUL PLACE for EVERYONE!
LOVE,
Dr. Ed