2024 Kawasaki Disease Parent Symposium Q&A Questions (Transcribed)
Q: How can families participate in the KD genetic studies? Who qualifies to enroll and how?
A: Currently, DNA studies are focused on patients with aneurysms. Families with a child who has aneurysms and wish to participate can request a mouthwash kit for at-home testing; a swab kit is available for infants. Contact KDGenetics@UCSD.edu to discuss obtaining a kit.
Q: Why is KD less commonly treated with steroids?
A: While it’s not common for Rady Children’s Hospital to treat KD with steroids, the use of steroids in combination with IVIG continues to be used around the world for children with aneurysms and with treatment-resistant KD. In San Diego, many sites have been using infliximab instead of steroids because of the biological data supporting the use of infliximab and what we know about the pathophysiology of KD; however, there has not been a head-to-head trial done to compare infliximab or steroids when combined with IVIG.
Q: Does being diagnosed with KD predispose you to any other disease? Is it an autoimmune disease? Does it allow you to develop any hyperautoimmune diseases?
A: KD is not an autoimmune disease, and there’s no evidence at this point in time that KD predisposes to autoimmunity later in life. An ongoing adult KD study with questionnaires is being conducted to study long-term effects; patients aged 15 or older can participate.
Q: Why does KD cause a high heart rate?
A: High heart rate is part of the acute KD presentation, and is one symptom looked at when KD is being considered. It happens because the heart muscle itself is inflamed in Kawasaki disease patients, so the heart is not working as efficiently, which causes the heart rate to be higher.
Q: At what age does exercise testing start? Why aren’t stress tests performed until age 12?
A: Every center conducts exercise tests differently. At Rady Children’s Hospital, stress testing is done using the protocol of getting on a stationary bike and riding it: patients get on a small stationary bike, pedal hard until their heart rate is around 200 bpm, and immediately jump off the bike and do an ECG. Many other centers use chemical stress to test younger children who aren’t mature or old enough to cooperate with this form of exercise testing, but Rady Children’s Hospital waits until the child is old enough, as chemical stress testing is a much more complicated procedure.
Q: Who should get exercise stress testing done?
A: Children who have had coronary artery abnormalities (2017 AHA statement).
Q: Should a child who will participate in contact sports get a stress test done?
A: Stress testing for KD is based on the coronary artery abnormalities. If there are no abnormalities, stress tests do not need to be done.
Q: Is there any increased prevalence in allergies after Kawasaki disease?
A: There is a difference of opinion in the literature. Some studies from Taiwan have associated allergies and Kawasaki disease being genetically linked; other studies from Japan and other places have not found that association. Whether they’re linked or not, it’s important to pursue exercise for a heart-healthy lifestyle and get a good allergist for medications and treatment.
Q: Is there any role for anti-inflammatory therapy (such as infliximab) a few years after KD diagnosis?
A: Generally, anti-inflammatory therapies are useful during the acute period of Kawasaki disease and sometimes a week or two later. After a certain point, treatment becomes long-term, and medications like direct oral anticoagulants and aspirin are used instead to make sure that no blood clot forms. The long-term use or late use of anti-inflammatory therapies in KD is not standard practice as of now; it is more used when the child presents initially.
Q: What is the likelihood of aneurysms happening later? If your child had a normal baseline echo, what would happen at the two-week or the two-month follow-up?
A: The majority of abnormalities of the coronary arteries (over 80%) present in the first week to 10 days. The recommendation is to have a follow-up one to two weeks after discharge from the hospital, which is especially important for our younger patients, to confirm no development of aneurysms. If a child has normal echoes in the hospital and then at that two-week follow-up, it is unlikely that anything will develop beyond that, especially two months or a year later.
Q: What is genetic counseling, and why is it important?
A: We educate families that KD is a genetically linked disorder from the very start of their first visit. This is also when we approach families about collecting DNA as part of the studies. We go more in-depth about genetics when patients graduate from our clinic, which tends to be between the ages of 15 to 18.
Q: If I had KD, how likely is it that my children would develop KD? What’s the risk of transmitting KD genetically to offspring?
A: Currently, the exact answer is unknown, but the hope is to conduct studies to calculate the heritable risk; this is one of the research questions of the long-term adult KD study. The likelihood is neither 0% nor 100%; it’s likely low, but there have certainly been parent-child pairs seen.
Q: Can you return to school after being diagnosed with KD?
A: Yes. KD is not contagious, and IVIG has antibodies that help fight inflammation in both KD and even some regularly circulating infections.
Q: Does IVIG help or protect patients, or does it lead to immune suppression?
A: Neither KD nor IVIG are immunosuppressive. In fact, families have reported that children get ill less frequently because of the protection from IVIG.
Q: Is there any link between KD and high cholesterol? Should that impact how parents see either what the kids can eat or physical activity limitations?
A: We can think about heart health in two ways: First, the child had Kawasaki disease, which can’t be undone, whether there are some microscopic changes in the heart or in the blood vessels. Second, there’s a kind of heart disease called atherosclerosis, which has specific risk factors and steps can be taken to mitigate them. Hypertension, obesity, exercise, and diet are all contributors to this adult-type heart disease. How people handle the fats in their diet is related to genetics, and at the one-year visit, cholesterol screening is completed to identify patient baseline and identify children who have inherited predispositions to high LDL (“bad cholesterol”). Parents largely control what patients eat and should shift towards healthier foods if there is a predisposition. The lipid panel also tests for a molecule called Lipoprotein(a) or Lp(a), another potent genetic risk factor which is inherited and can predispose for atherosclerotic adult-type heart disease and can also take steps to mitigate. Lipid panel results are discussed with the parents of KD patients.
Q: Is KD associated with postural orthostatic tachycardia syndrome (POTS)?
A: POTS is related to the neurologic part of our bodies called the autonomic nervous system, not primarily a cardiac problem. While POTS does seem to be a problem affecting many young people, particularly teenagers, there’s no evidence right now that POTS is directly or even indirectly linked to KD.
Q: What have we learned from the KD study about which diseases are associated with Kawasaki disease?
A: A preliminary look at the data published last year raised questions about an association between KD and migraine headaches; further analysis is being done.
Q: Does low-dose aspirin pose a risk for Reye’s syndrome?
A: There is no danger with low-dose aspirin and Reye’s syndrome. Reye’s syndrome is associated with influenza and varicella viruses, and higher aspirin doses used to control fever, not the low-dose aspirin that KD children are sent home on.
Q: Should you get the flu vaccine prior to discharge from the hospital?
A: Yes. Patients should get the flu vaccine for protection, especially while recovering from KD. There is no concern about the interaction between low-dose aspirin and flu vaccines.
Q: Are there any ideas about antigens, either environmental or infectious, based on the wind studies that have been done in terms of triggers for KD?
A: Research into antigens triggering Kawasaki Disease (KD) is ongoing. Studies suggest that increased wind speed might correlate with higher KD incidence, potentially linked to air pollution, such as urban or industrial pollution associated with industrial production and continental Asia and China, increasing over many decades. Researchers are exploring this connection through long-distance wind modeling and local measurements of heavy metals, but multiple hypotheses are still being investigated.
Q: Is there the implication that the wind velocity is associated with higher incidence of KD in the Asia Pacific region or the Americas and Europe?
A: Researchers aim to investigate if there are similar trends in the Asia Pacific region in South Korea, given its high KD incidence, but it’s too early to confirm the same trend in the Americas or Europe. Insights from Japan may help uncover regional differences. Climatology suggests that wind speeds may correspond to the amount of aerosol carried and the exposure of people breathing on the ground, which could be linked to KD.
Q: When are statins, particularly atorvastatin, used in children with KD who develop aneurysms?
A: Studies show that atorvastatin is safe for children aged two and older; it is sometimes prescribed longer-term for those with giant aneurysms to promote healing of the blood vessel wall lining. Statins are used not for cholesterol reduction but for vascular healing. Parents of children with persistent aneurysms are encouraged to discuss statin use with their cardiologists. While statin use is relatively new, there are ongoing research and clinical trials being done with younger children.
Q: Is there any reason not to put your kid on statins?
A: The current recommendation for statin use in children with Kawasaki Disease (KD) is specific to those with aneurysms, particularly giant aneurysms, as statins help heal injured vessel walls. Statins are not prescribed for children with normal coronary arteries or those with transient swelling that resolves. The use of statins in KD is not for cholesterol reduction but for vascular healing.
Q: What is a calcium score, and why is it important for KD patients? How often should it be done?
A: A calcium score is a great screening test for young adults unaware of heart complications after KD. It is highly recommended for all patients uncertain about their history of KD, to be aware of important coronary artery changes. Calcium scores are screening tests that use a CT scanner to look for calcium deposited in the wall of the coronary artery in the form of a scar. The calcium score is only positive in someone who had aneurysms; if it is above zero, the patient should discuss with a cardiologist and understand why it’s not zero. The test does not require an IV or any dye injection and is easy to get ordered by your patient care provider. It only needs to be done once, and should be at least 10 years after having KD; it is most applicable for people now in their 20s and 30s or older individuals.
Q: What’s typical for long-term follow-up?
A: Long-term follow-up depends on the community that you live in and the resources that are available. The Rady Children’s Hospital follow-up is as follows.
For children who have never had an abnormal echo: echo in the hospital, echo at two-week visit, lipid panel after a year, heart-healthy discussion after five years. At the one-year visit a lipid panel is taken for baseline study, as the patient is now fully recovered from inflammation, and results are discussed with parents. Heart-healthy counseling is conducted to educate families about heart-healthy lifestyles, modifiable risk factors, and KD’s genetic nature. At the five-year visit, there is a check-in for exercise and other aspects of a heart-healthy lifestyle. When patients are teenagers (age 15+ and before graduating from HS), the cardiologist will fill out a sheet together to help understand the patient’s medical history and KD experience: echo results, lipid panel results, blood pressure, percentile of height and weight, and any other relevant findings. Patients are advised to photograph the medical history sheet for reference in the future.
For children with aneurysms, Dr. Tremoulet created a specific program of transition of care and is happy to share with other centers around the country. The program is an online questionnaire to test patients starting at about age 12 if they have even remodeled aneurysms, and make sure they understand that the importance of follow-up and medications and how to refill a prescription. Patients are trained to be their own health advocates and maintain their health throughout their teenage years. Patients are also given genetic counseling to understand that KD is a genetic condition.
Q: What types of jobs are patients with aneurysms eligible for? What fields can they work in?
A: Patients with aneurysms should consider job types carefully, especially if they are on blood thinners, as jobs with an increased risk of injury and bleeding are not ideal. Roles that involve chronic stress might also not be suitable, as stress can add to the strain already on the heart with giant aneurysms. Desk jobs are a practical option, but incorporating healthy habits into lifestyle, such as using a standing desk, taking frequent breaks to move, and biking to work, can help maintain heart health. These considerations apply broadly to maintaining cardiovascular health, regardless of having Kawasaki Disease (KD).