A guide for what doctors and parents can do as Kawasaki disease kids grow up

A medical school lecture taught Dr. Samuel Kung a vital lesson: He needed to see a cardiologist.

蹒跚学步时, Kung had Kawasaki disease, an illness of unknown cause that tends to strike young children. 他不记得自己生病了，只记得一直延续到他十几岁的那些年.

他说，从他的儿科医生到成人专家的交接实际上是不存在的. “重点从来没有放在跟进某人对我来说有多重要."

That could have had life-threatening consequences for Kung, 他现在是拉霍亚的斯克里普斯诊所和斯克里普斯绿色医院的内科住院医师, 加州. 他也是美国心脏协会一份新的科学声明的合著者，该声明希望保护其他川崎病幸存者.

The statement, published Monday in the Journal of the American Heart Association, spells out steps to help patients, 他们的父母和儿科护理人员准备过渡到成人护理. 它还试图让成年心脏病专家更加了解有川崎病病史的人需要的专门关注.

“我们写这篇论文的真正目的是在所有各方之间架起桥梁”, 合著者博士说. 阿德里亚娜Tremoulet, 他是加州大学圣地亚哥分校川崎病研究中心的儿科教授和副主任. She called it a disservice to give people excellent care for Kawasaki disease, 然后把他们送到这个世界，却不知道他们可能患有危及生命的疾病."

Kawasaki disease is most common in Japan, where it strikes more than 1 in 100 children by their 10th birthday. 美国的利率.S. 大概是十分之一吧. 仍然, 这是儿童心脏病最常见的原因，不是先天性心脏缺陷, according to the statement.

症状包括发烧, 皮疹, swelling of the hands and feet, redness of the whites of the eyes, swelling of the neck glands and redness of the tongue, 嘴唇和喉咙.

It's rarely fatal in the short term. 但这种疾病会导致向心脏供血的动脉出现凸起或动脉瘤. 这些动脉瘤通常会消失，但患者面临复发的长期风险. 因此，血栓会形成并阻断流向心脏肌肉的血液，导致心脏病发作.

这份新报告关注的是患有此类动脉瘤的年轻人从儿科到成人护理的转变.

在儿童, Kawasaki disease care is generally overseen by a team, which can include a general pediatrician, a pediatric cardiologist and a pediatric infectious disease specialist, Tremoulet说. But the transition to adult care tends to be tripped up by two main issues.

The first is the patients' age. Kawasaki disease usually strikes patients who are younger than 5, so they don't remember much, 如果有什么区别的话, 关于生病.

之后, 这些孩子, who are likely to have little understanding of their illness, 成年后，他们会发现“一旦他们长大，没有一个真正合适的系统来照顾他们.“甚至找到一位了解川崎病的心脏病专家都是一项挑战.

The disease is expected to affect 1 in 1,600 people in the U.S. by 2030. To help those patients make the transition to adult care, 该声明呼吁采取“深思熟虑和协调一致”的一系列步骤，包括从12岁左右开始对这种疾病进行普通教育，并向青少年传授自我管理技能.

"And we're really aiming also to engage adult cardiologists," Tremoulet说. 她说，儿科心脏病专家和传染病医生都知道川崎病. “他们很关心它. 他们能理解." Adult cardiologists need to become "educated, 感兴趣, 订婚了, willing to take on these patients as their own patients, and then be able to provide the best care possible."

父母可以提供帮助, 她说, 通过与孩子们谈论他们的疾病，而不是成为“信息的保持者”, but the ones that pass it on."

Kung said patients need to be active, too. 随着年龄的增长, they need guidance with practical skills, 比如如何重新配药，以及如果他们遇到胸部症状该怎么做.

为他, he knew he had coronary aneurysms, 而是一个典型的大学生:“在那个年龄，我们有点觉得自己是不可战胜的." It took the medical school lecture about Kawasaki disease, followed by a conversation with the speaker, to make him realize he needed to see a cardiologist.

检查显示，他的动脉瘤已经长到一定大小，他需要开始服用血液稀释药物，以降低形成危险血栓的风险. “说实话, 很有可能我的儿科心脏病医生之前就打算让我服用药物,他说. But if that happened, the message didn't sink in.

他鼓励其他病人确保他们了解自己的病情, if they don't already have a cardiologist, 尽可能多地收集他们的病史，并与了解川崎病的人联系.

As he wrote in the statement, no patient should fall through the cracks. His outcome, he said, could have been disastrous. “我真的很幸运."

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