What is the appropriate treatment for restrictive lung disease?
One of the aspects of restrictive lung disease that I encourage patients to really think about and discuss with their physicians is whether the surgical intervention that are being proposed will actually either maintain their level of lung function or improve it because unfortunately, I think it's because there are so many different surgeries that are offered to patients with Marfan syndrome and they have to be responsible for understanding the literature and the history, the expectations for every one of these surgical interventions.
肺が狭められることで発症した疾患に関し、よく考えて主治医と相談するようにお勧めしていることは、提案された手術によって本当に肺機能が維持されたり、回復するのかを確認するということです。理由は、マルファン患者さんに提案される手術は多種多様であり、文献や既往、手術の予後などを理解することが求められるからです。
And for interventions in the chest, there's a component of what we call cosmesis; that's appearance and there's also a component of what we call function and those can be very much separated and divided.
胸部への介入については、外見に関わる美容という側面があり、その他に機能という側面もあります。これらは大きくかけ離れたものです。
For example, pectus excavatum is a very common, just deformity in Marfan syndrome and it's typically kind of manifest and many people have it who don't have Marfan syndrome; it's the most common chest wall disorder in the general population and what it is is a depression of the sternum or the central part of your chest.
例えば、漏斗胸はマルファン症候群では非常に一般的な胸部の奇形で目立つものですが、多くの健常者にも見られます。一般人に最も多い胸壁異常であり、胸の中央が陥没する所見です。
And for some people and for some kids especially who have this, it's a major source of discomfort, especially around other kids who may see it when they're in gym class, when they go swimming and what I encourage parents to do is to take that discomfort seriously and if there's an intervention plan, simply because you have a child who feels uncomfortable and finds this kind of a socially kind of disturbing situation, that's a reason to intervene.Unfortunately, that rationale for a surgical procedure gets very much tied into, "Will this improve my child's lung function?"
この所見をもつ成人の一部や特に一部の子供では、精神的苦痛の大きな理由となっています。とりわけ、水泳の授業などで感じるようです。私が親御さんに推奨しているのは、この苦痛を真剣に受け止めることです。お子さんが苦痛を感じていたり、こういった社会的状況を不安に思っている、という理由で介入（手術）を考えるであれば、正当な理由です。ですが、残念なことにそういった理由で手術に踏み切る場合、「手術で子供の肺機能が回復するのか？」という問題も避けては通れません。
And very often, a pectus will cause just a modest, a very small reduction in lung function but nothing that would cause any symptomatic problem or major limitation on exercise capacity.
非常に多くのケースで、漏斗胸が肺機能低下に及ぼす影響はごくわずかであり、自覚症状が出たり、運動能力が大きく制限されるということはないのです。
And so what I like to do is really clarify the rationale factually having a procedure done because unfortunately there's not any surgical procedure that doesn't have a potential complication and therefore parents really have to know why they're having the procedure done and the pectus is a classic example because I hear all the time from parents who say, you know, "Will it improve my child's lung function?" I've been told that he has reduced lung function or she has reduced lung capacity and I try to clarify the issue, knowing the intervention will not improve the lung function but if there's a cosmetic reason to have it done, then that's compelling.
私が望んでいるのは、現状に照らして手術をする場合の根拠を明確にするということです。なぜなら、合併症のリスクのない手術というものは存在しないからです。ですから、親御さんは手術の根拠について知っておく必要があります。漏斗胸はこういったケースでの典型例なのですが、それは私はいつも親御さんから「手術で子供の肺機能は回復するんですか？子供の肺機能や肺容量が低下しているそうですが…」という質問を受けるからです。私は手術では肺機能が回復しないということが分かっているので、手術を行う理由をはっきりさせようとします。外見上の問題だというのなら、やむを得ないと思います。
And I mean there I had an encounter a couple of years ago at one of the Marfan meetings where one of the parents was concerned about having the surgery and her son had a fairly deep pectus and brought her son into the room and we were talking about the pros and cons and I asked the son, "Are you concerned about having this pectus?" and he said, "Oh no! I think it's really cool because I can put coke cans and I could put lots of things in there when I hang out with my friends," and I asked him "Well, you know, do you feel odd because your chest is different from your friends?" and he said, "Well no, why would I want to look like everybody anyway!" and so I thought, "My gosh!" They have this exceptional child and, you know, it's wonderful. That's exactly what you want your child to say.
数年前にマルファンの会合で、かなりの深さのある漏斗胸の息子さんの手術について不安を抱えているお母さんがいました。部屋で（手術の）メリット・デメリットについてお話し、私が息子さんに「漏斗胸だと不安？」と訊くと、「全然。カッコいいと思うよ。だってコーラの缶が入るし、友達と一緒の時には色々なものを入れれるから」と彼は答えました。「友達と違う胸で変な感じしない？」「しないよ。なんでみんなと一緒じゃなきゃダメなの？」私はビックリ仰天しました。こんな子供がいるなんて。素晴らしいじゃないですか。それこそ、まさに子供に言ってもらいたい言葉です。
And because he obviously had an incredibly healthy self-concept, it didn't bother him and and so the major rationale for doing the surgery just wasn't there and I think it was very helpful for them to hear him say that in a very neutral environment and I like to encourage parents to actually have that conversation with their children.
彼は並外れて健全な自己認識を持っていたので、漏斗胸に動じることもありませんでしたし、手術の主な理由も他のところにありました。非常に公平な場で息子さんの考えを聞けたということは、ご家族にとって大きな助けとなったのではないかと思いますし、親御さんはお子さんとああいった会話をしてもらいたいと思っています。
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundationは、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.orgにアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
