Rare voices · Letter 12 of 12

A Letter to Amy from Representatives of Peutz-Jeghers Syndrome (PJS) Patients

A Letter to Amy from Representatives of Peutz-Jeghers Syndrome (PJS) Patients

English translation

Dear Amy,

I don't know what day at sea this is for you.

Right now, is it the sunshine, the starry sky, or the pitch-black wind and waves that keep you company? What difficulties have you encountered these past few days? When the waves beat against the boat again and again, when weariness and loneliness set in, we hope this letter of ours can cross five thousand kilometers of sea and bring you a little strength, a little warmth.

We are a group of patients with Peutz-Jeghers syndrome (PJS). This is a hereditary, tumor-related rare disease. From childhood, the dark spots on our lips have been like a mark we can't shake off; in many places, it is misunderstood as a symbol of “bad luck.”

As we grow up, we have to face the continuously growing polyps in our small intestine, face the risk of intestinal obstruction and intussusception, and face one abdominal surgery after another. Even heavier is our far higher-than-average risk of cancer. Our long lives seem to be filled with endless examinations, re-examinations, and surgeries. What many people fear most is not the pain before their eyes, but not knowing on which day the diagnosis that reads “cancer” will arrive—the suffocating feeling of that moment is enough to completely engulf a person.

We have no specific drug, nor an anti-cancer vaccine that could eliminate fear with a single shot. Even if we remove the polyps today, in the future they may still grow back. Repeated abdominal surgeries are like installing a zipper on our bellies, the intestines cut segment after segment, and body and heart become extremely fragile amid this repeated wearing-down.

But we have never stopped declaring war on the disease.

Over more than twenty years, through the joint efforts of patient friends and doctors, we have built a nationwide patient network, contacted experts in various places, and set up green channels for medical care. More and more patients can find, at the first opportunity, the doctor nearest to them who best understands PJS; more and more people have avoided unnecessary suffering; more and more cancers are detected at an early stage, winning precious treatment opportunities.

And behind all of this is a person we will never forget—Sister Meijuan. On June 15, 2026, she ended her own life due to depression.

She was an ordinary patient. She had undergone 1 abdominal surgery and 2 bouts of breast cancer; countless enteroscopies left her 170-centimeter frame weighing less than 90 jin (about 45 kg). Even so, over 19 years she devoted all her private time to QQ groups and WeChat groups, helping more than 2,000 patient families. She set up more than 80 groups for hospitals across the country, organized materials, and shared experiences, never taking any payment. She shared without reservation the expert resources and medical experience she had accumulated over many years, patiently listening to and comforting every patient friend in difficulty. She said: “With a rare disease, the more people who know about it, the better.”

Regrettably, in the end she did not get to travel, sing, and learn English after retirement, to realize those ordinary and beautiful dreams. She helped so many patients and families, yet she herself was crushed by depression. She once said with a smile: “In 19 years of being a rare-disease patient, I gained more than 2,000 good friends.” Her companionship to patients made us understand: when we bravely say “I am a rare-disease patient,” countless voices will respond, “So am I”; when we say aloud “I need help,” countless voices will say, “I need help too, and I am willing to help you.”

Amy, when you row with all your might in the vast sea, it's as if we see ourselves. You are contending with the wind and waves; we are contending with the disease. Before you is an ocean whose end cannot be seen; before us is a lifelong anti-cancer road whose finish line cannot be seen. But precisely because of this, we understand all the more the meaning of perseverance.

If there is ever a moment when you feel weary and want to give up, please remember: on the other side of the earth, more than two thousand PJS patients and families are cheering you on. We believe that every stroke you row forward will let more people hear the voice of rare disease; every wave you cross will bring hope to more patients.

Amy, please row on our behalf the far journey that Sister Meijuan was unable to complete, toward the other end of the Atlantic; perhaps this is Heaven's arrangement—that her unfulfilled wish continues forward by way of your oars. And please tell the world for us: the rare is not alone. Breaking the waves with our oars, we move toward the light.

May you safely pass through the storm and reach the far shore.

From the China PJS (Peutz-Jeghers Syndrome) Care Center

With respect

— — —— — —

Appendix: The only self-introduction Sister Meijuan left behind

“You need to get to the hospital quickly.” After I sent the message, I froze at the sight of the red exclamation mark on the screen. My thoughts were instantly pulled back to 22 years ago. Looking back at the surgical report from that time, my heart still races—the intussusception was as long as 30 centimeters; fortunately the bowel had not become necrotic. If I hadn't had surgery in time back then, I might not have had the chance to share my story here. If a colleague had not mentioned the detail that “dark spots are related to abdominal pain,” I might never have known that the lip spots I'd had since childhood were, as cause and effect, linked to the hundreds of polyps quietly growing in my digestive tract.

That was 2002; I was 24, and the abdominal pain began. A colleague at work told me that stomach pain might be related to polyps in the belly, and also connected to the dark spots on my mouth. As a child, I had noticed dark spots on my lips and oral mucosa, but my belly had never hurt, so I had never thought there could be any connection between the two. At the time, I didn't take my colleague's words to heart; after all, the abdominal pain passed after a while, and with the New Year approaching, I didn't pay much attention.

After the New Year, the abdominal pain became more and more frequent, and I suddenly remembered my colleague's words, so I tried searching online with the keywords “dark spots, polyps,” and was startled to realize that I might have a rare disease called Peutz-Jeghers syndrome.

This disease is mainly characterized by mucocutaneous pigmentation and multiple gastrointestinal polyps. Its prevalence is extremely low, only 1/200,000 to 1/8,000. PJS has onset from childhood; as the patient ages, gastrointestinal polyps gradually increase in number and size, which can cause various complications such as intussusception, intestinal obstruction, gastrointestinal bleeding, canceration, malnutrition, and developmental delay in children. In addition, patients have a higher risk of gastrointestinal canceration, and may also develop tumors such as pancreatic cancer, lung cancer, and gastric-type adenocarcinoma of the cervix. For a long time, among adolescent and young-adult PJS patients, most, because of repeated complications such as intussusception and intestinal obstruction, have been forced to undergo surgical treatment many times.

At that time, my abdominal pain was becoming more and more frequent—from once every month or two at first, to daily abdominal pain later—and I had to go to the hospital. Removing the multiple polyps in the digestive tract and preventing and treating the various complications they cause is the most important clinical treatment goal for this disease. Polyps in the stomach, proximal duodenum, and colorectum of PJS patients can be removed by gastroscopy and colonoscopy. In the outpatient department, I had polyps removed under colonoscopy and gastroscopy, but after staying in the hospital for over a month, my belly still hurt. I guessed that this must be caused by polyps in my small intestine.

Because PJS polyps can be distributed throughout the entire gastrointestinal tract, with the common sites, in order, being the small intestine, colon, and stomach, and most commonly the proximal small intestine such as the duodenum and proximal jejunum. Another study has reported that about half of patients show distribution throughout the entire gastrointestinal tract (with polyps in the stomach, small intestine, and colorectum), with a relatively large number of polyps, the small intestine being the site where PJS polyps most frequently occur. The polyps are mainly pedunculated, somewhat like grapes, with a small portion being sessile or broad-based polyps.

When searching for the disease online, I found only one post left by a patient friend seeking a consultation, in which he had left his mobile phone number. With trepidation, I dialed this phone number thousands of miles away. The patient friend told me that small-intestine polyp removal needs to be done by opening the abdomen while using an enteroscope in coordination. Simply put, after opening the abdomen, a small incision is made in the intestine, the enteroscope goes in and searches up and down the entire small intestine, and the polyps are all removed in one go. But the vast majority of regional hospitals do not have this technology, and even now there are still many hospitals that do not have this technique.

At that time I was still young, working alone away from home, with a meager income, and I didn't want to burden my family, so I chose to take antispasmodic drugs prescribed by the local hospital. At first they were effective—after taking them my belly stopped hurting—but after a while they lost their effect. Then I went to the local hospital to get Chinese medicine to drink; this Chinese medicine could relieve my abdominal pain symptoms—as long as I drank it my belly wouldn't hurt, and on any day I didn't drink it, it would hurt. I kept this up for over a year, and I knew I couldn't keep dragging it out like this; I had to deal with the polyps in my small intestine.

The patient friend told me that Shanghai Changhai Hospital could do it, so on March 15, 2005, I boarded the bus to Shanghai. At night I lay on a small bed in the living room of a guesthouse next to the hospital, tightly clutching the twenty thousand yuan I had borrowed, and didn't sleep all night for fear of it being stolen.

This time, at Shanghai Changhai Hospital, I received all-round treatment from Director Meng Ronggui of the anorectal department. Using a method combining enteroscopy and open abdominal surgery, Director Meng dealt with all the polyps hidden in my small intestine. This surgery not only solved my immediate suffering; more importantly, it spared me the risk of intestinal obstruction and intussusception that I might face in the future as the polyps grew, and the pain of the repeated abdominal surgeries that would come with them.

I remember that on the day I was discharged, the sunshine was so warm and the air so sweet—life turned out to be so wonderful!

While hospitalized at the local hospital, I also learned an exciting piece of news: a balloon-assisted enteroscope (enteroscope for short) that can fully examine the entire small intestine had come into being. Upon learning this news, I immediately searched online and found that this enteroscope could not only examine but also had the function of removing polyps. The enteroscope can enter the intestine through the anus or the mouth; once inside, it can clearly reveal the shape, size, number, and distribution of the polyps, and after finding them the doctor can loop a snare around the neck of the polyp and remove it using pure electrocoagulation or a mixed coagulation-cutting mode. If it could really succeed, then we would never again have to open the abdomen once every two or three years!

With an excited heart, I shared this discovery with my patient friend. He began to inquire everywhere about which hospitals had this equipment, contacting top hospitals and doctors across the country in hopes of finding a medical institution that could provide this advanced treatment. However, we found that at the time almost all hospitals used the enteroscope only for diagnosis, and most hospitals had reservations about—or were even unwilling to attempt—using it for polyp-removal treatment.

In early 2005, enteroscope technology reached an important turning point. This patient friend got in touch with Director Mao Gaoping of the gastroenterology department of the former Beijing Air Force General Hospital (“Kongzong” for short, now the Beijing Air Force Medical Center). After searching far and wide among relevant manufacturers, Director Mao specially custom-made a thin and long snare to suit the special needs of polyp-removal surgery under the enteroscope. In February 2005, Director Mao and his team successfully completed the first enteroscopic polyp-removal surgery for PJS, thus opening a new chapter of enteroscopic polyp removal in China.

The baton was passed from Director Mao to his student Director Ning Shoubin (now the director of the gastroenterology department at Kongzong). Under Director Ning's leadership, the department not only inherited the unique advantages of enteroscope technology, but also kept overcoming difficulties in practice and further refined this technique. Over 20 years, Kongzong has received more than 760 PJS patients, becoming the world's largest center for the enteroscopic diagnosis and treatment of PJS. During the busiest periods, the hospital even had 25 patients undergoing enteroscopic treatment in a single day.

Director Mao Gaoping and Director Ning Shoubin

Just when everything was going smoothly with the enteroscope, and we thought we would be worry-free as long as we removed the polyps regularly, a new challenge appeared: clinical data in recent years show that the incidence of gastric-type adenocarcinoma of the cervix in female patients is dozens of times higher than in ordinary people. This lurking killer is often missed by routine screening. As our patient community kept expanding and exchanges increased, we found that many patient friends had developed gastric-type adenocarcinoma of the cervix, and almost all of them were already at the invasive stage when diagnosed. It was not until the end of 2021 that we realized the danger of this cancer to our community.

At the end of 2021, through Dr. Chen Yiwei of Wandou SIR, we got to know Director Kang Yu of the gynecologic oncology department of Shanghai Red House Hospital, and set up a dedicated gynecology group. Director Kang has provided many green channels for our community, and also established the PJS Shanghai Multidisciplinary Alliance. Over the past three years, we have kept recommending patient friends to see Director Kang; so far more than a hundred patient friends have undergone examinations, but there are still many patient friends who have not yet had a gynecological examination.

Many patient friends, after problems are detected, can undergo timely surgery for early intervention and treatment. Some patient friends found to have cysts, lobular hyperplasia, or atypical lobular hyperplasia of the cervix, having understood their own condition, can choose regular monitoring to prevent the condition from worsening. And several lucky patient friends were found to have gastric-type adenocarcinoma of the cervix while it was still at the carcinoma in situ stage and had timely surgery, thereby avoiding more serious consequences.

A colleague's kind reminder, the help of patient friends, and doctors' brave exploration were like a seed that sprouted in my heart; I wanted to pass on this spirit of mutual help.

On February 24, 2006, at the suggestion of a patient friend, Sister Li, we set up a QQ patient group. Over 19 years, the group has brought together at least 2,000 patient family lines. Whenever a new patient friend joins, I can't wait to tell them everything I know.

I am more anxious than they are themselves, because I know the consequences of not getting proper treatment—that is, repeated abdominal surgery or even malignant transformation. This sense of urgency caused me to lose my sense of proportion when popularizing knowledge of the disease, causing panic among patient friends. People said that the daily discussion of illness in the group was frightening to see. Many chose to mute the group's messages, and some even suspected I was a medical tout (“yituo”) with some ulterior motive.

In the past, I always worried that patient friends would take detours: worried they didn't understand the disease and would thus delay their condition, worried they didn't know the medical channels and would thus fail to get the best treatment, worried they didn't take it seriously, worried they would avoid it… Especially in actual work, whenever something that moved me happened—such as a patient friend's canceration or death—I would unconsciously cause panic among everyone.

The establishment of the patient group meant we no longer fought alone or were left isolated and helpless. At first, I did not understand why they had an avoidant attitude. As time passed and with reminders from professional doctors, I realized that a healthy and optimistic positive mindset is the foundation for overcoming disease. I wondered: how to find a balance between publicizing knowledge of the disease and easing patient friends' state of mind? I made some changes—the patient group no longer allowed only discussion of illness, but also allowed relaxed topics. We also set up a chit-chat group, so that everyone would have a channel to release pent-up emotions. When publicizing knowledge of the disease, I also paid attention to priorities and to methods and approaches.

Perhaps many people wonder: why do I have such a complex about helping others?

Because I myself am a beneficiary of patient friends helping others.

I think summarizing my own medical experience can also reflect the improvement and progress in the medical situation of the PJS community over more than twenty years—a good way to encourage and inspire us and all rare-disease patient communities. Every rare-disease patient has gone through the journey from one person to two, to ten, and now to finding hundreds and thousands of patient friends.

When I typed out the welcome message for the 2,000th patient friend joining the group, the plane trees outside the window were putting forth new buds. On that night 20 years ago, curled up on the small guesthouse bed counting my banknotes, I never imagined that the moonlight slipping through my fingers would turn into starlight illuminating the whole community.

In today's chat group, a newborn's first cry and the “no abnormalities” of polyp re-examination reports flash across the screen in turn. The green channels of enteroscopy at more than twenty hospitals are like crisscrossing blood vessels, delivering hope to every life in need of treatment; healthy embryos conceived through third-generation IVF technology are stretching their spot-free skin in warm amniotic fluid; a message pops up at two in the morning—“just finished the enteroscopy,” all went well; and in the gynecology group, another gastric-type adenocarcinoma of the cervix has been caught by early screening!

Dawn never descends suddenly. It is the all-night light of the operating room on a winter night, the PJS Shanghai Multidisciplinary Alliance spontaneously founded by 18 experts, the “Guide to Seeking Medical Care” updated in relay by more than 2,000 patients in the group. These 22 years have taught me the most precious truth: hope is the most potent painkiller. The dark spots will not be branded on our lips forever; when we join hands and grow into a forest, those scattered, star-like marks will at last connect across the earth into a galaxy leading toward the dawn.

中文原文

黑斑息肉综合征(PJS)患者代表写给Amy姐一封信

亲爱的Amy姐:

我不知道今天是你在海上的第几天了。

现在陪伴你的是阳光、星空,还是漆黑的风浪?这几天你遇到了什么困难?当海浪一次次拍打船身,当疲惫和孤独袭来时,希望我们的这封信,能穿过五千公里的海面,给你带去一点力量、一点温暖。

我们是一群黑斑息肉综合征(PJS)患者。这是一种遗传性肿瘤相关的罕见病。从小,我们嘴唇上的黑斑就像甩不掉的标记,在很多地方,它被误解为“倒霉”的象征。

长大后,我们要面对不断生长的小肠息肉,面对肠梗阻、肠套叠的风险,面对一次又一次的开腹手术。更沉重的,是我们远高于常人的癌症风险。漫长的人生仿佛被无尽的检查、复查和手术填满。很多人最害怕的不是眼前的疼痛,而是不知道哪一天,会等来那份写着“癌症”的诊断书——那一刻的窒息感,足以把人彻底吞没。

我们没有特效药,也没有一针就能消除恐惧的抗癌疫苗。即使今天切除了息肉,未来它们仍可能重新长出来。反复的开腹手术,像在我们的肚子上装了一根拉链,肠子被切了一段又一段,身体和心,在一次次消耗中变得无比脆弱。

但我们从未停止与疾病宣战。

二十多年来,在病友和医生们的共同努力下,我们建立起覆盖全国的病友网络,联系各地专家,搭建就医绿色通道。越来越多的患者能在第一时间找到离自己最近、最了解PJS的医生;越来越多的人避免了不必要的痛苦;越来越多的癌症在早期被发现,赢得了宝贵的治疗机会。

而这一切的背后,有一个我们永远不会忘记的人——美娟姐。2026年6月15日,她因抑郁症结束了自己的生命。

她是一位普通患者,经历过1次开腹手术、2次乳腺癌,无数次小肠镜让她170厘米的身高只剩下不到90斤。即便如此,19年来,她把所有私人时间都用在QQ群、微信群里,帮助了2000多个病友家庭。她为全国各地医院建了80多个群,整理资料,分享经验,从不收取任何报酬。她毫无保留地分享自己积累多年的专家资源和就医经验,耐心倾听、安慰每一个身处困境的病友。她说:“罕见病巴不得更多人知道才好。”

遗憾的是,她最终没能等到退休后去旅行、唱歌、学英语,实现那些平凡而美好的梦想。她帮助了这么多患者和家庭,自己却被抑郁症击垮。她曾笑着说:“成为罕见病患者19年,我多了2000多位好友。”她对患者的陪伴,让我们明白:当我们勇敢说出“我是罕见病患者”,会有无数个声音回应“我也是”;当我们大声说“我需要帮助”,会有无数个声音说“我也需要帮助,我也愿意帮你”。

Amy姐,当你在茫茫大海中奋力划桨时,我们仿佛看见了自己。你在与风浪较量,我们在与疾病较量;你面前是看不到尽头的大洋,我们面前是看不到终点的终身抗癌之路。可正因为如此,我们更懂得坚持的意义。

如果有某个时刻,你感到疲惫、想要放弃,请记得:在地球的另一端,有两千多个PJS患者和家庭正在为你加油。我们相信,每向前划出的一桨,都会让更多人听见罕见病的声音;每跨过一道风浪,都会为更多患者带去希望。

Amy姐,请替我们把美娟姐未能完成的远行,划向大西洋的另一端;或许这是上天的安排——让她未竟的心愿,借由你的船桨继续前行。也请替我们告诉世界:罕见,并不孤单。以浆破浪,向光而行。

愿你平安穿越风暴,顺利抵达彼岸。

来自中国PJS(黑斑息肉综合征)关爱中心

敬上

— — —— — —

附:美娟姐留下的唯一一篇自己介绍

“你要快点去医院”发出消息后,看到屏幕上的红色感叹号我怔住了。思绪瞬间被拉回到22年前,回看当时的手术报告,我仍心有余悸——肠套叠竟长达30厘米,幸好肠管没有坏死。如果当时没有及时手术,我可能就没有机会在这里分享自己的故事了。如果不是同事提及“黑斑与腹痛相关”的细节,我或许永远不会知道,自幼伴随的唇部黑斑,竟与消化道里悄然滋生的数百枚息肉互为因果。

那是2002年,我24岁,腹痛开始了。单位同事告诉我,肚子痛可能与肚子里的息肉有关,还跟我嘴上的黑斑有联系。小时候,我就发现嘴唇和口腔黏膜上有黑斑,但肚子从未痛过,所以我从未想过这两者之间会有任何关联。当时,我并未把同事的话放在心上,毕竟腹痛一会儿就过去了,又临近年关,便没太在意。

年后回来,腹痛愈发频繁,我忽地想起同事的话,便试着以“黑斑、息肉”为关键词在网络上搜索,结果惊觉自己可能罹患了一种名为黑斑息肉综合征的罕见病。

这种疾病以皮肤黏膜色素沉着、胃肠道多发息肉为主要特点。患病率极低,仅为1/200000~1/8000。PJS自幼发病,随着患者年龄增长,胃肠道息肉逐渐增多、增大从而可引起各种并发症,如肠套叠、肠梗阻、消化道出血、癌变、营养不良及儿童发育迟滞等。此外,患者发生胃肠道癌变的风险较高,也会伴发胰腺癌、肺癌、子宫颈胃型腺癌等肿瘤。长期以来,在青少年及年轻成人PJS人群中,多数患者由于反复发生肠套叠、肠梗阻等并发症而被迫多次接受外科手术治疗。

那时的我,腹痛已经越来越频繁,从起初的一两个月痛一次,到后来每日都腹痛,我不得不前往医院就诊。切除消化道多发息肉、防治由其引起的各种并发症是这个疾病临床最主要的治疗目标。PJS患者胃、十二指肠近端及结直肠息肉可通过胃镜和结肠镜进行切除。在门诊我做了结肠镜和胃镜下切息肉,可在医院住了一个多月后,肚子还是痛。我猜测这应该是我小肠里息肉引起的。

因为PJS息肉可遍布于整个胃肠道,常见部位依次为小肠、结肠和胃,最常见于十二指肠及近段空肠等近段小肠。另有研究报道显示,约半数患者表现为全胃肠道分布(胃、小肠、结直肠均有息肉),且息肉数量较多,小肠是PJS息肉最好发的部位。息肉的形态主要是带蒂息肉,有点像葡萄,少部分为无蒂或广基息肉。

在网络上检索疾病时,我在仅找到一位病友留的问诊帖子,帖子里他留下了自己的手机号。我怀着忐忑的心情拨通了这个远在千里之外的电话。病友告诉我,小肠息肉切除需要在开腹的同时用肠镜配合。简单地说,就是开腹后在肠子上开一个小口,肠镜钻进去对整个小肠进行上下搜索,一次性切干净息肉。可是绝大部分地区医院没有这个技术,直到现在仍然有很多医院没有这项技术。

那时年纪尚轻,独自在外工作,收入微薄,又不想拖累家人,于是我选择服用本地医院开的解痉挛药。起初还有效,喝了肚子就不痛了,可时间一长便失效了。接着我又去本地医院开中药喝,这中药能缓解我腹痛症状,只要一喝这药,肚子就不痛,哪天不喝肚子就会痛。就这样我坚持了一年多,我知道我不能再这样拖下去了,我必须得去处理小肠里的息肉。

病友告诉我上海长海医院可以做,于是我在2005年3月15日那天踏上了去上海的大巴车,晚上躺在医院旁边民宿客厅的小床上,怀里紧紧抱着借来的两万块钱,一夜没睡,生怕被偷。

这次,我在长海医院肛肠科孟荣贵主任那得到了一次全方位的治疗。孟主任运用联合肠镜联合开腹手术方式把我小肠内隐藏的息肉都处理了。这次手术不仅解决了我当下的病痛,更重要的是,它避免了我因为息肉增长后在未来可能面临的肠梗阻、肠套叠风险,以及由此带来的反复开腹手术的痛苦。

记得出院那天,阳光是那么的温暖,空气是那么的清甜,人生原来如此美好!

在当地医院住院时我还得知了一个令人振奋的消息:一种能够完全探查整个小肠的气囊辅助式小肠镜(简称小肠镜)已经问世。得知这个消息后,我立刻在网上进行了搜索,发现这种小肠镜不仅能够检查,还具备切除息肉的功能。小肠镜可以经肛经口进入肠道,进入后镜子可明确看出息肉的形态、大小、数量、分布,在发现息肉后医生就可通过圈套器套住息肉颈部,通过纯电凝或凝切混合模式切除息肉。如果真的能成功,那我们就再也不用两三年开一次腹了!

我怀着激动的心情,将这一发现分享给了病友。他开始四处打听哪家医院有这个设备,联系全国各地的顶级医院和医生,希望找到能够提供这种先进治疗的医疗机构。然而,我们发现当时几乎所有的医院都仅将小肠镜用于诊断,对于使用它进行息肉切除的治疗,大多数医院都持保留态度,甚至不愿意尝试。

2005年初,小肠镜技术迎来了重要的转机。这位病友联系到了原北京空军总医院(简称“空总”,现为北京空军特色医疗中心)消化内科的毛高平主任。毛主任经过多方寻找相关厂家,专门定制了一种又细又长的圈套器,以适应小肠镜下息肉切除手术的特殊需求。2005年2月,毛主任及其团队成功完成了首例PJS小肠镜下息肉切除手术,,从而开启了中国小肠镜下切除息肉的新篇章。

接力棒从毛主任传给了他的徒弟宁守斌主任(现空总消化内科主任),在宁主任的带领下,科室不仅继承了小肠镜技术的独特优势,还在实践中不断攻克难题,进一步完善了这一技术。20年来,空总已经接诊了760多位PJS患者,成为全球最大的PJS小肠镜诊治中心。在最繁忙的时期,医院一天内甚至有25位患者同时接受小肠镜治疗。

毛高平主任和宁守斌主任

在小肠镜一切顺利,我们以为只要定期切息肉就万事无忧时,新的挑战又出现了:近年临床数据显示,女性患者宫颈胃型腺癌的发病率较常人高出数十倍。这个潜伏的杀手常被常规筛查遗漏。随着病友群体的不断扩大和交流的增多,我们发现很多病友都患上了宫颈胃型腺癌,而且基本上确诊时都已是浸润阶段。直到2021年底,我们才意识到这种癌症对我们群体的危害性。

2021年年底,通过豌豆SIR的陈懿玮博士,我们结识了上海红房子妇瘤科的康玉主任,并建立了专门的妇科群。康主任为我们群体提供了很多绿色通道,还建立了PJS上海多学科联盟。近三年来,我们不断推荐病友去康主任那里就诊,目前已有上百位病友接受了检查,但仍有许多病友尚未进行妇科检查。

很多病友在查出问题后,及时手术可以尽早干预治疗。有些病友查出宫颈有囊肿、叶状增生或不典型叶状增生,了解了自己的病情后,可以选择定期监测,防止病情恶化。更有多个幸运的病友,在宫颈胃型腺癌还处于原位癌阶段时就被查出,及时手术,从而避免了更严重的后果。

同事的好言提醒,病友的帮助,医生的勇敢探索就像是一颗种子,在我心里发芽,我想把这种互帮互助传递下去。

2006 年 2 月 24 日,在病友黎姐的建议下,我们建起了 QQ 病友群。19年来,群里汇聚了至少 2000 个患者家系。每当新进来一个病友,我就会迫不及待地想把自己知道的全部告诉他。

我比他们自己都更急,因为我知道得不到合理治疗的后果,那就是多次开腹甚至恶变。这种急迫的心情导致我在普及疾病知识时没有把握好度,引起了病友的恐慌。大家说群里每天讨论病情,看到就害怕。很多人选择屏蔽群消息,甚至有人怀疑我是医托,是否有所图。

以前,我总担心病友走弯路:担心他们不懂疾病知识从而延误病情,担心他们不知道就医渠道从而得不到最好的治疗,担心他们不重视,担心他们逃避…...尤其在实际工作中,每当有病友癌变、离世等触动我的事发生时,我总是会不自觉地引起大家的恐慌。

病友群的建立,让我们不再孤军奋战,不再孤立无援。起初,我不理解他们为何会有逃避的心态。随着时间的推移和专业医生的提醒,我意识到健康乐观的积极心态是克服疾病的基础。我在想,如何在宣传疾病知识和缓解病友心态之间找到平衡?我做了一些改变,病友群不再只允许讨论病情,允许聊轻松话题。我们还建立了闲聊群,让大家有渠道释放压抑情绪。宣传疾病知识时,也会注意轻重缓急和方式方法。

或许很多人会疑惑,为什么我会有这样的助人情结?

因为,我就是病友助人的受益者。

我想,总结我的就诊经历也能够体现出PJS群体二十多年来就医处境的改善和进展,是一个鼓舞和激励我们及所有罕见病患者群体的好方法。每个罕见病患者都经历过从一个人到两个人、十个人,再到现在找到了成百上千病友。

当我在键盘上敲下第2000个病友的入群欢迎词时,窗外的梧桐树正抽出新芽。20年前那个蜷缩在民宿小床上数着钞票的夜晚,我从未想过,那捧从指缝间漏下的月光,会化作照亮整个群体的星光。

如今的聊天群里,新生儿的第一声啼哭与息肉复查报告单的"无异常"交替刷屏。二十多家医院小肠镜的绿色通道如同纵横交错的血管,将希望输送到每个需要救治的生命;三代试管技术孕育的健康胚胎,正在温暖的羊水里舒展着没有黑斑的肌肤;深夜两点弹出的消息“刚做完小肠镜”,一切顺利;妇科群里又早筛一个子宫颈胃型腺癌!

黎明从来不是突然降临的。它是冬夜里手术室的彻夜长明,是18位专家自发成立的PJS上海多学科联盟,是2000多个患者在群里接力更新的《就诊攻略》。这22年教会我最珍贵的道理:希望是最强效的镇痛剂 。黑斑不会永远烙印在我们的唇边,当我们挽手成林时,那些星星点点的印记,终将在大地上连成通向曙光的银河。