by Werner Kratz, December 2025
I have Parkes Weber syndrome—an inherited vascular disorder that manifests through arteriovenous malformations and, in my case rather atypically, also through a huge port-wine stain extending from my sacrum down to the tips of my toes. My condition was therefore impossible to miss from birth. As a small child, my parents took me to every specialist they could find, but at the time medicine was still largely helpless in the face of this disease. I was documented as a medical curiosity, without any truly effective therapeutic approaches. I was also fortunate to live through my childhood without significant symptoms, which spared me further medical visits for many years.
The first problems did not appear until my mid-twenties, and at first they only earned me compression stockings. At 29, I encountered the full dimension of the disease for the first time. Suddenly a deep, poorly healing wound formed under the ankle on my foot. This ulcer caused severe pain, especially when lying down. Since then I have known how exhausting it is to fall asleep only for minutes at a time night after night before the pain jolts you awake again. Even youth could not counteract the strain of sleep deprivation.
My doctor immediately referred me to the university hospital in Aachen. The question was what treatment was indicated now. It became a long day in the hospital, being passed from doctor to doctor. No treatment option emerged right away—only one thing became clear: “the professor has to look at this.” I would hear variations of that sentence many times in my life. “May I show this to my students?” is entirely fine—if “this” is my leg, I am being asked directly, and “No” is an accepted answer depending on the day. Back then, however, I was usually still the “this,” and the question was addressed primarily to the doctor who had examined me—students were not even mentioned at first.
So my odyssey through the hospital ended at the door of a monumental clinic that bears some resemblance to Paris’s Centre Pompidou—only bigger. Its exterior elevator shafts and pipes protruding from the building are not architectural extravagance; they arose because a building with an undersized air-conditioning system had been built on sand. The odyssey ended in front of a door where a whole line of patients were already waiting for an audience with the professor.
I had taken a full day off work, and I was relieved that my visit would finally serve the purpose for which I had come: to obtain a treatment perspective for the hole in my foot. I was finally invited through the door—and to my complete surprise I found myself in front of a seminar room full of medical students. The professor asked me to show my leg, and I complied, reluctantly. After all, my future depended on his decision. And the pain and the sleepless nights it caused had left their mark on me. So I stood in my underwear in front of the assembled students while the professor lectured about my condition.
Then the professor’s talk ended. The sound of many chairs being pushed back at once suddenly filled the room. All the students stood up simultaneously and surrounded me. Everything happened in sync, without warning. A student pulled my underwear aside from behind so that the port-wine stain on my bare buttocks could be seen as well. While holding on to my underwear, she asked me a question—effectively fixing me in place for the examination. Any attempt to move away would only have exposed me further. Another student pressed a finger onto my port-wine stain without asking. When do you ever get the chance to perform a blanching test on a living subject? If you press long enough, the compressed spot briefly fades. It worked on my leg, too. From somewhere behind me the professor said to a student, “Ms. X—this is exactly your topic, we need to document this carefully.” By then, the “this”—that is, me—had become a pillar of salt. I no longer reacted consciously; I disappeared into the situation.
I came back to myself after I had apparently stared for a long time at the pattern of the carpet outside the seminar room. A patient who had entered the room right after me came back out in tears. “Why didn’t any of you say something?” she shouted into the waiting area. At the time I had no answer—why didn’t I say anything? I didn’t know. Somehow it felt like my personal failure. Only decades later did I understand that the whole procedure had left me with no real alternatives. In a situation where neither fight nor flight is an option, freezing is the only response the body still has available. The entire sequence—the ambush of the “this”—was very effective at providing the future doctors with an indispensable learning experience. How the objects of the examination felt was presumably not considered relevant. It worked, it was passed down from one generation of doctors to the next, and only in the 2000s was it finally abandoned.
I asked the nurse who had led me into the room what would happen now, what the professor had decided. The professor, I was told, was already no longer in the hospital. I should “just make a new appointment.” That was enough for the day. In my hand I still had a note someone had pressed into it during the examination in the lecture hall: I was supposed to report to the medical photography unit. But I had had enough—the note ended up crumpled in the next trash can. My wound was still open, and the dressing I had removed for the examination had not been replaced. After some inquiries, a civilian service worker (“Zivi”)(*1) was assigned to put on a new dressing.
The dressing did not hold for long. As soon as I stepped out of the hospital, I noticed I was dragging it behind me like a white flag, half unrolled. I tucked the end into my shoe and limped to the bus stop. Somehow fitting for that day: a defeat could not have felt more complete. I capitulated.
I never entered the Aachen hospital again as a patient. My leg was operated on in another hospital—with only moderate success. A vein stripping procedure would no longer be performed on a patient like me today. Back then, the range of treatment options was still very limited. It took almost a year for the surgical wounds to heal, and I internalized a rule: avoid hospitals—especially university hospitals—whenever possible.
With the turn of the millennium, my job brought me to Switzerland. For many years I had a local phlebologist in Solothurn who helped me through various pain episodes and minor inflammations. But in my mid-fifties I developed another major ulcer on my foot. My doctor said a specialist needed to see it, and on the recommendation of a vascular specialist in Solothurn I ended up at the outpatient clinic of Bern’s Inselspital. Only there did I learn that, at least in Switzerland, angiologists are the specialists who deal with congenital vascular malformations like mine.
I learned that over the past two decades there had been tremendous progress in treating vascular malformations; that there might even be an option to treat the disease with medication; and that Inselspital is actively conducting research in this field. The resident who examined me was committed and took time to show me entirely new perspectives on my condition. That gave me hope. I trusted them, but I hesitated about the photographic documentation of my condition. Still, I had an open wound on my foot—any treatment progress had to be documented.
More appointments followed—examinations, and even a genetic test—which unfortunately made the indication for drug therapy seem more questionable again. I had obviously “hit the jackpot.” Right after the genetic test, I learned that I had been “upgraded” from Klippel–Trénaunay syndrome to its aggressive little brother: Parkes Weber syndrome. In my case the syndrome is additionally characterized by a mutation combination across several genes that has not been described before. PWS is more aggressive because it is marked by fast-flow arteriovenous fistulas. In connection with the genetic test, I signed a general consent form.
New symptoms have surprised me throughout my life. It seemed important to contribute so that future generations would receive answers—and perhaps even a perspective, a treatment. In addition to allowing my genetic and other data to be used, I explicitly signed that my personal data would remain protected. I knew that photos were not mentioned there. After all, under Switzerland’s data protection law (DSG), the disclosure of personal—particularly sensitive—health data requires the patient’s explicit and informed consent. Since the revised law came into force in September 2023, substantial fines are potentially on the table for violations.
Alongside various examinations, photographic documentation was continued at every visit. Especially in the context of optional drug therapy, I began to inform myself and read numerous medical articles. I was surprised how often patients were pictured, particularly in Swiss papers. I wasn’t used to that, and I found the way people were sometimes shown rather exposing: one side of the underwear was pulled up to make the entire malformation visible. I have always rejected this kind of documentation. I was clearly not alone, as I learned in conversation with a nurse who photographed me during an exam: “Good—someone finally says something.” After all, the patients remained recognizable to people in their surroundings.
And then came November 2023. I had been discussing with colleagues my preference for traveling first class on trains during peak hours. I am almost two meters tall, and because of my condition I quickly have problems when I sit cramped for a long time. On that occasion I also mentioned my illness—and how ultra-rare Parkes Weber syndrome is. Three days later a colleague told me there was a medical article about me.
The medical article (*2), published under a Creative Commons license by a U.S.-based publisher, was written by authors from Bern, Belgium, and Germany. They described Parkes Weber patients from Inselspital in a collection of short case reports. In my case my exact age was also mentioned indirectly. Only my age during the pandemic was stated—but since everyone knows which two years the pandemic affected us, my age was pinned down to within two years. For my colleagues, that was enough to be sure it was about me. In the months that followed, the article became a recurring topic at the office coffee machine. The reactions were consistently empathetic and made me a bit more open about my condition.
What made it unbearable for me, however, was that the article also contained two photographs of my leg. Body positivity may be a helpful approach—but it should be up to the owner of the “body” to decide when to present it positively. I felt like a fairground attraction: “the man with the red leg,” put on display for the entire world. It felt as if, decades later, I had picked up again the slip of paper I had been handed during my visit to Aachen—in order to bring the humiliation of that time to an end. It was the first time in decades that my thoughts returned to that long-ago event.
For me it was clear: these photos had to go. Since I didn’t know the first author, I first searched online for who might be responsible and ended up at the Ethics Committee of the Canton of Bern—only to learn that their website was offline in November and December 2023, placing the committee out of reach for me as a patient. However, I personally knew a few of the co-authors, so I wrote to them. Somehow the whole thing hit me harder than I wanted to admit. When I received a call back, my hand was trembling so much that the phone almost slipped from my grasp.
Fortunately, we quickly agreed. An erratum was published removing the photos from the article. The revised article was online in early 2024. In this context I also had direct contact with the journal’s publisher, which may have accelerated the process. Still, it took until spring 2025 for what was, for the time being, the last copy of the original article to go offline. Clearly, the mechanisms an institution like Inselspital uses to spread information are much better suited to dispersal than to recapturing it.
Ultimately, Inselspital decided to adjust its internal processes and in the future ask all patients whose photos it intends to publish. An important step in the right direction. For me, however, the article ended my participation in research under the general consent. I did that because the medical article links my unique genetic defect, at least for a larger group of people, to my person. With this text here, I expand that link significantly once again. The case report is a kind of public key, and any follow-up publication would be traceable back to me. In addition, it was unclear to me how the photos— which identify me personally due to the large capillary malformation and thus render anonymization meaningless and pseudonymization ineffective—would have been handled. And for me the idea that the photo might surface elsewhere again was not acceptable.
The whole incident prompted me to look for ways out of my own helplessness in such a situation. I first joined the German “Bundesverband Angeborene Gefässfehlbildungen,” and there I met the people with whom we later founded a patient association in Switzerland: the “Verband Angiodysplasie Schweiz.” I also had the opportunity to talk to other patients with Parkes Weber syndrome. Interestingly, they too often described their experiences with university hospitals as feeling like a fairground attraction. I was not the only one who repeatedly felt put on display.
And yet research is of course extremely important if we want to secure the great advances that are emerging in the treatment of vascular malformations. But how should research be conducted if it is to do justice to patients?
For me, there are some key demands:
• Any use of patients’ data should be made transparent in its full scope. Patients must be enabled to make an informed decision about supporting research so they can decide what risks they are willing to take.
• This means, in particular— as is already common practice in other countries— that an explicit, modular consent must also be obtained in Switzerland for photographic documentation.
• If case reports about patients with ultra-rare diseases are to be published, it is essential that patients are explicitly asked for consent. After all, patients take on a significant risk of re-identification.
• This also means that when signing a general consent, patients should be informed that rare—and especially ultra-rare—diseases carry a significant risk of re-identification.
• It also means that within SPHN—through which Swiss research institutions will become increasingly interconnected—the decision on what to do when research activity entails a significant re-identification risk should not be left to ethics committees but to the patients themselves. FAIR (Findable, Accessible, Interoperable, Reusable) is only “capitalized” for patients if patients can consciously take on risks themselves. And a data catalog like SPHN’s could, over time, be expanded by an additional layer that would allow patients to manage their consent to data use dynamically.
• It also means that the composition of ethics committees—still often primarily made up of distinguished physicians and lawyers—should be adapted to our increasingly digital world so that this important body can fulfill its oversight function.
Research will be supported by patients if they are not seen merely as subjects. Everyone wants to be seen as an autonomous person who can make self-determined and informed decisions about supporting research. Advancing the global networking of medical science offers new opportunities to develop therapies for rare and ultra-rare diseases. We patients are certainly ready to contribute as partners and to help move this development forward. We will not play the attraction of a global medical fairground.
(*1) In the 1990s, civilian service workers (“Zivildienstleistende,” “Zivis”) played a central role in German society—especially in healthcare, social services, and nursing. Civilian service was the alternative to military service for men who refused armed service on conscientious grounds, and it existed until 2011.
(*2) Andreoti, Themis-Areti A.; Tuleja, Aleksandra; Döring, Yvonne; Maiolo, Massimo; Schaller, André; Vassella, Erik; Zweier, Christiane; Boon, Laurence M.; Vikkula, Miikka; Rössler, Jochen; Bernhard, Sarah M.; Baumgartner, Iris. Parkes Weber Syndrome: Contribution of the Genotype to the Diagnosis. Journal of Vascular Anomalies 4(4):p e076, December 2023. | DOI: 10.1097/JOVA.0000000000000076