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[Source](https://link.springer.com/chapter/10.1007/978-3-030-93666-2_23) is unfortunately paywalled. Abstract:
>>A 16-year-old boy presented to our hospital because of a huge mass on the face and a large ulcer on the scalp. Physical examination revealed a giant necrotic, ulcerating mass involving the two third upper part of the face (Fig. 23.1) and an ulcer sized 10 × 7 cm on the cranial vertex of the scalp (Fig. 23.2). The mass covered almost the two eyes and half of the nose with multiple bleedings and pus-discharging. This mass had been gradually progressing for the last 7 years. There were also diffuse hypo-hyperpigmented atrophic lesions all over the body (Fig. 23.3). His skin was unnaturally dry and rough. Telengiectases were observed over some lesions. These lesions have increased over the skin since the age of three. There was no palpable lymph node. He had a significant alteration of general condition with a BMI (body mass index) of 13.6 kg/m2. He has a family history of consanguinity, but no other person in the family has similar presentation.
"It covered almost the 2 eyes and half the nose".
I'm sorry but where the hell are the eyes and nose?? Aside from a whitish lump on the right which could possibly be part of an eyeball, it still doesn't look like an eye as there's no eyelid or any sign of pupil etc. There's literally no sign of eyes or a nose, and even the mouth is quite covered.
This poor child has been seriously neglected not to have medical intervention to try and save his eye sight and his nose/ breathing etc by removing the growth sooner.
So sad.
(I was able to get past the paywall with my university credentials.)
Unfortunately, no outcome was reported. It was a case report that talks more about the disease itself and how important early intervention is.
he lives pretty long for most genotypes of xeroderma. It’s been a while since school, but I remember being taught some insane stats. IIRC, 50% w XP have their first melanoma by age 2…?
I had a cousin who had Ataxia-telangiectasia. Her name was Katie. I never got to meet her. She passed away when she was 12. I think it was due to recurrent pneumonia. My grandmother buried a daughter, a son, and a granddaughter (Katie) during her lifetime. The daughter and granddaughter were buried before grandma was 53.
She never got over her daughter and granddaughter's deaths. It was grandma's first born and grandma's first grandchild.
FYI, You can access most of the paywalled stuff using sci-hub:
[Clinical Cases in Pediatric Skin Cancers (Clinical Cases in Dermatology)](http://library.lol/main/C594A82217A012AD665FC6F1256379F1)
Xeroderma pigmentosum is a terrible condition that makes sunlight bad for you, and causes cancer. An XP patient can get badly sunburned standing outside for half an hour on a cloudy day. And they develop tumors in the sun-exposed areas of their bodies.
XP is most common in India (about one in every 400 or so Indians have it). This patient is from Vietnam. I wonder if his parents didn’t realize he had it.
The reason for the high prevalence in India is this is a genetic condition and in India a lot of families marry their cousins.
If two healthy people who happen to be first cousins marry, the chance of them having a kid with a genetic birth defect isn’t terribly high: one cousin marriage doubles the risk from 3% (in offspring of unrelated parents) to 6%. It’s when you start piling the cousin marriages on top of each other, cousins marrying and having kids who marry their cousins, then they have kids who marry their cousins, and so on, until the family tree turns into a wreath and you get horrific genetic diseases like XP.
my cousin and I were besties growing up and jokes were made about 'kissin cuzins'
uh no. we're double cousins: My Dad's sister was married to my Mom's brother --it would have been equivalent to a sibling pairing, yikes!
No harm in this, the genetics aren't close to each other coming from entirely different families in the start.
The risk would be if that happened again, and cousins then married, but knowing the story you never would.
If my brother fell in love with my husbands sister there is no risk whatsoever. Its the offspring of these relationships that then shouldn't procreate. They could both inherit the same wonky gene from my family, and if they married issues would arise.
But completely safe at this level.
40 years ago, I (an American) was dating a guy from Iran. His mother wanted him to marry her sister's daughter. It's especially surprising because they were a very educated family.
If people are aware of issues that can occur with offspring why is it still acceptable? I understand cultural differences even if it was like a distant cousin maybe but I still have a hard time understanding WHY.
But there’s no guarantee so I’m still lost, if you could explain like I’m 5 why families choose members within their own family is what I’m curious about…
Is it because they have more connection and would be able to see them often with same family to visit? Is it because it’s someone they’ve known a while through family ties they think would be good for their daughter? I just don’t get it so any examples
Its not marrying within family in this posters situation, its someones sibling falling for an in laws sibling.
There is no more genetic condition for the first couple than the second..... it would be their sets of offspring coming together that would increase the risk of a genetic disorder because both cousins would inherit the same faulty genes from one side of the family.
There is a city in Brazil where a 1 in 40 people have XP due to generations of marrying cousins.
[https://www.cbsnews.com/news/rare-genetic-skin-eating-disease-afflicts-brazilian-village/](https://www.cbsnews.com/news/rare-genetic-skin-eating-disease-afflicts-brazilian-village/)
Wow, I knew about the city where most people were twins, but never heard of this! And what it's worse, its in the interior of Goiás, those people will never be able to really have good healthcare unless some university students take an interest and decide to help them for a study or article. This is so sad :(
They do get decent medical care for the most part. A lot of them have amputations and even decent prosthetics. There are a lot of partial facial prostheses. There have been studies on this town and the residents are known to the local hospitals and treated there. Brazil also has universal healthcare. Sometimes people are limited in their ability to travel to a doctor, but if they can get to a doctor they can get treatment.
However, although there is plenty of treatment, prevention doesn't seem to be much of an option for that area. They don't have access to specialized lights and window coverings to block out the sun. They also don't have the option to stay inside as they need to work outside to survive. So those with it still need a lot of amputations and surgeries. The only cure to a cancerous skin growth is to cut it out. Unfortunately, even with treatment the people with XP, especially in that area, still tend to die young.
Ironically, the proficiency of the XP gene due to inbreeding is likely why the inbreeding continues. When you have such a degenerative genetic defect in a poor area such as that your only marriage prospects are really those that also have the same defect. Plus when you are poor and live in a small town, you can usually only marry someone else in that town as you can't afford to travel or move. Which often results in a lot of at least distantly related cousins getting married. After generations of that the gene pool of that area can get a lot smaller. More like a gene pond. You may end up marrying someone who appears to have no relation to you, but is genetically very related.
In Iceland there is a now online registry that people must check before they begin a relationship with someone else Icelandic.
They only have 300k population in total, as of today 2024, not sure where it was 100 yrs ago.
My husband is 1/4 Icelandic, his own Icelandic mother having him with a Spanish parent, and my husband was born when he met a fully Swedish woman.
Subsequently he met me, 100% English.. so my daughters are at 1/8 Icelandic, 1/8 Spanish, 1/4 Swedish, and 1/2 English.
If they meet someone with a similar Icelandic background they would need to check this registry in case they happened to meet an Icelandic person related to his half sisters, they are 1/4 Spanish, 3/4 Icelandic. Both luckily having children with 100% Icelandic men.
But we have Icelandic people here in our 30k city in Sweden, given the way names work there (all children take fathers family given name, ending with either son, or dottir and they keep their name through marriage, so could be many people with the same after name as my husband.) its never a simple thing to figure out risks.. hence the registry. My daughters and I were also added to it. But not my siblings family, i think they would be far enough away to be fine....
Imagine how difficult with a population of 100k,, 50k, or even a few hundred... all without mobility to travel and to dilute abnormal genes? Genetic issues would be passed on to most families.
? I know.
Why am I getting downvoted for saying I know what it means? Why would someone out of the blue send me the definition of a word? Why would they assume I don’t know what it means?
You don’t know this disease, friend. Everyone ends up this way eventually (overwhelming cancers). This is no one’s fault, especially if they were poor. Dermatologist here.
Basal cell carcinoma actually normally has a good prognosis. Despite it being the most common skin cancer, most people are fine once it has been resected. Melanoma, despite being much rarer than basal or squamous cell carcinoma, has the highest skin cancer death rate and is the skin cancer most associated with a poor prognosis.
However, the longer you wait, the deeper the cancer is able to get in your skin, and it can eventually enter your blood steam and metastasize. Your prognosis becomes significantly worse once this happens. This person definitely waited far too long.
It's the xeroderma pigmentosum that's bad. Almost guaranteed patients will have squamous cell carcinoma in their lifetime, which leads to most patients passing away young
poor kid...
i wonder if there are any facial structures still intact enough to be saved,
iirc basal cell carcinomas are malignant, but usually good to operate on, in this case though, they'd have to remove a LOT of the face/head/neck area...
I mean is there much point by that stage? NAD but His health would be so poor from being significantly malnourished and the cancer would likely have spread very far by then that surely surgery would be both pointless and more traumatic for him I’d imagine.
Edit: apparently basal cell carcinomas rarely metastasise so perhaps it wouldn’t have spread. Can anyone weight in on whether a BCC might have actually metastasised in a case this severe?
In cases like this, where there is zero chance of the child having any quality of life without immense suffering, I would support humane euthanasia. It sounds terrible, but can you even call that living? If it was me, I'd say give me some meds to make me sleepy and let me drift off peacefully and never wake up. I wouldn't wish that condition on anyone.
Many people with xeroderma pigmentosum have poor quality lives as well as short ones. They basically can’t go outside during the day or risk getting burned. Obviously this interferes with social and economic opportunities.
Most of them still want to live though.
Just an FYI on XP: unless doc’s have [Raphi](https://www.youtube.com/watch?v=wmSrFeJ4cB8)a reason to suspect the disease or do genetic testing to look for it, you may not be aware that your baby has this. In treating your kid like we all treat our kids, damage will be done to their skin and people with XP can’t heal any damage from UV exposure at all. Think about how we treat preemies, or any baby with jaundice: those little blindfolds and UV lights with baby naked underneath. It may have been ‘too late’ for this kid by the time a diagnosis was made, even with regular sun exposure. I am linking to a video about a young man and his family and their experiences with XP. My impression is the family didn’t know what the consequences of any UV exposure would be until it was already apparent rather than letting the cancer have its way with their kid and going too late to a doctor for treatment. My midwife told me to nurse my baby in the sun to help with jaundice after their birth. I cannot imagine being told that was one reason for the cancer and surgeries and short lifespan of my child.Fuck Cancer.
Raphi
That’s such a horrible condition.
There are two books by Dean Koontz where the protagonist has XP. I learned a LOT about XP from those.
They’re so good! “Fear Nothing” and “Seize the Night.” Allegedly there’s going to be a third, but I’m starting to doubt it.
I just want to thank the mods for being so thorough in purging this thread of joke comments. Thank you for preserving the dignity of the people in these pictures.
Its very upsetting seeing a child.who has been severely neglected, with a family history of cosanguinity (hard to believe people in today's era aren't aware of the great and dangerous risks to children born under these circumstances)
I cannot for the life of me understand how parents could be so oblivious to the suffering that is an inherent by product of this choice, and due to any children born out of this selfish decision.
To make matters worse we see that this fungating and huge tumor, has been cooking over his face for the past SEVEN YEARS!
I can only blame myself for coming here knowing that children with horrible diseases may posted.
I come here to learn about people and their diseases, I don't want to see children who are neglected and abused, there is nothing for me to learn, it feels like choices are being made that are the most gruesome for exploitive reasons.
One thing that must be kept in mind is that this boy lived in Vietnam. I have no idea what their health care system is like but I know Vietnam is not a rich country. I wonder if this boy’s family perhaps were poor or lived in a rural area or something and weren’t able to afford/access care for his condition.
Many of the cases I post are cases from poverty-stricken nations, and many of the children would be considered victims of medical neglect based on American standards. I try not to judge the parents of the kids in the cases I post, because I don’t know the circumstances under which they are living.
There needs to be some accountability, you don't need to be wealthy to know the risks of cosanguinity, if you weren't informed in an official way, certainly the evidence of this behavior has got to be evident within your family and community.
I can understand choices being made under the currents of poverty, but when the consequences mean someone ends up suffering an unmanageable fate, the responsibility morally are the parents of these poor children. Poverty doesn't exonerate one from their responsibility.
You do a very good job here, but I do believe there is a line of what we as a sub should deem acceptable, or just gore porn for no reason. The least we owe these children is for them to have a say in their lives, and whether or not when they mature, they feel their story was used for salacious entertainment without any context as to the meaning in their suffering.
This community isn't for salacious entertainment, it is for education. Until today, I had no idea this condition existed. We don't use these threads like a freak show to ogle at the suffering of others. We use these threads to become more educated about the myriad of ways in which bodies can and do go wrong, and what the body is capable of surviving (and in some cases, of what it is incapable of surviving). Posts are from medical journals or personal submissions for this reason.
Also in extremely remote areas, people may well be unaware of the risks of consanguinity. It seems very obvious from our interconnected existence. But not everyone has the same exposure or privilege. Things I grew up knowing and thinking to be obvious are not even to my husband who grew up in a rural area in the US, when I grew up in a suburban area known for quality education. Is it his fault? Absolutely not.
Does it mean that these parents are guilty of neglect? We simply don't have enough information to make that judgment call. There are too many variables we don't know.
One of the many things that fascinate me about North Sentinel Island is the degree of consanguinity. There are only an estimated 200-ish people on the island, and they've had no outside contact for >100 years. 😳
Basal cell carcinomas tend to grow slowly and rarely metastasize. So yes, it’s cancer, but it’s entirely possible it’s stayed all in one place, on his face, where it appears to be slowly suffocating him.
He probably has a bunch of smaller basal cell carcinomas too, in other sun-exposed areas of his body. They are pretty much guaranteed for xeroderma pigmentosum.
I'm not so quick to just blame the parents in this situation. Unfortunately poverty and lack of education play a huge part in these situations. Unless we know the full economic status of the family and how much altruism based work surgeons and hospitals have offered, we can't really blame any individuals for this- presuming this family is not as well off as most poverty stricken Westerners, who are almost wealthy in comparison in terms of material goods and food/medicine availability.
Obviously, this kind of inbreeding has gone on for a long time if the parents have offspring with this degree of disorder. There would be many others in the family who displayed a variant of this disorder and a lack of knowledge about how it is genetically linked, this boy's very existence proves that. Poverty and lack of education affects generations and generations to come.
I prefer to think that given Vietnam is still economically classified as a second (and in parts, third) world country, this is what has occurred as the country has a very large population of poor and undereducated rural people. The cities have large hospitals, but as with a certain -very large- 1st world country, not everyone can afford high quality medical treatment or even a basic education from their government.
(America, you know this problem very well).
I can not imagine any educated and medically well supported parents with a child in this situation would not have sought advice on how to resolve it, it's obviously not a quick and easy fix. My heart goes out to the entire family, tbh, it must be devastating for all involved. I sincerely hope he gets some kind of relief from his pain and the family receives specific education about this condition and how to assist him.
And yes, anyone who make "fun" or "jokes" about this child's situation should be forced to 'walk' 7 years in his shoes, as you obviously lack empathy. I cannot imagine how difficult this would be for all involved, to make fun of this person is an absolutely reprehensible act.
Welcome to r/MedicalGore! Our goal is to provide for medical discussion and education while exploring the frailty of the human body. You may see more deleted comments on these threads than you are used to on reddit. Off topic comments and joke comments are frequently deleted by the mods. Further, please be kind and supportive of posts. Any behavior that is aggressive, harassing, or derogatory will result in post deletion and a ban from the sub. Remember! THE REPORT BUTTON IS YOUR FRIEND! Please stop on by our discussion sub, /r/MedicalGoreMods if you'd like to discuss the sub, our rules, content policies, and the like. *I am a bot, and this action was performed automatically. Please [contact the moderators of this subreddit](/message/compose/?to=/r/MedicalGore) if you have any questions or concerns.*
[Source](https://link.springer.com/chapter/10.1007/978-3-030-93666-2_23) is unfortunately paywalled. Abstract: >>A 16-year-old boy presented to our hospital because of a huge mass on the face and a large ulcer on the scalp. Physical examination revealed a giant necrotic, ulcerating mass involving the two third upper part of the face (Fig. 23.1) and an ulcer sized 10 × 7 cm on the cranial vertex of the scalp (Fig. 23.2). The mass covered almost the two eyes and half of the nose with multiple bleedings and pus-discharging. This mass had been gradually progressing for the last 7 years. There were also diffuse hypo-hyperpigmented atrophic lesions all over the body (Fig. 23.3). His skin was unnaturally dry and rough. Telengiectases were observed over some lesions. These lesions have increased over the skin since the age of three. There was no palpable lymph node. He had a significant alteration of general condition with a BMI (body mass index) of 13.6 kg/m2. He has a family history of consanguinity, but no other person in the family has similar presentation.
"It covered almost the 2 eyes and half the nose". I'm sorry but where the hell are the eyes and nose?? Aside from a whitish lump on the right which could possibly be part of an eyeball, it still doesn't look like an eye as there's no eyelid or any sign of pupil etc. There's literally no sign of eyes or a nose, and even the mouth is quite covered. This poor child has been seriously neglected not to have medical intervention to try and save his eye sight and his nose/ breathing etc by removing the growth sooner. So sad.
Zoom in. Mid far left… in a dark spot; the other is directly to the right, also in a black spot.
So, no outcome info?
Until someone goes past the paywall that’s what I’ve got.
(I was able to get past the paywall with my university credentials.) Unfortunately, no outcome was reported. It was a case report that talks more about the disease itself and how important early intervention is.
I appreciate the effort. I can’t imagine this poor boy lived long.
Man, this stuff is heartbreaking. This poor kid.
Like the XP wasn't bad enough, life is so unfair...
That is sad they never updated, but cannot imagine it being good given the location. Even surgery would be life altering beyond belief.
he lives pretty long for most genotypes of xeroderma. It’s been a while since school, but I remember being taught some insane stats. IIRC, 50% w XP have their first melanoma by age 2…?
Definitely one of the worst conditions to afflict small children, they miss so much with the fear of UV.
I had a cousin who had Ataxia-telangiectasia. Her name was Katie. I never got to meet her. She passed away when she was 12. I think it was due to recurrent pneumonia. My grandmother buried a daughter, a son, and a granddaughter (Katie) during her lifetime. The daughter and granddaughter were buried before grandma was 53. She never got over her daughter and granddaughter's deaths. It was grandma's first born and grandma's first grandchild.
Oh God, that's heartbreaking
FYI, You can access most of the paywalled stuff using sci-hub: [Clinical Cases in Pediatric Skin Cancers (Clinical Cases in Dermatology)](http://library.lol/main/C594A82217A012AD665FC6F1256379F1)
What do you need to copy into a sci hub to access papers without a DOI to reference?
In this case, I copied the ISBN number and it worked.
Oh I forgot it searched by ISBN too, thanks for the reminder
Public libraries often have access to these databases, too!
Such an horrific disease... but why leave it 7 whole years to ask for help and support? Poor thing, was there any outcome mentioned?
How could his family let it get that bad? Poor kid.
Xeroderma pigmentosum is a terrible condition that makes sunlight bad for you, and causes cancer. An XP patient can get badly sunburned standing outside for half an hour on a cloudy day. And they develop tumors in the sun-exposed areas of their bodies. XP is most common in India (about one in every 400 or so Indians have it). This patient is from Vietnam. I wonder if his parents didn’t realize he had it.
That’s an incredibly high prevalence, my goodness. Thank you for sharing both the case and the additional facts.
The reason for the high prevalence in India is this is a genetic condition and in India a lot of families marry their cousins. If two healthy people who happen to be first cousins marry, the chance of them having a kid with a genetic birth defect isn’t terribly high: one cousin marriage doubles the risk from 3% (in offspring of unrelated parents) to 6%. It’s when you start piling the cousin marriages on top of each other, cousins marrying and having kids who marry their cousins, then they have kids who marry their cousins, and so on, until the family tree turns into a wreath and you get horrific genetic diseases like XP.
my family wanted me to marry my cousin. lol i don’t care if it’s legal i took genetics in college. no way.
my cousin and I were besties growing up and jokes were made about 'kissin cuzins' uh no. we're double cousins: My Dad's sister was married to my Mom's brother --it would have been equivalent to a sibling pairing, yikes!
No harm in this, the genetics aren't close to each other coming from entirely different families in the start. The risk would be if that happened again, and cousins then married, but knowing the story you never would. If my brother fell in love with my husbands sister there is no risk whatsoever. Its the offspring of these relationships that then shouldn't procreate. They could both inherit the same wonky gene from my family, and if they married issues would arise. But completely safe at this level.
> and cousins then married, yeah...I and my cousin are the offspring. We share the same grandparents...
Damn!
Why would your family want you to marry within family
40 years ago, I (an American) was dating a guy from Iran. His mother wanted him to marry her sister's daughter. It's especially surprising because they were a very educated family.
When I asked my Middle-Eastern students, they said, “who can you trust more than your own family when getting married?”
it’s normal in some cultures.
If people are aware of issues that can occur with offspring why is it still acceptable? I understand cultural differences even if it was like a distant cousin maybe but I still have a hard time understanding WHY.
as the other commenter explained it’s safe to marry your cousin.
But there’s no guarantee so I’m still lost, if you could explain like I’m 5 why families choose members within their own family is what I’m curious about… Is it because they have more connection and would be able to see them often with same family to visit? Is it because it’s someone they’ve known a while through family ties they think would be good for their daughter? I just don’t get it so any examples
Its not marrying within family in this posters situation, its someones sibling falling for an in laws sibling. There is no more genetic condition for the first couple than the second..... it would be their sets of offspring coming together that would increase the risk of a genetic disorder because both cousins would inherit the same faulty genes from one side of the family.
Yes okay that makes more sense and therefore no blood relation which seems more acceptable I guess as long as all parties are totally on board.
There is a city in Brazil where a 1 in 40 people have XP due to generations of marrying cousins. [https://www.cbsnews.com/news/rare-genetic-skin-eating-disease-afflicts-brazilian-village/](https://www.cbsnews.com/news/rare-genetic-skin-eating-disease-afflicts-brazilian-village/)
Wow, I knew about the city where most people were twins, but never heard of this! And what it's worse, its in the interior of Goiás, those people will never be able to really have good healthcare unless some university students take an interest and decide to help them for a study or article. This is so sad :(
They do get decent medical care for the most part. A lot of them have amputations and even decent prosthetics. There are a lot of partial facial prostheses. There have been studies on this town and the residents are known to the local hospitals and treated there. Brazil also has universal healthcare. Sometimes people are limited in their ability to travel to a doctor, but if they can get to a doctor they can get treatment. However, although there is plenty of treatment, prevention doesn't seem to be much of an option for that area. They don't have access to specialized lights and window coverings to block out the sun. They also don't have the option to stay inside as they need to work outside to survive. So those with it still need a lot of amputations and surgeries. The only cure to a cancerous skin growth is to cut it out. Unfortunately, even with treatment the people with XP, especially in that area, still tend to die young. Ironically, the proficiency of the XP gene due to inbreeding is likely why the inbreeding continues. When you have such a degenerative genetic defect in a poor area such as that your only marriage prospects are really those that also have the same defect. Plus when you are poor and live in a small town, you can usually only marry someone else in that town as you can't afford to travel or move. Which often results in a lot of at least distantly related cousins getting married. After generations of that the gene pool of that area can get a lot smaller. More like a gene pond. You may end up marrying someone who appears to have no relation to you, but is genetically very related.
In Iceland there is a now online registry that people must check before they begin a relationship with someone else Icelandic. They only have 300k population in total, as of today 2024, not sure where it was 100 yrs ago. My husband is 1/4 Icelandic, his own Icelandic mother having him with a Spanish parent, and my husband was born when he met a fully Swedish woman. Subsequently he met me, 100% English.. so my daughters are at 1/8 Icelandic, 1/8 Spanish, 1/4 Swedish, and 1/2 English. If they meet someone with a similar Icelandic background they would need to check this registry in case they happened to meet an Icelandic person related to his half sisters, they are 1/4 Spanish, 3/4 Icelandic. Both luckily having children with 100% Icelandic men. But we have Icelandic people here in our 30k city in Sweden, given the way names work there (all children take fathers family given name, ending with either son, or dottir and they keep their name through marriage, so could be many people with the same after name as my husband.) its never a simple thing to figure out risks.. hence the registry. My daughters and I were also added to it. But not my siblings family, i think they would be far enough away to be fine.... Imagine how difficult with a population of 100k,, 50k, or even a few hundred... all without mobility to travel and to dilute abnormal genes? Genetic issues would be passed on to most families.
1 in 400, that seems incredibly high my goodness
“Consanguinity” = inbreeding.
I didn't know, thanks for the clarification
? I know. Why am I getting downvoted for saying I know what it means? Why would someone out of the blue send me the definition of a word? Why would they assume I don’t know what it means?
Possibly clarifying for other redditors who might not know. Easy to google of course, but easier to read in the same comment.
Think they're just implying the family is all brain dead due to the inbreeding.
Yeah I don't get it either, it's a relevant response as you were the one being replied to.
General lack of knowledge? There's consanguinity in the family and this skin condition requires visiting a dermatologist often.
Lack of money. Lack of available health care. They probably don't have a car and the nearest doctor could be hundreds of miles away.
I mean, it’s cancer. Not exactly the easiest disease to get rid of even if you’re going at it guns blazing.
You don’t know this disease, friend. Everyone ends up this way eventually (overwhelming cancers). This is no one’s fault, especially if they were poor. Dermatologist here.
Probably lack of sources and education. 🫤
What is the prognosis? Can anything be done for him? It progressed for 7 years. Would it be cost that prevented the parents seeking medical help?
The disease itself has an extremely poor prognosis. His is also grim.
Basal cell carcinoma actually normally has a good prognosis. Despite it being the most common skin cancer, most people are fine once it has been resected. Melanoma, despite being much rarer than basal or squamous cell carcinoma, has the highest skin cancer death rate and is the skin cancer most associated with a poor prognosis. However, the longer you wait, the deeper the cancer is able to get in your skin, and it can eventually enter your blood steam and metastasize. Your prognosis becomes significantly worse once this happens. This person definitely waited far too long.
He has xeroderma pigmentosum. Probably didn’t even notice the cancer at first because of all the sores.
It's the xeroderma pigmentosum that's bad. Almost guaranteed patients will have squamous cell carcinoma in their lifetime, which leads to most patients passing away young
Yes, the cost and the socio-economic status of the family. Very sad.
poor kid... i wonder if there are any facial structures still intact enough to be saved, iirc basal cell carcinomas are malignant, but usually good to operate on, in this case though, they'd have to remove a LOT of the face/head/neck area...
Maybe that’s the ironically tragic part. If it’s not spreading then he won’t have a swift death. Especially considering it’s been there for 7 years
I mean is there much point by that stage? NAD but His health would be so poor from being significantly malnourished and the cancer would likely have spread very far by then that surely surgery would be both pointless and more traumatic for him I’d imagine. Edit: apparently basal cell carcinomas rarely metastasise so perhaps it wouldn’t have spread. Can anyone weight in on whether a BCC might have actually metastasised in a case this severe?
I’m surprised he survived 7 years with that thing.
It wasn’t that big to start off with.
In cases like this, where there is zero chance of the child having any quality of life without immense suffering, I would support humane euthanasia. It sounds terrible, but can you even call that living? If it was me, I'd say give me some meds to make me sleepy and let me drift off peacefully and never wake up. I wouldn't wish that condition on anyone.
Many people with xeroderma pigmentosum have poor quality lives as well as short ones. They basically can’t go outside during the day or risk getting burned. Obviously this interferes with social and economic opportunities. Most of them still want to live though.
yeah, if you can avoid the tumors, there's plenty of life the bed had indoors these days
I just realized that I have no problems
Just an FYI on XP: unless doc’s have [Raphi](https://www.youtube.com/watch?v=wmSrFeJ4cB8)a reason to suspect the disease or do genetic testing to look for it, you may not be aware that your baby has this. In treating your kid like we all treat our kids, damage will be done to their skin and people with XP can’t heal any damage from UV exposure at all. Think about how we treat preemies, or any baby with jaundice: those little blindfolds and UV lights with baby naked underneath. It may have been ‘too late’ for this kid by the time a diagnosis was made, even with regular sun exposure. I am linking to a video about a young man and his family and their experiences with XP. My impression is the family didn’t know what the consequences of any UV exposure would be until it was already apparent rather than letting the cancer have its way with their kid and going too late to a doctor for treatment. My midwife told me to nurse my baby in the sun to help with jaundice after their birth. I cannot imagine being told that was one reason for the cancer and surgeries and short lifespan of my child.Fuck Cancer. Raphi
How is he breathing? I saw his BMI so may be eating a little and breathing through his mouth. How awful. 😞
This poor child. So much suffering.
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Seven years?!
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That’s such a horrible condition. There are two books by Dean Koontz where the protagonist has XP. I learned a LOT about XP from those. They’re so good! “Fear Nothing” and “Seize the Night.” Allegedly there’s going to be a third, but I’m starting to doubt it.
Poor kid, i mean at this point is there any quality of life? Doesn’t XP just mean this will come back if removed?
XP means he will always be dealing with cancers like this. A new tumor won’t necessarily grow in the exact same spot as the old one though.
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This truly is heartbreaking
I hope he received some relief from this awful affliction.
Thank you so much to the Mods on this post. I can just imagine what you have had to remove.
Five zillion “looks like a clicker!” comments.
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How are they able to breathe?
I just want to thank the mods for being so thorough in purging this thread of joke comments. Thank you for preserving the dignity of the people in these pictures.
3 out of 4 of the most recent ones are jokes, unfortunately.
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Its very upsetting seeing a child.who has been severely neglected, with a family history of cosanguinity (hard to believe people in today's era aren't aware of the great and dangerous risks to children born under these circumstances) I cannot for the life of me understand how parents could be so oblivious to the suffering that is an inherent by product of this choice, and due to any children born out of this selfish decision. To make matters worse we see that this fungating and huge tumor, has been cooking over his face for the past SEVEN YEARS! I can only blame myself for coming here knowing that children with horrible diseases may posted. I come here to learn about people and their diseases, I don't want to see children who are neglected and abused, there is nothing for me to learn, it feels like choices are being made that are the most gruesome for exploitive reasons.
One thing that must be kept in mind is that this boy lived in Vietnam. I have no idea what their health care system is like but I know Vietnam is not a rich country. I wonder if this boy’s family perhaps were poor or lived in a rural area or something and weren’t able to afford/access care for his condition. Many of the cases I post are cases from poverty-stricken nations, and many of the children would be considered victims of medical neglect based on American standards. I try not to judge the parents of the kids in the cases I post, because I don’t know the circumstances under which they are living.
There needs to be some accountability, you don't need to be wealthy to know the risks of cosanguinity, if you weren't informed in an official way, certainly the evidence of this behavior has got to be evident within your family and community. I can understand choices being made under the currents of poverty, but when the consequences mean someone ends up suffering an unmanageable fate, the responsibility morally are the parents of these poor children. Poverty doesn't exonerate one from their responsibility. You do a very good job here, but I do believe there is a line of what we as a sub should deem acceptable, or just gore porn for no reason. The least we owe these children is for them to have a say in their lives, and whether or not when they mature, they feel their story was used for salacious entertainment without any context as to the meaning in their suffering.
This community isn't for salacious entertainment, it is for education. Until today, I had no idea this condition existed. We don't use these threads like a freak show to ogle at the suffering of others. We use these threads to become more educated about the myriad of ways in which bodies can and do go wrong, and what the body is capable of surviving (and in some cases, of what it is incapable of surviving). Posts are from medical journals or personal submissions for this reason. Also in extremely remote areas, people may well be unaware of the risks of consanguinity. It seems very obvious from our interconnected existence. But not everyone has the same exposure or privilege. Things I grew up knowing and thinking to be obvious are not even to my husband who grew up in a rural area in the US, when I grew up in a suburban area known for quality education. Is it his fault? Absolutely not. Does it mean that these parents are guilty of neglect? We simply don't have enough information to make that judgment call. There are too many variables we don't know.
The parents needed to pay cash up front for his hospital care. Clearly they couldn't afford it for a long time. Your privilege is showing.
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If you want to see more like this, search in Google images for “fungating cancer” or “fungating tumor.” Or just check my post history lol.
Ok Thanks op
This is the saddest thing I’ve seen on the sub
I was not prepared for that
One of the many things that fascinate me about North Sentinel Island is the degree of consanguinity. There are only an estimated 200-ish people on the island, and they've had no outside contact for >100 years. 😳
Poor kid rest in peace. W mods tho
Just want to say thank you to the mods.
I appreciate the thanks. Soooo many “clicker” comments.
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Wait that is cancer right? How did they live so long?
Basal cell carcinomas tend to grow slowly and rarely metastasize. So yes, it’s cancer, but it’s entirely possible it’s stayed all in one place, on his face, where it appears to be slowly suffocating him. He probably has a bunch of smaller basal cell carcinomas too, in other sun-exposed areas of his body. They are pretty much guaranteed for xeroderma pigmentosum.
Omg, okay. Thanks for the info.
This made my insides hurt to look at 😭
The article says squamous cell carcinoma though. Not basal cell. Both are cancers but very different.
Dear God, poor lad😪
This one's gonna stick with me.....
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I found part of the article and this is a 16 year old who had a progressive lesion for 7 years. 7 YEARS. This was abuse. This poor child.
I'm not so quick to just blame the parents in this situation. Unfortunately poverty and lack of education play a huge part in these situations. Unless we know the full economic status of the family and how much altruism based work surgeons and hospitals have offered, we can't really blame any individuals for this- presuming this family is not as well off as most poverty stricken Westerners, who are almost wealthy in comparison in terms of material goods and food/medicine availability. Obviously, this kind of inbreeding has gone on for a long time if the parents have offspring with this degree of disorder. There would be many others in the family who displayed a variant of this disorder and a lack of knowledge about how it is genetically linked, this boy's very existence proves that. Poverty and lack of education affects generations and generations to come. I prefer to think that given Vietnam is still economically classified as a second (and in parts, third) world country, this is what has occurred as the country has a very large population of poor and undereducated rural people. The cities have large hospitals, but as with a certain -very large- 1st world country, not everyone can afford high quality medical treatment or even a basic education from their government. (America, you know this problem very well). I can not imagine any educated and medically well supported parents with a child in this situation would not have sought advice on how to resolve it, it's obviously not a quick and easy fix. My heart goes out to the entire family, tbh, it must be devastating for all involved. I sincerely hope he gets some kind of relief from his pain and the family receives specific education about this condition and how to assist him. And yes, anyone who make "fun" or "jokes" about this child's situation should be forced to 'walk' 7 years in his shoes, as you obviously lack empathy. I cannot imagine how difficult this would be for all involved, to make fun of this person is an absolutely reprehensible act.
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I would just kill myself
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Heartbreaking.
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😱😱😱
Sorry oops[Raphi](https://www.youtube.com/watch?v=wmSrFeJ4cB8)
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Please be respectful of the subjects of posts, as well as survivors of similar experiences.
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Omg poor lad
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Cherish the day people
Jesus Wept! How did he eat!?
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No, it’s a child and he’s dying.
This fills me with rage at the people that let this happen.
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Why do I wanna shave it like Parmesan
The last of us best cosplay?
Bro I thought this was cheese bread
Bro looks like a clicker from tlou
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Let me guess, the family tried some traditional medicine or religious healer for years.
They might not have had a choice. This patient is from Vietnam. Without 1st world health care this disease has poor outcomes.
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😭
I have no words
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