Toxic epidermal necrolysis: What is it ?
Lyell's Syndrome (T.E.N)
Lyell's syndrome (or TEN, toxic epidermal necrolysis) is a rare (1 in 1 million a year) and severe (death rate around 25/30%). This syndrome is a serious toxidermia, related to taking a drug. We also talk about Stevens Johnson syndrome (the difference is the percentage of body surface area affected).
It is characterized by a necrosis of the skin and mucous membranes (eyes, mouth, genitals). It can also reach vital organs. The syndrome is described as the most severe dermatological condition.
The care of this syndrome is to drive the patient in a burn unit. The treatment consists in stopping the drug which caused the TEN, a strict asepsis and intravenous rehydration. There is no treatment to stop the TEN…
The survivors of Stevens Johnson Syndrome or Lyell’s Syndrome have many after effects (ocular, risk of blindness, respiratory, cutaneous, oral, psychological ....).
My sources came from : WIKIPEDIA / PASSEPORT SANTE
The beginning
Here we are. I’m going to tell you all about my TEN. All victims of TEN and SSJ will know that this is very difficult to tell. This disease happens very brutally and without warning. These words were very hard to write, but things are what they are, and I have to give a sense to this dramatic experience!
This is my story.
Friday 29th September
Everything was fine. I quit my job, new start for me and my fiance. I’m so excited and hurry to start a new chapter of our life. My fiance was not around at that time! Not a problem, he was going to come back to me soon.
The same evening I spend my evening with my good friend M. I’m so happy because my good friend C. is there too! The evening is going on. I had some headaches. I’m not worry about that, I have a lot of headaches. I'm also starting to have an itchy eye. Not worry too, I have a lot of allergies to pollen / animals.... I do not worry. !!
I go back home for my first sleep in my new life..
Sunday 1st October
In the morning nothing has changed. Mom drives me back to the doctor. I remember the way people looked at me. I felt like a monster. What was happening to me? It's so hard.. I was scratching as hard as I can!
The doctor received me. Diagnosis: chickenpox. But I got it when I was a child! Well, we can reoffend. OK. The doctor was very nice, he gave me a pill of morphine. We went back home, I went directly to bed. I wasn’t able to get up well. I felt more and more bad. I never felt a pain like this before.
In the evening I took a shower to relieve myself again. I get out of the shower gets dressed. BAM 1st black out. First time in my life.. My father heard me fall. They put me back in bed.
I fell asleep, thanks to the morphine.
Tuesday 3rd October
I suffered to the point of telling my mother that I want to stop everything. It has to stop. It wasn’t possible to suffer so much. Does a chickenpox make you want to die? I suffered so much.
She called the Ambulance.
"No madam, a 28-year-old woman with chicken pox, I'm not moving an ambulance for that. "
So, my parents took me to the Emergency of Avignon by their own…
4:00 am: I woke up very brutally, because of important itching in my back.I was wondering if a spider decided to sleep with me. I went back to sleep.
In the morning, the itching is still there. My eye is still itching. I talked to my mom who advises me to wash my sheets and empty a small bottle of saline in the eye.
Nothing changed. I felt weak.
New surprise, it appears quite strange lesions in my mouth. Blood spots / small bubbles in my mouth. Never see that before ! That worried me. We were Saturday, I have to go to the medical house of Avignon.
Once there I see a doctor. He tells me that the lesions in my mouth are residues of angina that I had in early September. Small spots are allergic reactions, nothing serious. He still prescribed me cortisone. In the evening I have to go to my sister for a girls’ party.
The symptoms do not pass. It became very scary, I have more and more spots in the back and I start to have some on the face. I have to go back to my parents' house, because I felt so badly. I began to express my strong anxiety to my parents, who told me to calm down, that stress must play.
In the night my condition got worse. More and more spots all over my body. I got up very regularly at night to take hot showers…
Saturday 30th September
We called the family doctor who came to see me. She said it's chickenpox, but the meds did nothing to me to get better. I have 40 degree of fever, it was impossible to got up.
My mouth is more and more impressive, my tongue has become completely white. I took morphine again, to calm down.
In the evening I did a second black out when I wanted to take a shower. It got worse and worse…
Monday 2nd October
Highway to hell….
It was impossible for me to walk, they put me in a wheelchair and then on bed. Then, we were in box with my mother. An intern arrived and said "No, it's nothing like a chickenpox. Wait, you will see a dermatologist.” The time was long. The never ending story.
They had to drive me to the dermato unit. A man said, "I do not touch that. " Holly sh*t. The nurses drove me to the dermato unit. I had to waint in a corridor, a mask on the mouth. People looked at me like a monster. During this moment, I’ve just wanted something to stop the pain.
The dermatologist received me. I am asked to undress. The lesions are spread on the trunk, back, calves, a little in the arms, + face, and mucous membranes. My parents joined me. The doctors were 3 around me. They talked about "Steven Johnson" (who's that one?) Or a Polymorphous Erythema (CHAT THE HELL IS THAT?) These names did not tell us anything. I am asked to list ALL the meds I have taken during the last month.
"We are not able to take care of you here, we have to transfer you in Marseille, in the burn unit of the “Conception Hospital”.”
My mother was crying. Why ? What's happening ? Am I goig to die ? Burn Unit? At this moment the only thing I asked was morphine, because I could not stand to suffer any more. They did a biopsy (with local anesthesia) and send me back to my box before being transferred. Before leaving I get a perfusion with something like morphine.
The transfer went wrong. I close my eyes, impossible to reopen them. I was in the dark. All alone.
I did not know at that time that it was only the beginning of a long journey in hell.
I arrived at “the Conception” Hospital on Tuesday 3rd October 2017 around 18:00.
Before leaving the Emergency in Avignon, the lesions were spread over 30% of my body surface (Steven’s Johnson Syndrome). Arrived at the Conception, I was at 70%.
When I arrived, my eyes were closed, it was impossible to open them. The nurses reassured me. Everyone who were here introduce himself by giving me his name. They told me what they were going to do. They put an arterial catheter in my right arm. It does not hurt thanks to local anesthesia. This part was going well. Then I have to have an urinary catheter. That’s hurt me a lot. But the nurses were so king, and sweet with me, so I forgot this moment quickly. After a while, they opened my eyes.
I was so cold because of fever, they raised the temperature to + 30 degrees. It was better! They began to explain to me that they have to bandage me from head to toe. I stilled do not understand anything.
The hospitalization
The process began: they clean the body, cover me with sanitized linens for 10 minutes, then bandage me. During the cure, I remember that there was a doctor and a nruse. These two were so kind. They spoke to me a lot, about everything. They were joking. It was good.
Except that at one point, the nurse told me very nicely, "Camille, you must know. It is possible that tomorrow we are going to shave your head. I prefer to tell you so that you prepare yourself " I was crying. I did not understand. What disease can lead me to make me bend like a mummy, and lead me to shave my head?
3 hours after that I saw my parents. I looked like a mummy. It was very hard for my parents. They left the hospital, and I was alone. With my pain, all the catheters . I could not move because of the bandage. It was so hard..
I spend a very painful first night. I didn’t sleep.
Very early in the morning there is a problem. The arterial catheter didn’t hold. The nurses try several times fix it. Then in my other arm. It ends up working, I think.
Around 8:00 am / 9:00 am the exams began. Radios from the ribcage (I did not know at all why - it was to check if the organs were affected). I had to get new bandage..
The body surface was 90% affected.
D. (the medicine resident) came in my room. The first impression that I have was not really good. He wasn’t kind, sweet. I mean, it’s like he was here just to see the freak show.. The worst day of this episode begins.
"Well, what did you hear about what was going on? "
" Nothing "
"We have to make sure your organs are not affected"
"mh, ok. "
He has to put the central line. I did not know it at all. A catheter that is placed at the level of the neck and goes to the heart (it seems to me).
D. began the manipulation. As if it were not enough that I was suffering, he was surrounded by first-year students, to whom he begins to ask theoretical questions like "So, what are the main arteries of the human body? ".Meanwhile my face was covered.
It was just so hard to breathe. I had to ask that we raise this thing to get some air. He missed his gesture once.
I felt an embarrassing pain in my lungs. It was worse and worse. So I told him that I really hurt my lungs.
"Ah. I probably send you some air in your lungs. That’s happen sometimes". He has never apologize. He repeated his gesture and succeeds this time.
It’s not the end of the nightmare "We’re going to do biopsies."
Nobody gave me something for the anesthesia. Shit.
So yes, D. did 4 biopsies, just like that..
I was crying, screaming and asking him to stop.
Why 4 biopsies? Once the 3rd was finish he said "I missed it. I’m going to do it again.” He still does not apologize. This person really traumatized me. He was not kind at all. If he had conditioned me by saying "it's going to be painful, it's a bad time to pass. I'm going to count to three and we're going." But he did not. At this moment I was a number, on a bed. I wanted to die. On this table. People were coming into the room to take pictures of me. I felt like a laboratory animal. Welcome to the freak show.
Then suddenly, a ray of hope. The one that made me want to live a little more: the ophthalmologist M. arrived. She talked to me. She was so nice to me. She gave me injections (yes, in the eyes). I felt anything. She was the most beautiful thing that could happen to me that day..
End of this morning. Still looked like a mummy. Still from head to toe. This time they made bandages with silver, on the face and hands they put me a paste made of betadine. The pain is difficult to manage. I had the sensation of having acid on my face. I asked for a sedative, trying to complain as little as possible. The pain went away, thanks morphine! In the eyes: they are also affected, I have injections every 2/3 days, and drops to put every hour a lot of different drops. My mouth was the most painful.
02:00 pm: the time of the visits. My parents were coming. I told them that the morning was very difficult to live.
D. came into the room. "Well, since the arterial catheter in your arm does not fit, I’m going to put one on in the femoral artery." He left the room, I started to cry like a baby. A nurse arrived. I told her that I didn’t want to see him again. I didn’t want him to touch me anymore. She understood.
From there, the D. never took care of me again. (And, he never apologized)
[The following photos may offend the sensitivity of some people.]
The first week, so I was in the intensive care unit of the burn department. It was Dr. W. who took care of me. She was great. Kind, sweet. She told me that they would not shave my head. What a good news it was. My hair is the only part of my body that ties me to the person I was before.
During this period, I had radios every morning, silver dressings were changed every 3 days. They uncovered my face and hands on Sunday, October 8th. I cried every single day.
The lesions spread to my trachea. During 3 days it was very hard to talk, and breath. This passed fairly quickly but was very hard for me and also for my family. I could not sleep. The effect of sleeping pills was not at all relaxing. So they gave me “lexomil” quickly. I took some before sleeping, just to be less anxious.
During this first week, no one told my family what I had. They talked about a Lyell’s syndrome (TEN) when my I was stabilized. And I thanks God! At least they did not get alarmed on the internet by reading everything related to this serious disease.
I did not want to have many visits during my hospitalization. I only wanted my family, and friends really close. I had a silencing for 2/3 days because of the choking. I slept only a few hours at night. Maybe 3 hours..
Conclusion of my first week
I keep a lot of hard memories about the period when I was in intensive care. I remember the pain, the stress, the fear, my despair, the change of sheets that was a real obstacle each time, the pain of seeing my fiance and my parents leave the room each night, etc. Emotional roller coaster. Fortunately, the nurses were what they were, they made those dark days a little brighter. They have my eternal gratitude and I really admire them for being who they are! <3
The second week was "better".
I mean that the room was less scary. I still didn’t eat. I did a lot of rehabilitation, I was able to walk again quickly, mid-week it seems to me. Freedom. Same thing when they removed the urinary catheter, then disconnected me from everywhere. I had to irrigate 3 times a day. It was hard. But the nurses were so kind that I supported it well. I continued the drops every hour for my eyes .
My mouth lesions were important. Every cup of soup was a torture. But I had to eat. Protein were very important to recover, I knew I had to eat a lot. I fought against myself to make it happen.
Conclusion of my second week
Mentally it was the roller coaster. I cried every day. Sometimes it was better. Finding my independence was a step to my recovery. My condition was stable, but I cracked easily. The nurses reassured me: "You have no organ damage, your eyes are not too affected, there is no sign of regrowth, you really recover quickly for a TEN". Wait, what? Yes, we can make a regrowth ... Not for me…. But I had lesions on 90% of the body. Nothing will ever be the same again. The silver dressings are rubbing on my skin. I am brown from trunk to feet.
I knew I was disfigured. If I was in the burn unit and had bandages on my face, that was not because my twin was Emma Watson. Acceptance of the loss of my face was hard to handle. Fortunately, the time has done its job. Today I don’t see the marks on my face anymore. : )
The third week.
At the end of the 2nd week, one day a doctor came to see me "So I don’t know if it's good or bad news, but we are going to transfer you to La Timone (another hospital in Marseille), Dermatology department, we need the room.” I was scared, I had to start gathering my stuff, quickly. I didn’t have time to say goodbye to the nurses and caregivers I used to see every day. They were so nice to me! I wanted to see them before leaving.
So we came to LA TIMONE. I had a single room. Small bathroom with tub, the window opens, I can heard the birds singing and felt a little bit of wind. And big surprise: I could have someone sleeping with me. Best news I could receive. We also had the results of the Biopsy! It is a Lyell’s syndrome (TEN). They gave me my allergy card.
The day after my transfer, my bandages are removed to see the evolution of healing! Good news ! It's great, we could remove everything !!!!!No more bandages! Well I had the body all brown but it was ok! They helped me to take a shower. It was very hard to learn how to touch this body, which was not mine. I reassure you, over time, I love and accept it :-)
The care I had during this week was “Cold Cream” in the morning, the drops in the eyes , and twice a day I had a care done with a vaporizer Lucas on the lips, so detach the crusts. The condition inside my mouth has gotten worse. I spend two days without being able to speak. Before eating I took a a shot of “acupan”, then I eat.
My parents asked that I have permission to go home one night and one day. Doctors accepted.
Back to the hospital, my fiance was going to sleep with me for several nights on a small camp bed. We were happy to be together!
I lost 6KG in two weeks at Conception. I gradually regain strength. I took showers, even if I put a lot of time. I started to look at myself in a mirror.
After the third week the doctors made my prescriptions and gave me the procedure to start my new life. I had a thousand questions to ask them. I’ve got appointments in consultation with a gynaecologist, a cardiologist, and I have to return to the hospital in 3 weeks for a dermatology consultation.
24th October 2017 I leave the hospital. Reassured, but scared too.
Go back home
Going back home wasn’t as easy as you might think. The first few days I stayed in my room, locked up. I was afraid of everything, the sun, the death, and also the way that people could look at me.
My first step outside was a real catastrophe, to the point of don’t wanted to go out for a week. Today I go out all alone or with friends! It's really good to find the outside world.
I quickly started a therapy. Everyone who had a TEN or a SJS should do a therapy, because of the trauma!
The first nights I couldn’t sleep. I felt like I was at the hospital again. I was afraid of having to go back there. So I also started kinesiology sessions! Practice that I did not know, and that I had never experienced. I recommend this practice. Thanks to this I was able to sleep normally quickly.
The second week after I left the hospital, I had a lot of visits from close friends. It was good for me. I could talk, and laugh.
I also started to go out step by step. First in my garden. The appetite came back very quickly, I paid attention to what I ate.
Days after days, I learn how to enjoy life again, I take pleasure again to take care of myself, make me beautiful and go out <3 I do not see things the same way, that’s sure :-)
My after effects
The after-effects left me by my TEN are mainly esthetic, at the time of writing, we are 3 months after leaving the hospital (January 2018).
I have second-degree burn scars all over my body, so I put moisturizer every day on the body. I also use other moisturizers that I find comfortable. About my face, I hydrate my skin in the morning and in the evening. When I go out, I use sun cream SPF50+ on my face and on my neck.
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About my eyes : I really take care of them. I use 3 kinds of drops when I need it (about 15/20 times a day);
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About my mouth : my tongue is full of scars, but I have not lost the taste. My lips are not as elastic as before. I hydrate them every day too.
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Miscellaneous: My nails broked (feet / hands). It’s a casual after effect that we find a lot after these syndromes. It is not painful. Just do not touch it and it's okay. We have to be very patient. My hair are fine!
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Psychological part : trauma, so I started a therapy.
Today I'm writting my new life. After going through hell, we open our eyes to many things. Life is beautiful, and it becomes more beautiful when you go through hard times. This is the truth for every kind of disease which is falling from nowhere, to ruin our life, our dreams, our projects.. My TEN changed my whole life, but for the best !
My Lyell syndrome is probably the most beautiful lesson about life I could to have. I didn’t pull through it without a scar, but I survived. And I intend to draw something positive! So here I am today to share my experience, my tips and send a lot of positive vibes to all people who like me, fight every day against the disease to survive it, and be stronger. <3