Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Jim Shannon Excerpts

Wednesday 13th July 2016 Westminster Hall

[Dame Margaret Hodge (Barking) (Lab)]

I beg to move,

That this House has considered awareness and funding for treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis.

It is a delight to move a motion under your chairmanship for the first time, Mrs Gillan, and I am grateful to the Speaker for selecting this issue for debate. I am also grateful to the Minister and look forward to having a positive exchange with him.

I had never heard of Stevens-Johnson syndrome or toxic epidermal necrolysis—my pronunciation of some of these medical terms may leave a little to be desired—until my constituent Nadier Lawson, who had suffered from the condition, contacted me. She has set up an awareness group, SJS Awareness UK, which is based in my constituency. It was because of her and that group that I asked for the debate.

SJS—and its much more severe form, TEN—is a severe reaction that affects the skin. It is caused by a whole range of standard medicines that we all use regularly. The reaction is most commonly caused by drugs used to treat epilepsy; some antibiotics, such as penicillin and sulphonamides; over-the-counter drugs such as ibuprofen; and medications that are commonly used to treat HIV and gout. The adverse reaction triggered by those drugs is devastating. I have seen pictures of children and adults who have had such a reaction, and they are truly shocking. People start with a skin rash, which rapidly develops into excruciating blistering across their skin, which starts to peel off. The condition particularly attacks the mucus membranes in the body—in the mouth, eyes, nasal passages and guts—and is similar to having third-degree burns on the skin. The condition is classified according to how much of the body surface is blistered: if it is less than 10%, the condition is called Stevens-Johnson syndrome; if it is 11% to 30%, it is called overlap syndrome; and if it is over 30%, it is called toxic epidermal necrolysis.

The initial symptoms that people experience are non-specific. Someone can take a pill one day and feel nothing for up to a week or two but then start to feel unwell and develop a rash, which is often assumed to be chicken pox, and may experience flu-like symptoms. A key problem associated with the condition is that all too often, it takes far too long to identify. Obviously, the first thing to do is to stop taking the medication that is causing the condition. Failure to identify the condition early enough can lead to terrible lasting effects, including permanent damage to the eyes—resulting at its most extreme in blindness—and lungs, loss of nail beds, arthritis and chronic fatigue syndrome. At the very worst, people die. Around one in 10 people with SJS, the mildest form of the condition, and up to a quarter of those with TEN, the most severe form of the condition, die.

I have a whole lot of case studies, but I thought it worth reading out just one, which was given by a young man who came to an event that I held in the House to bring together people who had experienced the condition. He is called Stuart Doyle, and he wrote and said this:

“Nine years ago I had a TENS reaction. I burned from the inside out and lost around 95% of my skin, all through second and third degree burns with permanent scarring. My finger and toenails burned off and have never grown back. The enamel on my teeth burned away. Mouth, throat, lungs and stomach all burned. My eyes burned and ulcerated, then fused to my upper and lower eyelids. My tear film was destroyed, as was my tear production and I lost all saliva production too. I also had inner ear burning and am now partially deaf in one ear. My genitals burned.”

I will skip a bit and give just a summary of what he said and wrote. He continued:

“I spent six weeks in a ketamine-induced coma, which I was placed in just two days after I arrived at my first hospital. I arrived with what seemed to be meningitis, it was textbook and it was moving fast. Two days later my oxygen SATS had dropped to the point where brain damage had begun its process. They acted quickly; they had already started treating me, my son, and my partner for meningitis. It wasn’t until after the lumbar puncture results came back, that they realised it was not what they first thought it was.

The high doses of anti-biotics were stopped, by this point my throat and lungs had begun burning and blistering and a rash now covered more than half of my body. It was the lungs and throat burning that had begun to close up my airways and provoked the need for a ventilator to keep my brain intact. The ketamine-induced coma was to try and get my heart rate back down from the 180 beats per minute mark caused by the pain of the internal burning. If they’d not done that, I’d have certainly died from cardiac arrest there and then. I was to stay in the coma on full life support for six weeks; my total hospital stay was three months. I woke up in a different city.”

He goes on to describe how the condition has impacted his life, saying:

“I hoped I’d die, I wished every night for 3 years after my reaction that I’d not wake again. I had more surgeries than I can recall, my eyes were in a terrible state.”

This is the treatment that he requires today:

“My eyes require a tremendous amount of work. My day starts before 6am and ends around midnight. I have to change my lenses at least 20 times a day, put in more than 100 drops, both lubricants and steroids, and then there is the ever present pain. But, it’s totally worth the effort and I am so lucky, and grateful for all the work that my doctors put in to get me to here.”

He then says:

“The hardest thing about my new life, is the chronic pain”.

[Jim Shannon (Strangford) (DUP)]

I congratulate the right hon. Lady on raising awareness of this issue. As far as I am aware, this is the first time that this condition has been brought to the attention of Westminster Hall and the Minister. The background information about the condition indicates that it can be triggered by normal medicines such as paracetamol. Is it time for the Minister and the NHS to address the issue by raising awareness of the condition among GPs, consultants and everyone else? The condition affects only one or two people in every million, but it is an important issue.

[Dame Margaret Hodge]

I completely agree. We are raising awareness through the debate, and I hope that the Minister will take action so we can get early identification and therefore prevent people from suffering the condition’s worst impacts.

The condition is rare, and therein lies the problem. I would appreciate it if the Minister addressed the following issues. There is a lack of awareness among many medical professionals, who just do not come across the condition. Insufficient attention is paid to the condition and its symptoms in the education and training of all health professionals. Survivors whom I have talked to all talk about that. A young man, Laurence McCalla, went to my local hospital, Queen’s hospital. They gave him antibiotics; it took about 24 hours to identify the condition. At one point he had 20 doctors and consultants looking at him, because it was new to them and they wanted to learn from it as a case study. Another lad, from Worcester, said:

“There is one big thing that stands out the most from this though. It astonishes me that so many doctors I have seen do not know about it.”

Debbie Hazel was misdiagnosed three times, as doctors thought she had chicken pox. She says:

“One of the problems was the lack of knowledge doctors have about the condition.”

The mum of a 13-year-old son, who lives in Surrey, says:

“My son was so ill and I couldn’t hold him or kiss him. He was screaming because his skin was so raw. I felt helpless. Nobody could tell us what was happening because nobody knew.”

My first ask to the Minister, therefore, is for a commitment that the condition, and its symptoms and treatment, should be taught to medical students, nurses and pharmacists as part of their educational courses. SJS Awareness, the organisation in my constituency, has a fantastic poster about the symptoms and how to spot the condition. Those posters could, if the Minister were to help us, be distributed to all GPs. Early diagnosis, and therefore awareness, would not just alleviate suffering; it would save lives.

Guidance has also been prepared on the clinical pathway by the British Association of Dermatologists. What steps will the Minister take to ensure that that guidance is followed throughout the country so that identification and treatment of the condition is not a postcode lottery? SJS Awareness has asked me to ask the Minister whether we could have an SJS awareness week for the general public. We are talking about such regularly used medicines—Optrex, ibuprofen, penicillin. They are standard medications, which we do not even think about using. We need to raise our awareness about the potential side effects.

Finally, because the condition is rare, money for research on it is limited. However, there is a cost to the NHS from not understanding the condition or recognising it early and understanding how to treat it. I understand the cost of treating skin reactions is about £500 million a year and it simply makes economic sense, as well as being a question of people’s lives, of course, to use research to get better at understanding why some people have such a reaction to drugs. An interesting key finding on genetic testing is that, in China, there is a gene in the population that predisposes people to different types of skin reaction, putting them at a higher risk from the drug carbamazepine. I do not know whether the Minister has come across that. In China and Taiwan, doctors test for the gene before they administer the drug. If we did more work here, we could manage that.

We also want research on new and better ways of treating severe reactions. From what I gather, more treatment should happen in burns units; often that is not understood and people are put into the intensive care unit and given the wrong medication. Finally, it is very important that we should have research on better understanding the features of drugs that make them more liable to cause the reactions in question. Those are three hugely important areas of research. I hope that the Minister can give us some comfort on that point.

People who saw the title of today’s debate would not have had a clue what I was going to talk about, yet the condition could affect any one of us, because we all take the medications concerned—they are standard. I have a file full of tragic cases of people affected by Stevens-Johnson syndrome, yet many people would not have a clue what we are debating. I ask the Minister, therefore, to do some practical things: to help us to raise awareness; to improve the training and development of all medical professionals, so that they understand the syndrome; and to get money for research so that we can understand the causes and prevent recurrences of this terrible condition in our population.