SJS-TEN Patient Guide : simplebooklet.com

INTRODUCTIONThis patient guide was designed to help survivors and theirfamilies affected by SJS/TEN. Throughout this guide, youwill find questions to ask your doctor or healthcareprofessional along with space at the end of the booklet towrite down their answers. The content of this booklet is forinformational purposes only and is not intended todiagnose, treat, cure, or prevent any condition or disease.No material in this guide is intended to be a substitute forprofessional medical advice, diagnosis or treatment.Please consult with your own physician or healthcarespecialist regarding the suggestions andrecommendations made in this guide. The use of thisguide implies your acceptance of this disclaimer.

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DIFFERENCE BETWEENSJS & TENTEN is a more severe form of SJS.Differences between the two arebased on the total body surfacearea (TBSA) affected. Patients canbe classified into three groups: AntibioticsAnti-epilepsy or seizure drugsAllopurinol Non-steroidal anti-Inflammatory drugs(NSAIDs), such as IbuprofenThe most frequent cause of SJS/TEN is animmune reaction to a medication. Inchildren, viruses can cause similarsymptoms. Almost any drug can cause thisreaction but the most common are: WHAT IS SJS & TEN?Stevens-Johnson Syndrome (SJS)and Toxic Epidermal Necrolysis(TEN) are two severe and life-threatening reactions, commonlytriggered by medications, thatcause the skin tissue to developpainful blistering rashes, die anddetach from the body. SJS andTEN affect the external surfaceof the body, as well as themucous membranes of themouth, nose, eyes, genitalia,gastrointestinal, and lowerrespiratory tracts. They are notcommon, so they are treated inspecific Burn Centers and need avery specialized andmultidisciplinary team. THE FACTS ABOUTSTEVEN JOHNSON SYNDROME & TOXICEPIDERMAL NECROLYSIS (SJS-TEN)SJS covers < 10% of TBSA SJS-TEN covers between10-30% of TBSA TEN covers > 30% of TBSACAUSES OF SJS & TEN SIGNS & SYMPTOMS1-9 cases permillion developSJS & TEN 5-30% risk ofdeath with SJS &TEN, respectively[4]Symptoms can begin 1-3 weeks after amedication exposure. Initial signs mayinclude a fever and flu-like symptomsfollowed by a red to purple rash, skintenderness, skin blistering, and peeling. Fever FatigueHeadacheJoint painCoughBurning redeyesEarly Symptoms:Blistering of theskin, mouth, eyes,& genitalsRed rash on anypart of the bodySkin peelingBurning eyesLate Symptoms:Source: JAMA Dermatol. 2017

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Depending on the severity of your SJS/TEN,you may be hospitalized in an IntensiveCare Unit or a specialized BurnUnit/Center. Treatment may include antibiotics toprevent infection, IV fluids to bring backfluids lost through the skin that hassloughed off, and medications for painrelief. WHAT TO DO?MANAGEMENT AND FOLLOW UPRECOMMENDATIONSRecognizing the early symptoms of SJS/TENand seeking medical attention are the mostimportant steps to minimize the disease.Stop the culprit drug IMMEDIATELY. After discharge, keep up with allappointment visits, including specialtyvisits. Try not to be afraid of all new medications.The immune response is highly specificonly to the drug that caused your SJS/TEN.Talk to your doctor before starting a newmedication, even over-the-counter, andbe sure they know your history. Wear sunscreen (SPF > 30) reapplyingevery 2 hours, and protective eyewear asthe skin and eyes are sensitive to sunlightand may take months to heal. Follow all instructions, including use ofmoisturizers, eye and mouth care. For more information, visit the specialtycare and support group section. [5]

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SJS/TEN & SKINPatients with SJS/TEN require immediate hospitalization as symptoms can be life-threatening. When possible, patients are treated in a burn center or intensive care(ICU) unit along with a team of specialists. Possible complications of SJS/TEN include infection, difficulty breathing due to fluidin the lungs, and organ failure. In addition, patients may be unable to open their eyes,have a hard time eating, and suffer large amounts of fluid loss or dehydrationbecause water escapes through the areas of lost skin. Areas of the skin may becomescarred or discolored. Lifelong hair loss can happen along with loss or scarring of thefingernails or toenails. Due to the severe complications of SJS/TEN and healing time,hospitalization may last weeks to months. SKIN SPECIALTY CARETREATMENT Treatment of SJS/TEN will vary dependingon how much of the body is affected andany complications that happen. Treatmentmay include: Avoid sun exposure for severalmonths and use sunblock anytimethere is sun, the higher the numberof SPF the better! Follow all instructions forchanging your bandages at home,including putting on prescriptionointments or creams. Keepwounds moist and covered. If your wound or bandage developsa bad smell or pus, seek immediatehelp as this may be a sign ofinfection. You may be instructed to use amedicated shampoo whenshowering. For areas of skin withhair, use baby shampoo. If you have mouth sores, aprescribed mouthwash and specialtoothbrush or sponge may help. Scarring such as on the face, mayrequire special treatment oncehealed. A physical therapist can help youregain your strength to walk andget back to your normal activities.[6]FOLLOW-UP CAREWound Care: Hospital staff will help keepyour skin clean and moist. Dead skin issometimes removed, and bare patchesare covered with a special dressing,topical antibiotics, and moisturizers toprevent infection and promote healing. IV Fluids: To prevent dehydration,multiple fluids are given into the vein.Nutrition: The skin needs protein torebuild and heal. Wounds in the mouth orthroat may bleed and cause difficultieswith swallowing. A tube that goes intothe stomach through the nose may beused to ensure a high calorie diet andrecovery. Airway Management: A breathing tubemay be required for patients withdifficulty breathing along with givingoxygen.Pain Management: SJS/TEN can be verypainful, and painkillers are given to helpease any pain when needed.

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SJS/TEN & FEMALE GENITAL ORGANSGenital drynessItchingBurning or bleeding afterintercourseSexual dysfunction Painful sexual intercourse oreven inability to haveintercourse can occurProblems with urination SJS/TENS can affect the vulva and vagina. In the beginning, the vulva can appear redand form larger blisters while the outermost surface of the vagina can peel off.Resolution of these lesions may range from weeks to months if not treated. Long term complications of SJS/TEN could lead to the labia sticking together and thevaginal entrance tightening. Scarring inside the vagina could lead to narrowing andrarely, closure of the vagina. Common secondary symptoms patients can developdue to long term complications include: GYNECOLOGYSPECIALTY CARETREATMENT FOLLOW-UP CARE Suppressing your menstrual cycles(we don’t want you to have yourperiod) Placing a vaginal dilator with steroidointment or a barrier cream toprevent labial adhesions Placing a urinary catheter toprevent adhesions near the urethra Anti-virals to suppress genitalherpes in affected patients Yeast treatment Continuation or start using a dilatorwith a steroid ointment such asclobetasol or betamethasone. Youcan also use a dilator with lidocainejelly later on for pain control.If the vulva is not yet completelyhealed, consider application of asteroid or barrier cream around thelabia minora and majora to preventadhesions along with taking sitzbaths. Until healing of the vulvovaginal areais complete, continue menstrualsuppression.Your Gynecologist can do a vaginalexamination for any vaginalnarrowing or adenosis (patchesinside the vagina that could rarelylead to cancerous changes).Referral for pelvic floor physicaltherapy may also be considered.After discharge, it is important to followup with a Gynecologist when the vulvaor vagina is involved. Follow uprecommendations may include: [7]If female organs are present, aGynecologist should be consulted soonafter admission to the hospital forassessment of vulvovaginalinvolvement and counseling. During thehospital stay, treatment may include:

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Corneal ScarringDry EyesIngrowing EyelashesPhotosensitivity as well as othercomplicationsEyes with ocular involvement fromSJS/TEN, require acute and chroniccare. Most SJS/TEN survivors maydevelop eye symptoms includingvision changes. The common effectsof SJS/TEN on the eye include: The decrease in vision for some mayget worse over time (months oryears), while others can have severevisual problems right away. The moresevere vision loss can be described asa “functional blindness” becausemost survivors may have someresidual vision (about 1-15% of normalvision). However, the quality of visionis very distorted and can be affectedby environmental conditions such asheat, bright lighting, and sunlight. Theinflammatory process in the eyes cankeep going long after patientsrecover from the initial acute phaseof SJS/TEN. OPHTHALMOLOGYSPECIALTY CARESJS/TEN & EYESWHAT IS AMNIOTIC MEMBRANEGRAFTING SURGERY?If there is eye involvement,determined by an eye exam by anOphthalmologist, sometimes topicalmedication is used, and if the eyedisease is severe, sometimes anAmniotic Membrane Grafting Surgeryis needed. It is especially important for everypatient to schedule a follow up examwith an ophthalmologist (notoptometrist).TREATMENT[8]The procedure involves applying ahuman amniotic membrane madeof a combination of tissue andcells as a biological dressing tohelp reduce the risk of vision loss,scarring of the eyes, and promotebetter wound healing of the eye. The procedure is performed by acornea specialist and should bedone within 7 days of the start ofthe skin rash. The membrane isless effective if performed later. FOLLOW-UP CAREA cornea specialist will perform acomprehensive baseline eyeexam to monitor for any changesof the eye disease over time. Management of eye inflammationmay include medical or surgicaltreatment such as topical steroidor other anti-inflammation drops,antibiotics, serum blood tears ortherapeutic contact lenses to helpprotect the eye and relieve anypain from dry eye syndrome. Surgical management mightinclude eyelid repair which whenpointed inward, can lead to thelashes rubbing on the surface ofthe eye. Adhesions between theeyelid and eyeball may alsorequire surgical repair.

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SPECIALTY CAREPULMONOLOGYUROLOGYPSYCHIATRY/PSYCHOLOGYSPECIALTY CARESPECIALTY CARECoping with SJS/TEN can involve intense emotionsfor both the patient as well as family members.What you have gone through is traumatic and willtake time to heal. Professionals may be helpfulduring the hospital admission and followingdischarge. A psychiatrist or psychologist canprovide treatment for mental health concerns,such as post-traumatic stress disorder, related toyour SJS diagnosis. After SJS/TEN, more than half of patients haveabnormal oxygen flow while breathing. However,severe long-term lung complications are rare. Apulmonologist will assess your breathing using atest that involves breathing through amouthpiece. It is recommended that SJS patientsfollow up with a pulmonologist for 1 year after thehospital. The penis and vagina can be affected by SJS/TENand there can be pain with urination. Blood in theurine and difficulty emptying the bladder whenurinating are also possible. While in the hospital,these issues may require a catheter to be placedin addition to evaluation by a urologist. [9]

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FAQs03. WILL I HAVE PERMANENT VISIBLEDAMAGE TO MY SKIN? 01. WHAT ARE THE LONG-TERMCOMPLICATIONS OF SJS/TEN?Scaring happens if the reaction was deepenough, and follow-up care will help let youknow what you expect. Not all patients willhave long-term skin damage, but possiblechanges include darkening or lightening ofthe skin, scars, nail changes, hair changes,and eczema or dry skin. In some cases,scarring can be treated with laser therapy. SJS and TEN can leave victims with reducedvision (mild to severe vision loss includingblindness), damaged lungs or other internalorgans, chronic skin pain, and scarring.Patients can suffer from anxiety, depression,or even post-traumatic stress disorder afterthis highly painful experience. 02. HOW LONG WILL IT TAKE FOR MY SKINTO HEAL? The amount of time your skin will take to healdepends on the severity of the reaction.Recovery can take anywhere from weeks tomonths, sometimes years. [10]

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FAQs[11]04. IS SJS/TEN GENETIC?Currently, genetic tendency for developingSJS/TEN has been reported. However, thishas only been seen with some specificethnicities and associated with specificmedications. Most prescribers are aware ofthese risk factors and should discuss genetictesting with patients at high risk if necessary.Otherwise in most cases, there is no definitegenetic link, but SJS/TEN running in familieshas been reported. 05. WHAT TREATMENT OPTIONS AREAVAILABLE?At this time, there is no definitive cure ortreatment for SJS/TEN. The most importantthing is to never take the offending drugwhich caused your disease again. Yourdisease may be treated with a combinationof ointments that go on your skin or eyes,oral pills, IV medications, steroids, andantibiotic eye drops. For more severe diseaseamniotic membrane grafting may be doneearly on. It is important to keep the skinmoist and continue that at home and be sureto follow up with multiple specialistsfollowing discharge.

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FAQs08. CAN I QUALIFY FOR DISABILITY AFTERSUFFERING THROUGH SJS/TEN?07. SHOULD I BE CONCERNED ABOUT THECOVID-19 VACCINE IF I'VE HAD SJS/TEN INTHE PAST OR MORE RECENTLY?Many SJS/TEN patients are unable to workdue to lingering side effects. The sooner youcontact apply for disability, the better asapproval can take months to be approved.You may also need to contact Social Servicesto receive Medicaid, or your employer forshort or long-term disability benefits. Mosthospitals have a patient advocate and socialwork on staff to help.It is understandable that survivors ofSJS/TEN would be hesitant to take anymedication and vaccines even outside thedrug that was implicated in their reactiongiven the severity of the disease. However,those who have recently experiencedSJS/TEN and survivors of SJS/TEN can bereassured that receiving the SARS-CoV-2vaccine is a safer option than naturalinfection.[12]06. WHO CAN GET SJS/TEN?SJS/TEN can affect patients of all ages,races, ethnicities, and genders. More caseshave been reported in women compared tomen. Patients with a previous or familyhistory of SJS/TEN, and immunodeficiencymight be at an increased risk.

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SUPPORTGROUPSSTEVENS-JOHNSON SYNDROMEFOUNDATIONWebsite: www.sjsupport.orgEmail: sjsupport@gmail.com Be assured , you are not alone! The SJS Foundation is a non-profit organization thatprovides support and resources to patients andfamilies affected by SJS/TEN. More information can befound at http://sjsupport.org/ [13]LOYOLA STEVENS-JOHNSONSYNDROME SUPPORT GROUPContact: Heidi MaidlPhone: (219) 263-3334Email:babybellissima3@yahoo.comSJS AWARENESS UKWebsite: www.sjsawareness.org.ukEmail: info@sjsawareness.org.ukSJS KIDS SUPPORTWebsite: sjskidssupport.webs.com

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This SJS-TEN patient guide was led by Dr. Eden Lake and DjoniElkady at the Loyola University Medical Center with the supportof various specialty experts and contributors we'd like to thankbelow. Contributions By: Alexandra Kiszluk, BS Anjay Khandelwal, MD Charles S. Bouchard, MD Esther Fuchs, MD Taylor Starnes, MD Arthur P. Sanford, MD Elizabeth Simmons, PsyD This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. This booklet is intended for informationaland distributive purposes only. Any other use is strictly prohibited without priorwritten consent. [14]ACKNOWLEDGEMENTS

SJS-TEN Patient Guide

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