Her body burned by drug, not fire

[nursebedlam]

Her body burned by drug, not fire

18-year-old has severe reaction to prescription

09:59 AM MST on Tuesday, May 4, 2004

By Carla McClain / Arizona Daily Star

A beautiful 18-year-old Tucson softball player has suffered severe burns over 80 percent of her body but has been nowhere near a fire.

In an extremely rare, often fatal and little-known severe toxic reaction to a medical drug, Samantha Grasham's body caught fire - from the inside out - blistering most of her skin, as well as her mouth, throat, esophagus and airway, perhaps leaving her scarred for life.

She spent nearly three weeks in the Burn Unit at St. Mary's Hospital, as doctors there worked to save her life and her skin.

As happens in most of these strange cases of what is known as Stevens-Johnson syndrome, there was no warning that Grasham would react so severely to a medication.

Most people never know they are at risk, and doctors often do not recognize the syndrome when they see it. Yet many common drugs - including prescription painkillers and antibiotics - even over-the-counter pain and fever medicines for children - are linked to this "fire in the flesh."

In the worst cases, people are not only scarred, but also blinded and dependent on breathing and feeding tubes - if they survive. In its severe form, when the skin is literally burned off the body and even internal organs are damaged, about half die.

The U.S. Food & Drug Administration has issued dozens of warnings about drugs that can trigger Stevens-Johnson syndrome (SJS) - including the one given to Grasham - but the warnings receive little publicity.

"I think it is commonly misdiagnosed, especially in the early stages," said Dr. Norman Levine, longtime University of Arizona dermatologist, who sees about one case a year.

"But a physician will seldom miss it in a very sick patient, once it has severely progressed. When that happens, it can be devastating."

Although the syndrome is rare - occurring at the rate of about 1 to 7 cases per million people per year - some say it is starting to increase significantly as more antibiotics and anti-inflammatory drugs hit the market.

"The problem is that the drugs causing this syndrome are unbelievably common - things like ibuprofen, that people take every day," said Levine. "Something in the drug leads to a very inappropriate, wildly inflammatory immune response that causes the skin to die.

"It's completely unpredictable. Someone can be taking a drug safely for years, then all of a sudden have a horrible reaction to it. Those who get it are not highly allergic. This is just plain bad luck."

In the case of Samantha Grasham, the syndrome stopped her life cold, ending her athletic scholarship, her education, her life plans.

"I was always so active and involved - in sport and games. I loved college, and traveling," she said. "Now there is nothing - just these scars all over my body." Like many victims, she now battles chronic fatigue.

The former softball star at Desert View High School entered El Paso Community College in Texas last fall on a two-year athletic scholarship.

But in early January, during the first day of softball practice, she had an unexpected, one-time seizure that caused her to fall and black out.

After a series of tests back home in Tucson, Grasham was placed on an anti-seizure drug, Lamictal, by a neurologist, who told her she could return to school and go on with her life normally.

She did, but about 10 days after starting the drug, she noticed a small rash on her arm. Though she played first base in a softball game the next day, the rash by then had spread to her arms, legs, back and face.

With the rash worsening by the hour, now itching and burning, her ears and mouth starting to blister, Grasham went to urgent care in El Paso. A doctor there correctly diagnosed an allergic reaction to the drug she was taking but never told her to stop taking it - the first terrible mistake. After getting an allergy medicine, she was sent home.

Two days later, "my face was on fire," as Grasham describes it, and the burns were moving down her airway, interfering with her breathing. She went back to urgent care, where again no one told her to stop the drug. Instead they gave her another drug, prednisone, also linked to cases of Stevens-Johnson syndrome.

The next day, her whole body now blistering and burning, her face completely inflamed, Grasham's family made an emergency trip to El Paso.

"Oh my God, I couldn't even speak when I saw her. I couldn't even cry - I was so shocked," said her mother, Patricia Grasham. "Her mouth was one big blister; her face was all red and swollen. Her eyes were burning so bad we had to keep wet towels on them all the way home. The blisters were all over her mouth and down her throat. She was drooling, and in so much pain."

Driving straight to the emergency room at Tucson Medical Center, the family finally found out what had attacked their daughter. An emergency physician immediately diagnosed Stevens-Johnson syndrome and nailed Lamictal as the likely cause.

Grasham remembers many doctors and nurses coming into her room to see this highly unusual and bizarre eruption, many of them for the first time.

"Once you see the mucous membranes blistering, and you get a history of how this progressed in the patient, an emergency physician should know what they're looking at," said Dr. Ron Widman, chief of emergency services at TMC.

"But this is not very common, so yes, certainly, we want our young doctors to see it."

Although Grasham was finally off Lamictal, the syndrome kept worsening - possibly fueled by adding prednisone to the other drug. She was struggling to breathe as the burns marched down her respiratory tract.

Two days later, on March 5, with the severity of her burns reaching second degree, Grasham had to be moved to St. Mary's burn unit.

SJS patients who reach this stage of damage - when more than 35 percent of the body is blistered or burned - are diagnosed with toxic epidermal necrolysis, or TENS. At that point, the mortality rate is around 50 percent.

But after 18 days of intensive treatment - including extremely painful debriding of her burned skin, bandaging, infection control and special nursing care - Grasham finally left St. Mary's, alive but scarred. She has continued daily treatment as an outpatient.

"If only they had known what they were looking at in (El Paso) urgent care, I don't think this would have gotten so bad," her mother said.

But many doctors will never see a case of Stevens-Johnson syndrome, said Dr. Jeffrey Nelson, a plastic surgeon and burn specialist at St. Mary's burn unit.

Nelson - obviously familiar with it in his specialty - has treated about 10 to 12 cases during his nine years at St. Mary's, with antibiotics and anti-convulsants the most common trigger.

"It feels like I've seen a lot more cases in the last three years than in the three years before that, but I have no way of knowing if that is an accurate trend," he said. SJS is not a reportable disease.

But warnings about the SJS-drug link have been emerging more frequently in recent years. In 1997, the Food and Drug Administration posted a warning on the drug Grasham took, Lamictal, and the drug's maker, Glaxo Wellcome, added a boxed, boldface warning to the drug's label saying the drug has caused "severe, potentially life-threatening rash, including Stevens-Johnson syndrome and, rarely, toxic epidermal necrolysis."

The warning notes that the reaction is occurring in about one of every 1,000 adults taking the drug, but at dramatically higher rates in children - as high as one in 50.

"Lamictal is not approved for use in patients below the age of 16," the label now reads.

Today, with no scholarship, her dreams of higher educations shot, she is at a loss over her future.

"I just don't know. I need to find a job," she said softly. "This is such a nightmare."

http://www.fox11az.com/news/local/stories/KMSB_20040504_drugburn_dsbp.18be1123e.html

[gwenith, BSN, RN]

I am moving this thread to the burns forum as we are trying to increase the resources and stories on SJS.

[jnette, ASN, EMT-I]

How terribly tragic for this young woman.

And how incomprehensible that she was never advised (twice, no less!) to dc the Lamictal, especially after being dx'd. with an allergic reaction. I don't get it... :stone

[Nur_1996]

Wow, what a story, I learned something new today. I was'nt aware of this syndrome. What a terrible tragedy. Makes it scarey to even take any meds.

[psychomachia]

Here is a good summary of SJS: http://www.emedicine.com/emerg/topic555.htm

[2ndCareerRN]

And how incomprehensible that she was never advised (twice, no less!) to dc the Lamictal, especially after being dx'd. with an allergic reaction. I don't get it...

My personal belief is that she was "told" to d/c the meds, but it was never put in writing (document, document, document). Because of this she will have a civil case against the hospitals, clinics and providers she saw at the onset of the disorder. I would give you odds that she has a lawyer already.

I have seen this in 1 person, it is a devastating illness. This person will need much therapy and probably reconstructive surgery. It is a shame, but, given the rarity of the disease, I can see how it was missed at the first visit.

I still find it hard to believe she was not told to d/c the meds. That is as basic as breathing when a person has been dx with an allergic reaction to a drug.

bob

[gwenith, BSN, RN]

Thank-you for the resource Psychomachia -

As I said I am trying to put some resources together here in this forum on SJS and TENS.

Having nursed 7 cases it is my hope that we can make this a place where nurses can come and find some answers.

I put some more links on this thread.

https://allnurses.com/forums/showthread.php?t=62017

[Reabock]

We had one of these cases a few months ago, the cause seemed to be the OTC med Abreva for cold sores. I never saw this pt before she was transferred,the same evening, as I was too busy with my own pts down another wing, but I can still remember hearing her scream in pain. We heard later that she did die. I did hear the Docs on the phone and SJS was their thought at the time. Very scary!

[louloubell1]

I've seen this in one patient. It was indeed horrible.

[Hellllllo Nurse, BSN, RN]

A new, schleral contact lens has been developed, which has restored vision to some people blinded by Stevens-Johnson Syndrome, and people whose vision is distorted due to keratoconus.

http://www.bostonsight.org/giftofsight.htm

NEWS AND PUBLICATIONS

April 2, 2003

BOSTON SIGHT FOUNDATION RECEIVES GRANT TO EXPAND

AVAILABILITY OF SIGHT-RESTORING LENS

Grant from VISTAKON® to Train Eye Care Experts on its Boston Scleral Lens

Boston, MA., April 2, 2003 - Boston Foundation for Sight today announced that VISTAKON®, Division of Johnson & Johnson Vision Care, Inc., will provide a grant to establish and train a network of eye care professionals in the United States and abroad who can help restore functional sight to patients with severe corneal disease.

The grant will enable a $240,000 program to train Eye Care Professionals associated with academic eye care centers on the fitting technology of the Boston Scleral Lens, a specially designed contact lens device that provides a non-surgical means of restoring vision in eyes affected by many corneal disorders.

The Boston Scleral Lens (BSL) developed by the Boston Foundation for Sight is made from an advanced oxygen-permeable plastic that permits the eye tissues to breathe through the lens. Designed to rest entirely on the white tissue (sclera) of the eye, it arches over the damaged cornea creating a space that is filled with artificial tears. The effect is to create a smooth optical surface over the diseased cornea that replaces its distorted surface that improves vision, often dramatically. This oxygenated compartment of fluid also protects the extremely sensitive and fragile corneas suffering from severe ocular surface disease from exposure to air and the friction of blinking, both of which can cause severe, debilitating pain and extreme photosensitivity.

Dr. Perry Rosenthal, M.D., founder of Boston Foundation for Sight and assistant clinical professor of ophthalmology at Harvard Medical School, said there is a considerable need to increase the number of professionals who can help these patients and fit them with this life-altering contact lens. According to Rosenthal, scleral lenses had a checkered history and despite their unique advantages for eyes

-more-

with corneal disorders, they had been abandoned because of their propensity to cause serious and permanent complications.

By incorporating the elements of advanced polymer chemistry and the flexibility and precision of state-of-the-art non-rotationally symmetrical lathe technology driven by a program based on advanced mathematical spline functions, the Boston Foundation for

Sight has significantly advanced the successful use of gas-permeable scleral lenses. The Boston Scleral Lens incorporates unique radial channels in its bearing surface that facilitate fluid-tear exchange while excluding the intrusion of air bubbles. This has been critical to its success in managing some of the most devastating corneal diseases that had been previously refractory to all available treatment options.

"Patients from all over the world come to our Foundation desperate for relief from devastating corneal disorders," he said. "The vast majority of our patients are young and in the prime of life and have exhausted all available treatment options. Vistakon's considerable gift will make a significant impact in helping the Foundation build a network of professionals trained in providing these sight-restoring contact lens devices for the patients, many of whom had been given no hope of regaining functional vision or finding relief from the unremitting eye pain and disabling photosensitivity that relegated them to living in the dark, both figuratively and literally."

Dr. Rosenthal estimates that there are approximately 50,000 people in the U.S. who suffer from corneal diseases that can be helped by its Boston Scleral Lens when other modalities fail. Conditions such as keratoconus, Stevens-Johnson syndrome, anesthetic corneas, ocular cicatricial pemphigoid, disabling dry-eye disorders, distorted corneal transplants, corneal scars, eyelid abnormalities and other corneal disorders have all been successfully treated with the BSL in Rosenthal's Chestnut Hill, Massachusetts, clinic-currently the only site in the United States that can provide and fit the Boston Scleral Lens.

"For many of the tens of thousands of people in the United States blind from corneal disease, the Boston Scleral Lens, currently manufactured exclusively at the Chestnut Hill, Massachusetts facility, is the only option for regaining functional vision," said Phil Keefer, President, VISTAKON® Americas "Until now the Foundation's outreach to those in need has been limited to patients who are able to travel to the Boston clinic for the fitting process that can take up to two weeks. Although the BFS turns no one away because of their inability to pay (since most health insurers refuse to accept fitting this lens a covered service, the BFS provides free care for almost 50% of patients), traveling to its clinic is still a hardship for many. We are proud that our grant will help establish a network of satellite clinics in the U.S. and abroad to substantially increase the availability of the device by practitioners skilled in this technology."

The grant will help the Foundation prepare appropriate teaching materials and conduct intensive workshops to train eye care professionals on the technical details of fitting the BSL. Through the Foundation, the program will also offer a 6-week fellowship to selected tertiary eye-care centers abroad to help bring this sight-giving technology to patients around the world. In 2005, the Foundation expects to host its first annual symposium to provide practitioners with the opportunity to share experiences and advance the knowledge of caring for these patients.

-more-

About Dr. Perry Rosenthal

According to Dr. Rosenthal, "The mission of the Foundation is to provide our vision-restoring contact lenses to the hundreds of thousands of people worldwide who are visually disabled from corneal diseases and can benefit from our technology-regardless of the patient's ability to pay or where they live." Dr. Perry Rosenthal, president and founder of the Boston Foundation of Sight, assistant clinical professor of ophthalmology at Harvard Medical School and founder of the Massachusetts Eye and Ear Infirmary's Contact Lens Service, has developed a unique family of contact lenses that have rehabilitated the vision of hundreds of patients with corneal diseases, more than half of whom had no other option for recovering functional eyesight. Unlike conventional contact lenses, the quarter-size Boston Scleral Lens is the result of over 15 years of research that incorporates state-of-the-art polymer chemistry and a patented computer design/manufacturing program. Dr. Rosenthal's clinical team has achieved a success rate of over 80% in rehabilitating vision and eliminating the eye pain for people who suffer from the most painful of blinding eye diseases for which there had been no definitive treatment in the past.

About Johnson & Johnson Vision Care, Inc.

VISTAKON®, Division of Johnson & Johnson Vision Care, Inc., pioneered the concept of disposable contact lens wear in 1987 with the introduction of ACUVUE® Brand Contact Lenses. The ACUVUE® Brand family of lenses is the most prescribed by Eye Care Professionals worldwide today. VISTAKON®, headquartered in Jacksonville, Fla.,

###

ACUVUE® is a trademark of Johnson & Johnson Vision Care, Inc.

Perry Rosenthal, M.D.

Founder and President

1244 Boylston Street, Suite 202

Chestnut Hill, MA 02467-2115

tel [phone]

fax [phone]

patient appt. 617.735.8810

[email protected]

http://www.bostonsight.org

Board of Directors

Harry H. Birkenruth, Chair

Bonnie H. Bader

Nancy Comenitz

Harry T. Daniels

William A. Guthrie

Rabbi Donniel Hartman

Florence Koplow

Brian Levy, O.D.

Edwin Miller

Richard D. Muzzy

Thurman F. Naylor

Steven Nevins

Renée Rapaporte

Robert E. Remis

Hillary Salmons

Robert Shapiro

Cynthia A. Sheppard

Alan J. Strassman

Kazuo Tsubota, M.D.

James Vanecko

[redwinggirlie]

This was a patient of mine. Great attitude and great support system. It's a condition which warrants more attention.

[maureeno]

thank you for this and for the links

lamictal is being used for a mood stabilizer

and I am on the lookout for this rash I hope never to see