Saving Princess Amira
Following a ravaging row-house fire, a young child’s fight for her life leaves an indelible image on the members of the pediatric burn team who cared for her.
"As she grows and develops into that beautiful
young woman she’s going to be, hopefully
she’ll always take with her that she is who she is.
- Physician Assistant, Amy Woodcheke
Amira ambles down the stairs to her living room in East Baltimore, moving the best she can with limbs twisted and constricted by deep burns she suffered in a row-house fire two years earlier that claimed the lives of six members of her family. She was 3 then, a month shy of 6 now, and in most of the time between—569 days—she was at the Johns Hopkins Children’s Center and affiliated institutions fighting with doctors, nurses and therapists for a life that many thought was lost.
Typically, patients with burns over 80 percent of their body do not survive. With deep burns over 95 percent of her body, Amira is the patient pediatric burn team members mention first when asked about most challenging cases—she was the patient who was not supposed to live. Yet at the bottom of the stairs she turns her head and makes eye contact and smiles through scars suddenly non-existent, supplanted by her personality perhaps. She is animated and very much alive.
I’m Princess Amira,” she says. “I’m Princess Amira because I live in a castle, and I’m special natural.” Nearby, Chrissy Thomas, Amira’s mother, laughs: “She likes to play with words. Yes, she is special natural.”
You’d be hard-pressed to find any members of Amira’s care team who would argue that one. There had to be something special in this child to survive the burns she sustained and to undergo aggressive and often painful treatments and surgeries over weeks and months. They work equally hard for each patient, they say, but add that Amira’s fight attracted them, drew them in and perhaps pushed them even more to save her.
“She was very sick, and it was always in the back of my mind that she could die any day,” says physician assistant Amy Woodcheke. “But she had that spontaneity, that push. And if I hadn’t seen it in her, I don’t know if I would have pushed as hard.”
This is the story of Amira and that spirit, how it touched the members of her pediatric burn team. This is also the story of the medicine and science these clinicians brought to Amira, from her admission in the Emergency Department (ED) to her months-long stay in the Pediatric Intensive Care Unit (PICU) to some 30 surgeries in the OR—including a new skin-grafting technique in which Amira’s own skin cells were used to grow new skin.
“Without the cultured skin,” says pediatric plastic surgeon Rick Redett, “she would not stand a chance of surviving.” But she did, and here’s how.
In the early morning hours of May 22, 2007, Amira Williams came to Hopkins through the adult ED, where members of the pediatric trauma team, including Woodcheke, quickly assessed her. They were stunned by the extent of her burns. Then Woodcheke heard Amira’s voice, eerily through the inhaled smoke coming out of her mouth: “‘Help me,’ she cried out,” says Woodcheke. “‘Help me.’”
With no signs of trauma, Amira was rushed to the PICU where critical care specialist Jamie Schwartz and her team took over. Like Woodcheke, Schwartz was startled to hear Amira speak: “We’re used to taking care of children in circumstances so extreme they’re usually not talking to us. ‘Ow, ow, it hurts,’ she cried. ‘Ow, ow, it hurts.’”
Those words would be Amira’s last for a long time, though they would linger for Schwartz and Woodcheke during her lengthy hospitalization. Knowing her airway—like the rest of her body—would swiftly swell up in response to the burns, Amira was heavily sedated and intubated with a breathing tube, which was hooked up to a mechanical ventilator to help her breathe.
At the same time PICU staff searched for access for intravenous lines to provide the enormous amounts of salt-containing fluids Amira would need to replace what she lost, and to perfuse her organs and resuscitate her body. They worked to keep her warm, too—little skin meant a significant risk of hypothermia.
The team looked for signs of infection, too, as burns this severe automatically suppress the patient’s immune system and, with no skin barrier, make them susceptible to infection at the same time. All along they monitored Amira’s heart and vital signs while trying to get her kidneys to remove the escalating amount of toxins from the muscle and tissue breakdown in her body. “Amira’s body was just so swollen,” says Woodcheke, “and working so hard not to shut down.”
In ways, the fire Amira had initially survived had ignited another fire in her body that now threatened to kill her. Deep, high-percentage body burns, explains PICU Medical Director Ivor Berkowitz, set off a systemic inflammatory response that, while designed to protect the body, attacks it as well.
White blood cells become highly activated and stick to blood vessels in burned areas of the body to ward off impending infections. But in the same way these white blood cells target other, non-affected areas of the body, like the kidney, liver and lungs. Blood vessels in these organs become leaky, putting patients at risk of pulmonary edema, respiratory distress, and kidney and liver failure.
“Even though the target of these white blood cells is just the skin,” says Berkowitz, “you can get this multi-organ failure.”
Indeed, despite the PICU team’s best efforts, Amira was rapidly progressing to both respiratory and renal failure.
Knowing the rapid fluid shifts that occur in the acute period following burn injury were now relentlessly attacking Amira’s body, the PICU team constantly checked Amira’s albumin, calcium, glucose and other markers to direct her fluid replacements. “In cases like this there’s an enormous amount of effort and attention to detail,” says Berkowitz. “You’re not just managing the burn, but a very disturbed physiology.”
“We were getting ready for what was essentially going to be a long-term battle to keep her alive,” adds Schwartz.
Enroute to the PICU, Redett was about to join the battle. The pediatric plastic surgeon had heard about the fire, knew his patient’s mother was also burned and now at Johns Hopkins Bayview Medical Center, and knew that other family members had not survived. What he did not know was the extent of Amira’s burns.
“My initial impression was she may not survive,” says Redett. “She had lost 95 percent of her skin, almost her entire body down to muscle.”
Redett was flooded with thoughts of the child, her parents, as well as what he’d have to do in surgery, assuming she made it to the OR.
As much as Amira needed to be stabilized in the PICU, she also needed surgery as soon as possible to remove the dead skin—another source of infection—and to relieve her body of the enormous edema threatening her circulation. Normal skin is elastic and can tolerate such swelling, but deeply burnt skin, called eschar, is firm and more leathery than elastic.
So that very first night Redett was in the OR making long cuts through the eschar covering Amira’s arms and legs, relieving the pressure and restoring circulation in her hands and feet. What dead skin Redett removed he replaced with cadaveric skin, giving Amira a temporary covering for her body, which in effect was one open wound.
But getting Amira’s body covered was no easy chore. Redett had to stage this initial surgery through multiple operations over the course of the week to reduce the risk of blood loss, hypothermia and infection. All along it was touch and go for Amira – during one operation her heart stopped and had to be revived, and each time she returned to the PICU in even more critical condition. The operations were designed to save her life, but each surgery put another stress on her body.
“If we don’t do the escharotomies,” says Schwartz, “she’ll get infections, but if you do them they cause an even greater inflammatory response and make the lungs, heart and kidneys even sicker.”
Similarly, each treatment in the PICU posed another potentially life-threatening problem for Amira. She needed fluids but fluids raised the risks of respiratory and renal failure. Diuretics were given to clear out the lungs and increase urine flow, but diuretics can throw a vulnerable burn patient’s electrolytes out of whack.
Amira needed central lines placed for fluid resuscitation and transfusions, but each line placed increased the risk of blood-borne infections. Mechanical ventilation helps patients like Amira breathe, but mechanical ventilation may lead to a chronic lung disease, even fatal pneumonia. “It’s like two steps forward, three steps back, and some days it’s two steps forward and four steps back,” says Schwartz. “These burns really are the worst injuries we see.”
While Schwartz and the PICU team were working to keep Amira alive, clinicians at Johns Hopkins Bayview were treating Amira’s mother for burns to her arm. There, Arceneaux was building a rapport with the mother and helping her through the loss of her own mother and two brothers, as well as the possible loss of Amira.
“The main issue was whether Amira would live or not, and how the mother would handle sharing that information with her siblings,” says Arceneaux. “It was difficult for her to see Amira. There were multiple layers of family trauma.”
The mother and her family were also beginning to face the same emerging ethical issues facing the care team.
The question was not only how much could Amira take but how much should she have to take? Should heroic efforts be made given the high mortality in patients with burns over 80 percent of their body? Even if Amira survived, what would be her quality of life? Did she suffer a noxious injury that deprived her brain of oxygen? Would she suffer serious cognitive and developmental issues as a result? Would she be confined to a wheelchair for the remainder of her life? Would it be better to let a child like this go because she was likely to go, rather than undergo multiple and often painful surgeries and treatments?
“There’s always this balance between the burden of therapy, the pain and lack of efficacy, on the one hand, and the hope that what we’re doing is going to benefit the patient,” says Berkowitz. “But it’s not a one-time decision. The direction of therapy and the goals are constantly being re-evaluated in consultation with the family.”
For Woodcheke, the answer was a no-brainer. She saw Amira from the moment she arrived, and she heard a voice that filled her with hope. You’d fight for a heart or liver transplant patient, so why not fight for a severely burned patient? “What makes a burn patient different sometimes is the physical appearance,” says Woodcheke. “It is hard for people to understand what could be.”
At one team meeting, Arceneaux replied that quality of life varies from patient to patient based upon individual beliefs. Importantly, she added, “Amira is telling us she’s fighting.”
Redett agreed: “She had multiple chances to die and she didn’t. No one would have been surprised if she had.”
Schwartz saw and heard the fight in Amira, too, which pushed her to push ahead. Had there been signs of brain damage, she might have felt differently.
“In some ways you feel like you’re hurting her more because we have to do so much to her to get her better,” says Schwartz. “But you knew she was in there, that her brain was okay and that she was going to be able to play and blow bubbles and enjoy her mother if we could get her to the other side. We really felt so motivated to keep going.”
So did the mother. “I recall the heart-wrenching talk with her mom,” says Arceneaux. “She said, ‘I’m not afraid. You guys are going to save her.’”
Back at Hopkins Children’s, however, the pediatric burn team hardly felt Amira was out of the woods. Redett had completed the initial surgeries, including replacing Amira’s dead skin with cadaveric skin, and harvesting skin tissue from unburned areas of Amira’s scalp to help reconstruct her face—a technique he likes to use because the scalp matches the face in skin tone and, because he took only razor-thin layers of scalp, hair could still grow on Amira’s head.
But the more daunting challenge was how to provide permanent skin for the 95 percent of Amira’s body that was deeply burned. Cadaveric skin, which is usually rejected within 14 days, is only a temporary solution, and without a skin covering and soon, Amira would be even more vulnerable to life-threatening inflammation, infections, bleeding and hypothermia. With almost no healthy skin to harvest, there was only one option—using Amira’s own skin cells to grow new skin.
So, Redett took postage-stamp-size skin biopsies from Amira’s groin and sent them to a lab in Boston, Ma., where, over the course of about 10 days they would be co-cultured with mouse cells to form epidermal skin grafts about the size of playing cards.
Because these grafts are grown from a patient’s own skin cells, typically they are not rejected by the patient’s immune system. But because there’s no dermis in the cultured skin, it’s thinner than natural skin, which makes it more prone to contracting and a scarring.
While Redett waited for the new skin, Amira returned to the PICU where her critical condition continued to fluctuate, requiring even greater coordination of her care. PICU staff rounded with Redett and other surgeons in the morning, met among themselves in the afternoon, consulted with the myriad subspecialties as needed.
There were teams within the team of some 35 clinicians who cared for Amira, and someone needed to connect them all—Woodcheke’s position allowed her to take on the role. One issue was the ever-changing rotation of residents that occurs in a teaching environment. Almost daily new faces were facing a learning curve called Amira.
“It took a lot of time to make sure everyone knew the updates, what the labs were,” says Woodcheke.
At the same time, members of the team became increasingly invested in her outcome. In team meetings, psychologist Arceneaux reminded some staff of the bonds that can be easily forged with a patient like Amira, and the need to keep some boundaries to focus on the immense job at hand.
“We had never had a patient with 95 percent burns, and that was part of it,” says Arceneaux. “Not to say we don’t feel a genuine affection with other patients, but we particularly felt protective of Amira.”
And all along Amira kept on plugging along, surviving each surgery and each critical period afterward in the PICU. Then it happened—she started to get better.
“She slowly improved,” says Berkowitz. “Did it surprise me? Yes.”
Indeed, Amira was well enough to be weaned somewhat off her highly sedated state. A tracheotomy tube replaced her ventilated breathing tube, and a gastric tube, or G-tube, was put in so she could receive food to help her body grow and heal. Now, as Amira became more alert, Arceneaux’s focus switched to helping her manage the painful dressing changes and the limitations of being in an intensive care unit. With the team, Arceneaux helped set up a daily schedule for Amira, giving her choices to give her control.
“She wanted to be a normal 3-year-old and play,” says Arceneaux. “That was a good sign. It meant Amira was healing.”
Then the cultured skin grafts were delivered to Redett, who transplanted them, patch by patch, onto Amira. The grafts were completed in two 2 ½-hour stages to minimize blood loss and hypothermia. Amira, says Redett, took the new grafts “amazingly well.”
Things were looking up. With the skin grafts complete, Woodcheke says she and the team believed Amira had survived the burns, though they were very much aware of the continuing related threats to her body as it healed.
Also, there were still unknowns about her future quality of life, how whole Amira would be. But, for Woodcheke, those concerns were allayed when she saw the individuality that emerged as Amira came out of the induced coma: “Right away we could see this attitude.”
Child Life Specialist Rebecka Carlson saw it, too, when after four months in intensive care Amira turned 4 and was transferred to a regular room on the fourth floor. Seeing the extent of burns on Amira’s body, her initial reaction was, “Wow, this is going to be a long road.” Then, when she put her hand in Amira’s, she felt a squeeze. “There was this little personality that came out of her from the very beginning,” says Carlson. “She was fighting beyond the medications we were giving her.”
As a Child Life specialist, Carlson says her job is to help patients and families navigate the emotional and psycho-social dynamics of being sick and in the hospital, to be there for the patient and the family. But never had she worked with a patient with such burns, a family with such losses.
The biggest challenge was getting Amira through painful dressing changes. Staff provided medications as needed, but also employed distraction alternatives, which for Amira became Dora the Explorer. Carlson read books about Amira’s new hero, showed her videos, sung her songs. At times the treatment room was filled with a chorus of nurses singing along. Still, the dressing changes were Amira’s most anxious moments.
“She’d get upset, cry and kick her legs,” says Carlson. “The good news was she was regaining the ability to communicate.”
Working closely with Arceneaux, Carlson sought creative ways to help Amira re-engage with her environment, providing predictability in her day, and positive touches for a child who had very limited places where she could be touched.
The more Carlson interacted with Amira, the more feistiness she saw in Amira. She saw her impatience, too, especially when she tried to talk. Amira, who her mom says wore the pants in her family and always told her sister what to do, now couldn’t say a word because of her trach tube.
“When she wanted something she got very expressive,” says Carlson. “You could see this frustration.”
A playful, confident energy typical of a 4-year-old was coming out of Amira, too, a good sign for her team. Carlson recalls donning a pair of bunny ears, only to have Amira reach around and pull them off.
“She wanted them, and Amira wants what Amira wants,” says Carlson. “But it was incredible to see this critically ill child get to the point where she was regaining control of her situation.”
Woodcheke saw Amira’s emerging control, too, along with her sassiness, especially after she learned she could breathe without the trach tube: “You’d go into her room and she’d be swinging the trach tube around. ‘Amira, did you take that trach tube off?’ She’d reply, ‘Yeah, I was lonely.’ When we saw that we could tell not only was she surviving, she was thriving.”
So, the trach tube was removed and Amira could be heard again—and staff did indeed hear her. “She was very demanding, so we worked on manners and I told her it’s important to say ‘please.’ So she looked at me and said, ‘Well PLEASE get out of my room,’” recalls Arceneaux. “Well, she did use her manners.”
But then came the stark moments, says Arceneaux, when Amira first noticed the changes in her skin: “She just stared at her hands for awhile. I encouraged her to touch her hands: ‘This is your skin. It’s new skin, but it’s your skin.’”
Then there was the new face she saw in the mirror. “Her initial reaction was anger, but it was very brief,” says Arceneaux. “I don’t remember her exact words, but they were something like, ‘I’m still beautiful. I’m still a princess.’ She was saying ‘Okay, I look different, but you know what, I’m still a good person.’ Most 4-year-olds don’t have such a strong inner-awareness.”
In ways, despite her wounds—or perhaps through surviving them—the team could now see the wholeness of Amira. She was not completely well—but well enough to be transferred to Mt. Washington Pediatric Hospital for physical rehabilitation.
For Amira, leaving would not be easy. Being at Hopkins Children’s for so long, she had developed a deep dependency on staff. She was nervous, says Carlson, and to ease the transition the Child Life specialist would accompany Amira in the ambulance to Mt. Washington on November 19, almost six months to the day she had been admitted. Excited about going outside for the first time since the fire, Amira gasped with her first breath of cold air.
Yet she didn’t seem to notice the house fire they passed on the way. She was more concerned about the speed bumps, says Carlson. At Mt. Washington, she was calmed by the puffy white clouds above her bed and a new Elmo.
For the members of Amira’s team at Hopkins Children’s, her departure was bittersweet. Many felt even more protective. “You develop that bond,” says Arceneaux, “but then you realize you have to let them go, for their sake as well as yours.”
But as much as they knew they would miss Amira, they were now confident of a full recovery. “By the time she left for Mt. Washington,” says Woodcheke, “we all thought she was going to make it.”
Little did Woodcheke and the team know then, however, that Amira would soon be back, her life again in danger.
Doctors weren’t certain what triggered Amira’s respiratory distress and cardiac arrest at Mt. Washington less than a month later. Among the possibilities were bronchitis, a coincidental acquired infection, a respiratory virus, pneumonia, or perhaps simply the culmination of the continued stress of the burn injuries on her tiny body.
What was certain, a cardiac echocardiogram showed after she was brought back to Hopkins PICU, was that Amira was in profound heart failure. Inotropic drugs were administered intravenously to stimulate and strengthen the contractions in her heart, but Berkowitz knew they were just buying time.
Her only option appeared to be ECMO, or extra-corporeal membrane oxygenation, a heart and lung bypass system that takes over the functions of these organs, allowing them to heal. But ECMO posed life-threatening risks, too, including infection and stroke. Also, ECMO, designed to treat reversible diseases with a defined duration of one or two weeks, had never been used at Hopkins for a severely burned patient like Amira.
“She was deteriorating and, for lack of anything else, it looked like if we didn’t do this she would probably not survive,” says Berkowitz. Berkowitz met with Amira’s mom, who agreed to the path of treatment. Meanwhile, members of Amira’s team were stunned to see her on ECMO.
“That was hard. I was devastated, very angry,” says Carlson. “If she was going to die, why did she have to go through all this? I thought I was saying goodbye.”
So did Arceneaux, who on Amira’s 10th day on ECMO saw her body still, the bypass machine silent: “My God, I thought she had passed.”
But then the PICU fellow told Arceneaux that Amira had been heavily sedated so that they could take her off ECMO. “I went in and said ‘Amira, it’s Dr. Lisa.’ She immediately opened her eyes and looked at me and held my hand. She was fine.”
But Amira wasn’t fine. Coming off ECMO she had suffered kidney failure and had to undergo peritoneal dialysis from late December until early March 2008. She survived that, too, and was transferred to the floor again until she was discharged to the Kennedy Krieger Institute for physical rehabilitation. She finally went home on December 10, 2008, but would continue outpatient physical rehab for another six months. How did she survive it all?
“Clearly, she herself was one tough patient with an enormous amount of resilience,” concludes Berkowitz. “I don’t think you can exclude the will to survive.”
“This is a miracle baby right here,” says Thomas. “She amazed me.”Redett would be among the first to acknowledge the miraculous determination of Amira, but credits “good nursing care” and a persistent multi-disciplinary burn team for saving her. “It was a tour de force,” he says. “It’s pretty labor intensive to get a child through injuries like this.”
Members of the team say they may have given Amira her life back, but she gave them something, too.
“When we face the next patient with a disease process that is not survivable, or the next high-percentage body burn, Amira will give us more hope and make us push harder,” says Schwartz. “For sure, none of us will ever forget Amira.”
In June 2009 Amira began, for the first time, attending public school in Baltimore. Last August she turned 6. She still faces significant and potentially life-long issues related to her injuries and appearance, say team members, but they have no doubt that her acceptance and confidence will carry her, and that she’ll not only survive, but surprise the world.
“I have no doubt that Amira is going to do amazing and wonderful things with her life,” concludes Carlson. “Whether she becomes a doctor, a social worker, a teacher, an artist—she will continue to have a huge impact on everyone around her.” Adds Woodcheke, “She’ll keep on plugging and as she grows and develops into that beautiful young woman she’s going to be, hopefully she’ll always take with her that she is who she is. She’s special.”
Getting Physical with Burns
As deep burns heal, scarred skin gets tighter and tighter, restricting patients’ range of motion and, in some cases, resulting in contractures that can only be relieved through surgery. Being severely burned and chronically ill in the hospital for a long time can also result in brittle bones because of the skin’s failure to make adequate vitamin D. The condition, called osteoporosis, is a disease of aging, but it resulted in a fractured femur for Amira at the age of 5.
The people at Hopkins Children’s who help patients like Amira counter these other consequences of burns are physical and occupational therapists like Kerry Peterson and Francesca Crupi. Through daily stretching and range-of-motion exercises, and through working with patients on transfers from bed to wheelchair or chair, they aim to restore patients’ ambulation and mobility to where it was before they were burned. But the regimen is difficult and not without pain.
“We have to do certain things like prolonged stretching to get their arms and legs moving fully and to prevent contractures,” says Peterson. “This initially causes more pain, but we know if they don’t do this now, they’re going to be ten times worse later on.”
Incorporating coping strategies, games and play into the exercises helps cut through patients’ resistance. The therapists stress that they try to push patients but not overwhelm them. Overdo the exercises and healing skin may breakdown and cause ulcers.
It’s all about building trust, says Peterson: “We tell them first what’s going to happen and why we’re there – not to cause pain but to make them whole again.”
To discuss a case or refer a patient call +1.443.287.6499.