The Leader-Telegram
Eau Claire, WI
April 6, 2008
Halting the operation on the 11-year-old boy barely clinging to life went against everything Dr. Marjorie Arca believed in. During her five years as a pediatric surgeon at Children's Hospital of Wisconsin in Wauwatosa and three years before that at an Ohio hospital, Arca had operated on plenty of patients with unusual, serious, challenging diseases. Arca always did whatever was necessary to save her patients' lives. That's what doctors do.
But this case was different. When Joshua Holm of Eau Claire arrived at Children's Hospital at the end of January, doctors didn't know what was wrong with him. He was subsequently diagnosed with undifferentiated embryonal sarcoma of the liver, an extremely rare, highly aggressive cancer that occurs most often in children. The diagnosis was a death sentence of sorts. Average life expectancy after diagnosis typically is about two months, and only a handful of people have become cancer free.
Josh's case was problematic even for this difficult disease. The tumor surrounded most of the left side and part of the right side of his liver. The rapidly growing cancer had spread beyond the liver and filled his abdomen, surrounding major blood vessels, stomach and other organs.
During the previous month, Josh's weight had ballooned from 101 to 162 pounds. He gained an improbable 11 pounds in just one day despite ingesting no food during that time. His legs swelled to several times their normal size, making his toes appear like tiny spikes protruding from club feet. His ankles disappeared as his body retained an increasing amount of fluid.
To accommodate Josh's growing size, his grandmother Judy Holm bought extra-large men's clothing for him. Still, the socks had to be cut to fit over the boy's swollen feet. "I remember one day I peeked under the blanket to look at his legs and thought, 'My God, that's just not right,' " Judy said, her voice wavering as tears streamed down her cheeks. "I'd never seen anything like it. I knew it was Josh, but it sure didn't look like him."
Initially, doctors had difficulty diagnosing Josh's condition. Once they pinpointed the disease, doctors treated the tumor with chemotherapy, but that effort failed to curb the cancer's growth, and Josh's condition continued to deteriorate.
On Feb. 27, a team of physicians overseeing Josh's case presented his parents, Pete and Darla Holm, with a seemingly hopeless choice: Let Josh die peacefully during the next several days or perform an exceedingly risky operation that would almost certainly kill him.
The Holms opted for the surgery. At 8 a.m. Feb. 29, Arca began the procedure. She knew it would be dicey.
The enormous tumor and the surrounding gelatinous, cancerous goo filled much of Josh's torso from his chest to his waist. The tumor had collapsed one of Josh's lungs and part of the other, forcing him to use a machine to breathe. It also pushed his stomach and other organs down near his pelvis, squeezing them together while stretching his esophagus. Eating had become virtually impossible.
Complicating the surgery was the fact that the tumor was intertwined with blood vessels that fed the cancer. Removing the tumor amid the myriad arteries and veins without damaging them and causing Josh to bleed to death would be like navigating a minefield. During a much simpler biopsy procedure Arca had performed several weeks earlier, Josh's tumor had bled profusely, requiring a blood transfusion.
Now, minutes into the operation to remove the tumor, Arca's fears were realized. She cut into Josh's lower abdomen where the blood vessels would be least numerous. But the boy began to bleed anyway. And the cancer was everywhere.
"I've never seen anything like it," Arca thought as she gazed at Josh's tumor. "It's absolutely huge."
Arca and attending physicians drained more than a liter of the cancerous liquid from the lower abdomen and pumped blood into him, replacing what was lost to his bleeding during the procedure. She had planned to siphon cancer from more of Josh's torso, but now she wasn't sure that was possible.
"I figured we might be done at that point," Arca said. "I thought for sure he was going to bleed out, and I didn't know if I could continue."
On Jan. 15, the usually energetic Josh stayed home from school after complaining of fatigue. The youngster rarely missed school, in large part because he enjoys the company of his Crestview Academy classmates so much that he fights with his parents to attend class, even if he isn't feeling well.
This time Josh seemed unusually tired and "looked a little green," his mother said. Still, his parents figured he had the flu bug and would bounce back in no time.
Instead, Josh felt increasingly worse, and after several days his parents took him to Sacred Heart Hospital. Tests showed he was anemic, so doctors prescribed extra iron.
However, Josh's condition continued to worsen, and several days later his parents noticed his stomach had begun to swell noticeably. Worried, they took him to the emergency room where tests led doctors to believe the boy was suffering from a pancreatic condition. The next day, Josh was transferred to Marshfield Clinic in Marshfield for more tests. That night, Darla, at home with her 9-year-old son, Jamie, received a phone call from Pete telling her she needed to come to Marshfield right away.
The news was grim. Doctors had discovered a huge tumor on Josh's liver that spread to the surrounding area. They were uncertain exactly what type of cancer the boy had and informed Darla and Pete that they didn't have the expertise to treat it.
The couple tried to get their son transferred to St. Jude Children's Research Hospital in Memphis, Tenn., but were unable to do so. Medical personnel recommended the Holms seek medical care for Josh at Children's Hospital of Wisconsin in Wauwatosa, and they decided to do so despite misgivings about the Milwaukee area.
"We were looking for answers, but we didn't have any," Darla said. "We were scared to death."
When it comes to serious diseases, Children's Hospital of Wisconsin physicians have just about seen them all.
The Wauwatosa facility has gained renown for its successful treatment of a variety of unusual medical cases, including a much-publicized case in 2004 in which doctors devised an innovative procedure to save the life of then-15-year-old Jenna Giese of Fond du Lac who was admitted for a rabies infection after being bitten by a bat. Before that case, rabies had been considered fatal in unvaccinated patients such as Giese.
Still, Josh's case perplexed Children's Hospital physicians. Upon his arrival in late January, he underwent a battery of tests and a biopsy. But doctors still couldn't determine the exact type of liver cancer Josh had.
"The uncertainty of not knowing what Josh had and watching him get worse was awful," Darla said.
Finally, the family received the grim news.
Any cancer is bad. Because of the importance of the liver in regulating a number of important bodily functions, cancer of that organ is especially problematic. And on the roulette wheel of diseases, undifferentiated embryonal sarcoma of the liver is about as unlucky as it gets.
According to medical journals, during the past three decades there have been only about 150 documented cases of the disease worldwide, and Josh is believed to be among a handful of people in the United States with the affliction. The odds of his contracting the condition are somewhere around 1 in 50 million.
Doctors at Children's Hospital hadn't dealt with the illness. In fact, there is so little information about how to treat that specific form of liver cancer that Children's Hospital doctors were charting their own treatment course for Josh.
Anne Warwick, assistant professor of pediatric oncology, and other Children's Hospital doctors searched for clinical studies detailing successful treatment of the disease, but most of them included only a couple of individuals instead of the usual larger numbers.
"We don't have enough data to determine the best course of treatment," Arca said. "It's just not available."
The size of Josh's tumor -- and its rapidly increasing size -- added to treatment difficulties. By the time Josh arrived at Children's Hospital, the fast-growing mass already caused his stomach to bulge abnormally. As the mass grew, it not only endangered Josh's organs but squeezed his veins, restricting blood flow to his limbs. Doctors knew time was running out.
The team of doctors treating Josh arrived at a couple of options. They could surgically remove as much of the tumor as possible. But the process would be extremely risky, and they figured Josh most likely would bleed to death during the operation.
Instead, they recommended chemotherapy. The chemical cocktails that target cancer cells worked well initially, and Josh felt slightly better for a short time. But the tumor continued to grow, and Josh's condition worsened again. He was placed on a ventilator after his lungs became too compressed by the tumor to supply his body with oxygen and expel carbon dioxide. Josh's heart rate raced, pounding along at 170 and 180 beats per minute, and his kidneys' function waned as the tumor compressed them.
The growing tumor caused another problem for Josh: extreme discomfort. The mass was crushing his organs. He was unable to eat or drink, and when he tried he vomited repeatedly.
The healthy, energetic boy who had enjoyed playing video games and socializing with friends had been replaced by an immobile child with a badly swollen stomach and legs who slept much of the day, struggling to breathe.
"It was so hard to watch," Pete said of his son's difficulties. "Josh was slipping away."
Pete and Josh Holm have always been close. They like to toss a football around. They enjoy watching the "Spongebob" TV show or squaring off in video game competitions. Most of all, they have an affinity for hanging out together.
That bond grew closer during their time at Children's Hospital. Wherever Josh went, his father was by his side. In fact, Pete has remained at the hospital since Josh arrived, leaving his job at the Midwest Labor employment agency in Eau Claire. Darla, Jamie and other relatives and friends visit often, but Pete has been the constant in Josh's life during his ordeal.
"Somebody had to be here 24 hours a day," Pete said. "And as sick and emotional as he was, it had to be the same person, so he had that comfort level. On the first day here I told him I'm going to be with him until we come home."
He provided comfort when Josh underwent one painful test and procedure after another. He pushed the pain medicine button intended to numb the agony when Josh was too weak to do it himself. He reassured Josh when things went wrong. He spoke with his son even though the boy couldn't talk back because of the ventilator he needed to keep breathing. He calmed Josh when his son sobbed with frustration. He laughed with his son.
And he was there to cry.
"There have been plenty of tears," Pete said.
Darla has shed her share of tears too. She cries each time she makes the four-hour return trip home from the hospital with Jamie as they try to maintain their lives in Eau Claire while wishing they could remain at Josh's side.
"I think about Josh all the time, how I wish I could be with him and help him somehow," she said.
The charismatic Jamie worries about his brother too. And Josh's illness has made him ponder his own mortality.
A few weeks ago, while home with the flu bug, Jamie asked his mother if he had contracted cancer too. He continued to fret despite Darla's assurances that he would be OK.
The Holms aren't strangers to medical difficulties. Nine years ago, Darla gave birth to Jamie, who was born six weeks premature and required extensive medical care. Just three months later, Darla was diagnosed with stage four cancer of her milk ducts. The cancer had spread to her lymph system, and the prognosis wasn't good.
But Darla was a fighter. For two years, she underwent chemotherapy treatments before the disease was knocked from her body. She had beaten the odds.
When Josh was diagnosed, the Holms couldn't help but wonder why cancer had revisited the family. Hadn't they suffered enough?
But in a strange way, Darla's illness has provided the family with a ray of light amid the darkness. If she could beat a tough form of cancer so could Josh, they reasoned.
"We know the odds aren't in his favor," Pete said. "But what happened with Darla does give us hope."
The initial chemo treatment for Josh's cancer had failed, but doctors kept searching for ways to curb its growth.
They continued to consult with physicians at medial facilities elsewhere, such as Duke University Medical Center in Durham, N.C., and St. Jude Children's Research Hospital, and decided on a process known as chemoembolism in which arterial blood flow to tumors is blocked, cutting off its nutrient supply. Chemotherapy drugs are then pumped into the arteries, allowing for a more specific targeting of tumor cells.
The portion of the tumor on the right side of Josh's liver was a prime candidate for the process because of the many arteries there. Tests showed the process has shrunk cancer cells in that area.
But the treatment wasn't feasible on Josh's left side because of a lack of blood vessels. That part of the tumor, which comprised the majority of the mass, continued to grow, and Josh's condition deteriorated.
"It was awful," Darla said of watching helplessly as her son moved closer toward death. "The doctors were trying so hard, but they were running out of options."
Some of Josh's fifth-grade classmates at Crestview and their parents journeyed to Children's Hospital to visit him. The visitors boosted Josh's spirits and those of his family, but on most occasions he was too sick for visitors.
During one afternoon when Josh felt particularly weak, friends and family extended their hands to his bed and prayed for him to get well again.
"At that point we felt like prayer might be our only hope," Judy, Josh's grandmother, said.
By Feb. 27, there weren't many options left. Josh's cancer-ridden body had swollen to 160 pounds as his stomach protruded grotesquely. Even with the help of a machine, Josh labored to breathe.
Doctors met with the Holm family that day to give them the bad news. Josh likely would die within a few days without surgery. So far they had avoided surgery because it was deemed too risky. Now Pete and Darla wondered whether it represented the last opportunity for their son's survival.
Michael Kelly, a Children's Hospital pediatric oncologist, got involved with Josh's case. Kelly didn't mince words. He had pushed for a more aggressive chemotherapy regimen for Josh. Now he urged the Holms to opt for surgery.
Pete and Darla asked whether they could delay the decision until Monday, but Kelly told them if they waited that long, Josh would die. However, Kelly and others doctors were blunt about the slim chance that the surgery would save Josh's life. The best-case scenario was he would survive the procedure, buying perhaps as much as two weeks of life.
"And that was a pie-in-the sky goal," Arca said. "We just hoped to get him out of the surgery alive, to buy him a little more time so his family could say their final goodbyes."
The gravity of the situation hit the Holm family like a heavy weight on their hearts.
"When they told us he's not leaving this hospital, that he's going to die here no matter what you do, that was the very bottom," a teary-eyed Pete recalled.
The couple debated whether to put their son through another painful procedure that was unlikely to save his life. Unexpectedly, Jamie helped them reach a decision.
"You need to do it," Jamie told his parents. "Josh would want us to."
They agreed. The surgery was scheduled for Feb. 29.
Like the other doctors involved with Josh's case, Arca had her doubts about whether the surgery would work.
"I was pretty fatalistic about it," she recalled. "I said maybe we could take the breathing tube out and have him talk to (his family) one last time. I just didn't think we could do anything to make this tumor any better."
Despite her doubts, Arca felt a duty to try to save Josh's life. Fearful of the delicate dangerous procedure, she sought inspiration at the hospital chapel before the surgery.
As she scrubs up before each surgery, Arca asks God to guide her hands. This time, however, she wanted more guidance.
"I figured this warranted a longer conversation than my usual two minutes. So I went to (God's) house. I told him I really needed his help with this one," she said.
Meanwhile, the Holms were saying prayers of their own. For six weeks, they had seen Josh's condition go steadily downhill. Each attempt to improve his situation had failed. Now he faced his last chance to live.
"We knew in our minds he probably wouldn't make it," Judy said. "But in our hearts we were saying, 'Yes! Yes! You've got to live!' "
About an hour into the surgery, the Holms received a phone call informing them that Arca didn't know whether she could continue the procedure. Despondent, they immediately left the pancake house where they had gone for breakfast and returned to the hospital, fearing the worst. On the drive back they prayed for Arca to forge ahead with the surgery.
When they arrived at the hospital, they received another call. This time they were told Arca had continued with the procedure and that some of the cancer had been successfully removed.
"At that point we really didn't know what was going on," Pete said. "We were on pins and needles. All we could do was wait and hope for the best."
Meanwhile, Arca and her team continued to remove the tumor, bit by bit. Arca's surgical strategy wasn't textbook. Instead of making one large incision and removing as much of the tumor as possible at once, she would make numerous smaller cuts, remove smaller portions of cancerous material, then stitch the incisions in an effort to reduce Josh's chances of bleeding to death.
Cut, delicately remove tumorous material, stitch. Then repeat. And hope to keep bleeding to a minimum.
Even with the small incisions, initial heavy bleeding prompted Arca to consider stopping the operation. She didn't want Josh to die on the operating table.
But Arca had recruited a secret weapon for the occasion. She had requested a specific anesthesiologist for the procedure. He was a parent, and she knew he would push her to continue the procedure when it became difficult.
"He helped me think about it not only like a doctor but also like a parent," Arca said. "All of us in that room were committed to doing whatever we could to keep Josh alive."
Besides the possibility of Josh bleeding out, the sheer amount of cancer to be removed proved daunting. The tumor, a mix of soft tissue and a softer, cancerous gel, was huge. All told the surgical team removed about 24 pounds of cancerous material from Josh's body.
Despite challenges and her initial doubts, the surgery worked better than Arca had anticipated. She was able to remove nearly all the tumor on Josh's left side, and he lost relatively little blood during the process.
"As the surgery progressed I was more emboldened because (Josh) wasn't bleeding out," Arca said. "So I just kept going and hoping for the best."
At 1:30 p.m., five and a half hours after the operation began, Arca walked into the waiting room to meet with the Holms, a smile lighting her face. The surgery had been a success. Arca and Josh had beaten the odds. The boy was alive.
A combination of surgical skill, determination and maybe just plain luck had pulled Josh through a surgery his doctors didn't think he would survive. As he visited with his family after the procedure, Josh proudly showed off his much-smaller stomach, its surface lined by a fresh 13-inch scar.
As she had done weeks before the surgery, Judy peeked under the bedcovers to check on her grandson's legs. She could barely believe they were back to their normal size, and she fell to the floor crying with gratification.
But Josh wasn't out of the woods yet. Not by a long shot.
With the removal of the tumor and subsequent fluid, Josh's weight plummeted to less than 90 pounds. The procedure and his illness had left him extremely weak. He was unable to perform even simple tasks, like sitting, without help.
Bit by bit, day by day, with the aid of nurses, therapists and other medical professionals Josh began to rebound. There was simple therapy like trying to squeeze a small piece of foam between his fingers to regain coordination and strength. Then, as he progressed, he was able to sit up, first with help, then by himself. Every gain, no matter how small, was cause for celebration.
Throughout the process, Josh has continued to exhibit a fighting, stubborn spirit. When doctors asked him to do an exercise 10 times, he would do 20. When they wanted him to take 15 steps on the first day he walked with the assistance of a walker, Josh shook his head no and quietly said, "I'm going to do more." He ended up pacing off 61 before stopping, exhausted.
There were other milestones. Days after the surgery, Josh was able to eat part of a Popsicle. It was the first time since January he had been able to keep food down without vomiting.
Josh spent part of that same day with several friends. They talked, played video games and just hung out. That night, Josh slept with a smile on his face.
"It's the first time in a long time I'd seen that smile," an emotional Pete said.
Throughout Josh's illness and recovery, Pete has been amazed at his son's resiliency.
"He's doing things nobody expected he'd be able to do," Pete said, his pride in Josh evident in his voice. "He's bound and determined that no matter what, he's going to beat this."
First one tepid, shaky step, then another, then another.
Josh spent part of a recent afternoon struggling to walk again. Relearning that task has been perhaps the most challenging aspect of his recovery. He can walk short distances with a walker, or holding his father's hand. But he's unable to amble along on his own for long.
"He gets tired so fast," Pete said.
Shortly after his surgery, Josh began another round of chemo treatments. Last weekend, tests showed that while the tumor on his right side has shrunk, the cancer on his left side has started growing again. So doctors started him on another chemo regimen.
Josh endured the first couple of days relatively well, but as the week wore on the chemo took its toll. On Friday, he came home for the first time since January so he can attend a fundraiser today arranged to help his family pay medical bills.
Chemo-induced nausea and vomiting nearly derailed the trip. Pete had to stop during the drive back to Eau Claire as Josh was overcome with wave after wave of sickness.
As Pete's white truck pulled into the driveway, Darla scurried outside, opened the truck door and embraced her son. A moment later, Josh took a few uncertain steps from the truck to a wheelchair.
Friends and relatives greeted Josh inside the Holms' house. He smiled a couple of times and chatted softly on occasion, but mostly he sat quietly, overwhelmed by nausea and eager for bed.
"He's so, so tired," Darla said. "But it's good to have him home."
Josh's doctors express amazement that he's survived this long, that he survived his Feb. 29 surgery at all. But, when pressed, they said they believe his cancer will kill Josh.
The Holms know that's a distinct possibility. But they also know that their son already has beaten the odds. And they know that, nine years ago, Darla beat the odds too.
They credit Josh's unlikely survival so far to a combination of the efforts and the skills of Children's Hospital physicians, the assistance of friends and their unwavering faith in God.
"There have been a lot of tough times, and there will be tough times ahead," Darla said. "But we have faith that things will turn out.
"All you can do is take it one day at a time and hope for the best. You've just got to keep believing."
Emerson can be reached at 830-5911, 800-236-7077 or julian.emerson@ecpc.com.
