Upstate Health, fall 2020

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Meet the surgeon who never gave up One way to control Crohn’s Results of tick testing Serving on the pandemic front lines Docs who rock

Thriving with sickle cell

From the experts at

Fall 2020

ICYMI IN CASE YOU MISSED IT

What’s up at Upstate and umbilical cord after childbirth.

Cord blood that is not donated is discarded as medical waste. Cord blood is a rich source of the hematopoietic stem cells that have the potential of being used in the treatment of dozens of diseases, like blood cancers and bone marrow diseases such as sickle cell disease.

Upstate is a COVID-19 testing laboratory for a large part of the state’s school population, including K-12 pupils in Onondaga County as well as college students throughout the State University of New York’s 64-campus system and other campuses.

The lab tests include wastewater and pooled saliva samples. Upstate is partnering with Quadrant Biosciences on the “spit tests,” and SUNY recently bought five testing machines for Upstate, allowing the lab to test up to 120,000 samples a week.

With the recent addition of five testing machines from SUNY, Upstate has further enhanced its lab capacity to perform rapid tests for COVID-19.

Researchers from Upstate and Quadrant — a Start Up NY company based on the Upstate campus in Syracuse — received emergency use authorization from the U.S. Food and Drug Administration for a saliva-based diagnostic test they developed called Clarifi COVID-19.

The pooled tests allow from 10 to 25 people to be tested at a time, using self-administered saliva swabs. A negative test means all people in that pool are presumably virus-free. If the pool comes back positive, each individual sample can be tested immediately to determine who carries the virus. The quick turnaround time is crucial to tracing the virus’s spread.

Testing samples of wastewater provides an early warning of the virus’s presence in a town, a neighborhood or even a college dormitory, as Syracuse University has done, and helps determine whether individual testing of a population is needed.

Upstate partners with Onondaga County to offer free, rapid testing to K-12 students or school employees in the county and has sent vans to offer drive-through testing at various sites in Central New York.

SUNY Chancellor Jim Malatras visited the Upstate campus twice to see the testing procedures firsthand, and the SUNY Board of Trustees has commended Upstate and interim President Mantosh Dewan, MD, for their “exceptional leadership” in combating the pandemic, including clinical trials for vaccines and treatments. l

St. Joseph’s Hospital Health Center joins Crouse Hospital and Upstate Community Hospital in allowing new parents to donate their newborn baby’s umbilical cord blood to the Upstate Cord Blood Bank at Upstate Medical University.

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Umbilical cord blood is blood that remains in the placenta U P STAT E H E A LT H l fall 2020 l upstate.edu

PHOTO BY RICHARD WHELSKY

Matthew Elkins, MD, PhD, medical director of the Upstate Cord Blood Bank, says, “through their generous donations of their child’s cord blood, they are helping others in need, either through transplantation or further medical research.”

The bank collects, tests, processes, stores and distributes umbilical cord blood donated by families throughout Central and Northern New York to be used by those in need of lifesaving medical treatments and for medical research. l

More than 200 iPads were installed around Upstate University Hospital and Upstate Community Hospital to help patients and families stay connected during the COVID-19 pandemic. Like all hospitals in New York, Upstate had suspended visits for health and safety reasons.

The iPads also were used by medical staff as a safe way to check in with patients from outside the patient’s room, thereby conserving the hospital’s supply of personal protective equipment.

The iPads were purchased through donations to the Upstate Foundation’s Virtual Visitation Fund for Patients, with the largest gift — $50,000 — coming from the technology company, Cisco. l

Upstate has expanded the size, scope and coverage area of the neurosurgery department with the addition of three new surgeons.

Harish Babu, MD, PhD, specializes in epilepsy and skull base neurosurgery. Timothy Beutler, MD, is Upstate’s first neurosurgeon dedicated to critical care neurosurgery.

And Ali Hazama, MD, will focus on spine and minimally invasive spine surgery. In addition, neurologist Ruham Nasany, MD, joins Upstate to lead a new program in neuro-oncology. u For more news, visit upstate.edu/news

Health C O N T E N T S UPSTATE

fall 2020 PATIENT CARE Surviving a massive aortic dissection

page 4

ON THE COVER Michael Rodriguez, 6, of North Syracuse, one of the kids with sickle cell disease at a family event at Upstate.

PHOTO BY ROBERT MESCAVAGE SEE STORY, PAGE 20.

Need a referral?

Contact Upstate Connect at 315-464-8668 or 800-464-8668, day or night, for appointments or referrals to the health care providers on these pages or anywhere at Upstate or for questions on any health topic.

Science Is Art Is Science Microscopic image takes first place

UPSTATE HEALTH ExEcutIvE EDItoR

Leah caldwell Assistant vice President, Marketing & university communications

EDItoR-In-chIEf

Amber Smith 315-802-9152 or smithamb@upstate.edu

wRItERS

DESIgnER

In Case You Missed It Mantosh Dewan, MD, left, interim Upstate president, greets SUNY Chancellor Jim Malatras on a visit to Upstate’s main campus in Syracuse page 2

Jim howe charles Mcchesney Emily Kulkus Amber Smith Susan Keeter

FROM OUR EXPERTS

He got a new kidney during the pandemic page 6 A fecal transplant provided relief

page 8

back cover

IN OUR COMMUNITY

Scientist predicts disease trends through studying ticks page 11

Visit us online at www.upstate.edu For corrections, suggestions and submissions, contact Amber Smith at 315-802-9152 or smithamb@upstate.edu

ADDITIONAL COPIES: 315-464-4836

Upstate Health offices are located at 250 Harrison St., Syracuse, NY 13202

Upstate Health magazine is a community outreach service of Upstate Medical University in Syracuse, New York. Upstate is an academic medical center with four colleges (Medicine, Nursing, Health Professions and Graduate Studies); a robust research enterprise and an extensive clinical health care system that includes Upstate University Hospital, Upstate Community Hospital, the Upstate Cancer Center and the Upstate Golisano Children’s Hospital. Part of the State University of New York, Upstate is Onondaga County’s largest employer.

How sepsis is similar to COVID-19 page 12 She served on the front lines of the pandemic Investigating a rare disorder in the Dominican Republic page 14

IN OUR LEISURE Banding together

page 22

page 16

PHOTO BY N. SCOTT TRIMBLE/SYRACUSE MEDIA GROUP

Patients needed companionship; medical students stepped up

page 17

In their words: Mother and daughter share their health crises

page 18

Kids with sickle cell disease – and their families – learn to thrive

page 20

upstate.edu l fall2020 l U P STAT E H E A LT H

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PATI ENT CARE

Kim Gutfleish Sklow, top row center, and cardiac surgeon G. Randall Green, MD, top right, on a video conference reunion with Sklow’s family and the cardiac surgical team one year after her near-fatal aortic dissection.

PROVIDED IMAGE

Miraculous save A main artery ruptured. Her heart stopped. She credits her survival to a ‘wonderful, supportive’ staff and a surgeon who wouldn’t give up

BY AMBER SMITH

or 12 hours, the chief of cardiac surgery worked

F

to repair an aortic dissection that threatened the

life of his patient, a middle-aged teacher brought

by helicopter to Upstate University Hospital.

Most people don’t survive a rupture of the main artery in their body. It poses one of the most challenging cases for even the most experienced surgeon. But this patient would survive.

The woman had a tear along the length of her aorta, from where the vessel exits the heart all the way to her pelvis, a much greater area in need of repair than for most aortic dissections. Her tissue was thin and uncooperative. Blood collected in her chest. A team lead by G. Randall Green, MD, surrounded her in the operating room, working diligently.

Finally, shortly before dawn, Green completed the repair.

That’s when the woman’s heart stopped. Using medications and chest compressions, Green and the team brought her back. Her heart stopped again. And again, they brought her back. Her blood was not clotting the way it should. She had lost a lot of blood. Her heart rhythm was abnormal. It did not seem like she would survive.

“There was nothing more that we could possibly do,” Green recalls of Kim Gutfleish Sklow.

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U P STAT E H E A LT H l fall 2020 l upstate.edu

Her family was in the waiting room, having hurriedly traveled hundreds of miles. Green spoke to them, explaining that he wanted them to be able to see her, but he wasn’t sure Sklow would survive from the operating room to the intensive care unit.

After the surgery, in which much of her aorta was replaced by synthetic fabric tubing, inflammation was so severe that Green could not fit her heart back into her chest. So her heart rested atop her chest. Staff from the operating room carefully covered Sklow with sterile sheets. They gave her more medications to help her blood clot.

She remained unconscious and connected to multiple machines.

Green kept close watch of his patient and her vital signs, while loved ones said their goodbyes. Suddenly he asked everyone to clear the room. Sklow’s blood pressure was improving. Maybe her blood was clotting now. He wanted one more chance to save her.

PATI ENT CARE

For eight more hours, Green ordered small adjustments to her medications. She received transfusions. She got more medications, and more adjustments. Slowly, it started to seem like Sklow might survive.

That was June 2019.

A year later, Sklow has a renewed life, and she’s giving back. “I mean, they saved my life,” she says of Green and his colleagues at Upstate.

The family mantra while she healed had been to put one foot in front of the other. With that in mind, Sklow planned to walk a 5K on the June 26 anniversary of her near-death experience. But because she injured her ankle, family members instead pushed her in a wheelchair and collected about $12,000 to donate to the hospital. They also had a reunion on a Zoom call with the doctors and nurses and staff who cared for Sklow when she was hospitalized.

Sklow, 57, speaks with a raspy voice now, a consequence of being on a ventilator for so long. She is an eighth-grade teacher who lives in New Jersey, and she directs a summer sleepaway camp at Raquette Lake in the Adirondacks, about 45 minutes east of Old Forge. She considered herself in good health.

Arrival in the nick of time In June 2019, she prepared for her 14th year at camp by posing with the entire staff for a photo the evening before campers would arrive. As the photos were snapped, Sklow felt a strange sensation. “I thought maybe this is what it feels like when you’re going to faint.”

Her right side became numb. She developed a severe back- ache. Sklow was in so much pain, she laid on her side in the back of an ambulance for the 1½-hour drive to a hospital in Utica.

Relatives got word to her children, 22-year-old twins.

They had both graduated from college, and her daughter, Charlotte Sklow was in Dallas, helping her son Kenan Sklow settle into an apartment. The pair raced to the airport.

They flew standby to New York City, borrowed a car and drove to Syracuse.

Sklow was flown by helicopter to Upstate after tests revealed the aortic dissection.

“She got here right in the nick of time,” Green says, noting that Upstate physicians care for 30 to 50 people with aortic dissections each year.

Robbie Birnbaum, Sklow’s significant other, rode in the helicopter with her to Upstate. He says it felt like an episode of the television show “ER.” He was escorted from the helipad. Then Green was explaining to Birnbaum how he would take care of Sklow, while the anesthesiologist was accompanying the patient to the operating room.

That anesthesiologist, Syed Ali, MD, would later tell Birnbaum that in his 25 years of service, he’s never seen a case like Sklow’s, where a patient with such a lengthy aortic dissection pulled through.

To celebrate her recovery, Kim Gutfleish Sklow’s family organized a 5K walk on the anniversary of her lifesaving surgery. Pictured are Sklow, center, with her children, Kenan and Charlotte, right, and partner, Robbie Birnbaum, left.

PROVIDED IMAGE

Of course, Sklow’s memories are fuzzy. In her postoperative fog, she imagined she was at the camp’s dining hall, and she called nurses by the names of camp staffers.

In reality, she was recovering in the intensive care unit, her heart atop her chest. As her body healed and the swelling reduced, Green took Sklow back to the operating room to sew her heart back into her chest.

“Dr. Green was beyond amazing,” she says. “The entire team, the nurses on the cardiac intensive care unit were not just wonderful to me, but they were especially wonderful and supportive to my family. None of us are from Syracuse, so some of them were there for a month, staying in a hotel.

“Everybody did everything they could to just make it as easy as they could for us.”

It was about three weeks later when Sklow was well enough to get out of bed. She made it to the door of her hospital room, turned around and returned to the bed, exhausted.

She was discharged to a rehabilitation center closer to her home in Englewood, New Jersey. She completed three months of cardiac rehabilitation. When she was well enough, she returned to teaching eighth grade at a private school in Manhattan. Then the pandemic hit, and, like her colleagues, Sklow began teaching from home. Her summer camp was cancelled for 2020.

Over the last year, she has had follow-up appointments with a variety of medical specialists. She’s told the story of what she went through to each of them. She says, “Every single doctor and nurse has had the same reaction: They say, ‘And you’re alive?’” u upstate.edu l fall 2020 l U P STAT E H E A LT H

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PATI ENT CARE

SUNY Potsdam music professor Lonel Woods had a kidney transplant at Upstate University Hospital at the height of the coronavirus pandemic. He stayed at Sarah’s Guest House during his recovery and is pictured at nearby Upper Onondaga Park.

PHOTO BY EMILY KULKUS

Getting a kidney during COVID-19 Extra precautions ensure successful transplant for music professor

BY EMILY KULKUS

onel Woods was on the kidney transplant list for

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three years. During that time, Woods had gotten used to, as much as one can, performing self-

dialysis treatments five times a day, including once in the middle of the night. Each treatment took about 40 minutes, which accounted for more than three hours of the SUNY Potsdam music professor’s day.

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Woods, 52, is suffering from the same kidney disease afflicting his twin brother and the same disease that took his mother’s life at 56. His last several months have been filled with incredible highs and incredible lows. In January, he was minutes from an operation to receive a new kidney when doctors explained that the organ was not an ideal match. On a stretcher outside the operating room, Woods says he cried at the news. Then a few months later, the coronavirus pandemic swept the nation and put all surgeries – organ transplants

PATI ENT CARE

included – in jeopardy. Woods, a vibrant man who looks and sounds a decade younger than his age, thought he might never receive the kidney he so desperately needed.

transplant patients are given a strong immune-suppression medication during the procedure and then several doses afterward to prevent the body from rejecting the new organ.

Then, at 10:30 p.m. on April 22, Woods received a life-changing phone call from Upstate University Hospital.

A kidney that appeared to be a good match had become available. If he could get to the hospital by 7 a.m. the next morning, it would be his.

“Doctors altered it to include a safer option for immune suppression,” Rivera said. “When the immune system is depleted, patients are at risk for getting a virus or bacterial infection and currently the transplant team is increasingly worried about COVID. It would be devastating if someone who received a new transplant were to get COVID and their immune system is not capable of protecting them.”

Woods hastily packed a bag, made sure his apartment was clean and drove the nearly three hours to Syracuse.

He arrived at the hospital just before 7 a.m. where Upstate nurses and staff began prepping him for surgery.

‘Gracious and kind’ hospital staff With a potential match arising at the height of the COVID19 pandemic, Upstate and Woods had to take many extra steps to ensure everyone’s safety. Before even coming to the hospital, he had to answer an extensive questionnaire about his current health and potential exposure to the coronavirus: Was he feeling well, had he been quarantined and had he been exposed to anyone with the virus? Woods’ answers met the criteria, and he headed for Syracuse by himself, as no visitors were allowed during the pandemic.

Nurse Galina Rivera is a certified clinical transplant coordinator at the Upstate Transplant Center. Rivera was on call the night Woods’ kidney became available and was tasked with calling him at his home in Hannawa Falls to make the arrangements. After several unanswered calls to Woods, Rivera feared she might have to call the local police for help tracking him down.

“It was very late at night, and I could not reach him,” Rivera says, noting that luckily, she also had contact information for one of his friends, and with her help they were able to connect. “I was really happy when I saw his name come up, especially as he missed out on the transplant in January.”

Before being admitted to the hospital, Woods completed a rapid COVID-19 test in the transplant clinic, which came back negative. The donor was also tested before the organ came to Upstate, Rivera says.

An organ’s “cold time” is a measure of time that an organ can be outside the body. After about 24 hours the function of the kidney begins to decline and risks not being usable.

Woods’ new kidney was coming from a deceased 42-year-old in Illinois. It arrived at Upstate via ground transportation at about 7 a.m. on April 23. At about 2:30 p.m. that day – 16 hours after that first phone call – Woods was wheeled into surgery. The procedure took five hours, during which time Upstate nurses called family members in Chicago and South Carolina to update them on his progress.

“Everyone at the hospital was gracious and kind,” he says.

Regaining his strength An additional precaution had to be taken during Woods’ operation because of COVID-19, Rivera says. Traditionally,

The Upstate Transplant Center, which specializes in kidney and pancreas transplants, has performed 34 transplants since mid-March, when the pandemic began shuttering institutions and halting many medical procedures. That rate is the same as last year, says social worker Tania Lyons, the transplant quality assurance supervisor.

Woods says he awoke at about 10:30 p.m. that evening and spent several days in intensive care. He was both surprised and thrilled that nurses got him out of bed to sit in a chair and walk around just a day later. When Woods was released from the hospital, he went to Sarah’s Guest House, a Syracuse respite facility where people can stay while loved ones are in the hospital, so he could more easily attend his twice-weekly checkups. He takes about 10 medications a day, including anti-rejection and anti-infection drugs to help his body adjust to the new organ.

“They’ve been saying things are going really, really well so I’ve been very pleased,” he says. “There have been no issues, and I’m gaining strength all the time.”

Feeling blessed, feeling better Despite recovering from a major operation, Woods continued teaching online classes at Potsdam. He even conducted an online lecture from his hospital bed at Upstate, he said. As co-chair of music performance in the Crane School of Music at Potsdam, Woods is eager to sing again.

His resume includes a stint on Broadway in the 1990s.

He specializes in opera, oratorio and musical theater.

As part of routine post-transplant care, the Upstate Transplant Center recommends that patients avoid crowds to limit infectious exposures to prevent them from getting sick, Rivera says, adding that during the pandemic, that’s more important than ever. Woods followed her advice, remaining quarantined at Sarah’s Guest House. He filled his days with teaching online, naps and walks to nearby Upper Onondaga Park in the Strathmore neighborhood of Syracuse. Overall, he says he feels “wonderful,” and not having to perform the self-dialysis is his favorite part.

“That is the best thing,” he says. “My body is still waking up (in the middle of the night) because I had to do it for so long. But now I do a little work, or I watch something and then I go right back to sleep. It’s a great pleasure.

“My energy is up. I feel much better, and I feel blessed that this was able to happen.” u upstate.edu l fall 2020 l U P STAT E H E A LT H

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PATI ENT CARE

Prateek Wali, MD, encourages Ava Acker to manage her own medication and diet.

PHOTOS BY WILLIAM MUELLER

You want to do what?!

Unusual treatment offers relief for recurrent bowel infections

BY AMBER SMITH

A

How it started

va Acker did not like what her doctor proposed.

To treat the recurrent infection she had — which was making her inflammatory bowel disease worse — pediatric gastroenterologist Prateek Wali, MD, wanted to try a fecal transplant.

He would infuse a stool specimen from a healthy donor during a colonoscopy procedure, providing her body with a dose of healthy bacteria.

“They explained it to me, and I was like ‘what?’” recalls Ava, 14, of New Hartford.

Wali explained that an overgrowth of a bacteria called Clostridium difficile, or C. diff, was causing inflammation and worsening her bowel disease. “A donor stool sample with a different ratio of bacteria will sometimes help to bring that ratio in your colon to a more normal level,” he said.

If Wali could get rid of the infection through the fecal transplant, the regular medication Ava was taking for her bowel disease should work better.

Once she understood how the procedure could improve her life, Ava was game to try it.

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She only told her closest friends about her condition, and the treatment she would undergo.

U P STAT E H E A LT H l fall 2020 l upstate.edu

Ava was in kindergarten when she started having chapped lips off and on.

She describes: “They used to hurt so bad I couldn’t smile sometimes. I felt really tired all the time. And my stomach wasn’t right. I didn’t feel normal.”

Her mother, Debra Acker, who is a nurse, says it seemed like Ava used the bathroom a lot. In 2015, when she was 8, her pediatrician began suspecting she might have Crohn’s disease, a chronic bowel disease affecting the lining of the digestive tract.

“Everyone thought she was a nervous kid, and that wasn’t the case,” Debra Acker says.

They were referred to Wali, the division chief of gastroenterology at Upstate. They saw him on a Friday, and endoscopy and colonoscopy tests were scheduled for the following Monday.

Those procedures allow doctors to

PATI ENT CARE

examine the insides of the upper and lower gastrointestinal tracts to confirm the diagnosis of Crohn’s disease.

The cause of Crohn’s disease is unknown, but doctors believe a combination of genetics and environmental factors may play a role. A great uncle of Ava’s has Crohn’s disease. Her twin brother, Aidan, does not. The disease can be treated, but there is no cure.

Treatment options Wali first prescribed a medication that’s also used to treat cancer.

Later, he tried Ava on a variety of medications that had to be infused over time, or injected, in a search to find something to help her long term.

She also takes vitamins and follows a healthy diet.

Debra Acker says Ava keeps track of her medicine and diet on her own.

“Dr. Wali wanted us to instill that in her, so she would be compliant as a teenager,” she says.

Wali says researchers are constantly learning about the microbiome in our gut, which varies tremendously from one person to another but includes a variety of bacteria and other organisms, many of which help protect the body from infection.

People can become sick if the ratio of bacteria is disrupted, which can happen while taking certain antibiotics or other medications. C. diff is one of the most serious infections that may develop, often bringing abdominal pain and cramping, diarrhea and fatigue.

When it became clear that Ava was

battling C. diff, and medication was not helping, Wali proposed the fecal transplant. The procedure is designed to help restore intestinal balance and is approved by the Food and Drug Administration. Fecal transplants can be effective and even life-saving for some patients, he says.

Among patients who did not have underlying bowel disease, fecal transplants had a 91% cure rate, according to a study in the Journal of Pediatric Gastroenterology and Nutrition in March 2019. The cure rate was only 75 percent for those who had a bowel disease in addition to recurrent C. diff infection.

Finally some relief Ava underwent the fecal transplant in May 2019.

Her procedure was similar to a regular colonoscopy, during which time Wali placed the stool specimen in Ava’s colon. She describes feeling pressure when she initially woke up, and then it subsided.

Debra Acker noticed an improvement in her daughter almost immediately.

“She was a different kid. She had more energy. She looked better.” Ava stopped having to run to the bathroom seven or eight times a day, and she’s able to sleep through the night. She also regained the energy to go up and down stairs and carry her own books at school.

“I actually feel a lot better,” Ava says. u

Ava Acker had a fecal transplant to treat complications from Crohn’s disease. A gram of stool includes about 100 billion bacteria, 100 million viruses, 100 million microbes known as archaea (distant relatives of bacteria), 10 million cells from the lining of the gut, and a million spores of fungi, according to research published in the scientific journal PLOS Biology.

Where stool specimens come from The stool specimens used for fecal transplants at Upstate come from OpenBiome, a nonprofit organization in Cambridge, Massachusetts that was founded by a team of doctors, scientists and public health advocates.

sought but couldn’t find a clinician who could perform the treatment for him. When he finally received a life-changing fecal transplant, the effect was remarkable. Within a couple of days, he had his life back.”

They explain why they launched OpenBiome on their website, OpenBiome.org: “In 2011, a close friend contracted a C. diff infection after a routine surgery, and antibiotic treatment wasn’t working. For 18 months, we watched him suffer with this debilitating illness and several rounds of failed treatment. Knowing the evidence supporting the use of fecal transplantation to treat recurrent C. diff infection, he

Today, the group collects donations of stool from healthy adults up to age 50 who live nearby and who pass a series of screening tests.

In addition to treating patients with C. diff infections at more than 1,000 medical centers, some specimens are used in more than 30 clinical trials as a treatment for ulcerative colitis, irritable bowel syndrome and other disorders. upstate.edu l fall 2020 l U P STAT E H E A LT H

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Foundation

Impacting patient care, education, research, and community health and well-being through charitable giving.

Upstate Foundation family touched by COVID-19 h

Earlier this year — as Upstate Medical University was rising to meet the threat of COVID-19, and the Upstate Foundation was introducing new funds to help with the crisis — the outbreak struck close to home. Patrick Penfield, son of Upstate Foundation staffer Kristen Penfield, tested positive for the virus.

A junior at Syracuse University, Patrick was a couple of months into a semester abroad when the virus began Lto spread across that continent.

“I was studying in London, but I wanted to travel as much as possible,” said Patrick, 20. “I went to Paris, Madrid, and then, about a week before the coronavirus hit the headlines and people became concerned about traveling, I went to Barcelona and the Canary Islands over Spring Break.

Because of the timeline, I believe I got sick in one of those last two places .”

“ w h B I p

Overseas studies were cut short, and Patrick returned to his home in Lysander and immediately self-isolated. Sure enough, he — like other students from the same program —began showing symptoms and tested positive for novel coronavirus.

At that point, his voluntary quarantine became a court-ordered one, as his family brought food to his bedroom and maintained social distancing.

The isolation was challenging, admits Patrick. But family support, twice-daily check-ins from the Onondaga County Health Department, reading, learning Chinese, and watching Netflix helped him through the fear, distance and the barrage of virus-related news.

Deprived of his coursework in London, Patrick still learned a few lessons: “First, I’ve got newfound respect for health care workers; the people doing my tests were putting their lives at risk every single day.”

h

He also appreciates how the Upstate Foundation, where his mother works, responded with COVID-19 funds that offered support to Upstate Medical University students and employees facing financial hardship from the virus outbreak. “I’m fortunate to have my family. We’re able to get groceries and basic necessities. But there are lots of people who don’t have that.”“I appreciate how my oncology

d

Patrick’s scary brush with coronavirus has driven home the value of giving back — starting with his willingness to help inform others about the disease and by donating his blood and plasma to Upstate as a recovered COVID-19 patient, to help treat others who are ill.

As always, the Upstate Foundation is here for you.

that they don’t need to be afraid;

As part of the Upstate family, we fully appreciate the critical work being conducted in this unprecedented time by our colleagues all across Upstate Medical University.

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In partnership with your support, we are fully committed to helping our community overcome this crisis and have established the following COVID-19 related funds: • Support of Upstate Employees Fund • Virtual Visitation Fund for Upstate Patients • Student Retention Emergency Fund To learn more about these funds and give a donation, call 315-464-4416 or visit www.UpstateFoundation.org/covid-19

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Where your gift impacts the health of the entire region.

Patrick Penfield, second from left, and his family

FROM OUR EXPERTS

Send in the ticks Studying the pests helps predict disease emergence across the state

BY AMBER SMITH

pstate’s Citizen Science Tick

He also receives quite a few dog ticks, and Lone Star ticks.

Testing Program invites the

Thangamani’s lab tracks the emergence of a dozen

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public to send in ticks they

find to be tested for Lyme and other

species of ticks that are most prevalent in New York state.

Researchers strive to understand the geographical expansion of the ticks and tick-borne diseases. They analyze weather information to predict whether the climate influences tick

disease-causing agents.

migration. And they study how ticks transmit various

The majority of the ticks that are submitted are deer ticks, and one-third of those carry Borrelia burgdorferi, the bacteria that causes Lyme disease, says Saravanan Thangamani, PhD, a professor of microbiology and immunology who leads the Vector-borne Infectious Diseases Laboratory. (Learn more at thangamani-lab.com)

diseases and which host species — animals or human — the different ticks prefer.

Based on data he collected from 2019, Thangamani says human New Yorkers are only the second favorite host for Lyme-positive ticks. Turns out cats are No. 1. u

Where ticks attach

7%

Deer ticks collected between July 2019 and July 2020 through Upstate’s Citizen Science Tick Testing Program were found all over their human hosts.

Here are the spots on the human body that were most popular with the blacklegged arachnids:

5%

6%

8%

8%

7% 16%

16 8 8 7 7 7 6 6 6 5 5 4

percent on the thigh percent around the waist percent on the stomach percent on the groin percent on the upper back * percent on the scalp percent upper arm percent behind the knee percent calf or shin percent in armpit percent on the neck percent on the lower back * those found on the upper back were most likely to carry the bacteria that causes Lyme disease) SOURCE: SARAVANAN THANGAMANI, PhD

5% 7%

4%

6%

6%

upstate.edu l fall 2020 l upstate health

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FRO M OUR EXPERTS

Eyeing the storm Sepsis research targets treatment for cytokine storm, which may also benefit COVID-19

BY EMILY KULKUS

new type of sepsis treatment investigated by a

A

team of researchers at Upstate could bolster

survival rates — and be useful in treating

severe cases of COVID-19.

Sepsis is a potentially life-threatening condition caused by the body’s response to an infection. It affects 1.7 million adults in the United States each year and contributes to more than 270,000 deaths.

When the body’s immune system fights an infection, it releases a group of proteins called cytokines, which control inflammation in the body. Sometimes the body goes into overdrive, releasing more cytokines than it should, producing what’s known as a cytokine storm. This can lead to organ failure and death.

A cytokine storm or hyperinflammation syndrome has been documented in many patients with severe cases of COVID-19, tied to the coronavirus pandemic.

Researchers believe their new therapy might help.

“We are targeting the cytokine storm and inflammatory mediators,” describes Juntao Luo, PhD, an associate professor of pharmacology. He leads the team that has been studying a way to neutralize severe inflammation during sepsis for the last three years.

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“Inflammation is a double-bladed sword, as it is meant to control infection naturally but can cause tissue damage if unchecked,” he explains.

“Inflammation in a sepsis patient is really hard to control, and for many patients they cannot overcome that and may die.”

Sepsis is usually treated with antibiotics plus supportive measures, including intravenous fluids, medications to support blood pressure and mechanical ventilation or dialysis, to support injured lungs or kidneys.

Luo worked with professor and Chair of Surgery Robert Cooney, MD, to develop new nanotrap technology that absorbs excessive inflammatory mediators in the blood. When paired with antibiotics, their new treatment dramatically increased survival rates in experimental models.

PATIOUR ENT EXPERTS CARE FROM

“With antibiotics alone to control infection we get a 50 to 60 percent survival rate,” Luo explains. “Our new approach to control inflammation also gives us a 50 to 60 percent rate.

“But when they are combined, the survival rate is 100 percent. This technology can be used as a blood-cleaning therapy and is promising to improve the survival rate of severe sepsis.”

Juntao Luo, PhD

Illustration of COVID-19

A paper describing their work — “A Nanotrap Improves Survival in Severe Sepsis by Attenuating Hyperinflammation” — was published recently in the scientific journal Nature Communications. The summary says that “in addition to reducing the inflammatory effects of infection, the nanotrap technology decreased injury to lung, kidney and liver and also improved survival.”

Li Chen from Baylor Scott and White Medical Center in Temple, Texas, and Matthew Dai, formerly of Upstate and now from Brown University, also contributed.

The research is supported by several grants from the National Institutes of Health. Other Upstate contributors to

Doctors at Upstate have been pooling their expertise through the Sepsis Interdisciplinary Research Center since

the project are: Changying Shi, Xiaojing Wang, Lili Wang, Qinghe Meng, Dandan Guo and Guirong Wang.

Robert Cooney, MD

the summer of 2019. Luo’s work is one of the center’s primary projects.

Cooney says Upstate physicians and researchers involved in the center are studying sepsis, infection and organ failure. The group meets regularly to share research and collaborate on projects such as this one to develop integrative therapies for sepsis and critical illness.

“Sepsis is a critically important public health problem,” he says. “Upstate is trying to combine our energies and expertise to improve outcomes.”

Learn more about sepsis research at www.upstate.edu/pharm/sirc u

CONNECTED FOR YOUR CARE

We now offer telehealth visits for patients seeking second opinions on their cancer diagnosis.

During this visit, our physicians are ready to help you understand your options or to offer the reassurance that you are on the right path for treatment. Should you need to be seen for care, Upstate has the dedicated experts and technologies already in place to treat your specific type of cancer.

YOU HAVE OPTIONS FOR YOUR CANCER CARE.

SYRACUSE

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ONEIDA

l

OSWEGO

UPSTATE.EDU/OPTIONS 315 464-HOPE (4673) upstate.edu l fall 2020 l U P STAT E H E A LT H

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FROM OUR EXPERTS

Associate professor Frank Middleton, PhD, medical student Alfred “AJ” Espinoza, and clinical research associate Rhianna Ericson, all from Upstate, are part of a group studying the rare illness PKAN in the Dominican Republic. Research team members are shown at a sign for the town of Cabral, where they are studying clusters of the disease. From left are Carlos Muniz, MD; Middleton; Craig Bass and his wife, Susan Baser, MD; Ericson; Madeline Santana, MD; and Espinoza. Santana is Dominican, and the others are from the United States.

PHOTO COURTESY OF FRANK MIDDLETON, PhD

Investigating a rare nerve disorder Scientists explore cluster of cases of inherited condition that has no cure and few treatments

BY JIM HOWE

n a remote corner of the Caribbean,

I

Upstate researchers are taking part in an international effort to study

and perhaps alleviate a rare degenerative disease.

This inherited condition, known by its initials as PKAN (pronounced PEA-kan), has an unexplained cluster of cases in a poor provincial town in the Dominican Republic, where affected children and teens gradually lose control of their muscles, similar in some ways to Parkinson’s disease.

Symptoms and their intensity vary from person to person and can include: l l l l

14

frequent falls, leading to broken teeth and other injuries; tongues hanging uncontrollably out of mouths and often getting bitten; bodies becoming clumsy, spastic or tightly twisted; trouble speaking or swallowing, which can lead to problems of getting sufficient nutrition;

U P STAT E H E A LT H l fall 2020 l upstate.edu

l l

tremors and involuntary movements; eventually needing help with everyday tasks like combing hair, using the bathroom or walking;

l

vision problems;

l

death in childhood or early adulthood.

The condition stems from a genetic mutation that causes iron to accumulate in the basal ganglia, a part of the brain associated with movement. PKAN, which stands for pantothenate kinase-associated neurodegeneration, has no known cure and few available treatments, which typically offer only limited, temporary relief.

“As iron accumulates in the basal ganglia, patients slowly lose their ability to control their movements. Their ability to think, however, remains unaffected. They are prisoners in a body which refuses to obey their commands,” is how a video documenting PKAN, titled “Tough as Iron,” sums up its effects.

In most of the world, PKAN occurs in about one to three people per million. But in the dusty, sun-bleached Dominican town of Cabral, with a population of about 16,000, the rate is at least a thousand times above the world average. Cabral has at least 40 known cases, and likely more undiscovered

FROM OUR EXPERTS

cases. A few other rural Dominican towns also show dramatically elevated rates of PKAN. Upstate’s Frank Middleton, PhD, is among an international team of researchers studying the disease, to seek ways to halt or reverse its effects and to help families who have a member with PKAN. This team, working under the PELICAN Study (see box), has been interviewing affected families, obtaining DNA samples for gene sequencing studies and supplying clothing, gifts and food, since those with PKAN can have a high metabolism and need extra calories. The team hopes eventually to establish more and faster DNA testing and genetic counseling for carriers of the mutation.

To get the disease, both parents must be carriers — people who carry the genetic mutation but do not have the disease.

If two carriers have children, each of their children has a 25% chance of having the disease, a 50% chance of being a carrier and a 25% chance of neither having the disease nor being a carrier. The town has an estimated 2,500 carriers, about 15% of the population, so the chances of two carriers mating in a town like Cabral is relatively high.

“What is unique about this situation is that because the prevalence is so high, there are opportunities to understand precisely how it starts and develop treatments that you simply could not do anywhere else in the world,” says Middleton, an associate professor with appointments in neuroscience and physiology, biochemistry and molecular biology, pediatrics and psychiatry and behavioral science.

Calling PKAN “an emerging pediatric health crisis” in the Dominican Republic, he notes that certain factors of life in Cabral and other affected towns probably contribute to the high incidence, such as: l

l l

l l

frequent intermarriage among cousins in a relatively isolated town, some of whom are not aware of the familial relationship; a lack of awareness that one is a carrier and a general lack of understanding of genetics; beliefs and superstitions that reject scientific explanations for PKAN, instead thinking it is a curse or that it is spread by infection; lack of funding for and availability of genetic testing, analysis and research. difficulty in getting to Cabral — three hours over bad roads from the capital — let alone transporting sensitive medical equipment there that might allow, for example, brain scans for early detection of the telltale iron accumulation.

Middleton’s research into PKAN included the use of saliva samples as an additional tool for screening, as well as identifying subgroups of PKAN by their particular symptoms.

By studying the underlying mechanisms of PKAN, the scientists may also discover connections to other neurodegenerative movement disorders, including Parkinson’s disease. u

This young man appears in “Tough as Iron,” a documentary on PKAN and its prevalence in some small towns in the Dominican Republic.

A quick look at a rare disease PKAN: The initials stand for pantothenate kinaseassociated neurodegeneration. This rare, inherited disorder stems from a mutation of the PANK2 gene, which leads to abnormal iron accumulation in the brain and progressively affects muscles and movement, resembling Parkinson’s disease in some ways. It is found in unexplained clusters in a few towns in the Dominican Republic, which allows for in-depth study. Symptoms can show up in early childhood and vary widely in severity and form. There is no cure. Current treatments — such as physical therapy, chelation to extract excess iron and Botox injections to relieve muscle spasms — usually offer at most limited, temporary relief.

NBIA: PKAN is the most common type of this group of disorders. The initials stand for neurodegeneration with brain iron accumulation. As the name states, NBIA disorders are characterized by an abnormal accumulation of iron in the brain and progressive degeneration of the nervous system.

PELICAN Study: Starting in 2011, a team of doctors and scientists began studying the disease and providing medical care and family support, including gifts and food supplements. The name stands for PKAN Evaluation in Cabral Natives, Cabral being a Dominican town with a high incidence of the disease.

INFORMATIONAL VIDEO: “Tough as Iron” shows how PKAN affects people who have it and their families and talks with experts about efforts to alleviate it, in layman’s terms. About five minutes long, Google “Tough as Iron PKAN” to find it. upstate.edu l fall 2020 l U P STAT E H E A LT H

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IN O UR CO MMUNITY

Patricia Goodyear has five children, ages 6 to 12. “As a single parent in the military, you have to have a plan. I knew that my children would stay with my friend if I ever needed to leave,” says Goodyear, a colonel in the Army Reserve.

PHOTO BY N. SCOTT TRIMBLE/SYRACUSE MEDIA GROUP

Battling COVID-19 Nurse practitioner deployed to NYC during the worst of the pandemic

BY AMBER SMITH

rmy Reserve Col. Patricia Goodyear is a nurse

A

practitioner at Upstate University Hospital who

spent two months at Queens Central Hospital

caring for patients with COVID-19, when New York City was hardest hit by the pandemic.

Here’s more about her experience:

She joined the Army Reserves at age 23.

“I joined back in 1988. Most of my family members had been in the military at some point, so it was always something I wanted to do.”

She mobilized in March with two day’s notice.

16

“I was not really given any information about where I was going. I was simply informed that I was being mobilized for the campaign against COVID-19. We were all brought to Utica to begin with, and then parts of our group were divided off into other places to form 85-man urban task forces to deal with this disease.”

U P STAT E H E A LT H l fall 2020 l upstate.edu

She was glad to serve.

“Honestly I thought it was an amazing chance to be able to help in a situation where you’re watching on the television news day after day a city within our state just suffering with this disease and needing health care providers.

“I also felt like for me, because I have five children — my oldest is 12, and my youngest is 6 — I didn’t have to worry about bringing it home to my kids every night.

“I won’t say I was excited because it is hard to leave home and leave your children, but I was glad to be able to help.”

continued on page 17

IN OUR C OMM UNI TY

She worked long hours.

“I have an acute care and critical care background, so they put me in the medical intensive care unit, which had some very severe needs at that time. Quite a few of their staff had contracted COVID-19. They had exposed themselves without realizing it because in the beginning, if the patient didn’t come in with symptoms of COVID-19, the staff didn’t take precautions.

“We worked 12 to 14 hours a day six days a week. Queens Central Hospital uses EPIC for their electronical medical records, and we use EPIC at Upstate. That made my transition that much easier, because I knew how to use the charting system.”

She saw patients who did not survive.

“I had a few patients that I thought were going to do OK. In particular there was a patient that we had extubated

(removed him from a ventilator) who was very young, in his 30s. After he was extubated, he seemed to be doing well and got transferred out of the ICU. When I went back into the records in EPIC, his name showed up. It said “discharged to morgue.” I couldn’t believe it.

“Most of the patients I treated were young. It was very hard for me.”

She and her team stayed healthy.

“Handwashing, hand sanitizer, wearing a mask and social distancing does a lot more than people give it credit for.

“We had 85 people in my task force, and there was another task force nearby. None of us caught COVID-19, and we were involved in caring for patients with COVID-19 daily for over two months. What was universal among all of us was social distancing, wearing a mask, using good handwashing and hand sanitizing.” u

Patients needed companionship; medical students stepped up

BY CHARLES McCHESNEY

The COVID-19 pandemic presented hospitalized patients with an unusual challenge: No visitors.

To combat the spread of the novel coronavirus, hospital leaders, including those at Upstate University Hospital, have allowed visitors only in the most dire situations. That left hospitalized patients — all patients, not just those with COVID-19 — without the companionship and support offered by visits from family and friends.

Recognizing the issue, Brian Changlai, MD, an assistant professor of medicine at Upstate, turned to students from the Rural Medical Scholars Program, known as RMED, for help. Changlai undertook the task in his role as associate director of patient experience for the hospitalist division at Upstate.

“The students were available to the patients as not only a clinical resource to help answer questions about COVID or other medical issues, but also as an outlet for the patients in solitude,” he explains. Working from their homes, the students would call patients on their hospital room phones.

RMED student Danielle Clifford had been doing clinical work at the Clifton Springs Hospital and Clinic when, to help ensure the safety of students and the community, Upstate prohibited students from all direct patient care opportunities. That didn’t change a simple fact for Clifford and others who volunteered: “We’re in medical school. We want to be able to still

help, to hopefully make a difference.”

Danielle Clifford

Working off a spreadsheet from Changlai, student volunteers phoned patients. They had a preset list of questions, but they also simply conversed. “Would you like to talk today?” was often the first question. They asked how patients were feeling, if they had questions about their care and if they understood their diagnosis.

Shortly after the project started, students got access to electronic patient records, so they could be more informed when talking with patients.

Patients seemed to appreciate the conversation, having “someone just to say, ‘I care how you are doing,’” says student volunteer Claire Englert.

Jacqueline Maier

Claire Englert

Other times, the call was vital. Patients alerted students to difficulties they were having, or to pain.

Student volunteer Jacqueline Maier had one patient who complained about her breathing. Maier sent a text to the team caring for the patient, and “they rushed in and gave her an albuterol treatment.”

Englert recalled a patient with whom she had been talking who had a simple issue she had not mentioned.

“Anything else I can help you with?” Englert asked at the end of the call. It turned out the patient was hungry. Englert was able to contact the care team and get her a meal.

Changlai praised the volunteers. They were “exceptional across the board — and are promising future doctors.” u upstate.edu l fall 2020 l U P STAT E H E A LT H

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IN OUR C OMM UNI TY

IN HER OWN WORDS

A stroke at 34 Upstate nurse shares her ordeal

BY LINDSAY SHAW

ct. 6, 2014, was the day after my 34th birthday, and I was looking forward

O

to a dinner with friends to celebrate. I woke up with a headache but just

shrugged it off, thinking I was overtired from working late nights as a nurse

and having two little kids (at the time, Stella was 4, and Tristan was 3.) I took some 18

ibuprofen and got my kids packed into the car for our daily errands.

U P STAT E H E A LT H l fall 2020 l upstate.edu

IN OUR C OMMUNITY My headache wasn’t getting any better as the day went on. We got home, and I put my kids down to rest, and I took some more pain medication.

I bent over to get something out of my tub, and when I stood, I had intense pain in the back of my neck and head.

It was a thunderclap headache. I couldn’t really move my head, the pain was so intense. I felt like I was going to pass out from all the pain.

I lay down, and the pain just got more and more intense. I felt like my brain was having labor contractions in the back of my skull. I knew something wasn’t right.

My husband, Dave, came home to stay with the kids, and my sister, Carrie Higginbotham, took me to the emergency department at Upstate University Hospital. I thought I did something to my neck. Never did I think it was my brain.

After a computed tomography scan of my head, I heard urgent pages for the neurologists. I just knew they were calling them for me and that it wasn’t good.

Within minutes I had a slew of doctors at my bedside saying I needed to be moved immediately to the Intensive Care Unit because I had a subarachnoid hemorrhage, which is a type of stroke where you are bleeding in the space between your brain and your skull.

It had gone from me thinking, “Oh, it’s probably muscle spasms, and I’ll be home soon” to “I’m on my way to the ICU, and I may be here for weeks.”

My husband rushed to the hospital, where doctors tried to prepare us for what could happen in the next 24 hours to two weeks. Within hours, I was

uncontrollably vomiting, and the pain was absolutely unbearable.

The plan was for a procedure the next morning to find the cause of the bleed and fix it; but it turned out, there was no aneurysm or anything to repair.

I had a spontaneous hemorrhage, and the doctors told us the bleeding would clot and stop on its own. The next five or six days were a blur, because I was heavily medicated. They have to keep your blood pressure under tight control. They were working to keep me stable and to prevent another stroke, which can happen as a result of the blood vessels in my brain constricting from my original stroke.

It was really scary for my kids. My husband would bring them in. For the first week, I would wake up and say “oh, hi” and fall back to sleep. I’d wake back up thinking it was five minutes later, and it would be half a day later. It got better for them when I could sit up, when I started to eat and be able to move around and spend time with them.

Even after I went home, after 12 days in the ICU, I had some vision problems and severe vertigo for weeks. Today, more than five years later, there’s some lingering pain in my eye and some memory issues.

It was a long, four-month recovery before I was able to go back to work part time.

When I experienced that thunderclap headache on Oct. 6, 2014, I could have said, “oh it’s just a migraine.” I had a history of migraines. But something was just not right. So my message is: listen to yourself and your body. u

What’s a thunderclap headache?

An abrupt onset of a severe headache that hits with full force in less than a minute and lasts for at least five minutes is called a “thunderclap” headache.

The pain may cause nausea or vomiting, or other signs and symptoms including an altered mental state, fever or seizures. Thunderclap headaches

usually require urgent medical care.

They can signal a stroke or another dangerous condition, but they can also occur because of benign conditions.

Patients experiencing such severe headaches may need to undergo medical imaging scans or other tests to determine the cause.

SOURCE: AMERICAN MIGRAINE FOUNDATION

Her daughter recalls heart surgery at 7

BY STELLA SHAW

I’m 10 now, but when I was 7, I woke up one morning sick with flu-like symptoms. I told my mom that my heart felt like it was going to beat out of my chest. I stayed home from school, and my mom brought me to my doctor. My doctor told us that she heard a murmur that wasn’t there before. She sent us right away to see a cardiologist, just to be safe.

When I was at the cardiologist, I got an echocardiogram (an ultrasound image of the heart muscle and valves) and an EKG (an electrocardiogram to measure electrical activity.)

He said, “This is serious.”

I had a coarctation of the aorta. He said I would need heart surgery to fix it. I had to take medicine all summer to get my heart ready for my surgery.

I had my surgery in November 2017. I was really scared, but I knew it would be OK because my mom was with me the whole time, and I had a lot of care from good people.

I was in the hospital for a week and had to be out of school for a month.

I had to see my cardiologist a lot after my surgery to make sure everything was healing OK, but now I only have to see him once a year. All my checkups have been great.

Before my surgery, I always got really tired riding my bike and had a hard time running far because my legs would hurt and get really tired, but I never knew why. We found out that it was because my heart had not been pumping blood to my legs like it was supposed to. Now, I can keep up with my friends on my swim team in the pool and my brother on my bike.

I might have to have surgery as a grown-up to fix my valve, but it is working OK for now. u

What’s a coarctation of the aorta?

It is a congenital condition in which the main artery that feeds the heart is unusually narrow and there is a bicuspid aortic valve, meaning that it has two rather than three leaflets. upstate.edu l fall 2020 l U P STAT E H E A LT H

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IN OUR C OMM UNI TY

Striving to thrive A family-centered approach to treating youth with sickle cell disease

BY JIM HOWE

Syracuse teens Knai Bridges, left, and Malyzha Espey have sickle cell disease. They met at an event held at Upstate for young people with sickle cell and their families. Knai, who was a senior at Nottingham High School when this photo was taken, wrote a college application essay that described the years of pain and hospital visits the disease had caused but included the affirmation: “I have sickle cell. Sickle cell doesn’t have me.”

PHOTO BY ROBERT MESCAVAGE

t’s the world’s most common inherited blood

I

disorder, yet it is widely misunderstood or even unknown to much of the public.

Children with sickle cell disease must learn from an early age to avoid infections, temperature extremes or changing temperatures, and physical or emotional stress.

Their parents can feel they are fighting a lonely battle as they talk to teachers, gym instructors, school nurses and even relatives and neighbors about their children’s needs and limitations.

The children’s family members must learn how to help out and to deal with the challenges the disease brings to their households.

In response to these challenges, the pediatric sickle cell program at Upstate aims to treat the illness as something that affects all aspects of a family’s life.

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U P STAT E H E A LT H l fall 2020 l upstate.edu

Pediatric psychologist Jill Majeski explains how the sickle cell team addresses patients’ needs, such as:

l medical: doctor visits and hospitalizations, medications, pain episodes (called crises) and infections. l social: stigmas related to mistaken ideas about the disease; its impact on school and relationships.

l psychological: how the disease affects mood, worries and self-esteem.

Upstate’s program treats about a hundred patients from infancy through age 21 across a wide swath of Upstate New York. Adults are treated through a separate program.

IN OUR C OMMUNITY To reach out to families of sickle cell patients, the pediatric sickle cell program held an event in late 2019 called “Thriving Together: Starring Kids With Sickle Cell Family Event,” which was underwritten by charitable groups, such as The Kara Fund, a local charitable group that helps children with life-threatening diseases and their families.

The sickle cell team hopes to repeat the event.

“Thriving Together” allowed people touched by the disease to meet and to hear a talk on the disease, advice on being a child’s advocate and how to cope with the disease’s complications.

UNDERSTANDING SICKLE CELL When both parents have sickle cell disease

Sickle cell disease and sickle cell trait are inherited.

This chart shows the likelihood of any child being affected by the disease or the trait.

KEY

Each child will have sickle cell disease Zero chance any child will have sickle cell trait Zero chance any child will be unaffected

—has sickle cell disease

Insights from Thriving Together Playing a game using elbow macaroni to teach about sickle cell, showed Ryleigh Farrell, 8, of Syracuse that “when parts of my body hurt, like my arms or legs, it’s your blood clumping together like a crescent moon.” Iliyana Funderburg, 10, of Syracuse, who played the game with her, thought it was ”really cool” to see how her blood cells are affected. She noted that neither adults nor children at school usually understand her sickle cell disease: “My mom has to tell my teachers every year of what to do if I get cold or sick at school,” she said.

Emanuel “Manny” Liles, 15, of Liverpool, who has sickle cell disease, describes it as “complicated to talk about and hard for people to understand.” His mother, Sheryl Liles, appreciates the emotional support of meeting other people who are dealing with what can be an “overwhelming” condition. Anything that helps spread accurate information about sickle cell disease is helpful, she added.

Antoinette Singleton of Syracuse, who attended the event with her grandson, Kyren Herring, age 8, who has sickle cell disease, agreed that meeting others facing the condition was “wonderful.” She said it added another aspect of support to the medical, social and educational help provided by the members of Upstate’s pediatric sickle cell team, which she said “has been nothing but positive.”

“A lot of success with kids relates to how parents are instructing and teaching them,” said Kathryn E. “Katy” Scott, MD, the director of the pediatric sickle cell program.

Among the program’s goals are preparing the young people to understand and manage their disease, so they will be able to deal with it in adulthood.

Since pain is often an issue, a second goal is to minimize the use of medications when possible. “I teach parents about how to help children with chronic pain, coping when we can’t make it go away,” said Majeski. “I work with kids and parents to focus on those two goals.”

While there is no universal, risk-free cure for sickle cell disease at the moment, there are promising developments, such as voxelotor, a recently approved drug for older teens and adults; stem cell transplants, which carry risks but can result in a cure; and treatments still being researched, including gene therapy and new drugs.

Scott is hopeful that a cure will be available to everyone with sickle cell disease in the next several decades, within the lifetime of the young patients she sees now. u

—has sickle cell trait —is unaffected (has neither disease nor trait)

When one parent has When one parent has sickle cell disease and one sickle cell disease and parent has sickle cell trait one parent is unaffected

50% chance any child will have sickle cell disease 50% chance any child will have have sickle cell trait Zero chance any child will be unaffected

Zero chance any child will have sickle cell disease Each child will have sickle cell trait Zero chance any child will be unaffected

When both parents have sickle cell trait

When one parent has sickle cell trait and one parent is unaffected

25% chance any child will have sickle cell disease 50% chance any child will have sickle cell trait 25% chance any child will be unaffected

Zero chance any child will have sickle cell disease 50% chance any child will have sickle cell trait 50% chance any child will be unaffected

SOURCE: SUNY UPSTATE MEDICAL UNIVERSITY

Sickle cell disease is an inherited DNA mutation Sickle cell disease can turn normally flexible and round red blood cells into hard, sickle-shaped (or C-shaped) cells.

This disrupts normal blood flow and can lead to painful episodes known as sickle cell crises as well as anemia, infections, fevers, yellowed eyes, organ damage and early death.

People with sickle cell trait (sometimes called sickle cell carriers) do not have sickle cell disease but can pass the chance of getting it to their children.

Sickle cell disease affects about 100,000 Americans and occurs more often in people whose ancestors came from Africa, the Mediterranean region, the Middle East and other parts of Asia. Various treatments can alleviate the disease, but there is no universal cure at the moment.

SOURCES: AMERICAN SOCIETY OF HEMATOLOGY, SICKLE CELL DISEASE ASSOCIATION OF AMERICA INC.

upstate.edu l fall 2020 l U P STAT E H E A LT H

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IN OUR LEI SURE

Docs who rock band comprised of five Upstate doctors won “best audience participation”

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during the “Rockin’ the Redhouse” benefit concert earlier this year. The

event raised money for Redhouse scholarships and provides an opportunity

for garage bands to perform on stage at the Landmark Theatre in Syracuse.

The Bronze Puppies got their name from a group text. A message referring to their vocalist’s “golden pipes” was somehow auto-corrected to “bronze puppies.” Here’s a look at how each makes music a part of leisure time:

Jean Ball, DC

Lawrence Chin, MD

Instrument: vocals.

Instrument: drums.

Career: assistant professor, cell and developmental biology

Career: neurosurgeon, dean of the College of Medicine.

Began music lessons: ”I have been singing since high school with some lessons along the way.”

Began music lessons: Piano at the age of 8. “I still play piano but infrequently and know how to play the guitar but don’t due to lack of talent.

I am mostly a drummer these days.”

How do you practice? Just with the band.

Why music is part of her life: “Music communicates a message and tells a story in a way that plain words can not do.”

U P STAT E H E A LT H l fall 2020 l upstate.edu

Best concert he attended: Steely Dan any year or location, but most recently at the Landmark Theatre in 2019 in Syracuse.

James Greenwald, MD Instrument: bass.

Graduated medical school: 1987.

Career: doctor of family medicine.

What he does just before taking the stage: Takes deep breaths and visualizes the crowd cheering.

Began music lessons: “I joined the choir in my local church when I could still sing soprano. I started trombone lessons with my school music teacher, Lawrence Bunny, in fifth grade. I taught myself how to play bass.”

All-time favorite song: Steely Dan’s “Kid Charlemagne” or “Peg.”

22

Why music is part of his life: “It’s relaxing and enjoyable, but also challenging and creative.”

IN OUR LEI SURE

The Bronze Puppies performing at “Rockin’ the Redhouse” at the Landmark Theatre. Page 22, from left: Jennette (Jean) Ball, DC; Lawrence Chin, MD; and James Greenwald, MD. This page: Gary Johnson, MD; Rachel Hopkins, MD; and Paul Klawitter, MD, PhD. PHOTOS BY ROBERT MESCAVAGE

Graduated medical school: 1978.

What he does just before taking the stage: Gets scared and sits by himself.

All-time favorite song: The Jussi Björling and Robert Merrill version of “Au Fond du Temple Saint,” known as “The Pearl Fishers’ Duet,” from Georges Bizet’s 1863 opera, “Les Pecheurs de Perles.” v

Why music is part of his life: “I’m pretty sure it is hard-wired into my brain.”

Favorite concerts from the 1970s: UFO at Chicago’s International Amphitheatre, Pink Floyd at Chicago’s Soldier Field, and Blue Öyster Cult at Capital Centre in suburban Washington, D.C. Favorite concerts from the 1980s: The Kinks at Capital Centre, and John Hiatt at the former First Baptist Church facing Columbus Circle in Syracuse, also known as the Mizpah Tower.

Why music is part of her life: “Music is a fun, fulfilling way to connect with other people. There’s also just something deeply and uniquely human about music.”

Paul Klawitter, MD, PhD Instrument: guitar.

Career: emergency physician.

Began music lessons: Clarinet lessons in third grade.

Graduated medical school: 1993.

Rachel Hopkins, MD

Earned his doctorate: 2002.

The performer he channels: Phil Lesh, formerly of the Grateful Dead.

Instrument: vocals.

Gary Johnson, MD

Began music lessons: “I’ve actually never had a guitar lesson.”

Began music lessons: “I took some voice lessons in high school, approximately 100 years ago. I also took piano lessons for a couple of years as an adult when my kids started lessons.”

What he does just before taking the stage: Runs through the set list in his head; tries to trick himself into not being anxious.

Graduated medical school: 1986.

Graduated medical school: 1999.

Why music is part of his life: “Music is a hobby, and once you get comfortable with screwing up on-stage, it’s a lot of fun and good fellowship.”

What she does just before taking the stage: Try to stay calm and not succumb to the terror.

Instrument: guitar.

Career: emergency physician.

Career: endocrinologist.

How she practices: Sings in the shower and her car all the time.

All-time favorite song: “This is an unanswerable question. I suggest following the Rolling Stones’ advice: Make every song you sing your favorite tune.”

Best concert he attended: Johnny and the Triumphs in the mid- to late 1980s at a Potsdam bar called Django’s.

The performer he channels: British songwriter/guitarist Mark Knopfler, who cofounded the band Dire Straits.

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SCIENCE IS ART IS SCIENCE IS ART IS SCIENCE IS

glowing image of a pinpoint-sized worm won

A

first place in a scientific society’s contest for

“The Assembly of a Worm Rocket!” is a composite image of Caenorhabditis elegans, a roundworm barely visible to the naked eye, magnified about 40 times and including two stains in addition to GFP. Worm muscle is studied for its similarity to human heart muscle.

Sundaramurthy said the image suggests a rocket and movement.

Upstate’s Sumana Sundaramurthy. She is a

doctoral candidate in cell and developmental biology in the laboratory of David Pruyne, PhD. The American Society for Cell Biology ran a contest in 2019 to celebrate 25 years of green fluorescent protein, or GFP, as a biological tagging tool.

Sumana Sundaramurthy

The society’s December 2019 newsletter ran Sundaramurthy’s photo, “The Assembly of a Worm Rocket!” on its cover. Inside, it ran an additional photo she submitted, titled “Movement of Screw-Propelled Worms!” which was in three-way tie for second place.

PHOTO BY JIM HOWE

20.079102035.165mQMC sk

GFP glows brightly under certain types of light and can be used, for example, to mark and observe a protein within a cell.

GFP can also create striking images, which is what the contest celebrated. u

“Movement of Screw-Propelled Worms!”

MICROSCOPIC PHOTOS BY SUMANA SUNDARAMURTHY, COURTESY OF THE AMERICAN SOCIETY FOR CELL BIOLOGY’S GFP 25 IMAGE AND VIDEO CONTEST