Patient Stories | Mount Sinai Today

A Rare Bladder Condition, and in Need of a Kidney Transplant: Little Lexi’s Extraordinary Medical Journey

Updated on Jun 30, 2022 | Featured, Patient Stories, Your Health

Lexi Isler got a kiss from her father, Luther, before the kidney transplant surgery in January.

In 2009, when a prenatal ultrasound showed an abnormal kidney, Luther and Ashley Isler knew that their daughter, Lexi, would be born with a urinary problem. What they did not expect, however, was that Lexi would also be born without a bladder, a condition known as bladder agenesis that has only 20 known survivors worldwide.

Today, 8-year-old Lexi is among those remarkable survivors as she continues an extraordinary medical journey supported by her loving family and an interdisciplinary team of Mount Sinai physicians that has included pediatric urologists and nephrologists, and transplant surgeons, along with nurses and countless other caring staff over the years.

When Lexi was 2 years old, doctors at Mount Sinai Kravis Children’s Hospital confirmed by magnetic resonance imaging that urine had been backing up into her kidney—because she did not have a bladder. While she was in diapers, not having a bladder was not immediately obvious, but as she grew older, Lexi would need a way to both hold and pass urine. Meanwhile, her kidneys—damaged by her urinary outflow anatomy—had been steadily deteriorating and doctors realized that she would eventually need a kidney transplant.

Lexi’s urology and pediatric doctors formed a close team to strategize about her case from the earliest stages. “Long before a transplant, our team first had to think about how to make a bladder and how to get it to work,” says Jeffrey M. Saland, MD, Chief of Pediatric Nephrology and Hypertension at Mount Sinai Kravis Children’s Hospital and Medical Director of the Pediatric Kidney Transplant Service.

When Lexi was 5 years old, Jeffrey A. Stock, MD, Associate Professor of Urology, and Pediatrics, Icahn School of Medicine at Mount Sinai, and Chief of the Division of Pediatric Urology at Mount Sinai Kravis Children’s Hospital, performed the complex, six-hour surgery to create an artificial bladder.

“We removed part of her colon and connected the ends to create a bowel pouch that functioned as a bladder,” says Dr. Stock. “Then, we used her appendix to create a urethra to drain urine from the neobladder.” The neobladder can store, but not empty, urine so Lexi has learned to use a catheter in the bathroom to empty her new bladder. The neobladder procedure is rare. Dr. Stock, who specializes in the surgical reconstruction of genitourinary birth defects, has done only four such procedures in his 25 years of practice.

By January 2018, Lexi’s kidney function was so compromised that at one point, she was unresponsive for days. The situation remained critical all year with very little improvement, and in December 2018, the need for a kidney transplant could not be deferred. Fortunately for Lexi, when she was 3, her father had been identified as a match for her. “It was an easy decision,” says Mr. Isler. “I felt blessed that I was a match.”

Victoria Caselnova, RN, BSN, left, and Stacey Brooks, RN, BSN, with a smiling Lexi Isler on the day she was discharged from the hospital.

In January 2019, as Lexi was about to go into kidney failure, surgeons from the Recanati/Miller Transplantation Institute at Mount Sinai performed the transplant using her dad’s kidney. “We had all of our firepower available,” says Ron Shapiro, MD, Professor of Surgery, and Surgical Director of the Kidney and Pancreas Transplant Program, referring to the expert team of surgeons involved with the transplant and the bladder creation. A smiling Lexi was discharged after spending nine days in the hospital for the transplant surgery.

Still, says Dr. Saland, “This is a work in progress. Lexi remains at higher risk for urinary tract infections and we’ll need to make ongoing adjustments for drainage as well as in her anti-rejection medications. But at Mount Sinai, we believe we have all the expertise and resources we need to treat all aspects of Lexi’s condition.”

“I have the utmost respect for the doctors and nurses at Mount Sinai,” says Ms. Isler. “They all treated Lexi with so much respect and love. The nurses even came to her bedside when their shifts were over to paint with her.”

Despite periodic hospital stays, Lexi is happy and back at school, facing her health challenges with grace and an amazingly positive attitude for a girl her age, says Ms. Isler. Lexi recently found the courage to stand before her classmates and tell them about the condition she was born with and how it was treated. “Her classmates have been very supportive, and they don’t treat her like she’s different,” says Ms. Isler.

At home, the child who had been taking dance classes since the age of 2 has newfound energy and dances around the house with a lack of inhibition, Mr. Isler says. “With a lot of support, our goal is to enable Lexi to live a long, happy, and healthy life,” says Dr. Stock.

To learn more about organ donation, visit www.LiveOnNY.org.

Learn more about organ donation at LiveOnNY.org

White Coat Event Inspires Nursing Students

Mar 19, 2019 | Community, Patient Stories

The Phillips School of Nursing at Mount Sinai Beth Israel gave students white coats and a mission of compassionate care.

The Phillips School of Nursing at Mount Sinai Beth Israel recently held its second annual White Coat Ceremony for nursing students in the Accelerated Bachelor of Science in Nursing program. The event, held on Tuesday, February 5, at Stern Auditorium, was designed to instill a commitment to providing compassionate care among the next generation of registered nurses.

The White Coat ceremony, long a rite of passage at medical schools, was introduced to nursing in 2014, said Elizabeth Cleek, PsyD, Chief Program Officer and Vice President of the Arnold P. Gold Foundation, a sponsor of the event. Dr. Cleek advised the students in the years ahead “to take that extra moment—despite the pressures, despite the new technologies that may hold some of the answers—to be fully present with the person or people in front of you.”

During the ceremony, each student was cloaked with a white coat provided by the School of Nursing and received a pin with a gold Mobius loop from the Gold Foundation that symbolizes the continuous bond of trust, respect, and communication that connects nurses with their patients.

“I leave you with this quote by Maya Angelou,” said the keynote speaker, Laly Joseph, DVM, DNP, APRN, Senior Associate Dean of the Phillips School of Nursing. “‘They may forget your name, but they will never forget how you made them feel.’”

Todd F. Ambrosia, DNP, APRN, FNAP, Dean of the Phillips School of Nursing, closed the ceremony by reminding students that “while nursing is rooted in knowledge and evidence-based practice, it is equally rooted in caring for the whole person, and not just an illness.” He also encouraged them “to always care for yourselves and for each other.”

A Celebratory Reunion for Pediatric Patients

Mar 19, 2019 | Community, Patient Stories, Pediatrics

Pediatric cardiology patients enjoyed popcorn, cotton candy, and face-painting at the 33rd Annual Valentine’s Reunion Party held on Wednesday, February 13, in the Annenberg West Lobby.

The carnival-themed event brought children and their families together with the doctors, nurses, and medical staff who previously administered vital care.

Organized by the Children’s Heart Center—located within Mount Sinai Kravis Children’s Hospital and part of an alliance with Children’s Hospital of Philadelphia—the festivities were also supported by the nonprofit organizations Project Sunshine, Harboring Hearts, and the Congenital Heart Defect Coalition.

“It is amazing to see the kids we take care of outside the clinical setting,” said Peter Pastuszko, MD, Co-Director of the Children’s Heart Center, and Chief of Pediatric Cardiac Surgery and Director of Pediatric Cardiovascular Services for the Mount Sinai Health System. “We may see some patients only once or twice after they are discharged. Parties like this are the best chance to see the results of our care and what we have been able to give them. It is incredibly rewarding.”

Meriel Simpson, PA-C, Senior Physician Assistant, Pediatric Intensive Care Unit, The Mount Sinai Hospital, with Olivia Malpica.

Peter Pastuszko, MD, with Miriam Pugo and her son Joel Vivar.

Children created their own slime at the event.

Research Is Customizing Artificial Pancreas for Pregnancy

Mar 19, 2019 | Featured, Patient Stories, Research

From left: Carol J. Levy, MD; Camilla Levister, NP; Co-Investigator Grenye O’Malley, MD, Assistant Professor of Medicine (Endocrinology, Diabetes and Bone Disease); and Clinical Research Coordinator Selassie Ogyaadu, MD, MPH.

An artificial pancreas system tailored to the specific, and daunting, challenges faced by pregnant women with type 1 diabetes is the goal of a study to be conducted by a consortium of four leading institutions, including the Icahn School of Medicine at Mount Sinai. The project, funded by the National Institutes of Health, is the first of its kind in the United States.

“Pregnant women with type 1 diabetes typically test their blood sugars seven to eight times per day, and many wear glucose sensors, but they still struggle tremendously to keep their blood sugar levels in target ranges,” says Carol J. Levy, MD, Clinical Director of the Mount Sinai Diabetes Center, and the project’s principal investigator at the Icahn School of Medicine.

If blood sugar levels are too low for a prolonged period of time, a pregnant woman can feel poorly, or pass out, and risks seizures. If levels rise too high, there are risks to her unborn child, including malformations, delayed lung maturity, placental malfunction, or fetal death. “The use of customized technology provides an important opportunity to improve patient and fetal outcomes,” says Dr. Levy, Associate Professor of Medicine (Endocrinology, Diabetes and Bone Disease), Icahn School of Medicine at Mount Sinai. “We are excited to be part of the team evaluating this important area of research designed to improve care and reduce patient burden.”

The clinical trials will be conducted by specialists at three sites: Mount Sinai; the Mayo Clinic in Rochester, Minnesota; and the Sansum Diabetes Research Institute in Santa Barbara, California.

The overall principal investigator is Eyal Dassau, PhD, an expert on algorithm design and Director of the Biomedical Systems Engineering Research Group at Harvard University’s John A. Paulson School of Engineering and Applied Sciences.

Each patient participating in the artificial pancreas portion of the study will wear a Dexcom G6 continuous glucose monitoring device and an insulin pump. Both devices are linked with a software algorithm on a smartphone, which identifies a personalized blood-glucose range and prompts doses of insulin with reduced input from the patient. This is also known as a closed loop system because it uses a customized algorithm to close the decision-making loop between the glucose reading and the delivery of insulin, with the goal of improving blood sugar control.

In the artificial pancreas, or closed loop system, a controller—consisting of a software algorithm on a smartphone—receives readings from a continuous glucose sensor and prompts doses from an insulin pump, with reduced input from the patient.

The only artificial pancreas approved by the U.S. Food and Drug Administration is the Medtronic 670G. But it is designed for blood glucose targets between 120 milligrams/deciliter (mg/dL) and 180 mg/dL, while pregnant women with type 1 diabetes aim for a narrower range—under 90 mg/dL while fasting and 130 to 140 mg/dL one hour after meals.

“This is not easy to do, even for the most meticulous patients,” says Dr. Levy. Another challenge is that hormonal shifts in each trimester change the level of resistance to insulin, making the proper dose “a moving target.”

The first clinical trial in the grant—Longitudinal Observation of Insulin Requirements and Sensor Use in Pregnancy (LOIS-P)—is now enrolling 50 pregnant women with type 1 diabetes and will follow their glycemic outcomes into the postpartum period, providing guidance in refining the algorithm. The trial is named for the late Lois Jovanovic, MD, who was a former director of the Sansum Institute and a role model for many in the study of diabetes, including Dr. Levy. The overall project is intended to progress from a sequence of in-clinic studies to a safe and effective at-home clinical trial.

“I view this work as critical for patients, and I have a personal perspective as well, since I have had type 1 diabetes for 48 years,” Dr. Levy says. “I managed my diabetes with my health care team through two pregnancies, and every day was a challenge. Caring clinicians are important, and any support that people with type 1 diabetes can have provides a huge difference. Every time I enroll a patient in the LOIS-P study, I feel as if we are one step closer to a real answer for many women.”

For more information about the clinical trials, call 212-241-9089.

Continuity of Cardiac Care for South Nassau Patients

Mar 13, 2019 | Featured, Patient Stories

Patient Bill Regan with, from left: Robin Varghese, MD; Pilar Stevens-Haynes, MD; and Jason
Freeman, MD—members of the coordinated team that treated him on Long Island and in Manhattan.

Even before an official partnership was finalized in December 2018, specialists from the Mount Sinai Health System were collaborating with physicians at South Nassau Communities Hospital to provide advanced and coordinated care to Long Island patients. For 67-year-old Bill Regan, a retired financial services executive from Massapequa, this new relationship proved to be life-saving.

It was in March 2018 that Mr. Regan decided to try a new primary care physician—this time, at South Nassau Primary Care at Bellmore. During his first visit to Carolyne McHyman, DO, Mr. Regan provided the details of his medical history, which included a carotid endarterectomy, a surgical procedure to clear blockages in the carotid artery in the neck, and prescriptions for high blood pressure and high cholesterol. The former long-time smoker told Dr. McHyman that he was “generally in good health,” but when he also mentioned some daytime fatigue and left leg discomfort when he walked long distances, she ordered several diagnostic tests.

They included a chest CT scan, as part of The Mount Sinai Hospital’s Early Detection Lung Cancer Screening program; an ultrasound of the abdominal aorta to check circulation and to rule out an aortic aneurysm, an abnormal bulge in the aorta; a consultation to rule out a sleep disorder; and a recommendation to see his cardiologist, Pilar Stevens-Haynes, MD, for an evaluation.

The CT scan of the lungs, while negative for lung cancer, showed calcification in his aorta. The ultrasound of the abdominal aorta also showed narrowing of the left iliac artery in his pelvis, while a magnetic resonance angiography, which provides detailed images of blood vessels in the body, revealed a 90 percent blockage of that artery. “Honestly, I didn’t worry about my health, but these test results blindsided me,” recalls Mr. Regan.

Meanwhile, Dr. Stevens-Haynes recommended a cardiac catheterization to check for any blockages of his coronary arteries. “Bill didn’t have previous symptoms of chest pain, but coronary artery calcification can be associated with major adverse cardiovascular events, and we needed to get to the bottom of it,” says Dr. Stevens-Haynes.

When Jason Freeman, MD, Director of Interventional Cardiology at South Nassau Communities Hospital, performed the cardiac catheterization on April 24, he discovered that Mr. Regan’s left main and circumflex coronary arteries that supply blood to his heart were significantly blocked and could not be treated with stents, expandable tubes used to open clogged arteries. Now, Mr. Regan would need open-heart surgery—a diagnosis that was made immediately by Robin Varghese, MD, Associate Professor of Cardiovascular Surgery at the Icahn School of Medicine at Mount Sinai, who was at South Nassau that day.

South Nassau’s Center for Cardiovascular Health has long been known for its outstanding care. It is a recipient of Healthgrades’ 2019 Coronary Intervention Excellence Award and five stars for its performance in coronary interventional procedures, placing it among the top 10 percent in the nation; however, it does not perform cardiac surgery.

“When I met with Dr. Varghese, the heart surgeon, I was very anxious, but I came out feeling this is going to be okay,” Mr. Regan says. “He walked me through what the procedure entailed. He had a diagram of the heart and the arteries, and he was positive, calming, and reassuring. He made me feel like ‘we got this and this is what we do every day.’”

Long Island patient Bill Regan was able to receive postoperative care, including cardiac rehabilitation, close to home in South Nassau.

On April 30, Dr. Varghese and his team at The Mount Sinai Hospital performed the three-hour surgery to restore blood flow to Mr. Regan’s heart. “After surgery, we took Bill to our Cardiac Surgery Intensive Care Unit,” says Dr. Varghese, who is also Director of Cardiovascular Critical Care for the Mount Sinai Health System. “He was sitting up in the chair in no time and looking great. He surprised us all.” Within four days, Mr. Regan was released from the hospital and was back home on the South Shore under the supervision of South Nassau’s Homecare program, attending church two days later in Massapequa.

“My entire experience was coordinated and supported by the most wonderful people from South Nassau and Mount Sinai,” says Mr. Regan. “I feel blessed, thankful, and overwhelmed with gratitude to everyone, and I mean everyone, involved in my treatment and recovery at South Nassau and Mount Sinai.”

After he was released from South Nassau’s Homecare program, vascular surgeon Xenophon Xenophontos, MD, performed a successful angioplasty of the left iliac artery. Mr. Regan also participated in South Nassau’s Cardiac Rehabilitation program at South Nassau’s Sports Medicine and Rehabilitation Therapy (SMART) Center in Oceanside, which he completed three months ago. Mr. Regan says he plans to return to the SMART Center this month for additional exercise programs.

Having access to advanced specialty care at The Mount Sinai Hospital while being able to stay close to home for pre- and postoperative care offers many benefits for patients. “Continuity of care is a key component of quality of care, especially when treating patients with multiple conditions,” says Adhi Sharma, MD, Executive Vice President for Clinical and Professional Affairs and Chief Medical Officer at South Nassau. “The care was not only life-saving for Bill, but a seamless journey and patient experience.”

New Program Achieves Early Benchmarks For Improving Patient Care Post-Discharge

Mar 6, 2019 | Featured, Patient Stories

The Transitions of Care Center team includes, from left: Amanda Anderson, MSN, MPA, RN; Claudia Colgan; and Eunice Reynoso, BSN, RN, Clinical Nurse Manager.

When a patient is admitted to a hospital within the Mount Sinai Health System, planning for discharge begins almost immediately to ensure a smooth transition to the next level of care, whether it is at home or at a skilled nursing facility. Studies show that effective discharges lead to improved patient health, reduced readmissions, and decreased health care costs.

Toward that end, the Mount Sinai Health System has established the Transitions of Care Center (TOCC), a centralized telephone-based discharge program staffed by Mount Sinai registered nurses who are specially trained in hospital discharge protocols and are led by a clinical nurse manager.

The TOCC has been tasked with improving patient satisfaction, ensuring patients have access to post-hospital care and are following discharge instructions, and preventing avoidable readmissions. Staff work from a dedicated office on East 16th Street near Union Square in Manhattan, where 11 registered nurses call Mount Sinai patients 24 to 72 hours after they leave the hospital—reviewing and addressing each patient’s unique discharge plan of care.

The Center initially was launched in December 2016 as a pilot program aimed exclusively at patients deemed at high risk for rehospitalization. The program quickly expanded and is now servicing patients regardless of readmission risk or diagnosis who are discharged home from The Mount Sinai Hospital, Mount Sinai Queens, Mount Sinai West, and Mount Sinai St. Luke’s. In March, the TOCC will add discharged patients from Mount Sinai Brooklyn, with a goal to expand to Mount Sinai Beth Israel in the coming months.

Since the program’s start, nurses have made more than 40,000 phone calls, spoken to nearly 19,000 patients, and provided close to 11,000 interventions to assist patients with post-discharge care needs. “The results have been positive,” says Claudia Colgan, Vice President, Care Coordination, Mount Sinai Health System, and Vice President, Operations, The Mount Sinai Hospital. “In the 12 months leading up to August 2018, patients who were contacted by the TOCC had a 16 percent lower-than-expected readmission rate.”

The Transitions of Care Center nursing staff includes Verisa Robinson BSN, RN, center, and from left: Alla Aminova, BSN, RN; Maki Lahori, BSN, RN; Kristina Davanzo, BSN, RN; Amanda Mendez- Paranavitana, BSN, RN; Connie Fong, BSN, RN; and April Schott-Auerbach MSN, RN, CNL.

While speaking to patients, nurses assess their health status and determine if they are adhering to their discharge care plan. They ensure that patients have filled their prescriptions and are taking their medications properly, that they have the medical equipment they need, and that they are able to attend follow-up appointments. As needed, they perform nursing triage for active symptoms, referring patients to in- and out-patient providers. They work closely with interdisciplinary care management teams at the hospital where the patient was treated and collaborate with ambulatory teams, home health agencies, and community-based service organizations.

According to TOCC data from January 2017 through November 2018, nurses had 6,290 patient interventions that involved providing educational resources and reinforcing overall discharge instructions, and 1,013 interventions that resolved medication needs. They also helped escalate the management of active symptoms for 825 patients.

“No matter how well the discharge team explains next steps of care, patients often do not fully understand or remember what to do,” says Ms. Colgan. “This is pervasive across the entire patient population, regardless of education or language proficiency or socioeconomic status. At Mount Sinai, we understand that they may be overwhelmed with new directives.”

Mount Sinai is one of the few facilities in the country with a dedicated team of on-staff nurses whose full-time job is to call patients. “Our success lies with our registered nursing team,” says Amanda Anderson, MSN, MPA, RN, Associate Director of Care Transitions for the Mount Sinai Health System. “The TOCC nurses are highly trained by Health System service-line leaders. They understand the Mount Sinai culture of putting patients first, and they collaborate with other hospital staff on the patient’s behalf when necessary.”

“It’s great when we are able to triage and provide a service to someone who really needs it,” says TOCC Senior Nurse April Schott-Auerbach, MSN, RN, CNL. “One of my patients was experiencing increasing shortness of breath and it was clear he needed assistance but was unable to get to an urgent care facility or go back to the emergency room. I was able to triage and collaborate with the Health System Community Paramedicine team, which provided the patient with the more immediate assessment he needed.”

According to Ms. Schott-Auerbach, patients are happy knowing that someone is following up with them. One of her patients told her, “I never had such amazing service from other hospitals. It really makes me feel like Mount Sinai cares about me.”

A team picture: The Transitions of Care team.

« Older Entries

Next Entries »