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E-News for Referring Offices

Cancer Predisposition Clinic Aims to Change the Trajectory of Genetically Based Cancer

At least 10% of children with cancer harbor a disease-associated pathogenic variant in a known cancer predisposition gene. While few can be prevented, regular surveillance can, at the very least, find malignancies early when treatment is most effective.

That’s the idea behind Children’s of Alabama’s Cancer Predisposition Clinic, now in its third year.

“We provide comprehensive care and screening for these patients with the goal of detecting cancers at an early stage in order to provide the best care and outcomes for the patients,” said Elizabeth Alva, M.D., assistant professor in the University of Alabama at Birmingham (UAB) Division of Pediatric Hematology and Oncology. “It used to be there was nothing we could do about this terrible diagnosis,” she said. “Now we know there are definitely ways to look for cancers early and help patients.”

In addition, the clinic provides psychological support for families and determines if the genetic condition affects other family members.

Typically, primary care physicians follow children with cancer predispositions. But Alva provides another level of evidence-based surveillance that the pediatric offices may not be able to offer.

That’s why these clinics are a growing area in the pediatric hematology/oncology world, particularly at the larger children’s hospitals. “We felt that we definitely needed to provide that same level of care here in Alabama,” Alva said.

Alva and neuropsychologist Avi Madan-Swain, Ph.D., are currently following about 25 patients. Patients come to them through the Pediatric Cancer Genetics Clinic, where families are tested and counseled. Alva provides the screening, which ranges from regular ultrasounds to CT scans and MRIs, while Madan-Swain addresses the family’s psychological needs.

One benefit to the clinic is that if there is a cancer diagnosis, the child and family are already comfortable with the hospital and the clinical team.

Alva is building a database of patients to gain a better understanding of disease development and progression, while Madan-Swain plans research around the psychological impact on families that have a child with a predisposition syndrome.

Nephrology

Cutting Out Sugar Intake, One Kid at a Time

The average American consumes almost 152 pounds of sugar a year, about three pounds a week or 42.5 teaspoons a day — more than triple the recommended amount.[1] While sugar consumption isn’t the only cause of the country’s obesity epidemic, it is definitely a major contributing factor — particularly in children. And the problem is not only obesity, says pediatric nephrologist Daniel I. Feig, M.D., Ph.D., who directs the University of Alabama at Birmingham (UAB) Division of Pediatric Nephrology at Children’s of Alabama, but all the downstream health effects of being overweight, including cardiovascular disease, hypertension, liver disease, kidney disease and type 2 diabetes.

One reason for the high sugar intake is economic. Over the past 35 years, the price of fruits and vegetables has tripled, he said, while the price of sugar-sweetened foods such as beverages fell 75%. “The availability of calories and nourishment in a low-sugar fashion is much more expensive than it was a few decades ago,” he said. “We can talk until we’re blue in the face in low-income, urban clinics about eating fruits and veggies, but that isn’t the only barrier to kids not eating them; their families can’t afford it.

Then there’s the issue of high-fructose corn syrup (HFCS), used as a sweetener and preservative in many foods. Research from Feig and others has found that HFCS is not simply sugar in another form but has a high relative fraction of fructose compared to glucose, which alters cellular carbohydrate metabolism. This results in a greater rise in triglycerides and uric acid than with sugar from sugar cane or sugar beets.

Researchers have also demonstrated that high levels of uric acid stiffen and thicken blood vessel walls, resulting in hypertension, as well as activating the renin-angiotensin system system, causing immediate vasoconstriction.

Clinical trials find that lowering uric acid levels in hypertensive adolescents, but not adults, improves blood pressure. “So we have a window of opportunity in children to reduce their long-term cardiovascular and renal risk factors by controlling sugar intake,” Feig said.

That’s why clinicians and nutritionists at the hypertension clinic at Children’s counsel patients and their families about the effects of sugar as well as where the sugar is found (i.e., the sweet tea that is ubiquitous throughout the South). “Adolescents get about 48% of their sugar from sugar-sweetened beverages,” Feig said, “so it isn’t a function of just telling them not to eat candy.”

“When I see a child in our hypertension clinic with obesity-related hypertension, about a third of the time very high sugar and caloric intake in their beverages, up to 2,000 calories a day, is a major contributing factor,” he said. “Simply eliminating those liquids could make a huge difference in their health.”

He cites a recent study that polled new parents about the sugar content of various foods. More than 80% of parents underestimated the sugar content of foods with a “health halo,” like fruit juice and yogurt. “We have an educational deficit in terms of dietary literacy,” he said.

“So a big push in our clinic is helping families learn more about the nutritional content of food.”

Blood Pressure Control

Learn more about the hypertension clinic at Children’s of Alabama.


[1] Department of Health and Human Services. How Much Sugar Do You Eat? You May Be Surprised! https://www.dhhs.nh.gov/dphs/nhp/documents/sugar.pdf.

Cardiology, Uncategorized

Understanding Xenotransplantation’s Potential to Save Babies

The issue is simple: there are simply not enough hearts for all the children who need them. So 17% of all children who need a heart transplant die while waiting; this translates to 20% to 25% of infants.[1]

The University of Alabama at Birmingham (UAB) and Children’s of Alabama aim to change those dismal statistics with one of the most revolutionary approaches since the first heart was transplanted from one human to another in 1967 – xenotransplantation.  

Thanks to a $19.5 million grant from biotechnology magnate United Therapeutics Corporation, UAB and Children’s have launched one of the top programs in the world dedicated to developing genetically modified solid organs from pig models for transplantation.

The idea isn’t new. Pig tissue has been used to replace heart valves for years, said cardiothoracic surgeon David Cleveland, M.D., MBA, who leads the program at Children’s. The greatest challenge with solid organs, he said, is overcoming immunological and physiological barriers.

If they can do that, “We believe that there’s huge potential to improve the lives of children,” he said.  

Supporting Evidence

Earlier this year, Cleveland presented preliminary results from a study showing little reactivity in an infant’s blood to cells from a triple-knockout (TKO) pig. The pig had been genetically modified to delete the three major antigens that react with natural human anti-pig antibodies. Even those human cells that did react demonstrated a very mild reaction.

“We found that very promising,” Cleveland said.

Another area of interest is producing immune tolerance by transplanting porcine thymus tissue to “re-educate” the immune system to accept the pig heart, said cardiac intensivist Leslie Rhodes, M.D. The idea comes from the fact that children can develop an immune system via a human thymus transplant. “We wonder if we could we train their immune system to be tolerant to the pig thymus transplant,” she said.

Infants are the ideal starting place, Cleveland said, not only because they have the highest wait list mortality of any other demographic waiting for a solid organ transplant, but because their immune systems are still naïve. Indeed, they do not develop antibodies to pig glycans during at least the first three months of life, Cleveland and his team wrote in a recent journal article, providing a “window of opportunity” for the transplant.[2]

The next step is a transplant in a non-human primate. “The FDA won’t even consider it until we can prove consistent survival in a non-human primate,” Rhodes said. They hope to perform their first transplant later this year.

Societal Concerns Addressed

The team is also aware of the societal issues around xenotransplantation. To address that, they surveyed the families of patients on the transplant list and the nurses and physicians who will care for these children.

”I was surprised by how positive they were,” Cleveland said. “I thought there would be more pushback than there was.” Still, he said, “I think there has to be major education,” once xenotransplantation becomes a reality. “The idea of replacing a heart with a pig heart will take some people a little time to get over.”

He’s confident it will happen, though. “UAB is going to be one of the centers in the world with the potential to make this happen,” he said. “We have children living in our ICU because there’s not enough cardiac function; they are having their birthdays here. It totally changes entire families to have a child in the hospital forever. There has to be another way.“


[1] Dipchand AI. Current state of pediatric cardiac transplantation. Ann Cardiothorac Surg. 2018;7(1):31–55. doi:10.21037/acs.2018.01.07

[2] Cleveland D, Adam Banks C, Hara H, Carlo WF, Mauchley DC, Cooper DKC. The Case for Cardiac Xenotransplantation in Neonates: Is Now the Time to Reconsider Xenotransplantation for Hypoplastic Left Heart Syndrome? Pediatr Cardiol. 2019;40(2):437-444.

Cutting-Edge Research

Learn more about various research areas at the University of Alabama at Birmingham.

Pulmonology

Using Antisense Oligonucleotides to Improve CFTR Function in Cystic Fibrosis

oligotherapeutics research

Therapeutic development in cystic fibrosis (CF), a fatal pediatric lung disease affecting 1 in 3500 newborns annually, is rapidly advancing. “Although exciting progress has been made,” said Children’s of Alabama pediatric pulmonologist William T. Harris, M.D., an associate scientist at the Gregory Fleming James CF Research Center at the University of Alabama at Birmingham (UAB) who treats patients at Children’s of Alabama, “we are just midstream.” 

Where previous advances focused on the downstream consequences of disease, such as malnutrition, chronic infection and mucopurulent secretion, recent drug developments target genetic mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein itself. Dr. Harris’ research focuses on improving the efficacy of these agents, called CFTR modulators.

One key therapeutic clue is to identify why certain children with exactly the same CFTR genotype have widely different disease trajectories. Dr. Harris has studied the mechanisms behind this disease disparity and has discovered the mechanism through which transforming growth factor beta (TGF-β), a leading gene modifier of CF lung disease severity, inhibits CFTR functional expression. Dr. Harris now targets this mediator of disease progression as a therapeutic opportunity to optimize CFTR modulator response.

His research discovered that small, non-coding nucleotide sequences called microRNA (miRNA) regulate CFTR function. miRNA diminish gene expression by degrading the gene transcript or inhibiting protein translation. In CF, TGF-β stimulates miR-145 expression, which binds to and degrades the CFTR gene transcript. This prevents protein expression and diminishes channel function. Both TGF-β and miR-145 are markedly increased in CF lungs and airway epithelia, posing a significant barrier to effective intervention. 

Introducing miR-145 antagonists to airway epithelia reverses TGF-β suppression of CFTR and potentiates CFTR modulator response. However, TGF-β signaling and miR-145 activity are involved in multiple functions throughout the body, raising concern for off-target consequences. “As CF outcomes improve, the tolerance for side effects becomes very low,” he said. “Thus, the therapeutic intervention must be highly specific with a clearly defined target that only blocks the effects on CFTR.”

Antisense oligonucleotides (ASOs) offer that option. These short nucleic acid sequences bind to specific molecules of RNA, regulating expression of the gene. The FDA has already approved a handful of ASOs to treat congenital pediatric diseases such as Duchenne muscular dystrophy and spinal muscular atrophy.

 “ASOs are appealing in CF because they can be delivered directly to the lungs (via inhalation) to bypass systemic side effects,” Dr. Harris said. He is partnering with Ionis Pharmaceuticals, a leader in the development of ASOs, to test an ASO that prevents miR-145 binding to the CFTR transcript. This approach is called target site blockade (TSB).

“TSB offers a nuanced strategy to address the problem of CFTR inhibition without interrupting TGF-β/miR-145 availability for other regulatory processes,” he explained.  “I expect oligotherapeutics to benefit CF patients across genotype and improve next-generation therapeutics whether that be small molecule correctors or evolving gene editing strategies.”

Get More Information
Learn more about the Cystic Fibrosis Center’s resources for physicians.

Hematology and Oncology

Cancer Predisposition Clinic Aims to Change the Trajectory of Genetically Based Cancer

Li-Fraumeni syndrome. Von Hippel-Lindau. Rhabdoid tumor predisposition syndrome. Beckwith-Wiedemann syndrome. Retinoblastoma.

These are just a few of more than a dozen rare genetic conditions that predispose a child to cancer. Overall, at least 10% of children with cancer harbor a disease-associated pathogenic variant in a known cancer predisposition gene. While few can be prevented, regular surveillance can, at the very least, find malignancies early when treatment is most effective.

That’s the idea behind Children’s of Alabama’s Cancer Predisposition Clinic, now in its third year.

“We  provide comprehensive care and screening for these patients with the goal of detecting cancers at an early stage in order to provide the best care and outcomes for the patients,” said Elizabeth Alva, M.D., assistant professor in the University of Alabama at Birmingham (UAB) Division of Pediatric Hematology and Oncology. “It used to be there was nothing we could do about this terrible diagnosis,” she said. “Now we know there are definitely ways to look for cancers early and help patients.”

In addition, the clinic provides psychological support for families and determines if the genetic condition affects other family members, she said.

Typically, primary care physicians follow children with cancer predispositions. But those doctors may not be aware of or able to provide the level of evidence-based surveillance that Alva offers.

That’s why these clinics are a growing area in the pediatric hematology/oncology world, particularly at the larger children’s hospitals. “We felt that we definitely needed to provide that same level of care here in Alabama,” Alva said.

Alva and neuropsychologist Avi Madan-Swain, Ph.D., are currently following about 25 patients. Patients come to them through the pediatric cancer genetics clinic, where families are tested and counseled. Alva provides the screening, which ranges from regular ultrasounds to CT scans and MRI, while Madan-Swain addresses the family’s psychological needs.

One benefit to the clinic is that if there is a cancer diagnosis, the child and family are already comfortable with the hospital and the clinical team.

Alva is building a database of patients to gain a better understanding of disease development and progression, while Madan-Swain plans research around the psychological impact on families that have a child with a predisposition syndrome.

Understanding Genes
Learn more about the UAB Department of Genetics.

Neonatology

Initiative Targets Pain Management in NICU Babies

neonatology_pain

Adults and children can tell you when they’re in pain. Infants can’t. Which is why Children’s of Alabama is participating in a national quality improvement initiative called Erase Post-Op Pain designed to reduce pain after invasive procedures. The initiative is part of the Children’s Hospital Neonatal Consortium (CHNC), an international group of children’s hospitals dedicated to improving care in the neonatal intensive care unit (NICU).

“There is really no ‘gold standard’ for pain assessment in preverbal children,” said NICU Associate Medical Director Allison Black, M.D. “Nor is there much data on the best way to treat pain in neonates.” However, there is data showing that preterm  babies who experience repeated pain can develop physiologic instability, altered brain development and abnormal stress response systems that persists into childhood. “The immature brain can potentially have a more diffuse and exaggerated response to pain,” she said.

The Erase initiative is designed to apply a multidisciplinary approach, including physicians, bedside nurses, pharmacologists, and even parents, to implement a standardized method to assess, document and manage postoperative pain.

The first action the team took was to adopt a single objective pain assessment tool, the N-PASS score, which measures sedation and pain based on vital signs such as heart rate and breathing, as well as behavior such as agitation, crying, facial expressions and neurologic resting tone. “These are things parents can help us assess as well,” Black said. Parents will also complete a survey after each procedure about how well they thought their baby’s pain was assessed and controlled.

The NICU pharmacist worked closely with other team members to develop different guidelines and different algorithms of what medications to use for each specific patient. Each guideline is unique, and the algorithm used depends upon the invasiveness of the procedure, whether the patient has had similar drugs in the past and if they are breathing spontaneously or with the help of assisted ventilation.

“By considering the history of the patient, the type of procedure performed. and looking closely at each drug’s  time to onset and duration of action, the treatment should be more effective,” Black said.

The initiative dovetails nicely with another CHNC performance improvement project, the STEPP-IN initiative. STEPP-IN works to reduce perioperative stress and instability  in NICU patients through improved handoffs and communication. “I think the projects will compliment each another and help improve our overall care of these small infants during the high-risk perioperative period,” Black said.

Babies in Need

Learn more about the Neonatal Intensive Care Unit at Children’s of Alabama.

Pulmonology

Secondhand Smoke Exposure in Kids with Cystic Fibrosis May Impact Treatment Efficacy, Researchers Suspect

The introduction of cystic fibrosis transmembrane conductance regulator (CTFR) modulators, which target the basic genetic defect in cystic fibrosis (CF), has revolutionized the treatment of the disease over the past five years. With a new, triple CTFR modulator expected to be approved by the end of 2019, in the next year, 90% of those with CF may benefit from these new drugs. However, studies of currently available modulator therapies find that between 20% to 25% of patients who should respond based on their disease’s genetic fingerprint don’t.[1][2]

Researchers from the University of Alabama at Birmingham (UAB) think they may know why: patients’ exposure to secondhand smoke. Now they have embarked upon a study to test this hypothesis.

While it might seem counterintuitive that families with a child with CF would expose them to secondhand smoke, approximately one-third of pediatric CF patients are exposed to tobacco smoke, half of whom have been around a smoker in the past 3 months.[3] And yet, said Gabriela Oates, Ph.D., assistant professor in the UAB Division of Pediatric Pulmonary and Sleep Medicine at Children’s of Alabama and an associate scientist in the UAB Cystic Fibrosis Research Center, “many think their child isn’t exposed to the smoke if the family member is smoking outside.”

But that’s simply not true.

“We’re not just talking about secondhand smoke but also about thirdhand smoke exposure,” Oates said. Tobacco particulates remain on the hair, skin and clothes of the smoker, even if he or she smokes outside, and are also found on household surfaces. “You can find relatively high level of nicotine metabolites in the urine of kids whose parents smoke out of doors,” she said. In fact, children demonstrate exposure even if their household members do not smoke but they live in multifamily housing that shares a wall with a smoking household.

This all ties into the new CFTR modulators because in-vitro, animal and non-CF studies indicate that “even indirect exposure to tobacco smoke actually blunts the effect of the drug,” she said. So while the CFTR modulators are designed to correct the underlying genetic mutation that causes the disease, “the smoke exposure undermines that.”

Her project will define the consequences of secondhand smoke on CF respiratory decline and CFTR modulator response using both self-reported and objective measures of exposure such as urine biomarkers. Results will underscore the necessity of clinically driven smoking cessation programs for CF families and will inform recommendations for smoke exposure screening and control.

Given that most children exposed to smoke are clustered in the low-income segment of the CF population, this becomes a health equity issue, Oates said. “It’s particularly concerning because the smoke exposure may be outside of the household and there’s nothing the family can do about it,” she said. “I worry that in the era of CFTR modulators we may see an increased gap in CF outcomes between kids living in poorer environments and their advantaged counterparts. This issue needs to be watched carefully.”

Oates also fears that if her hypothesis is supported, payers may institute smoke exposure screening programs and base drug coverage on the results. This creates quite the conundrum for researchers like herself. “As scientists, we have a responsibility to determine why drugs work or don’t work,” she said, “yet we have little control over how the results of our science are used.” If her study does show that smoke exposure limits the benefits of CFTR modulators, she said, “the very first step is major education on several levels, including CF families, clinicians and insurance providers.”

Her team is being proactive in this regard, already holding interviews with current and former smokers who have a family member with CF, as well as with CF clinicians and other stakeholders. The goal is to develop materials to better inform caregivers and clinical providers about the impact of second-hand smoke and to test a smoking cessation intervention tailored to CF families. “You would be amazed that there is not a single U.S. study evaluating smoking cessation programs in the CF community,” Oates said.


[1] Hebestreit H, Sauer-Heilborn A, Fischer R, Kading M, Mainz JG. Effects of ivacaftor on severely ill patients with cystic fibrosis carrying a G551D mutation. J Cyst Fibros. 2013;12(6):599-603.

[2] Taylor-Cousar J, Niknian M, Gilmartin G, Pilewski JM, investigators VX. Effect of ivacaftor in patients with advanced cystic fibrosis and a G551D-CFTR mutation: Safety and efficacy in an expanded access program in the United States. J Cyst Fibros. 2016;15(1):116-122.

[3] Ong T, Schechter M, Yang J, et al. Socioeconomic Status, Smoke Exposure, and Health Outcomes in Young Children With Cystic Fibrosis. Pediatrics. 2017;139(2).

Breathe Easier

Learn more about the Cystic Fibrosis Center at Children’s of Alabama.

Hematology and Oncology

Meet Girish Dhall, MD, the New Division Director for the Hematology, Oncology, and Blood & Marrow Transplantation Program

Girish Dhall, M.D.

In January, Girish Dhall, M.D. was named division director for the Hematology, Oncology, and Blood & Marrow Transplantation program in the University of Alabama at Birmingham Department of Pediatrics and Children’s of Alabama. In May, he and his wife moved from Los Angeles, where he was an associate professor of pediatrics and director of the Neuro-oncology Program at Children’s Hospital Los Angeles, to Birmingham.

We talked to Dr. Dhall about his vision for the department and the transition to Alabama.

Q: What brought you to Children’s UAB?

A: What brought me here was the quality of the program at Children’s Hospital and UAB and the people here. I was looking for a new challenge and this program was looking for a leader who could help build and elevate the program. When I came here, I was really impressed by the high caliber of the people here, not just in the clinic but also in the research arena, as well as the existing infrastructure at Children’s and UAB, which made my decision quite easy.

Q: What do you mean by infrastructure?

A: The ability to conduct clinical trials and research is very solid on the UAB side, and the clinical care is very strong on the Children’s side. Plus, there are so many subspecialists you don’t have to send patients out of the hospital, which makes treating patients with chronic conditions like cancer much easier.

Q: What’s your top priority for the division of heme-oncology as its new leader?

A: My biggest priority is to build a well-rounded program — a program that is not only strong in its clinical mission, which is to provide the best treatment possible for children with cancer and blood diseases, but one that also has a strong research base, which includes clinical, basic, and translational research.

We already have a strong clinical program with top-notch faculty providing outstanding care to our patients as well as a strong clinical and translational research programs in the area of brain tumors, sickle cell disease, and survivorship. I hope to build on the existing strengths of the program and add clinical and translational research programs in leukemia and sarcomas. Eventually, my hope is that these efforts will lead to providing more cutting-edge therapies for children with cancer and blood disorders in the state of Alabama and the surrounding area for decades to come.

Q: Where do you see the fight against cancer in 10 years?

A: Over the last five decades, we have made significant strides in our fight against cancer. We are now able to cure approximately 80% of all childhood cancer patients compared to 10% in in the 1960s. However, the therapies we use, such as radiation therapy and chemotherapy, sometimes have lasting side effects on our patients, especially young children.

In the last decade or so, there has been an explosion of scientific techniques that have helped us understand the biology of these cancers and what makes them grow. In the next 10 years, I hope that we will have a shift in designing treatments that are directed specifically at the cancer cells and genetic derangements within them and spare normal organs and tissues, i.e., provide therapies that are more effective and less toxic.

Q: You chair the Young Investigator’s Committee for Children’s Oncology Group. What advice do you have for young researchers?

A: Research can be extremely, extremely frustrating in the sense that it takes a long time to complete and obtaining funding is a huge challenge because it’s extremely competitive. So my advice is don’t give up.

Q: What are you most excited about regarding living in Birmingham?

A: We’re having a great time. We go on a walk with our dog in the evening and we do less walking and more talking with the neighbors. We love the people and their Southern hospitality, the bar-b-que, the blue skies and less traffic.

Q: And the summer heat?

A: Everyone told us July and August would be a killer, but it hasn’t been that bad.

The Latest on Transplantation

Learn more about the UAB Blood and Marrow Transplantation & Cellular Therapy Program.

Nephrology

Children’s of Alabama Becomes First to Safely Provide Dialysis to Tiny Babies

Didactic and hands-on teaching on the use of CRRT using the Aquadex Pureflow.

Despite the frequent use of dialysis for critically ill children and adults, the procedure has historically been used sparingly in neonatal intensive care units (NICU) because dialysis  machines designed for adults can cause severe complications in babies. That’s no longer the case at Children’s of Alabama.

The problem is that continuous renal replacement therapy in these tiny patients requires at least 100 ml of blood to initiate the therapy. This can be half or even more of the baby’s entire blood volume, said David Askenazi, M.D., MSPH, who directs the Pediatric and Infant Center for Acute Care Nephrology. “Many times, when we started the machine, we had to open the crash cart to resuscitate infants who were coding,” he said.

That changed in 2013, when Askenazi realized that a machine designed to remove fluid and sodium from blood in adults with heart failure — the Aquadex FlexFlow® System — could be repurposed for neonate dialysis.

“If we could adapt a machine that requires one-third of the blood of the traditional machine volume to do what we needed, we knew we could improve our ability to support these babies,” he said. So the team learned as much as they could about the device, developed a safety net of processes to maximize the likelihood of success and convinced the hospital to buy its first machine.

Today, the hospital has fiveAquadex machines and two or three babies are typically receiving dialysis at any one time. “Now we have complete control over their fluids, electrolytes and waste products,” Askenazi said, “while the nurses feel comfortable doing the therapy and the babies don’t even know they’re on it.” Last year, babies in the NICU spent a total of 800 days on dialysis compared to just 30 days in 2013.

“For our babies born with diseased or absent kidneys, Aquadex has given them a chance at life,” said NICU nurse practitioner Kara Short, MSN, CRNP, “because in the past, there were no options to treat these patients.”

The team published the results of its first 12 patients in the journal Pediatric Nephrology in 2016. Since then, they have treated more than 90 patients, the smallest just 1.2 kg (2 pounds, 7 ounces) and taught nephrologists at several other children’s hospitals around the country to use the Aquadex. However, there are still only a handful of hospitals offering the procedure.

“We have shown we can now support these babies safely,” Askenazi said. “The impetus now is on us to make sure the patients who can benefit from this therapy make it to Children’s so we can give them a chance for life.”

And the machine’s manufacturer? It is now pursuing a pediatric indication for Aquadex.

A Team Effort

Learn more about the neonatology program and team at Children’s of Alabama.

Cardiology

Children’s of Alabama CVICU Embraces Quality Improvement Projects

What if you could proactively identify patients who might go into cardiac arrest and intervene before the unthinkable happens? If you do what Children’s of Alabama did, you end up with fewer children having arrests and improved response times because of faster medication administration when an arrest does occur.

That’s all thanks to a multi-institutional quality improvement project — Cardiac Arrest Prevention (CAP) — centered around a resuscitation action plan, which focuses on rescuing patients before they arrest.

The project, led by cardiovascular intensive care unit (CVICU) Medical Director Santiago Borasino, M.D., and cardiac intensivist Hayden Zaccagni, M.D., is part of the Pediatric Cardiac Critical Care Consortium (PC⁴), made up of 52 of the country’s top children’s hospitals. The consortium maintains a focused CVICU registry designed to share real-time data and outcomes between institutions and participates in quality improvement projects to improve outcomes.

The CAP project is just one of the data-driven, collaborative learning initiatives the group has implemented.

Comprehensive Effort

“CAP is a joint effort with bedside nurses, respiratory therapists, administrative nursing staff and trainees, whether fellows or advanced practice practitioners, to not just identify at-risk patients but have a common mind-set and goals to prevent arrests,” Zaccagni said.

Once a patient meets certain criteria putting them at risk for cardiac arrest, the attending intensivist completes a paper report that remains bedside. Clinicians then round separately on these patients and, if warranted, give the bedside nurse the ability to start the treatment plan without waiting for separate orders. Resuscitation medications are kept at the bedside for immediate use if the patient demonstrates any danger signs. “The goal is to expedite interventions to prevent the arrest from occurring,” Zaccagni said.

Borasino, in collaboration with two former Children’s intensivists, Kimberly Jackson, M.D., and Jeffrey Alten, M.D., started the original resuscitation program. “Dr. Alten was our medical director and section chief until 2017 and he was instrumental in starting this project and then taking it to the national stage,” Borasino said, while Jackson coordinated the local effort when it began in 2013. Alten, now at Cincinnati Children’s Hospital Medical Center, is still the coordinating head of the national initiative, while. Jackson has moved to Duke University Medical Center in Durham, North Carolina, where she leads its initiative.

The official PC4 initiative began in October 2018, but Children’s had something similar in place for three years, Zaccagni said. However, because the PC4 initiative includes more than 10,000 patients, there is more data available on best practices. So, for instance, Children’s adjusted the bedside rescue medications so they are easier to deliver.

Although the new initiative had only been in place eight months when this article was written, “anecdotally, I’d say we’ve reduced the number of cardiac arrests,” Borasino said.

More Quality Improvement Initiatives

Two other quality improvement projects are also demonstrating results:

Star Track. Geared towards less-complicated patients who have cardiac surgery, this initiative involves standardizing patient care to remove unnecessary equipment sooner. This improves patient comfort and enables them to transfer soon out of the CVICU. “The patient comfort is our main goal,” Borasino said. “Patients undergoing these types of surgeries are older and don’t require the level of invasive monitoring our unit provides.”

A secondary benefit is patient flow. “We are a busy unit, so this allows us to care for more patients as needed,” Borasino said. In addition, removing devices reduces the risk of infection from invasive equipment like Foley catheters and central lines.   

Alarm reduction. This initiative is geared toward reducing the number of alarms in the unit in an effort to reduce “alarm fatigue” while improving the overall atmosphere. Alarm parameters are reviewed every 12 hours to ensure they are still accurate given the continually changing status of the patient. To date, the number of alarms has dropped by a third. “We’re trying to diminish that even more,” Borasino said. “We’d like all alarms to be meaningful alarms.”

Quality Improvement

Learn more about the cardiovascular intensive care unit at Children’s Alabama.