Bringing new hope to patients and their families, CHOC Children’s is now among a few hospitals in the country to offer treatment for a rare genetic brain condition that has previously been considered a death sentence for children.
CHOC has been fast tracked to commercially provide Brineura, the first and only treatment for CLN2 disease, also known as late infantile Batten disease. The condition typically begins with language delays and seizures before age 3, and rapidly progresses to dementia, blindness, loss of the ability to walk and talk, and death in childhood.
Bringing Brineura to CHOC is the product of three years of work by metabolic specialist Dr. Raymond Wang.
“This is huge,” Dr. Wang says. “You’re taking a progressive and fatal disease and stopping it. Having seen how heartbreaking it is for families to see the child they know get slowly robbed from them, the fact that we can offer these families hope, is tremendous. Something like this is the very reason I went into medicine and specialized in metabolic disorders: to provide hope to families affected by rare disorders such as late infantile Batten disease.”
Dr. Wang works closely with CHOC neurosurgeon Dr. Joffre Olaya to administer the medicine. Each patient has an Ommaya reservoir implanted under their scalp, which allows the medicine to be infused directly into their brains.
In a sterile procedure every two weeks, Dr. Olaya and a team of highly trained nurses insert a needle into the reservoir to administer the medication. The infusion lasts four hours, and after four hours of observation, the patients can go home.
While not a cure, the drug can slow the progression of the disease. Over a three-year period, patients treated during clinical trials showed no progression of the disease, which was radically different from the disorder’s natural course. The medication improves quality of life and buys patients critical time as researchers continue to search for a cure.
Having the treatment available close to home is a game changer for the Bowman family. A participant in the clinical trial, Ely Bowman, 4, would travel every 10 days with his parents from Orange County to Columbus, Ohio, for treatment.
But now, the Bowmans need only to drive a few miles to CHOC for this critical treatment.
“For Ely to be home and have consistency and we can still have some fun is wonderful,” his mother, Bekah, says. “We can see him thriving.”
An internationally recognized pediatric ophthalmologist with expertise in strabismus, amblyopia, pediatric cataracts and glaucoma has joined CHOC Children’s. Dr. Rahul Bhola is the newest division chief of ophthalmology with CHOC Children’s Specialists.
“The biggest reason I was inspired to join CHOC was the mission of the hospital. I feel that CHOC’s mission to nurture, advance and protect the health and well-being of children is in close alignment with my personal goals as a physician,” Bhola says. “I seek to nurture the health care of children by delivering state-of-the-art ophthalmology care to our community. CHOC has the resources, reputation and experience to provide excellent care.”
Dr. Bhola comes from a family of physicians. His parents practiced internal medicine for more than 40 years in India, and the empathetic and holistic care they provided to their patients inspired him to pursue a career in medicine.
“Very early on in medical school, I developed a special interest in pediatrics, and the surgical finesse of ophthalmology later cemented my passion for pediatric ophthalmology. The gift of vision is the most important sense a child can have,” Dr. Bhola says. “Giving a ray of light to those who struggle with vision is very gratifying to me. Treating children is important to me because they have their entire lives ahead of them, and improving their vision positively impacts their entire family.”
Dr. Bhola attended medical school and completed an internship at University College of Medical Sciences in Delhi, India. He completed two residencies in ophthalmology at Maulana Azad Medical College in New Delhi, India and the University of Louisville, Kentucky. He pursued fellowships in pediatric ophthalmology at the Jules Stein Eye Institute at the University of California Los Angeles and the University of Iowa.
Dr. Bhola has received numerous awards both nationally and internationally and has extensively published in peer-reviewed journals. He has participated as an investigator in many NIH-sponsored trials and has been named to the “Best Doctors in America” and “America’s Top Ophthalmologists” lists consecutively for many years. Dr. Bhola recently started studying the ocular effect of excessive smart device usage in children. His research includes tear film composition in children who are consistently overexposed to smart devices, thereby establishing a link between dry eyes in children and excessive smart device usage.
At CHOC, Dr. Bhola will provide comprehensive eye care, treating patients with a variety of eye diseases and disorders. In addition to treating refractive errors (the need for glasses), Dr. Bhola will provide more specialized care for diseases like amblyopia (lazy eyes), pediatric and adult strabismus (crossing or drifting of eyes), blocked tear duct, diplopia (double vision), pediatric cataracts, pediatric glaucoma, tearing eyes, retinopathy of prematurity, ptosis (droopy eyelids), traumatic eye injuries and uveitis.
Dr. Bhola is among the very few surgeons nationally skilled in treating pediatric glaucoma surgically using the illuminated microcatheter. This highly-specialized, minimally-invasive approach of canaloplasty has been used for treating pediatric glaucoma only within the last few years. Childhood glaucoma, though uncommon, can be a blinding disease causing severe visual impairment if not detected early and treated promptly. The onset of juvenile glaucoma often occurs between the ages of 10 and 20 and can be multifactorial. Glaucoma in pediatric population can also be secondary to trauma occurring from any form of injury including sports injuries.
As a Level II pediatric trauma center, and the only one in Orange County dedicated exclusively for kids, CHOC’s trauma team treats a variety of critically injured children from across the region. This includes children who have sustained sports injuries, during which damage to the structure of the eye can cause glaucoma.
Dr. Bhola is very passionate about educating primary care physicians on the need for regular pediatric vision screenings. For example, children complaining of headaches may be taken to a neurologist. However, eye problems such as refractive errors, convergence insufficiency and strabismus can result in headache from excessive straining of the eyes, which may affect school performance and even social withdrawal in some children. These conditions are likely to be identified at regular vision screenings.
Dr. Bhola’s philosophy of care is to treat his patients as if they were his own children.
“My main philosophy is to deliver patient-centered care with compassion and excellence. I remember their life events and celebrate their achievements with them. It’s important that a patient remembers you in order to start to build trust with them. I love when my patients send me holiday cards and copies of their school photos and let me know how they are doing. They became part of my family. I always treat every patient like they are my own child,” Bhola says.
He also focuses on treating the whole person rather than the disease, and involving patients in their care.
“I don’t treat the disease, I treat the individual. Healing is more than treating the disease. I want to be at their level so I always talk to them directly and not only talk to their parents. I involve their entire group during treatment,” he says.
At CHOC, Dr. Bhola is eager to provide holistic eye care for his patients.
“My practice will offer complete comprehensive vision care to all patients, which includes both medical as well as surgical care. Our patients come to us for glasses, contacts, regular ocular screenings, and we also provide more specialized care like glaucoma, cataract and strabismus surgeries,” Bhola says. “A lot of systemic disorders such as diabetes, sickle cell anemia, juvenile rheumatic disease and lupus, have co-occurring eye issues that may go undetected if children aren’t seen for regular eye screenings. CHOC patients with systemic disorders such as diabetes now have better access to holistic care.”
As division chief for CHOC Children’s Specialists ophthalmology, Dr. Bhola is passionate about providing state-of-the-art care to patients and training the next generation of pediatric ophthalmologists.
“My main goal is to build a leading ophthalmology division, not only delivering excellent patient care but also engaging in cutting-edge research and disseminating education to the next generation of ophthalmologists and referring providers,” Bhola says.
When not treating patients, Dr. Bhola enjoys cooking, practicing yoga and meditation, and spending time with his wife and two daughters.
To contact Dr. Bhola or refer a patient, please call 888-770-2462.
In this CHOC Children’s grand rounds video, Dr. Chantal Boisvert, neuro-ophthalmologist, addresses optic neuritis in pediatric patients. Specifically, she discusses how the presentation and outcome can be different for children ...
The honor recognizes emergency departments across the nation for demonstrating excellence and innovation in leadership, practice, education, advocacy and research.
Recipients of the award went through a rigorous application process, during which they had to provide outcome metrics, as well as descriptive examples that highlighted the following:
Practice – qualities that foster professional pride, confidence and a community of support for emergency nurses
Leadership – operational improvement initiatives, including new systems and processes that positively impact department operations
Education – quality and accessible education that instills knowledge and enhances competencies
Advocacy – efforts that enhance the emergency nursing profession and quality patient care
Research – quality improvement research and the evaluation of clinical outcomes
“Our team is dedicated to providing safe, quality care based on evidence-based practices. We are honored to have our commitment recognized by the Emergency Nurses Association, and will continue to push ourselves to provide the best possible emergency medicine and trauma services to the children and families who depend on us,” says Frank Maas, RN, BSN, MBA, director of the CHOC ED.
The 22,000-square-foot, full-service CHOC ED is exclusively dedicated to the treatment of pediatric patients. It features 31 treatment rooms, including several rapid evaluation and discharge rooms and three triage suites.
The department will be presented with its Lantern Award at the Emergency Nursing Conference 2017 in St. Louis, Missouri later this summer.
The Lantern Award honors the legacy of Florence Nightingale, referred to as the “Lady of the Lamp” for her actions during the Crimean War. Working throughout the night, she would bring a lantern to help her see and tend to the wounded soldiers. She is credited with changing nursing from an untrained job to a skilled, science-based profession.
After spending time with a relative with a mental illness, Dani Milliken knew as early as age 6 that she wanted to help people who struggle with mental illness when she grew up.
“From that point on, I dreamed of working as a psychiatric nurse,” Dani says. “I am so fortunate that I get to come to work every day and love what I do. My passion in life is psychiatric nursing; it always has been and it always will be.”
Dani will put that passion – as well as a wealth of experience in establishing a pediatric inpatient mental health unit – to good use as clinical director of the CHOC Children’s Mental Health Inpatient Center, set to open next spring.
“The community is in need, and CHOC is stepping up to the plate in a big way,” she says. “I have been impressed with everyone’s commitment to developing a safe environment that patients, families and staff feel proud of.”
Before joining CHOC in June, Dani helped design and operationalize a pediatric inpatient mental health unit at Nationwide Children’s Hospital in Columbus, Ohio. Previously, she spent four years at Twin Valley Behavioral Healthcare, a state-owned and -operated inpatient psychiatric hospital in Ohio. There for four years, she served in a variety of roles, most recently as assistant director of nursing.
Dani hopes her work at CHOC will set the standard of care for psychiatric nursing across the country, as well as help remove a stigma that persists around patients with mental illness and the clinicians who treat them.
“Unfortunately, there can be incredible amounts of stigma surrounding not only the patients on an inpatient psychiatric unit, but also the staff that works there,” she says. “I look forward to teaching everyone about quality psychiatric treatment, and what it means to be a real psychiatric nurse.”
Dani earned her bachelor’s degree in nursing from Mount Carmel College of Nursing, and went on to receive her master’s degree in nursing administration. She is currently working toward a doctorate in health care administration.
Though much of her free time is spent getting settled in Orange County after a cross-country move, Dani looks forward to hiking and biking with her husband and dog, as well as quilting and sewing.
In her short time at CHOC, Dani already has been impressed with the culture of collaboration, accountability and respect.
“I am inspired at the amount of teamwork, collaboration and respect that I have encountered during meetings with various disciplines,” she says. “All of these things speak loudly about the level of quality care being delivered to patients here at CHOC. I am thrilled to be a part of this amazing team!”
Upon its opening, CHOC Children’s Mental Health Inpatient Center will be the first pediatric inpatient mental health center in Orange County to accommodate children younger than 12.
With 18 private rooms in a secure and healing environment, the center will provide a safe, nurturing place for children ages 3 to 18, and specialty programming for children younger than 12.
The center is the cornerstone of CHOC’s efforts to create a pediatric system of care for children, teens and young adults in Orange County with mental illness. One in five children – about 150,000 in Orange County – will experience a diagnosable mental health problem.
Learn more about CHOC’s efforts to help children with mental illness.
In addition to treating newborn babies requiring critical care, neonatologist Dr. Irfan Ahmad strives to involve family members in the care of their infant, which he says is essential for providing the best possible care for babies in the CHOC Children’s neonatal intensive care unit.
“I always include parents as part of the care team when treating a baby in the NICU, especially the mother. A mother and her baby were a single unit up until right before the delivery,” Dr. Ahmad says. “Parents are an essential part of the healing team, and building a strong physician-parent relationship is an important aspect of patient- and family-centered care.”
The program will take up residence in CHOC’s recently-opened NICU, which features 36 private rooms with the latest technology and innovations in neonatal care. The 25,000-square-foot unit is nearly triple the size of CHOC’s prior NICU space, and will allow parents to stay overnight with their babies.
“We strongly believe in mother-baby bonding and the value of breast feeding, and our new private NICU rooms are designed to optimize that,” he says.
The recently-opened NICU also features three rooms with surgical lights, allowing minor procedures to be performed at the bedside.
The only Surgical NICU on the West Coast, CHOC’s program is comprised of a multidisciplinary team including neonatologists, pediatric surgeons and anesthesiologists.
“What inspires me the most about care being delivered at CHOC is the combination of passion for helping babies, multidisciplinary interactions, use of modern technology and an atmosphere of teaching,” Dr. Ahmad says. “From dedicated neonatologists present 24 hours a day in the NICU, nurses constantly advocating for best care, nutritionists and pharmacists rounding with the team, physical therapists, wound care teams, lactation specialists and social workers all working together to help a fragile small baby has no parallel.”
In addition to neonatologists, the dedicated ECLS team is composed of cardiothoracic and pediatric surgeons, intensive care physicians, nurses, respiratory therapists and cardiopulmonary perfusionists who are experts in their fields and have received additional education to manage the complex equipment and medical needs of the children needing this life-saving technology.
In addition to stewarding the Surgical NICU, Dr. Ahmad’s special clinical interests include caring for babies who require surgery, including those born with structural abnormalities such as diaphragmatic hernia, intestinal obstruction and imperforate anus. His clinical interests also include babies who develop the intestinal infection necrotizing enterocolitis or who have intestinal perforation. His most common diagnoses include intestinal obstruction and trachea-esophageal fistula.
Mandibular Distraction Program
Dr. Ahmad is especially passionate about caring for babies with difficulty breathing due to an undersized or recessed lower jaw, which can be caused by a condition called Pierre Robin Sequence.
In 2008, Dr. Ahmad helped launch a mandibular distraction program at CHOC. Dozens of infants have benefited from mandibular distraction osteogenesis, which involves a plastic surgeon placing a special device in the small lower jaw to expand it, prompting new bone growth over a period of two to three weeks.
Traditionally, babies with this condition have been treated by placing a tracheostomy that remains in place for several years until the child outgrows the condition. Mandibular distraction is a more permanent solution that takes a few months to complete, allowing a baby to go on to have a normal, healthy development.
Constant quality improvement
Passionate about quality improvement, Dr. Ahmad serves as director of quality improvement for NICUs affiliated with CHOC Children’s Specialists. He has participated in several quality improvement initiatives with Vermont Oxford Network and California Perinatal Quality Improvement Collaborative. This includes a project to improve the transition of care for surgical cases from one team to another, decreasing delivery room intubations and preventing premature newborn babies from developing hypothermia.
As the director of quality improvement for CHOC’s network of nine NICUs, he partners with quality improvement teams at each unit in carrying out improvement projects based on local needs. The team currently has nine simultaneous quality improvement projects in the hospitals where CHOC neonatologists round.
Passionate about educating the next generation of pediatricians and neonatologists, Dr. Ahmad also serves as NICU education director for UC Irvine’s pediatric residency program and is an associate clinical professor of pediatrics at UC Irvine. He also trains neonatology fellows through CHOC’s partnership with Harbor-UCLA Medical Center’s neonatal-perinatal medicine fellowship program.
His current research efforts include studying the breathing patterns of full-term babies in order to refine inclusion criteria for the mandibular distraction procedure. He is also currently studying the clinical outcomes of CHOC’s surgical NICU program.
Pursuing his calling to care for children
Dr. Ahmad attended medical school at Aga Khan University in Pakistan. He completed a residency in pediatrics at the University of Oklahoma and a fellowship in neonatal-perinatal medicine at UC Irvine. He has been on staff at CHOC for 10 years. He knew from an early age that he wanted to care for children, so pursuing a pediatrics residency after medical school was a natural choice.
“I was exposed to various specialized fields like cardiology and oncology, but I wanted to take care of the whole patient. I also wanted to see when I could have the most impact on the life of a person,” Dr. Ahmad says. “During my residency when I worked in the NICU, I noted that good care in the first few minutes of life was so critical. Effective resuscitation, followed by intensive care in the NICU could make all the difference for the patient, who can then live a long and accomplished life.”
Dr. Ahmad finds inspiration in the strength of his patient’s families, and is continually renewed and humbled by their gratitude.
“I have been impressed by the strength of the families who have a sick little baby in the NICU. It is extremely difficult to have your newborn on a ventilator struggling for life. Yet, we see the moms and dads holding on to hope and being there for their baby,” Dr. Ahmad says. “Neonatology is a very difficult field with long hours taking care of very sick babies. The gratitude you get from parents when the baby is finally well and going home and the amazing photographs and cards that are sent to us makes everything worthwhile.”
In his spare time, Dr. Ahmad enjoys golfing with his children and developing his photography skills.