I’ll bet you a quarter that you do not treat children with cerebral palsy (CP) in Nepal as does David A. Spiegel MD from the Children’s Hospital of Philadelphia.
For every quarter I pay out here, I’m guessing I’ll get enough back to cover a year’s worth of bad hospital coffee.
Why, then, should we use the Editor’s Spotlight/Take 5 space in Clinical Orthopaedics and Related Research®—the part of the Journal we devote each month to the article with the greatest general-interest message—to cover such a seemingly narrow topic? That’s easy.
It’s because this story inspires us. It inspires us to find ways to make a difference in the lives of those with less, whether around the globe or around the corner. It inspires us to think about what it takes to start a program that meets a critical need. It should inspire gratitude—for the things that we have, and for the fact that there are, in the world, people like Dr. Spiegel and his team.
This story starts in Philadelphia, but I suspect Dr. Spiegel might say that this month’s Spotlight paper  really started in Nepal, with Dr. Ashok Banskota, who founded the Hospital and Rehabilitation Centre for Disabled Children (HRDC) in Banepa, Nepal. Dr. Banskota and his team (who coauthored this month’s Spotlight paper) has made it possible for many thousands of Nepali children to walk, play, and, eventually, work; the latter is especially critical in a part of the world where safety nets hardly exist. Dr. Spiegel has served as an honorary consultant at HRDC and began participating in a range of activities at the hospital in 1996.
Dr. Spiegel’s interventions around the world on behalf of children has been recognized numerous times, not least by the Pediatric Orthopaedic Society of North America and the American Academy of Orthopaedic Surgeons, both of which have honored him with their respective Humanitarian Awards . Likewise, Dr. Banskota received the World of Children “Health Award” in 2016, and its “Hero Award” in 2011; since receiving that first award, his hospital has evaluated more than 100,000 children, and operated on more than 5000 . The laurels they’ve received have been well earned.
The study they published in this month’s CORR® found that children with the spastic hemiplegia form of CP in Nepal—perhaps unsurprisingly—experience much-more-profound levels of physical disability than do children with CP who live in more-developed countries. What was surprising, at least to me, is that despite the more-severe level of physical disability observed among Nepali children (as measured by Gross Motor Function Classification System, and the Manual Ability Classification System), Nepali children with spastic hemiplegia were far-less impaired in terms of their ability to communicate in society as measured by the Communication Function Classification System (CFCS). In fact, despite their rather-profound levels of motor impairment, 97% of children with spastic hemiplegia in Nepal scored a I or II on the CFCS (which is scored from I to V, with I representing independent, effective communication), compared to only 67% of children with this diagnosis in more-affluent countries. These differences were not observed for other forms of CP, including spastic diplegia or quadriplegia.
These findings call for an explanation, because some are, on the surface, confusing. For example, why would Nepali children with CP have less impairment in terms of communication than children with the same diagnosis elsewhere? But more importantly, these findings call for intervention, and there is a high-value opportunity here: Children with CP in Nepal have more-severe upper- and lower-extremity motor impairments than do patients with the same diagnosis in more-affluent countries, but—as mentioned—the Nepali children are far-less impaired in terms of their social interactions. This suggests that simple orthopaedic interventions, like orthoses or soft-tissue releases, are likely to have an outsized benefit, since this population is essentially naïve to treatment. And once treated, their high CFCS scores suggest that these children and adolescents will become productive members of their communities.
These findings also should cause us to take a step back and look at the underlying contexts in which children with these diagnoses receive care, both in resource-constrained environments and countries with ample medical infrastructures, in order to see what part each of us can play in alleviating the world’s suffering.
Join me in the Take-5 interview that follows with Dr. Spiegel for a learned perspective on those topics, as well as to find out ways to get involved with the kinds of international efforts that he has spent a career developing.
Take Five Interview with David A. Spiegel MD, senior author of “Socioeconomic Status Influences Functional Severity of Untreated Cerebral Palsy in Nepal: A Prospective Analysis and Systematic Review”
Seth S. Leopold MD: Congratulations on this eye-opening study. Research of this sort depends on categorizing the conditions, and measuring them using objective functional scales, which you’ve done well. But as you know, the numbers don’t tell the whole story. What nuances have you gained about caring for these patients from the time you've spent in Nepal that does not come through in a scientific report like yours?
David A. Spiegel, MD: While the numbers help the reader to gain some sense for how the patients scored on standard assessments of function including gross motor, upper extremity, and the ability to communicate, the challenge lies in contextualizing these findings and using them to improve the care of children in low-income or resource-poor settings like Nepal. Each reader will have his or her own internal frame of reference, based on their perceptions of what CP is from his or her own experiences. What our paper attempts to communicate is that one’s perception of CP needs to be restructured and reframed according to context. Specifically, the most appropriate orthopaedic treatment for CP or any other orthopaedic condition is defined by the local context, including the presentation and stage of disease (often delayed presentation), the resources available at the health facility (imaging, equipment/supplies, access to immediate rehabilitation services), community resources for ongoing rehabilitation and maintenance of orthoses or assistive devices, as well as social and cultural variables. In Nepal, geospatial barriers are a substantial obstacle hindering access to orthopaedic care, particularly for patients living in smaller communities up in the mountains, often far from motorable roads. The field of orthopaedics is essentially recreated at the site of service delivery based on local variables, although the goals of treatment remain the same.
Dr. Leopold: Your work will inspire many. Who, in your career, has inspired you, and how has that person’s example changed you as a surgeon and a person?
Dr. Spiegel: I have had many mentors and have been influenced by several great orthopaedic surgeons both in the United States and abroad. I’ve been especially inspired by Dr. Ashok Banskota, whom I first met just after I completed residency. He has been a mentor, close friend, and collaborator for 22 years. Dr. Banskota is humble, kind, and thoughtful, and he is an excellent technical surgeon who is extremely dedicated to his patients and to tackling challenging procedures in a setting where resources are limited. I was amazed at his creativity and resourcefulness, his knowledge of each patient, and his vision. In addition to developing a successful practice and building his own hospital, he also founded and has dedicated countless hours to the HRDC, a children’s hospital that has impacted the lives of more than 80,000 patients to date, most of whom are from remote and underserved communities in Nepal. He also started one of the first residencies in Nepal and has trained more than 50 orthopaedic surgeons. My close interactions with Dr. Banskota and his team of talented surgeons during the years has permanently altered my perception of orthopaedic conditions, and my philosophy and approach to patient care whether in Philadelphia, Kathmandu, Iraq, or Pakistan. It has been my pleasure to collaborate with Dr. Banskota’s team on education, research, and developing clubfoot and CP programs at HRDC. I have also become close with his son, Bibek, who has now taken over administrative responsibilities at HRDC in addition to being a staff surgeon.
Special mention must also go to Drs. James Urbaniak and Denis Drummond, mentors during my residency and fellowship, as well as Drs. Thamer Hamdan and Ali Al-Iedan in Basra, Iraq, Dr. Syed Muhammad Awais in Lahore, Pakistan, and Dr. Meena Cherian from Geneva, Switzerland, all of whom have inspired me.
Dr. Leopold: You first went to Nepal as a resident. What can residents do if they want to get involved in this kind of effort?
Dr. Spiegel: There is greater interest in offering residents an international elective in recent years [2-4, 6, 8], exposing them to the challenges of orthopaedic surgery in settings with limited resources. I believe there is great educational value in exposing the resident to uncommon presentations of familiar conditions—for example, the delayed presentation of fractures and infections—and the common presentations of unfamiliar conditions such as tuberculosis and polio. Residents learn to appreciate the difficulties in providing care with limited availability of implants, the value of time-honored methods of care such as traction, and the importance of social and cultural variables that impact the choice of treatment. They also appreciate how orthopaedic services are delivered in the setting of a fragmented and often dysfunctional health system.
These observations appear to be gaining traction among orthopaedic educators, and many programs have started offering overseas electives [2-4, 6, 8]. Indeed, Schultz and colleagues  found that 26% of programs offered such an experience as of 2015. This remains a work in progress, as there are financial and logistical challenges to coordinating trips given the requirements for coverage, and the requirement for a partnering institution . But there is potential in officially recognizing an overseas experience as time in training since in many instances, these short-term experiences come at least partially from a resident’s vacation time. There may be opportunities available through faculty at the resident’s institution; certainly, they are available through charitable and/or nongovernmental organizations such as Orthopaedics Overseas (https://hvousa.org/ourwork/programs/orthopaedics) which offer experiences with elements of service, teaching, or both.
Dr. Leopold: One definition of “value” is benefit derived divided by cost. You’ve made a compelling case that intervening in resource-starved areas like Nepal is a high-value proposition: These children have profound but often readily treatable functional problems, and since they scored well on the communications scale—higher, in fact, than children in more-affluent countries—they stand to benefit tremendously even from simple interventions. But the denominator in the value equation remains the challenge: There is so little money, so little resource available there. How can that change?
Dr. Spiegel: Advocating for specialty services for a heterogeneous and chronically disabling condition like CP is a daunting task, especially in environments where access to basic health care is often lacking and specialty surgical services are rarely available. Children’s physical disability can hardly be prioritized when basic care for injuries and infections cannot be assured. Health systems are fragmented, there are gross deficiencies in human resources, and it is impractical for patients from many areas to come for the regular followup and/or receive ongoing services in their home communities. The needs extend throughout growth and development and into adulthood. Deficits within the system level are often partially offset or compensated by subspecialty organizations and/or institutions such as HRDC, which rely upon funding from donors.
Given this situation, the case must be made for supporting the treatment of CP relative to competing priorities, and value decisions must be made. In the present case, nearly 20% of patients presenting to HRDC and its mobile camps throughout the country are diagnosed with CP. This is a significant burden, and we wanted to better characterize the patients presenting for treatment before determining how to address their needs. This study helped us achieve that goal.
Regarding treatment strategies, we believe that “cerebral palsy” must be redefined in the Nepali context, and that value can be achieved despite challenges including limited resources/finances, difficulties with maintaining regular followup (geospatial barriers), deficiencies in formal rehabilitation and medical services in patients' home communities, and a hesitance among many patients to use orthoses. While the process has been evolving, we understand that a contextually appropriate, community-based approach is required, and HRDC has utilized community-based rehabilitation workers as a linkage to our patients' communities. Any system will include: (1) Screening to identify patients, (2) rehabilitation services focusing on home therapy and monitoring by community-based health workers, (3) surgical services, (4) fabrication and maintenance of orthoses and assistive devices, (5) appropriate referral to institutions that address the nonorthopaedic needs of this patient population, and finally (6) community-based followup and monitoring to provide longitudinal services and promote social integration.
While the surgical procedures themselves have been practiced with regularity, the criteria for offering surgical care are stringent and based on a high likelihood of improving function. Many children will benefit more from assistive devices, vocational rehabilitation, and modifications to their home environment than from a surgical procedure. Our team is working to refine indications and to establish realistic goals for both ambulators and nonambulators. Strategies for tone reduction such as botulinum toxin, rhizotomy, and baclofen pump are impractical, although oral baclofen and phenol blocks have been utilized in selected patients.
Development and scaling up of a “program” for patients with CP will require greater financial inputs into our system, so that funds are not diverted from other services we deliver, especially with the goal of extending our services to patents in the most disadvantaged communities or even the entire population. We believe that research projects such as this, which capture or estimate the burden, coupled with future projects to study function, participation and ease of care using validated patient reported and/or disease specific outcomes can help make a better case to the donor community. Research focusing on systems for service delivery will also be useful, given the complexities of providing long-term care for diverse communities in a setting like Nepal. While we don’t need to prove that heel cord lengthening can reliably improve dorsiflexion at the ankle, we do need to demonstrate that the different services offered improves function, participation in activities, and/or ease of care in our setting. Patient-reported and/or other functional outcomes should be measured with contextually relevant tools, a challenging proposition when disease-specific instruments are evolving and must be translated and ideally validated. While some might argue that time and money expended on research comes at the expense of clinical care when money is scarce, we feel that studies are an essential component of setting up systems for clinical care, such as clubfoot and CP, and monitoring the delivery of services. Research should focus on contextual solutions.
Dr. Leopold: I understand why children with CP in a country with little medical infrastructure might have more-profound levels of motor impairment, but I confess that I don’t completely understand why Nepali children with this diagnosis would demonstrate less impairment in communication skills than did children from more-affluent countries. Why do you think this is?
Dr. Spiegel: Cerebral palsy is a developmental disability with a spectrum of clinical manifestations resulting from damage to the immature brain. While abnormalities in movement and posture are most evident, there are also several associated problems including seizures, gastrointestinal abnormalities, and intellectual deficits and/or behavioral problems that heavily influence the clinical manifestations in a given patient.
While the number of children diagnosed with CP does not differ substantially between economically developed and economically underdeveloped regions (1 to 3 out of 1000 live births), my impression is that most common etiologies likely differ between low- and high-income settings. In economically underdeveloped regions such as Nepal, most children are born at home, often without prenatal care and/or the assistance of a trained birth attendant, and without access to emergency obstetric services. The most-common suspected causes of CP in this context are suspected to be events at the time of birth and neonatal jaundice, and to a lesser extent postnatal causes such as encephalitis. They survive in the absence of neonatal intensive care services, formal nutritional services, gastrostomy tubes, shunts, and/or pulmonary services, so it is reasonable to suspect that they would have a lesser degree of injury to their brain than a group of patients with the same diagnosis from an economically developed country in which a significant number of patients are associated with prematurity and low birth weight, and there is usually access to a full complement of services including neonatal intensive care.
By contrast, in higher-income regions, more patients who are neurologically devastated survive into adult life due to the availability advanced medical services. Perhaps this might explain why scores on the CFCS were higher in Nepal. The neurologic concerns in CP include weakness, difficulties with controlling movement (and also unintentional movements), problems with balance and proprioception, and abnormalities of muscle tone such as spasticity or dystonia. Within this background and in parallel comes the musculoskeletal manifestations, which include myostatic muscle contractures and problems with bony lever arm function from torsional malalignment and foot deformities that progress over time and contribute to the disability. Although the neurologic injury remains static, the musculoskeletal manifestations may progress throughout growth and development, particularly in the absence of formal treatment. This may help explain why the functional scores are lower for gross motor function and manual function.