Approximately one in every thousand children is born with a congenital upper limb difference. These conditions can include complete or partial absence of a limb, failure of fingers to separate, duplication of fingers, overgrowth and undergrowth as well as constriction ring syndrome. Along with the physical differences this presents as a child grows and develops, there can also be mental and emotional challenges.
A recent study led by Donald S. Bae, MD, a pediatric orthopedic surgeon in the Hand and Upper Extremity Program at Boston Children’s Orthopedics and Sports Medicine Center, evaluated the physical level of function as well as the mental and emotional health of almost 600 children with congenital upper limb differences. Ultimately, the study found that while children with congenital hand differences had decreased upper limb function, they have better peer relationships and positive emotional states compared to population norms.
Building a national registry
The study was performed over a two-year period, in which 586 children with congenital upper limb differences from Boston Children’s and St. Louis Children’s Hospital were enrolled in the Congenital Upper Limb Differences registry. Functional outcomes were assessed in 301 patients from the registry using Pediatric Outcomes Data Collection Instrument (PODCI) and Patient-Reported Outcomes Measurement Information System (PROMIS). The areas assessed were upper extremity function, pain, anxiety, depression and peer relationships.
“In the interest of not only providing high-quality care, but also investigating the patient-centric or patient-reported outcomes of our treatment, we’ve helped spearhead the formation of a multi-center registry,” Bae explains. This study is one of the first to use the national registry, and is a combined effort between Boston Children’s and St. Louis Children’s at Washington University.
Differences in physical, mental and emotional health
The results show that, although patients with hand and upper limb differences weren’t as proficient in terms of physical function as population norms, in general they did not score much lower — particularly in the adolescent population. Not surprisingly, patients who had bilateral involvement or patients whose conditions involved more than just one hand had lower scores than children with one-sided involvement of just the hand alone. While this is an important takeaway from the study, Bae believes that there is much more to the story.
“As musculoskeletal providers and as surgeons, of course we want to improve physical function, but I think what this study also underscores is the importance of an individual’s participation, peer interaction and emotional health.”
Improvements through adaptation
Both younger patients and adolescents reported higher scores for peer relationships and lower levels of depression and anxiety than population norms — a finding that is consistent with prior studies. However, few studies have looked at emotional and social health in this group of patients. Despite this, the consistency in findings between publications that have studied this area may be due to the ability of children with upper limb differences to adapt and compensate for physical function in ways that don’t negatively impact their social and psychological development. In fact, in some ways these coping mechanisms may improve certain areas of their development.
“You wonder sometimes if because a child with a congenital difference has learned to adapt to who they are in order to perform daily tasks and participate in activities, that maybe this puts them in a place where they can actually cope and deal with stressors better,” Bae postulates. While stressing that he’s not a psychologist nor developmental pediatrician by training, Bae was not surprised by these results based on his interactions with many patients with hand differences.
Bae believes that these findings can be used as a tool in clinic to help reassure parents that their child is not at an increased risk for mental or emotional health issues.
“We can assure families that their children will be able to participate and have really meaningful relationships and emotionally do very well,” says Bae. “This allows us to focus on what we can do to improve the hand or upper limb function, while being mindful that we’re not just focused on the hand, we’re focused on the whole child.”
Learn more about Boston Children’s Hand and Upper Extremity Program.