<b><i>Children with Unilateral Congenital Below-Elbow Deficiency</i></b> <b><i>"How the parents accept a limb deficiency and how well they cope with it has a great deal to do with how well the child does, either with or without a prosthesis." -Yoshio Setoguchi, MD</i></b> <img style="float: right;" src="https://opedge.com/Content/OldArticles/images/2008-04_12/1-1.jpg" hspace="4" vspace="4" /> Caroline is a lively, redheaded fifth-grader. She is a straight-A student who is into dancing, cheerleading, gymnastics, acting, and modeling. She has competed in soccer, played the violin, and now is eager to learn to play the guitar. Caroline is confident, outgoing, and has many friends, says her mother Chris. Like most youngsters, she sometimes squabbles and competes with her siblings: fraternal twin sister Annie and older brother Will, 17. Caroline was born missing her right hand and part of her right arm below the elbow. That certainly has not slowed her down, however. She was fitted with a passive prosthesis when she was eight months old and began using a myoelectric prosthesis at 18 months. She used the prosthesis to play the violin and when riding her bike because she thought it would help her balance. Today, however, her prosthesis sits on a shelf. Caroline found it cumbersome and does well without it, according to her mother. "I was worried about her socially, growing up and looking 'different', but her peers have totally accepted her," Chris says. What has helped Caroline to be so outgoing, happy, and confident, both with and without a prosthesis? Experts in pediatric prosthetics often point to the important role of the parents and family in helping a youngster with limb loss or deficiency, whether acquired or congenital like Caroline's. <table class="clsTableCaption" style="float: right;"> <tbody> <tr> <td><img src="https://opedge.com/Content/OldArticles/images/2008-04_12/1-3.jpg" alt="Setoguchi" /></td> </tr> <tr> <td>Setoguchi</td> </tr> </tbody> </table> Yoshio Setoguchi, MD, medical director of the Child Amputee Prosthetic Project (CAPP), Shriners Hospital for Children, Los Angeles, California, says, "My feeling is that how the parents accept a limb deficiency and how well they cope with it has a great deal to do with how well the child does, either with or without a prosthesis." "If the parents aren't handling [the limb deficiency] well, then about 90 percent of the time the child isn't either," says Alicia J. Davis, MPO, CPO, FAAOP, associate director of residency training, Orthotics and Prosthetics Center, University of Michigan Health Systems, Ann Arbor. "I think one of the greatest suggestions came from a child himself: 'I just want to be treated like anyone else.'" <table class="clsTableCaption" style="float: right;"> <tbody> <tr> <td><img src="https://opedge.com/Content/OldArticles/images/2008-04_12/1-4.jpg" alt="Davis" /></td> </tr> <tr> <td>Davis</td> </tr> </tbody> </table> Says Caroline's mother: "I think it has helped that Caroline has a twin because I treat them both the same. It's best if you treat them like they're no different than anyone else... It's the attitude of the parents and the love and support of friends and family that give these children the confidence to shoot for the moon." Under the umbrella of working to help children achieve self-esteem, confidence, and reach personal goals, there are varying clinical approaches and prosthetic options-including, in some cases, the option of not using a prosthesis. Prosthetic care of children with unilateral congenital below-elbow deficiency (UCBED) especially has been an area of controversy. Should children with UCBED be fit with a prosthesis? If so, how early? And with what type of prosthetic design? The experts emphasize the need to recognize, evaluate, and assess the needs of each child individually-there's no place for an assembly-line approach. "Each child is definitely a separate entity and has specific things he or she wants to do," Davis says. "So each one has to be handled as a very specific and special case-to issue a blanket statement [about treatment protocols] would be a disservice." <h4>Psychological Issues</h4> If a child does appear to have self-esteem or body image issues, or the parents seem to be having difficulty coping with their child's limb deficiency, counseling may be in order. But Setoguchi and Davis advise referring the patient and family to a social worker or psychologist rather than a prosthetist or anyone untrained in counseling. "Even if they mean well, they may not be helping the patient," Setoguchi says. When psychological issues with the patient or family become apparent, Davis generally refers them to a psychologist on staff and possibly to a peer support group, which can share experiences, camaraderie, and practical advice. "I have the I-Can support group [International Child Amputee Network, <a href="https://opedge.com/3115">www.child-amputee.net </a>] who I can call on day or night, and someone will have been there, done that," says Caroline's mother. <img style="float: right;" src="https://opedge.com/Content/OldArticles/images/2008-04_12/1-2.jpg" hspace="4" vspace="4" /> When a baby is born with a congenital limb deficiency, there are often feelings of shock and guilt. "What did I do to cause this? Why did it happen? Will my child be able to have a normal and happy life?" These are some of the questions that confront parents. Early intervention and support are most important for families, and they are encouraged to see the clinic team as soon after the baby's birth as possible, according to "Developmental Approach to Pediatric Upper-Limb Prosthetic Training," by Joanna Grace Patton, BS, OTR, a chapter in the <i>Atlas of Limb Prosthetics, </i>Second Edition (American Academy of Orthopaedic Surgeons, 1992, reprinted 2002). The opportunity to discuss feelings and concerns and receive appropriate information about the baby's limb deficiency and future treatment are very valuable, Patton wrote. Family involvement and commitment is crucial. "In any type of rehabilitation, the family has to be involved because they're the ones that are going to be caring for the child and following through on recommendations," Setoguchi says. The parents must be willing to make the commitment to the time and effort needed to successfully carry out the treatment program, he emphasizes. <h4>Why Fit a Prosthesis?</h4> Early prosthetic fitting for UCBED children has been considered by many clinicians to help children reach essential developmental milestones and provide other functional benefits. "In a general sense the most important reason for early intervention is to facilitate attainment of developmental milestones; e.g., balance issues, ambulation issues," Davis says, "but secondary to avoid overuse injuries in later life." Clinicians need to see not only the infant or child, but also must look ahead 30 to 50 years to the adult who has been relying on his intact limb to do the majority of the work, which can lead to overuse injuries. "These injuries can be debilitating and even life-altering," Davis says. "We all appreciate the individual who overcomes all odds to become a one-handed baseball or football player or someone who becomes a firefighter and has only one leg. But in clinical practice, I'm seeing clinical manifestations of overuse developed from the attempt to compensate for what has been lost." Therefore, an important goal is to create a prosthesis that becomes an integral part of the person's life and will help offset possible overuse of the intact limb and resulting complications, she points out. Some therapists believe that earlier fitting results in better incorporation of the prosthesis into the child's body image and that early fitting may be essential for normal neuromuscular development, notes an article reporting results of a survey of North American child amputee clinics ("Early Upper Limb Prosthesis Fitting: When and What Do We Fit," by Julie Shaperman, MSPH, OTR; Samuel E. Landsberger, ScD; and Yoshio Setoguchi, MD, <i>Journal of Prosthetics and Orthotics, </i>Vol. 15, No. 1, 2003; <a href="https://opedge.com/3116">www.oandp.org/jpo/library/index/2003_01.asp</a>). The article adds this caveat, "While intuitively appealing, neither of these ideas is supported by published documentation." Early prosthetic fitting provides the opportunity to develop bimanual skills, useful prehensile activities, along with incorporating the prosthesis into the body image, according to the study, "Juvenile Upper-Limb Amputees: Early Prosthetic Fit and Functional Use," by R.C. Tervo, MD, and J. Leszczynski, MD (Association of Children's Orthotic-Prosthetic Clinics [ACPOC], <i>Inter-Clinical Information Bulletin</i>, Vol. 18, No. 5, 1983). Early fitting may ultimately contribute to better prosthetic tolerance and wear patterns and may prevent an asymmetrical posture and spinal curvature, the authors add. <h4>Is There Value in Prosthetic Fitting?</h4> While the approach described above represents the status quo for at least the last 25 years, a recent study by Michelle A. James, MD, et al., published in the <i>Journal of Bone and Joint Surgery, </i>November 2006, goes against the current of conventional thinking. "Prostheses may help with social acceptance or may be useful as tools for specialized activities, but they do not appear to improve function or quality of life, which are nearly normal for children with unilateral congenital below-the-elbow deficiency regardless of whether they wear a prosthesis," according to the research article titled, "Impact of Prostheses on Function and Quality of Life for Children with Unilateral Congenital Below-the-Elbow Deficiency." The multi-center outcomes study involved 489 children with UCBED; 321 wore a prosthesis and 168 did not. The Unilateral Below-the-Elbow Test (UBET) was designed, validated, and administered to these children along with several outcomes measures, including the Pediatric Outcomes Data Collection Instrument (PODCI), the Pediatric Quality of Life Inventory (PedsQL), and the Prosthetic Upper Extremity Functional Index (PUFI). <table class="clsTableCaption" style="float: right; width: 30.8971%;"> <tbody> <tr> <td style="width: 100%;"><img src="https://opedge.com/Content/OldArticles/images/2008-04_12/1-5.jpg" alt="Photograph courtesy of Hanger Prosthetics and Orthotics Upper Extremity Prosthetics Program." /></td> </tr> <tr> <td style="width: 100%;">Photograph courtesy of Hanger Prosthetics and Orthotics Upper Extremity Prosthetics Program.</td> </tr> </tbody> </table> Use of a prosthesis was not associated with any clinically relevant differences in PODCI or PedsQL scores, the researchers found. Non-wearers performed either the same as or better than wearers on the UBET, according to the study. "When queried [with use of the PUFI] about performance of various tasks, non-wearers scored themselves higher than wearers. Children with a unilateral congenital below-the-elbow deficiency scored the same as or higher than the general population on the PedsQL. They scored significantly lower than the general population on the PODCI Upper Extremity Physical Function Domain and higher on the Happiness Domain, but the differences were small." The authors conclude, "These findings call into question the standard practices of fitting infants with prostheses and encouraging young children to wear the prosthesis." Even if a prosthesis does not meet all of the child's needs and if its use is not continued, "a prosthesis...may serve a short-term purpose or benefit during a certain developmental period," observes a study involving a retrospective chart review of 298 children at five Shriners Hospitals for Children ("Predictors of Continued Prosthetic Wear in Children with Upper-Extremity Prostheses," Joanne Shida-Tokeshi, MA, OTR/L, et al., <i>Journal of Prosthetics and Orthotics, </i>Vol. 17, No. 4, 2005). The 236 children (79 percent) in the study who wore a prosthesis for at least three years appeared to receive some benefit, the study found. However, not continuing to wear a prosthesis should not be assessed as a failure or as a negative outcome, the researchers note. "It is clinically observed that a prosthesis cannot meet or fulfill all the needs of the family or child. It may be an appropriate outcome or natural occurrence for a certain percentage of children to find the prosthesis unnecessary." <h4>When to Fit a Prosthesis</h4> If the choice is to use a prosthesis, the previously cited survey of North American child amputee clinics reveals that most clinics prefer to fit at six months, when children generally can sit up independently, and to add an active control system, either body-powered or external (myoelectric), by 18 months. "I agree with most literature that prosthetic fitting should be paired with developmental milestones," Davis says. However, she feels there are times to deviate. For instance, if a child is delaying in reaching developmental milestones, clinicians may still fit the child with a prosthesis if they believe it will help the child advance developmentally. Generally, the first fitting is a passive device, which enables the infant to oppose the sound hand for gross two-handed grasping and develops a prosthetic wearing pattern that will ease the transition into an activated limb, note Jack Uellendahl, CPO, and Jamee Riggio-Helan, OTR/L, in an article published in <i>Physical Medicine and Rehabilitation: State of the Art Reviews</i>, June 2000. The baby is encouraged to include the prosthesis to stabilize body weight when creeping on all fours or when pulling to stand. The prosthesis can help the infant bear weight on the affected side while reaching with his dominant hand and allow natural muscle development in both upper extremities. The prosthesis also promotes eye-hand coordination as the infant explores different objects in the environment, according to Uellendahl and Riggio-Helan. <h4>The Team Approach: Optimizing Resources</h4> The team approach is considered vital, since each member contributes to a successful outcome for patients at all levels and types of musculoskeletal or developmental conditions. The CAPP team includes the pediatrician, orthopedic surgeon, occupational therapist, physical therapist, social worker, and prosthetist. The first visit by the patient and family generally takes more than two hours and includes a social history as well as a complete medical history and physical. For the social history, the social worker asks such questions as how the parents felt when the child was born, their reactions, what type of support they have had, and their questions and expectations on coming to the clinic. The patient and family then see the occupational therapist for an assessment of their upper-limb progress in activities of daily living (ADL), and psychosocial function, and a physical therapist for assessment of the lower-limb function. They see the prosthetist if the patient is a candidate for prosthetic fitting. If the patient needs surgery, the child and family are referred for an orthopedic consultation. After all of the team members have evaluated the patient, they meet as a group without the patient and family, discuss their findings and concerns, and then establish a list of recommendations for a treatment program. They then meet with the family to discuss their recommendations, answer any questions, and then set up the appropriate appointments. Approximately 12 to 20 patients are seen in the clinic each day. The University of Michigan medical team is similar. Davis notes that they see a far larger number of upper-extremity patients of all ages with limb loss or deficiency than the average private prosthetic practice since they hold two clinics a month, each with 14 to 16 patients-about 30 patients monthly. A prosthetist in a smaller practice not associated with a hospital or university medical school can often utilize a team approach when he realizes that the patient and family have needs outside his scope of expertise. Setoguchi recommends contacting the patient's primary care physician first to help locate resources. The prosthetist can start to build a base of resources to refer patients for help. <i>[Editor's note: For more information on crafting a team approach, see "Making a Team Work: Assembling a Rehabilitation Team in O&P Private Practices," </i>The O&P EDGE, <i>July 2007,</i><a href="https://opedge.com/3117"><i>www.oandp.com/edge/issues/articles/2007-07_01.asp</i></a><i>]</i> <h4>What Defines Success?</h4> Lastly, what really is a successful outcome for the child with UCBED-or any other prosthetic or orthotic patient? It's not all about how well the child adapts to and uses the device, according to Setoguchi. Even in the "normal" population, one can't predict who will achieve a happy, successful life, he points out. "The key element is how well they function in society-as adults, do they have a job, a family, and are they happy in what they do and happy with their life?" If so, that's a successful outcome, whether or not they're using a prosthesis. <i>Miki Fairley is a contributing editor for</i> The O&P EDGE <i>and a freelance writer based in southwest Colorado. She can be contacted via e-mail at</i><a href="mailto:miki.fairley@gmail.com"><i>miki.fairley@gmail.com</i></a> <h4>Survey Explores Early Prosthetic Fitting Clinical Practice</h4> Highlights from a survey of North American child amputee clinics, "Early Upper Limb Prosthesis Fitting: When and What Do We Fit?" by Julie Shaperman, MSPH, OTR; Samuel E. Landsberger, ScD; and Yoshio Setoguchi, MD <i>(Journal of Prosthetics and Orthotics </i>, Vol. 15, No. 1, 2003) are presented below. The researchers received responses from 45 of 80 clinics contacted (56 percent); the clinics were identified from the Association of Children's Prosthetic-Orthotic Clinics (ACPOC) and the Amputee Coalition of America (ACA). <ul> <li>The median age for first fitting was six months, with a range of three to 48 months. Two-thirds of the clinics prefer the first fitting at five to seven months of age.</li> <li>All but four clinics fit a passive terminal device with the first prosthesis. The other four reported using body-powered systems (three voluntary opening, one voluntary closing).</li> <li>Regarding developmental signs used to determine readiness for a more advanced terminal device/control system than the one first fitted, most responses referred to increased developmental maturity indicating that the child could be taught to operate an active control system. Developmental signs guiding the change included cognitive (e.g., awareness of cause and effect); predictors of use of the prehensile function of the terminal device (e.g., attempts to hold objects, to open the terminal device manually, and to insert objects); and predictors of ability to participate in a training program (e.g., willing to follow directions). The study noted that a confounding variable was that it is often practical to add the new terminal device and control system at the time the child has outgrown the first prosthesis, even if preferred age and other developmental indicators are absent.</li> <li>The median age for changing to a more advanced terminal device and activation of the control system was 18 months, with a range of 12 to 60 months.</li> <li>Regarding the use of objective tests to measure skill and use of the terminal device, ten clinics reported using the University of New Brunswick test, four had designed their own test, and 31 did not use any objective tests.</li> <li>Improvements desired in first or second terminal devices for young children included increased grip strength, improved appearance, easier operation, simpler mechanisms, better hold on objects related to size of terminal device opening, and ease of object positioning. Also mentioned were lighter weight, wrist motion, and harness comfort.</li> </ul>
