Chronic hip pain in a boy with mental retardation and cerebral palsy

Jonny is a 13 year old boy with spastic quadriparesis and severe mental retardation following Haemophilus influenza type B (HIB) meningitis at 2-months of age. Signs of meningitis started on the evening of his 2-month immunizations that included the HIB vaccine. He presented to his pediatrician with left hip pain that occurred intermittently for a few years and more frequently in the past six months. His parents initially attributed the pain to whizzing around the back yard in a motorized wheelchair. An earlier evaluation of hip pain led to bilateral femoral osteotomies for hip dysplasia. Obesity, associated with inactivity and a tendency to consume fatty foods, complicates Jonny's disabilities. His only activity is a modest amount of physical therapy at school and "floor time" for about one hour each day at home. In the office of his pediatrician, Jonny is friendly, smiling, and verbalizing a few words with his limited expressive vocabulary. He is resistant to a hip examination and grimaces with manipulation of his left hip. Spasticity of the left leg appears increased compared to previous examinations. He has nonpitting edema of his lower legs and feet, a cryptorchid left testicle, and a somewhat tender left inguinal area. Jonny lives with his mother and father in a small house on a busy street less than one-half mile from the pediatrician's office. Jonny's pediatrician often sees him in his wheelchair, accompanied by his mother or grandmother, and waves or stops to chat. He has van services to school, and there is a Hoyer lift in the home, but his parents do not own a van. Recently, Jonny's father finds it more difficult to lift him. The family has also been challenged by the mental health problems of Jonny's two older brothers, and a serious eye injury suffered by his middle brother in a motor-vehicle accident. Jonny's pediatrician has cared for him and his two brothers since birth. Although the parents continue to believe that the HIB vaccine caused his catastrophic illness, they remain with the pediatrician. In general, they are satisfied with the individualized educational plan at a local public school. When he was 6.5 years old, Jonny's school aid reported that he attempted to touch her in the genital area. The pediatrician attended the meeting to review this incident and successfully advocated for Jonny by pointing out that this was an isolated incident; it did not occur again. At 6 years old Jonny functioned in the 1.5-2.5 year old range with motor skills in the 6-12 month level according to the Bayley Scales of Infant Development and the Vineland Adaptive Behavior Scales. In the past a neurologist and a physiatrist saw Jonny, but both of these individuals moved from the community. He had prior evaluations at a children's orthopedic clinic at a small community hospital and at the local Shriner's hospital. He had a tonsillectomy and adenoidectomy at 7 years old. He is currently treated for constipation and receives dental care at a clinic for people with disabilities. His pediatrician has always respected the parents for their care and obvious love for their disabled child. However, parental resistance to addressing major issues such as obesity has frustrated his pediatrician. When the pediatrician suggested that Jonny was eligible for the state's managed care program, which would convert Medicaid coverage to a state sponsored program with more extensive services and case management, Jonny's mother repeatedly said that she would "think it over." At the current visit, the pediatrician recommended an adjustment of Jonny's wheelchair, a hip x-ray, a referral to Shriner's Hospital, and an appointment with a pediatric surgeon to address the undescended testicle and possible hernia. Jonny's mother mentioned that he had been to Shriner's Hospital for hip pain two years earlier but was told nothing could be done "because nothing was wrong with his bone." The hip x-ray was normal as well as a complete blood count and a C-reactive protein. The pediatric surgeon did not find a hernia and deferred treatment of the cryptorchid testicle. His parents contacted the wheelchair company to arrange adjustments. The pediatrician called the medical director at the Shriner's Hospital to discuss Jonny's case, but 2 months after the initial visit, the parents had not arranged for an appointment at the Shriner's Hospital. Jonny's hip pain persisted. The pediatrician now wonders how he can more effectively address Jonny's current problems and improve overall care for him and his family.