The Children’s League helps three generations with inherited disease
Members of the Lepley family meet with The Children’s League staff. From the left are Jenny Vought, physical therapist; Sharen Lepley; Dr. John Tis, pediatric orthopedic surgeon; Bentley Lepley; Cody Lepley; and Cathy Growden, executive director, The Children’s League.
For the Cumberland Times-News Oct 10, 2017
CUMBERLAND — Three generations of the Sharen Lepley family have a strong connection to The Children’s League. Sharen Lepley, her son Cody and grandson Bentley have all been helped by the League and treated for the same inherited disorder known as Charcot-Marie-Tooth. The CMT disease is a group of inherited disorders that causes nerve damage. The damage is mostly in the arms and legs. CMT is also called hereditary motor and sensory neuropathy. The disease results in smaller, weaker muscles. It also can cause loss of sensation, muscle contractions and difficulty walking. Foot deformities such as hammertoes and high arches are also common. Symptoms usually begin in the feet and legs, but may eventually affect the hands and arms. Symptoms of CMT disease typically appear in adolescence or early adulthood, but may also develop in midlife.
Sharen Davis Lepley was born in 1964 and at that time her family was not aware that she carried the inherited gene for CMT. Her mother, Theresa Davis, was secretary for George Schwarzenbach, a local businessman and a member of the League board of directors. He encouraged Davis to take Sharen to the League to see Dr. Kirby von Kessler, a new pediatric orthopedist who saw children at the League.
At just 2 years old, Sharen had her first orthopedic visit with von Kessler for marked pronation of both feet and tight heel cords. She continued with routine visits in League clinics and was eventually diagnosed with having CMT, a disease that already affected several members of her family. Sharen had her first of many foot surgeries at age 14 at Children’s Hospital in Baltimore. She began brace wear and physical therapy to treat her CMT. Once she reached age 21 and was too old for League services, she continued necessary visits and surgeries with the good physicians who cared for her as a child.
CMT affected her feet and toes as a youngster, and as an adult Sharen Lepley has experienced the effect of CMT in her hands and fingers as well. For the 19 years that she was a patient at the Leauge, von Kessler was the orthopedist who cared for her. “CMT has never stopped any member of my family and it is not about to now,” Sharen Lepley said. “With The Children’s League giving us the support we need, we will be fine.” She continues a personal contact with the League through being an active member of The Children’s League Alumni Association. The group of past patients and families meets quarterly, offering support to one another and to current League patients and families.
Aware of her family’s history of CMT, she had great concern of the inherited disorder when her son Cody was born in 1990. With a careful watch from Mom and Cody’s pediatrician for any signs of CMT, Cody was eventually referred to The Children’s League when he was 6 years old. Ironically, von Kessler was still holding orthopedic clinics at the League and saw Cody in November 1996 for heel cord tightness and pronated feet. He continued yearly visits at the League and in 2002 was declared to have the inherited CMT. Cody was diagnosed with a scoliosis curve in 2005 and continued to be followed for that as well as the CMT. His CMT progressed, which affected his feet and toes, but he has not required any surgeries for either the CMT or the scoliosis. As an adult and knowing that CMT can progress even into adulthood, he is checked routinely. He stated, “With the League behind us, we will give CMT a swift kick.”
Sharen and her son share the same hereditary disorder of CMT. So with the arrival of Cody’s son Bentley in 2012, the family was once again concerned and faced with the possibility of CMT affecting the third generation of the family. As with his grandmother and his father, Bentley also became a patient of The Children’s League. Cody, knowing the effects of CMT that his mother lived with, wanted his son to be evaluated in an orthopedic clinic at the League. In 2014, Dr. John Tis, pediatric orthopedic surgeon, Johns Hopkins Hospital, evaluated 2 ½ year old Bentley for turning in of his feet and an unusual gait. Because of the long-standing family history of CMT, Tis recommended that Bentley have genetic testing to declare the positivity of him having CMT. It was also recommended that Bentley wear a foot ankle orthotic or a well-fitting, high top shoe to give support to the ankle and foot.
Bentley completed genetics testing in October 2015 and it was declared that he does carry the inherited disorder of CMT. Bentley continues his routine clinic visits with Tis and also receives weekly physical therapy at The Children’s League. Dad, grandmother, Jenny Vought, his physical therapist, and Tis are all keeping a close watch on this young man for any signs of the CMT progression and hoping that Bentley will not need any surgeries until he is a teenager.
Cathy Growden, executive director of The Children’s League, said, “Helping three generations of a family, getting to know each one personally and observing the compassionate medical care given by the League pediatric orthopedists and therapists is what I have come to appreciate and love so much about The Children’s League. The League’s commitment of caring for and serving children for 83 years is truly remarkable.”
The Children’s League serves children birth to 21 from Maryland, West Virginia and Pennsylvania, providing free orthopedic, cerebral palsy and cleft lip/palate clinics as well as free speech evaluations/therapy and physical therapy. To have a child scheduled or for information about the League or the Alumni Association, call the League office, 301-759-5200.