Day 2: 3rd Annual Achondroplasia Research Conference recap
Day 2 sparked our curiosity. We know that the achondroplasia and skeletal dysplasia communities are divided on the controversial topics of limb lengthening surgery and Vosoritide. Generally speaking, growing taller is not a goal for the majority of these communities. This means that a “cure” for achondroplasia is not a desired outcome. The MCDS-Therapy team believes that the primary aim of any therapy should be to help patients with the more challenging aspects that come with living with achondroplasia or other skeletal dysplasias. It should not include a needless focus on height. Our primary aim is to listen to all the voices in the achondroplasia community and let people make their own decisions.
We were keen to sit in on an open conversation around these topics to learn more about their pros and cons. We were grateful that Chandler gave us the safe environment to do so at her conference. We encourage you to read this section of our recap with an open mind whichever way you fall on the subjects.
Day 2
The last day of the conference began with John E. Herzenberg, Director of Pediatric Orthopedics at Sinai Hospital and Director of the International Center for Limb Lengthening at the Rubin Institute for Advanced Orthopedics.
Dr. Herzenberg opened his presentation by saying, “What fits one person, doesn’t fit another.” He acknowledged that limb lengthening surgery doesn’t treat medical problems and can be seen as a cosmetic procedure over a medical one. He combatted that response by explaining how the world wasn’t made for short stature individuals. Everything is out of reach, whether it be the gas pump, light switches, gas pedals or kitchen appliances. This reality is experienced behind closed doors for those living with achondroplasia and other skeletal dysplasias.
Dr. Herzenberg stated that there are really only two options for these patients: change the environment or change the individual. He understood how harsh that sounds, but reassured the room the goal of any limb lengthening surgery is to achieve functional height to improve a patient’s quality of life. It’s not cosmetic.
We learned the following during Dr. Herzenberg’s insightful and thought-provoking presentation:
- The average achondroplasia male stands at 4 feet 3 inches.
- The average achondroplasia female stands at 4 feet 1 inch.
- The functional height limb lengthening surgery looks to achieve is 4 feet 10 inches.
- External fixation is used to correct a patient’s bowed legs after they have reached full, adult height. The goal is to realign the legs, not grow or look taller.
- Achondroplasia patients are unable to straighten their arms, which results in limited reach and range of motion.
- Achondroplasia patients can choose to undergo humeral lengthening to increase the function and reach of their arms. This is done safely around 95% of the time.
- Limb lengthening surgery can be done when a patient is age 10-11. He or she can opt to have his or her arms, legs or both lengthened.
- Arm and leg lengthening are done in stages. If a patient opts for limb lengthening surgery at age 10-11, he or she will return for another surgery when he or she is 12-13 and then again when he or she is 16 years old for the final surgery. After all three surgeries are complete, a patient can gain up to 8.5 inches in length.
Continuing on with this interesting topic, physical therapy expert, Anil Bhave, took to the stage. Anil has 28 years of experience working in physical therapy. He currently works as the Clinical Director of Orthopedic Rehabilitation and Director of Wasserman Gait Laboratory International Center for Limb Lengthening, ICLL, RIAO, at Sinai Hospital. Anil explained how his job is to maintain a patient’s range of motion, bone health and muscle health. By doing so, he works to increase a patient’s flexibility over time.
Muscles naturally resist being lengthened and prefer to be stretched at a slower rate than bones. Lengthening a patient’s muscles results in an increased tension in his or her knees and legs. Not all muscles respond the same to the stretch that occurs during limb lengthening surgery. In fact, the calf muscle, femur, hamstring and hip flexor muscles can all cause the most problems when it comes to limb lengthening. The humorous is more adaptable to the surgery, but all patients undergoing a limb lengthening surgery are required to have physical therapy afterward.
Depending on the patient’s age, muscle strength loss can be a problem. If the patient is under 25 years old, then his or her muscle strength should return. If the patient is in his or her 30s, 40s and 50s, he or she could lose his or her range of motion. Hydrotherapy pools and anti-gravity treadmills can be used to promote active mobility in patients. Deep tissue stimulation can also be done to increase mobility.
Anil ended his presentation with a few recommendations for those needing physical therapy after limb lengthening surgery. They are:
- Patients should have physical therapy five times a week.
- Physical therapy should be reduced down to one to two times a week after the bones consolidate and external fixations are removed.
- To ensure that patients maintain their full range of motion, dynamic splinting, casting or bracing can be used. Do four hours on and one hour off.
- To keep muscles strong post limb lengthening, patients should use electrical stimulation for 60 to 80 minutes a day.
- Perform self-stretching, but try not to flex the hip and knee together to avoid nerve tension. Instead, try extending the hip and bending the knee to take the pressure off the nerves. Certain stretches can also help to decompress the spine.
