How Are Pes Cavus and Scoliosis Safely Managed in FRDA?

In Friedreich ataxia (FRDA), changes to your skeleton like high arches (pes cavus) and spinal curvature (scoliosis) largely result from progressive neuromuscular imbalance and growth-related alignment changes ^[1][2]. As the nerves lose their ability to sense the body’s position (proprioception) and control movement, some muscles become weak while others become tight, pulling the bones out of alignment over time ^[1][3]. Managing these changes requires an individualized, proactive team approach. While you cannot permanently “fix” or reverse the structural changes with exercise alone, a combination of physical therapy, orthotics, bracing, and in some cases, surgery can help preserve your mobility, reduce pain, and keep you functioning as well as possible ^[4][5].

The Neuromuscular Connection

Skeletal changes in FRDA are very common, especially in individuals who develop symptoms earlier in life (typical or early-onset FRDA) ^[6][7]. The root cause of these visible changes is multifactorial, driven by the nerves rather than the bones themselves:

• Sensory and Postural Loss: FRDA damages the sensory pathways that tell your brain where your body is in space. To compensate for this loss of balance and coordination, your brain alters how you stand, walk, and sit, which places uneven stress on your spine and feet ^[1][2].
• Muscle Imbalance and Tightness: As the disease progresses, certain muscles weaken, while others—particularly the calf muscles—can become very tight. This tightness can result from muscle shortening, contractures (permanent shortening of the muscles and tendons), or in some cases, spasticity (a specific type of nerve-related muscle stiffness) ^[3].

Because these changes are driven by progressive nerve damage, they can begin very early in the disease course. High arches and scoliosis often appear around the same time as the onset of ataxia, and sometimes even before noticeable coordination issues ^[8][7].

Managing Pes Cavus (High Arches)

Pes cavus is a hallmark of FRDA ^[8]. Whether you are walking or using a wheelchair, the goal of foot management is to keep the foot flexible, prevent skin breakdown, reduce pain, and maximize safe function.

• Physical Therapy: An individualized program of gentle range-of-motion exercises, strengthening, and balance training prescribed by a physical therapist can improve short-term motor function ^[4][9]. It is important not to stretch aggressively into pain, as this can cause injury in a weak limb.
• Orthotics and Custom Footwear: Special shoe inserts can distribute your weight more evenly and cushion bony areas. However, because FRDA reduces sensation in your feet, you may not feel rubbing or a developing sore ^[1]. Any new insert must be professionally fitted and broken in gradually, with daily skin checks.
• Bracing: If the foot begins to drop, your doctor may recommend an ankle-foot orthosis (AFO). This brace supports the foot and ankle, providing a stable base. Like inserts, AFOs require daily skin checks to ensure they aren’t causing pressure sores.
• Surgical Options: If the foot becomes rigid, painful, prevents shoe-wearing, or causes recurrent skin problems despite conservative measures, orthopedic surgery may be considered to release tight tendons or realign the bones. Surgery is highly individualized, and recurrence of the deformity is possible as the condition progresses.

Managing Scoliosis (Spinal Curvature)

Scoliosis is very common in early-onset FRDA (developing in over 90% of these patients), with major curve progression typically occurring during growth spurts and puberty ^[7].

• Individualized Monitoring: Your orthopedic team will individualize your clinical exams and standing X-rays based on your age, growth, curve size, and rate of change ^[7][10]. They will measure the Cobb angle (an X-ray measurement of the curve’s size) to track progression while balancing the need for information with radiation exposure.
• Bracing and Physical Therapy: A back brace may be prescribed for growing children or for adults needing seated posture support. In neuromuscular diseases like FRDA, bracing may improve comfort or temporarily slow progression, but it does not typically stop the curve entirely and must be monitored for its effects on breathing and skin comfort.
• Surgical Intervention: For severe, progressive curves, spinal fusion (an operation that joins selected vertebrae to correct or stabilize the curve) may be an option. More than half of those with the most severe spinal curves eventually undergo corrective surgery ^[7]. Surgery may improve sitting balance, reduce pain, and improve overall quality of life ^[5]. While available studies have not consistently shown a loss of walking ability following spinal surgery, functional decline remains a possible individual risk and should be discussed during shared decision-making ^[10].
• Surgical Preparation: Because FRDA often involves hypertrophic cardiomyopathy (a thickened heart muscle) and respiratory changes, major surgery requires meticulous planning ^[11]. Postoperative complications can occur, so your surgical team should include specialists in orthopedics, cardiology, anesthesiology, and pulmonology to thoroughly assess and manage your risk ^[11].

When to Contact Your Care Team

Contact your care team promptly if you notice:

• A new foot wound, blister, or persistent redness from a brace or shoe.
• Rapidly worsening back pain or changes in your spinal curve.
• A sudden loss of physical function, balance, or repeated falls.
• New difficulty breathing, particularly when sitting or lying down.