Charcot-Marie-Tooth Disease: Information for School Nurses

Charcot-Marie-Tooth disease or CMT is a progressive neurological disorder causes damage to nerves connecting the brain and spinal cord.

Fast facts:

  • CMT is both an inherited genetic disorder that can run in families and also a spontaneously occurring condition.
  • Approximately 3 million or more people across the world suffer from CMT.
  • There are approximately 100 genetic mutations of CMT known currently but more research is being done.
  • CMT can present at any age and in all genders.
  • CMT is a type of muscular dystrophy
  • CMT does not affect the patient’s ability to think and learn although it can create anxiety and emotional concerns which can lead to difficulty learning.
  • CMT is not fatal in most cases although the progressive disability can be extremely detrimental.
  • Onset of symptoms is usually noted in adolescence or early adulthood, occasionally in mid-adulthood.

Symptoms

  • Muscle pain
  • Muscle weakness
  • Frequent tripping, ankle sprains, general clumsiness
  • Hand tremors
  • Cold hands and feet
  • Drop foot
  • Burning, shooting, stabbing, stinging nerve pain
  • Chronic fatigue
  • Numbness
  • Curled fingers
  • Muscle atrophy of legs and/or arms
  • Curled toes
  • High arches or flat feet (no arches)
  • Difficulty with fine motor skills
  • Difficulty breathing
  • Rare symptoms include breathing difficulties caused by respiratory muscle weakness, swallowing or speaking difficulties, neurogenic bladder, hearing loss, optic neuropathy and vocal cord paralysis.

Diagnosis

  • Genetic testing in-utero if family history is known. This requires CVS testing (amniocentesis).
  • Genetic testing through blood or saliva for the patient.
  • Nerve conduction study to assess the relationship of the nerves to the muscles
  • Electromyogram
  • Review of symptoms and physical exam by neurologist including sensation exams, muscle strength assessment, visualization or limbs and feet.

Treatment

  • Physical and occupational therapy
  • Hearing aids for hearing loss
  • Pain medications for more severe pain
  • Supportive aids such as braces, walkers or wheelchair
  • Surgery to repair malformed joints
  • Assisted ventilation for those with breathing difficulties or sleep apnea (CPAP), in more severe cases patients may need to depend on a ventilator for respiratory support due to muscle weakness.

Managing CMT in a school setting

  • Student should be receiving PT and OT – sometimes at school
  • Student should be educated on safety measures at school such as navigating hallways, playground, cafeteria
  • Activities such as safety using the bathroom should be accommodated
  • PE class should be modified for the student to avoid competition and strength training and focus more on the recreation aspect. Depending on students health status student could also be excused from PE.
  • Consider 1:1 aid for assistance with ADL’s and navigation of daily activities at school.

504 accommodation suggestions

  • May receive physical therapy and/or occupational therapy at school
  • 1:1 aid to assist with eating, using the bathroom, ambulation, general safety.
  • Student will use wheelchair, walker, or supportive devices in school.
  • Modified PE to accommodate student’s abilities: student should not be required to compete or progress in activities but be permitted to participate as their abilities allow for recreational purposes.
  • Additional textbooks provided to prevent transport of books to home or changing classes
  • Individuals with CMT may have difficulty with dine motor skills causing difficulty with handwriting and using a keyboard. Student should be granted extra time to complete tests and classwork, and potentially the option to take tests and do classwork orally as opposed to written.
  • Elevator access if available
  • When possible, student’s needs should be taken into consideration when creating class schedule ie: student assigned to classes that are closer together to minimize travel throughout the building.
  • Student should be seated in the classroom in a location that is conducive to their ability to navigate the classroom.
  • Emergency evacuation plan should accommodate the student. A school staff member should be assigned to the student in case of emergency (if no 1:1 support). A “safe room” should be established in case student is unable to be evacuated and this information should be readily available to the fire department and rescue teams.
  • School field trips should be evaluated for safety concerns for the student ie: dropping student off at front door of the destination, ensuring that location is handicapped accessible, having a wheelchair lift for the bus, ensure that all staff present on the trip are aware of student’s needs.
  • Take each student’s case individually and tailor additional accommodations to the student’s needs.

CMT and COVID risk: CMT does put the patient at higher risk for COVID related complications, especially those with breathing problems such as a weak diaphragm or reduced lung capacity. Click this link for some general guidelines.

CMT Fact sheet from MDA.org

School Accommodation Recommendations from MDA.org

Some of the websites I’ve used to gather this information are:

CMTAUSA.ORG

MDA.ORG

Hopkinsmedicine.org

Published by Emme Mauer M.Ed., BSN, RN, CSN

Mom to two preemies, anxiety sufferer, postpartum depression survivor, and school nurse extraordinaire.

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