Children and Adolescents With Marfan Syndrome: 10,000 Healthy Steps and Beyond
Condition: Marfan Syndrome
MeSH terms: Marfan Syndrome; Arachnodactyly; Syndrome
Study type: Interventional

Summary: 
      Marfan patients are at risk of sudden death due to weakening of the wall of the large blood
      vessel leading from the heart (aorta). The wall of the aorta weakens and dilates which can
      rupture, leading to death, and sometimes during intense exercise.

      There is some evidence in Marfan patients that a stiffer aorta increases risk for rupture.

      For some time, clinical care has focused on what type of exercise these patients should avoid
      due to risk for aortic dissection. Little clinical emphasis has been placed on encouraging
      patients to engage in routine and safe exercise such as walking. Informed by this evidence,
      the investigators propose to collaboratively investigate whether regular exercise improves
      aortic health in adolescent Marfan patients.
    

Detailed description: 
      Marfan syndrome is an inherited disorder of the connective tissue, which provides material
      and support for the skeleton, muscles, and blood vessels. Marfan patients are at risk of
      sudden death due to weakening of the wall of the large blood vessel leading from the heart
      (aorta). The wall of the aorta weakens and dilates which can rupture, leading to death, and
      sometimes during intense exercise.

      Typically, the weakening process starts when elastin fibers in the aorta become fragmented.
      There is some evidence in Marfan patients that a stiffer aorta increases risk for rupture.

      For some time, clinical care has focused on what type of exercise these patients should avoid
      due to risk for aortic dissection. Little clinical emphasis has been placed on encouraging
      patients to engage in routine and safe exercise such as walking. These young patients also
      frequently choose sedentary lifestyles, most likely due to limitations imposed by parents as
      well as adolescents' own perceptions of what is safe for them and their physical
      capabilities. Another complicating factor is that these patients often experience difficulty
      coping with their diagnosis. Informed by this evidence, the investigators propose to
      collaboratively investigate whether regular exercise improves aortic health and coping skills
      in adolescent Marfan patients.
    

Intervention name(s): Exercise
Intervention type(s): Behavioral
Intervention description(s): Across studies in adolescents in general population, 60 minutes of recommended daily exercise level is achieved, on average, within a total volume of 10,000-11,700 steps. The investigators will assess baseline physical activity quantitatively by using an accelerometer worn on the wrist during waking hours over a 7-day period. Each participant will be given a Garmin device to place on their wrist that will track their steps. Then patients will be asked to complete 10,000 steps daily for 6 months (acclimation over a week), which will be encouraged by daily text messages or e-mail, and weekly phone calls by the intervention team in addition to a Garmin and Facebook peer group.
Intervention model: Single Group Assignment
Masking: None (Open Label)
Primary purpose: Prevention