The nurse is providing teaching to the parents of a child with Marfan syndrome. Which topic is the priority for the nurse to address?

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11ecbb05_cac8_5600_b433_679840c02aa8_MD0009_00 Marfan syndrome is an autosomal dominant disorder affecting the connective tissues of the body. Abnormalities are mainly seen in the cardiovascular, musculoskeletal, and ocular systems. Clients with Marfan syndrome are very tall and thin, with disproportionately long arms, legs, and fingers. Cardiovascular manifestations of Marfan syndrome include abnormalities of the aorta and cardiac valves, including aneurysms, tears (dissection), and leaky heart valves that may require replacement or repair. Therefore, competitive or contact sports are discouraged due to the risk of cardiac injury and sudden death (Option A). (Option B) The client may also experience crowding of the teeth from a very high-arched palate. Preventive antibiotics prior to dental work may be needed to provide prophylaxis against infective endocarditis, especially in clients with an artificial valve replacement. However, this is not a priority. (Option C) These clients have an increased risk for scoliosis, especially during the adolescent years of increased growth; therefore, the child should be monitored regularly for curvature of the spine. This is not a priority. (Option D) Ocular problems (eg, lens dislocation [ectopia lentis], retinal detachment, cataracts, glaucoma) can be common for the child with Marfan syndrome. Annual eye examinations with an ophthalmologist are important to monitor for developing issues. Educational objective: Aortic root disease (ie, aneurysm, dissection) is the major cause of morbidity and mortality in Marfan syndrome. Therefore, competitive or contact sports are discouraged due to the risk of cardiac injury and sudden death.

The Nurse Is Providing Teaching to the Parents of a Child