What is included in patient education about Hermansky-Pudlak syndrome (HPS)?

Updated: Dec 13, 2019
  • Author: Jaclyn Scholtz, MD; Chief Editor: Dirk M Elston, MD  more...
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Patients need extensive education regarding visual impairment and visual enhancement. They need to understand the implications of photophobia, low-vision aids, nystagmus, and strabismus. The benefits of protective sunglasses must be explained to patients. Sunglasses with ultraviolet protection minimize the harmful visual effects of ultraviolet rays. Further, blue blockers, yellow tint, or polarized glasses may decrease photophobia in these patients. Low-vision aids enhance the lives of patients with Hermansky-Pudlak syndrome, as they can facilitate educational activities. Additional resources may include telescopic lenses, high-contrast school materials, and font magnifiers. Grønskov et al [22] have suggested that people with diseases such as Hermansky-Pudlak syndrome (1) use glasses (possibly bifocals) and dark glasses or photochromic lenses to abate effects of reduced visual activity and photophobia and (2) consider having strabismus and nystagmus corrected if necessary.

It must be stressed to patients that their skin is sensitive to the sun. They must wear broad-spectrum sunblock with a high SPF daily and avoid sun exposure. Wearing wide-brimmed hats is also encouraged. Patients should perform monthly self-skin examinations and have frequent dermatologic evaluations to evaluate for skin cancer. Patients and families need to understand the implications of the increased bleeding tendency. Patients must avoid contact sports and physical trauma. Surgery should be undertaken with caution.

Patients should be encouraged to follow up closely with their healthcare provider, given that they may develop complications related to their lung and heart function.

Since patients with Hermansky-Pudlak syndrome have many health issues, they might require psychological, educational, and social assistance from appropriate specialists.

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