The Blue People of Kentucky

• They're known simply as the "blue people" in the hills and hollows around Troublesome and Ball Creeks. Most lived to their 80s and 90s without serious illness associated with the skin discoloration. For some, though, there was a pain not seen in lab tests. That was the pain of being blue in a world that is mostly shades of

white to black.

Fugate Descendants

• We learned that the matriarch of the family, Mary Fugate, who moved to Kentucky in the 1800s, was a carrier of a rare disease that prevented her blood from carrying the usual amount of oxygen.

• The disease, Methemoglobinemia, is genetically inherited and causes an enzyme deficiency that results in cyanosis, "a bluish color to the skin."

Biochemistry

• Red blood cells (RBC) from blue people are deficient in NADH diaphorase activity and cannot reduce the oxidized iron in methemoglobin (metHb).

Genotypes

• bb = affected individuals

• Bb = carriers

• BB = unaffected individuals

• The “blue” gene is located on chromosome 22

Symptoms

• Lung problems (shortness of breath)

• Heart disease

• Feeling blue

Frequency

• Position Name Position Name

• I-1 Benjamin Fugate (bd ca. 1750) II-1 Martin Fugate (bd ca. 1783)• I-2 Hannah ?? (bd 1783) II-2 Mary ??(Ritchie?/Smith?) (bd 1780)• I-3 William Smith II-3 Richard Smith (bd 1771)• I-4 ?? Betty Ritchie (bd ca. 1750)/Eunice ?? II-4 Alicia Combs (bd 1775/80)• I-5 Nicholas Combs (bd before 1764) II-5 Levi Fugate (bd 1795)• I-6 Nancy Gribsby (bd ca. 1750) II-6 Haney Noble (bd 1790)• I-7 William Fugate (bd 1760/65) II-8 Jane ?? • III-1 Hannah Fugate (bd 1811/12) IV-1 Martin Ritchie (bd 1829)• III-2 James Ritchie (bd ca. 1806) IV-2 Rebecca Williams (bd )• III-3 Andrew Fugate (bd 1808) IV-3 William Fugate (bd 1838)• III-4 ???? IV-4 Juda Campbell (bd 1837)• III-5 Zachariah "Ball Creek Zach" Fugate (bd 1817) IV-5 John "Blue" Fugate (bd 1832)• III-6 Mary Smith (bd 1814) IV-6 Zachariah Fugate (bd 1845)• III-7 Martha Smith (bd 1798) IV-7 Polly Campbell (bd 1844)• III-8 John Campbell (bd 1796) IV-8 Lettie M. Smith (bd 1834)• III-9 Sara "Sally" Fugate (bd ) IV-9 Elizabeth Campbell (bd 1827)• III-10 Lorenzo Dow Smith (bd 1816) IV-10 Henry Hudson (bd 1827)• III-11 Elizabeth Smith (bd 1825) III-12 Martin Fugate (bd 1820) • V-1 James "Big Jim" Ritchie (bd 1857) VI-1 Gabriel Fugate (bd 1890)• V-2 Hannah Fugate (bd 1856) VI-2 Zachariah "Big Man" Fugate (bd 1884)• V-3 Eleanor Fugate (bd 1860) VI-3 Elizabeth "Aunt Bessie" Fugate (bd 1876)• V-4 Lorenzo "Blue Anze" Fugate (bd 1854) VII-1 Mahala Ritchie (bd 1854)• V-5 Polly "Poss" Ritchie (bd 1886) VII-2 Levi Fugate (bd 1844)• V-6 Manuel "Man" Fugate (bd 1879) V-7 Nancy Hudson (bd 1849/50)• VIII-6 Luna Fugate (bd 1889) V-8 Charles Fugate (bd 1846)• VIII-7 John C. Stacey