Hemoglobin D (Hb D) is not a common disease but it when it does rear its ugly head it usually does so in India, Pakistan or Iran. It was first discovered in 1951 and differs from the other hemoglobin diseases by producing clinically significant conditions like sickle cell anemia and chronic hemolytic anemia of moderate severity (Desai 2004)
Hb D usually presents with a mild hemolytic anemia and a mild to moderate splenomegaly. Differentiating Hb D with other hemoglobin diseases is usually done by the zero thalassemia and the fact that it has also been reported to be associated with hematological malignancies. (Desai 2004 pg 13)
The disease itself is relatively rare and rather difficult to study due to its rarity. When it is found it is normally done so in north west India or additionally in Pakistan, Iran and other Asian countries such as Thailand. A common variation of the Hb D has been named Hb D-Punjab due to its occurrences in the Punjab region.
Basic biochemical properties for Hb D include the normal Hb properties with a substitution of the G AA C AA substitution at condon 121. In addition to that substitution, there is the -28 (A¡uG) mutation for the thalassemia alleles. One recent study sho
" (Landola 2007) The question that arises is what a community (whether that community is a small city, town, metropolitan or nation) can do to overcome and service a disease such as sickle cell that usually only affects a certain segment of the population. (Smith 2007) Many groups are attempting to raise both awareness and funds due to insurer's lack of coverage of the disease, especially for young people between the ages of 18-24 who may not have the capabilities needed to work full-time, and who often find themselves on state Medicaid programs because they cannot get coverage. These two Md's summarized the disease by stating that the patients with sickle cell hemoglobin D disease can usually be discovered with the following symptoms; osteo-articular pains, pallor and jaundice. (Desai 2004 pg 14) Hb D was first presented by Itano(1) in 1951 and from that time until 1965 only an additional eight cases were discovered. If, by promoting studies, such as the ones on Hb D, can enlighten those who control the purse strings, and that more financial support can be lent to the cause, then it is their duty to do so. One such patient is 21-year old Candice Johnson who has been dealing with sickle cell disease her entire life. Because sickle cell was for so long a disease that often claimed lives in childhood, the network of care often is weak and spotty for patients who reach age 21, with poorer insurance coverage, fewer doctors and less follow-up care for adults. A-Globin gene haplotype analysis demonstrated, for the first time, that all these Asian AD-Punjab globin genes were associated with haplotype --, previously undescribed in other populations. Hb D has also been reported to be associated with hematological malignancies. According to federal health statistics, sickle cell occurs in about one in every 600 African-American births and about one in every 1,000-1,400 Hispanic-American births. (Restrepo 1965 pg 1303) Additionally, they said that macrocytic and hyprochromic anemia has also been described in connection with the disease as well. (Lemondola 2007) Other current event topics confirm Lemondola's remarks and there are plenty of groups, regional and national in scope, that are working diligently to inform the public, and raise funds to combat the effects of this disease, especially in the African-American communities where there is a higher prevalence of the disease than with other groups.