Hemoglobinopathy Treatment Drugs; Help Relieve Pain and Minimize Complications
Hemoglobinopathy treatment drugs include hydroxyurea
(Siklos, Hydrea, and Droxia), ACE inhibitors, antibiotics, and analgesics.
Blood transfusions should be given only when strictly indicated.
Hemoglobinopathy is a genetic disorder in which there is an abnormal structure
or production of a hemoglobin molecule. This group of disorders includes
thalassemias, sickle cell anemia, hemoglobin S-C disease, and hemoglobin C
disease. They fall into two main groups: thalassemia syndromes and structural
hemoglobin variants (abnormal hemoglobin). Common symptoms of the condition
include enlarged spleen, shortness of breath, severe pain, and growth problems
in children.
Hemoglobinopathy is a serious condition that can lead to
complications in many parts of the body. These include cardiovascular disease
and heart attack. Hemoglobinuria, the clinical term for this condition, occurs
when the body does not properly use the circulating blood's supply of red blood
cells, leading to an accumulation of the substance in the body. This often
occurs in the extremities such as the ankles, feet, and legs. The most common
symptoms include pain and swelling in the areas where the body normally
produces hemoglobin; nausea and vomiting; fatigue; and low energy levels. Moreover,
hemoglobinopathy has the potential to spread to the lungs, resulting in a
pulmonary embolism, which can be life-threatening.
Hemoglobinopathy
treatment drugs include oral medication, such as deferiprone
(Ferriprox) or deferasirox (Jadenu and Exjade). Another drug, deferoxamine
(Desferal), is given by needle. These drugs are used to treat thalassemia. An
individual with thalassemia trait has a normal life expectancy. However, heart
complications can make this condition fatal before the age of 30 years.
Consuming beetroot, lentils, sweet potatoes, and banana can help reduce
symptoms of thalassemia, as these foods have folic acid that can help in
producing more red blood cells. Thus, with the increasing prevalence of
hemoglobinopathies such as thalassemia, the demand for hemoglobinopathy
treatment drugs is also increasing.
According to the National Organization of Rare Disease
(NORD), thalassemia is relatively rare in the United States, but is one of the
most common autosomal recessive disorders in the world. The incidence of
symptomatic cases is estimated to be approximately 1 in 100,000 individuals in
the general population.
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