The use of psychological treatment to help people manage thalassemia

Plain Language Summary

Thalassaemia is a group of genetic blood disorders. Blood transfusion is needed for serious forms of the condition treated, but it leads to a high level of iron in the body. The excess iron is usually removed by drugs through 'chelation therapy'. Other less conventional treatment is bone marrow transplantation, which is restricted by age, and hydroxyurea, a drug designed to raise the level of fetal hemoglobin in collecting and anemia reduction . Psychological support would seem appropriate that thalassemia is a chronic disease with a major role for the control of a self-management. We were unable to find any trials to include in this review. There is a some reports that people learn about their condition improve compliance with chelation therapy. But from the information currently available, we can not recommend the use of specific psychological therapy in thalassemia. We believe there is a need for a big test the value of psychological therapy for thalassemia evaluate.

Abstract

Background: Thalassaemia is a group of genetic blood disorders characterized by the absence or reduction in the production of hemoglobin. Severity is variable from less severe anemia by thalassemia intermedia, deeply serious anemia (thalassemia major). In thalassemia major other complications that delayed growth, bone deformation, and an enlarged spleen. Blood transfusion is needed for severe forms of thalassemia treatment, but this results in excessive accumulation of iron in the body (iron overload), removed mostly by a drug called Desferrioxamine by chelation therapy. Non-routine treatment is bone marrow transplantation (that age limit), and possibly hydroxyurea, designed to raise the level of fetal hemoglobin to raise, and thus anemia. In addition, psychological therapy seems appropriate for improving the outcome and adherence to medical treatment.

Objectives: proof that people with thalassemia, psychological treatments improve the ability to handle the situation, and the improvement of both the medical and psychosocial outcomes research.

Search methods: We went to the Cochrane cystic fibrosis and genetic disorders haemoglobinopathies Group Trials Register comprising references from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Search the Internet are also exported.

Date of most recent search of the Group's Trials Register haemoglobinopathies: October 27, 2011.

Selection criteria: All randomized or quasi-randomized controlled trials comparing the use of psychological intervention (psychological) intervention in people with thalassemia.

Data collection and analysis: No trials of psychological therapy were found in the literature for inclusion in this review.

Main results: There are currently no results reported.

Authors' conclusions: As a chronic disease with a major role for the control of a self-psychological support seems appropriate for the management of thalassemia. But from the information currently available, no conclusions about the use of specific psychological therapy in thalassemia. This systematic review has clearly identified the need for well designed, adequately powered, multicenter, randomized controlled trials to assess the efficacy of specific psychological interventions for thalassemia.

Demography

Thalassemia trait is found in all populations, but it is most common in people from the following origins:

  * Southeast Asia (Vietnam, Laos, Thai, Singaporean, Filipino, Cambodia, Malaysia, Burma,and Indonesia)
  * Chinese
  * East India
  * African
  * Middle East
  * Greek
  * Italian
  * Trans Caucasian (Georgian, Armenian and Azerbaijani)


There are two main types of thalassemia trait: alpha thalassemia and beta thalassemia trait characteristic. Individuals who have beta thalassemia trait have a normal beta globin gene and one such that little or no beta globin change. There are subtypes of alpha thalassemia trait. Individuals with alpha thalassemia silent trait 'lacks an alpha globin genes. When two alpha globin genes are missing, an individual said that alpha thalassemia trait might have. There are two different ways as well. The 'cis' type of alpha thalassemia trait occurs when two genes of the same chromosome missing. This type is most common in those from Southeast Asia, Chinese, or Mediterranean descent. The "trans"-type of alpha thalassemia trait occurs when two genes from different chromosomes is missing. This type is most common in African Americans.

Thalassemia trait is generally not thought to cause health problems, although women with the trait more likely to have anemia of pregnant women develop without the characteristic can be. Obstetricians sometimes treated with folate supplementation. Most types of thalassemia traits cause the red blood cells to be smaller in size than normal, a condition called microcytosis. Sometimes it is incorrectly referred to as "low blood". Since iron deficiency is the most common cause of microcytosis, doctors sometimes mistakenly prescribe iron supplements to individuals with thalassemia trait. Iron supplements for that reason, doctors must rule out thalassemia trait and / or perform laboratory tests to evaluate iron levels. A person with thalassemia trait can also iron deficient, but if he or she is, iron supplements can lead to an excess of body iron. Excess iron can deposit in many parts of the body, causing organ damage in the long term.

The inheritance of thalassemia


The instructions for alpha globin production are present in duplicate, two genes on each chromosome 16 for a total of four. The instructions for beta globin production on chromosome 11, one gene on each chromosome for a total of two.


Half a woman's genetic information in each egg, including one chromosome 11 and chromosome 16. The same applies to the formation of the seed. At fertilization, the total recovery of 46 chromosomes. Which chromosome 11 or 16 are transferred randomly determined. There is nothing a mother or father (or not do) to establish that chromosome, and therefore which allele is transmitted to his or her children.

The recessive inheritance

The thalassemias are a group of recessively inherited conditions. Remember that genes come in pairs. In recessive both members of the pair associated with the condition should be changed, or mutated. This is a difference of a dominant position, only one copy of the gene needs to be changed in order to manifest symptoms.

In recessive conditions, a favorable trait or carrier state exists where an individual has both a normal and a mutated copy of the gene. The term "normal" is a convention to describe only the copy of the gene, an allele is called, which is most often seen in the general population. The normal allele is able to compensate for the missing or altered function of the mutant allele. Therefore, the individual with the characteristic symptoms seen in the disease. It is generally thought that each of us carry 7-8 of these recessive traits that disease if present in a "double dose causes."

It is not uncommon for a person with a recessive disease for the first person in his or her family, the condition to be. Typically, this happens when both parents carry the trait. This is a difference of a dominant inherited condition in which the members of a family in several successive generations are affected.

Some recessive conditions
Attached earlobes
Sickle cell anemia
Cystic fibrosis
Thalassemia

Some dominant conditions
Tongue rolling (try)
Huntington's disease