According to a research carried out by ABRASTA in 2009, with 74 people with intermediate and major thalassemia, representatives from 13 States of Brazil, most of them reported that their treatment depends not only on the hematologist (the physician specialized in diseases affecting the blood), but on the assessment and monitoring of other professionals, especially the cardiologist (being searched by 43.84% of the interviewees) and the endocrinologist (periodically consulted by 35.62%). It occurs for the own characteristics of the disease which incline the person with thalassemia to certain problems or for the side effects of the treatment. Check below the major complications, why they occur and how to avoid them:

Heart

the iron overload is the major villain for this vital organ, even when the person with thalassemia strictly follows the chelation therapy. It occurs because the actual accumulation of the mineral in the heart is not always diagnosed, and the dose of chelating agent administered may not be ideal, for example. With advancements in the treatment, such as modernization of the chelating agents and, consequently, greater adhesion to the therapy or even the possibility to make the magnetic nuclear resonance exam with T2*, which precisely reveals the amount of iron accumulated in the heart, the risks of a person with thalassemia to develop a cardiopathy has been decreasing. But even so a lot of attention is needed with the "bosom friend".

PREVENTION
As the cardiac complications are still the major death cause among people with thalassemia, the periodical monitoring by a cardiologist, from the adolescence, is essential – especially if the young person starts to show symptoms that may indicate some problem in the heart, such as quivers, dismays, breathlessness, pain above the stomach, excessive tiredness and swelling.

Vessels and arteries

In people with thalassemia, these are the path to receive the red blood cell concentrate, rich with the healthy hemoglobins which will assure long and quality life. Ironically, these same vessels and arteries may also impair the health of a person with thalassemia. More and more researches attest that the disease which causes an inherited and chronic anemia is also associated with a greater risk of thrombosis, a health problem characterized by formation of thrombus (clogs) inside the veins making it difficult for the passage of blood. According to experts, the chances of such obstruction occurring increase when the person with thalassemia already developed or has a history of thrombosis in the family, when he/she doesn't have the spleen, suffers from hepatic cirrhosis, uses catheter to receive transfusion and chelating agent or, we still don't know well why, if the person has intermediate thalassemia.

For people with thalassemia, the most severe consequence of the thrombosis has been pulmonary hypertension, in which situation the thrombus (clogs) reach the artery connecting the heart to the lung, preventing the flow in the region. (Pathophysiology of PI is much more complex: chronic anemia and hemolysis play also a relevant role) The chances of this complication increase with the age and deserve attention because according to experts, currently, when the death cause of a person with thalassemia is cardiac, the fault lies on the iron overload in the organ or on the pulmonary hypertension.

PREVENTION
To avoid the thrombosis, it is recommended to avoid or being attentive to situations which favor appearance of the problem – in practice, one should stop smoking, tell the surgeon on the diagnosis of thalassemia before going through any surgical intervention and, in the case of women, frankly talk to the gynecologist if she is to begin using contraceptives or making any other type of hormonal replacement.

Hormones

The iron overload in the organism of the person with thalassemia usually has as target certain glands and organs responsible for production of hormones – which may lead, if chelation and specific medical monitoring do not appropriately occur in each stage of life, to a hormonal unbalance capable of affecting growth, delaying beginning of the puberty and even cause diseases.

When the target is...

Pituitary gland

Located in the brain basis, it is responsible for production of several hormones, among them that of growth and the gonadotrophins, associated with the hormonal revolution which marks the arrival of the puberty.

Consequences: low height and delay in the sexual development (hypogonadism), such as growth of the mammae and testicles.

Thyroid

Gland that lies on the neck and has the function to keep the metabolism pace. Depending on how it is affected, it may accelerate or decrease the "speed" of the organic functions, respectively giving rise to the hyperthyroidism (marked by tachycardia, cases of diarrhea, loss of weight) or hypothyroidism (sleepiness, cold, mental and physical slowness and gain of weight). In case of the thalassemic patients, the hypothyroidism occurs.

Consequences: sleepiness, cold, mental and physical slowness and gain of weight.

Pancreas

The beta cells of this organ are responsible for production of the hormone insulin, which controls the levels of glucose (sugar) in the blood. If, for any reason, these cells are prevented from working, insulin starts to be missed to metabolize the glucose ingested (in this stage the organism starts to suffer from intolerance to glucose) and then the sugar can no longer penetrate inside the cells and starts to be accumulated in the blood (now, the status is for diabetes).

Consequences: according to data from the Thalassemia International Federation (TIF), nearly half the people with major thalassemia suffer from intolerance to glucose and out of 10% to 30% of these people develop diabetes mellitus (diabetes type 2) at some point in life.

PREVENTION
From the 10 years of age, periodical consultation to the endocrinologist and check-up by means of specific blood exams that may identify the problems mentioned above.

Bones

One of the most common problems of people with thalassemia is presenting a fragile and thin bone structure, favoring appearance of an early osteoporosis. The anemia itself and the hyperactive bone marrow of the thalassemic people are behind this status, but not only: the excess iron in the organism, as well as the endocrinological problems – delay in the puberty and sexual development, for example – also interfere absorption of calcium by the bones and, consequently, the bone density.

PREVENTION
The ideal, according to experts, is that a person with major thalassemia would be submitted as of the 15 years of age to bone densitometry exam, which assesses the formation and the bone state, in addition to identifying signs of osteoporosis. Unfortunately, there are few specialized centers in Brazil that make an assessment aimed at adolescents, with appropriate interpretation of the exam results. On the other hand, the person with thalassemia may collaborate to indirectly protect the bones, with his/her adhesion to the transfusions and the chelation therapy, the practice of regular exercises, a diet rich in calcium, consumption of supplements of vitamin D – when recommended by the physician – and the abandonment of bad habits, such as smoking.

Blood Security

After all advancements of the last decades in the donation process, selection and preparation of the blood pack, quality control of the blood banks and transfusion, the chances of a donor transmitting to the receiver a disease by the bloodstream – such as syphilis, hepatitis B and C, HIV, barber bug fever – are minimal, but still exist.

PREVENTION
Ffrom the first blood transfusion and every six months, the person with thalassemia who needs the classic treatment should be submitted to serological exams to discard the contamination by diseases transmitted by the blood.

ATTENTION TO THE EMOTIONS
It is not easy for anyone, especially children and adolescents, to have to deal with a chronic disease, submitting to the treatment of blood transfusions and chelation on a monthly basis and still going through the periodical assessment of an extensive and varied medical team to control side effects that affect the self-esteem, such as low height, bone deformities and delay in the sexual development. Because of that, a psychological or psychiatric monitoring in some stages of life may help the person with thalassemia and, also, assure adhesion to the treatment.

Complications for usage of chelating agents

Such as every medicine, the chelating agent also causes some side effects that need to be taken into account, since the person with thalassemia periodically uses this type of medication. Learn what each one of the three drugs currently indicated for chelation may cause to the health and how to protect. It is worth mentioning that frequent usage increases the risks to certain problems, but it doesn't mean that they will necessarily occur:

Deferoxamine: high-frequency hearing loss and hearing deficit, cataract and reduction in the growth of the backbone.

PREVENTION
Making every year, respectively, hearing assessment (audiometry), fundus exam and control of weight and height.

Deferiprone: neutropenia, that is, fall in the amount of white blood cells – the cells that defend the organism --, which may favor opportunistic infections.

PREVENTION
Blood count every seven or 10 days and stop immediately treatment in case of fever or signs of infection and seek for medical advice and white blood cell count.

Deferasirox: renal problems.

PREVENTION
Preferably every three months, the execution of exams to assess the good functioning of the kidneys, responsible for filtering the blood and indirectly controlling the arterial pressure.