Daniel Akill-Attieh's Blog | 310: THALASSAEMIA Part I – Make the necessary effort to search for, diagnose, and treat the root-cause; otherwise………

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310: THALASSAEMIA Part I – Make the necessary effort to search for, diagnose, and treat the root-cause; otherwise………

03 July, 2008

THALASSAEMIA Part I – Make the necessary effort to search for, diagnose, and treat the root-cause; otherwise………

Thalassaemia, UK, thalassemia, USA: is normally or usually an inherited autosomal recessive blood disease. The genetic defect results in a reduced rate of synthesis of one of the globin chains that make up hemoglobin, which causes the formation of abnormal hemoglobin molecules, and which in turn causes the anaemia which is the characteristic presenting symptom of thalassemia. However, thalassaemia can be caused by vector-borne diseases of which malaria is the main and worst culprit – read my postings #292, 293, 294, 295, 296, and 297 on Mosquitoes and Malaria.

Autosomal: a chromosome other than one that determines sex

Globin: the protein component of hemoglobin

Haemo or hemo: a prefix – definition: blood. Greek haima

Haemoglobin or hemoglobin: red blood cells containing iron and protein that transport oxygen to all parts of the body

Thalassemia is a quantitative problem of too few globins synthesized, whereas sickle-cell disease – another product of malaria – is a qualitative problem of synthesis of non-functioning globins. Thalassaemia usually results in under-production of normal globin proteins, often through mutations in regulatory genes that imply structural abnormalities in the globin proteins themselves. The two conditions may overlap, however, since some conditions which cause abnormalities in globin proteins – sickle-cell disease – also affect their production – thalassaemia.

Thalassaemia is a hemoglobin-pathogenic condition. It is particularly prevalent among Mediterranean peoples and this geographical association was responsible for its name: Thalassa (θάλασσα) Greek for sea, and Haema (αίμα) Greek for blood

In combination with other hemoglobin-pathogenic conditions:

Thalassemia can co-exist with other hemoglobin-pathogenic conditions, of which the most common are: Hemoglobin E-Thalassemia: common in Cambodia, Thailand, and parts of India; clinically similar to β-Thalassemia-major or Thalassemia-inter-media

Hemoglobin S-Thalassemia: common among African and Mediterranean populations; clinically similar to sickle-cell anaemia, with the additional feature of splenomegaly: an enlargement of the spleen, which usually lies in the left upper-quadrant of the abdomen. Splenomegaly is usually associated with an increased workload, and a response to hyper-function. It is therefore not surprising that splenomegaly is associated with any disease process that involves abnormal red blood cells being destroyed in the spleen – malaria. Other causes include congestion due to portal hypertension and infiltration by leukemia and lymphoma – all of these and more are, and can be, caused by malaria.

Hemoglobin C-Thalassaemia: common among Mediterranean and African populations

Hemoglobin C/β^o-Thalassaemia: a moderately severe hemolytic anemia/splenomegaly and Hemoglobin C/β⁺-Thalassaemia: a milder disease

Anyone with – or suspect they have – thalassaemia should consult a properly qualified haematologist who should in turn, and with the assistance of an oncologist, make the necessary effort to search for, diagnose, and treat the root-cause; otherwise the treatments, prescriptions, blood-transfusions, surgeries, and bone-marrow transplants shall all come to naught, and the patient will die, eventually – after much pain, discomfort, misery, sleepless nights and restless days, and anxiety……Etc…to all involved and/or concerned

Thalassemia may co-exist with, or be the result of, folic acid and iron deficiency, only in Thalassemia-Minor. Folic Acid is part of the Vitamin B-Complex, and Iron is ferrous sulfate, which is administered to patients suffering from anaemia. Iron cannot be well- assimilated without Folic Acid

Thalassemia-Major and Inter-media

Thalassemia-Major Patients are given frequent blood transfusions that lead to iron overload. Iron-chelation treatments are necessary to prevent iron overload damage to the internal organs in patients with Thalassemia-Major. Because of recent advances in iron- chelation treatments, patients with Thalassemia-Major can live long lives if they have access to proper treatment. Popular chelating agents: deferoxamine and deferiprone and of the two, deferoxamine is preferred since it is associated with fewer side-effects

Chelation treatment: administering chelating agents in order to remove or get rid of an overload of heavy metals such as lead or iron from the bloodstream

The most common complaint by patients is: difficulty in complying with the intravenous chelation treatments because they are painful and inconvenient. The oral chelator Deferasirox was recently approved for use in some countries and may offer some hope

Untreated Thalassemia-Major eventually leads to death usually by heart failure therefore birth screening is very important

In recent years, bone-marrow transplant has shown promise with some patients suffering from Thalassemia-Major. Successful transplants eliminate the patients’ dependencies on blood transfusions

All Thalassemia patients are susceptible to health complications that involve the spleen, which is often enlarged and frequently removed, and gall stones. These complications are mostly prevalent in Thalassemia-Major and Inter-media patients

Thalassemia-Inter-media Patients vary a lot in their treatment needs depending on the severity of their anaemia

Thalassemia-Minor

Contrary to popular belief, Thalassemia-Minor Patients should not avoid iron-rich foods by default. A serum ferritin test can determine what their iron levels are and guide them to further treatment if necessary. Thalassemia-Minor, although not life threatening on its own, can affect quality of life due to the effects of mild to moderate anaemia; as well as psychic disorders: panic attacks, depression, anxiety, irascibility, sometimes confused states, and mood disorders: digestive disorders: anorexia and/or bulimia; respiratory disorders: coughs, dyspnea, asthma, or intermittent asthmatic crises. In Thalassaemia-major and Inter-Media Patients, these Physical and Psychic (Mental) diseases and/or side-effects are more severe

A spectrophotometer-study showed that the antioxidant, indicaxanthin, found in beets, are more effective than either Trolox or Vitamin C. Collectively, the results demonstrate that indicaxanthin can be incorporated into the redox machinery of β-thalassaemia RBC and protect the cells from oxidation, reducing or interfering with perferryl-Hb, a reactive intermediate in the hydro-peroxide-dependent Hb degradation

Daniel: This means, eat as many beets as you can, whether in season, fresh and cooked or boiled; or out of season, in cans or jars

Haematology or hematology is the branch of biology (physiology), pathology, clinical laboratory, internal medicine, and pediatrics that is concerned with the study of blood, the blood-forming organs, and blood diseases. Haematology includes the study of etiology – the causes of various phenomena - diagnosis, treatment, prognosis, and prevention of blood diseases. The laboratory work related to the study of Haematology is performed by Medical Technologists

Blood diseases affect the production of blood and its components, such as blood cells, hemoglobin, blood proteins, the mechanism of coagulation, etc

Leukaemia or leukemia: Greek leukos λευκός white, and haima αίμα blood; is a cancer of the blood or bone marrow and is characterized by an abnormal proliferation (production by multiplication) of blood cells, usually white blood cells (leukocytes). Leukemia is a broad term covering a spectrum of diseases. In turn, it is part of the even broader group of diseases called hematological neoplasm

Haematological neoplasm or malignancies are types of cancer that affect blood, bone- marrow, and lymph nodes. As the three are intimately connected through the immune system, a disease affecting one of the three will often affect the others as well: although lymphoma is technically a disease of the lymph nodes, it often spreads to the bone marrow, affecting the blood and occasionally producing a Para-protein

Chromosomal translocations are a common cause of these diseases, although this is uncommon in solid tumors. This leads to a different approach in diagnosis and treatment of haematological malignancies

Although hematological malignancies are a form of cancer, they are generally treated by specialists in Haematology, although in many hospitals Oncology specialists also manage or assist in these diseases

Anorexia Nervosa: eating disorder marked by an extreme fear of becoming overweight that leads to excessive dieting to the point of serious ill-health and sometimes death

Bulimia: a condition in which bouts of overeating are followed by under-eating, the use of laxatives or self-induced vomiting. It is associated with depression and anxiety about and over putting on weight

Neoplasm: Cancer (medicine), any of more than 100 diseases characterized by excessive and uncontrolled growth of abnormal cells, which invade and destroy other cells

Oncology: the branch of medicine that deals with the study and treatment of malignant tumors (cancer) and seeks to understand their origin, development, diagnosis, treatment, and prevention. A medical professional who practices oncology is an oncologist. The term originates from the Greek onkos (ογκος), meaning bulk, mass, or tumor, and the suffix -ology, meaning the study of

Oncologists often coordinate the multidisciplinary care of cancer patients, which involve physiotherapy, counselling, and clinical genetics, to name but a few. On the other hand, oncologists often have to liaise with pathologists on the exact biological nature of the tumors that are being treated

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