The blood disorder beta thalassemia
Thalassemia
An inherited blood disorder characterized by the formation of abnormal form of hemoglobin.
What is the prognosis of beta thalassemia?
Those suffering from hemoglobin H disease or beta thalassemia intermedia may show moderate to severe symptoms and anemia; They can lead a normal life, with regular monitoring and occasional transfusions; Those suffering from beta thalassemia major may present with more severe symptoms and may need frequent blood transfusions, almost every few weeks
How do you treat beta thalassemia?
The patients of beta thalassemia, according to doctors, need lifelong care that includes regular blood transfusions and medicines. The disease is quite common, like sickle cell anemia, among the Tharu community.
What is the best treatment for thalassemia?
Your healthcare provider will figure out the best treatment based on:
- Your age, overall health, and medical history
- How sick you are
- How well you can handle certain medicines, procedures, or therapies
- How long the condition is expected to last
- Your opinion or preference
What is the treatment for beta thalassemia?
Your doctor is likely to ask you a number of questions, including:
- Do symptoms occur all the time or come and go?
- How severe are the symptoms?
- Does anything seem to improve the symptoms?
- What, if anything, appears to worsen the symptoms?
Which of the following is the possible complication of beta thalassemia?
Beta thalassemia major causes major problems and can result in early death. Complications may include delayed growth, bone problems causing facial changes, liver and gall bladder problems, enlarged spleen, enlarged kidneys, diabetes, hypothyroidism, and heart problems.
What are the long term effects of thalassemia?
When left untreated, this condition can lead to problems in the liver, heart, and spleen. Infections and heart failure are the most common life-threatening complications of thalassemia in children. Like adults, children with severe thalassemia need frequent blood transfusions to get rid of excess iron in the body.
How long can a beta thalassemia patient live?
Survival of thalassemia patients The cumulative survival rate from birth until 10 years old was 99%. After reaching the age of 20 years, 88% of the patients survived until 30 years, 74% survived until 45, 68% survived until 50, and 51% survived until 55 years old.
What is the permanent treatment for thalassemia?
Stem cells are the cells inside bone marrow that make red blood cells and other types of blood cells. A stem cell transplant is the only treatment that can cure thalassemia.
How does thalassemia affect the brain?
Brain damage was more severe in patients with sickle cell-thalassemia disease who had more crises per year. Conclusion: This study suggests that patients with thalassemia intermedia and those with sickle cell-thalassemia disease may have asymptomatic brain damage.
Is beta thalassemia life threatening?
Beta thalassemia major (also called Cooley's anemia). People with beta thalassemia major have life-threatening anemia. They need regular blood transfusions and other medical care.
Do blood transfusions shorten your life?
Results: The median length of survival was 95.0 (+/- 2.5) months. Twenty-four percent of patients died within 1 year after the transfusion, 30 percent within 2 years, 40 percent within 5 years, and 52 percent within 10 years.
Can thalassemia patients marry?
Abstract. Aim: With good care, patients with transfusion-dependent thalassemia major (TDTM) can reach older ages, marry and reproduce.
Can beta thalassemia have kids?
Parents who have beta thalassemia trait can have a child with beta thalassemia disease or sickle cell disease (Sβ+ Thalassemia or Sβ0 thalassemia disease). This is why it is important to understand how beta thalassemia trait is passed on, and how it can affect the health of your children and grandchildren.
How is β thalassemia currently treated?
Stem cell or bone marrow transplants are the only cure for thalassaemia, but they're not done very often because of the significant risks involved. Stem cells are produced in bone marrow, the spongy tissue found in the centre of some bones, and have the ability to develop into different types of blood cells.
Can thalassemia be cured permanently?
Bone marrow transplant This is the only treatment that can cure Thalassemia permanently. It is a heavy treatment that is only offered in the most severe forms of the disease.
What is the fastest way to increase red blood cells?
5 nutrients that increase red blood cell countsred meat, such as beef.organ meat, such as kidney and liver.dark, leafy, green vegetables, such as spinach and kale.dried fruits, such as prunes and raisins.beans.legumes.egg yolks.
What are the complications of beta thalassemia?
The blood disorder beta thalassemia can bring complications that include things like bone damage, heart problems, and slow growth in children. Treatment can help you or your child avoid many of these problems.
Why does beta thalassemia make your spleen bigger?
Just like a muscle grows when you use it more, overuse makes your spleen get bigger. If your spleen gets too large, you may need an operation called a splenectomy to remove it.
What is the name of the virus that causes thalassemia?
People with thalassemia (as well as other hemolytic anemias) require a high rate of new red blood cell production. Parvovirus B19 is a virus that causes a classic illness in children called Fifth Disease.
Is thalassemia a blood disorder?
The complications associated with thalassemia are partially determined by the severity of your particular type of thalassemia and the treatment you require. Because thalassemia is a blood disorder, any organ can be affected.
Can beta thalassemia cause heart problems?
Heart issues are not uncommon in people with beta thalassemia major. Enlargement of the heart occurs early in life due to anemia. With less blood, the heart needs to pump harder, causing enlargement. Transfusion therapy can help prevent this from occurring.
Does splenomegaly increase RBC?
In an attempt to do this, the spleen grows in size (splenomegaly). This RBC production is not effective and does not improve anemia. Early initiation of transfusion therapy can prevent this. If the splenomegaly causes an increase in transfusion volume and/or frequency, splenectomy (surgical removal of the spleen) may be required.
What Are The Long Term Effects Of Thalassemia?
Thalassemia is the condition of production of abnormal hemoglobin synthesis due to the mutation of genes regulations its synthesis. The severity of the disease is defined on the basis of number of related genes that gets mutated. Thalassemia, if not managed properly, can lead to various complications in the long run.
Conclusion
Various long term effects due to thalassemia involve severe anemia, splenomegaly, hepatomegaly, bone deformation, increased risk of fracture, iron toxicity and organ failure.
What is thalassemia major?
Thalassemia major is a hereditary anemia characterized by little or no ß-globin production, which results in hemolysis ...
Who developed HSCT for thalassemia?
Lucarelli and his colleagues at Pesaro initially pioneered HSCT in thalassemia and described a standard "Pesaro" risk stratification scheme based on liver size by physical examination, fibrosis detected on liver biopsy, and chelation history.
Does beta thalassemia have a higher QOL?
The hypothesis is that patients who had beta thalassemia who have undergone a hematopoietic stem cell transplant (HSCT) and are >1 year post-HSCT will have less long term comorbidities and a higher quality of life (QOL) as compared to those with beta thalassemia who are maintained on supportive care. In order to assess quality of life, ...
Does thalassemia improve life expectancy?
Although improvements in conservative treatment have improved the prognosis of thalassemia considerably disease and transfusion related complications in affected patients progress over time, causing severe morbidity and shortened life expectancy.
Is thalassemia a long term outcome?
Morbidity in thalassemia patients (both transplant related and non-transplanted) is attributed to iron overload associated end organ damage. Although there is data on long term outcomes in chronically transfused thalassemia patients, the data on long-term outcomes after HSCT for thalassemia remains limited.