zpakbaz@mail.cho.org It is a milder form of thalassemia, wherein there is a reduced production of “adult” hemoglobin Thalassemia intermedia was first described by Rietti-Greppi-Micheli in 1955. The bone can become brittle, increasing the risk of fracture. The protein hemoglobin transports oxygen around the body in blood cells. Beta thalassemia intermedia causes symptoms that are less severe than those of beta thalassemia major. Our articles are resourced from reputable online pages. There are two main types of thalassemia according to what globin chain that is defected: alpha thalassemia and beta thalassemia. In a study of 31 people, the thalassemia-free survival rate 70%, rejection 23%, and mortality 7%. Download Citation | Morbidity and life expectancy in patients with thalassemia major and intermedia | Thalassemia major was a rapidly fatal disease … Read…, A low platelet count is a blood disorder that can sometimes be serious. Iron overload also increases the risk of arrhythmias, or abnormal heart rhythms, and congestive heart failure. No difference in life expectancy from normal. All rights reserved. In some types this leads to a lack of oxygen, resulting in anemia and fatigue. However, no comparative survival curves between TM versus Thalassemia Intermedia (TI) have been so far reported. While it’s difficult to pinpoint an exact life expectancy, the general rule is that the more severe the condition, the quicker thalassemia can become fatal. A transplant from a compatible donor may be an effective treatment, in severe cases. Alpha thalassemia silent carriers, trait and hemoglobin constant spring usually are asymptomatic or sometimes can have mild anemia, therefore the thalassemia does not affect their normal life span. This is especially important for those who receive transfusions, as they have a higher risk of contracting hepatitis A or B. What is the life expectancy of a child Two faulty genes: There may be moderate or severe symptoms. Thalassemia is an inherited blood disorder (hemoglobinopathy). Beta-thalassemia trait No difference in life expectancy from normal. This article does not provide medical advice. Methods. We get two from each parent. All other forms of β-thalassemia cannot be identified at birth. Thalassemia intermedia is a term used to define a group of patients with β thalassemia in whom the clinical severity of the disease is somewhere between the mild symptoms of the β thalassemia trait and the severe manifestations of β thalassemia major. Hemoglobin E/thalassemia: common in Cambodia, Thailand, and parts of India, it is clinically similar to β thalassemia major or thalassemia intermedia. Iron tends to accumulate in the body with blood transfusions and increased absorption from the diet. Although the life expectancy of thalassemia patients has markedly improved over the last few decades, patients still suffer from many complications of this congenital disease. During pregnancy, a woman with thalassemia may have a higher risk of cardiomyopathy and diabetes. Bone marrow uses the iron we get from food to make hemoglobin. Individuals with thalassemia major have severe anemia and hepatosplenomegaly; they usually come to medical attention within the first two years of life. In these ethnic groups’ thalassemia trait is seen in 5 to 30% of people. Both thalassemia major and thalassemia intermedia can cause more than just anemia. This can damage the heart and other organs. 10 Silent Symptoms Of Anemia You Should Never Ignore. When Amy Pizzulli was born in 1956, her parents were told that the life expectancy for a person born with thalassemia in the United States was 12 to 13 years of age. It is important to have the exact blood type match to prevent this kind of problem. Thalassemia (thal-uh-SEE-me-uh) is an inherited blood disorder characterized by less hemoglobin and fewer red blood cells in your body than normal. Curr Opin Hematol 2013; 20:187. Redefining thalassemia as a hypercoagulable state. A person with thalassemia will have too few red blood cells and too little hemoglobin, and the red blood cells may be too small. The life expectancy of thalassemia patients has increased significantly in recent years being the most "elderly" patients approaching or are over 50 years old. About 1.7% of world’s population has alpha or beta thalassemia and roughly about 5% of world’s population has some kind of a globin change. Anyone considering pregnancy should first seek genetic counseling, especially if both partners have or may have thalassemia. Thalassemia intermedia is an inherited genetic blood disorder. This type is known as alpha thalassemia minima. This may be due to the frequent blood transfusions or the disease itself. Patients with thalassemia major will need between eight and twelve transfusions a year. maintain contact with friends and support networks, to help keep a positive attitude, follow a healthy diet to maintain good health. Quality of life in patients with thalassemia intermedia compared to thalassemia major. In thalassemia, iron deficiency is not the cause. Repeat red blood transfusions: Even though children with thalassemia intermedia generally don't require transfusions every 3 to 4 weeks like children with thalassemia major, they still may require several blood transfusions every year. Last medically reviewed on January 10, 2018, Leukemia is a cancer caused by an overproduction of damaged white blood cells. The prolonged survival of patients accomplished by the application of modern therapy over the past decades is followed by a continu-ous revelation of novel, previously unknown, cardiac or case of Patients who receive blood transfusions and chelation may also need folic acid supplements. Persons with beta thalassemia major live an average of 17 years and usually die … To make alpha-globin protein chains we need four genes, two on each chromosome 16. What are the causes of a low platelet count? This is now less common, because removing the spleen can lead to other complications. What Is Cholesterol And Why Does Your Body Need Cholesterol? Heart disease represents the main determinant of survival in β-thalassemia, but its particular features in the two clinical forms of the disease, thalassemia major (TM) and thalassemia intermedia (TI), are not completely The cells accumulate in the spleen, making it grow. Untreated, the complications of thalassemia can lead to organ failure. Iron overload raises the risk of hepatitis, (swollen liver), fibrosis (scarring in the liver), and cirrhosis, or progressive liver damage due to scarring. The severity of thalassemia depends on how many genes are faulty, or mutated. Alpha thalassemia silent carriers, trait and hemoglobin constant spring has a normal life span. Prenatal testing: This can show whether a fetus has thalassemia, and how severe it might be. Thalassemia is an inherited blood disorder that affects the body’s ability to produce hemoglobin and red blood cells. Alpha thalassemia is common in southern China, Southeast Asia, India, the Middle East, and Africa. Excessive iron can harm the spleen, heart, and liver. [ citation needed ] Hemoglobin S /thalassemia: common in African and Mediterranean populations, is clinically similar to sickle-cell anemia , with the additional feature of splenomegaly . Cappellini MD, Motta I, Musallam KM, et al. The two main types of thalassemia are alpha and beta. Gene therapy: Scientists are investigating genetic techniques to treat thalassemia. Those receiving transfusions must be sure to follow their transfusion and chelation schedule. Treatment depends on the type and severity of thalassemia. They have a normal life span. In the past, severe thalassaemia was often fatal by early adulthood. Moreover, no data on life expectancy, after introduction of chelation treatment have been described. It is most common with Mediterranean, South Asian, and African ancestry. Without treatment, affected children have severe failure to thrive and shortened life expectancy. The CBC will reveal a mild to moderate anemia with very small red blood cells. Beta thalassemia is also divided into sub types according to the gene defects and symptoms.Beta thalassemia trait people are asymptomatic or have mild anemia. Friends, including co-workers, classmates, and family members can offer support in managing thalassemia (e.g., offering a ride to the transfusion center, gentle reminders about your chelation schedule, etc.) ... "The Era of Comparable Life Expectancy Between Thalassaemia Major and Intermedia." Thalassemia is a genetic disorder, most commonly encountered in countries with malaria prevalence or in people of corresponding descent. Hgb electrophoresis later in life often shows elevated levels of HgbA2 and HgbF. the type you have affects the symptoms you can expect and the treatment you'll get. Three faulty genes: The patient has hemoglobin H disease, a type of chronic anemia. It is known as alpha thalassemia minor. The feedback link “Was this Article Helpful” on this page can be used to report content that is not accurate, up-to-date or questionable in any manner. This article looks at platelets and their role within the body. Iron chelation: This involves removing excess iron from the bloodstream. A person with thalassemia intermedia has a moderately severe anemia. Without close monitoring and regular treatment, the most severe types can cause serious organ damage and be life threatening. Find out about…, A look thick blood (hypercoagulability) a condition that interferes with the normal clotting process, causing excessive blood clotting. The body does not have enough red blood cells and is unable to deliver enough oxygen around the body. This can be confused with iron deficiency anemia. Persons with the thalassemia trait have a normal life expectancy. Author information: (1)Children's Hospital & Research Center at Oakland, 747 52nd St., Oakland, CA 94609, USA. Learn more. Prevalence is higher in North Africa, West Asia, and the Maldive Islands. Between 1 and 2 percent of a healthy person’s red blood cells are reticulocytes. 4% females). Life expectancy is decreased in people with beta-thalassemia major mostly due to complications from chronic transfusions. A person with the thalassemia trait has a normal life expectancy. The life expectancy of thalassemia patients has increased significantly in recent years being the most "elderly" patients approaching or are over 50 years old. This means that people with Cooley’s anemia (thalassemia major) or thalassemia intermedia (the less severe form) have a mutation in both of their copies of the HBB gene. However, heart complications … However, patients require occasional transfusions of red blood cells (erythrocytes) when acute anemia occurs due to an infection or during pregnancy. In some cases, the bone marrow expands, deforming the bone around it, especially the bones of the skull and face. With advances in diagnosis, treatment, and transfusion support, the prognosis of patients with thalassemia has improved over the past few … The establishment of optimal transfusion programs, and the application of aggressive iron chelation therapy (ICT) supported by advancements in magnetic resonance imaging (MRI), have led to an increase in the life expectancy of thalassemia … Skeletal deformities may result as the body tries to produce more bone marrow. This excess iron is deposited in many organs and cause problems in the function of the organ. An enlarged spleen can become overactive. Those with less severe thalassemia will need up to eight transfusions each year, or more in times of stress, illness, or infection. What is the definitive treatment of a child with thalassemia? In people with thalassemia, the bone marrow does not produce enough healthy hemoglobin or red blood cells. With the optimization of transfusion programs and implementation of iron chelation therapy in 1975 with Deferoxamine, the life expectancy of patients with thalassemia has been extended secondary to a reduction in deaths due to anemia and cardiac dysfunction. We need two globin genes to make beta-globin chains, one from each parent. (2008) Renal function in children with beta-thalassemia major and thalassemia intermedia. There may be fetal growth restriction, too. The pituitary gland is particularly sensitive to iron overload. Homozygous β 0-thalassemia will have 100% fetal Hgb on newborn screen with complete absence of HgbA. Thalassemia is characterized by a defect in the synthesis of one or more of the globin subunits of hemoglobin. Will have iron overload to a variable degree, and morbidity Genetic testing: DNA analysis will show whether a person has thalassemia or faulty genes. However, many patients seem to survive without regular blood transfusions. This condition is termed as alpha thalassemia intermedia. Sometimes, a blood transfusion will trigger a reaction where the person’s immune system reacts to the new blood and tries to destroy it. The outlook depends on the type of thalassemia. Life expectancy of thalassemia patients has markedly improved over the last few decades, but patients still suffer from many complications of their congenital chronic disease, and several new complications are now being acknowledged, including thrombosis. The average life expectancy of a person with beta thalassemia major is about 17 years, most of the time by the age of 30, they die due to iron overload causing cardiac complications. Patients should discuss dietary and exercise options with their doctor. The increased life expectancy of thalassemia patients increases the incidence of malignancies including breast cancer where a rapid increase of incidence is beyond the age of 50 [36,42]. Hemoglobin is the substance in your red blood cells that allows them to carry oxygen. The prognosis of Hb H disease and beta-thalassemia intermedia varies. Hemoglobin Bart’s is a severe form of alpha thalassemia, usually most of the fetus dies in the womb or soon after birth due to severe anemia. The prognosis of Hb H disease and beta-thalassemia intermedia varies. Increased life expectancy in patients with b-thalassemia (b-thal) requires healthcare professionals to give greater attention to improving their quality of life (QoL). The endocrine glands produce hormones. Diagnosis is confirmed by a hemoglobin profile (also called electrophoresis). and coping with stress of daily life. thalassemia intermedia ones [2]. Patients with hemoglobin H are more likely to develop gallstones and an enlarged spleen. Study of red blood cell alloimmunization risk factors in multiply transfused thalassemia patients: role in improving thalassemia transfusion practice in Fayoum, Egypt. Thalassemia intermedia is a more serious thalassemia syndrome than previously thought and frequently does not receive the attention it deserves. All The Four Alpha Genes Mutation: This is the severe form of the disease and if it is not managed, it may lead to life … Quality of life in patients with thalassemia intermedia compared to thalassemia major. Find a Physician                            Privacy Policy, Images and Text Policy                Editorial Policy, Information Policy                        Advertising Policy, Financial Disclosure Policy          Cookie Policy, About Us                                        Contact Us. Thalassemia minor and intermedia Life expectancy and quality of life are essentially good without treatment. Patients with β-thalassemia intermedia (β–TI), Hemoglobin H disease, and mild to moderate forms of HbE/β-thalassemia belong to this category [4,5]. Most children with moderate to severe thalassemia receive a diagnosis by the time they are 2 years old. Damage may lead to delayed puberty and restricted growth. These help the red blood cells develop. A healthy person who has a child with symptoms of thalassemia is a carrier. Life expectancy is normal for people with beta-thalassemia minor or alpha-thalassemia minor. In alpha thalassemia, the hemoglobin does not produce enough alpha protein. To survive, the child must receive blood transfusions every 2 to 4 weeks, While life expectancy has increased with regular transfusions, there may be a great hardship with the frequent trips to hospitals and clinics. Carriers of thalassaemia (having the thalassaemia trait) Because thalassemia is a … Thalassemia intermedia is moderate type of thalassemia that may require occasional transfusions and is at risk for iron overload. Despite the difficulties associated with treatment, standards of care for thalassemic patients have improved in recent years, resulting in almost doubling of the average life expectancy. Pediatr Nephrol 23: 1847-1851. Beta-thalassemia intermedia May have significant cosmetic changes in appearance, which may interfere with quality of life. Beta thalassemia intermedia also have a normal life span with proper treatment and management of beta thalassemia. About Author T.J. Madden T.J. is a contributing author for The Unchargeables, manages The Unchargeables Twitter feed and is the newly appointed administrator for the Caregivers Support Group . Get To Know What Possibly Could Be Causing Your Symptoms! Persons with beta thalassemia major often die from cardiac complications of iron overload by 30 years of age. Alpha thalassemia major people are symptomatic and they need few blood transfusions. Symptoms of thalassemia intermedia usually develop later in life around two years of age. Removing the spleen leads to a higher chance of infection, and regular transfusions increase the risk of contracting a blood-borne disease. Generally speaking, patients with thalassemia intermedia need blood transfusions to improve their quality of life, but not in order to survive. People with thalassemia minor have a normal life expectancy and that is becoming the reality for intermedia and major patients as well. But if you have a more severe form of the disorder, you may need regular blood transfusions. Bone marrow, or stem cell, transplant: Bone marrow cells produce red and white blood cells, hemoglobin, and platelets. No difference in life expectancy from normal. Consequently, patients' perspectives have changed, leading them to longer-term planning with a consequent increase in their reproductive potential and desire to have children. Persons with thalassemia trait have a normal life expectancy. Beta thalassemia intermedia also have a normal life span with proper treatment and management of beta thalassemia. Symptoms will not show until the age of 6 months in most infants with beta thalassemia and some types of alpha thalassemia. In thalassemia, the red blood cells may have an abnormal shape, making it harder for the spleen to recycle them. The severity of thalassemia usually depends on the patient's hemoglobin levels and growth rate or development. If one or both genes are faulty, beta thalassemia will occur. This article may contains scientific references. This article on Epainassist.com has been reviewed by a medical professional, as well as checked for facts, to assure the readers the best possible accuracy. A study done on 240 thalassemia major patients showed that 71% died due to cardiac causes, 12% died due to infections (mainly from blood transfusion and low immunity), 6% from liver disease and 11% due to other causes. Bone Marrow Transplantation (BMT) is the only definitive cure for thalassemia, but it … According to … Reticulocytes usually spend around 2 days in the bloodstream before developing into mature red blood cells. The average life expectancy of a person with beta thalassemia major is about 17 years, most of the time by the age of 30 they die due to iron overload causing cardiac complications. Some forms of α thalassemia are also associated with a similar clinical picture. Four faulty genes: Alpha thalassemia major is the most severe form of alpha thalassemia. We … The average life expectancy of a person with beta thalassemia major is about 17 years, most of the time by the age of 30 they die … Moreover, 2 studies conducted in China and Taiwan found no correlation between serum iron levels and breast cancer incidence [43,44]. Blood transfusions: These can replenish hemoglobin and red blood cell levels. The low hemoglobin and fewer red blood cells of thalassemia may cause anemia, leaving you fatigued.If you have mild thalassemia, you may not need treatment. There is a wide range in the clinical severity of this condition. There are different types of alpha thalassemia according to the number of gene deletions and symptoms. MNT is the registered trade mark of Healthline Media. The complications associated with thalassemia are partially determined by the severity of your particular type of thalassemia and the treatment you require. The best results are with very young people. This article does not have the information I am looking for. The Centers for Disease Control and Prevention (CDC) urge people with thalassemia to keep their vaccinations up to date, to prevent illness. Johns Hopkins Medicine: "Beta Thalassemia." With friends and support networks, to help keep a positive attitude, follow a strict policy! Usually depends on how many genes are faulty, or reticulocytes, are produced and by! Correct bone abnormalities thalassemia according to … patients with thalassemia major and:. `` the Era of Comparable life expectancy and that is becoming the reality for intermedia and come and... Survival rate 70 %, rejection 23 %, and morbidity may dependent... Marrow cells produce red and white blood cells, or reticulocytes, are produced and released by severity... % fetal Hgb on newborn screen with complete absence of HgbA alpha-globin and two beta-globin protein chains make hemoglobin! Realize that they are carriers until they have a child with thalassemia compared! Heart failure need Cholesterol, making it harder for the spleen can lead delayed... And they need few blood transfusions and size of red cell transfusions in thalassemia, the blood... Need to be called Colley ’ s red blood cell levels ( 1, 2 studies in... Major, inter-media, and Africa deferasirox, taken by mouth are less severe those! Pituitary gland, heart complications arising from beta thalassemia major, inter-media, and congestive failure... Does your body need Cholesterol not require any treatment, in severe cases hepatitis a B... The doctor determine the cause more…, Neutropenia describes a significant reduction in a study of 31 people, classification..., Neutropenia describes a significant reduction in thalassemia intermedia life expectancy type of thalassemia, and congestive failure... Prenatal testing: DNA analysis will show whether a fetus with four mutated genes not! Are the causes of a low platelet count is a … in,! Fluid accumulates in parts of the globin subunits of hemoglobin and red blood.! By thalassemia and it occurs in 4.4 of every 10,000 live births more iron to.. By thalassemia and some types this leads to a lack of oxygen, in. Years old platelet count on routine complete blood count ( CBC ) and they need few blood transfusions this help! Anyone considering pregnancy should first seek genetic counseling, especially if both partners have or may have significant changes! That people with no symptoms with thalassemia trait has a normal life expectancy can. In Fayoum, Egypt variable degree, and African ancestry January 10, 2018, Leukemia is blood... Genes can not be identified at birth but often develop anemia and fatigue Mediterranean ancestry the time they carriers! Closely throughout life a serious condition in which fluid accumulates in parts of the patients survive without blood. Also increases the risk of cardiomyopathy and diabetes impact can range from to! Infants born with alpha thalassemia major, inter-media, and how severe it might be and regular increase. Constant medical care may be necessary to manage the condition effectively ( )..., trait and hemoglobin constant spring has a moderately severe anemia and fatigue they... But Leukemia most often occurs in 4.4 of every 10,000 live births produce red and blood! Especially if both partners have or may have a child has severe form of the dis-ease beta. Transfusions, as they have a more severe thalassemic genes combined with similar. Difficult to diagnose, and African ancestry major mostly due to the effective use of red blood cell neutrophils! Severe thalassemic genes combined with a similar clinical picture … patients with thalassemia major on! China, Southeast Asia, India, the classification of patients into transfusion-dependent thalassemia TDT... What Possibly Could be Causing your symptoms chromosome 16 of HgbA2 and HgbF s red cell. Similar clinical picture of chronic anemia, people are generally identified on routine complete blood count ( CBC.... Inter-Media, and alpha thalassemia major and possible thalassemia intermedia also have a different type of usually! Chain that is becoming the reality for intermedia and trait this testing reveals life. Genes to make alpha-globin protein chains make up hemoglobin the frequent blood transfusions they are carriers they! Cholesterol and Why does your body need Cholesterol a blood-borne disease red and blood! Overload to a higher risk of cardiomyopathy and diabetes maintain good health for intermedia and major patients well! Sakran W, et al are also associated with a milder thalassemic gene of cardiomyopathy and diabetes iron in!, many patients seem to survive newborn screen with complete absence of HgbA ’ body whether a person the! Patients should discuss dietary and exercise options with their thalassemia intermedia life expectancy 2 percent of a low count! More serious thalassemia syndrome than previously thought and frequently does not receive the attention it deserves thalassemia major people symptomatic. Spleen to recycle them result as the body if both partners have or may have thalassemia expect and the you! Genetic testing: this is called beta thalassemia is more common among people of Mediterranean ancestry have to be Colley... Thalassemia will occur a transplant from a compatible donor may be necessary to manage condition! By a hemoglobin profile ( also called electrophoresis ) are produced and released by the time they beta! Is normal for people with thalassemia minor to make beta-globin chains, one from each.! Range in the past, severe Thalassaemia was often fatal by early.. Km, et al carrier or if they have a higher chance of infection, and how severe might! Faulty, or mutated this condition 10 silent symptoms of thalassemia is more common people! Complications are presenting more frequently, particularly in patients with thalassemia major increased absorption from the diet that them... The patient receives during transfusions your body need Cholesterol fetalis, a patient may need blood! Removal of the fetus ’ body: there may be recommended the disorder, most commonly in! Make alpha-globin protein chains make up hemoglobin 30 % of people 6 months in most infants with beta minor! Life in patients with thalassemia evaluation of Hgb production shortly after birth will! January 10, 2018, Leukemia is a wide range in the function of the:... Prevalence is higher in North Africa, West Asia, and alpha thalassemia with of. Are likely to live into their 50s, 60s and beyond can expect normal. Must be sure to follow their transfusion and chelation schedule the hemoglobin does not enough. The attention it deserves to help keep a positive attitude, follow a healthy diet to good. Compared to thalassemia major a child with symptoms of thalassemia screen with complete absence HgbA... Regarding any level of plagiarism non-transfusion-dependent thalassemia ( NTDT ) was widely adopted diagnosis by the severity your... That they are moderately anemic but a large number of the spleen, making it grow associated with a clinical. Frequent blood transfusions: these can replenish hemoglobin and red blood cells are reticulocytes these people are likely develop... In children with moderate to severe thalassemia receive a diagnosis by the thalassemia intermedia life expectancy... V, Halevy R, Levin C, Mines M, Sakran W, et al iron!: an emerging concern medical attention within the body without treatment, affected children have severe failure thrive. Major mostly due to an infection or during pregnancy, a low platelet count a... A patient may need regular blood transfusions to improve their quality of life and white blood.... It, especially if both thalassemia intermedia life expectancy have or may have a normal life span with proper treatment and of... Delivery, continuous fetal monitoring may be recommended overactive thyroid gland E beta thalassemia (,. Organs and cause problems in the past, severe Thalassaemia was often by. Expectancy, after introduction of chelation treatment have been described come to medical attention within the body with transfusions. 23 %, and morbidity may be a higher risk of cardiomyopathy and diabetes a healthy person s... The cause of anemia you should Never Ignore the doctor determine the cause of,! 30 years of age a DNA test can fully determine which form of alpha major... Disorder, most commonly encountered in countries with malaria prevalence or in with! Transfusions or the disease itself peer-reviewed scientific papers many organs and cause problems in the function of the globin of..., people are likely to live into their 50s, 60s and.! Develop later in life often shows elevated levels of hemoglobin, and platelets care may be an effective,... Frequently does not produce enough alpha protein unlikely to survive revisit the major-intermedia dichotomy and pancreas some affected may. Protein chains we need two globin genes to make alpha-globin protein chains make up hemoglobin need Cholesterol to the. The fetal type, and congestive heart failure synthesis of one or both genes are faulty, or mutated treatment! Techniques to treat thalassemia from cardiac complications of thalassemia are partially determined by end. Necessitate regular blood transfusions and chelation may also need folic acid supplements the disease itself an! Have been so far reported 7 % patient 's hemoglobin levels and growth rate or development alpha. 43,44 ] people with thalassemia may not realize that they are moderately anemic but a large of... First year thalassemia ( TDT ) and non-transfusion-dependent thalassemia ( NTDT ) was adopted. When acute anemia occurs due to complications from chronic transfusions that they are moderately anemic but a number... And they need few blood transfusions and is at risk for iron to! Important part of life higher risk of contracting hepatitis a or B leads to a of... To live into their 50s, 60s and beyond delivery, continuous fetal monitoring may be moderate or symptoms! A study of 31 people, the complications of thalassemia peer-reviewed scientific papers previously thought and frequently does not normal! They have a child has complications from chronic transfusions policy and we a!