Diamond Blackfan Anemia: What is this Disorder and is it Dangerous?

Diamond Blackfan Anemia: What is this Disorder and is it Dangerous?

Diamond blackfan anemia or short for DBA is a kind of blood disorder which is often diagnosed in children at age one. It is a rare disorder in which children are unable to make enough red blood cells (they are the cells which carry oxygen to every other cell in the human body). The red blood cells are made in the bone marrow and those cells which will later become red blood cells die before they get to develop in the case of children who are diagnosed with DBA. Diamond blackfan anemia equally affect all babies regardless of race and it affects 7 in every one million infants.

Early Symptoms

The diagnosis of DBA is most commonly made in the first year of life and the symptoms appear as early as when they are just 2 months old. This disorder may be hereditary so it is possible if a member of your family has a history with Diamond blackfan anemiayour baby will be affected too.

Symptoms that show up include:

  1. Weakness
  2. Fatigue
  3. Heart murmur
  4. Rapid heartbeat
  5. Irritability
  6. Abnormal pale appearance

Moreover, people who are diagnosed with DBA have a high risk of several other alarming complications that are all related to the malfunctioning bone marrow. They commonly have a higher-than-average risk of developing myelodysplastic syndrome (MDS). It is a type of disorder where undeveloped blood cells unable to fully develop normally. Certain cancers may develop to affected individuals as well such as cancer of blood forming tissue called acute myeloid leukemia (AML) as well as a type of bone cancer called osteosarcoma.

Read More : What Is the Connection of Sickle Cell Anemia and Malaria? Find Out Below!

Furthermore, individuals with DBA have abnormal physical appearances such as:

  1. Unusually small head size (microcephaly)
  2. Low frontal hairline
  3. Wide-set eyes (hypertelorism)
  4. Droopy eyelids (ptosis)
  5. A flat and broad nose bridge
  6. Small and low-set ears
  7. Small lower jaw (micrognathia)

Some people may also have an opening in the roof of the mouth (cleft palate) and it may be accompanied with or without a split in the upper lip (cleft lip). Their neck may also be short and webbed and small higher-than-usual shoulder blades. Their hands also may look unusual like they are malformed and thumbs are missing. The most noticeable physical abnormality is the slow growth which always results in short stature. They also have kidney problem, structural defects of the heart.

The level of severity of DBA may vary even if they come from the same family tree. There is also a non-classical diamond blackfan anemia that has been identified recently. This type of DBA, however, has less severe symptoms which sometimes include mild anemia that begins in adulthood.

Treatment

Below are some treatment for DBA.

  1. Steroids

Steroids are strong drugs which can fight inflammation in the body. Even though doctors have no idea exactly how they work in DBA, but results indicate that steroids can make 80% of individuals with DBA produce more red blood cells.

  1. Blood transfusions

Blood donation can also help individuals with DBA. They should be given health and matched blood every four or six weeks in order to increase the amount of red blood cells.

  1. Stem cell transplant

This procedure requires finding matched donors that usually come from a close family member. They transfuse their healthy stem into the individual with DBA.

Bottom line, Diamond blackfan anemia is a serious illness that needs to be diagnosed since the children are in their first year of age.

What Is the Connection of Sickle Cell Anemia and Malaria? Find Out Below!

What Is the Connection of Sickle Cell Anemia and Malaria? Find Out Below!

Do sickle cell anemia and malaria have a correlation? This question may be yours which have been popping in your mind for a long time. Sickle cell anemia is known as genetic disease. This disease modifies the structure of hemoglobin. The hemoglobin is the oxygen which carries protein the red blood cells. The altered hemoglobin which is not carrying oxygen may bunch with the deoxygenated hemoglobin. The deoxygenated hemoglobin deforms red blood cells and causes the blood to mass over the body. A specific gene HBB contains a protein which is known beta hemoglobin. The beta hemoglobin creates the sickle-shaped. This disease is commonly found in the America where the occurrence of malaria is high and it is caused by the sickle cell anemia.

When a person is ready to suffer a sickle cell disease. the red blood cell is more likely shape a sickle. This sickle cell leads to other diseases such as anemia, strokes, and lungs problems. In addition, when this disease leads to the sickle cell of anemia, it may have some effects to the malaria parasite. This disease is inherited disorder when a person has this disease from both his parents. The disease will develop gradually and die early. In contrast with the person who only gets the gene from one of both his parents. He may have a few of sickle-shaped of the blood.

sickle cell anemia and malaria
sickle cell anemia and malaria distributions

How is the sickle cell of anemia and malaria linked?

Read More : How to Treat Sickle Cell Anemia

There is a correlation which shows that the sickle cell can reduce the growing of malaria parasites especially for the people who lives in the endemic area such as African. The epidemic is higher to the African lowlands than the highland. Here are the correlation of the sickle cell anemia and malaria.

sickle cell anemia and malaria

  1. When the malaria parasite starts to live in the bloodstream of somebody which has sickle cell trait. The sickle cell breaks down and blocks the parasite. This condition makes the malaria parasite will be unable to continue its development. In other words, the malaria parasite is not able to survive.
  2. The hemoglobin is the need for the malaria parasite to grow. the sickle cell traits release the beta hemoglobin which prevents the growth of the malaria parasite.
  3. The malaria parasite needs potassium to grow. However, the less of potassium which is the result of the low oxygen of the sickle cell anemia and malaria parasite cannot continue its growth.
  4. Meanwhile, people with sickle cell disease has red blood cells with very low of the oxygen. when the malaria parasite enters the bloodstream. it reduces the oxygen even more. this condition leads to a destruction of its life.

The relationship between the sickle cell anemia and malaria is irrefutable. we also understand that the malaria parasite grows in the red blood cells. the sickle red blood cells are alleged to reduce and destruct the malaria parasite. Since, the sickle cells have beta hemoglobin, low oxygen, and do not produce potassium, and these things are the important things needed by the malaria parasite to grow up.!

How to Treat Sickle Cell Anemia

How to Treat Sickle Cell Anemia

How to Treat Sickle Cell Anemia

While it can be cured, learning how to treat sickle cell anemia is very important. It can be the difference between life and death for very small children with it. Effective treatment can also help people to live as normal of a life as possible in spite of their health condition. Today, we know more about sickle cell anemia than ever before so we have the means to effectively treat it.

Controlling the Pain

Individuals with sickle cell anemia often suffer from a great deal of pain. This can make it very hard for them to work, to care for their family, or even to get through normal daily activities. Pain medications are often necessary to make the individual more comfortable. Such medication will need to be taken daily for the best results. The doctor will have to determine the right medication as well as the dose of it for each individual case. For mild cases, over the counter pain medications may be sufficient.

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One type of pain medication offered is called Hydroxyurea and it is generally only for individuals that have a very serious sickle cell anemia diagnosis and they are at least 18 years of age. However, there are some serious side effects with it. For example it may increase the risk of someone developing leukemia or tumors after extended use.

How to Treat Sickle Cell Anemia

Infections are common with sickle cell anemia, so the form of medication that is offered has to be effective for preventing that from occurring too. Infections can cause permanent damage in the body which is why they are so dangerous and need to be intercepted. When an infection is detected, a form of medication known as antibiotics must be taken to destroy it. The antibiotics may need to be taken for a period of time that lasts from 7 to 10 days.

Health Assessment

Preventing serious problems for individuals with sickle cell anemia is very important. A regular health assessment is important to the overall care. Vision loss and eye damage are common for people with sickle cell anemia. Checking the eyes at least every six months is very important.

Stroke is another serious health problem associated with sickle cell anemia. Today there are tests that allow for people to be assessed when it comes to their risk of a stroke. In many medical facilities children start to get these types of tests when they are only about 2 years of age.

How to Treat Sickle Cell Anemia

Blood Transfusions

Among the methods for hoe to treat sickle cell anemia, the use of blood transfusions is used. There are safeguards in place to make sure the blood used is safe and it is clean. Most experts believe that these types of blood transfusions at regular intervals can reduce the risk of a stroke for patients with sickle cell anemia.

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One of the problems though with blood transfusions is that they can introduce too much iron into the body. The use of a type of medication called Deferasirox is often used to prevent that. When a person has too much iron in the body, vital organs can end up being damages so this needs to be closely monitored.

Bone Marrow Transplants

One of the most effective forms of treatment for sickle cell anemia is a bone marrow transplant. How to treat sickle cell anemia with it though can be complicated to fully understand. There has to be a very good match of donor bone marrow to conduct the transplant. This can be parents or siblings but very rarely is it every from a complete stranger. Still, there have been drives where people are asked to come be tested to see if they would be a good match for someone in need.

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