Sickle cell anemia, cause of sickle cell anemia, symptoms of anaemia, Diagnosis and treatment

 What is sickle cell anemia,.

An inherited blood disorder in which abnormal hemoglobin cause the RBC to distort and attain sickle like shape is termed as sickle cell anemia ( or sickle cell disease) . 

The term inherited indicates that the disease is passed by genes from parents to their children. These sickled RBC are fragile and rapture frequently.thus the number of RBC reduce causing anaemia

Sickle cell anemia images 

The healthy RBC are flexible and round so easily travel through the blood vessels. However sickle cell anemia the RBC Becomes rigid, sticky and sickle - shaped. 

Due to these irregular shape the RBC block small blood vessels.thus showing down or blocking blood flow and oxygen to different body part. 

This result in a pain and organ damage sickle cell anemia is a life-long disease. It's severity varies from person to person.

Causes of sickel cell anemia: 

Sickle cell anemia is a genetic disorder in which the affected individual in herit.  From each parent a gene for hemoglobin S (HBS).

Sickle cell anemia trait is a condition in which the individual have only one copy of gene are carriers of sickle cell gene and deformed RBC however they do not develop sickle cell anemia.

Sickle cell image 

Hemoglobin play a major role in sickle cell anemia .

Hemoglobin molecules present in each RBC are the carries of oxygen from lungs to different organ and tissue and then they carry carbon dioxide to the lungs 🫁 for removal.

In condition of  Sickle cell anemia after delivering oxygen some of the hemoglobin molecules group together to form long road like hard structure which becomes like a sickle.

The normal RBC remain in the blood circulation for up to 120 days but sickle cell RBC remain only for 10 to 20 days. since this abnormal cell are not replaced frequently the blood Become deprived of RBC resulting in anaemia.

Sign and symptoms of sickel cell anemia

Symptoms of anaemia include:

1. Fatigue
2. Breathlessness
3. Rapid heartbeat 
4. Headache
5. Cold hands and feet
6. Paleness
7. Chest pain'
8. Sickle cell crisis
9. Fever 
10. Sudden temperature change 

Diagnosis of sickle cell anemia

Sikal sel anemia can be diagnosed by its symptoms and the result of routine blood test.

It should be diagnosed as early as possible in children. Hemoglobin electrophoresis is a simple, inexpensive blood test which diagnoses this condition even new borns.

This taste is performed simultaneously with the other routine in newborn screening test using the same blood samples.

 If the sample shows the presence of hemoglobin S, the hemoglobin electrophoresis test is considered to be positive. However the taste is repeated to confirm that the child is a sickle cell anemia cell trait carrier .

Treatment of sickle cell anemia

medication

1. Antibiotics:  Penicillin can be given to affected children from 2 month of their  age and  continued till they are at least of 5 year to prevent life-threatening infection (such as pneumonia).
2. Pain relieving medication: These are taken to ease pain during a sickle cell crisis.
3. Hydroxyurea : regular administration of this drug reduce to frequency of painful crises and also the need for frequent blood transfusion and hospitalisation.  hydroxyurea act by triggering the production of foetus hemoglobin to prevent sickle cell formation.
4. Assessing Stoke risk: the doctor can determine which children have a higher risk of stocke by using a special ultrasound machine. This test is painless and measures blood flow by using sound wave in children of 2 year age regular blood transfusion also decrease the risk of stocke.
5. Vaccination to prevent infection: the affected children's should be given childhood vaccination because they can be suffer from sever infection.
6. Pneumococcal vaccine and annual Flu shot are important for both affected children and adults.
7. Blood transfusion: In this treatment RBC of the donor are collected and administrated intravenously to the patient.
8. Bone marrow or  stem cells transplanting: in this treatment bone marrow of the affected individuals  is replaced with healthy bone marrow of the match donor.In cases when donors are not available stem cell from umbilical cord are used. Bone marrow plant due to its only suggest for children having significant symptoms and problems from sickle cell anaemia.