Sickle cell anemia (sickle cell) is a disorder of the blood that results from inherited abnormal hemoglobin. Hemoglobin is an oxygen-carrying protein in red blood cells that, if abnormal, leads to the formation of sickle-shaped red blood cells. In this case, the red blood cells will be brittle and prone to rupture. Excessive rupture of red blood cells leads to a decrease in their number and anemia. This condition is called sickle cell anemia. Sickle cells are visually irregular and can clog blood vessels. Sickle cell anemia is one of the most common inherited anemias.
Sickle red blood cells block or completely block normal blood flow to various tissues in the body, leading to pain crises in these patients. The patient normally feels pain and the pain often affects the bones. Touch-sensitive abdominal pain is common and can resemble appendicitis pain. Pain crises are often accompanied by pain. Dehydration should be prevented to prevent further tissue damage, and in some cases, intravenous fluids may be needed. Other methods, such as biofeedback, self-hypnosis, or electrical nerve stimulation may be helpful.
Sickle cell anemia is an autosomal recessive inherited disease in which erythrocytes are sickle-shaped or crescent-shaped (normal erythrocytes are round, flat, concave in the middle) due to abnormal hemoglobin carrying oxygen in the red blood cells. Erythrocyte function is impaired and their lifespan is shortened.
Sickle cell anemia is life-threatening if the erythrocytes begin to break down en masse or the bone marrow is unable to produce enough new erythrocytes (for example, in infectious disease, when the bone marrow produces more cells to fight the virus/bacteria).
People who have inherited a defective hemoglobin gene from only one parent, ie are carriers of the defective gene, do not develop blood vessel blockages, resulting in tissue damage, and excessive erythrocyte breakdown. However, prolonged, strenuous physical exertion/exertion is dangerous for them, and sudden death can occur.
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Do you know the complications of sickle cell anemia?
SCD can cause severe side effects. Sickle cells can spread to different parts of the body and block blood vessels. Clogged arteries can be painful, which is called a sickle cell crisis.
Sickle cell crisis can occur in a variety of situations, including:
- temperature changes
In addition, sickle cell anemia can cause some other complications, including:
Anemia is caused by a lack of red blood cells, in which case the microbial cells are easily broken down and so-called hemolyzed. Normal red blood cells generally live for about 120 days, but sickle cells survive for a maximum of 10 to 20 days.
Hand and foot syndrome
Hand and foot syndrome occurs when sickle-shaped blood cells clog blood vessels in the hands or feet, causing swelling or sores in the hands and feet. Swollen hands and feet are often the first sign of sickle cell anemia in infants.
Spleen destruction _ sequestration
Sickle cells can block the arteries of the spleen, causing the spleen to separate and cause a sudden and painful enlargement of the spleen. The spleen may be removed from the patient due to complications of sickle cell disease in an operation known as splenectomy. In some patients with sickle cell anemia, the spleen shrinks and stops functioning. Patients without a spleen are at higher risk for bacterial infections such as Streptococcus, Haemophilus and Salmonella.
Delayed growth often occurs in people with SCD. Children with sickle cell anemia are usually short but are more likely to grow taller in adulthood. But in these children, puberty may be delayed because the sickle cells may not be able to provide enough oxygen and nutrients.
Seizures, strokes, or even coma can be side effects of sickle cell disease. They are caused by blockage of cerebral arteries.
Blindness due to blockage in the arteries supplying the eye is another side effect of sickle cell anemia that can damage the retina.
If small arteries in the legs become blocked due to sickle cell anemia, skin sores can form on the legs.
Heart disease and chest syndrome
Sickle cell anemia can lead to heart problems including heart attacks, heart failure, and abnormal heart rhythms.
Lung damage overtime associated with decreased blood flow can lead to high blood pressure in the lungs (pulmonary hypertension) and lung ulcers (pulmonary fibrosis). Lung damage makes it harder for the lungs to carry oxygen to the blood and can lead to more sickle cell crises.
Complications of pregnancy
Sickle cell anemia can increase the risk of high blood pressure and blood clots during pregnancy. It can also increase the risk of miscarriage, premature birth, and low birth weight babies.
Gallstones are not a complication of clogged arteries. Instead, they are caused by the destruction of red blood cells. Due to the destruction of red blood cells and the formation and excessive accumulation of bilirubin can lead to gallstones.
- Chest syndrome is a severe form of sickle cell crisis.
- The disease causes severe chest pain and is accompanied by symptoms such as cough, fever, sputum, shortness of breath and low blood oxygen levels.
The best foods for anemia
Now that you know the importance of iron and vitamin B12, it makes sense for patients with anemia to follow a diet rich in these nutrients. Unfortunately, one of the best foods for patients with anemia is also one of the most unsafe examples: Liver.
Beef or chicken liver has significant levels of iron and vitamin B12. In one study, many patients with anemia who added a few servings of liver and onions to their weekly diet reported significant benefits in improving mood and energy levels.
Other foods rich in iron and / or vitamin B12 include:
- Beef cattle fed with grass
- Mutton fed on grass
- Wrinkled oysters
- Double shell oysters
- Black oysters
Non-meat and seafood sources of iron or vitamin B12 include:
- Swiss beet
- Pumpkin seeds
While dark leafy vegetables are good for patients with anemia, their iron and vitamin B12 content is not comparable to their meat counterparts. Accordingly, people who follow a vegetarian or vegan diet have a higher risk of developing anemia.
What are the symptoms of sickle cell anemia?
Symptoms of sickle cell anemia usually appear at a young age. They may even occur in infants as early as 4 to 6 months of age. While there are different types of sickle cell anemia, they all have the same symptoms, with varying degrees of severity.
These symptoms include:
- Sleep disorder
- Recurrent infections
- Dizziness in infants
- Jaundice, especially yellowing of the eyes and skin
- Swelling and pain in the hands and feet (dactylitis)
- Pain in the chest, back, arms or legs
- Excessive fatigue or irritability due to anemia
- Aseptic necrosis and bone infarction (partial death of bone)
Many symptoms typically occur in certain age groups. Babies with sickle cell anemia have no symptoms in the first few months of life because the hemoglobin produced by the developing fetus (fetal hemoglobin) protects red blood cells from the disease. But symptoms may start at 4 months of age.
Signs and symptoms of this disease in infants and younger children are:
- stomach ache
- Enlargement of the spleen
- Pneumococcal bacterial infections
- Painful swelling of the hands and feet (dactylitis)
More common symptoms occur in adolescents and young adults:
- Foot ulcers
- Aseptic necrosis
- Eye injury
Symptoms in adults are usually due to damage to the bones, muscles, or internal organs of the body, with intermittent onset and sensation of pain.
What are the various types of sickle cell anemia
Hemoglobin is a protein found in red blood cells that carries oxygen. Hemoglobin normally has two alpha chains and two beta chains.
The four main types of sickle cell anemia based on gene mutations in hemoglobin chains are:
Hemoglobin SS disease
- Hemoglobin SS (Hb SS) is the most common type of sickle cell disease.
- Hb SS occurs when a patient inherits copies of the hemoglobin S gene from both parents. People with Hb SS experience the worst symptoms of the disease.
Hemoglobin SC disease
Hemoglobin SC disease is the second type of sickle cell disease and occurs when a patient inherits the Hb C gene from one parent and the Hb S gene from the other parents. Get the code. People with Hb SC have the same symptoms as people with Hb SS, but they have severe anemia.
Hemoglobin SB + beta thalassemia
If a person inherits the Hb S gene from their parents, they get the disease. Thalassemia hemoglobin SB + (beta) affects the production of the beta-globin gene and reduces the size of red blood cells. Hemoglobin SB + thalassemia does not cause severe symptoms.
Beta zero hemoglobin thalassemia (SB 0)
- Sickle cell thalassemia beta zero is the fourth most common type of sickle cell disease.
- Its symptoms are similar to Hb SS anemia, and sometimes the symptoms become more severe and the disease does not have a good prognosis.
SD hemoglobin, SE hemoglobin and SO hemoglobin
These types of sickle cell disease are very rare and usually do not have severe symptoms.
Sickle cell adjective
People who inherit only one mutated hemoglobin S gene from one parent are said to have sickle cell symptoms. Of course, it is possible that the symptoms will not occur
Home remedies to help fight against sickle cell anemia
There are cases that patients with this disorder can consider at home to help relieve their symptoms, including:
- Regular exercise to reduce stress
- Use heating pads to relieve pain
- Take folic acid supplements as recommended by your doctor
- Proper nutrition, especially enough fruits, vegetables, and whole grains
- Drink plenty of water to reduce the risk of sickle cell crisis
- Prompt and timely referral In case of infection, early treatment of the infection may prevent a full-blown crisis
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