World Sickle Cell Day is observed each year on June 19th to raise the public awareness regarding this disease and its treatment methods. Sickle Cell disease is an inherited transmitted deformity/abnormality of haemoglobin. It is an inherited form of anaemia. This abnormality generates changes in the shape of red blood cells from round to crescent, which reduces the oxygen carrying capacity and slows down the flow of blood to various parts of the body. 

Generally, red blood vessels are round and flexible, passing easily through the blood vessels. The red blood cells become sticky and rigid in sickle cell anaemia. Sickle cells last 10 to 20 days, which causes anaemia because of the disorder of red blood cells, while healthy red blood cells could live up to 120 days.

Millions of people around the world are affected by Sickle Cell Anemia. There is no specific cure for this disease as Sickle cell anaemia is not contagious, it is hereditary. Sickle cell anaemia is named so for the reason that red blood cells are crescent-shaped or sickle-shaped, and anaemia signifies lack of blood. There is a difference between iron-deficiency anaemia and sickle cell anaemia.

Symptoms

Sickle Cell Disease (SCD) has many symptoms which includes: serious anemia, episodes of suffering, additionally a range of complications, heart failure, dizziness, chronic kidney disease (CKD), acute chest syndrome (ACS), chest pain, gallstones, pulmonary hypertension, immune deficiency, paralysis or stroke, splenomegaly, joint damage mainly in the hip joint, foot ulcers, delayed puberty, retinopathy, and endocrine disorders.

Diagnosis

Sickle Cell Disease is diagnosed by blood tests.

Treatment

Bone marrow transplant or stem cell transplant in patients usually below the age of sixteen is the only available cure for the disease.

Aim of the World Sickle Cell Day

The aim of the World Sickle Cell Day is to support and promote research for the patients in order to help them upgrade their overall quality of life to the maximum possible extent. It also aspires to increase awareness for sickle cell disease disorder in the general public so that they know how to deal with patients suffering from the disease. Moreover, the World Sickle Cell Day has the objective of raising awareness of the importance among generations of pre-marital screening to lessen the transmission of Sickle Cell Disease.

Target Groups for Awareness Programs

Though it is desirable that everybody knows about Sickle Cell Disease, the target groups for the awareness programs include health decision-makers, sickle cell anaemia sufferers and their families, health organizations and associations, people who are planning to get married, and the health workers including health educators, physicians, and nurses.

On the World Sickle Cell Day, different societies provide advocacy, information, and advice for raising awareness. They also provide educational and welfare grants, support services, annual children s holiday, home help, hospital visits, mentoring and befriending through holding talks, assisting into research and training, lobbying for better service provision, patient education seminars, and campaigning.

World Sickle Cell Day - What Should You Know?

Iron is an essential nutrient and mineral that is required by adults and children alike. Iron helps move oxygen from the lungs to the rest of the body and helps muscles store and use oxygen. It is especially important for children because it aids development and prevents anaemia. Untreated iron deficiency in children can cause physical and mental delays. It can lead to less healthy red blood cells in the child's blood stream which will cause a delay in the growth of physical and mental faculties.

Risk factors for iron deficiency in children

Infants and children at highest risk of iron deficiency include:

Babies who are born prematurely or have a low birth weight
Babies who drink cow's milk before age 1
Breast-fed babies who aren't given complementary foods containing iron after age 6 months
Babies who drink formula that isn't fortified with iron
Children ages 1 to 5 who drink more than 24 ounces (710 milliliters) of cow's milk, goat's milk or soy milk a day
Children who have certain health conditions, such as chronic infections or restricted diets
Children ages 1 to 5 who have been exposed to lead
Adolescent girls also are at higher risk of iron deficiency because their bodies lose iron during menstruation.
Symptoms of iron deficiency anaemia

The signs and symptoms of iron deficiency anaemia in children may include:

Pale skin
Fatigue or weakness
Slow cognitive and social development
Inflammation of the tongue
Difficulty maintaining body temperature
Increased likelihood of infections
Unusual cravings for non-nutritive substances, such as ice, dirt or pure starch
Prevent iron deficiency in children

Take steps to prevent iron deficiency in your child by paying attention to his or her diet. For example:

Breast-feed or use iron-fortified formula. Breast-feeding until your child is age 1 is recommended. If you don't breast-feed, use iron-fortified infant formula.
Encourage a balanced diet. When you begin serving your baby solids, typically between ages 4 months and 6 months, feed him or her foods with added iron, such as iron-fortified baby cereal. For older children, good sources of iron include red meat, chicken, fish, beans and dark green leafy vegetables. Between ages 1 and 5, don't allow your child to drink more than 24 ounces (710 milliliters) of milk a day.
Enhance absorption. Vitamin C helps promote the absorption of dietary iron. You can help your child absorb iron by offering foods rich in vitamin C, such as melon, strawberries, kiwi, broccoli, tomatoes and potatoes.
Consider iron supplements. If your baby was born prematurely or with a low birth weight or you're breast-feeding a baby older than 4 months and he or she isn't eating two or more servings a day of iron-rich foods, talk to your child's doctor about oral iron supplements.
Make sure that you watch out for the tell tale signs of iron deficiency and take the necessary precautions to avoid the same. If you wish to discuss about any specific child related problem, you can consult a specilized pediatrician and ask a free question.

Iron Deficiency In Children

Did you know that Thalassemia is a common blood disorder in India, that?s passed through families (just the way it was passed to Abhishek Bachchan because megastar Amitabh Bachchan is a carrier). It is characterized by excessive destruction of red blood cells or genetic mutation, leading to anaemia. Today is World Thalassemia Day, lets understand about the disease.

Thalassemia can be classified into alpha and beta, depending on the globin genes that are affected.

1. Thalassemia Minor - Usually doesn?t have any symptoms, except for minor anaemia.

2. Beta Thalassemia - Has two serious types.

2.A) Thalassemia Major: This type usually happens before a child?s second birthday and requires regular blood transfusions. The condition is life-threatening. Symptoms include anaemia, fussiness, frequent infections, paleness of the skin, poor appetite, enlarged organs and jaundice.

2.B) Thalassemia Intermedia: It is a less severe form and doesn?t require blood transfusions. The symptoms include anemia, jaundice, extremely large spleen and malnourishment.

3. Alpha Thalassemia: Only in rare cases this severe form of Thalassemia may be present. Known as Hydrops Fetalis, this condition usually occurs before birth and children are either stillborn or die soon afterwards.

How to live with Thalassemia:

Take steps to be as healthy as possible. Follow a balanced diet eating plan as per the recommendation of your doctor. Take iron supplement only if prescribed. It is recommended that patients get themselves vaccinated whenever needed, especially if their spleen has been removed. Meanwhile, always be on a lookout for the signs of infections (such as fever) and lower your risk of infections. Thalassemia can spark fear, anxiety, depression and stress. That?s why, doctors recommend that patients receive adequate emotional support.

Treatment:

The treatments depend on the kind of Thalassemia an individual has. Usually, treatment procedures include blood transfusions, bone marrow transplants, removal of spleen or gallbladder specific medications and supplements.

If you have any more questions on this topic, click on consult privately to ask me.

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Read Dr. Jatin Soni's Health Tip on The Occasion of World Thalassemia Day

What do we mean by the term Anemia?

It is described as a decrease in the total number of red blood cells, or hemoglobin in the blood, or a decreased oxygen-carrying capacity of blood.

How to know if an individual has Anemia?

An individual with anemia may show one or more of the following signs or symptoms:
Shortness of breath, chest pain, and headache are especially seen with exercise as the oxygen supply decreases
Difficulty in concentration which is mostly seen in children
Easy tiredness, fatigue, and loss of energy
Dizziness because of low blood volume leading to hypotension
Hair loss especially while combing, washing hair. Also, hair becomes brittle and dry due to anemiaInsomnia (lack of sleep)
Cramps in legs especially calf muscle 
Rapid heartbeat with exercise as the demand for oxygen increases during exercise, so your heart beats faster than usual
Craving for indigestible substances, such as pica, paper, mud, ice, dirt, etc.
Koilonychia (spoon-shaped nails or upward curvature of the nails)
Soreness and cracks at the corners of the mouth
Very heavy menstrual periods or irregular bleeding because of low hemoglobin count
Pale skin is especially seen over the palms of your hands and under your nail beds. Also, the conjunctiva looks pale
Anemia due to Deficiency of Vitamin B12 Dementia (loss of memory)
Tingling sensation in the hands or feet
Loss of the sense of touch
Difficulty in walking
Stiffness in the arms and legs
Delayed growth and development in children
Swelling of the hands and feet
Episodes of severe pain in the joints, abdomen, and limbs as the red blood cells obstruct capillaries
Anemia by Chronic Red Blood Cell Destruction/Hemolytic anemia

Jaundice (yellowish or greenish pigmentation of skin and sclera of the eyes)
Brown or red colored urine due to the destruction of red blood cells
Fever due to the destruction of red blood cells
Small bruises under the skin
Abdominal pain because of enlargement of the spleen and liver
Black stools due to the destruction of the red blood cells
Anemia Caused by Chronic Lead Poisoning
Level of lead in blood is higher than 50  g/dLA blue-black line is seen on the gums. It is also known as a lead line
Abdominal pain
Constipation
Vomiting
Investigations 

Complete blood count: This test will help an individual know the percentage of hemoglobin in blood as well as red blood cell count. Also, mean cell volume (MCV) is used to categorize anemia.
Serum Ferritin Test: Ferritin levels below 15 20 micrograms/L in a person confirms iron-deficiency anemia.
Total Iron-Binding Capacity: It measures the blood's capacity to bind iron with transferrin.

How Can Anaemia Be Diagnosed?

Thalassemia is a genetic (which is passed from parents to children through genes) blood disorder. People with Thalassemia disease are not able to make enough hemoglobin, which causes severe anemia. Hemoglobin is found in red blood cells and carries oxygen to all parts of the body. When there is not enough hemoglobin in the red blood cells, oxygen cannot get to all parts of the body. Organs then become starved for oxygen and are unable to function properly.

Symptoms

The symptoms depend on the type of thalassemia:

Thalassemia Minor: Thalassemia minor usually doesn t cause any symptoms. If it does, it causes minor anemia.
Beta-thalassemia: Beta-thalassemia comes in two serious types, which are thalassemia major, or Cooley s anemia, and thalassemia intermedia. The symptoms of thalassemia major generally appear before a child s second birthday. The severe anemia related to this condition can be life-threatening. Other signs and symptoms include:
fussiness
paleness
frequent infections
a poor appetite
failure to thrive
jaundice, which is a yellowing of the skin or the whites of the eyes
enlarged organs
This form of thalassemia is usually so severe that it requires regular blood transfusions.

Alpha-thalassemia: Alpha-thalassemia also has two serious types, which are hemoglobin H disease and hydrops fetalis. Hemoglobin H disease can cause bone issues. The cheeks, forehead, and jaw may all overgrow. Hemoglobin H disease can cause:
jaundice, which is a yellowing of the skin or the whites of the eyes
an extremely enlarged spleen
malnourishment
Hydrops fetalis is an extremely severe form of thalassemia. It occurs before birth. Most individuals with this condition are either stillborn or die shortly after being born.

Thalassemia - Symptoms Based on Types of Thalassemia 

Anemia is a condition where the red blood cell count is reduced due to deficiencies within the system. This is usually caused by a lack of iron, folate and other related nutrients in the diet. Anemia is a major public health concern in India as over 50 percent of women are known to be anemic. This pushes up the mortality rate of young mothers and even children.

Some symptoms of Iron deficiency anemia
Anemia has a wide range of symptoms which varies from person to person. However there are some generic symptoms which can be noticed in most people. Some of these are
1. Pale appearance of skin - Many people exhibit this symptom where their skin appears to be pale as there is a reduced amount of oxy - hemoglobin in the skin.
2. Physical weakness, light headedness and fatigue - This is a general symptom, which many tend to suffer from and complain about. Fatigue and weakness may come in very quickly even with the slightest effort.
3. Hair loss - Many women may complain about hair loss due to Iron deficiency  anemia.

Some other symptoms are -
a. Breathlessness
b. Constipation
c. Missed or irregular periods
d. Numbness or burning sensations
e. Depression
f. Tinnitus among others

Causes of Iron deficiency anemia
The primary cause of Iron deficiency anemia is the lack of iron in the diet which causes the hemoglobin in the blood to be reduced. Some of the patients may still have Iron deficiencyanemia as they have developed gastrointestinal disorders which prevents the absorption of nutrients like iron within the body.

How to treat Iron deficiency anemia
Diet related anemia can be easily cured or controlled with changing diets. Inclusion of certain foods within the diet can easily reverse the Iron deficiencies. Some of the food items that help with deficiencies are:
1. Fortified cereals
2. Green dark leafy vegetables
3. Poultry items
4. Eggs
5. Meat
You may also require certain iron supplements if you have certain underlying conditions which is causing issues with iron absorption.

How to prevent Iron deficiency anemia
It is important, especially for women to go for regular checkups to your doctor and also a hemoglobin check up. Your doctor or dietician can tell you to include certain foods in your diet that include folate, iron and vitamin B 12 so that the level of hemoglobin in your blood can be maintained at healthy levels.

All About Iron Deficiency - Anemia

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