What are the different types of sickle cell disease?

Sickle cell disease or sickle cell disorder is a term often used to talk about a group of diseases affecting hemoglobin. It is the oxygen-carrying element of blood that makes up 2 alpha globins and 2 beta globins. Sickle cell disease happens due to beta-globin mutation and depending on the abnormality in mutation.

Types-
Sickle cell diseases can be of many types depending on the gene mutation in the blood. Some of the common types are-

Sickle hemoglobin s disease (ss)
Hemoglobin ss disease or sickle cell anemia is the most popular type of scd. It takes place when hemoglobin s gene mutation is inherited from both mother and father. In simple words, it happens when the child inherits one substitution beta-globin gene from each parent. In this type, the body only produces hemoglobin s. It is mostly found in people of african and indian origin.

Sickle hemoglobin- c disease (sc):
Patients with sickle hemoglobin sc disease have a moderately dissimilar swapping in their genes of beta-globin. Here, both hemoglobin c and hemoglobin s are inherited from each parent. It may cause similar symptoms like anemia but remains relatively l. People of west african and mediterranean origin are more prone to this.

Hbs beta-thalassemia
People with this type of scd inherit one sickle cell gene s and one gene for beta-thalassemia from each parent. Zero and plus are the two common types of beta-thalassemia. While beta 0 thalassemia usually takes a severe form, beta + thalassemia is a milder form of the disease.

Sickle beta-zero thalassemia
Beta-zero thalassemia takes form when the child inherits the beta s gene from one parent and beta 0 thalassemia gene mutation from the other. With similar symptoms to anemia, it is a slightly severe form of beta-thalassemia.

Sickle beta-plus thalassemia
Substitutions in both beta-globin genes take place in patients with sickle beta thalassemia (sb) disease. This may even lead to the requirement of blood transfusion if no normal beta globin is produced. Mediterranean and caribbean people are more likely to have this disorder.

Hemoglobin sd, hemoglobin se, and hemoglobin so
These are the few rare and not less common types of sickle cell diseases. They are also less severe. People with these forms of scd inherit one sickle cell gene s and one gene from uncommon hemoglobin (d, e, or o) from each parent.

Sickle cell trait
Hbas
People with sct have one sickle cell gene s and one normal gene a from both mother and father. Ones with this disorder show few to no signs of the disease and usually spend a normal life. However, the risk to their future generations still exists.
 

What Are The Different Types Of Sickle Cell Disease?

People with sickle cell disease often experience myriad symptoms of the disease that can be controlled by the right nutrition. Reports suggest that nutrition is an increasingly significant factor to prevent symptoms such as slow growth, lower body mass, joint issues, infections, pain crises, and anemia.

There is a greater than the typical requirement for both calories and micronutrients.

The nutritional requirement of a sickle cell patient emphasizes more on vegetables, fruits, whole grains, and more similar food items. These can prevent any deficiencies in the body and also decrease any chances of disease exacerbation.

 


	
	High-calorie nutrient-dense diet
	


The usual energy intake in a sickle cell patient is normally lower than the average values. It is a case even when the condition is asymptomatic where the requirement drops to half the level. As a result, people with sickle cell diseases (especially kids) are at higher risk for impaired growth and considerably lesser fat and fat-free mass, with obesity also posing a risk. It is suggested to have adequate energy supplements in the diet to reach the minimum sufficient requirement of the body that can be done only by proper nutritional assessment.


	
	Adequate fluids
	


Avoiding dehydration is another significant factor to avoid any severe symptoms of sickle cell disease. Sickling of the erythrocytes aggravates in patients who perform hardcore physical work in the heat without drinking enough fluids. This leads to dehydration and can even result in constipation.


	
	Micronutrients
	


Micronutrient deficiencies can be found in sickle cell patients up to a great extent. Several vitamins and minerals like vitamin a, b6, c, e, carotenoids, zinc, and magnesium are often found in fewer numbers in scd patients. A significant drop in blood-antioxidant status may result in acute chest syndrome. The supplementation of these vitamins and minerals can reduce the number of sickled cells in the body. Plant phenols and flavonoids may also help in lowering oxidative stress.


	
	Calcium-rich food
	


Getting plenty of calcium-rich food like dairy products, leafy vegetables, cereals, and fruit juices aids the child's growth and development. This may lead to a stronger physical structure and a greater resistance to the disease.


	
	Omega-3 fatty acid
	


There are reduced proportions of omega-3 polyunsaturated fatty acids in sickle cell patients. These acids aid the fluidity of red blood cell membranes. As a result, they prevent sickle cell crises. Supplemental docosahexaenoic (dha) and eicosapentaenoic (epa) have shown numerous therapeutic benefits in scd patients.


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Role Of Nutrition In Sickle Cell Disease

Atherosclerosis refers to a medical condition in which the arteries become hardened and narrowed due to the accumulation of cholesterol plaque in the walls of the artery, obstructing the flow of blood.

What are the causes?

Atherosclerosis is a slow but progressive disorder that starts with the damage of endothelium   cells lining the interior surface of lymphatic and blood vessels. This allows bad ldl cholesterol to build up in the artery walls. Over time, a plaque made of calcium, macrophages, and cholesterol grows to a certain size to clog up arteries, disrupting blood flow around the body.

Other factors leading to atherosclerosis to include  

High blood pressure

High levels of triglyceride in the blood

High cholesterol levels

Certain medical conditions   diabetes, obesity, insulin resistance

Smoking/use of tobacco

Inflammatory conditions like lupus and arthritis

Symptoms of atherosclerosis

Atherosclerosis may develop in childhood or during adolescence. Mild conditions may not reveal observable symptoms unless the arteries are clogged, and blood supply to the tissues and organs are restricted.

Signs of moderate to severe atherosclerosis depend on the affected arteries   it could be the arteries leading to your brain, your heart, legs and arms, or the kidney. The symptoms of atherosclerosis include  

Weakness and fatigue

Difficulty breathing/concentrating

Headaches

Facial numbness

Paralysis

Vomiting

Chest pain

Anxiety

Loss of appetite

Swelling in the feet and hands

If any of the above symptoms seem familiar to you, consult a doctor right away for treatment.

Can atherosclerosis be prevented?

The best way to avoid cholesterol plaque from building up in the artery walls is to prevent the onset of atherosclerosis with these preventive measures.

Eat healthy   avoid eating foods that are rich in saturated fats   red meat, butter and cheese. These increase your cholesterol levels drastically. Instead, include foods that contain unsaturated fats, like avocados, olive oil, fatty fish, nuts and seeds because these help to keep ldl or bad cholesterol levels down.

Stay active   exercising and staying active can help regulate your cholesterol levels and prevent certain conditions that might put you at risk for atherosclerosis.

Quit smoking   people who smoke should quit smoking because it is a major risk factor for atherosclerosis   it increases blood pressure, which in turn, affects your cholesterol levels.

Atherosclerosis can lead to complications. With precautionary measures and proper treatment, the condition can be avoided.

Know All About Atherosclerosis Here

The world celebrates world blood donor day on the 14th of june to raise awareness of the need for safe blood and blood products and to thank voluntary and unpaid blood donors for their life-saving gift of blood. 

Importance of blood donation

The importance of donating blood is not only to save the lives of thousands of people who are deprived of life but also to save the lives of many more who are affected by the various diseases. ;

 

It has also been seen that when people; e have donated their blood, they have gained many health benefits as it helps in weight loss, helps maintain healthy liver and iron levels, it helps reduce the risk of heart attack and cancer. ;

 

A blood donation can save a life of- ;


	
	Victims of natural disasters, including earthquakes
	
	
	Patients with hemophilia, sickle cell disease, or cancer
	
	
	People who suffer traumatic injuries from being in a car accident
	
	
	Surgery patients
	
	
	Shooting or stabbing victims
	
	
	Women who suffer complications during childbirth
	


 

Blood types that are needed most

It s good to donate if you re healthy. However, there s always a need for specific blood and plasma which are universal and can be used when a patient blood type is unknown. For newborn infants or if a patient's particular blood type is unavailable.

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	Type o negative blood is always needed because it can be used in patients regardless of their blood type.
	
	
	Type ab positive plasma can also be transfused to patients with other blood types. 
	


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Donations are constantly needed

Because of their short shelf life of 5 days after being collected, platelet donations are constantly by the blood blank. Red blood cells also have a short shelf life and needed to be used within 42 days after the collection. Cryoprecipitate and plasma can be frozen and stored for up to a year after donation.


 

Some misconceptions about blood donations


	
	The donor will not experience any kind of pain while donating. ;
	
	
	There will be no scarcity of blood in the body after donating the blood as even after the donation the body will have a surplus quantity of blood. 
	
	
	The common misconception everyone has is that they will faint after donating their blood. ;
	
	
	You will be able to resume your everyday activity immediately after donating the blood. 
	
	
	You will not get infected with aids if you donate your blood. ;
	


 

Donating blood is a great way to give back to your community and potentially save a life by only giving up an hour of your time.

World Blood Donor Day: How Can We Save Life By Donating Blood?

All types of viral hepatitis diseases are contagious and they can be transmitted from one person to another easily. Although, the modes of transmission may vary.

 

Experts recommend some preventive steps for every type of infection to lower your risk of acquiring or spreading hepatitis infection.

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Here are some preventive measures that may help in preventing the infection:

 

Vaccines

Vaccines for hepatitis a and hepatitis b are the best ways to prevent these viruses. Vaccines are not for all types of hepatitis infections. However, hepatitis a vaccination can help prevent the infection caused by the hepatitis a virus. The hepatitis a vaccine is generally administered in 2 doses. Similarly, there are vaccines available for hepatitis b as well. It is administered within 6 months duration. The same vaccine is helpful to prevent hepatitis d as well. There are no vaccines for hepatitis c and hepatitis e currently.

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Reducing exposure

Hepatitis diseases can be transmitted through bodily fluids, water, and food containing the infection. One can lower the risk of contamination by minimizing contact with these agents. Practicing proper and good hygiene is a way to avoid getting hepatitis through these mediums.

 

You can decrease your risk of coming in touch with fluids containing these viruses by avoiding:

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	Sharing used needles and razors
	
	
	Using someone else s toothbrush
	
	
	Touching spilled blood
	
	
	Not using condoms and dental dams during sexual contact
	


 

Handwashing

One must trust in personal hygiene to lower the risk of spreading or acquiring the hepatitis virus. Here are some preventive steps you can follow by washing hands:

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	Washing your hands thoroughly after accessing the restroom
	
	
	When you get in touch with an infected person's blood, or stool
	
	
	After daycare routine for caretakers
	
	
	After a diaper change, and before serving food.
	


 

Avoiding contaminated consumption:

Contaminated or infected food and water may become the primary causes of hepatitis infection. One should take these precautions to avoid any contaminated consumption of food or water:

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	Avoid eating raw shellfish
	
	
	Beware of fruits that have been washed in contaminated water
	
	
	Avoiding unhygienic street vendors
	
	
	Use bottled water for brushing or oral cleaning in areas where clean water is not available
	
	
	Bringing the water to a 1-minute boil is a must in doubtful areas
	
	
	Heated food to be eaten right away
	


Hepatitis: Some Ways To Prevent Yourself

World sickle cell day is observed annually on the 19th of june to raise awareness of sickle cell-related diseases. It is observed to increase people's knowledge and the understanding required to face the challenges posed by the disease.


 

What is sickle cell disorder (scd)?

Sickle cell disorders or sickle cell diseases are genetic blood disorders that cause an abnormality in the red blood cells. The rbcs turn into sickle-shaped cells that stick together and block the blood flow in the body.


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Types

There are four major classifications of sickle cell diseases-


	
	Hemoglobin ss disease
	
	
	Hemoglobin sc disease
	
	
	Hemoglobin sb+ thalassemia
	
	
	Hemoglobin sb 0 thalassemia
	



 

Symptoms

One can start noticing symptoms of sickle cell in a patient at around 6 years of age. However, they can differ from person to person. Some of the most typical symptoms are-

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	Anemia
	


A shortage of red blood cells occurs due to the excessive early dying in 10-20 days. It leads to an oxygen shortage in the body.


	
	Excessive pain
	


Blockage of blood flow due to the change in shape leads to periodic cycles of pain in the body.


	
	Swelling in the body
	


As the blood circulation becomes irregular, swelling becomes a regular phenomenon.


	
	Infections
	


Sickle cell can be damaging to the spleen. It increases the risk of ailments in the body that can be prevented only by antibiotics.


	
	Delayed puberty
	


Oxygen and nutrients carried by the blood are required for growth. As a reason, irregular blood flow can lead to slow growth or delay in puberty.


	
	Vision issues
	


Infected blood vessels carrying blood to the eyes can cause damage to the retina. It can lead to visual problems.


 

Traits and treatment

As sickle cell is genetic, it is suggested to take a test that can detect any trait of the disease. It can prevent any life-threatening risks to the newborn. However, we don't have a complete cure for sickle cell disorders. There are preventive measures that reduce the adversity of the disease to an extent. Procedures like blood transfusions help greatly in minimizing the effects of the disorder.

World Sickle Cell Day: Know All About Sickle Cell Disease

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