Abstract

Background: it is essential to have an understanding of life expectancy and risk factors among patients with sickle cell disease (sickle cell anemia, hemoglobin diseases, and thalassemias). These are required to counsel the patients, target treatment, and plan the right clinical trials.

 

Methods: a research followed 3764 people ranging from birth to 66 years of age and suffering from scd. The procedure was done to understand the life expectancy and calculate the average age at death. Moreover, the study understood the reasons for the death in all 209 adults. It used the proportional-hazards regression analysis to know the risks for an untimely death in adults with sickle cell anemia.

 ;

Results: when the results for young kids and adults with sickle cell anemia were observed, it was found that the average age at death was 48 for women and 42 for men. Moreover, suffering from hemoglobin c disease had an average mortality age of 60 for men and 68 years for women.

 

In people with sickle cell disease, 18% of deaths took place in patients with mainly renal organ failures. 33% of the tested patients were medically free of organ failures but episodes of pain crisis took their life. Out of these patients, 78% experienced chronic pain, chest syndrome, or both, while 22% suffered a stroke that took their lives.

 ;

Clinical modeling showed that people with sickle cell anemia faced acute chest syndrome, seizures, kidney failure, a white-cell count of over 15, 000 per cubic mm, and a low hemoglobin level were related to a risk of early death.

 

Conclusions: results suggested that 50% of patients with sickle cell anemia lived beyond the age of 50. A huge share of those who lost their lives suffered no noticeable chronic organ failure. However, most of them died due to acute episodes of pain or stroke. Early death was seen highest among patients who had symptomatic disease. High levels of fetal hemoglobin suggested improved chances of survival and are assumably a credible source to foresee the risks and other factors related to adult life expectancy.


 ;

Life Expectancy And Risk Factors Involved In Sickle Cell Disease

Kidney - prolonged periods of stress can indirectly hurt your kidneys. So you need to be stress free to keep your kidneys healthy.

Stress And Kidney Failure: Are They Connected?

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?

Blood donation is a process that is carried out when a person voluntarily offers for blood to be extracted from his body. There are countless myths about blood donation that need to be squashed and awareness is the only way of doing it.

While blood donation brings multiple health benefits to both the donor and recipient, some facts are known to only a few. These facts will not only open doors to endless possibilities, but will make people feel confident about it.

Facts

Here are some lesser-known facts about blood donation- every person should know-

 


	
	Approximately 112.5 million units of blood are collected from blood donation globally.
	
	
	Around 30 million units are transfused annually.
	
	
	Any person in sound health, at least 18 years old, and weighing at least 45 kilograms can donate blood every 56 days.
	
	
	Your one unit of donated blood can save up to 3 lives.
	
	
	A, b, ab, and o are the primary blood groups. While ab is a universal recipient, o negative is a universal donor.
	
	
	The blood donation process normally takes less than 10-12 minutes.
	
	
	Donating blood does not make you physically weak or decrease your strength.
	
	
	Only 1% of india's population donates blood each year, leading to an annual deficit of two million units.
	
	
	All donated blood units are tested for diseases like hepatitis b, hepatitis c, hiv, and syphilis before transfusion.
	
	
	Red cells, plasma, cryoprecipitate, and platelets are the various types of transfusable elements derived from the blood. The procedure takes place using a method called apheresis.
	
	
	Red blood cells carry oxygen to the organs.
	
	
	Red blood cells survive for about four months in the system of circulation.
	
	
	Platelets encourage blood clotting and provide leukemia patients and other cancer patients with better treatment.
	
	
	Plasma is a pale yellow combination of proteins, salts, and water.
	
	
	Plasma is 55 percent of the volume of blood and 90 percent water.
	
	
	White cells are the chief defense of the body against infections.
	
	
	There is no alternative for blood donation as blood cannot be manufactured.
	
	
	You cannot contract aids or other infectious diseases due to blood donation.
	


Lesser Known Facts About Blood Donation

C-reactive protein ;is a type of protein produced by the liver in response to an infection ;or inflammation. When your body contracts an infection, the white blood cells produce proteins to fight it. Some of these proteins activate the liver into producing c-reactive protein. For many years, the crp ;blood level has been used to ascertain the level of infection or inflammation.

Assessing the risk of heart disease ;with c-reactive protein

The level of crp is a potential indicator of how likely you are to develop cardiovascular diseases. This is because the development of atherosclerosis is linked with inflammation within the walls of your blood vessel. This results in higher levels of c-reactive protein in atherosclerosis patients than in those without the disorder.

The greater the crp levels, the more chances you have of being affected by cardiovascular disorders. The test also determines the risk of a second attack.

The value of knowing your crp levels depends on whether you come under one of three cardiovascular risk groups.


	
	High risk   not important to know crp because you should be combining lifestyle changes and aggressive treatment to reduce risk
	
	
	Intermediate risk   helpful to find out crp levels, as it can provide vital information crucial in deciding the next line of treatment
	
	
	Low risk   even if your crp levels are high, you will not be at risk   due to negligible risk factors and low cholesterol levels   that should require more than just lifestyle changes
	


To find out crp levels you will have to take a blood test. C-reactive protein is classified into  


	
	High   equal to or greater than 3
	
	
	Intermediate   between 1 and 3
	
	
	Low   below 1
	


If you are having a c-reactive protein test to check for heart diseases, your healthcare provider may recommend a cholesterol test as well. A number of other tests may also be prescribed to evaluate your risk further.

How C-reactive Protein Can Pose A Risk For Heart Disease?

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.


 ;

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-

 ;


	
	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

Don't wait any longer, manage your Case Control Study online with a Lybrate doctor today

Consult for Case Control Study - Online and Easy!

FilterClear All

COMMON SPECIALITIES

COMMON ISSUES