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

Urinary tract infections (uti) can occur anywhere in the urinary system   the bladder, kidneys, urethra, and ureter. Most of these infections affect the lower part of the urinary system   urethra and the bladder.

An infection ;confined to the bladder can be frustrating and painful. You may experience pain during urination, and pus or blood in the urine. However, the condition becomes worse if the infection spreads to the upper part of the urinary system, particularly to the kidneys, triggering nausea, ;vomiting, and severe pain in the back.

If uti is left untreated, it can potentially lead to kidney damage   a condition known as pyelonephritis.

How does untreated uti affect the kidneys?

Untreated urinary tract infections can cause the kidneys to swell up and damage them permanently. The infections initially begin in the lower tract of your urinary system. Then the bacteria slowly enter your body through the urethra and begin multiplying and spreading up to the bladder. The bacteria eventually travel to your kidneys through the ureters.

Some people are at higher risk for kidney damage due to urinary tract infections. This includes  


	
	Pregnant women
	
	
	Elderly people
	
	
	People with kidney problems
	
	
	Someone with a weak immune system
	


Signs to watch out for

An untreated infection in the bladder that has spread to the kidneys is associated with symptoms, such as the following  


	
	Chills and ;fever
	
	
	Vomiting and nausea
	
	
	Pain in either side of your lower back
	


You should visit a doctor right away if you notice the above uti symptoms. The condition requires immediate medical intervention in order to prevent damage to your kidneys.

Symptoms of painful utis can be managed by combining medications and lifestyle remedies. Consult a specialist to diagnose the condition early and seek appropriate treatment to avoid complications.

Untreated Urinary Tract Infections And Kidney Damage: Know The Connection

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?

Headaches are extremely common   we all are familiar with mild or persistent throbbing ;pain. A number of conditions can result in headaches. Some of these include  


	
	Fatigue
	
	
	Lack of sleep
	
	
	Stress/tension
	
	
	An underlying condition, like migraine ;or sinusitis
	


Recent studies have revealed a lesser-known cause for headaches   ;hypertension, or what is commonly referred to as high blood pressure.

The connection between hypertension and headaches

Hypertension occurs when the force of the blood circulation in the arteries is elevated   this particular condition can lead to steady pain throughout the head, particularly at the back of the head or in forehead.

Hypertension-induced headaches usually affect both sides of the head, particularly the temple regions. However, a person may not even realize that the headaches he/she is experiencing are induced by high blood pressure.

Since hypertension is an asymptotic condition, it is often referred to as  the silent killer . When blood pressure rises above 180/110, the condition is known as  hypertensive emergency .

Different types of hypertension leading to headaches

There are three types of hypertension conditions that are followed by mild to severe headaches:


	
	Malignant hypertension- this refers to a sudden, rapid increase in blood pressure that calls for medical attention. This is more common among young adults and women with pregnancy-induced hypertension.
	
	
	Idiopathic ;intracranial hypertension- it is characterized by elevated blood pressure around the brain. Symptoms include vision problems, nausea, ;shoulder pain, thumping noise in the ears, and severe headaches.
	
	
	Hypertensive encephalopathy- this is an extreme condition of high blood pressure, which leads to swelling in the brain. A person with this condition experiences severe headache that becomes worse.
	


Uncontrolled hypertension can be dangerous   headaches induced by such a condition could also become frequent or worsen over time. However, with proper medications and certain lifestyle changes, high blood pressure can be kept under the normal measure. Once your blood pressure normalizes, the headaches will disappear in no time.

How Are Hypertension And Headaches Related?

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?

People suffering from sickle cell disease (scd) usually start showing symptoms very early. They can appear to show up even during the first year of life. Symptoms of a sickle cell vary from one person to another and can be mild to serious depending on the condition.

 

Sickle cell disease is a health condition that worsens with time. Despite the absence of any full-proof solution, some treatments prevent further complications. These treatments can help patients receive aid for a lifetime. Simply said, sickle cell diseases can be managed medically.

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Treatment

Treatment in a sickle cell patient depends on the severity of the symptoms. Preventing complications and relieving pain is the primary aim of the diagnosis. They can range from medications to blood transfusions.

 

Concerns

Some of the concerns that need to be taken care of during the treatment of scd are-

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	Prevent painful scenarios
	


A painful episode or sickle cell crisis can be avoided by consuming sufficient fluids, wearing warm clothing, and staying away from sudden temperature changes.

 


	
	Avoid infections
	


Children with sickle cell anemia are made safe from infections by using penicillin. Adults with anemia might need to take penicillin all their lives. Doctors should ensure that all vaccinations in children and adults are periodically done to avoid any infections. However, during the covid-19 pandemic sickle cell patients should take extra care.

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Medications

While there can be numerous ways to manage sickle cell treatment, medications help in a significant way. Some of the prescribed medicines are-


	
	Hydroxyurea- it reduces the frequency of sickle crises. Pregnant women should stay away from this according to experts.
	
	
	Crizanlizumab- this drug injected into the patient can help reduce the sickle crises in adults and children over the age of 16.
	
	
	Voxelotor- it is an oral drug used in children over 12. It lowers the risk of anemia by improving blood flow.
	
	
	Pain-relievers- narcotics to prevent excessive pain can also be used during the treatment.
	


 

Surgical procedures


	
	Blood transfusion
	


It is used to prevent stroke and other related complications in patients. This can increase the number of red blood cells in the body that can help reduce the symptoms.

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	Stem cell transplant
	


It is also called a bone marrow transplant and is used to replace affected bone marrow with a healthy one. This procedure is carried out between matched donor and recipient. Although it is a known cure for sickle cell disease, various risks involved make it a less used practice.

 

Can Sickle Cell Be Cured?

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