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

The moment you are diagnosed with a tumour, the first thing your doctor will do is find out whether the tumour is benign or malignant   this will determine the course of your treatment. In simple words, benign means non-cancerous while malignant means cancerous.

Benign cancer

When tumour cells grow at a normal pace, it is known as benign or non-cancerous growth. In the case of benign cancer, the cells grow slowly and do not spread to other parts of the body or invade the adjacent tissues. Most often, these do not exhibit serious symptoms or pose a threat to your health. So, they are not a cause of concern. However, sometimes a benign tumour can press against nerves, tissues or the blood vessels, causing pain and discomfort.

Some common types of benign cancer are  


	
	Adenomas
	
	
	Fibromas or fibroids
	
	
	Hemangiomas
	
	
	Lipomas
	


Benign tumours, although tend to be harmless, may need to be operated. This is true for tumours that have grown to become large and weigh heavy. When these develop in the brain and cluster around to press against block channels or vital organs, the condition can be potentially dangerous. Moreover, certain types of non-cancerous tumours like intestinal polyps are precancerous   meaning they have the potential to turn cancerous. ;Surgery to remove this type of tumour can help prevent it from becoming malignant.

Malignant cancer

Malignant cancer refers to cancerous tumours that can potentially be fatal. Unlike benign cancer, malignant cancer progresses quickly and metastasizes to invade other organs, tissues or parts of the body.

Here are a few examples of tumours that are cancerous in nature  


	
	Carcinoma
	
	
	Sarcoma
	
	
	Germ cell tumour
	
	
	Blastoma
	


A tumour that has turned malignant, or has spread through the lymphatic system or bloodstream, may require aggressive treatment. Based on the stage of cancer, your ;oncologist will devise a treatment plan that might include immunotherapy, ;radiation therapy, chemotherapy ;or surgery.

Being diagnosed with a tumour can be hard to deal with. To find out if the tumour is benign or malignant, consult a specialist and discuss your concerns.

Benign Cancer Vs Malignant Cancer: Know The Difference!

When abnormal cells grow inside the brain and are in the form of a mass the condition is called a brain tumour. There are various types of brain tumours that exist. Some of the brain tumours which exist are malignant or cancerous while other may be non-cancerous. A brain tumour can begin in any part of the brain and spread to other parts of the body or vice-versa. The growth rate of brain tumours is different for each individual suffering from it. As per studies, every 2 in 1, 00, 000 people are said to be affected by a brain tumour in india. However, it may come as a relief to some as not all brain tumours are cancerous or life-threatening. Some are benign tumours and seldom pose any threat to the affected individual.

 

Types of brain tumor

Brain tumours may be primary or secondary.

Primary brain tumour

In this type of brain tumour, the abnormal mass of cells that originated in the brain does not metastasize to the other organs. Based on their location in the brain, the primary brain tumours may be of the following types.


	
	Gliomas: one of the most common types of primary brain tumours, gliomas can have their origin in the spinal cord or the brain. Some of the common types of gliomas include astrocytomas (originates in the cerebrum of the brain), glioblastomas (these tumours form from the astrocytes which form the supportive tissues of the brain), ependymoma (these develop from the ependymal cells which are present within the cns), oligodendrogliomas (these tumours are found to arise from the oligodendrocytes).
	
	
	Schwannomas: also called acoustic neuromas, these primary brain tumours form on the nerves that play an important role in regulating the balance and hearing from the inner ear to the brain.
	
	
	Meningiomas: these types of tumours are usually benign and have their origin in the meninges (membranes surrounding the spinal cord and the brain).
	
	
	Primitive neuroectodermal tumours (pnets): primitive neuroectodermal tumours (pnets) are rare malignant tumours that originate from the fetal (embryonic) cells in the brain.
	
	
	Pituitary adenomas: as the name suggests, these tumours arise from the pituitary gland located at the base of the brain.
	
	
	Medulloblastomas: common among children, these malignant primary tumours develop in the brain (lower back) and gradually spread via the spinal fluid.
	


Other types of primary brain tumours include craniopharyngiomas and germ cell tumours.

Secondary brain tumours ;

Unlike the primary brain tumours, these tumours have their origin in the other body parts and organs (mostly in the lungs, skin, colon, breast, and kidney) and then gradually metastasize to the brain. Secondary brain tumours are found to be more common.

 

What Are The Different Types Of Brain Tumours?

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?

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

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

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