Introduction
Sickle Cell Disorder, a health condition characterized by the predominance of hemoglobin S (HbSS), was first described in 1910 by James Herrick , referring to sickle shaped red blood cells that were found in a West Indian youth in 1927. Further research showed that the sickle shape of the cells - instead of the normal roundish shape of red blood cells - was related to low levels of oxygen.

Even though Sickle Cell Disorder is still a major cause of serious health challenges in both children and adults that suffer from the condition - and a cause of significant emotional and financial challenges to their loved ones, the prospects for people with the disorder have improved tremendously.

With early detection, adequate understanding of the disease and proper management, people living with the disease can lead very productive lives, pending a major breakthrough that provides a cure.
In this report we shall focus on the most common form of sickle cell disorder, homozygous SS.
How Does A Person Get Sickle Cell Trait?
Sickle Cell Disorder is not a contagious condition. It happens when a child inherits 2 sickle hemoglobin genes from both parents (SS). When people inherit only one gene, they do not have Sickle Cell Crisis, even though they are carriers of the Sickle Cell Trait.
What Is Sickle Cell Crisis, And What Leads To It?

Normal red blood cells are roundish in shape. But when the blood cells of people who have Sickle Cell do not receive enough oxygen, they grow longer, curve and change into the shape of a crescent moon which looks like the blade of the cutting tool called the sickle, hence the name Sickle Cell. Because of their new sickle-like shape, the cells have difficulty moving through the smallest blood cells, and often get stranded in blood vessels. As a result, blood is sometimes unable to circulate freely to other parts of the body leading to Sickle Cell Anaemia.
When such occurs, naturally, pain will occur and sometimes, damage to the body’s organs. Areas that are often clogged up include the blood vessels in the chest, legs, arms or abdomen.
When People Living With Sickle Cell have their blood vessels blocked by sickle-shaped red blood cells and this leads to intense pain, they are said to be in crisis. It can be a very difficult period in their lives.
A typical crisis period can last from as little as several hours to as much as several days. Perhaps only a woman in labor or cancer patient can describe such a state of intense pain.

There Are Several Types Of Crisis That Could Occur
1. Vaso-occlusive Crisis: this is when the sickle shaped blood cells obstruct capillaries, resulting in premature death of living tissues which could lead to significant organ damage.
2. Splenic Sequestration Crisis: this very painful crisis forces the spleen to expand. The function of the spleen is to clear defective blood vessels. During this crisis, the abdomen becomes bloated and hard almost like a stone. Numerous small blood vessels open up in the endocrine glands, the liver, lymphoid tissue, bone marrow and in the spleen itself, resulting in flooding of the spleen, circulatory problems and lower blood volume.
3. Aplastic Crisis: This crisis is caused by a virus called Parvirus B19, which is responsible for the childhood rashes called Fifth Disease. During crisis, the virus invades the blood, multiplies and destroys red blood cells.
4. Hemolytic crisis: this occurs in individuals who have what is called co-existent G6PD deficiency. Hemoglobin levels plummet, leading to rapid loss of red blood cells.
All four types of crisis worsen the patient’s baseline anemia, and can lead to ailments like:
• Pale skin as a result of lack of oxygen
• Increase in heart rate
• Pain syndromes
• Fatigue
• Acute chest syndrome
• Priapism; that is when an erect penis or clitoris doesn’t return to its flaccid state
• Persistent damage to the eye
• Cellular death of bone components of the hip as a result of interruptions of blood supply
• Leg ulcers
• Stroke

The Costs Of Sickle Cell Disorder
They include:
• Trauma of patients and their loved ones
• Loss of productivity
• Frequent absenteeism from school
• Frequent hospital visits
• High hospital bills
• Impacted quality of life
Home Remedies And Preventive Measures
• Drink at least 8 cups of water or and other fluids each day, because dehydration (lack of fluid) can make cells to become sickle-shaped.
• Rest a lot to avoid fatigue
• Take frequent rest breaks during strenuous exercise
• Avoid or manage stress
• Reduce any intake of alcohol as alcohol can cause dehydration
• Avoid oxygen-reducing practices like cigarette smoking and high altitude climbing
• Try to stay warm because exposure to cold air and water can cause a painful event. So keep body temperature cool
• Try to take a balanced diet and dietary supplements like folic acid. Take folate-rich vegetables.
• Use mild analgesics (pain killers) but don’t use Aspirin for children
• Take regular dose of any prescribed antibiotic for the prescribed periods
• Take regular dose of pain relievers your doctor may have prescribed to treat any severe case of pain
• Take Ciklavit nutritional supplement regularly. The manufacturer recommends a dosage of 10 ml or 2 tea spoons of Ciklavit for children below 5 years; and 20ml of two table spoons of Ciklavit for children above 5 years and adults.

According to experts, mildly painful events that don’t last too long can be treated at home with a combination of pain- reliving medicines, adequate intake of fluids and comfort measures.
When To Contact A Doctor
A medic’s attention should be sought if the patient begins to experience any of the following:
• Difficulty breathing, including shortness of breathe
• Pain that takes too long
• If skin or nail beds suddenly become pale
• Serious vomiting or diarrhea
• Unusually high frequency in urination
• Erection of the penis or clitoris that refuses to go soft

Extremely Though Economic Conditions Of Poor Children Living With Sickle Cell
Children Living With Sickle Cell Disorder whose parents are either poor or dead face double difficulty coping with the condition. An example is BoyX (names withheld). His parents had died and he was living with his guardian. His guardian refused to continue financing his education, telling him pointedly that he (the guardian) could not continue pay his school fees, as according to him, there is no guarantee that the boy would live long and therefore spending money on his education would be a waste. This boy, a child, boldly refused this pronouncement and proceeded to beg for money to pay his school fees, as according to him, ‘if his uncle wouldn’t help him, he had to help himself’. Unfortunately after a courageous battle to make something out of this life, in spite of a condition he didn’t put on himself, he lost the battle of life.

Another child living with Sickle Cell Disorder was repeatedly sexually abused by her stepfather, and even got thrown out of their house by the same stepfather when one evening her mother discovered what was happening. This repeatedly abused child, her mother and her brother –who also happens to be suffering from Sickle Cell Disorder - were left to live in the streets with its cold and stressful nights by the stepfather and they endured threats in addition to that.

The Prince And Princess Charles Offokaja Foundation (CAC IT no 75980) runs a programme that aims to give help to indigent children living with Sickle Cell Disorder, to protect their health and make their lives more comfortable and joyful.
You can encourage us today by liking our Facebook page
Source: [url]https://www.facebook.com/permalink.php?story_fbid=106492719716014&id=101940420171244
[/url]

Interesting

Humanitarian Bump!