Folic Acid And Penicillin
From birth to five years of age, penicillin daily, due to the immature immune system that makes them more prone to early childhood illnesses, is recommended. Dietary supplementation of folic acid had been previously recommended by the WHO. A 2016 Cochrane review of its use found “the effect of supplementation on anaemia and any symptoms of anaemia remains unclear” due to a lack of medical evidence.
Social Isolation Of People With Sickle Cell Disease
The deeply rooted stigma of SCD from society causes families to often hide their family members’ sick status for fear of being labeled, cursed, or left out of social events. Sometimes in Uganda, when it is confirmed that a family member has sickle cell disease, intimate relationships with all members of the family are avoided. The stigmatization and social isolation people with sickle cell disease tend to experience is often the consequence of popular misconceptions that people with SCD should not socialize with those free from the disease. This mentality robs people with SCD of the right to freely participate in community activities like everyone else SCD-related stigma and social isolation in schools, especially, can make a life for young people living with sickle cell disease extremely difficult. For school-aged children living with SCD, the stigma they face can lead to peer rejection. Peer rejection involves the exclusion from social groups or gatherings. It often leads the excluded individual to experience emotional distress and may result in their academic underperformance, avoidance of school, and occupational failure later in life. This social isolation is also likely to negatively impact people with SCD’s self-esteem and overall quality of life.
What Causes Sickling Each Red Bloodcell Is Densely Packed Withhemoglobin Proteinshealthy Red Blood Cells Are Packed Witha Form Ofhemoglobincallednormal Adult Hemoglobinhba Looks And Functions Properly All The Time In Sickle Cell Red Blood Cells Are Packed Withhemoglobin Sickle Proteins Proteinsare Created Due To Having Two Copies Of Anabnormalhbbgene Thesemoleculesproteinslook And Work Normally In A High
Each red blood cell is densely packed with hemoglobin proteins. Healthy red blood cells are packed with a form of hemoglobinHemoglobina protein in red blood cells that helps carry oxygen throughout the body called normal adult hemoglobinNormal adult hemoglobin healthy form of adult hemoglobin produced when there is no mutation in the HBB gene and it does not stick together to cause sickling . HbA looks and functions properly all the time.
In sickle cell, red blood cells are packed with hemoglobin sickleHemoglobin Sickle an abnormal form of hemoglobin that can stick together and cause cells to become rigid, sticky, fragile, and sickle-shaped proteins. HbS proteins are created due to having two copies of an abnormal HBB gene. These proteins look and work normally in a high-oxygen state , but they can change to an abnormal shape when they are in low-oxygen state.
This change in shape can cause these HbS molecules to stick to one another. When there are many HbS molecules in a cell, they clump together and form long chains called polymers in a process called polymerizationPolymerizationa process in which smaller molecules combine to create a larger group of molecules called a polymer.
The amount of HbS molecules you have compared with other forms of hemoglobin in the celland their subsequent polymerizationare the ultimate cause of sickling in red blood cells as well as the associated symptoms and complications of sickle cell.
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How Does Sickle Cell Anemia Affect People
Babies born with sickle cell anemia may not have symptoms for several months. When they do, symptoms include extreme tiredness or fussiness from anemia, painfully swollen hands and feet, and jaundice. Babies may also have spleen damage that affects their immune system and increases their risk for bacterial infections. As people with sickle cell anemia grow older, they may develop different and more serious medical problems that happen when organ tissues dont receive enough oxygen. People with sickle cell anemia are at increased risk for stroke and lung, kidney, spleen and liver damage.
What Are The Treatments For Sickle Cell Disease
The only cure for SCD is bone marrow or stem cell transplantation. Because these transplants are risky and can have serious side effects, they are usually only used in children with severe SCD. For the transplant to work, the bone marrow must be a close match. Usually, the best donor is a brother or sister.
There are treatments that can help relieve symptoms, lessen complications, and prolong life:
- Antibiotics to try to prevent infections in younger children
- Pain relievers for acute or chronic pain
- Hydroxyurea, a medicine that has been shown to reduce or prevent several SCD complications. It increases the amount of fetal hemoglobin in the blood. This medicine is not right for everyone talk to your health care provider about whether you should take it. This medicine is not safe during pregnancy.
- Childhood vaccinations to prevent infections
- Blood transfusions for severe anemia. If you have had some serious complications, such as a stroke, you may have transfusions to prevent more complications.
There are other treatments for specific complications.
To stay as healthy as possible, make sure that you get regular medical care, live a healthy lifestyle, and avoid situations that may set off a pain crisis.
NIH: National Heart, Lung, and Blood Institute
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Living With Sickle Cell Disease
Sickle cell disease is a life-long condition. Although the complications of sickle cell disease may not be able to be prevented entirely, living a healthy life-style can reduce some of the complications.
It is important to eat a healthy diet with lots of fruits, vegetables, whole grains, and protein, and drink lots of fluids.
Do not take decongestants because they cause constriction of blood vessels and could trigger a crisis.
Other factors that may trigger a crisis include high altitudes, cold weather, swimming in cold water, and heavy physical labor.
Avoid infections by getting an annual flu shot, washing your hands frequently, avoiding those who are sick, and getting regular dental exams.
The Occurrence Of Symptoms
The symptoms of sickle cell anemia normally appear for the first time when a newborn reaches 5 to 6 months of age. Every individual reacts to the disease differently, so the signs, the degree of severity, and the relapse frequency greatly vary from person to person.
To better understand what symptoms people usually have during the crisis of Sickle Cell Anemia, Dr. Sebi Journal interviewed Hehleena Cardosa, a top Sickle cell expert in Washington. She helped us understand how symptoms of Sickle cell Anemia manifest. Heres some parts of the interview
I always feel agonizing whenever I see an SCD patient approaching me with a wild pain, she recalls. It is so heartbreaking. In my 23 years of being an SCD expert, I have observed that Pain is an everyday struggle in most SCD patients.
Adding further to it, Hehleena says: Let me tell you, most SCD patients who come here deal with a great degree of
welling on their hands and feet.Pale skin is also common, which indicates the presence of Jaundice. Fever is another symptom people usually get during the crisis of SCD.
She told us in her interview that patients with sickle cells are anemic and debilitated. If your child relatively has slow body growth, it may be a sign of Sickle cells. Frequent relapse of infection is also common in SCD patients.
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Pregnant Women With Sickle Cell Disease
Women living with SCD who become pregnant often face extreme discrimination and discouragement in Uganda. These women are frequently branded by their peers as irresponsible for having a baby while living with sickle cell disease or even engaging in sex while living with SCD. The criticism and judgement these women receive, not only from healthcare professionals but also from their families, often leaves them feeling alone, depressed, anxious, ashamed, and with very little social support. Most pregnant women with SCD also go on to be single mothers as it is common for them to be left by their male partners who claim they were unaware of their partner’s SCD status. Not only does the abandonment experienced by these women cause emotional distress for them, but this low level of parental support can be linked to depressive symptoms and overall lower quality of life for the child once they are born.
What Is Sickle Cell Disease
Sickle cell disease is a group of inherited red blood cell disorders. Red blood cells contain hemoglobin, a protein that carries oxygen. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has SCD, the hemoglobin is abnormal, which causes the red blood cells to become hard and sticky and look like a C-shaped farm tool called a sickle. The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious complications such as infection, acute chest syndrome and stroke.
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How Is Sickle Cell Anemia Treated
A number of different treatments are available for SCD:
- Rehydration with intravenous fluids helps red blood cells return to a normal state. The red blood cells are more likely to deform and assume the sickle shape if youre dehydration.
- Treating underlying or associated infections is an important part of managing the crisis, as the stress of an infection can result in a sickle cell crisis. An infection may also result as a complication of a crisis.
- Blood transfusions improve transport of oxygen and nutrients as needed. Packed red cells are removed from donated blood and given to patients.
- Supplemental oxygen is given through a mask. It makes breathing easier and improves oxygen levels in the blood.
- Pain medication is used to relieve the pain during a sickle crisis. You may need over-the-counter drugs or strong prescription pain medication like morphine.
- helps to increase production of fetal hemoglobin. It may reduce the number of blood transfusions.
- Immunizations can help prevent infections. Patients tend to have lower immunity.
Bone marrow transplant has been used to treat sickle cell anemia. Children younger than 16 years of age who have severe complications and have a matching donor are the best candidates.
Complete Blood Count Test And Sickle Cell Disease
A complete blood count test can help the care team monitor your childs sickle cell disease. This test can help the care team find out if treatments are working.
The complete blood count test measures levels of:
- Red blood cells
Does Haplotype Correlate With The Clinical Severity Of Sca
In an individual patient, the clinical severity of SCA can range from mild to severe. The degree of phenotypic variation in a disease caused by a single-base substitution illustrates the importance of other genetic loci in modifying disease severity. Recent anecdotal observations among the Arabian/Indian and Senegal haplotypes suggest they are associated with milder forms of SCA, whereas the Bantu haplotype is associated with a more severe clinical course . Interestingly, this observation correlates with differences in the mean levels of fetal hemoglobin in patients with different haplotypes. Individuals with the Arabian/Indian haplotype had an average 17% FH , those with the Senegal haplotype had an average 12.4% FH , and those with the Bantu and Benin haplotypes had even lower average FH. However, within each haplotype group, the range of FH levels is very broad.
Patients with SCA tend to maintain perceptible levels of FH in their RBCs throughout childhood. This persistence has a protective effect on SCA severity, presumably by blocking the assembly of the long polymers of abnormal hemoglobin. Reactivating or increasing production of FH is the rationale for administering hydroxyurea, the only drug licensed to ameliorate the symptoms of SCA. By increasing FH content in the blood of some patients, hydroxyurea administration has been shown to decrease the incidence of painful episodes and serious vaso-occlusive incidents .
Misconceptions About Sickle Cell Disease
The stigma around the disease is particularly bad in regions of the country that are not as affected. For example, Eastern Ugandans tend to be more knowledgeable of the disease than Western Ugandans, who are more likely to believe that sickle cell disease resulted as a punishment from God or witchcraft. Other misconceptions about SCD include the belief that it is caused by environmental factors but, in reality, SCD is a genetic disease. There have been efforts throughout Uganda to address the social misconceptions about the disease. In 2013, the Uganda Sickle Cell Rescue Foundation was established to spread awareness of sickle cell disease and combat the social stigma attached to the disease. In addition to this organization’s efforts, there is a need for the inclusion of sickle cell disease education in preexisting community health education programs in order to reduce the stigmatization of sickle cell disease in Uganda.
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What Is The Difference Between Normal Red Blood Cell And Sickle Cell
The key difference between normal red blood cell and sickle cell is the shape of the cell. That is normal red cells are round in shape while the sickle cells have a sickle shape. Also, a further difference between normal red blood cell and sickle cell is that normal red blood cells are flexible while sickle cells are rigid and sticky.
Moreover, normal red cells contain haemoglobin A and sickle cells contain haemoglobin S. So, this is another difference between normal red blood cell and sickle cell.
The below infographic presents more information regarding the difference between normal red blood cell and sickle cell.
When Should I Call The Doctor
Get emergency medical care right away if your child has any of these problems:
- fever of 101°F or higher
- pain that isn’t getting better with medicine
- severe stomach pain or swelling
- shortness of breath or trouble breathing
- extreme tiredness
- skin that’s yellow or very pale
- a penile erection that is not going away or is painful
- sudden change in vision
- weakness or trouble moving part of the body
- loss of consciousness
- numbness or tingling
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General Immune System Considerations In Scd: A Broad Overview
SCD impacts many aspects of the immune system, from innate to adaptive immunity. Outside of transfusion medicine, the most recognized consequence of immune dysregulation in SCD is an increased risk of infection infection has historically been a leading cause of morbidity and mortality world-wide in people with SCD . Along these same lines, responses to vaccines appear to be less sustained in people with SCD . Although hyposplenism presumably impacts the risk of infection as well as vaccine responses, it is also likely that alterations in white blood cell subsets , platelets, RBCs, and complement are involved to some extent. Elevated WBC counts were described decades ago in people living with SCD . Activated neutrophils from people with SCD have been described to be associated with disease complications such as pain crises and they also contribute to neutrophil extracellular traps . Thrombocytosis in people with SCD contributes to the inflammatory milieu , with people with hemoglobin SS disease having higher platelet counts than those with sickle cell trait or those with Hgb AA . Further, people with SCD also have high levels of soluble CD40L and baseline platelet activation . The generation of free heme from ongoing RBC breakdown activates the alternative pathway of complement and increased levels of complement C3-C5 have been noted in people with SCD compared to controls .
What Are The Symptoms Of Sickle Cell Disease
The following is a list of symptoms and complications associated with sickle cell disease. However, each person may experience symptoms differently. Symptoms and complications may include:
The symptoms of sickle cell disease may look like other blood disorders or medical problems. Always consult your health care provider for a diagnosis.
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Who Is At Risk For Sickle Cell Disease
In the United States, most of the people with SCD are African Americans:
- About 1 in 13 African American babies is born with sickle cell trait
- About 1 in every 365 black children is born with sickle cell disease
SCD also affects some people who come from Hispanic, southern European, Middle Eastern, or Asian Indian backgrounds.
Symptoms Of Sickle Cell Disease
People born with sickle cell disease tend to have problems from early childhood, although some children have few symptoms and lead normal lives most of the time.
The main symptoms of sickle cell disease are:
- painful episodes called sickle cell crises, which can be very severe and last up to a week
- an increased risk of serious infections
- anaemia , which can cause tiredness and shortness of breath
Some people also experience other problems, such as delayed growth, strokes and lung problems.
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Other Pathways Or Cells Of Potential Interest In Humans With Relevance To Rbc Alloimmunization
A customized single nucleotide polymorphism panel was utilized to evaluate 700 sequence variants in a multi-center case-control study of people with SCD living in France and The Netherlands . The group did not find any large-effect SNPS associated with RBC alloimmunization, though 19 SNPs that were determined to be moderately associated with RBC alloimmunization were identified in the secondary analysis. Of these, the authors concluded that rs5743618 in TLR1 was likely the most important risk factor for RBC alloimmunization of the SNPs studied, with a four-fold increased risk of alloimmunization per A-allele. A SNP in TANK was also identified as being of interest. SNPs found to be associated with alloimmunization prevention included those in STAM , STAT4, and IFNAR . A different group reported a regulatory locus on Chromosome 5 to be associated with RBC alloantibody formation , with the same group reporting no large-effect SNPs in a smaller study of alloimmunized and non-alloimmunized people with SCD .