Sickle cell disease, an inherited blood condition, shows itself as sickle cell anemia. Your red blood cells change shape when you have sickle cell anemia, going from being round, flexible discs to stiff, sticky sickle cells that restrict blood flow. Treatments can reduce suffering and aid in avoiding disease-related complications thanks to early discovery and modern therapies.
Sickle cell disease (SCD) is the most common genetic blood disorder. That implies that it is inherited within families. You have SCD from birth. It cannot be acquired or developed later in life.
In red blood cells, hemoglobin is responsible for transporting oxygen throughout the body. Hemoglobin in a healthy adult is rounded, smooth, and flexible. As a result, red blood cells can go through your bloodstream without difficulty. However, the hemoglobin’s structure changes if you have SCD. Red blood cells become curved and rigid as a result. The strangely formed cells prevent blood flow. Extreme pain, anemia, and other side effects are possible with this risky substance.
Symptoms of Sickle Cell Disease
Sickle cell anemia symptoms usually first appear in children. They may occur in infants as young as four months old., although they often start around six months. SCD comes in a variety of forms, but they all have a set of symptoms that can range from mild to severe.
The signs and symptoms of sickle cell disease:
- Anemia
- Episodes of pain
- Vision problems
- Infants’ uneasiness
- Frequent infections
- Delayed growth or puberty
- Swelling and pain in hands and feet
- Pain in the chest, back, arms, or legs
- Excessive fatigue or irritability from anemia
- Bedwetting from associated kidney problems
- Yellowing of the eyes and skin.
Most people with sickle cell anemia are more likely to experience the onset of new illnesses. Some of these diseases pose a severe risk to life. However, by becoming aware of their conditions and symptoms, people with sickle cell anemia can seek medical attention immediately to treat the problem.
Sickle Cell Disease Causes
Sickle cell anemia is a hereditary condition passed down from one’s biological parents. The gene that aids in producing healthy red blood cells mutates or changes in sickle cell anemia. Sickle cell anemia affects people who receive the defective hemoglobin protein gene from both biological parents. Sickle cell trait is present in people who receive one normal gene and one sickle cell gene (SCT). SCT patients typically do not exhibit any SCD symptoms but can convey the trait to their offspring.
Complications
Your child has a 25% chance of being born with sickle cell anemia if you and your partner are sickle cell trait carriers. There is a 50% chance that your child will be born with sickle cell trait if just one of you has sickle cell trait. However, your child won’t have sickle cell anemia. The complications of sickle cell disease are:
- Stroke
- Priapism
- Leg ulcers
- Gallstones
- Blindness
- Organ damage
- Deep vein thrombosis
- Splenic sequestration
- Acute chest syndrome
- Pulmonary hypertension
- Pregnancy complications
According to studies, sickle cell anemia people constantly struggle to control pain, which often compromises their quality of life. They occasionally experience severe pain due to sickled cells obstructing blood flow. This is a medical emergency and a VOC. Others experience chronic pain, which is discomfort that lasts for more than three to six months. If you endure ongoing pain, think about speaking with a pain management expert. They can offer a variety of pain management strategies.
How to Diagnose Sickle Cell Disease?
Sickle cell disease-related hemoglobin deficiency can be detected with a blood test. Adults, older children, and babies can all take this test. This test is a commonplace component of newborn screening in the United States.
The doctor might advise additional testing if the child has sickle cell disease. For example, the doctor could suggest a specialized ultrasound to determine your risk for stroke. In addition, they might offer a genetic counselor for you and your child (since you both carry the gene). Finally, an amniotic fluid test can reveal whether your unborn child has sickle cell illness or trait, whether you or your spouse have sickle cell disease or both. To learn more, consult your doctor.
Sickle Cell Disease Treatment
Blood transfusions, antibiotics to cure infections, and drugs to relieve symptoms caused by sickle cell anemia complications are commonly utilized by healthcare practitioners to treat sickle cell anemia. Hydroxyurea, Voxlelotor, L-Glutamine Therapy, and Crizanlizumab maybe some of these drugs.
Preventing Infections
Penicillin may be administered to kids with sickle cell anemia between 2 months and at least five years old. This reduces illnesses like pneumonia, which can be fatal to kids with sickle cell anemia.
Adults with sickle cell anemia may need penicillin for the rest of their lives if they’ve undergone pneumonia or spleen surgery.
All children should receive their first round of vaccines to help avoid sickness. However, due to the potential severity of their infections, they are notably more crucial for children with sickle cell anemia.
Your children’s doctor should ensure that your child obtains the full complement of recommended childhood immunizations, including those for hepatitis B, pneumonia, meningitis, and meningococcal disease, as well as an annual flu shot. Vaccinations are crucial for adults with sickle cell anemia as well.
Blood Transfusions
These are utilized by those who have sickle cell disease to treat and prevent consequences, including stroke. For example, red blood cells are taken from donor blood and supplied through a vein to a patient with sickle cell anemia during a red blood cell transfusion. This raises the quantity of healthy red blood cells, which helps lessen signs and side effects.
Stem Cell Transplant
This procedure (bone marrow transplant) involves replacing sickle cell bone marrow with healthy bone marrow from a donor. A sibling or other matched donor free of sickle cell anemia is typically used in the surgery.
The Final Word
Families and medical professionals have battled the painful effects of babies born with sickle cell anemia for over a century. Few newborns survived to reach their fifth birthday. Healthcare professionals made significant advancements in mistreating sickle cell anemia ten years ago. A newborn today will probably live well into their 50s. Research has made it possible to manage sickle cell anemia as a chronic condition by medical professionals. However, it is a disease with significant, occasionally life-threatening, medical implications. If you or your child has sickle cell anemia, you might appreciate the previous developments while looking forward to the future. The treatment of sickle cell anemia is the primary focus of scientists and medical professionals.
