Sickle Cell Disease In Children: Early Detection, Intervention, And Long-term Management

Sickle cell disease is a genetic disorder that affects the red blood cells, causing them to become misshapen and break down more easily. It is an inherited condition that primarily affects people of Middle Eastern, African, Mediterranean, and Indian descent. Early detection, intervention, and long-term management are critical for improving the quality of life and outcomes for children with sickle cell disease.

The red blood cells’ capacity to carry oxygen or hemoglobin, is affected by sickle cell disease. Because normal hemoglobin cells are rounded, smooth, and flexible, much like the letter “O,” they may easily pass through the blood veins in our body. When oxygen is removed from sickle cell hemoglobin cells, which are hard and clingy, they take on the shape of a sickle or the letter “C.” These sickle cells frequently group together and find it difficult to pass through blood channels. The cluster creates a bottleneck and prevents the flow of sound, regular, oxygen-carrying blood. The painful and harmful complications of sickle cell disease are brought on by this obstruction.

Sickle cells have a lifespan of only 10 to 20 days, compared to the 120-day lifespan of normal hemoglobin. Additionally, because of their rigidity and structure, sickle cells run the risk of being destroyed by the spleen. Blood infections are filtered out by the spleen, and sickled cells become caught in this filter and perish. A person with sickle cell disease is chronically anemic as a result of the lower quantity of hemoglobin cells circulating in their body. The sickled cells’ obstruction of healthy, oxygen-carrying cells causes harm to the spleen as well. These people are more susceptible to infections because their spleens are not working normally.

Early Detection The Secret to Fighting Sickle Cell Disease

When a child receives a diagnosis of sickle cell disease, vigilant health monitoring becomes paramount. This hereditary blood disorder demands close attention, especially in infant and early childhood, as young patients face a heightened vulnerability to life-threatening infections where youngsters under the age of 3 years old may experience painful swelling in their fingers and toes, known as dactylitis, as an additional challenge associated with the condition.

Hemoglobinopathy testing, or checking newborns for hemoglobin abnormalities, is now commonly done in many states as part of newborn screening blood tests that are regularly done to find serious, life-threatening disorders. State regulations mandate that infants be tested between the ages of two and seven days, even if they appear healthy and don’t exhibit any symptoms of illness. Early diagnosis is crucial for delivering the right preventative care for some of the fatal illness consequences.

How Does Sickle Cell Disease Affect People?

Sickle cell disease primarily affects people of African descent and those of Hispanic descent with Caribbean ancestry, while it has also been observed in people with Middle Eastern, Indian, Latin American, Native American, and Mediterranean ancestry. It is a genetically based disease that runs in families. Sickle cell disease consequences affect millions of people globally.

Symptoms of Sickle Cell Disease in Children

Most Sickle Cell Disease patients begin to exhibit symptoms during the first year of life, frequently around 5 months. The symptoms can differ for every child. They could be minor or major. Some symptoms include:

• Anemia is the most typical symptom in children who have sickle cell disease. Anaemia results from having fewer red blood cells. A kid with anemia may appear pale and worn out.
• Jaundice is a yellowing of the eyes, mouth, and skin. This symptom is typical. Sickle cells have a shorter lifespan than regular red blood cells. They pass away more quickly than the liver can remove them. When red blood cells die, a chemical called bilirubin is released, which gives the blood its yellow colour.
• Sickle crisis, or a pain crisis. Small blood arteries can cause sickle cells to become stuck as they go through them. Pain results from this blood flow obstruction. Although this acute pain can strike anywhere, it typically affects the chest, arms, and legs. Sickle cell disease children and infants may have painful finger and toe swelling. Blood flow obstructions can also result in tissue aging.
• Acute chest syndrome occurs when sickle cells congregate and obstruct the flow of oxygen to the lungs’ minuscule blood capillaries. This might be fatal. When the body is under stress from an infection, a fever, or fluid loss (dehydration), it frequently happens suddenly. There may be fever, pain, and a severe cough, and it may appear to be pneumonia.
• Sequestration or pooling of spleen. Sickle cells accumulate in the spleen, causing it to expand and hurt. There are fewer red blood cells that can move. A sudden reduction in hemoglobin may result from this. If not treated right away, it could be fatal.

Sickle Cell Disease symptoms can resemble those of other diseases or medical conditions. Always consult your child’s doctor or the specialist for a diagnosis.

Treatment of Sickle Cell Disease in Children

The course of treatment depends on the symptoms, age, and general health of the child. Furthermore, it will depend on how serious the problem is. Treatment of this condition involves early detection and avoiding new issues. Your child’s doctor will suggest a hematologist to you. An authority on blood problems, this. Patient care may also include the involvement of other specialists.

• Medication for pain as these are applied to pain emergencies.
• 8 to 10 glasses of water per day is recommended. In doing so, pain crises are avoided and treated. IV (intravenous) fluids may be required in specific circumstances.  
• Transfusions of blood as these are used to treat splenic sequestration, acute chest syndrome, persistent pain, anaemia, and chronic pain, as well as to avoid stroke.
• Antibiotics and vaccines that serve as an infection preventative.
• Acid folic to lessens the risk of severe anaemia.
• Regular examinations of the eyes. These are carried out to check for retinopathy, an eye disease. Every year, have your child’s eyes tested.
• Transplant of stem cells as some kids with sickle cell diseae can be cured through transplants. These treatments are still being researched. Speak to your child’s medical professional. Only specific hospitals perform transplants and if you are looking for a better healthcare facility for your child you can opt for Bone Marrow Transplant in India as the country has some of the best medical facilities.
• Hydroxyurea is a medication that has the ability to lower the blood’s sickle cell count. Complications, unpleasant occurrences (crises), and hospital stays are decreased.

How Can I Support The Survival Of My Child Who Has Sickle Cell Anaemia?

The life-threatening effects of sickle cell anaemia have decreased thanks to improvements in preventive treatment and new medications. However, it is still a serious, persistent, and occasionally fatal condition. Specialists should take good care of your child. What determines how your child is handled is:

• Your child’s type of sickle cell anaemia
• How bad the illness is
• The frequency of your child’s difficulties
• How diligently you and your child practise prevention
• Has a balanced diet
• Sleeps enough
• Drink a lot of water

Childhood Sickle Cell: Key Takeaways for Parents

• A genetic blood condition known as sickle cell disease is present at birth. This indicates that it is inherited through a parent’s DNA.
• Red blood cells with Sickle Cell Disease have an unusual C shape. They obstruct blood flow by becoming lodged in tiny blood arteries.
• This obstruction may be painful and infectious. It can potentially result in a stroke and harm a child’s vital organs.
• As part of a series of screening exams, newborns are examined for sickle cell disease.
• In the first year of life, typically around five months, the majority of youngsters will begin to exhibit symptoms.

Reference

• https://www.nationwidechildrens.org/family-resources-education/family-resources-library/early-diagnosis-key-to-dealing-with-sickle-cell-disease
• https://www.hindustantimes.com/lifestyle/health/sickle-cell-disease-in-children-early-detection-intervention-and-long-term-management-101690538047062.html
• https://www.sciencedirect.com/science/article/pii/S0268960X1300057X
• https://emedicine.medscape.com/article/205926-treatment