Anemia, often known as sickle cell disease (SCD), is an inherited blood condition that damages the hemoglobin in red blood cells. Both kids and adults can develop acute chest syndrome (ACS), a serious lung-related consequence of sickle cell disease. One of the main causes of morbidity, hospitalizations, and fatalities in children and adults with sickle cell disease, it results in a sickness that resembles pneumonia and causes hospitalizations. ACS develops when sickle red blood cells obstruct pulmonary blood vessels. This is a serious matter that could endanger your life.

 

Causes 

 

Acute chest illness has no clear cause as of yet, according to scientists. However, they think it has something to do with one or more of the following:

 

• Bone marrow necrosis - The soft tissue found inside most bones is called bone marrow. In the event of bone marrow necrosis, particles may circulate in your blood. Acute chest syndrome may be exacerbated by particles that enter your lungs.
• Fat embolism - A fat embolism can also be brought on by bone marrow necrosis. A chunk of fat like this gets lodged in a blood vessel and prevents blood flow. Acute chest syndrome could result from it.
• Infection - Acute chest syndrome can be brought on by infections like viral or bacterial pneumonia.
• The most common cause of acute chest syndrome in children is infection. Fat embolism frequently contributes to the problem in adulthood.

 

Symptoms 

 

You might be coughing or struggling to breathe. You can be wheezing a lot as well. While extremely unpleasant, these symptoms may mimic a cold or another breathing issue.

 

Of those with SCD, around one-third have acute chest syndrome. You have an 80% chance of receiving it again if you already had it once. Many people delay seeing a doctor since the symptoms can also be related to other illnesses. Additionally, unless your doctor is aware that you have SCD, these symptoms may not always cause them to take notice.

 

Who is at Risk?

 

Although there is no one specific cause of ACS, certain circumstances can make it occur. When the body is under stress from an illness or dehydration, ACS can appear abruptly.

 

• Medical Conditions - Infections including pneumonia and asthma, pulmonary embolism (blood clots from other areas of the body moving to the lungs), pulmonary fat embolism (bone marrow moving from the bones to the lungs), and pulmonary artery thrombosis (blood clots growing in the lungs).
• Vaso-occlusive crisis (VOC) - An episode of sudden onset discomfort is known as a vaso-occlusive crisis (VOC). The pain is frequently localized in the arms, legs, chest, or back, as well as the extremities. A side effect of VOC might be ACS.
• Low oxygen levels - The development of sickle cells is caused by low oxygen levels in the blood and lungs. This could be the effect of a lung infection.
• Low Fetal Hemoglobin (HbF) - The primary form of hemoglobin in a fetus is called fetal hemoglobin, or HbF. Since HbF remains in red blood cells after birth, it prevents sickling. The sickling of blood cells can result from low levels of HbF.
• Genotype - Sickle The sickle gene S results in hemoglobin. Individuals with SS have the most severe form of SCD because genes reside in pairs. SS may coexist with another hemoglobin gene variant, such as C, which results in a less severe form of the illness.
• Age - Any age can experience ACS. Ages 2 to 4 are when the greatest onset of ACS occurs in children with SCD.
• Recent trauma or surgery - You might have shallow breathing and low oxygen levels after surgery as a result of pain and medicine, which can lead to ACS-causing sickling in the lungs.
• Active or passive smoking - Children who smoke or are exposed to secondhand smoke have more severe episodes and a higher risk of developing ACS.

 

Diagnosis 

 

Rapid diagnosis of acute chest illness can prevent death. If you have SCD and are in the hospital, you need to undergo routine testing and monitoring.

 

Medical professionals can diagnose the problem using the following tests:

 

• Analysis of arterial blood gasses to determine your blood's pH, oxygen content, and carbon dioxide levels.
• Complete blood counts, which assess red blood cells, white blood cells, and platelets, are one type of blood test.
• An X-ray of your chest to check for any lung infiltrates or infections
• Cultures can check bodily fluids like blood, spit, or urine for different illnesses
• Your healthcare professional may assign a mild, moderate, or severe grade to acute chest syndrome depending on the test results and your symptoms