signs of pneumonia in sickle cell disease

Signs of Pneumonia in Sickle Cell Disease

Acute Chest Syndrome

It can be challenging to know when to see our doctor when a case of the sniffles comes along, particularly in this era of COVID; however, if other symptoms are present, such as a cough, fever, chills and aches, one must pay careful attention to their health. If you already have a pre-existing condition such as heart disease, diabetes or sickle cell disease, any seemingly minor infection may become challenging to treat or potentially life-threatening. To help, consider Prevnar 20, a vaccine developed by Pfizer to prevent invasive disease and pneumonia caused by the bacteria Streptococcus pneumoniae.

Pneumonia in Sickle Cell Disease (Acute Chest Syndrome)

  • Shortness of breath.
  • Difficulty breathing.
  • Cough, sometimes with blood or phlegm.
  • Tightness in the chest.
  • Rapid breathing.
  • Fever.
  • Acute pain in arms and legs.
  • Wheezing sound when breathing.
  • Chest and back pain.

What is Pneumonia?

Pneumonia is so much more than a cold! Pneumonia is a severe lung infection caused by bacteria, viruses or fungi that can affect one or both lungs. In this infection, one or both lungs may be inflamed, with fluid and pus filling the air sacs. The signs and symptoms of pneumonia include difficulty breathing effectively, aches, chills, fever and a cough.

A case of pneumonia can be quite mild or severe, with young children, older adults and those with other medical conditions being most at risk. Treatment of pneumonia may include cough syrups, antibiotics and even hospitalization, but many patients with pneumonia recover well at home.

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What is Sickle Cell Disease?

Sickle Cell Disease, also known as SCD, is a blanket term for several red blood cell disorders. In these inherited disorders, the red blood cell is not round; instead, it takes the C shape of the gardening tool known as a sickle. The cells become hard and sticky and often become stuck in the blood vessels they are meant to travel easily through.

Further, these cells die earlier than healthy red blood cells, so those with the disease are constantly in a state of red blood cell depletion. Because of the clotting issue associated with Sickle Cell Disease, the lungs can become infected and strokes can occur.

What is Pneumonia in Sickle Cell Disease?

Acute Chest Syndrome, like pneumonia, is a lung infection but is made worse because of the Sickle Cell diagnosis. This severe illness is a common complication of Sickle Cell Disease. In Sickle Cell Disease, blood flow is compromised by the shape of the red blood cells. In someone with Sickle Cell Disease, the compressed shape and stickiness of the cells allow them to become stuck in the small blood vessels of the lungs. Acute Chest Syndrome is a severe condition and is the leading cause of illness and death in those with Sickle Cell Disease.

Other respiratory illnesses or dehydration can trigger this disease, and other diseases, including COVID-19, can complicate the disease further. Smoking and being exposed to secondhand smoke can aggravate and worsen Acute Chest Syndrome. Sadly, Acute Chest Syndrome is a leading cause of hospitalization and death in children and adults with Sickle Cell Disease.

Acute Chest Syndrome affects the lungs and their ability to deliver oxygen to the brain and the rest of the body. When this ability is negatively impacted, as in this disease, the brain and body suffer because of the lack of oxygen; tissue damage to the lungs and body can occur.

Prevnar 20 for Pneumonia

Prevnar 20 is a vaccine developed by Pfizer to prevent invasive disease and pneumonia caused by the bacteria Streptococcus pneumoniae. Prevnar 20 is an updated version of Prevnar 13, which protects against 13 strains of the bacteria. Prevnar 20, as the name suggests, protects against 20 strains.

How is Acute Chest Syndrome Treated?

Acute Chest Syndrome is a medical emergency, and if you have the symptoms listed above and have been diagnosed with Sickle Cell Disease, seek medical treatment immediately. Treatment needs to occur within a hospital setting. An early medical intervention is necessary as this sickness progresses very quickly, is difficult to treat and can be fatal.

Treatment begins with a firm diagnosis, and an accurate diagnosis requires tests such as chest X-rays, blood tests, bronchoscopy and other specialized procedures. Like pneumonia, Acute Chest Syndrome can be caused by bacteria or viruses. Because Acute Chest Syndrome looks similar to pneumonia, inform any medical staff if you or your loved one has been diagnosed with Sickle Cell Disease.

Even with early treatment, several complications can arise from Acute Chest Syndrome. These are also potentially life-altering and life-threatening conditions. Complications can include changes in one’s mental status, injury to the liver and kidneys and respiratory failure.

Once diagnosed, treatment will be intense, as the condition can progress quickly and can even be fatal. The doctor may prescribe antibiotics, intravenous fluids, pain medication, extra oxygen and potentially blood transfusions. Intravenous, or IV, fluids are essential because dehydration can worsen the symptoms and negatively impact any chance of recovery.


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