How to Treat Acute Chest Syndrome in Sickle Cell Disease

What is Acute Chest Syndrome

Acute chest syndrome (ACS) remains a leading cause of premature mortality in SCD. ACS is the second-most common reason for hospitalization in SCD patients. The severity of ACS is variable, ranging from a mild illness to a severe life‐threatening condition. ACS is often precipitated by infection but treating ACS as a purely infective episode may lead to progression and rapid clinical deterioration.

The clinical features of ACS may not be evident at the time of admission. Nearly half of patients present initially with a painful vaso‐occlusive crisis and then develop this complication while in the hospital. ACS will often develop 24–72 hours after the onset of severe pain (Gladwin & Vichinsky, 2008). It is important to note nearly 50% of ACS occurs in patients hospitalized for other causes (notably, pain).

The most common respiratory symptoms of ACS are:

• Cough
• Chest pain
• Shortness of breath
• Rib and sternal pain
• Chills
• Wheezing
• Hemoptysis

ACS may occur secondary to infection, and infectious organisms were identified in 38% of cases who underwent detailed investigations including blood culture, nasopharyngeal sampling for viral culture, sputum culture, and serum samples for antibody response and bronchoscopy (Vincinsky, et al, 2000). The identification of a specific infectious organism is less likely with standard investigations alone. The most common bacterial organism identified in adults is C. pneumoniae and in children is Mycoplasma pneumoniae, and the most common virus identified is the respiratory syncytical virus (RSV).

Thymulin is produced in the thymus and promotes proper T cell activity. Studies have shown for decades that a lack of zinc results in a decrease and eventual absence of thymulin. This causes fewer natural killer T cells to mature, which means there are less cells to fight pathogens.

Zinc deficiency is common in patients with sickle cell disease (SCD). SCD patients increased hemolysis, the breakdown of red blood cells, creating a loss of zinc. This event creates a free zinc in the blood plasm; however, the zinc is not reabsorbed by the kidneys due to, in many cases, renal abnormalities created by repeated vaso-oclusive crisis. For this reason, almost all of those with sickle cell disease lack normal levels of zinc.

Zinc supplementation was actually reported to increase thymulin, the presence of T cells, the T4/T8 ratio, and the presence of Immunoglobulin M (IgM). (IgM) – the largest and first antibody to respond to an antigen – is almost nonexistent in zinc-deficient SCD patients. IgM is responsible for many immune-related responses, including activating complement, supporting T cell responses, and providing memory of antigens to lymph tissues in the body. An absence of IgM results in a very weak immune system.

As a result of increasing immunity, one study found zinc supplementation in patients with sickle cell disease reduced the frequency of clinical infections, especially:

• Acute bronchitis
• Upper respiratory tract infections
• Pneumonia

These infections, especially Mycoplasma pneumonia and Chlamydia pneumonia, are the most common causes of chest crises in patients with SCD. So, through long-term zinc supplementation, patients with SCD will suffer fewer chest crises. Treatment with a daily dosage of zinc will vastly improve patients’ quality-of-life.

It was also suggested zinc helped reduce pain crises by moderating the activity of red blood cells, endothelial cells (which line the walls of vessels), and leukocytes. Through a number of other randomized placebo-controlled studies, it is estimated even using regular zinc supplements for a month yields benefits in treating sickle cell disease.

Zinc is a safe and effective therapy for those living with the painful manifestations of sickle cell disease. The advantage of using zinc is that it is widely available. It’s very easy to find and take as a regular daily supplement pill. However, it is important to look more closely into the role of supplementation to reduce death and complication rates of sickle cell.

By actively taking zinc, which is readily available and inexpensive, those affected by sickle cell can normalize zinc levels and reduce the instance of chest crises. Controlling crises using natural ingredients is an excellent way that sickle cell patients can enjoy good health without the negative side effects common to conventional medications.