Sickle Cell Crisis - Pain Crisis

What Is Sickle Cell Disease?

A sickle cell disease is a group of inherited blood disorders involving red blood cells. It is mostly seen in African-Americans. Sickle cell disease leads to sickle cell anemia and sickle cell crisis. The disorder is observed at the time of birth in the affected individual and results in abnormal production of hemoglobin. Generally, hemoglobin is a protein on the surface of red blood cells and helps transport oxygen and essential nutrients to all body parts. Red blood cells are disc-like and have flexibility, allowing them to travel through small and narrow blood capillaries. In sickle cell disease, the production of abnormal hemoglobin makes the red blood cells rigid and sickle or ‘C’ shaped. These sickle-shaped red blood cells get stuck in the blood capillaries and block the blood flow, causing pain and infection. The main clinical feature of sickle cell disease is an acute painful crisis that often needs hospitalization. This can result in some serious conditions like stroke, multiple organ failure, acute chest syndrome, and premature death.

What Is the Etiology of Sickle Cell Disease?

Sickle cell disease is a genetically inherited disorder involving abnormal production of hemoglobin. It shows an autosomal recessive pattern of gene mutation. Sickle cell disease involves nucleotide gene mutation on chromosome 11, which substitutes the position of glutamic acid to valine at position six on the beta-globin subunit resulting in the abnormal production of hemoglobin.

What Is the Sickle Cell Crisis?

Sickle cell crisis refers to a condition that is associated with sickle cell anemia. In sickle cell anemia, the sickle or ‘C’ shaped red blood cell gets stuck in the small blood capillaries blocking the flow of the blood in the various parts of the body and resulting in episodes of extreme pain. The pain may vary in intensity and last for several hours or weeks. In some cases, chronic pain causes damage to joints and bones, ulcers, and other complications.

The word sickle cell crisis is used to describe several other crisis-related conditions associated with sickle cell anemia, such as -

Vaso-occlusive Crisis

In a vaso-occlusive crisis, the following signs are observed

• Patients may suffer from moderate to severe pain.

• The intensity and frequency of the pain may vary in the different affected individuals.

• Children may experience severe pain and swelling in their hands and feet.

• The pain is present in the lower and upper extremities, chest, and back.

• Fever.

Splenic Sequestration Crisis

In a splenic sequestration crisis, the following signs are observed

• In sickle cell disease, spleen infarction is involved in childhood.

• The spleen is involved due to its narrow blood vessels, and it plays a crucial role in the lymphoreticular system.

• The splenic sequestration crisis results in acute extreme pain and a sudden drop in hemoglobin level, which can be a life-threatening condition.

Aplastic Crisis

In an aplastic crisis, the following signs are seen

• In sickle cell crisis, a sudden drop in hemoglobin resulting in pallor and weakness is caused by the aplastic crisis, and it is accompanied by reticulocytopenia.

• It is also observed that it is triggered by a viral infection caused by parvovirus B19 which directly affects the bone marrow by suppressing the production of red blood cells. This leads to a shortening of the life span of red blood cells and a dropping level of hemoglobin level in the blood.

• The aplastic crisis is a self-limiting condition that lasts for seven to ten days.

Acute Chest Syndrome

In acute chest syndrome, the following are the conditions that are observed

• Acute chest syndrome is triggered by hypoxia (low levels of oxygen in body tissues) due to the hypoventilation caused by the vaso-occlusive crisis. Hypoxia leads to the sticking of the red blood cells in the tiny vessels of the lungs.

• It can also be caused by pulmonary embolism originating from the vaso-occlusive crisis.

• These symptoms include fever, coughing, tachypnea (fast and shallow breathing), chest pain, wheezing, and pulmonary failure.

Hemolytic Crisis

In a hemolytic crisis, the following are the signs that are observed

• A sudden drop in the hemoglobin concentration.

• Patients may also experience glucose-6-phosphate dehydrogenase deficiency.

Others

Some other conditions are also observed in the sickle cell crisis

• Femoral and humeral necrosis.

• Proliferative retinopathy.

• Renal complications.

How to Investigate the Sickle Cell Crisis?

The evaluation of sickle cell crisis is done on the basis of the past family history of the affected individual as it is an autosomal recessive disorder.

Routine laboratory investigation involving

1. Complete Blood Count - A blood test helps determine the count of red blood cells in the blood.

2. Packed Cell Volume - It helps determine the hemoglobin content in the blood.

3. Reticulocytes Count - Increased reticulocyte count, helps to determine the production of new red blood cells.

4. X-Rays - Imaging tests are also performed to check the effect of the crisis. For example - A chest X-ray is performed to identify acute chest syndrome.

5. CT Scan (Computed Tomography) and MRI (Magnetic Resonance Imaging) - Are performed to investigate the structure and function damage to different organs.

6. Ultrasound Sonography - It helps to check the abdomen and some abnormalities like cholelithiasis.

Other investigations like liver function tests and renal function tests are also performed.

What Are the Treatment Options for Sickle Cell Crisis?

Treatment of the sickle cell crisis depends on the intensity and frequency of the pain. As the pain arises, a shot of analgesic can help the patient. Depending upon the pain's severity, the drug administration route is decided. In mild pain, oral administration of analgesics may help. Rapid hydration and monitoring vitals are very important. In serious conditions, patients are advised to get hospitalized and require supportive treatment.

Conclusion

In a sickle cell crisis, an affected individual should be aware of the possible condition to avoid any mishandling of the situation. However, sickle cell disease is genetically inherited and has no permanent cure, making day-to-day life challenging. The main rule for treating sickle cell crisis is pain control and hydration and avoiding the triggering factors. It is always advisable to consult an experienced hematologist for such blood-related disorders.