The symptoms and signs of Sickle Cell Anemia Crisis are usually caused by exacerbation of the sickling effects of the red blood cells. Sickle cell anemia crisis is a manifestation of the deformation of the red blood cells leading to the different types of Sickle cell crisis. There are factors that trigger or precipitate sickle cell crisis and knowing these factors help to prevent occurrence of the crisis and hence prevent any complication that may arise. The management depends on the presentation and type of crisis.

Sickle cell anemia crisis does not occur in the first 6 months of life, which means that babies that have sickle cell anaemia and are less than 6 months old would not show the symptoms. The reason for this is because part of the fetal hemoglobin is still in circulation in much amount that it makes the manifestation of sickle cell crisis not possible. After the fetal hemoglobin becomes replaced by adult hemoglobin, which usually occurs after 6 months, only then will the sickle cell crisis begin. Some sickle cell crises are milder while that of others are severe. The more the amount of fetal hemoglobin the child has, the milder the sickle cell anemia crisis.

Sickle cell anemia vs Sickle cell crisis

In order not to be confused, it is good to differentiate between sickle cell anemia and sickle cell crisis. Sickle cell anemia is the disease itself while a crisis occur because of a trigger that causes the red blood cells change their shapes to that of a sickle, hence the name Sickle cells. When these red blood cells change to shapes like sickle, they occlude small blood vessels and the CRISIS is started. Sickle cell anemia crisis therefore is the worsening of symptoms as a result of the red cells becoming less malleable and bursting or destroyed easily while the sickle cell anemia is the disease that can cause the crisis.

Sickled red blood cells as seen in sickle cell crisis and also Normal red blood cells

Types of Sickle cell anemia crisis

1. Hand-foot syndrome
2. Vaso-occlusive crisis
3. Acute chest syndrome
4. Acute splenic sequestration crisis
5. Aplastic crisis
6. Hyperhemolytc crisis
7. Central Nervous system disorders
8. Susceptibility to Infections

Hand Foot syndrome

This is referred to as Painful dactylitis which is the painful swelling of back of hands and feet. This painful swelling of the hands and foot develop suddenly and may last for about a week or two. It normally starts in children from 6 months of age to 3 years and it is not seen when the child becomes older than age 5. This might be mistaken for osteomyelitis, which also presents with painful swelling of the hand and other parts of the body as a result of infections to the bones.

Acute Splenic sequestration crisis

This is a medical emergency and can lead to death unless prompt treatment is carried out. It is usually due to rapid enlargement of the spleen as a result trapping of circulating blood in the spleen. This causes drastic reduction in the blood that flows to other organs of the body. There will be life threatening anemia and high reticulocyte.Blood transfusion is necessary followed by removal of the spleen (splenectomy) in children that are older.

Aplastic crisis

This is caused by infection with Parvo virus B-19 and the same virus causes a disease known as the fifth disease characterized by fever, felling of being sick (malaise) and rash. Aplastic crisis causes impairment of the bone marrow to produce red blood cells. As a result of this, there is a rapid drop in Hemoglobin (the protein needed for transport of oxygen in the blood). The bone marrow usually recovers after about 7 to 10 days and normal red blood cell production continues.

Hyper-hemolytic crisis

This is triggered by infections. Over destruction of the red blood cells by the infection causes anemia (there would be reduced haematocrit) and jaundice (yellowish discoloration of the eyes and skin). The yellow eyes may deepen in colour if there was an existing jaundice prior to onset of the haemolytic crisis. There is usually Reticulocytosis (increased in the number of immature forms of red blood cells in circulation) as the bone marrow tries to produce red cells.

Vaso-occlusive crisis

This is the same as the hand foot syndrome that occurs in children less than 3 years but has different manifestations as the child grows. It is due to reduced supply of blood flow (ischemia) to organs and tissues of the body. The shortage in blood supply causes pain. It can involve the long bones of the body, the trunk and can affect the central nervous system and other systems of the body. Depending on the organ affected, it manifest with different symptoms.

Symptoms of Vaso occlusive crisis in Sickle cell anemia crisis

1. In the lungs, it causes acute chest syndrome
2. In the brain, it causes stroke
3. In the eyes, it causes retinopathy
4. In the bones, it causes bone necrosis
5. Nephropathy can occur in the kidneys
6. In the penis, it causes priapism. Priapism involves the persistent erection of the penis that is often painful and it is not associated with any sexual excitement.
7. Chronic leg ulcers can occur as a result of this

Acute chest syndrome

This may follow an infection, pulmonary infarction (obstruction of blood flow to an organ or tissue) or fat embolism resulting from bone marrow infarction. It usually starts with infarction of the ribs and atelectasis (lung collapse) may occur. It is an emergency that needs immediate treatment to prevent death.

The symptoms of acute chest syndrome in sickle cell anemia crisis

1. Chest pain
2. Fever
3. Cough
4. Respiratory distress (evidenced by flaring of the nose and use of accessory muscles of respiration)
5. Leucocytosis (increase in the number of white blood cell)
6. Pulmonary infiltrates are seen on chest x-ray

Central Nervous System involvement

Stroke is the commonest CNS manifestation that may occur as a result of sickle cell crisis. Weakness of one side of the body (Hemiparesis) usually occurs if the CNS is involved; convulsion may occur frequently and sometimes, infarction may occur without any obvious manifestation and cognitive function and behaviour may be affected (there might be learning difficulties).

Leg ulcers

Ulcers or wound may occur around the malleoli (prominences at the inner and outer aspects of the foot). This is due to poor circulation as sickling occurs. Infarction then follows. There is usually delay in healing.

Susceptibility to infections

Sickle cell anemia patients are susceptible to infections as a result of the inability of their spleen to function properly. There is a defect in their Properdin/Alternative pathway of the complement system. The complement system helps in elimination of harmful microorganisms in the body as it is an important part of the immune system. This defect in the complement system causes inability to deal with encapsulated microorganisms in the body leading to infections.

Treatment of a boy in sickle cell anemia crisis by administering oxygen when in respiratory distress

Management and treatment of sickle cell anemia crisis

1. Bed rest
2. Fluid therapy
3. Blood transfusion of packed cells when there is severe anemia
4. Analgesics to relief pain
5. Antibiotics to treat any infection
6. Surgery to remove spleen in order to prevent recurrence
7. Oxygen can be administered when there is respiratory distress

Prevention of sickle cell anemia crisis

1. Daily folic acid and vitamin C supplements
2. Daily Proguanil for malaria prophylaxis
3. Use of insecticides treated mosquito nets to prevent malaria infection
4. Prompt treatment of malaria and other infections
5. Vaccination against Hepatitis B, Hepatitis C, Pneumococcal bacteria and Haemophilus influenzae type b
6. Preventing precipitating factors that trigger sickle cell anemia crisis

Precipitating factors of Sickle Cell Anemia crisis

1. Avoid infections
2. Avoid exposure to cold
3. Avoid stress
4. Avoid becoming dehydrated. Take enough water
5. Avoid climbing or staying in high altitude areas
6. Sleep in a room with enough ventilation

These are the factors that trigger the sickle cell crisis. Avoiding these triggers has great benefits of reducing sickle cell anemia crisis.