If you suffer from sickle cell, then you would at one point in time in your life have been admitted to hospital. What a lot of people don’t know is that there are different kinds of crisis. Many people don’t know this and people living with sickle cell might not know either. Every crisis can be different in terms of pains and when one is asked questions such as ‘surely, you should be used to the pain?’ No, it is impossible to get used to the excruciating pain that is sickle cell and as you will read below, you will see that the pains are dissimilar. There are six different types of crisis that people with sickle cell have to endure. The most common is vaso-occlusive but let us read a bit more about the others.
Vaso-occlusive crisis is a painful sickle cell crisis (or vaso-occlusive crisis). It is the commonest manifestation of sickle cell disease and characterised by recurrent episodes of acute, severe pain from tissue ischemia as a result of vasco-occlusion. Sickle cell anemia patients often experience episodes of acute pain that are caused by vaso-occlusive crisis. Patients with Sickle cell anemia may experience VOC several times per year. Pain is unpredictable and frequently occurs spontaneously but may also occur after infection, exposure to cold, dehydration or in uncommon situations after emotional stress, exercise or alcohol, often no precipitation cause can be found, as triggers of VOC are not entirely understood.
VOC crisis is responsible for around 90 per cent of hospital admission and visits in patients with sickle cell disease. Legs, arms, back, chest and abdomen are often involved in VOC. VOC might cause leg ulcers, stroke, kidney insufficiency and spontaneous abortion.
Acute splenic sequestration is the enlargement of the spleen that can be life-threatening. There are two types of splenic sequestration in sickle cell disease (acute and chronic). Chronic splenic sequestration may not cause problems and the doctor may choose to record the size of the spleen at each visit to make sure it is not getting any larger. ASS can happen when sickled red blood cells get trapped in the spleen, causing the spleen to enlarge. Acute splenic sequestration crisis is a life-threatening complication associated with sickle cell disorder that consists of an acute fall in hemoglobin produced by red blood cell sickling within the spleen. It is also one of the leading causes of death in children with sickle cell disease.
The chances of having another episode of ASS are high. If the first episode was severed or if a second ASS occurs, the doctor may recommend splenectomy (removal of the spleen). A person can live without a spleen because other organs in the body perform the same function as the spleen. So, a person can have a healthy life without the spleen.
Infarctive crisis (obstruction of the blood supply to an organ) of the bone marrow leads to release of inflammatory meditators which activate afferent nerve fibres resulting in severe generalised bone pain. Almost any bone may be involved and there is a tendency for the infarcts to become infected. Sometimes infection may occur.
Aplastic crisis is when the body does not make enough new red blood cells to replace the ones that are already in the blood. Normally, the soft tissue at the centre of your bones called bone marrow constantly makes new RBCs. These cells go into your blood supply and carry oxygen through the body. If you have SCD, your body needs to make a lot of new RBCs but during AC, the bone marrow stops making RBCs for a short time. This is called transient (temporary) AC. It normally lasts for 7-10 days; after which the bone marrow starts making RBCs again.
AC is usually caused by a virus. Parvovirus B19 is one of the most common causes. The virus causes the bone marrow to stop making a new RBCs for 7-10 days.
Haemolytic crisis is an acute drop in hemoglobin level marks this crisis. It is common in patients with co-existent G6PD deficiency. HC is the rupturing of RBCs and the release of their contents into surround fluid (e.g. blood plasma). One cause of hemolysis is the action of hemolysins, toxins that are produced by certain pathogenic bacteria or fungi. Another cause is intense physical exercise. Hemolysins damage the RBCs. HC is an acute accelerated drops in hemoglobin level. The RBCs break down at a faster rate.
Bone crisis is caused by bone marrow ischemia or infarction. These crises usually start after age 2-3 years and occur as gnawing, progressive pain, most commonly in the humerus, tibia, and femur and less commonly in the facial bones. Periarticular pain and joint effusion, often associated with a sickle cell crisis, are considered a result of ischemia and infarction of the synovium and adjacent bone and bone marrow.
Patients with acute bone pain crisis usually present with fever, leukocytosis, and warmth and tenderness around the affected joints. This process tends to affect the knees and elbows, mimicking rheumatic fever and septic arthritis.
Bone involvement is the commonest clinical manifestation of sickle cell disease both in the acute setting such as painful vaso-occlusive crises, and as a source of chronic, progressive disability such as avascular necrosis and the long-term effects of sickle cell disease on bone mineral density, growth and chronic bone and joint damage.
Individuals with sickle cell disease are functionally asplenic and are at risk for infections that may be life-threatening, and other bone and joint complications can cause severe pain and immobility that significantly interfere with functioning and quality of life.
If you would like to know more about types of sickle cell crises, my book, “HOW TO LIVE WITH SICKE CELL,’’ is now available on Okadabooks and on Amazon. If you would also like to get in touch with me about this column or about sickle cell, do contact me on: [email protected] and do visit my column: http://www.howtolivewithsicklecell.co.uk
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