• Vaso-occlusive – painful episodes: intense pain in any part of the body that can be concentrated on the limbs, chest, pelvis area, joints or it can be visceral. The pain is a result of the clogged sickled red blood cells in small cross-sectioned vessels. It is the most usual consequence of vaso-occlusive episodes and it has to be cured immediately with the proper pain treatment (during intense pain episodes induction/aggressive treatment is necessary with non-steroidal, opioid analgesics), thus, cause of damage and tissue necrosis are avoided.
•Acute chest syndrome: An acute pathological condition of the respiratory system that causes intense chest pain, possibly fever and/or respiratory symptoms: shortness of breath and hypoxia. Being one of the most dangerous conditions for the life of the sickle cell patient, it needs urgent medical assistance because it may also cause long-term consequences.
• Acute abdominal pain: abdominal pain due to caging situation caused by the lock-in of the red blood cells in the spleen or the liver. Splenic caging is an extremely urgent case that prompt action is required. Furthermore, the spleen usually due to multiple infracts, is shrinking and develops functional asplenia (inclination to infections is also one of its results). Many patients also develop liver disease and gallstones. In rare cases mesenteric vessel blockage can occur.
• Priapism: Painful erection of the penis due to corpus cavernosum sickling.
2. Chronic Painful Outbreaks
• Bone and joint diseases: bone lesions such as avascular necrosis of the femoral head of the hip joint or other joints like the shoulder, digitalis, osteomyelitis, osteoporosis, degenerative spinal conditions caused by biconcave deformation in the vertebrae episodes, large and small joint infarcts and arthritis. Patients are usually undergoing surgical replacement of their bone femoral head due to intense chronic pain and claudication. One of the main causes of chronic pain is the multiple vessel-caging episodes in the vertebrae and to other bones as well.
• Leg ulcers: Due to ischemia, ulcers are usually located in the lowest sections of the lower ends. They affect the adults more often and they also tend to be implicated with infections.
• Vascular diseases a) Eye lesions: incidental damages (central retinal artery occlusion, other vascular occlusions, optic nerve stroke) b) Acute hearing loss: due to vascular infarction c) Vascular encephalopathy: any cerebral vessel could be clogged up and present as a transient ischemic stroke, thrombotic episode or hemorrhagic stroke. Moreover, vascular damages may present as silent cerebral infarctions, stroke or transient ischemic attack, intracranial hemorrhage and advancing deduction of cerebral functions. Moreover, vascular damages may present as silent cerebral infarctions, stroke or transient ischemic attack, intracranial hemorrhage and advancing deduction of cerebral functions. Chronic headaches and migraines are reported with an increased frequency in sickle cell anemia patients.
Pulmonary vascular disease – Pulmonary hypertension: it’s a very common and serious complication (32% of the patients) that occurs with ageing.
a) Renal complications –
Kidney disease: Inability of condensation and tubular operation disruption are the most common effects. Also, two more kidney diseases related to sickle cell anemia are glomerulonephritis and papillary necrosis. Papillary necrosis usually develops macroscopic hematuria while glomerulonephritis develops varying types of hematuria and proteinuria. After all, a correlation between the sufferers and the carriers of the sickle cell anemia has been observed. Initially, mostly between African descendants but ultimately also between Caucasian – White descendants with the developing of Renal medullary carcinoma that affects mostly men below their 20s and has a bad outcome. Especially between Sickle cell disease carriers, those individuals are mostly involved in athletics. b)Chronic Liver disease – Sickle Cell liver: liver vaso-occlusive episodes (acute hepatic crises), acute intrahepatic cholestasis (which may manifest itself as fulminant liver failure), chronic intrahepatic cholestasis, liver caging episode, gallstones, cirrhosis (serious condition that usually requires liver transplant.) Additionally, infections derived from transfusions are noticed and also iron deposition in the liver (secondary hemochromatosis). Transfusions entail the risk of iron overload, too. This major problem may occur in the long-term and that’s why the patient needs to be monitored regularly and get the appropriate deferrisation treatment if needed. Formerly, transfusions entailed another high risk: hepatitis B, hepatitis C and HIV infection, however during the last few years, blood and blood derivatives are safer due to the implementation of new monitoring procedures. c)Chronic Cardiomyopathy: due to chronic myocardial ischemia and anemia mostly during adult life but also often as a result of iron deposition. Other heart complications of sickle cell disease may be: diastolic dysfunction of the left ventricle and irregular heartbeat.
• Organ failures: Endocrine disruptions: they may involve bone metabolism of: the thyroid gland, the pituitary and the gonads as well as various vitamin deficiencies such as D3 and calcium.
• Reproductive system disorders
• Anemia: is the interpretation of the chronic type of hemolysis, while the sudden decrease of the hemoglobin may be a result of a hemolytic episode (it may be caused by the destruction of the red blood cells mainly because of their morphology. It is common among a large number of sufferers.), either as an aplastic episode (the bone marrow stops producing platelets, red blood cells and white blood cells.), or because of destruction due to a hypersplenism case or blood loss (e.g. Splenic caging). In cases of extensive bone marrow necrosis besides aplastic anemia, intense bone pain, fever and absence of reticulocyte cells are noticed. In some cases, acute anemia is caused by hemolytic reactions due to transfusion in a small percentage of patients who receive them.
• Infections: patients are more likely to catch certain germs (pneumococcus, meningococcus, streptococcus, staphylococcus, mycoplasm, salmonella, hemophilus, cytomegalovirus, yersinia etc.) as a result of immune system disorder. There is feedback between infections, episodes, or other outbreaks of sickle cell disease which results in difficulty at the diagnostic approach. That’s why initially, fever during sickle cell anemia is considered as an infection.
• Psychological effects: social isolation, low self-esteem, sense of incompetence, defeatism, depression, distress, and insomnia are the main psychological consequences of the disease.
