Sickle Cell Disease

Sickle Cell Disease
Physical-Adult
• Eye problems. The retina, the "film" at the back of the eye that receives and processes visual images, can deteriorate when it does not get enough nourishment from circulating red blood cells. Damage to the retina can be serious enough to cause blindness.
• Jaundice, or yellowing of the skin, eyes, and mouth. Yellowing of skin and eyes. These are signs of jaundice, resulting from rapid breakdown of red blood cells.
• Delayed growth and puberty in children and often a slight build in adults. The slow rate of growth is caused by a shortage of red blood cells
• Pain crisis, or sickle crisis. This occurs when the flow of blood is blocked to an area because the sickled cells have become stuck in the blood vessel
• Anemia.
• Acute chest syndrome. This occurs when sickling is in the chest
• Increased infections
• Leg ulcers
• Bone damage
• Early gallstones
• Kidney damage and loss of body water in the urine
• Eye damage
• Multiple organ failure
Any and all major organs are affected by sickle cell disease. The liver, heart, kidneys, gallbladder, eyes, bones, and joints can suffer damage from the abnormal function of the sickle cells and their inability to flow through the small blood vessels correctly. Problems may include blindness or even death of the effected tissue with obstructed blood flow. Sickle Cell crisis occurs when flow of blood is blocked because a sickled cell has become stuck in a vessel.,

Mental-Emotional (adult)
No one should underestimate its mental and emotional impact. The patient endures not only the pain itself but also the emotional strain from unpredictable bouts of pain, fear of death, and social isolation at school and work. Both children and adults with sickle cell disease often suffer from depression. The financial costs of medical treatments combined with lost work can be very burdensome. Sickle cell patients and caregivers often face great obstacles in finding psychological support for the disease. Communities in which many sickle cell patients live generally lack services that can meet their needs. Computer on-line services are now valuable sources of support groups and access to research.

Anxiety Stress Reduction
Depression Cognitive-Behavioral Therapy.
Fear of Death On-Line Support Help
Isolation Support Associations

Sexual (adult)
Priapism. A painful obstruction of the penis by sickle cells. If not promptly treated, it can result in impotence. A reported 38 - 42% of males, including children, with sickle cell disease suffer from priapism. Priapism causes prolonged and painful erections that can last from several hours to days. If priapism is not treated, partial or complete erectile dysfunction can occur in 80% of cases. Sickle Cell has no effects to the female sex organs.
Pregnancy and Sickle Cell Disease
Women with sickle cell disease who become pregnant are at higher risk for complications such as miscarriage, premature birth, and low birth weight. Sickle cell disease symptoms often worsen during pregnancy and pain crises become more frequent. However, with careful prenatal care and monitoring, serious problems can be avoided. Maternal mortality rates have dropped significantly over the past decades. Most women with sickle cell disease can now anticipate favorable pregnancy outcomes
More than one third of pregnancies in women with sickle syndromes terminate in abortion, stillbirth, or neonatal death. Recently, a number of genes modifying the clinical severity of sickle cell anemia have been identified

Reference:
Genetic Disease Profile: Sickle Cell Anemia. http://www.ornl.gov/sci/techresources/Human_Genome/posters/chromosome/sca.shtml
Johns Hopkins Library- Sickle Cell Disease. http://www.hopkinsmedicine.org/healthlibrary/conditions/hematology_and_blood_disorders/sickle_cell_disease_85,P00101/
Source: http://www.umm.edu/patiented/articles/what_complications_of_sickle-cell_disease_their_treatments_000058_6.htm#ixzz2PkZiphyJ
Sickle cell anemia in the female patient. Obstet Gynecol Surv. 2006 May ;61(5):343-52.