Kidney Problems The main job of the kidneys is to filter waste out of the blood. More than 1 million tiny filters inside the kidneys, called nephrons , remove the waste. The collected waste combines with water, which also is filtered by the kidneys, to make urine. The urine leaves the kidneys and travels down the ureters into the bladder. When the bladder is full enough, the pressure causes a person to feel the need to go to the bathroom. Urine then leaves the body through the urethra. Sickle-shaped red blood cells can block the tiny vessels of the kidneys and damage the nephrons, causing the urine to be less concentrated than it should be. This results in an increased amount of urine and makes it very easy to become dehydrated. It also is the reason many children with sickle cell disease experience bed-wetting, known as nocturnal enuresis . These children can be treated the same as children who wet the bed and do not have sickle cell disease. Treatment for bed-wetting includes limiting fluids in the evening, using bed- wetting alarms, and, in some cases, taking medications. If the nephrons continue to be damaged by sickle cell disease, the kidneys may lose their ability to filter the blood. In the worst-case situations, kidney failure may occur. Kidney damage can be detected early with a simple urine test. The test checks the urine for a protein that the should have removed and for blood (hematuria). Urine sampled first thing in the morning gives the best indication of the kidneys’ health. A kidney specialist (nephrologist) may be consulted if there is blood or protein in the urine. Gallbladder Problems The gallbladder is an organ in the digestive system that stores bile, which is needed to help digest fats. Bile is made from bilirubin, a substance contained in red blood cells. When red blood cells break open, the bilirubin is released into the bloodstream. Because people with sickle cell disease have a high rate of hemolysis, they have excess bile that can cause gallstones to form in the gallbladder. Gallstones may cause sharp, sudden pains in the upper-right abdomen, which may worsen after eating fatty or spicy foods. These stones are diagnosed by an abdominal ultrasound. Sometimes the gallbladder can become inflamed, resulting in fever, pain, or both. When this happens, the gallbladder may need to be surgically removed. Eating a diet low in fat may make the attacks less painful. Joint Complications The blood supply to the body’s joints is limited. This is especially the case in ball-and-socket joints like those in the shoulders and hips. Avascular necrosis is a death of bone tissue that occurs in these joints after the blood flow is repeatedly blocked by sickle-shaped red blood cells. The femoral head, located at the top of the thigh bone, or femur, is at greatest risk for this problem. Avascular necrosis feels like arthritis pain, with stiffness and achiness, especially in the morning or after sitting for a while. If a child is still growing, physical therapy can be very helpful in healing the joint. For people who have finished growing, physical therapy can help relieve the pain and possibly delay the need for surgery. In some cases, joint- replacement surgery is needed.
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