Infections

Infants and children with sickle cell disease are especially prone to bacterial infections, such as meningitis (infection of the lining of the brain) and blood infections. Such infections are the leading cause of death. They can be prevented by immunization and the use of prophylactic antibiotics, such as penicillin. Infection, including pneumonia, gallbladder inflammation (cholecystitis), bone infection (osteomyelitis), and urinary tract infection may occur.

 

Pain episodes

It is the most common symptom of sickle cell disease. Pain episodes occur when sickle cells clog small blood vessels depriving the body of adequate blood and oxygen. Pain frequently occurs in the arms, legs, chest, hips, and abdomen. Episodes vary in length and frequency for each person.

 

Acute Chest Syndrome

This is a potentially life-threatening condition that should be treated in a hospital. It is caused by an infection or blocked blood vessels in the lung. The condition is can present with symptoms such as difficulty breathing, chest pain, and fever. This condition can be so severe as to require respiratory support on a ventilator.

 

Anemia

Red blood cells containing hemoglobin S do not live as long as normal red blood cells, resulting in a low blood count. When this happens, the destroyed blood cells releases bilirubin into the body fluids can make the eyes look more yellow and the urine darker than usual. Less red blood cells results in less oxygen provided to tissues.

 

Hand-Foot Syndrome

Often the first symptom in affected babies and young infants. Hands and feet swell when blood vessels become blocked by sickle cells.

 

Jaundice

Yellowish color of the eyes. Although jaundice is usually not painful, it is a signal of an abnormal blood condition. It is caused by coloring material from red blood cell breakdown.

 

Stroke

Stroke occurs when sickle cells block blood vessels in the brain. Stroke may result in permanent disability or learning problems. Doctors are now able to identify most children who are at an increased risk of having a stroke with regular transcranial doppler ultrasound. Aggressive prophylactic intervention is provided to patients with the increased risk.

 

Slow Growth

Anemia may cause a child to have slow growth, delayed puberty, pale complexion, fatigue, and experience shortness of breath. Children with sickle cell disease may continue to grow after their other children their age have stopped. After awhile, they can reach the height that would be expected from the size of their parents.

 

Tissue damage

When sickle cells block small blood vessels, the amount of blood and in turn, oxygen received by certain parts of the body is reduced and will eventually suffer damage.

 

Vision Problems

In some cases, blindness, may occur when blood vessels in the eye become blocked by sickle cells.

 

Problems with the spleen

In some children, the spleen may become large very quickly and begin to trap lots of blood cells. This “trapping of blood cells” in the spleen can cause the blood count to drop drastically. This can lead to heart failure and death if not treated promptly with a blood transfusion. Rapid spleen enlargement may require emergent removal of spleen.

 

Leg ulcer

A breakage in the skin that begins as a small sore on the lower leg around the ankle. It can be caused by injury and decreased blood flow.

 

Gallstones

They are formed from the waste products of broken down red blood cells. These cells collect in the gallbladder and form thick sludge or stones. Gallstones are not harmful. But if they get stuck in the gallbladder duct, they can cause a severe pain and allow for serious infections. Many children with sickle cell disease have gallstones and gallbladder removal may be recommended.

 

Problems with kidneys and urine

The kidneys help the body hold a proper fluid balance. In sickle cell disease, the sickle cells damage the kidneys so that even young children have trouble holding onto their urine. To stay hydrated, children with sickle cell disease should drink more fluids because they pass urine more often than other children. When a child becomes sick and drinks less than usual or loses fluid by vomiting, diarrhea, or fever, he or she can get dehydrated. Dehydration makes the blood vessels smaller,which can lead to more clumping of the red blood cells. Frequent urination can also be seen by bedwetting. This is common in many children with sickle cell disease. Bedwetting is often not due to an underlying psychological problem.

 

Priapism

It is a serious problem in boys that can occur if sickle cells block the blood vessels in the penis. When this happens, the penis becomes erect, hard, and painful. This problem can be a medical emergency and can occur at any age.

 

Disclaimer The Hina Patel Foundation for Sickle Cell Disease website is designed for educational purposes only. The information provided should not be used for diagnosing or treating. Seek medical advice from a physician if you have any health problems.