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Children's Medical Services - Special services for children with special needs
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Infant Toddler Development Training
Module 6, Lesson 5

Miscellaneous Medical Conditions

Sickle Cell Disease

Sickle Cell Disease is the most common genetic cause of a structural abnormality of hemoglobin, producing anemia in people of African and Caribbean descent. The disease causes an amino acid substitution during synthesis of food thus resulting in abnormal hemoglobin in the blood stream.

baby sleepingAlthough newborns with sickle cell disease appear to be normal at birth, by 4 to 6 months of age, the infants become anemic and some of their red blood cells appear to have a sickle shape rather than the nicely rounded edges of normal red blood cells. The sickled cells can cause vasoocclusion (blockages in the blood vessels). The infants show signs of swelling in their digits (fingers and toes) that is quite painful, and enlarged organs (organomegaly). These young children are at risk for overwhelming infections but, when detected, can be protected against certain bacterial organisms with antibiotic prophylaxis, and vaccination.

The vasoocclusion can cause local tissue hypoxia (lack of oxygen), and at times ischemia (disruption of blood flow in any area of the body that can lead to tissue death) and infarction (rupture of a blood vessel that prevents blood flow and can lead to tissue death). Infarction produces pain in the form of vasoocclusive crises, especially in the extremities. A person who has sickle cell disease is prone to leg ulcers later in life. The kidneys can have infarction resulting in blood in the urine. An infarction in the brain may result in a stroke. This is a devastating complication for children. Retinopathy with resulting visual loss can occur; though this is rare in young children.

An occlusion may occur in the gastro-intestinal tract and cause severe pain. An excessive breakdown of the red blood cells leads to "bilirubin" gallstones. This condition contributes to hepatomegaly (enlarged liver) and also enlarged spleen. The liver is also enlarged because more red cells are produced.

baby with tongue sticking outThe spleen becomes enlarged early in the disease, but as time goes on, the spleen quits functioning due to infarctions and shrinks. With a nonfunctioning spleen, the child becomes very susceptible to bacterial infections that are very resistant to antibiotics. Very rarely, the spleen will fill itself with a large quantity of circulating blood in a condition known as a "sequestration" crisis. This results in shock and the child requires immediate resuscitation.

Usually, the sickle cell disease is detected with newborn screening for abnormal hemoglobin. Early identification assists in the early planning for medical management and genetic counseling.

Clotting Disorders

Severe problems with bleeding do not occur with every child with a clotting disorder. However, in the most widely known clotting disorder, hemophilia A (Factor VIII deficiency), bleeding can occur several times a month, and require visits to the hospital or acute care clinic for clotting factor therapy.

A major issue for a child who has a clotting disorder is trauma. Trauma can produce major bleeding into the joints (hemarthroses). When the blood is reabsorbed, the joint lining is broken down and this leads to joint degeneration. Movement becomes painful and limited. Rehabilitation is uncomfortable, but necessary to prevent further debilitation.

Bleeds into the brain can destroy both gray and white matter. There is an increased risk of seizures leading to an increased incidence of learning difficulties.

The use of blood derived products for the treatment of hemophilia brings with it the risk of infections such as hepatitis B, hepatitis C, and Human Immuneodeficiency Virus (HIV). This is a major concern for both parents and other caregivers.

father putting child's coat onParents and caregivers must be constantly vigilant to safeguard the young child from injury. It is hard for parents to allow their children with hemophilia to be independent. Parents and caregivers must be aware that the use of corporal punishment or even grabbing a child's hand can result in a bleed.

Consider how the ITDS can help the family of a child with a clotting disorder. Name at least two ways.

 

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