Hematologic Disorders in Children

Hematopoietic Stem Cell Transplantation

• Hematopoietic stem cell transplantation is the intravenous infusion of stem cells from a compatible donor to reestablish marrow function in a child with deficient or nonfunctioning bone marrow.
• Sources of stem cells include bone marrow obtained by marrow aspiration, peripheral blood, or umbilical cord blood.
• Goal is to reestablish marrow function and treat hematologic disorders.

Hematologic Disorders

• Assessment: skin pallor, petechiae, bruising, lethargy, frequent infections, bleeding that is difficult to control, and diagnostic tests (CBC, iron, coagulation, and immune system status).
• Nursing diagnoses: knowledge deficit, altered nutrition, anxiety, pain, and family coping.
• Interventions: individual distraction techniques, blood sampling, blood or marrow transfusion, education on nutrition, pain management, and family support.

Anemias

• Definition: a condition where the number of RBCs and/or hemoglobin concentration is reduced below normal level.
• Causes: reduced oxygen carrying capacity, tissue hypoxia, and diminished oxygen available to tissues.
• Formation: RBCs are formed in the bone marrow under the stimulation of erythropoietin, a hormone formed by the kidneys.

Bone Marrow Aspiration and Biopsy

• Uses: determines the type and quantity of cells produced in the bone marrow.
• Sites: iliac crests or spines in children (rather than the sternum, which is commonly used in adults).

Blood Transfusion

• Types: whole blood, packed RBCs, washed RBCs, plasma, plasma factors, platelets, WBCs, and albumin.
• Uses: treats blood disorders and may include transfusions of blood or its products.

Digestive System

• Main function: prepares foods for absorption through mechanical and chemical breakdown.
• Anatomy: mouth, pharynx, stomach, small intestines, liver, gallbladder, pancreas, and large intestine.
• Physiology: peristalsis, rhythmic waves of muscular contraction, and mechanical functions of digestion immature at birth.

Cleft Lip and Cleft Palate

• Definition: a congenital defect resulting from failure of embryonic structures of the face to unite.
• Incidence: 1 in 1000 births, commonly associated with chromosomal abnormalities.
• Effects: interferes with oxygenation and nutritional needs, hampers normal process of children with parents bonding.

Esophageal Atresia and Tracheoesophageal Fistula

• Definition: congenital defects, upper segment of the esophagus ends in a blind pouch, and defect in which embryonic structures fail to divide into a separate esophagus and trachea.
• Assessment: copious oral and nasal secretions, choking, coughing, and difficulty suctioning or gavage.
• Interventions: prevention of aspiration, drainage tube, esophageal atresia end-to-end anastomosis, and feeding tube insertion through gastrostomy.

Diaphragmatic Hernia

• Definition: congenital defect, diaphragm forms between the thoracic and abdominal cavities, leaving an opening through which the esophagus and great vessels pass.
• Effects: abdominal protrusion through the chest, compression of lungs, and reflux of stomach contents.
• Therapeutic management: surgery, as soon as diagnosis is made.

Gastroesophageal Reflux (GER)

• Definition: backflow of gastric contents into the esophagus due to relaxation or incompetence of the lower esophageal or cardiac sphincter.
• Association: hiatal (diaphragmatic) hernia.
• Interventions: surgical repair, upright position, and conservative management.

Inguinal and Femoral Hernia

• Definition: protrusion of peritoneum through the abdominal wall in the inguinal canal (inguinal hernia) and femoral canal (femoral hernia).
• Interventions: place client in head-elevated position following feedings and at night, and surgical repair.