440 Unit 4 | Common Illnesses or Disorders in Childhood and Home Care
● Encourage the parents to seek emotional support from CAH support groups in their area or through a national organization.
Assessment Assessment for CAH includes the following measures. Clinical Presentation ● Males typically do not experience manifestations until later in childhood. These symptoms include early development of pubic hair, enlargement of the penis, or both; advancement in growth velocity; and advanced bone age compared with the child’s chronological age. ● In females, CAH manifests at birth with abnormal develop- ment of the genitalia called ambiguous genitalia. The clitoris may be enlarged and labial folds may be fused, giving the appearance that the female is male; however, the internal sex organs will be normal. ● Virilization (development of secondary sexual characteristics) occurs in females. Diagnostic Testing ● Newborn screening; however, the screening may show “false- positive” results. If positive, further testing may be needed (Held, Bird, & Heather, 2020). ● Serum 17-OHP is elevated. If levels remain elevated after birth, CAH is expected, because normal growth and development decrease 17-OHP as the infant matures (Prete et al, 2019). ● Chromosomal analysis will be performed to determine the infant’s gender in the case of ambiguous genitalia. ● In some cases, genitalia surgery is performed to meet the needs of the child’s determined sex. Nursing Interventions ● Early diagnosis is the key to successful treatment. The nurse must be able to carefully assess for prenatal and postnatal risk factors. ● Referral to a genetics clinic may be indicated. ● Dehydration is a complication of CAH, and the nurse must assess for signs and symptoms of dehydration, electrolyte imbalance, and hypovolemic shock. ● Careful monitoring of cardiopulmonary status is necessary. ● Frequently assess vital signs. ● Assess the parents’ emotional status related to the ambiguity of the infant’s genitalia. Understanding cultural and spiritual beliefs when counseling parents is important. ● Provide detailed education regarding the cause of the diagno- sis, symptoms, and available treatment options. ● If the infant’s gender is questionable at birth, refer to the infant as “your baby” and not “your son” or “your daughter.” Caregiver Education ● Emergency care involves teaching the parents and family how to administer medications. ● Teaching the family the signs and symptoms of adrenal crisis and the performance of stress dosing is crucial. ● Instruct the parents regarding the signs and symptoms of dehydration.
CRITICAL COMPONENT Administering Corticosteroids and Mineralocorticoids
Emergency care involves education regarding the administration of lifelong medications such as corticosteroids and mineralocor- ticoids. Emergency administration of corticosteroids given via injection should be taught to the parents for use when the child is in a crisis, such as febrile illness, surgery, trauma, or severe stress. The doses will need to be doubled or tripled during the period of crisis. This is referred to as "stress dosing." The most common medications administered are hydrocortisone and fludrocortisone (Florinef) (Prete et al, 2019). ● Educate the parents regarding future pregnancies and the importance of prenatal screening for CAH and refer them to a genetics clinic. ● Allow parents and family members to discuss concerns, feel- ings, and beliefs about their infant’s condition. Refer them to the appropriate center for psychological support; involve the social work team and offer spiritual support such as the clergy or designated chaplain for their faith. ● If surgery is indicated, provide education regarding preop- erative and postoperative care, and let the parents know that there may be more than one surgery to correct the genitalia.
ADRENAL INSUFFICIENCY ADDISON’S DISEASE
Insufficient cortisol and aldosterone production from adrenal glands leads to adrenal insufficiency. Usually, the cause is un- known. Children with adrenal insufficiency may experience hypoglycemia, especially during stressful times such as surgery or febrile illness. Symptoms are very individualized and may be mild unless the child gets sick. Assessment Assessment for Addison’s disease should include the following measures. Clinical Presentation ● Manifestations include but are not limited to weakness, fatigue, dizziness, and rapid pulse; dark skin that appears as if the child is very tanned; black freckles; bluish-black discolora- tion around the nipples, scrotum, and vagina; and weight loss, dehydration, loss of appetite, intense salt cravings, nausea and vomiting, and cold intolerance. Diagnostic Testing ● Low serum sodium, high potassium, and low blood sugar ● Diagnosed by assessing manifestations
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