Challenges of decision making for families of children with single or multiple chronic conditions.

When a child is diagnosed with a chronic illness, families begin a constant cycle of decision making. Many of these decisions surround health care treatment. These families can be divided into two groups: those with children newly diagnosed with a chronic condition and those with children who have a preexisting illness. If a new diagnosis represents a family's entrance into the medical community, the decision-making process can be confusing and difficult. For the family of a child with a preexisting condition, making repeated decisions about treatments can be additionally stressful and anxiety provoking because of their medical history. How do families with a child with a preexisting medical condition differ in their decision making? What factors affect their decisions? The nurse practitioner has a critical role interacting with both types of families and guiding the decision-making process. Therefore, understanding the family and their previous experience is crucial to determine how nurse practitioners can best tailor interventions to promote effective decision making.

Scoliosis in patients treated with growth hormone.

Two hundred fifty children being treated with growth hormone were screened for scoliosis by using the Adams and Bunnell techniques. If indicated, an anteroposterior radiograph was done and measured by the Cobb and Risser methods. Scoliosis was defined as a frontal curve of > or = 10 degrees; progression, as a sustained increase of > or = 5 degrees, and a progressive curve as one > or = 25 degrees and meeting our criteria for orthotic management. In 10 of the 250 patients, scoliosis developed. Six curves were double major thoracic and lumbar; three thoraco-lumbar; and one single thoracic. Six of the 10 patients had progressive curves and required an orthosis. Their average annualized rate of progression was 26 degrees. Progression was associated with double major curves and an earlier Risser stage. Despite bracing, progression continued to fusion in three patients. We conclude that growth hormone may increase the risk of progression of scoliosis. Furthermore, the progression is frequently rapid and requires special vigilance by the treating physician.

Arm span as measurement of response to growth hormone (GH) treatment in a group of children with meningomyelocele and GH deficiency.

Children with meningomyelocele (MMC) frequently have impaired linear growth. A number have associated structural brain defects with resultant GH deficiency (GHD). Reproducible measurements of height or length in MMC patients are often hampered by lower limb contractures, spasticity, and scoliosis. Arm span has been proposed as a more reproducible measure of linear growth. Five MMC children documented to have GHD were treated with recombinant human GH (hGH) for 1-3 yr. Their height, arm span, and growth velocity were compared with 32 children with idiopathic GHD treated similarly with hGH. These measures are compared with normal children by being expressed as standard deviation scores. The results of this study indicate that arm span measurements in GHD MMC patients are almost identical to height measurements in idiopathic GHD patients both before and during hGH therapy. The physical condition of children with MMC makes reproducible longitudinal height measurements difficult. Routine determinations of arm span measurements for children with MMC will assist in recognizing growth failure as well as monitoring treatment results.

The new highly sensitive adrenocorticotropin assay improves detection of patients with partial adrenocorticotropin deficiency in a short-term metyrapone test.

This study was designed to evaluate a short term metyrapone test using a highly sensitive (HS) IRMA ACTH assay and to evaluate the usefulness of a morning ACTH level as a screening test for partial ACTH deficiency. ACTH, 11-deoxycortisol and cortisol levels were evaluated over four hours in the morning after a single 40 mg/kg oral dose of metyrapone was administered at 0800 hours. 26 control children and 32 possibly pituitary deficient patients were evaluated. Based on 11-deoxycortisol levels alone, 17 of the patients passed the test, 11 patients failed the test and the result was inconclusive in four patients (12.5%). Evaluation of the increase in ACTH levels (delta ACTH) following metyrapone identified three of the above four with partial ACTH deficiency. The delta ACTH was consistent with the 11-deoxycortisol results in the remainder of patients. There was no difference in morning ACTH levels between controls and patients with partial ACTH deficiency. The measurement of ACTH using the HS IRMA assay, increases the sensitivity of the metyrapone test in detecting patients with partial ACTH deficiency. This test may be used safely in pediatric patients on a repetitive basis, especially in those children who may have progressive ACTH failure following hypothalamic-pituitary irradiation.

Growth hormone function and treatment following bone marrow transplant for neuroblastoma.

Previously, we reported that 26 children with stage III or IV neuroblastoma (NBL) treated with BMT grew poorly post-BMT and significantly worse than a comparison group of hematologic BMT patients. Furthermore, unlike the hematologic patients, there was no apparent catch-up growth. Six of these previously reported long-term (> 2 years) NBL patients surviving BMT were evaluated with growth hormone (GH) provocative testing, frequent (every 20 min) overnight GH sampling and IGF-1 determinations. Three of 6 patients were GH deficient based on subnormal responses to provocative stimuli and subnormal pooled 12 h GH values. Only one child had completely normal GH testing and his growth was normal. Four patients were tested with recombinant GH for a period of 12-21 months. Three patients demonstrated an improvement in their growth velocity on therapy. However, the overall response to GH treatment was significantly less than the growth response in children who are GH-deficient due to causes other than BMT. In summary, GH deficiency may be a frequent complication of BMT treatment of NBL. It also appears that the BMT treatment protocol employing total body irradiation and high-dose melphalan may induce GH resistance.