A Closer Look at Chronic Respiratory Disease States and Genetic Anomaly

1. Baby “A” A Closer Look at Chronic Respiratory Disease States and Genetic Anomaly

2. Health History • Birth Hx: born at 381/7 weeks via emergency C-section due to pre-eclampsia • Past Medical Hx: multiple past respiratory-related hospital admissions, respiratory failure 2oparaflu (hPIV), laringomalacia resolved by supraglottoplasty • Family Hx: 11q13.3 deletion on father’s side, has 2 brothers and one of them with developmental disabilities

3. Culture • No extraordinary considerations • Even though mom has two other boys to take care of, one with developmental issues as well, she is consistent in coming to the hospital right after she drops them off at school and cares for her daughter. It seems like this has become a normal part of life for her, and she manages to maintain a positive attitude through it all. Another month-long hospital trip has become routine for both.

4. HPI/Admission • A is a 10 month old infant who presented to the ER on 9/3/13 with “cold symptoms” since that Sunday in the setting of “wet lungs for weeks,” as stated by her mother. Acutely, she had been having a constant productive cough, runny nose & eyes, elevated WOB, and a fever of 101.5o F. Upon admission, her symptoms had worsened and she was unable to sleep.

5. Viral URI (Upper Respiratory Infection) MEDICAL DIAGNOSES Chronic Lung Disease

6. Patho & Treatment of URI • URI is also known as acute viral nasopharyngitis or the “common cold.” Though it would not pose a significant threat to a healthy person, in A’s case her already compromised lung function led to a month-long stay in the hospital. • Symptoms of nasopharyngitis are more severe in infants and children than in adults. Fever is common, especially in young children. Fevers can occur suddenly and are associated with irritability, restlessness, decreased appetite and fluid intake, and decreased activity. Vomiting and diarrhea may also be present. • Symptoms are mostly managed without any specific treatment or medication. Some might receive antipyretics, decongestants, cough suppressants, and plenty of rest is recommended for all. (Hockenberry & Wilson, 2011)

7. Pathoof CLD • CLD is also known as bronchopulmonary dysplasia (BPD). It develops primarily in ELBW and VLBW infants with RDS. BPD may also develop in infants with MAS, persistent pulmonary hypertension, pneumonia, and cyanotic heart disease. Infants who develop BPD are at risk for frequent hospitalization because of their borderline respiratory reserve, hyperactive airway, and increased susceptibility to respiratory infection. • Risk factors for BPD include assisted ventilation, oxygen administration, prenatal and postnatal infections, PDA, and fluid imbalance. • BPD begins with the immature lung that undergoes and initial injury leading to a chronic inflammatory process that results in recurrent injury and abnormal healing. • Growth and development are often delayed, which is related to difficulties in providing adequate nutrition and in part to the lack of normal sensory stimulation because of prolonged hospitalization.

8. Treatment of CLD No specific treatment Maintain adequate arterial blood gases with the administration of oxygen Stop progression of disease Corticosteroids are beneficial with BPD by decreasing pulmonary inflammatory response and improving oxygenation and gas exchange, though they are still considered a controversial therapy** Weaning infants from oxygen is difficult and must be accomplished gradually. These infants do not tolerate excessive or even normal amounts of fluid well and have a tendency to accumulate interstitial fluid in the lungs, which aggravates the condition. Oral diuretics are used to control interstitial fluid.** Nebulized or metered dose inhaler bronchodilaters (albuterol) and inhaled steroids may be effective and promote improvement in infants with BPD Oral electrolyte supplements are given to replace those lost with concurrent oral diuretics and renal water losses Metabolic needs are far greater than that of average infant, which are further compromised by GERD, a common complication of CLD – adequate intake of protein is particularly important (Hockenberry & Wilson, 2011)

9. Applicable Research • The use of postnatal steroids (PNS) for the treatment and prevention of CLD/BPD, is very controversial in the field of neonatology. Although PNS was once considered the mainstay for managing CLD, reports of adverse neurodevelopmental outcomes dramatically changed clinical practice. • Research demonstrating the efficacy and safety of PNS use remains limited. • No proven safe and effective therapies available for the prevention and treatment of BPD. • Follow-up data on adverse effects of dexamethasone are mixed: • Several studies found a higher incidence of adverse effects such as decreased growth parameters and increased neurodevelopmental impairment. • Some studies suggest that the benefits of PNS use may outweigh potential adverse effects in certain patient populations. For infants at higher risk of developing BPD, corticosteroid treatment reduced the risk of death or CP. (Forest, 2011)

10. Applicable Research • Diuretics are used in preterm infants to treat the symptoms of bronchopulmonary dysplasia (BPD), although there is little evidence of their effectiveness in improving long-term outcomes. Prescribing patterns and frequency of diuretic use in patients with BPD are unknown. Long-term diuretic administration to patients with BPD is commonly practiced despite minimal evidence regarding effectiveness and safety. • “Variation in the Use of Diuretic Therapy for Infants With BronchopulmonaryDysplasia”: collected data from 85% of all freestanding children’s hospitals in US in an effort to understand better the national usage of diuretics as a therapy for BPD/CLD. (Remember: there is no specific therapy for CLD) • A total of 1429 infants met the criteria for BPD at age 28 days, of which 1222 (86%) were treated with at least 1 diuretic dose. Patients received a median of 9 days (25th–75th percentile: 2–33 days) of diuretic therapy. • The range of infants receiving a diuretic course of >5 days duration varied by hospital from 4% to 86%, with wide between- hospital variation even after adjustment for confounding variables. (Slaughter, Stenger& Reagan, 2013)

11. Chromosomal Deletion 22q13.3 Secondary Diagnoses GERD Systemic hypertension Phelan-McDermid syndrome (22q13.3 deletion syndrome) is characterized by neonatal hypotonia, global developmental delay, absent to severely delayed speech, and normal to accelerated growth. Most individuals have moderate to profound intellectual disability(Phelan & Rogers, 2005).

12. Development • 10 month old A is in Erikson’s “trust vs mistrust” stage of psychosocial development. She is for the most part dependent on us nurses for her care, especially with her developmental delays, all she is able to do at this point is roll over on her own and pull her head up when on her stomach. Her development is really equal to that of a 6 month old. • For assessment purposes, the nurses were quiet and calm when speaking, gently yet firm in handling, doing the quiet procedures first and saving for last that which might irritate or upset her. When she was awake and alert, they would talk to and play with her. The nurse also involved mom in the care whenever possible, because this infant had already learned to trust mom and was more responsive to her.

13. Assessment • Neuro Developmentally delayed: cannot crawl, sit up, or stand. Exhibits significant cognitive delay. • CV PICC in right upper arm, both parts hep locked • Resp Coarse breath sounds bilaterally, slight retractions substernally. • GI JG tube, G tube to ferrell bag, J tube infusing cont feeds of Elocare 30 kcal @ 24 ml/hr • Skin Site around JG tube slightly reddened, mesh vest around abdomen • M/S Motor skills delayed.

14. Ineffective Breathing Pattern R/T chronic diseased lung state, inflammatory process Evidenced by: productive cough, resp depth changes, slight retractions Baseline O2 is ½ L continuous Meds: Albuterol, Pulmicort Ineffective Airway Clearance R/T infectious disease process, tracheobronchial secretions, mucus in nasal passages Evidenced by: cough, changes in depth of resp, runny nose, coarse lung sounds A.A. - 10 mo Viral URI Chronic Lung Disease Chromosomal deletion Risk for Caregiver Role Strain R/T significant home care needs for 2 out of 3 children, mom says they lose a home nurse every time A ends up in the hospital, frequent hospitalizations that last for weeks-months Risk for Infection R/T pre-existing infection, compromised immunity, PICC line, GJ tube, constant presence of secretions Contact/droplet precautions Delayed Growth & Development R/T genetic abnormality, chronic disease state, frequent hospitalizations Evidenced by: below 2% on height and weight growth charts, unable to meet developmental milestones for 10 months

15. Ineffective Breathing Pattern Pt will maintain effective breathing pattern and good O2sats (92-100%). Ineffective Airway Clearance Pt’s airway will be maintained free of secretions. Expected Outcomes Risk for Caregiver Role Strain Caregiver demonstrates competence and confidence in performing the caregiver role. Risk for Infection Pt remains free of additional infections during hospital stay. Delayed Growth & Development Pt will participate in developmental stimulation program to increase skill levels.

16. Nursing Interventions • This little girl has a lot going on, but the primary focus in her care is respiratory. The fact that she has chronic lung disease and is recovering from an upper respiratory infection puts her at risk for a significant amount of respiratory problems, specifically buildup of secretions and possible progression to pneumonia. She is at risk for other infections, aspiration, fluid overload (she gets crackles at the slightest bit of volume increase), and her oxygen sats depend on the ½ L of O2 going through her nasal cannula, which she keeps pulling off. Therefore, my primary nursing diagnoses were centered on providing nursing interventions for bettering of her respiratory status.

17. Nursing Interventions • Pt was assessed for respiratory rate & depth, patent airway, breathing patterns, breath sounds, nutritional status, height & weight, family resources, and available support system • Continuous pulse ox monitoring • HOB was kept elevated to 30o • Secretions in airway were suctioned regularly • Contact/droplet precautions were maintained throughout hospital stay • Occupational therapy collaborated with nurses to provide activities that help develop fine/gross motor skills, improve coordination and strength • Nurses would sit A up and continuously talk to her whenever they came in to assess • Relationship with caregiver was established

18. Teaching Needs • A’s mother was very informed about her condition since A had been in the hospital so many times previously with the same complications and they have been through discharge procedures multiple times. I did remind mom to surround herself with a support system so that she could get a break from time to time and focus on keeping her own health from deteriorating as well.

19. ? ? ? ? Any Questions?

20. References Hockenberry, M. J., & Wilson, D. (2011). Wong's essentials of pediatric nursing (9th ed.). St. Louis, MO: Mosby/Elsevier. Forest, S. (2011). Postnatal steroids for the treatment of bronchopulmonary dysplasia: a complex case presentation. Journal of Perinatal & Neonatal Nursing, 25(3), 292-293. Phelan, K. & Rogers, C. (2005). Phelan McDermid Syndrome. GeneReviews. Retrieved from http://www.ncbi.nlm.nih.gov/books/NBK1198/ Slaughter, J. L., Stenger, M. R., & Reagan, P. B. (2013). Variation in the Use of Diuretic Therapy for Infants With Bronchopulmonary Dysplasia. Pediatrics, 131(4), 716-723. doi:10.1542/peds.2012-1835