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Older Adolescent with Cystic Fibrosis: Transitioning to Adult Care.

Case Presentation

John is a 20-year-old Caucasian male with a diagnosis of cystic fibrosis (CF) who presents to his pediatric CF center for his annual visit. His last appointment with the CF center was nine months ago when he required hospitalization for pulmonary exacerbation. He was a "no-show" for his last two appointments (three and six months ago).

During the initial discussion with John, the nurse practitioner noted that John appeared tired and made limited eye contact with her. He did not smile much during their interaction, which is unusual for John, who has a history of playing practical jokes on members of the pulmonary team. When John was asked by the nurse practitioner how things were going at home, he replied, "It's all good; I am just tired of having CF mess up my life so much."


Past medical history. John had difficulty gaining weight as a newborn and was diagnosed with CF at 6 months. Genetic testing indicated that he had a homozygous F508del mutation. He experienced a relatively healthy childhood, with no hospitalizations for CF until he was 3 years old. He is knowledgeable about his treatments, including his medications, therapeutic diet (high calorie, high protein, and high fat), and chest physiotherapy procedures. In middle school, he had some negative peer encounters that resulted in him not taking his pancreatic enzymes during lunch, skipping his afternoon chest physiotherapy, and not adhering to his routine home medications, including oral antibiotics. This noncompliance resulted in him experiencing some decrease in his pulmonary functions and increased incidences of lung infections, which required hospitalizations several times each year. A sputum culture revealed colonization with Pseudomonas aeruginosa and Burkholderia cepaciaabout two years ago.

Growth and development history. John was born at 35 weeks gestation via cesarean section due to prolonged heart decelerations. His mother exclusively breastfed him for the first two weeks of his life. Weight loss was noted at his two-week checkup, so his pediatrician ordered high-calorie formula, which improved weight gain; however, he remained in the 30th percentile for height and weight throughout his adolescence. Due to hospitalizations in his teen years, he missed many peer functions, including band concerts and competitions (he played drums in the band), school dances, and football games, as well as other social functions. At age 18 years, he had a semen analysis that showed he is infertile due to congenital absence of the vas deferens. He was counseled on implications for family planning and genetic transmission of CF. John recently started a part-time job at a local bookstore and would like to get his own apartment with a friend in the future. He has enrolled part-time in a local university and is taking pre-engineering courses.

Family and social history. John is the second of three children. His older brother, who also had CF, died from complications following a double lung transplant 10 months ago. His younger sister is in middle school and does not have CF. His parents are divorced, and John currently resides in a home with his mother and sister. No other paternal or maternal relatives are known to have CF, although a great aunt passed away as an infant with an undiagnosed respiratory illness.

Recently, two friends with CF were placed on the transplant list for double lung transplants. John is concerned about losing them to similar complications that his brother experienced. His pulmonologist has discussed the need for him to transition his care to the adult hospital within the next year; he becomes very upset when this subject is broached.

When John was in high school, he drank beer with friends on weekends and occasionally smoked cigarettes. He denied use of any illegal or street drugs. He currently denies tobacco use but reports drinking one to two beers each evening.

Nutritional history. John eats two meals per day, with an occasional snack. He takes his pancreatic enzymes "occasionally, when I remember them." John reports eating a bowl of cereal with whole milk for breakfast and tuna or roast beef sandwiches on wheat bread for dinner. He usually skips lunch because he is "not hungry." He enjoys potato chips for a snack when playing video games. The nurse practitioner has made John aware of the need to work on maintaining his weight (125 to 158 lbs), to meet his body's nutritional needs.

Physical Assessment Findings

General: 20-year-old Caucasian male, alert and cooperative. Adequately groomed, appears thin and under-nourished, makes minimal eye contact and speaks quietly. Forced expiratory volume in 1 second (FEV1) 60% predicted. Ht. 1.72 m; Wt. 52 kg (115lbs); BMI=17.2.

Vital signs: Temperature=99.0 degrees Fahrenheit; Pulse=88/min; Respiratory Rates=28/min; Blood Pressure=130/68 mmHg.

Skin, hair, and nails: Skin pale and warm to touch. No lesions noted. Implanted port noted in left upper chest, not currently accessed. Hair brown, thin, and smooth in texture. No alopecia noted. Nail clubbing with fingers and toes. Cap refill 3 seconds, no cyanosis.

Head and neck: Head midline and erect. Skull normocephalic, no lesions. Facial features symmetrical. Neck symmetrical with trachea midline. No webbing, skin folds, or masses noted. No JVD distention or carotid artery prominence. Thyroid palpable, firm, non-enlarged, no nodules. Full ROM of neck without discomfort.

Eyes: Conjunctiva pink, sclera china white. Irises green, pupils 3 mm, PERRLA. EOMs intact, without nystagmus. Fundoscopic exam reveals red reflex bilaterally. Optic discs with well-defined margins, no hemorrhage or papilledema.

Ears: External ears without lesions, deformities, or drainage. Auricles symmetrical and aligned. Tympanic membranes pink, intact, with cone of light and bony landmarks visualized. Moves with insufflation. No effusion or retraction. Conversational hearing and speech appropriate. Responds to whispered voice.

Nose and sinuses: Symmetrical without deformities, tenderness, or discharge. Nares patent bilaterally. Two large polyps noted in right nare, none in left nare. Mucosa pink and moist. Sinuses non-tender.

Mouth and throat: Lips pink, no lesions. Buccal mucosa pink and moist. Teeth yellow, in various states of repair. No halitosis. Tongue and uvula pink and midline. Hard and soft palate without deformities. Tonsils 1+ without erythema, exudate, or discomfort. Swallows without difficulty.

Lymphatics: Lymph nodes non-palpable and non-tender.

Respiratory: Barrel chest. AP=transverse diameter. Chest expands symmetrically with respiration. Wet, productive coughs with deep breaths. Sputum thick, yellow, moderate amount. Intercostal retractions noted. Expiratory wheeze and crackles noted in right and left upper lobes. Hyperresonant sounds during percussion throughout lung fields.

Cardiovascular: Precordium without visible or palpable pulsations, thrills, or lifts. Neck veins non-distended. Peripheral pulses 2+. Apical pulse at 5th ICS in the MCL. S1 and S2 crisp. No S3, S4, murmur, click, or rub.

Gastrointestinal: Abdomen concave, soft, non-tender, without areas of visible peristalsis or pulsations. No visible masses or hernias. Well-healed scar from previous surgery noted in right lower quadrant. Hyperactive BS x 4 quadrants. Liver, spleen, and kidneys not palpable. No palpable masses.

Genitourinary: Tanner V. Circumcised penis. Testes descended without palpable masses or nodules. No discharge from urethral meatus. No swelling or tenderness. No CVA tenderness.

Musculoskeletal: Posture erect and spine straight without obvious deformities. Muscles and extremities symmetrical to inspection and palpation, full strength and ROM.

Neurological: CN II-XII grossly intact. Gait steady. Rapid alternating movements coordinated and smooth. Superficial touch, pain and vibratory sensation intact. DTRs 2+.

Tedra S. Smith, DNP, CRNP, PNP-PC

Laura B. Debiasi, Lynn Stover Nichols, and Sigrid Ladores

Laura B. Debiasi, DNP, MPH, CRNP, FNP-C, CNE, is an Assistant Professor, the University of Alabama at Birmingham, School of Nursing, Birmingham, AL.

Lynn Stover Nichols, PhD, RN, BC, SANE, is an Associate Professor and Interim Assistant Dean for Undergraduate and Prelicensure Programs, the University of Alabama at Birmingham, School of Nursing, Birmingham, AL.

Sigrid Ladores, PhD, RN, PNP, CNE, is an Assistant Professor, the University of Alabama at Birmingham, School of Nursing, Birmingham, AL.

Critical Thinking Case Studies is designed to test your problem-solving and decision-making abilities.

Instructions: Read the symptom(s) above. Then, outline how you would assess and manage the problem. Finally, compare your rationale and decision to that listed in the shaded area.

If you are interested in author guidelines for this column or would like to submit material, contact: Tedra S. Smith, DNP, CRNP, PNP-PC; Pediatric Nursing; East Holly Avenue/Box 56; Pitman, NJ 08071-0056;
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Title Annotation:Critical Thinking Case Studies
Author:Smith, Tedra S.; Debiasi, Laura B.; Nichols, Lynn Stover; Ladores, Sigrid
Publication:Pediatric Nursing
Article Type:Clinical report
Geographic Code:1USA
Date:Mar 1, 2018
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