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Guidelines for Management of Infants with CF

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Guidelines for Management of Infants with CF Cystic Fibrosis Foundation Evidence-Based Guidelines for Management of Infants with Cystic Fibrosis Drucy Borowitz, MD, Karen A. Robinson, PhD, Margaret Rosenfeld, MD, MPH, Stephanie D. Davis, MD, Kathryn A. Sabadosa, MPH, Stephanie L. Spear, PhD, Suzanne H. Michel, MPH, RD, LDN, Richard B. Parad, MD, MPH, Terry B. White, PhD, Philip M. Farrell, MD, PhD, Bruce C. Marshall, MD, and Frank J. Accurso, MD Newborn screening for cystic fibrosis (CF) offers the opportunity for early medical and nutritional intervention that can lead to improved outcomes. Management of the asymptomatic infant diagnosed with CF through newborn screening, prenatal diagnosis, or sibling screening is different from treatment of the symptomatically diagnosed in- dividual. The focus of management is on maintaining health by preventing nutritional and respiratory complications. The CF Foundation convened a committee to develop recommendations based on a systematic review of the ev- idence and expert opinion. These guidelines encompass monitoring and treatment recommendations for infants diagnosed with CF and are intended to help guide families, primary care providers, and specialty care centers in the care of infants with CF. (J Pediatr 2009;155:S73-93). ymptomatic diagnosis of cystic fibrosis (CF) is associated with short- and long-term complications including failure to thrive, stunting, wasting, vitamin and mineral deficiencies, recurrent pulmonary infections associated with decreased Slung function, and recurrent hospitalizations. Early identification of CF by newborn screening (NBS), prenatal diagnosis, or family history offers the opportunity to delay and potentially prevent many of these complications through early treatment. Although many treatment issues are the same for individuals with CF regardless of when they are diagnosed, some are unique to the population of newborn infants who may not have overt symptoms of the disease before referral to the CF care center. The CF Foundation convened a committee of experts to develop guidelines for care based on currently available evidence for this distinct population of newly diagnosed infants. Methods The CF Foundation convened a group of experts to identify issues in the care of infants with CF and commissioned an evidence review from Johns Hopkins University. Details of this evidence review, including methods and results, are provided in the ac- companying article.1 Committee members assessed this evidence in developing recommendation statements and, where pos- sible, made evidence-based recommendations. Recommendations were graded using the United States Preventive Health Services Task Force (USPSTF) grading system.2 Recommendations from published guidelines were used if available and appro- priate. The committee made consensus recommendations for topics not included in the evidence review, for topics where prior guidelines were available, and for topics for which there was limited or no evidence but the potential net benefit was assessed as at least moderate (Table I). A draft of the guidelines was posted on a secure web site for comment from CF Center care teams (physicians and ancillary care providers) and was revised as appropriate. As per the CF Foundation guidelines process, these guidelines will be assessed within 3 years to determine if revisions are necessary. AAP American Academy of Pediatrics P&PD Percussion and postural drainage BMI Body mass index PCP Primary care provider From the Women and Children’s Hospital of Buffalo, State CF Cystic fibrosis PERT Pancreatic enzyme replacement University of New York at Buffalo, NY (D.B.); Johns CFA Coefficient of fat absorption therapy Hopkins School of Medicine, Baltimore, MD (K.A.R.); University of Washington, Seattle WA, (M.R.); University of CFTR Cystic fibrosis transmembrane PFT Pulmonary function test North Carolina, Chapel Hill, NC (S.D.D.); Dartmouth conductance regulator PI Pancreatic insufficiency College, Hanover, NH (K.A.S.); Cincinnati Children’s Hospital Medical Center, Cincinnati, OH (S.L.S.); CT Computed tomography PS Pancreatic sufficiency Children’s Hospital of Philadelphia, Philadelphia, PA DHA Docosahexaenoic acid RTC Rapid thoracic compression (S.H.M.); Brigham and Women’s Hospital, Boston, MA (R.B.P.); Cystic Fibrosis Foundation, Bethesda, MD DRI Dietary Reference Intake RVRTC Raised volume rapid (T.B.W., B.C.M.); University of Wisconsin School of FE Fecal elastase thoracoabdominal Medicine and Public Health, Madison, WI (P.M.F.); and FTT Failure to thrive compression Children’s Hospital, Denver, CO (F.J.A.) MCT Medium chain triglyceride RSV Respiratory syncytial virus Please see the Author Disclosures at the end of this article. NBS Newborn screening USPSTF United States Preventive OP Oropharyngeal Services Task Force 0022-3476/$ - see front matter. Copyright Ó 2009 Mosby Inc. All rights reserved. 10.1016/j.jpeds.2009.09.001 S73 THE JOURNAL OF PEDIATRICS www.jpeds.com Vol. 155, No. 6, Suppl. 4 Guidelines for the Care of Newly Diagnosed Infants in greater depth how mutations in the CF transmembrane with CF conductance regulator (CFTR) gene cause CF and the impli- These guidelines are intended to be used by families, primary cations for other family members. The pivotal role that both care providers, and care centers. Even though a framework the parents and primary care provider (PCP) play as part of has been developed to standardize optimal care, it is expected the CF team should be emphasized at the early visits. The that care will be individualized to the needs of patients and positive outlook for newly diagnosed infants should be rein- families. Although the guidelines were developed in response forced and a sense of hope instilled. Last, information de- to the expansion of NBS programs in the United States, they scribing how to contact the CF center if questions or also apply to infants diagnosed prenatally or due to a family concerns arise before the next visit should be shared with history. Infants diagnosed with CF due to meconium ileus at the family. birth or due to a symptomatic presentation should be treated Recommendation #1 is in Table I. in a similar manner once their acute medical needs are ad- dressed. Infants diagnosed with CF through NBS are often la- Coordination of Care with Primary Care Providers beled ‘‘pre-symptomatic,’’ ‘‘asymptomatic,’’ or ‘‘subclinically A collaborative care model should be the goal, with regular affected.’’ However, many have clinical manifestations by 1 and open tri-lateral communication among the family, the month of age.3 Most infants diagnosed through CF NBS PCP and the CF Center. Families will be making numerous are at risk for some complication of the disease including visits to their PCP and CF Center during the first 2 years of hypoelectrolytemia, pancreatic insufficiency (PI), and lung life: standard pediatric visits are at age 1 to 2 weeks and at disease.4 2, 4, 6, 9, and 12 months in the first year of life; CF Center Diagnostic and treatment recommendations are listed at visits should be once monthly during the first 6 months, the end of each section and are summarized in Table I. and every 1 to 2 months in the second 6 months of life (Table III). These visits should complement each other, in Initial Visit that the expertise provided at primary and tertiary care sites Infants diagnosed with CF through NBS often appear to be differ. A written form to delineate appropriate contacts for totally healthy to the parents, and the diagnosis probably parents as problems and questions arise can facilitate efficient will be unexpected. Thus, the psychosocial impact of the di- communication and care (Figure 1). Insurers need to recog- agnosis of CF on the family must be carefully addressed at the nize that despite frequent visits early in life, the cost of care of initial visit. Infants diagnosed with CF through NBS should infants diagnosed with CF through NBS is lower than for be treated at an accredited CF care center, with the goal of those diagnosed following the onset of symptoms.7 Commu- an initial visit within 24 to 72 hours of diagnosis (1 to 3 work- nication between the PCP and CF Center is critical to ensure ing days in the absence of overt symptoms). At the initial visit that parents do not get conflicting messages, especially since to the care center, there should be adequate time for the fam- many CF care goals are different than those of standard pedi- ily to receive comprehensive education regarding CF care. atric care (eg, an emphasis on the need for the child with CF The duration of the visit may need to be as long as 2 hours to be slightly chubby versus concerns about obesity in the of direct face-to-face time between the care team and family; general population). Care providers should convey to each however, the length of the visit and the amount of the infor- other concerns about issues such as but not limited to poor mation addressed needs to be customized to each family. The weight gain, cough or wheezing, change of medications or most important issues to be discussed (based on expert opin- treatments, lack of adherence to the prescribed regimen, dif- ion) are listed in Table II. ficulties in administering prescribed medicines or treatments, Disbelief, anger, or anxiety about the new diagnosis may be worrisome infections in other family members, and immuni- present and retention of information may be a challenge.5 zation status. In addition, critical events such as admission to Families should be encouraged to invite extended family the hospital or change in medications should
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