Parents Of Cystic Fibrosis Children Research Articles

P258 Do smoking habits of parents of cystic fibrosis children differ from parents of diabetes mellitus type 1 children?

P258 Do smoking habits of parents of cystic fibrosis children differ from parents of diabetes mellitus type 1 children?

Nebuliser cleaning and disinfection practice in the home among patients with cystic fibrosis.

Nebulised delivery of different classes of drugs is of fundamental importance in therapeutic regimens relating to both the management of disease progression in cystic fibrosis disease and its associated complications. The aim of this study was to determine if current nebuliser hygiene practices in the home environment by paediatric and adult cystic fibrosis populations are appropriate to ensure appropriate infection control and prevention measures have been addressed. An Audit Questionnaire Study was completed with adult cystic fibrosis patients (n=20) or with parents of cystic fibrosis children (n=24), through a healthcare professional interview on a one-to-one basis, during either a home visit or during patient/parent attendance at cystic fibrosis clinic. Hygienic practices relating to nebuliser care varied, with paediatric carers more likely to clean and disinfect their devices. This study suggests there is much variation and confusion with regard to how to clean and disinfect nebulisers, as well as who is responsible for delivering this advice. The adult cystic fibrosis community in particular needs to be educated on practicalities associated with nebuliser hygiene and the reasons why this is important. Furthermore, to date there is a lack of a universally recommended guideline suitable for all types of cystic fibrosis nebulisers that all relevant pharmaceutical manufacturers advocate.

Identification of Dermatoglyphic Patterns in Parents of Children with Cystic Fibrosis

Background Dermatoglyphics could assist in the diagnosis of congenital abnormalities. The aim of this study was to identify the dermatoglyphic patterns (finger print pattern type, total ridge count of each finger, a-b ridge count, and articulotrochanteric distance [ATD angles]) in the parents of cystic fibrosis children. Materials and Methods We recruited 75 parents of children with cystic fibrosis for the study group and 341 parents for the control group. We recorded finger print pattern type, total ridge count (TRC), a-b ridge count, and ATD angles of all participants. Then we identified any asymmetry between the right and left hands of each person and characteristics of parents in two genders. Chi-square analysis, Mann-Whitney U test, and Fisher's exact test were used for data analysis. Results We observed significant differences in a-b ridge count on the right hand (P=0.02), and mean total ridge count on the right digit I (P=0.05), right digit IV (P=0.03), and right digit V in the fathers of children with cystic fibrosis compared to the control group(P=0.02). Conclusion According to the results, we suggest that the dermatoglyphic traits of parents could be used as a simple, appropriate, and supplementary screening method in the diagnosis of children with CF. Nevertheless, we need lager studies to precisely confirm that dermatoglyphics is a reliable method in the diagnosis of CF.

Inconclusive Cystic Fibrosis neonatal screening results: long‐term psychosocial effects on parents

Cystic Fibrosis (CF) Newborn Screening occasionally identifies neonates where a CF diagnosis can neither be confirmed nor excluded. To assess how parents of these infants cope with this ambiguous situation. Parents of 11 children with Ambiguous Diagnosis (group AD) were compared with parents of 11 children diagnosed with CF through neonatal screening [group Cystic Fibrosis Diagnosis (CFD)] and with parents of 11 Healthy Control children (group HC) matched for gender and age. The emotional reaction to the inconclusive result was less pronounced in AD than in CFD (p = 0.003), and AD parents considered their infants as healthy as controls. Parents' anxiety about their child's health is stronger in CFD than in AD (p < 0.05) and HC (p < 0.001). Long-term emotional distress was rated similarly in AD and CFD, and greater than in HC (p = 0.0003). The parent/child relationship was less influenced in AD than in the CF group (p = 0.03). Seven AD and CFD parents changed their family planning projects. Inconclusive neonatal screening results appear to be understood and associated with lower anxiety levels than CF diagnosis. Concern about the child's health is similar to healthy controls and lower than in parents of CF children.

Parental fears of Pseudomonas infection and measures to prevent its acquisition

Background and aim of the study: Chronic infection with Pseudomonas aeruginosa (PA) is associated with accelerated worsening of lung disease in patients with cystic fibrosis (CF). Fears of PA are widespread among parents of CF children, and many parents take precautions at home to prevent acquisition of the bacterium from the environment. The present study was undertaken to describe the type and intensity of these activities. Methods: Parents of 21 CF children (7 without prior PA infection, 10 with intermittent and 4 with chronic PA infection) were investigated using semistructured interviews. These were analyzed descriptively and with respect to predominant themes. Additionally, a German personality test was used to evaluate the influence of psychological factors. Results: The clinical impression of widespread parental anxieties of PA infection was confirmed. Misunderstandings concerning PA infections were related to a simplistic concept of the underlying biological mechanisms. Some parents which we classified as ‘bacterium-focussed’ thought that each contact with PA would lead to bacterial infection. These parents used a large variety of measures, which concerned both domiciliary and outdoor surroundings and activities. At the other end of the spectrum were parents which we classified as ‘child-focussed’ who mostly supported (and relied on) the child's defense mechanism instead of hygienic measures. Conclusions: Recommendations by physicians on how to prevent PA acquisition from the environment should take into account possible non-intended side effects, since some parents will exaggerate daily precautions to the detriment of the child's (and the parent's) quality of life.

Attitudes of parents of cystic fibrosis children towards neonatal screening and antenatal diagnosis

Information on parents' attitudes towards neonatal screening for cystic fibrosis (CF) and antenatal diagnosis by chorion villus biopsy (CVS) has been derived from a detailed questionnaire administered to parents of CF babies diagnosed early following newborn screening (18 babies), and later on account of clinical criteria (11 babies). Screening was by measurement of immunoreactive trypsin (IRT) on Guthrie card blood spots, which was the basis of the Wales/West Midlands IRT Screening Survey, 1985-1989. Families questioned were from Wales. Most parents supported screening: parents of 15/18 (83%) screened babies and 10/11 (91%) unscreened babies. Following antenatal diagnosis, 15/29 (52%) of families would abort a CF foetus. Neither standard of education nor social class correlated with attitudes to screening or antenatal diagnosis, although these factors were related to the parents' knowledge of CF in general. Several families emphasised the importance of minimal delay between the initial mention of the possibility of CF on IRT testing and confirmation (or otherwise) of the diagnosis. Four mothers acknowledge temporary rejection of their babies during the period of uncertainty or following the procedures of diagnosis, emphasising that neonatal screening for CF can have a psychological impact on the parent-child bonding. Although most families supported neonatal screening for CF, this study underlines some of the difficulties which may be encountered during the procedure of screening for CF by IRT.

Family adaptation and psychosocial adjustment to cystic fibrosis in the preschool child

The parents of 80% (41 of 51) of preschoolers with cystic fibrosis (CF) diagnosed at least 1 year prior to the study and attending the Hospital for sick Children (HSC) CF Clinic completed the Problem Inventory (PINV), Preschool Behavior Questionnaire (PBQ) and Family Assessment Measure (FAM). The mean age of the CF children was 3.7 years. Parents of a control group of 31 healthy daycare children with a mean age of 3.6 years completed the same questionnaires. Parents of healthy preschoolers reported more child-related problems for 2–5 year olds than did parents of CF children ( P < 0.001) suggesting that parents who have confronted the CF diagnosis go on to minimize the normal stresses of the developmental period. Considerable agreement was seen between PINV scores for mothers and fathers in each group, revealing that parents in a given family perveive similarly the impact their child has upon them. The mean PBQ for CF preschoolers was not significantly different from that of the control group, although there was some tendency toward hostile aggressive behavior in the CF group. Surprisingly, total FAM scores of all samples showed no significant differences with the exception of a better total FAM score for fathers of CF children when compared to control fathers revealing that the CF family is not, during the early years of relative health stability, adversely affected. Two subscales were significantly elevated, social desirability (for CF mothers and fathers) and denial (for CF mothers only), describing an important response style which may enhance mastery of long-term stress. Psychological measures and family demographic characteristics generally did not correlate with the physical status of CF preshoolers nor were significant differences found between CF boys and girls.