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Aims and objectives. The Family Support Program was created to support children and parenting when one of the parents has incurable cancer. We chose a family-based approach to support parent's coping and to help families pull together, identify strengths in the family and learn how to seek help. Background. Cancer is usually a new experience for young families. In most cases, parents do not have the necessary knowledge about their children's need for information and support about their parent's serious illness and impending death. Design. A qualitative evaluation study based on data collected through in-depth interviews focusing on parent's experiences with the Family Support Program. Methods. Participants were patients with incurable cancer and their partners and ex-partners with children aged between 5-- 18 years. Thirteen parents were in-depth interviewed. Results. Parents described how the Family Support Program helped them gain greater insight into their children's thoughts and reactions and into how the situation affected their daily living. Parents reported that conflicts were reduced, they could talk more openly about the situation in the family and that they were shown how to support their children's coping. Conclusion. The Family Support Program met the parents in the study's needs for more information and support about how to cope with their children during the patient's terminal illness. Relevance to clinical practice. The Family Support Program is described in detail in a manual that makes it easy for other health workers to use the same programme. The Family Support program was in use in outpatient clinics, oncology wards and palliative care units and was provided both from nurses and social workers trained in cancer care. Parent's in the study would like the Family Support Program to be available to all patients who receive the poor prognosis that their cancer cannot be cured. [ABSTRACT FROM AUTHOR]

Abstract Background Hydrocephalus is a heterogeneous disorder with multiple etiologies that are not yet fully understood. Animal models have implicated dysfunctional cilia of the ependyma and choroid plexus in the development of the disorder. In this report, we sought to determine the origin of the ventriculomegaly in four Bardet Biedl syndrome (BBS) mutant mouse strains as models of a ciliopathy. Methods Evans Blue dye was injected into the lateral ventricle of wild- type and BBS mutant mice to determine whether obstruction of intra- or extra-ventricular CSF flow contributed to ventriculomegaly. Transmission electron microscopy (TEM) was used to examine the ultrastructure of the choroid plexus, subfornical organ (SFO), subcommisural organ (SCO), and ventricular ependyma to evaluate their ultrastructure and the morphology of their primary and motile cilia. Results and discussion No obstruction of intra- or extra-ventricular CSF flow was observed, implying a communicating form of hydrocephalus in BBS mutant mice. TEM analyses of the mutants showed no evidence of choroidal papillomas or breakdown of the blood:CSF barrier. In contrast, structural defects were observed in a subpopulation of cilia lining the choroid plexus, SFO, and ventricular ependyma. These included disruptions of the microtubular structure of the axoneme and the presence of electron-dense vesicular-like material along the ciliary shaft and at the tips of cilia. Conclusions Abnormalities in cilia structure and function have the potential to influence ciliary intraflagellar transport (IFT), cilia maintenance, protein trafficking, and regulation of CSF production. Ciliary structural defects are the only consistent pathological features associated with CSF-related structures in BBS mutant mice. These defects are observed from an early age, and may contribute to the underlying pathophysiology of ventriculomegaly.

Objectives: The aim of this review was to provide a systematic overview of knowledge on how advanced cancer in a parent impacts the healthy parent's role in a family with children aged 6-12 years, and the types of help that they require in order to cope. Despite the large number of families living with a parent affected by cancer, the literature is limited concerning the needs and outcomes for the healthy parents and their need for support in managing their children's needs, when the partner is seriously ill or is in the palliative phase of cancer., Method: Comprehensive literature searches were undertaken by systematically searching for qualitative articles published during the period 1989-2009. The quality assessment was evaluated using a predefined "checklist to assess qualitative research.", Results: Seven articles met our eligibility criteria. Four distinct themes emerged that describe the healthy parent's role in the family, whose life is now characterized by uncertainty and who is attempting to maintain a balance between the needs of their children, the patient, and themselves: (1) new roles without a script, (2) attempting to maintain a safe and normal life, (3) feeling alone even within the family, and (4) support to help young family members., Significance of Results: The present review provides new knowledge and insight into how healthy parents manage the challenges in parenting young children, coping with their everyday lives, and taking on new roles when their partner has advanced cancer or is dying. The healthy parent cannot balance the needs of all family members. To reach the goals of palliative care, nurses and other health professionals are encouraged to offer the patient, the healthy parent, and the children practical and emotional support.

The grief experiences of young children and the interactional dynamics between parents and children leading to healthy grieving remain comparatively under researched. This article reports a qualitative evaluation of a Norwegian Bereavement Support Program where 8 parents described their young child's grief reactions and coping and how these intersected with their own grief. Successful parental coping with their child's grief involves understanding the child's genuine concerns following the death and an intricately holistic balance between shielding and including, between informing and frightening, and between creating a new life while cherishing the old.

Aims and Objectives: This study describes the implementation and evaluation of a new Body Awareness Programme (BAP) in bereavement support for adolescents. The BAP's aims were to provide information and insight into adolescents bodily reactions, to help them develop a deeper understanding of the reasons for bodily reactions and to introduce adolescents to healthy coping techniques., Background: Three main bodily reactions can follow a traumatic event such as the death of a loved one. The arousal of physical responses causes restlessness, concentration problems and disturbed sleep while 'flashbacks' of unpleasant memories contribute to increased tension. Active avoidance manifests as increased activity and avoidance of talking or thinking about unpleasant memories. These reactions may interfere with an adolescent's development and inhibit a healthy grieving process., Design: A qualitative, hermeneutic-phenomenological design., Methods: Data were collected using the BAP together with in-depth interviews with adolescents, focusing particularly on their experiences or recollections of their bodily reactions and coping. Seven adolescents participated, aged 13-18 years, who use our bereavement services., Results: The adolescents in our study internalised their struggles, and beneath their facade of coping, they reported having painful bodies that were stiff and restless. They were also anxious, experiencing painful thoughts of the deceased. The adolescents found the BAP helpful because they gained awareness of the body-behaviour-feelings connections, experiencing the techniques as helpful and possibly useful in their everyday lives., Conclusion: The results of this evaluation of the BAP are positive and suggest that this approach is both necessary and valuable in a bereavement support programme for adolescents., Relevance to Clinical Practice: Adolescents must recognise their own embodied reactions and understand their underlying causes before they can change their attitudes or seek appropriate help during bereavement. Health professionals should see beyond adolescents' facades and offer them support., (© 2012 Blackwell Publishing Ltd.)

Purpose: Mutations in the myocilin gene (MYOC) are the most common known genetic cause of primary open-angle glaucoma (POAG). The purpose of this study was to determine whether topical ocular sodium 4-phenylbutyrate (PBA) treatment rescues glaucoma phenotypes in a mouse model of myocilin-associated glaucoma (Tg-MYOC(Y437H) mice)., Methods: Tg-MYOC(Y437H) mice were treated with PBA eye drops (n = 10) or sterile PBS (n = 8) twice daily for 5 months. Long-term safety and effectiveness of topical PBA (0.2%) on glaucoma phenotypes were examined by measuring intraocular pressure (IOP) and pattern ERG (PERG), performing slit lamp evaluation of the anterior chamber, analyzing histologic sections of the anterior segment, and comparing myocilin levels in the aqueous humor and trabecular meshwork of Tg-MYOC(Y437H) mice., Results: Tg-MYOC(Y437H) mice developed elevated IOP at 3 months of age when compared with wild-type (WT) littermates (n = 24; P < 0.0001). Topical PBA did not alter IOP in WT mice. However, it significantly reduced elevated IOP in Tg-MYOC(Y437H) mice to the level of WT mice. Topical PBA-treated Tg-MYOC(Y437H) mice also preserved PERG amplitudes compared with vehicle-treated Tg-MYOC(Y437H) mice. No structural abnormalities were observed in the anterior chamber of PBA-treated WT and Tg-MYOC(Y437H) mice. Analysis of the myocilin in the aqueous humor and TM revealed that PBA significantly improved the secretion of myocilin and reduced myocilin accumulation as well as endoplasmic reticulum (ER) stress in the TM of Tg-MYOC(Y437H) mice. Furthermore, topical PBA reduced IOP elevated by induction of ER stress via tunicamycin injections in WT mice., Conclusions: Topical ocular PBA reduces glaucomatous phenotypes in Tg-MYOC(Y437H) mice, most likely by reducing myocilin accumulation and ER stress in the TM. Topical ocular PBA could become a novel treatment for POAG patients with myocilin mutations.

Bardet-Biedl syndrome (BBS) is a genetically heterogeneous autosomal recessive disorder with the primary clinical features of obesity, pigmented retinopathy, polydactyly, hypogenitalism, mental retardation and renal anomalies. Associated features of the disorder include diabetes mellitus, hypertension and congenital heart disease. There are six known BBS loci, mapping to chromosomes 2, 3, 11, 15, 16 and 20. The BBS2 locus was initially mapped to an 18 cM interval on chromosome 16q21 with a large inbred Bedouin kindred. Further analysis of the Bedouin population allowed for the fine mapping of this locus to a 2 cM region distal to marker D16S408. Physical mapping and sequence analysis of this region resulted in the identification of a number of known genes and expressed sequence tag clusters. Mutation screening of a novel gene (BBS2) with a wide pattern of tissue expression revealed homozygous mutations in two inbred pedigrees, including the large Bedouin kindred used to initially identify the BBS2 locus. In addition, mutations were found in three of 18 unrelated BBS probands from small nuclear families.