What are the information needs and concerns of individuals with Polycystic Kidney Disease? Results of an online survey using Facebook and social listening analysis.

BACKGROUND: Polycystic Kidney Disease (PKD) is a hereditary disorder that has no cure and can result in end stage kidney failure. Searching for health information online and via social media is a common phenomenon in many medical conditions. However, no recent studies have documented the information needs, online behaviours, and concerns of people with PKD. The aim of this study was to explore the information needs of individuals with PKD and their carers by documenting (i) the information needs (ii) online information health seeking behaviours (iii) the perceived challenges of living with PKD and (iv) dietary concerns. METHODS: A 17-item survey was constructed by undertaking a social listening analysis. This survey was then distributed via PKD related social media groups on Facebook. Seven groups distributed the survey with permission from the group owners. Open free text survey questions were analysed thematically using content analysis. RESULTS: A total of 536 respondents completed the online survey (70.9 % female, 77 % aged 35-70, 70.2 % diagnosed more than 10 years ago). The major information need expressed by participants with PKD was for dietary information. Information regarding medications, medical management and symptom control were also desired. The overarching themes arising from the free text responses to the major challenge of living with PKD included 'learning to navigate dietary ambiguities'; 'managing social, psychological and emotional needs'; and 'accepting an uncertain future'. In addition to a strong desire for practical and specific dietary information, participants expressed a need for more online information pertaining to management of fatigue, pain, complications and how to manage mental health. Online peer support was also highly regarded and desired. CONCLUSIONS: This study provides contemporary insights into the type of information desired by people with PKD. The results indicated that there was a strong desire for unambiguous information and guidance from health professionals to facilitate self-management, alleviate concerns, and address the complexities of living with Polycystic Kidney Disease. While diet is an important and frequently expressed need, there also remains a large demand for information on how to support psychological needs, and on medical management in order to support treatment decision making. Future work is required to develop specific, actionable and evidence-based resources for patients that are available online and through health professionals. Increased access to renal dietitians, peer support and additional training for health professionals could also improve patient-centered care and support self-management.

Understanding barriers to medication, dietary, and lifestyle treatments prescribed in polycystic kidney disease.

BACKGROUND: Autosomal dominant polycystic kidney disease (PKD) is the most common genetic renal disease and the fourth leading cause of end-stage renal disease in the United States. Although there is no cure for PKD, several treatments are considered to be beneficial, including blood pressure control, exercise, low-salt diet, and high volume water intake. However, levels of understanding of the importance of these treatments and adherence to these recommendations vary among patients. This study explores illness perception models of patients with PKD to reveal barriers in adherence to prescribed therapies; satisfaction with medical care; and sources of medical information. METHODS: We designed a phenomenological interview study to evaluate illness perception models of individuals with PKD. Patients were identified from the national PKD Foundation e-mail distribution list (N = 190) and responded voluntarily to an introductory survey (N = 50). Seventeen PKD patients in the Bay Area were scheduled for one-on-one in-depth interviews with one trained interviewer (W-CT). Open-ended questions administered with an interview guide were used to evaluate patients' beliefs. RESULTS: Mean age was 56.6 +/- 12 years (range 29-78); 65% were female. Many of the PKD patients in this study were highly motivated and willing to incorporate blood pressure, exercise, low-salt diet, and high volume water intake into their daily routines. Barriers to adherence to these therapies include personal beliefs and confusion due to unclear recommendations. CONCLUSIONS: These findings suggest there is variability between what patients understand about their disease and treatments and what they believe their doctors have told them. Not all physicians focus on lifestyle-based treatments, but the majority of PKD patients in our study are motivated and willing to incorporate blood pressure control, exercise, low-salt diet, and high volume water intake into their daily routines and would like specific recommendations on how to implement these. These findings support a role for further exploring patient beliefs about the disease and its necessary treatments in order to design strategies to improve communication and meet the needs of these patients.

Experiences and Perspectives of Polycystic Kidney Disease Patients following a Diet of Reduced Osmoles, Protein, and Acid Precursors Supplemented with Water: A Qualitative Study.

BACKGROUND: Salt, protein, acid precursors, and fluid intake have been identified as factors that influence cyst growth in ADPKD. Unfortunately, the feasibility of following these dietary restrictions/enhancements from a patient's point-of-view has yet to be studied. The purpose of this study is to understand better the experiences of patients following a relatively complex dietary prescription targeting these factors. METHODS: Twelve adults with ADPKD and kidney function >30ml/min/1.73m2 were recruited from the University of Kansas Medical Center Polycystic Kidney Disease clinic. In a qualitative design, semi-structured interviews of participants were conducted following a four week dietary intervention (experimental diet lower in sodium, protein, and acid precursors, and supplemented with water) either face-to-face or by telephone. All interviews were recorded, transcribed verbatim, and checked for accuracy. Transcripts were analyzed thematically for emerging themes. RESULTS: Participants reported that eating less meat and more fruits and vegetables were the easiest components of the diet, whereas reaching the daily goal amount of fruits and vegetables and tracking the diet constantly were the most difficult components. Participants had little difficulty with fluid intake and reported the prescribed fluid goal as achievable. The tracking system for fruits and vegetables and protein was reported to be both helpful and intuitive, but tracking their intake on paper was tedious. Eating out was the most significant barrier to following the diet with some individuals avoiding restaurants in order to comply with the dietary prescription. CONCLUSION: Participants on the experimental diet heightened their awareness of the consumption of dietary salt, protein, acid precursors, and fluid intake. Additionally, most participants believed adherence to the prescribed diet was feasible. However, participants wanted less cumbersome ways to track and monitor the diet, especially given that the prescribed diet is designed for lifelong adherence. Future studies should focus on targeting these specific dietary factors in larger groups of more ethnically and culturally diverse populations to help inform clinicians and how best to help diverse populations adhere to the dietary intervention. TRIAL REGISTRATION: ClinicalTrials.gov NCT01810614.

A painful inheritance-patient perspectives on living with polycystic kidney disease: thematic synthesis of qualitative research.

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a life-threatening genetic disorder and has multiple complications including, infection, pain, intracranial aneurysm and kidney failure leading to significantly impaired quality of life and reduced survival. These outcomes are well described, but patient perspectives and experiences of living with ADPKD are under-recognized. METHODS: MEDLINE, Embase, PsycINFO and CINAHL were searched to August 2014. Studies were analyzed using thematic synthesis. RESULTS: From 21 studies (n = 247), we derived five themes: unvalidated pain (medical trivialization, inadequacy of pain management); persisting uncertainties and ambiguities (lacking diagnostic clarity, disempowerment in self-care, unpredictable daily disruptions, inability to plan ahead, financial discrimination); genetic guilt and resentment (blaming parents, self-blame, constant burden of guilt); precariousness in pursuing parenthood (prognostic uncertainty, owning the decision, needing directive counselling); and defining parental responsibility for genetic testing and disclosure (preserving normality, doubting necessity of genetic testing, respecting the child's autonomy and hope in future technologies, facilitating preparedness). CONCLUSIONS: The erratic onset of pain contributes to the substantial unpredictability of daily living and prevents patients from establishing long-term life goals. Decisions about family planning, genetic testing of children and disclosure involves making profoundly difficult judgments about ethical parental responsibility. Patient engagement in pain management, strategies for self-care, counselling to reduce the burden of 'genetic guilt' and specific family planning decision support tools may be priorities for care to improve patient-centred outcomes in ADPKD.

Spontaneous manifestation of polycystic kidney disease following separation anxiety in a Persian cat.

A 4-year-old female Persian cat was referred with the history of separation from the owner. There was no clinical sign and everything had been reported as normal during the veterinary checkup just before separation and traveling. The history and clinical signs were fit to separation anxiety when the cat referred to us. Ten days after the diagnosis and starting of the treatment for separation anxiety, the cat was referred again with the clinical signs of chronic renal failure. Further assessments showed an inherited polycystic kidney disease. Spontaneous manifestation of polycystic kidney disease after separation anxiety indicate that this phenomenon may cause the chronic process of polycystic kidney disease to promote.

Personality dimensions of haemodialysis patients related to initial renal disease.

OBJECTIVE: The aim of the study was to investigate personality dimensions in uraemic patients undergoing haemodialysis (HD. The relationship between the disease that led to renal failure (and the subsequent need for haemodialysis) and the personality of the haemodialysis patients in question were investigated. METHODS: In the present study, which was conducted in three Greek hospitals, 103 patients were analysed and compared to 138 control patients matching the groups according to their age, sex and place of residence. The investigation was conducted using the Eysenck Personality Questionnaire (EPQ), a reliable method for clinical studies that measures three dimensions of personality: neuroticism, psychoticism and introverted/extroverted. RESULTS: The results from the EPQ were compared to the three disease categories that cause renal failure: glomerulonephritis, polycystic disease, and vascular disease. It was concluded that haemodialysis systematically affected all three scales of personality disorders in the patients. Male patients scored higher in neuroticism, while female patients appeared to score higher in psychoticism. Renal disease, the responsible factor for renal failure and the need for haemodialysis, significantly affected personality disorders. CONCLUSION: The findings show that dialysis modality influences the personalities of patients, and that initial renal disease must be strongly considered in the psychosocial evaluation and care of these patients.

Perceptions of genetic risk in individuals with a one in two chance of developing autosomal dominant polycystic kidney disease.

OBJECTIVE: To determine the level of understanding of personal genetic risk in people born with a one in two risk of having autosomal dominant polycystic kidney disease (ADPKD). DESIGN: Consenting individuals, contacted through affected family members, were asked standard questions about their knowledge of their own genetic risk. These questions were incorporated into a study investigating the use of DNA diagnostic techniques in ADPKD which was an opportunity to interview a substantial number of Australians at risk of inheriting this condition. SETTING: Interviews were conducted in the individuals' homes, or at the Royal Melbourne Hospital Renal Clinic or the Royal Children's Hospital Genetics Clinic. PATIENTS: Three hundred and eighty-seven individuals aged 15 years or more, born with a one in two risk but who had remained undiagnosed up until the time of study were identified within 46 families. Sixty-six were excluded from the study either because they resided too far away or they declined involvement. The remaining 321 were assessed clinically, 304 of whom were also interviewed about their understanding of their risk of having inherited this condition. RESULTS: Thirty per cent knew their risk at birth was one in two. Nine per cent were unaware that ADPKD was an inherited condition. Forty-one per cent were aware that ADPKD was inherited but had no opinion about their personal risk. Three per cent thought that they had no chance of having the condition, 3% thought that they were definitely affected, 8% considered their risk lay from 1%-49% and 6% thought their risk lay from 51%-99%. CONCLUSION: Within this group of people with a high risk of having an inherited condition with preventable and treatable components, the majority has a poor understanding of their genetic risk.

Organic manic syndrome associated with advanced uraemia due to polycystic kidney disease.

Mania secondary to advanced uraemia caused by polycystic disease of the kidneys in a 62-year-old woman involved a complex interplay of physical, psychological and social factors. Psychotherapy and thioridazine led to full recovery.

Education and attitudes in families with adult polycystic kidney disease.

The availability of methods for early accurate identification of individuals at risk of developing adult polycystic kidney disease (ADPKD) has raised a number of important clinical problems. As part of a project on clinical and genetic aspects of ADPKD, we have assessed the knowledge and attitudes of patients and their families. Most had a poor knowledge of the condition and its implications. Despite this, many were in favour of early, including antenatal, diagnosis. A videotape was therefore introduced as part of an educational programme to improve patients' knowledge. This resulted in a marked improvement in understanding of the condition. Although a significant number still wished to consider early diagnosis in their offspring, very few would consider terminating an affected pregnancy.

Adult polycystic kidney disease: knowledge, experience, and attitudes to prenatal diagnosis.

One hundred and ninety subjects from 100 adult polycystic kidney disease (APKD) families on the North Western Regional Genetic Register were interviewed to determine the likely demand for prenatal diagnosis. A detailed questionnaire was used to assess understanding and experience of clinical, therapeutic, and genetic aspects of APKD. Major features of the disease (presence of renal cysts which can lead to renal failure) and forms of therapy (dialysis and transplantation) were known; knowledge of less common features was related to experience. The cohort had had genetic counselling and the majority knew the risk to their own offspring, although the mechanics of the mode of inheritance was often misunderstood. Uptake of presymptomatic ultrasound testing was high, and some implications of early diagnosis are noted. A minority changed their reproductive behaviour as a result of APKD, and although the majority felt a prenatal test should be available, only 23% at high risk of passing on the disease and contemplating children felt they would be interested, and so far only one request for prenatal diagnosis has been received. Thus, demand appears to be low and to be related to perception of the seriousness of APKD.

Attitudes of at-risk and affected individuals regarding presymptomatic testing for autosomal dominant polycystic kidney disease.

One hundred forty-one individuals with autosomal dominant polycystic kidney disease (ADPKD) and 137 at-risk individuals from 107 kindreds answered a questionnaire to determine knowledge and attitudes about ADPKD and the use of gene linkage analysis for prenatal and postnatal presymptomatic diagnosis. Both groups of individuals displayed a high level of knowledge about the disease. Although 87% of affected individuals considered the recurrence risk of ADPKD high, only 11% of ADPKD did not have children for this reason after diagnosis. Ninety-seven percent of at-risk individuals would utilize gene testing for themselves. Eight-eight percent of ADPKD and 89% of at-risk individuals would test offspring (not significant, NS) and 65% of ADPKD and 50% of at-risk individuals between the ages of 18 and 40 would use prenatal testing (NS). Only 4% of ADPKD and 8% of at-risk individuals between the ages of 18 and 40 would terminate a pregnancy for ADPKD. A greater percentage would terminate a pregnancy for a serious medical problem. The factors influencing presymptomatic testing were analyzed. The data suggest that presymptomatic testing will not substantially modify incidence of ADPKD since it may only occasionally alter reproductive plans.

Genetic counseling in adult polycystic kidney disease in Israel.

45 patients with autosomal dominant polycystic kidney disease (APKD) were interviewed with regard to their knowledge about the familial nature of their disease. 22 patients (mean age up to 57 years) were treated with chronic dialysis, and 23 (mean age up to 49 years) had either normal serum creatinine or chronic renal failure without dialysis (serum creatinine range 1-7 mg/dl). Most of the patients knew the name and prognosis of their disease, but only 9% of them knew that half of their children might be at risk. Only 38% of the patients wanted to know that they had APKD before they had children and only 18% would not have had children if they had known beforehand that they were ill. 45% of the patients on dialysis and 78% of the patients not on dialysis would have had children in spite of their disease. The difference between the two groups is significant (p = 0.006). There was a correlation between the duration of follow-up of the patients and their children's knowledge (r = 0.38; p = 0.017). Genetic counseling in Israel is similar to that in other countries, but there is a large difference between the patients in Israel and others regarding their attitude towards childbearing. The finding of a linkage between a polymorphic region on chromosome 16 (3' HVR) to the locus of APKD makes a prenatal diagnosis of the disease possible. This could be a valuable tool for efficient counseling in the future.

Individual and family coping with polycystic kidney disease: the harvest of denial.

A multigeneration family with polycystic kidney disease (PCKD) was studied by personal interviews of all affected and most suspected-affected adults. A clear pattern of denial was identified, which strongly influenced individuals' awareness of PCKD and individuals' taking actions appropriate to that awareness, as well as family cohesion and communication. Time lags--in fact, availability, awareness and action-taking had serious consequences in terms of continued genetic transmission, future "burden" to the family and society, and the psychological stability of individual family members. The potentially important roles of the family physician in this type of familial disease are discussed as: a source of information, a facilitator of awareness and appropriate action, and a counselor in assisting adaptation to this major life stress.