M Richards

Leeds Teaching Hospitals NHS Trust, Leeds, England, United Kingdom

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Publications (28)91.95 Total impact

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    ABSTRACT: Objective: Risk assessment for venous thromboembolism (VTE) and thromboprophylaxis in those with risk factors is established in adult practice. Evidence to support efficacy and safety of this approach in adolescents is lacking. We aimed to describe thrombotic risk factors and to determine the proportion of potentially preventable events in a retrospective cohort study of adolescents with VTE. Design, setting and patients: Data were collected between 2008 and 2014 from eight tertiary UK centres. Qualifying events were radiologically confirmed VTE in subjects aged 12-17 years. Central venous line-related upper venous system events were excluded. Results: 76 cases were identified, 41 males, median age 15 years. Frequent risk factors were: reduced mobility, 45%; thrombophilia, 24%; malignancy, 20%; surgery, 18%; combined oral contraceptive pill, 12%; congenital venous anomaly, 5%. 28 (37%) had no significant underlying diagnosis and no provoking event/hospitalisation, presenting as outpatients with VTE which was considered 'unpreventable'. Of 48 where there had been opportunity for risk assessment, chemical thromboprophylaxis was not indicated in 26 and was contraindicated in 8. 14/76 (18%) had an indication to consider thromboprophylaxis and no contraindication. Of these, four had cerebral palsy, five malignancy and two inflammatory bowel disease. All had reduced mobility with recent surgery in eight. Four received chemical thromboprophylaxis prior to presentation. Conclusions: Among a cohort of adolescents with VTE, a small proportion (13%) had an indication to consider chemical thromboprophylaxis but did not receive it. VTE risk assessment and prevention should focus on adolescents with immobility or surgery, particularly in those with malignancy.
    No preview · Article · Jan 2016 · Archives of Disease in Childhood
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    M Jefford · J Rowland · E Grunfeld · M Richards · J Maher · A Glaser
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    ABSTRACT: Increasing cancer incidence together with improved survival rates are contributing to the growing number of cancer survivors. Survivors may encounter a range of potential effects as a result of the cancer itself or cancer treatments. Traditionally, the major focus of follow-up care has been on detection of cancer recurrence; however, the efficacy of such strategies is questionable. Traditional follow-up frequently fails to identify or adequately address many survivors' concerns. Aftercare needs to be planned to enable better outcomes for survivors, while using scarce health-care resources efficiently. This review focuses on provision of survivorship care, rather than on research. England's National Cancer Survivorship Initiative has developed principles for improved care of those living with and beyond cancer. These include risk-stratified pathways of care, the use of treatment summaries and care plans, information and education to enable choice and the confidence to self manage, rapid re-access to specialist care, remote monitoring and well-coordinated care. Many of these principles are relevant internationally, though preferred models of care will depend on local circumstances.British Journal of Cancer advance online publication, 20 December 2012; doi:10.1038/bjc.2012.554 www.bjcancer.com.
    Full-text · Article · Dec 2012 · British Journal of Cancer
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    ABSTRACT: There are no evidence-based guidelines on pain management in people with haemophilia (PWH), who may suffer acute, disabling pain from haemarthroses and chronic arthropathic pain. To review evidence and to investigate current clinical practice in pain assessment and management in PWH the European Haemophilia Therapy Standardisation Board undertook a literature review and a survey in 22 Haemophilia Treatment Centres (HTC), using a questionnaire and seven clinical scenarios. Consensus was sought on pain assessment and management in PWH. Few clinical studies on pain management in PWH were identified. The HTCs care for 1678 children (47% severe haemophilia, 84% on prophylaxis, 17% with arthropathy and 8% with chronic pain) and 5103 adults (44% severe haemophilia, 40% on prophylaxis, 67% with arthropathy and 35% with chronic pain). Analgesics are prescribed by HTCs in 80% of cases (median; range 0-100%) and in 10% (median; range 0-80%) are bought over the counter. Pain and analgesic use are assessed when reported by patients and at check-ups. Only eight centres use a specific pain scale and/or have specific pain guidelines. Two HTCs arrange regular consultations with pain specialists. For acute pain, the preferred first-line drug is paracetamol for children, and paracetamol or non-steroidal anti-inflammatory drugs (NSAIDs) for adults. Children with chronic pain are treated with paracetamol or NSAIDs, whereas adults usually receive Cox-2 inhibitors. Second-line therapy is heterogeneous. There is little published evidence to guide pain assessment and management in PWH, and clinical practice varies considerably across Europe. General and specific recommendations are needed.
    Full-text · Article · Apr 2012 · Haemophilia
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    ABSTRACT: Continuous infusion (CI) of factor VIII (FVIII) is an effective method for replacement therapy in haemophilia. Recently, concerns have been raised regarding association of CI with the development of inhibitors. The aim of this study was to gain information on the current practices in Europe regarding CI and the true inhibitor incidence after this mode of therapy. In a cross sectional study performed in 22 Comprehensive Care Centres (CCCs), we evaluated CI techniques, treatment protocols, efficacy, safety and complications of CI including inhibitors. Thirteen (59%) CCCs reported a total of 1079 CI treatments, given peri-operatively or for major bleeds, in 742 patients. Most centres used 'adjusted dose' CI aimed at median target FVIII level of 0.8 IU mL(-1). CI was haemostatically very effective with a low incidence of complications: median incidence of postoperative bleeding was 1.8%, six centres observed phlebitis in 2-11% of CI treatments. Only nine (1.2%) patients developed inhibitors (0.45% of 659 severe and 7.2% of 83 mild haemophilia patients). Additional analysis of inhibitor patients revealed several confounding factors (low number of prior FVIII exposure days, high steady-state factor levels during CI, high-risk genotype). In this unprecedentedly large cohort, CI treatment appears to be an effective and safe treatment that does not increase the risk of inhibitor development in patients with severe haemophilia. Thus, previous small case series reports suggesting that CI may increase inhibitors cannot be confirmed. Inhibitor risk in mild haemophilia could not be evaluated as the influence of other, potentially confounding, risk factors could not be excluded.
    No preview · Article · Apr 2012 · Haemophilia
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    ABSTRACT: Birth is the first haemostatic challenge for a child with haemophilia. Our aim was to examine the association between perinatal risk factors and major neonatal bleeding in infants with haemophilia. This observational cohort study in 12 European haemophilia treatment centres (HTC) incorporated 508 children with haemophilia A or B, born between 1990 and 2008. Risk factors for bleeding were analysed by univariate analysis. Head bleeds occurred in 18 (3·5%) children within the first 28 d of life, including three intraparenchymal bleeds, one subdural haematoma and 14 cephalohaematomas. Intra-cranial bleeds were associated with long-term neurological sequelae in two (0·4%) cases; no deaths occurred. Assisted delivery (forceps/vacuum) was the only risk factor for neonatal head bleeding [Odds Ratio (OR) 8·84: 95% confidence interval (CI) 3·05-25·61]. Mild haemophilia and maternal awareness of her haemophilia carrier status seemed to be protective (OR 0·24; 95%CI 0·05-1·05 and OR 0·34; 95%CI 0·10-1·21, respectively), but due to the low number of events this was not statistically significant. We found no association between neonatal head bleeding and country, maternal age, parity, gestational age or presence of HTC. Maternal awareness of carrier status protected against assisted delivery (unadjusted OR 0·37; 95%CI 0·15-0·90; adjusted OR 0·47 (95%CI 0·18-1·21).
    Full-text · Article · Dec 2011 · British Journal of Haematology
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    M Richards · J Corner · J Maher
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    ABSTRACT: The BJC is owned by Cancer Research UK, a charity dedicated to understanding the causes, prevention and treatment of cancer and to making sure that the best new treatments reach patients in the clinic as quickly as possible. The journal reflects these aims. It was founded more than fifty years ago and, from the start, its far-sighted mission was to encourage communication of the very best cancer research from laboratories and clinics in all countries. The breadth of its coverage, its editorial independence and it consistent high standards, have made BJC one of the world's premier general cancer journals. Its increasing popularity is reflected by a steadily rising impact factor.
    Preview · Article · Nov 2011 · British Journal of Cancer
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    ABSTRACT: Several studies have shown differences in survival trends between ethnic groups across adults with cancer in the UK. It is unclear whether these differences exist exclusively in the older adult population or whether they begin to emerge in children and young adults. Subjects (n=3534) diagnosed with cancer under 30 years of age in Yorkshire between 1990 and 2005 were analysed. Differences in survival rates for diagnostic subgroups were estimated by ethnic group (south Asian or not) using Kaplan-Meier estimation and Cox regression. When compared to non-south Asians (all other ethnic groups excluding south Asians) a significant increased risk of death was seen for south Asians with leukaemia (hazard ratio (HR)=1.75; 95% confidence interval (CI)=1.11-2.76) and lymphoma (HR=2.05; 95% CI=1.09-3.87), whereas south Asians with solid tumours other than central nervous system tumours had a significantly reduced risk of death(HR=0.50; 95% CI=0.28-0.89). This was independent of socioeconomic deprivation. We found evidence of poorer survival outcomes for south Asians compared to non-south Asian children and young adults with leukaemia and lymphoma, but better outcomes for south Asian children and young adults with other solid tumours. This needs to be explained, and carefully addressed in the on-going development of cancer services.
    No preview · Article · Sep 2011
  • Bob Phillips · Michael Richards · Rachel Boys · Miranda Hodgkin · Sally Kinsey
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    ABSTRACT: The maintenance phase of treatment for childhood acute lymphoblastic leukemia is characterized by daily oral chemotherapy dose-adjusted on the basis of toxicity, monitored by regular (1 to 2 weekly) blood counts. A traditional approach is undertaking this at out-patient clinics. A home maintenance program was commenced to reduce visits to hospital and associated family disruption. The program organizes blood tests arranged to be taken at or near the patients' home. The results are examined by a pharmacist and specialist nurse; changes in therapy are communicated by telephone call and written confirmation. Hospital attendance is reduced to monthly visits. To assess the program, tablet counting and before-and-after audits of parental satisfaction were undertaken. Results of the first 2 years are presented. Preliminary analysis to identify predictors of nonadherence was performed. Fifty families were included in the evaluation. There were no critical incidents. Poor adherence rates in the initial 3-month period (overall 24%) improved after increased support and advice were offered to 78%. Increasing age was correlated with good adherence (r=0.37, P=0.02). Partnership status of the child's caretakers was strongly associated with adherence (14% of poor adhering patients had caretakers in stable partnerships, compared with 87% of good adhering patients, P<0.01).
    No preview · Article · Jun 2011 · Journal of Pediatric Hematology/Oncology
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    ABSTRACT: In keeping with these data, the increased risk of intra-cranial and extra-cranial bleeding secondary to birth trauma appears to be mirrored in neonates with haemophilia (Ljung et al, 1994; Klinge et al, 1999; Stieltjes et al, 2005; Tarantino et al, 2007; Richards et al, 2009). In one of the first studies to address this issue, Ljung et al (1994) reported data on 117 severe and moderate haemophiliacs born in Sweden between 1970 and 1990. There were 17/117 (14·5%) cases of cranial bleeding, 4/117 (3·5%) ICH, 12/117 (10·3%) extra-cranial haemorrhage (ECH) and 1/117 (0·8%) retro-orbital bleeding (Ljung et al, 1994). Of the 12 subgaleal/cephalic haematomas, 10 had been delivered by ventouse extraction and among the four cases of ICH, one followed ventouse extraction, one followed premature delivery by caesarean section and the other two followed apparently normal vaginal delivery. There was therefore a clear relationship between ventouse at delivery and ECH, but a less clear association with ICH. Several subsequent studies did however show a more definite relationship between ICH and instrumentation at delivery (Klinge et al, 1999; Stieltjes et al, 2005; Tarantino et al, 2007; Richards et al, 2009). In a German registry study (Klinge et al, 1999) 9/11 cases of ICH were associated with trauma at delivery although the details were not specified and Stieltjes et al (2005) reported instrumentation at delivery in 7/10 cases of ICH. In a large population-based study the overall incidence of ICH in the presence of haemophilia or von Willebrand disease was 3·4%, which dropped to 1·9% following exclusion of ventouse deliveries and other co-morbidities (Tarantino et al, 2007).
    Preview · Article · May 2011 · British Journal of Haematology
  • P Cartledge · K Deakin · L McKecknie · M Richards

    No preview · Article · Mar 2011 · Haemophilia
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    ABSTRACT: Acute haemarthrosis is a frequent type of bleeding in individuals with haemophilia. Delayed and/or inadequate treatment can trigger a series of pathological changes within the joint, leading to a painful and disabling arthropathy. The early management of intra-articular bleeding has the potential to prevent chronic joint disease and may include a combination of factor replacement, rest, ice, rehabilitation and, in certain cases, joint aspiration. Little data are, however, available regarding the optimal management of acute haemarthrosis, especially with respect to replacement therapy and the use of adjunctive therapies (aspiration, avoidance of weight bearing and immobilization, as well as the use of anti-inflammatory medication and embolization). To provide more insight into the management of acute haemarthrosis in patients with haemophilia, a literature review was conducted. Concomitantly, current management was surveyed in 26 European haemophilia comprehensive care centres representing 15 different countries. The review highlights the need for future robust studies to better define the appropriate replacement therapy and the role of adjunctive therapies such as aspiration. The survey reveals much heterogeneity in the management of acute haemarthrosis across the EU. Within the constraints discussed, treatment recommendations are presented that reflect the literature, current practice and the clinical experience of the European Haemophilia Therapy Standardisation Board (EHTSB).
    Full-text · Article · Feb 2011 · Haemophilia

  • No preview · Article · Nov 2010 · Journal of Epidemiology & Community Health
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    ABSTRACT: With the advent of modern factor replacement therapy the most important remaining obstacle to successful treatment in haemophilia A is the development of inhibitory antibodies against Facto VIII (FVIII). This retrospective case control study examined genetic variables and early treatment patterns in severe haemophilia A patients who subsequently developed clinically significant inhibitors to FVIII compared with matched controls who did not. Seventy eight inhibitor patients were identified from 13 UK centers over 25 years (1982-2007). For each case an age matched control was selected. Data on potential genetic and treatment related risk factors were collected for cases and controls. Treatment related data was collected for the first 50 exposure days (EDs) for controls or up to inhibitor development for cases. Risk factors were compared for significance by univariate and multivariate analysis. Of the genetic risk factors, major defects in the FVIII gene and non-caucasian ethnicity were each responsible for approximately 5-fold increases in inhibitor risk. When treatment related variables are considered, high intensity treatment increased inhibitor risk around 2.5 fold whether represented by the presence of peak treatment moments or by high overall treatment frequency. This finding was significant regardless of the timing of the high intensity treatment. Periods of intense treatment associated with surgery for porta-cath insertion were however not found to be associated with increased inhibitor risk. No association was shown between inhibitor development and age at first FVIII exposure, type of FVIII product, or the use of regular prophylaxis. This study confirms treatment-related factors as important risks for inhibitor development in Haemophilia A.
    No preview · Article · Nov 2010 · Haemophilia
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    ABSTRACT: Few studies have examined epidemiological differences between ethnic groups for children and young adults with cancer. Subjects aged 0-29 years, diagnosed between 1990 and 2005 in the former Yorkshire Regional Health Authority, were included in the analysis. Ethnicity (south Asian or not) was assigned using name analysis program and Hospital Episode Statistics data. Differences in incidence (per 1,000,000 person-years) rates and trends were analysed using joinpoint and Poisson regression analysis. Overall cancer incidence was similar for south Asians (12.1, 95% CI: 10.7-13.5; n=275) and non-south Asians (12.6, 95% CI: 12.2-13.1; n=3259). Annual incidence rates increased significantly by 1.9% per year on average (95% CI: 1.2-2.6%), especially for south Asians (7.0%; 95% CI: 4.2-9.9%). If present trends continue, the higher rate of increase seen among south Asians aged 0-29 years in Yorkshire will result in three times higher cancer incidence than non-south Asians by 2020.
    Full-text · Article · Oct 2010 · British Journal of Cancer
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    Preview · Dataset · Sep 2010
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    ABSTRACT: It is unclear how many joint bleeds are needed to cause arthropathy in patients with haemophilia; this may be subject to inter-individual variation. Evidence has been presented in a prospective cohort study from Germany that the frequency of haemarthroses whilst receiving prophylaxis was significantly reduced if prophylaxis was started at the latest after a single joint bleed; furthermore the radiological joint score on follow up was significantly correlated with the number of joint bleeds that had occurred before prophylaxis (Kreuz et al, 1998). A study from the Netherlands confirmed that patients who start prophylaxis soon after the first joint bleed show little arthropathy in adulthood (Fischer et al, 2002a). Although it is theoretically attractive to introduce prophylaxis at an early age before the first joint bleed, this may present practical difficulties; the technical challenge posed by the intravenous administration of coagulation products in young children may necessitate the insertion of a permanent indwelling intravenous catheter. It has also been recognised that individuals with severe haemophilia may exhibit clinical phenotypes of varying severity. This has been reflected in the variable age at which patients with severe haemophilia suffer their first bleed and the variable time period that may elapse before they experience their second bleed. These observations have prompted the concept of individualized therapy, whereby patients with a mild clinical phenotype may be able to start prophylaxis at a later age and receive concentrate at less frequent intervals, using less concentrate to achieve a bleed-free exist-ence (Astermark et al, 1999; Astermark, 2003; Petrini, 2001).
    Preview · Article · Mar 2010 · British Journal of Haematology

  • No preview · Article · Jan 2010
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    ABSTRACT: Although most surgical and invasive procedures can be performed safely in patients with haemophilia, the optimal level and duration of replacement therapy required to prevent bleeding complications have not been established conclusively. For providing more insight into optimal therapy during invasive procedures, a literature review of surgical procedures in patients with haemophilia was conducted. Concomitantly, current practice was surveyed in 26 European Haemophilia Comprehensive Care Centres, representing 15 different countries. The review identified 110 original papers published between 1965 and 2007. Of these, only two studies were randomized controlled trials. Target levels and the duration of replacement therapy in the published studies were as follows. For major orthopaedic surgery: preoperative targets were 80-90%; postoperative targets showed a high degree of variation, with trough levels ranging from 20% to 80%, duration 10-14 days; for liver biopsy, 70-100%, 1-7 days; tonsillectomy: 90-100%, 5-11 days; indwelling venous access device insertion: 100%, 3-10 days; circumcision: 50-60%, 2-4 days; dental surgery: 30-50%, single treatment. With the exception of dental surgery, current practice in Europe, as assessed by the survey, was largely in agreement with published data. In conclusion, this study provides both a comprehensive review and a large survey of replacement therapy in patients with haemophilia undergoing invasive procedures; these data have informed the consensus practical treatment recommendations made in this paper. This study highlights the need for better-designed studies in order to better define minimal haemostatic levels of replacement therapy and optimal treatment duration.
    Full-text · Article · Jun 2009 · Haemophilia
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    ABSTRACT: We aimed to describe and contrast the epidemiology of haematological malignancies among 0-14 and 15-24-year-olds in northern England from 1990 to 2002 and compare clinical trial entry by age group. Incidence rates were examined by age, sex and period of diagnosis and differences were tested using Poisson regression. Differences and trends in survival were assessed using Cox regression. 1680 subjects were included comprising 948 leukaemias and 732 lymphomas. Incidence rates for acute lymphoblastic leukaemia were significantly higher for 0-14 compared to 15-24-year-olds, whilst Hodgkin lymphoma showed the reverse. No significant changes in incidence were observed. 60% of leukaemia patients aged 15-24 years entered trials compared to 92% of 0-14-year-olds. Survival rates were significantly lower and improved less markedly over time for 15-24 compared to 0-14-year-olds, particularly for leukaemia. Trial accrual rates need to be improved amongst 15-24-year-olds and a more structured follow-up approach adopted for this unique population.
    No preview · Article · Dec 2008 · European journal of cancer (Oxford, England: 1990)
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    ABSTRACT: Very few studies have addressed the question of adherence of haemophiliacs to their treatment. The aim of our study was to compare their levels of adherence to therapy and also to provide recommendations. Professionals of an international research company performed individual interviews with 30 patients in each of six European countries (France, Germany, Italy, Spain, Sweden and UK) resulting in a total of 180 patients. Twenty-eight interviews with haemophilia physicians and specialist nurses were also undertaken. Overall adherence to treatment was high (80-87% in each country). There was a positive correlation between greater adherence and younger age, prophylactic treatment, time spent with a haemophilia treatment centre (HTC) and the quality of the relationship with the haematologist and nurse. The four leading reasons for not using the prescribed amount of clotting factor or skipping the administration interval were reduction, fluctuation or disappearance of symptoms, forgetfulness, lack of time for treatment and convenience. These reasons differed according to the country and the age of the patient. The main suggestions made by patients to improve adherence related to HTC, environment and factor concentrates. Patients considered also that internet and electronic patient diaries were likely to improve adherence. In this selected group of European haemophilia patients, adherence to treatment appears higher than for most patients with other chronic diseases. However, it remains important to be aware of the possibility of non-adherence given the serious implications, particularly when considering a differently selected group of patients.
    No preview · Article · Sep 2008 · Haemophilia

Publication Stats

618 Citations
91.95 Total Impact Points

Institutions

  • 2007-2012
    • Leeds Teaching Hospitals NHS Trust
      • Department of Paediatric Oncology and Haematology
      Leeds, England, United Kingdom
    • Saint James School Of Medicine
      Park Ridge, Illinois, United States
  • 2003-2011
    • St. James University
      Сент-Джеймс, New York, United States
  • 2004-2005
    • University of Leeds
      Leeds, England, United Kingdom