Improving Accessible and Personalized Airway Protective Rehabilitation in Neurodegenerative Disease

Utilization of airway protective rehabilitation among individuals with neurodegenerative disease is astoundingly low. Yet, due to progressive decline in airway protective function and resulting health consequences such as aspiration pneumonia, the need for rehabilitation is clear. Moreover, a growing literature supports the benefit of airway protective rehabilitation in neurodegenerative populations. Therefore, it is a healthcare priority to increase treatment utilization in order to improve health and quality of life for individuals with neurodegenerative disease. Improving treatment accessibility and relevance are two approaches that have the potential to improve utilization. Despite the need to increase treatment accessibility and the growing evidence base to support the use of telehealth to increase access, a significant gap remains in our understanding of the feasibility and acceptability of telehealth to manage dysphagia in neurodegenerative disease. Moreover, little is known about patient perspectives which are critical to refine person-centered models of care that are relevant to patient’s needs. To address this important clinical research gap, this dissertation includes a series of three research studies aimed at improving accessible and relevant rehabilitation for airway protective dysfunction in neurodegenerative disease. Chapter 1 will provide an overview of the current literature as it relates to airway protective dysfunction in neurodegenerative disease, existing rehabilitation approaches, telehealth to manage dysphagia, and the need for personalized care. Chapter 2 will examine the feasibility of rehabilitating airway protection via telehealth in individuals with neurodegenerative movement disorders. Chapter 3 will then explore speech language pathologists’ (SLPs) perspectives and experiences using telehealth to manage dysphagia. Chapter 4 will characterize patient perspectives on airway protective dysfunction and treatment experience following cough skill training (CST). I will conclude (Chapter 5) by synthesizing the findings from chapters 2-4 and suggesting directions for future research

Gaucher disease: A cause of massive splenomegaly in a 15-year-old black African male

Patients with Gaucher disease (GD), a rare autosomal recessive lysosomal storage disease, commonly present to paediatricians with massive splenomegaly. While the diagnosis and management of patients with this chronic multisystem disorder has evolved significantly in recent years, the initial diagnosis represents a challenge. We describe the case of a 15-year-old black African male who presented with abdominal distension, delayed growth and fatigue. Initial laboratory studies revealed severe anaemia (haemoglobin concentration 8 g/dL) and moderate thrombocytopenia (platelet count 80 × 109/L). A computed tomography scan of the abdomen showed an enlarged liver of 173 mm and massive splenomegaly of 27 mm. The diagnosis of GD was confirmed by reduced beta-glucocerebrosidase activity and heterozygous mutations in the GBA1 gene. The patient was managed at a dedicated paediatric haematology unit with enzyme replacement therapy and regular clinical, biochemical and radiological monitoring

An environmental comparison of plastic and paper consumer carrier bags in South Africa: implications for the local manufacturing industry

The conventional consumer carrier bags have recently received considerable attention in South Africa. The choice of material for these bags, based on environmental preferences in the South African context, could significantly influence the local manufacturing industry. Life Cycle Assessment (LCA), an environmental management tool, has been applied to objectively evaluate and compare the overall environmental impacts of the complete life cycles (from raw material extraction to final disposal) of consumer plastic and paper carrier bags in South Africa. Paper bags have a higher carrying capacity compared to plastic bags and different use ratios were subsequently evaluated. Plastic bags have a lower environmental impact for use ratios of up to 2.5 plastic bags to one paper bag. Above this ratio the conclusions are not reliable. Paper bags would need to increase its recycled content significantly to be competitive in terms of environmental impacts. The re-use of thicker plastic, as proposed by the new plastic bag legislation, has the potential to significantly lower the impact of plastic bags.Die konvensionele gebruikersdrasakke het onlangs aansienlike aandag in Suid Afrika ontvang. Die keuse van materiaal vir hierdie sakke, wat gebaseer is op omgewingsvoorkeure in die Suid-Afrikaanse konteks, kan 'n wesenlike invloed uitoefen op die plaaslike vervaardigingsindustrie. Lewenssiklusanalise (LCA), 'n omgewingsbestuurgereedskap, is aangewend vir 'n objektiewe evaluasie en vergelyking van die algehele omgewingsimpakte van die totale lewenssiklus (vanaf grondstofekstraksie tot finale wegdoening) van gebruikersplastiek- en papierdrasakke in Suid-Afrika. Aangesien papiersakke 'n hoër drakapasiteit het in vergelyking met plastieksakke, is verskillende gebruiksverhoudings evalueer. Plastieksakke het 'n laer omgewingsimpak vir gebruiksverhoudings tot en met 2.5 plastieksakke vir elke papiersak. Die gevolgtrekkings van die studie is nie betroubaar vir hoër verhoudings nie. Die persentasie hergesirkuleerde materiaal in papiersakke moet verhoog word om mededingend te wees in terme van omgewingsimpak. Die hergebruik van dikker plastiek, soos voorgestel deur die nuwe plastieksakwetgewing, het die potensiaal om die algehele impak van plastieksakke wesenlik te verlaag.http://sajie.journals.ac.z

Behavioral Management of Respiratory/Phonatory Dysfunction for Dysarthria Associated With Neurodegenerative Disease: A Systematic Review

Purpose: This systematic review represents an update to previous reviews of the literature addressing behavioral management of respiratory/phonatory dysfunction in individuals with dysarthria due to neurodegenerative disease. Method: Multiple electronic database searches and hand searches of prominent speech-language pathology journals were conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses standards. Results: The search yielded 1,525 articles, from which 88 met inclusion criteria and were reviewed by two blinded co-investigators. A large range of therapeutic approaches have been added to the evidence base since the last review, including expiratory muscle strength training, singing, and computer- and device-driven programs, as well as a variety of treatment modalities, including teletherapy. Evidence for treatment in several different population groups—including cerebellar ataxia, myotonic dystrophy, autosomal recessive spastic ataxia of Charlevoix–Saguenay, Huntington\u27s disease, multiple system atrophy, and Lewy body dementia—were added to the current review. Synthesis of evidence quality provided strong evidence in support of only one behavioral intervention: Lee Silverman Voice Treatment Program (LSVT LOUD) in people with Parkinson\u27s disease. No other treatment approach or population included in this review demonstrated more than limited evidence, reflecting that these approaches/populations require urgent further examination. Conclusion: Suggestions about where future research efforts could be significantly strengthened and how clinicians can apply research findings to their practice are provided

Educating peers about human sexuality and birth control in natural settings: A social comparison perspective

Adolescents are being trained to play several important roles in educating peers about human sexuality and birth control. A social comparison perspective offers numerous advantages over a persuasive communication perspective in the design, implementation, and evaluation of such peer-education programs. In the social comparison perspective, peer educators serve as social referents, providing standards of knowledge and behavior for the contacts with respect to understanding sexuality and preventing pregnancy.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/24948/1/0000375.pd

AN ENVIRONMENTAL COMPARISON OF PLASTIC AND PAPER CONSUMER CARRIER BAGS IN SOUTH AFRICA: IMPLICATIONS FOR THE LOCAL MANUFACTURING INDUSTRY

<P>ENGLISH ABSTRACT: The conventional consumer carrier bags have recently received considerable attention in South Africa. The choice of material for these bags, based on environmental preferences in the South African context, could significantly influence the local manufacturing industry. Life Cycle Assessment (LCA), an environmental management tool, has been applied to objectively evaluate and compare the overall environmental impacts of the complete life cycles (from raw material extraction to final disposal) of consumer plastic and paper carrier bags in South Africa. Paper bags have a higher carrying capacity compared to plastic bags and different use ratios were subsequently evaluated. Plastic bags have a lower environmental impact for use ratios of up to 2.5 plastic bags to one paper bag. Above this ratio the conclusions are not reliable. Paper bags would need to increase its recycled content significantly to be competitive in terms of environmental impacts. The re-use of thicker plastic, as proposed by the new plastic bag legislation, has the potential to significantly lower the impact of plastic bags.</P><P>AFRIKAANSE OPSOMMING: Die konvensionele gebruikersdrasakke het onlangs aansienlike aandag in Suid Afrika ontvang. Die keuse van materiaal vir hierdie sakke, wat gebaseer is op omgewingsvoorkeure in die Suid-Afrikaanse konteks, kan 'n wesenlike invloed uitoefen op die plaaslike vervaardigingsindustrie. Lewenssiklusanalise (LCA), 'n omgewingsbestuurgereedskap, is aangewend vir 'n objektiewe evaluasie en vergelyking van die algehele omgewingsimpakte van die totale lewenssiklus (vanaf grondstofekstraksie tot finale wegdoening) van gebruikersplastiek- en papierdrasakke in Suid-Afrika. Aangesien papiersakke 'n hoër drakapasiteit het in vergelyking met plastieksakke, is verskillende gebruiksverhoudings evalueer. Plastieksakke het 'n laer omgewingsimpak vir gebruiksverhoudings tot en met 2.5 plastieksakke vir elke papiersak. Die gevolgtrekkings van die studie is nie betroubaar vir hoër verhoudings nie. Die persentasie hergesirkuleerde materiaal in papiersakke moet verhoog word om mededingend te wees in terme van omgewingsimpak. Die hergebruik van dikker plastiek, soos voorgestel deur die nuwe plastieksakwetgewing, het die potensiaal om die algehele impak van plastieksakke wesenlik te verlaag.</P&gt

Baseline characteristics of 32 patients with Gaucher disease who were treated with imiglucerase: South African data from the International Collaborative Gaucher Group (ICGG) Gaucher Registry

Background. Gaucher disease (GD) is a rare inherited autosomal recessive metabolic disorder with a prevalence in the general population of ~1 per 100 000. To optimise the recognition, diagnosis and management of patients with GD in South Africa (SA), it is important to have an understanding of local patterns of presentation of the disease. Objectives. To describe the baseline pretreatment characteristics of the SA cohort of patients enrolled into the International Collaborative Gaucher Group (ICGG) Gaucher Registry whowere treated with imiglucerase (Cerezyme; Sanofi Genzyme). Methods. The ICGG Gaucher Registry is an observational, longitudinal, international database that tracks the clinical, demographic, genetic, biochemical and therapeutic characteristics of patients with GD globally, irrespective of disease severity, treatment status or treatment choice. The study population included all SA patients reported in the ICGG Gaucher Registry as of 1 May 2020. Results. The registry included 49 SA GD patients, of whom 32 received imiglucerase as first primary GD therapy. All the patients had GD type 1, 59.4% were female, and mean and median ages at diagnosis were 14.7 and 9.8 years, respectively. The most common genotype was N370S/N370S (37.5%). At treatment initiation, 30.0% of patients had been splenectomised. Among patients for whom data were available, anaemia was present in one-third of non-splenectomised patients and 12.5% of those with splenectomy, and moderate or severe thrombocytopenia was reported in two-thirds of non-splenectomised patients. Bone pain was present in 30.8% and 57.1% of non- splenectomised and splenectomised patients, respectively. No bone crises were reported, and data relating to other bone complications were available for only ≤3 patients. Conclusions. Haematological findings and bone pain in this group are similar to those in the global ICGG Gaucher Registry cohort. Lack of baseline data for other bone complications limits interpretation in that regard. Clinicians who treat patients with GD are encouraged to submit accurate, complete and up-to-date information so that comprehensive data for the subset of SA GD patients can be maintained to improve recognition and diagnosis, and guide appropriate and effective use of treatment for SA patients