Pennsylvania prescription drug monitoring program: potential public health impact of a national database

Prescription drug misuse and opioid overdose death have increased significantly in recent years. Many states have implemented Prescription Drug Monitoring Programs (PDMPs) as a means to improve prescribing practices and mediate the ongoing opioid crisis in the United States (US). This comprehensive literature review examines the current structure of state-run PDMPs, and legislation surrounding them. More specifically, the Pennsylvania PDMP is examined. Limitations and barriers to use of PDMPs are explored. Recommendations are provided for improving current state-run PDMPs, and a proposal is made for the development of a national-level prescription drug monitoring database. Public Health Significance: The implementation of a standardized national-level prescription drug monitoring database could have a significant impact on reducing accessible opioids and other substances in the community. This could reduce opioid overdose death rates as discussed in prior studies of the effects of current state-run programs. A national program could also have an impact on decreasing the spread of infectious diseases such as HIV and Hepatitis C. Literature supports an association between substance use and increased risk of engaging in needle sharing and risky sexual behavior while under the influence of drugs or in seeking drugs. Improving accessibility of patient prescription data through a national database could lead to improved prescribing/tapering of opioid drugs, and improved screening and treatment for substance use; therefore, preventing the progression from prescription drug misuse to injection drug use

Sniff test: does what we measure at the nose reflect what happens in the chest wall?

Nasal pressure measured during sniff (SNIP) is a technically simple voluntary test. Since the contraction of the diaphragm expands the abdomen, the volume variation during sniff manoeuvre should therefore be predominantly abdominal in order to be considered a specific index of diaphragm strength. We aimed to verify if and how SNIP varied according to thoraco-abdominal volume variations. We measured abdominal volume variations, using opto-electronic plethysmography, during quiet breathing (ABQB) and sniff manoeuvres (ABSN) in supine position on 30 patients (age: 42; FVC:47.5%; FEV1:30%) on the waiting list for lung transplant. SNIP was measured simultaneously with ABSN. 68 sniff were analysed and classified into 4 groups according to ABSN: 16 with thoracic paradox, 24 predominantly abdominal, 16 predominantly thoracic and 12 with abdominal paradox. By definition ABSN was different (p<0.001) among the 4 groups, whereas ABQB (~75%; p=0.373) and SNIP (~53 cmH2O, p= 0.792) were similar (figure 1). SNIP did not change with the different thoraco-abdominal strategies. The diaphragm was not weak and leaded inspiration, therefore ABSN varied because the patients misperformed the manoeuvre. In order to not misunderstand the clinical significance of a sniff test, care should be paid also in thoraco-abdominal movement because SNIP, per se, cannot differentiate between thoracic or diaphragmatic manoeuvre with the risk to lose its specificity

Spontaneous breathing pattern as respiratory functional outcome in children with spinal muscular atrophy (SMA)

Introduction: SMA is characterised by progressive motor and respiratory muscle weakness. We aimed to verify if in SMA children 1)each form is characterized by specific ventilatory and thoracoabdominal pattern(VTAp) during quiet breathing(QB); 2)VTAp is affected by salbutamol therapy, currently suggested as standard treatment, or by the natural history(NH) of SMA; 3)the severity of global motor impairment linearly correlates with VTAp. Materials and methods: VTAp was analysed on 32 SMA type I (SMA1, the most severe form), 51 type II (SMA2, the moderate), 8 type III (SMA3, the mildest) and 20 healthy (HC) using opto-electronic plethysmography. Spirometry, cough and motor function were measured in a subgroup of patients. Results: In SMA1, a normal ventilation is obtained in supine position by rapid and shallow breathing with paradoxical ribcage motion. In SMA2, ventilation is within a normal range in seated position due to an increased respiratory rate(p0.05) while tachypnea occurred in type I NH. A linear correlation(p<0.001) was found between motor function scales and VTAp. Conclusion: A negative or reduced %ΔVRC,P, indicative of ribcage muscle weakness, is a distinctive feature of SMA1 and SMA2 since infancy. Its quantitative assessment represents a non-invasive, non-volitional index that can be obtained in all children, even uncollaborative, and provides useful information on the action of ribcage muscles that are known to be affected by the disease. Low values of motor function scales indicate impairment of motor but also of respiratory function

A New Method for Measuring Bell-Shaped Chest Induced by Impaired Ribcage Muscles in Spinal Muscular Atrophy Children

The involvement of the respiratory muscular pump makes SMA children prone to frequent hospitalization and morbidity, particularly in type 1. Progressive weakness affects ribcage muscles resulting in bell-shaped chest that was never quantified. The aims of the present work were: (1) to quantify the presence of bell-shaped chest in SMA infants and children and to correlate it with the action of ribcage muscles, assessed by the contribution of pulmonary ribcage to tidal volume (ΔVRC, p); (2) to verify if and how the structure of the ribcage and ΔVRC, p change after 1-year in SMA type 2. 91 SMA children were studied in supine position during awake spontaneous breathing: 32 with type 1 (SMA1, median age: 0.8 years), 51 with type 2 (SMA2, 3.7 years), 8 with type 3 (SMA3, 5.4 years) and 20 healthy children (HC, 5.2 years). 14 SMA2 showed negative ΔVRC, p (SMA2px), index of paradoxical inspiratory inward motion. The bell-shaped chest index was defined as the ratio between the distance of the two anterior axillary lines at sternal angle and the distance between the right and left 10th costal cartilage. If this index was &lt; &lt; 1, it indicated bell shape, if ~1 it indicated rectangular shape, while if &gt;&gt; 1 an inverted triangle shape was identified. While the bell-shaped index was similar between HC (0.92) and SMA3 (0.91), it was significantly (p &lt; 0.05) reduced in SMA2 (0.81), SMA2px (0.74) and SMA1 (0.73), being similar between the last two. There was a good correlation (Spearman's rank correlation coefficient, ρ = 0.635, p &lt; 0.001) between ribcage geometry and ΔVRC, p. After 1 year, ΔVRC, p reduced while bell-shaped chest index did not change being significantly lower than HC. The shape of the ribcage was quantified and correlated with the action of ribcage muscles in SMA children. The impaired ribcage muscles function alters the ribcage structure. HC and SMA3 show an almost rectangular ribcage shape, whereas SMA2, SMA2px and SMA1 are characterized by bell-shaped chest. In SMA, therefore, a vicious cycle starts since infancy: the disease progressively affects ribcage muscles resulting in reduced expansion of lung and ribcage that ultimately alters ribcage shape. This puts the respiratory muscles at mechanical disadvantage

Intellectual abilities, language comprehension, speech, and motor function in children with spinal muscular atrophy type 1

Background: Spinal muscular atrophy (SMA) is a chronic, neuromuscular disease characterized by degeneration of spinal cord motor neurons, resulting in progressive muscular atrophy and weakness. SMA1 is the most severe form characterized by significant bulbar, respiratory, and motor dysfunction. SMA1 prevents children from speaking a clearly understandable and fluent language, with their communication being mainly characterized by eye movements, guttural sounds, and anarthria (type 1a); severe dysarthria (type 1b); and nasal voice and dyslalia (type 1c). The aim of this study was to analyze for the first time cognitive functions, language comprehension, and speech in natural history SMA1 children according to age and subtypes, to develop cognitive and language benchmarks that provide outcomes for the clinical medication trials that are changing SMA1 course/trajectory. Methods: This is a retrospective study including 22 children with SMA1 (10 affected by subtype 1a-1b: AB and 12 by 1c: C) aged 3–11 years in clinical stable condition with a coded way to communicate “yes” and “no”. Data from the following assessments have been retrieved from patient charts: one-dimensional Raven test (RCPM), to evaluate cognitive development (IQ); ALS Severity Score (ALSSS) to evaluate speech disturbances; Brown Bellugy modified for Italian standards (TCGB) to evaluate language comprehension; and Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) to assess motor functioning. Results: SMA 1AB and 1C children were similar in age, with the former characterized by lower CHOP-INTEND scores compared to the latter. All 22 children had collaborated to RCPM and their median IQ was 120 with no difference (p = 0.945) between AB and C. Global median score of the speech domain of the ALSSS was 5; however, it was 2 in AB children, being significantly lower than C (6.5, p < 0.001). TCGB test had been completed by 13 children, with morphosyntactic comprehension being in the normal range (50). Although ALSSS did not correlate with both IQ and TCGB, it had a strong (p < 0.001) correlation with CHOP-INTEND described by an exponential rise to maximum. Conclusions: Although speech and motor function were severely compromised, children with SMA1 showed general intelligence and language comprehension in the normal range. Speech impairment was strictly related to global motor impairment

Understanding the total airway response to exercise: current perspectives and future challenges

Exercise places a unique set of physiological demands on the airway tract. Historically, most work in this area has focused on the lower airway response, however it is now becoming increasingly apparent that the structural and functional behaviour of the upper airway and large central airways is equally important. Dysfunction in these sections of the airway tract can act to either hinder or modulate the exercise ventilatory response and as such lead to an increased work of breathing and the development of troublesome respiratory symptoms. This article provides an overview of the way in which the entire airway tract is challenged by the heightened ventilatory state mandated by physical activity, highlighting recent developments in our understanding of the physiology of laryngeal, large central and lower airway function during exercise