Exploring the Quality-of-Life Impact, Disease Burden, and Management Challenges of GPP: The Provider and Patient Perspective [Podcast].

Generalized pustular psoriasis (GPP) is a rare, chronic, and debilitating disease characterized by flares of widespread erythema, desquamation, and pustule formation. GPP flares can be accompanied by systemic symptoms including fever, fatigue, malaise, and skin pain; severe cases may be fatal if untreated. Although GPP may occur concurrently with plaque psoriasis, they represent two distinct inflammatory conditions. Patients with GPP experience a substantial burden of disease, and the impact of GPP on an individual's mental health and quality-of-life (QoL) goes far beyond skin pain and discomfort. The rarity of GPP may result in a misdiagnosis, as the sudden onset of skin pustules may be mistaken for a primary infection. Misdiagnosis with a subsequent delay in treatment has tremendous negative consequences for the affected patient. In September 2022, spesolimab became the first FDA-approved medication in the US for the treatment of GPP flares in adults. Spesolimab has since been approved by regulatory agencies in numerous countries, including Japan, Mainland China, and the EU. Prior to spesolimab, the clinical management of GPP relied on the off-label use of systemic or biologic therapies approved for plaque psoriasis or other inflammatory conditions. There is a need for increased education among healthcare providers regarding the clinical diagnosis, risk stratification, and therapeutic management of this rare disease, including the other novel GPP-specific therapies in development. In this podcast, two dermatologists and a patient who has plaque psoriasis and GPP discuss the clinical presentation, symptoms, disease burden, QoL impacts, diagnostic challenges, and therapeutic strategies for the management of GPP.

Impact of Generalized Pustular Psoriasis from the Perspective of People Living with the Condition: Results of an Online Survey.

BACKGROUND: Generalized pustular psoriasis (GPP) is a rare disease characterized by episodic worsening (flares). Knowledge of the burden of GPP and the experience of affected individuals is limited. AIMS: To conduct a survey of people living with GPP to understand how they experience GPP flares, which therapies they have received and are receiving, and how GPP impacts their activities of daily living. METHODS: The online survey consisted of 43 questions answered by individuals recruited from an opt-in market research database. The research team performed a targeted outreach to identify individuals with GPP. The survey included screening questions to determine if potential participants qualified for inclusion. Eligible individuals were US residents aged ≥ 18 years who self-reported that they had been diagnosed with GPP. Respondents provided consent to participate and received compensation (fair market value) for their time. RESULTS: Between August 4 and 14, 2020, 66 people living with GPP in the USA were surveyed. Most participants were female, aged 40-59 years, had been diagnosed ≥ 1 year previously, and had experienced ≥ 2 flares in the past year. A substantial proportion of respondents had symptoms for years, had consulted multiple healthcare professionals, and experienced misdiagnoses before receiving a diagnosis of GPP. Emotional stress was the most common cause of flares and many respondents reported a fear of flares. Respondents defined flares by the presence of itching, an increase in the size of the affected area, more crusts or pustules, and fatigue. A change in mood was the most burdensome symptom. Most respondents were receiving topical corticosteroids and only approximately one-third felt their condition was well controlled. GPP had an impact on activities of daily living even in the absence of flares and many respondents felt that their physician did not understand the level of emotional, psychological, or physical pain caused by GPP. CONCLUSIONS: GPP imposes a substantial emotional burden on patients, with wide-ranging impacts on activities of daily living beyond the physical discomfort of skin lesions.

National Partnership for Maternal Safety: Consensus Bundle on Safe Reduction of Primary Cesarean Births-Supporting Intended Vaginal Births.

Cesarean births and associated morbidity and mortality have reached near epidemic proportions. The National Partnership for Maternal Safety under the guidance of the Council on Patient Safety in Women's Health Care responded by developing a patient safety bundle to reduce the number of primary cesarean births. Safety bundles outline critical practices to implement in every maternity unit. This National Partnership for Maternity Safety bundle, as with other bundles, is organized into four domains: Readiness, Recognition and Prevention, Response, and Reporting and Systems Learning. Bundle components may be adapted to individual facilities, but standardization within an institution is advised. Evidence-based resources and recommendations are provided to assist implementation.

National Partnership for Maternal Safety: Consensus Bundle on Safe Reduction of Primary Cesarean Births-Supporting Intended Vaginal Births.

Cesarean births and associated morbidity and mortality have reached near epidemic proportions. The National Partnership for Maternal Safety under the guidance of the Council on Patient Safety in Women's Health Care responded by developing a patient safety bundle to reduce the number of primary cesarean births. Safety bundles outline critical practices to implement in every maternity unit. This National Partnership for Maternity Safety bundle, as with other bundles, is organized into four domains: Readiness, Recognition and Prevention, Response, and Reporting and Systems Learning. Bundle components may be adapted to individual facilities, but standardization within an institution is advised. Evidence-based resources and recommendations are provided to assist implementation.

National Partnership for Maternal Safety: Consensus Bundle on Safe Reduction of Primary Cesarean Births- Supporting Intended Vaginal Births.

Cesarean births and associated morbidity and mortality have reached near epidemic proportions. The National Partnership for Maternal Safety under the guidance of the Council on Patient Safety in Women's Health Care responded by developing a patient safety bundle to reduce the number of primary cesarean births. Safety bundles outline critical practices to implement in every maternity unit. This National Partnership for Maternity Safety bundle, as with other bundles, is organized into four domains: Readiness, Recognition and Prevention, Response, and Reporting and Systems Learning. Bundle components may be adapted to individual facilities, but standardization within an institution is advised. Evidence-based resources and recommendations are provided to assist implementation.

A multicenter prospective study of neonatal outcomes at less than 32 weeks associated with indications for maternal admission and delivery.

BACKGROUND: Counseling for patients with impending premature delivery traditionally has been based primarily on the projected gestational age at delivery. There are limited data regarding how the indications for the preterm birth affect the neonatal outcome and whether this issue should be taken into account in decisions regarding management and patient counseling. OBJECTIVE: We performed a prospective study of pregnancies resulting in premature delivery at less than 32 weeks to determine the influence of both the indications for admission and their associated indications for delivery on neonatal mortality and complications of prematurity. STUDY DESIGN: This is a multicenter, prospective study in 10 hospitals where all data from the neonatal intensive care unit routinely was imported to a deidentified data warehouse. Maternal data were collected prospectively at or near the time of delivery. Eligible subjects included singleton deliveries in these hospitals between 23 0/7 and 31 6/7 weeks. The primary hypothesis of the study was to determine whether there was a difference in the primary outcome, which was defined as neonatal composite morbidity, between those neonates delivered after admission for premature labor vs premature rupture of membranes, because these were expected to be the 2 most frequent diagnoses leading to premature birth. The sample size was calculated based on a 10% difference in outcomes for these 2 entities. We based this hypothesis on the knowledge that premature rupture of membranes has a greater incidence of intra-amniotic infection and inflammation than premature labor and that outcomes for premature neonates are worse when delivery is associated with intra-amniotic infection. Additional outcomes were analyzed for all other indications for admission and delivery. Composite morbidity was defined as ≥1 of the following: respiratory distress syndrome (oxygen requirement, clinical diagnosis, and consistent chest radiograph), bronchopulmonary dysplasia (requirement for oxygen support at 28 days of life), severe intraventricular hemorrhage (grades 3 or 4), periventricular leukomalacia, blood culture-proven sepsis present within 72 hours of birth, necrotizing enterocolitis, or neonatal death before discharge from the hospital. A secondary composite of serious neonatal morbidity also was defined prospectively. RESULTS: The study included 1089 mother/baby pairs. Composite morbidity between those with premature labor (77.2%) and premature rupture of membranes (73.2%) was not significantly different (P = .29). A few neonatal complications were associated with indications for admission and delivery, but on logistic regression adjusting for gestational age and other confounders, suspected intrauterine growth restriction was the only indication for admission or delivery associated with an increase in serious morbidity (odds ratio 4.5, [2.1 to 9.8], P < .003). Other factors not related to the indications for admission including cesarean delivery, and low 5-minute Apgar were associated with an increase in morbidity. CONCLUSION: Studies of many single factors related to the indications for preterm delivery have been shown to be associated with adverse neonatal outcome. In this study evaluating all of the most frequent indications, however, we found only suspected intrauterine growth restriction as an indication for admission and delivery was found to be so. Thus, it seems that in almost all situations counseling patients can be based primarily on gestational age along with other factors including estimated fetal weight, sex, race, plurality, and completion of a course of antenatal corticosteroids.

Bakri balloon effectiveness for postpartum hemorrhage: a "real world experience".

INTRODUCTION: The Bakri balloon has been advocated for management of obstetric hemorrhage, based on several small studies (2-16 patients) where it was over 90% effective. OBJECTIVE: To estimate the effectiveness of the Bakri balloon for treating postpartum hemorrhage (PPH) in clinical practice at academic institutions. METHODS: This is a retrospective cohort study evaluating efficacy of Bakri balloon use in 35 women with PPH, performed as a chart review. Charts were reviewed to determine if balloon placement was deemed a "success" or "failure", as well as to abstract relevant demographic and clinical factors. Failure was defined as need for another form of hemorrhage control. RESULTS: Success rate was 67.57%. Bakri failure was associated with Cesarean section (67% versus 16%, p = 0.031) and predelivery Pitocin (67% versus 28%, p = 0.003) and had more ICU admissions (58% versus 4%, p = 0.0003), transfusions (5.4 red blood cell units versus 1.6, p = 0.007) and hospital days (5.65 versus 3.75, p = 0.011). Reasons for failure were continued bleeding or balloon extrusion. CONCLUSION: Our results suggest that the Bakri balloon is useful for treating PPH but not as effective as previously published. When balloon use is effective, however, the maternal morbidity is significantly decreased.

Evaluation of fetuses in a study of intravenous immunoglobulin as preventive therapy for congenital heart block: Results of a multicenter, prospective, open-label clinical trial.

OBJECTIVE: The recurrence rate of anti-SSA/Ro-associated congenital heart block (CHB) is 17%. Sustained reversal of third-degree block has never been achieved. Based on potential reduction of maternal autoantibody titers as well as fetal inflammatory responses, intravenous immunoglobulin (IVIG) was evaluated as preventive therapy for CHB. METHODS: A multicenter, prospective, open-label study based on Simon's 2-stage optimal design was initiated. Enrollment criteria included the presence of anti-SSA/Ro antibodies in the mother, birth of a previous child with CHB/neonatal lupus rash, current treatment with < or = 20 mg/day of prednisone, and <12 weeks pregnant. IVIG (400 mg/kg) was given every 3 weeks from week 12 to week 24 of gestation. The primary outcome was the development of second-degree or third-degree CHB. RESULTS: Twenty mothers completed the IVIG protocol before the predetermined stopping rule of 3 cases of advanced CHB in the study was reached. CHB was detected at 19, 20, and 25 weeks; none of the cases occurred following the finding of an abnormal PR interval on fetal Doppler monitoring. One of these mothers had 2 previous children with CHB. One child without CHB developed a transient rash consistent with neonatal lupus. Sixteen children had no manifestations of neonatal lupus at birth. No significant changes in maternal titers of antibody to SSA/Ro, SSB/La, or Ro 52 kd were detected over the course of therapy or at delivery. There were no safety issues. CONCLUSION: This study establishes the safety of IVIG and the feasibility of recruiting pregnant women who have previously had a child with CHB. However, IVIG at low doses consistent with replacement does not prevent the recurrence of CHB or reduce maternal antibody titers.

Collaboration between obstetricians and neonatalogists: perinatal safety programs and improved clinical outcomes.

Although collaboration between obstetricians and neonatologists may seem an obvious way to provide the best quality care to infants and their mothers, this has not always occurred. This article highlights the experiences of several recently published efforts demonstrating how coordinated care resulted in improved clinical outcomes.

Reduction of elective inductions in a large community hospital.

OBJECTIVE: Our goal was to lower unplanned primary cesarean deliveries by reducing elective inductions. STUDY DESIGN: To implement and sustain an induction management program, a committee of care providers reviewed induction rates. "Elective" and other categories were defined. An induction consent form was drafted. Consent compliance, induction rates, hours in labor and delivery and mode of delivery were evaluated. Outcomes were compared with historical data from 2 years earlier. RESULTS: A total of 10,166 nulliparas and 9869 multiparas attempted vaginal deliveries. Elective inductions decreased significantly, from 4.3% to 0.8% in nulliparas and from 13% to 9.5% in multiparas. A longer time to delivery was seen for both nulliparas (5.2 hours) and multiparas (4 hours) with elective inductions. Unplanned primary cesarean delivery rates are significantly lower in spontaneously laboring women, compared with those induced. CONCLUSION: A program aimed at reducing elective inductions was successfully implemented and sustained.