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Pulmonary fibrosis
Published in Anthony J. Hickey, Heidi M. Mansour, Inhalation Aerosols, 2019
Priya Muralidharan, Don Hayes, Heidi M. Mansour
Despite the etiology of IPF remaining elusive, inflammation, oxidative stress, and coagulation disturbances have been explored for their involvement in IPF (11). Unfortunately, there is no panacea for IPF, but clinical treatment includes pharmacological intervention, symptom relief, and co-morbidity management (12). Figure 18.2 identifies the different treatment modalities for IPF management as recognized by ATS. When these therapies fail to stabilize lung function, the patient is considered for single or bilateral lung transplantation. If the patient is deemed an appropriate lung transplant candidate, the wait time for a matching donor can be lengthy, hence emphasizing the need for a cure. Until recently, there was no therapies specifically marketed to treat IPF. Two drugs—pirfenidone (Esbriet®) and nintedanib (Ofev®)—were the first FDA-approved drugs for IPF treatment. Both the drugs received US FDA approval in 2014 following successful results from the ASCEND and INPULSIS clinical trials. Pirfenidone and nintedanib both slow the progression of the disease in mild to moderate IPF. Pirfenidone was approved in Japan in 2008, Europe in 2011, and in the United States in 2014. That same year, nintedanib also received approval for IPF treatment in the United States (13). This was seen as the first anticipatory step toward advancement in treating IPF, a devastating chronic respiratory disease. Both medications only slow disease progression, so a lack of curative drugs (14) is a motivating force behind drug development for this disease.
Life Care Planning for Organ Transplantation
Published in Roger O. Weed, Debra E. Berens, Life Care Planning and Case Management Handbook, 2018
Immediately upon arrival to the ICU, extensive and specific postoperative monitoring is begun to include laboratory work, hemodynamic monitoring, fluid assessment, and administration of immunosuppressive medications, generally cyclosporine or Prograf. Daily drug levels will be drawn to monitor cyclosporine or Prograf levels in the blood. Patients receiving liver, lung, or heart transplants will remain on mechanical ventilation for at least a few hours. For many lung transplants, mechanical ventilation is required for as long as 3 days.
Blood and immune system disorders
Published in Steve Hannigan, Inherited Metabolic Diseases: A Guide to 100 Conditions, 2018
This disorder can be diagnosed by blood tests that measure the level of alpha-1-antitrypsin and the phenotype in the blood. A good diet, exercise and the avoidance of smoking and alcohol will increase the likelihood of long-term health. Prolastin treatment is efective for lung disease, and antibiotics may be used to treat infections. A lung transplant may be necessary in some cases. The liver and lung symptoms can be treated with medication. Symptomatic treatment for liver problems is required, and a transplant may be necessary. Enzyme replacement therapy for this disorder is available.
The healthcare resource utilization and costs of chronic lung allograft dysfunction following lung transplantation in patients with commercial insurance in the United States
Published in Journal of Medical Economics, 2022
Ajay Sheshadri, Naomi C. Sacks, Bridget Healey, Phil Cyr, Gerhard Boerner, Howard J. Huang
Lung transplantation is the standard of care for patients with irreversible end-stage lung disease1. According to the Organ Procurement and Transplantation database, for over 30 years, nearly half (49.2%) of lung transplant recipients used commercial insurance as the source of payment for their procedure, and a majority (75.2%) were between 18 and 64 years old2,3. The long-term success of lung transplantation depends on selective donor-recipient pairing, effective surgery and post-surgical management, comprehensive screening for acute or chronic rejection, appropriate initiation and maintenance of immunosuppression, and mitigating the impact of infections4. Over time, outcomes among lung transplant recipients have improved, but median survival after lung transplantation remains only about six years, particularly low when compared with other organ transplantation procedures5,6.
Diagnosing and managing bronchiolitis obliterans in children
Published in Expert Review of Respiratory Medicine, 2019
Pediatric lung transplantation is a relatively rare procedure, with approximately 100 transplantations performed each year worldwide. The commonest indications in the pediatric population are cystic fibrosis, idiopathic pulmonary hypertension and BO [15]. Long-term survival of lung transplant patients is poorer than for other solid organs transplantations, with approximately 50% five-year survival. In the early stages post-transplantation, the greatest risks are infection and graft failure. The most common cause for long-term poor outcome is chronic lung allograft dysfunction (CLAD), of which bronchiolitis obliterans syndrome (BOS) is a subtype. BOS affects 50% or more of recipients who survive beyond 5 years and accounts for a considerable proportion of cases of lung allograft loss and recipient death beyond 3 months post-transplant [16].
An update on current and emerging treatments for pulmonary arterial hypertension in childhood and adolescence
Published in Expert Review of Respiratory Medicine, 2019
Julie Wacker, Robert Weintraub, Maurice Beghetti
Targeted therapies developed over the last two decades can slow clinical worsening in varying forms of PAH, but curative therapy remains elusive. Following the introduction of the first targeted therapies more than 10 years ago, new agents are developed among the families of drugs targeting the three known pathogenic pathways, with potential improvements of side effects, drug interaction and the number of daily administration. Continuous infusion of prostanoids (subcutaneous or intravenous) is still necessary in severe cases of PAH, as it remains the most potent agent, but is not without risks and side effects, and implies major life adjustments for patients and families. An oral formulation of prostanoid has been developed but has so far not proven to be substitutable for intravenous epoprostenol. Finding an oral equivalent of epoprostenol remains an ongoing challenge. The timing of lung transplant is difficult to determine, and the median survival after a lung transplant is only about 6 years. Surgical strategies (Potts shunt, atrial septostomy) can in some scenarios delay the need for a lung transplant. Selecting the right patients likely to benefit from those surgical interventions remains a challenge. In most centers, referral to lung transplantation service is only considered when patients are on functional class III or IV on full medical therapy or for patients with rapidly progressive disease.