Kevin Leon

Extensive surgical and comprehensive postoperative medical management for cystic fibrosis chronic rhinosinusitis

Background Chronic rhinosinusitis has a major impact on the quality of life of patients with cystic fibrosis (CF) and may contribute to progression of chronic lung disease. Despite multiple sinus surgeries, maxillary sinus involvement is a recurrent problem. The modified endoscopic medial maxillectomy (MEMM) permits debridement in the clinic, improves mucus clearance with nasal irrigations, and increases access for topical delivery of therapeutics. However, clinical outcomes of aggressive sinus surgery with regimented postoperative medical treatment have not been systematically evaluated. Methods CF patients completed the 22-Item Sinonasal Outcome Test questionnaires before sinus surgery (and bilateral MEMM) and at sequential postoperative visits. Objective measures included Lund-Kennedy endoscopic score and pulmonary function tests (forced expiratory volume at 1 second percent [FEV1%] predicted). Culture-directed antibiotic therapy, prednisone, and topical irrigations were initiated postoperatively. Results Twenty-two patients (mean age, 26.5 years; 4.9 prior sinus operations) underwent MEMM and sinus surgery. Symptom scores were significantly reduced at 60 days (primary outcome, 64.7 ± 18.4 presurgery versus 27.5 ± 15.3 postsurgery; p < 0.0001) and up to a year postoperatively (27.6 ± 12.6; p < 0.0001). Endoscopic scores were also reduced after surgery (10.4 ± 1.1 presurgery versus 5.7 ± 2.4 [30 days], 5.7 ± 1.4 [60 days], 5.8 ± 1.3 [120 days], and 6.0 ± 1.1 [1 year]; p < 0.0001)]. There were no differences in FEV1% predicted up to 1 year postoperatively, but hospital admissions secondary to pulmonary exacerbations significantly decreased (2.0 ± 1.4 versus 3.2 ± 2.4, respectively; p < 0.05). Conclusion Prospective evaluation indicates sinus surgery with MEMM is associated with marked improvement in sinus disease outcomes. Additional studies are necessary to confirm whether this treatment paradigm is associated with improved CF pulmonary disease.

Genetic and Reproductive Knowledge Among Adolescents and Adults With Cystic Fibrosis

Studies1,2 have shown that cystic fibrosis (CF) patients have limited knowledge of the genetics of CF. Previously, there was limited need to communicate this information: few CF patients lived to adulthood, and most who did could not reproduce.1,2 Genetic counseling focused on the patient’s parents, who were counseled about the recurrence risk at the time of the diagnosis. Today, CF is a disease of adulthood.3 In 2002, 40% of CF patients in the United States were 18 years old; by 2010, it will be 50%. Together with advances in assisted reproductive technology, reproduction and recurrence risk are now important issues for adolescent and young adult CF patients.3–6 A 19-item questionnaire was developed from the results of prior semistructured interviews with 18 CF patients aged 16 to 25 years. Knowledge-based questions (medical issues, inheritance, and reproductive options/risks) as well as communication patterns (preferred resources for learning about CF and preferred people with whom to talk about reproductive issues) were addressed. Recruited from the University of Alabama CF clinic population, 51 patients aged 15 to 29 years (mean, 21 years), 24 male (47%) and 27 female (53%), completed the questionnaire. The study was approved by the University of Alabama Institutional Review Board. Regarding autosomal recessive inheritance, only 33% knew that two carriers have a 25% chance of having a child with CF, and 25% knew that two carriers have a 50% chance of having a child who is a carrier. However, 82% knew that two carriers could have a child who did not have CF, and 52% knew that two carriers could have a child who did not carry CF. On their own reproductive risks, 59% knew that a CF patient had a 0% chance of having a child with CF if their partner was not a carrier, but only 26% knew that all their children would be carriers even if their partner was not a carrier. In the scenario of a CF patient with a CF carrier partner, 44% knew that a child had a 50% chance of having CF, and 24% knew that a child had a 50% chance of being a CF carrier. Most patients knew about their reproductive potential, as 96% responded that CF patients are able to have children. However, when asked about whether the chance for having children was different for male and female patients with CF, 65% answered that it was more difficult for men, 8% that it was more difficult for women, and 27% answered “not sure.” While 62% reported that they knew that there were options for male CF patients who wanted to have children, only 26% knew of assisted reproductive technology. Despite widespread availability, the lack of knowledge of adolescents and young adults with CF about the genetics of their disease continues. Furthermore, these patients are unaware of both modern technologies that could enable them to have biological children and the risk of those children having CF. This study illustrates the changing needs of patient education as medical knowledge progresses. CF patients would benefit from further genetic knowledge and counseling to enable them to make informed decisions about reproduction as they mature into adulthood.

Salvaging the dehisced lung transplant bronchial anastomosis with homograft aorta

This is a case of 50-year-old male who underwent left single lung transplantation for pulmonary fibrosis. He sustained a bronchial dehiscence with a pulmonary artery-bronchial fistula which was primarily repaired. One week later, there was complete bronchial dehiscence followed by a massive hemoptysis. At operation, following resection of necrotic donor bronchus there was a sizeable gap between donor and recipient bronchus, which was bridged with a cryopreserved aortic homograft. The homograft patch provided a satisfactory repair without malacia. The patient required retransplantation six months later for reasons unassociated with the repair. Homograft aorta proved useful material for salvaging the dehisced lung transplant bronchial anastomosis.

Left Atrial Mural Endocarditis Secondary to Mitral Valve Jet Lesion

A 38-year-old woman with Down syndrome and mitral valve prolapse presented with 2 weeks of fever and cough. She had presented to an outpatient clinic 1 week earlier and was prescribed empirical antibiotics for a suspected respiratory tract infection. Despite this therapy, she continued to have high spiking fevers and developed a fulminant rash, prompting emergent admission for further evaluation. She had previously been healthy without other risk factors for infective endocarditis. On admission to the intensive care unit, she was febrile, confused, tachycardic, and hypotensive. Examination showed peripheral signs of septic embolization, including Janeway lesions (Figure, A), splinter hemorrhages (Figure, B), Osler nodes (Figure, C), and conjunctival petechiae (Figure, D). An IV/VI plateau–shaped holosystolic murmur was heard in the mitral area with radiation to the axilla. Laboratory studies revealed neutrophilic leukocytosis and lactic acidosis. Transthoracic echocardiography showed myxomatous morphology of the mitral valve with holosystolic prolapse of the anterior mitral leaflet (Figure, E and Movie I in the online-only Data Supplement). Color-flow Doppler showed severe eccentric mitral regurgitation with a posteriorly directed regurgitant jet (Figure, F and Movie II in the online-only Data Supplement). Jet velocities in excess of 4 …

Educating the Adolescent and Young Adult With Cystic Fibrosis About Their Reproductive Risks and Options

Correspondence Affi liations: From the North Bristol NHS Trust Lung Centre , Southmead Hospital. Financial/nonfi nancial disclosures: The author has reported to CHEST that no potential confl icts of interest exist with any companies/organizations whose products or services may be discussed in this article . Correspondence to: Andrew R. L. Medford, MBChB, MD, FCCP, North Bristol NHS Trust Lung Centre, Southmead Hospital, Southmead Rd, Westbury-on-Trym, Bristol, BS10 5NB, England; e-mail: andrewmedford@hotmail.com

Implementation of a successful eradication protocol for Burkholderia Cepacia complex in cystic fibrosis patients

BackgroundInfection with Burkholderia cepacia complex (Bcc) results in a heterogeneous clinical course ranging from asymptomatic colonization of the airways to fulminant respiratory failure in patients with cystic fibrosis (CF). Early eradication of Pseudomonas aeruginosa improves clinical outcomes. The efficacy and clinical outcomes following implementation of an eradication protocol for Bcc are less well understood.MethodsWe developed and implemented a single center Bcc eradication protocol that included an intensive combination of intravenous, inhaled, and oral antibiotic therapies based on in vitro sensitivities. We conducted a retrospective cohort analysis of clinical outcomes compared to patients with chronic Bcc infection.ResultsSix patients were identified as having a newly acquired Bcc colonization and were placed on the eradication protocol. Sequential sputum samples after completion of the protocol demonstrated sustained clearance of Bcc in all patients. Lung function and nutritional status remained stable in the year following eradication.ConclusionClearance of Bcc from sputum cultures using a standardized protocol was successful at one year and was associated with clinical stability.

Lung injury and the cinnamon challenge: College students should beware this internet dare

A recent article in Pediatrics warns against inhaling cinnamon powder and cites experiments that demonstrated chronic inflammation and fibrotic changes to the pulmonary tissue of rats forced to inhale cinnamon powder, and was written in response to the growing popularity of the cinnamon challenge.1 The cinnamon challenge has existed for nearly a decade.2 This dare consists of consuming one tablespoon of cinnamon powder within one minute without drinking any fluids.3 The cinnamon challenge has become more popular among teenagers and young adults over the past four years.2 According to the American Association of Poison Control Centers (AAPCC), the number of calls to poison control centers concerning teenagers ages 13 to 19 who had undertaken the cinnamon challenge increased from 51 in 2011 to 222 in 2012. 4 In 2013, from January 1 to July 31, 37 such exposures were reported to poison control centers. 4 Not all persons who intentionally abuse or misuse cinnamon powder will need medical attention. Of the 122 calls classified as “intentional misuse or abuse” during the first three months of 2012, 30 required medical attention. 5 However, episodes in which medical attention is warranted may be underreported. Cinnamon is a caustic powder composed of cellulose fibers, which neither dissolve nor biodegrade in the lungs.1 The powder quickly dries out the mouth, which makes swallowing the powder very difficult. Coughing and burning sensations in the mouth, nose, and throat ensue. More serious symptoms include vomiting, epistaxis, and chest tightness1 and even atelectasis.6 Furthermore, the AAPCC warns that teenager with asthma or other pre-existing respiratory problems are at greater risk for developing respiratory distress from the cinnamon challenge.5 General internists and family practitioners who provide on-campus outpatient services to college students might encounter students with respiratory problems secondary to participating in the cinnamon challenge. Peer pressure is an appreciable factor among both children and young adults concerning participation in the cinnamon challenge.1 College students might undertake the challenge as part of their initiation into a student-led organization. Persons with an allergy to cinnamon or a pre-existing respiratory condition are encouraged to disclose their condition to friends or acquaintances who participate in the cinnamon challenge so that they are less likely to be pressured into following suit. In conclusion, college students are a population at risk for respiratory complications secondary to cinnamon toxicity. Educational efforts that highlight the dangers of the cinnamon challenge should be implemented on college campuses in addition to middle school, junior high school, and high school campuses. This Internet dare is not a concern just for pediatricians, but also for general internists, family practitioners, and pulmonologists.