Samya Nasr

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P191 Quality improvement project to improve pulmonary function in paediatric cystic fibrosis patients

Journal of Cystic Fibrosis, Jun 1, 2019

The Use of Home Spirometry in Pediatric Cystic Fibrosis Patients

Global pediatric health, 2017

Medication adherence is poor among pediatric cystic fibrosis (CF) patients, with adolescents havi... more Medication adherence is poor among pediatric cystic fibrosis (CF) patients, with adolescents having one of the lowest adherence rates. We wanted to identify an adherence intervention that would be acceptable to CF adolescents and assess its feasibility. We surveyed 40 adolescents with CF and asked about barriers to and motivators for their own adherence and to generate ideas for potential adherence interventions. Since most of the respondents chose frequent spirometry at home and medication reminders for interventions, we selected 5 subjects, 10 to 14 years of age, with CF to test the feasibility of home spirometry and medication reminders in pediatric CF patients. This article summarizes the results of both the survey and the feasibility pilot study.

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Hypervitaminosis A Causing Hypercalcemia in Cystic Fibrosis. Case Report and Focused Review

Annals of the American Thoracic Society, Oct 1, 2014

Hypercalcemia is a rare complication of hypervitaminosis A. We report a pediatric patient with cy... more Hypercalcemia is a rare complication of hypervitaminosis A. We report a pediatric patient with cystic fibrosis (CF) and pancreatic insufficiency who was found to have hypervitaminosis A causing hypercalcemia, complicated by nephrocalcinosis and renal impairment. The patient is a 4-year-old girl with pancreaticinsufficient CF, gastroesophageal reflux, oral aversion, and failure to thrive requiring gastrostomy tube placement. She was prescribed Source CF vitamins, but rarely received the full dose, due to emesis and intolerance. She had routine annual labs that revealed hypercalcemia with elevated blood urea nitrogen and creatinine, which were not present in her previous annual labs. Upon further questioning, her mother reported that she seemed more fatigued for a few weeks, had abdominal pain, and was urinating more frequently. Upon admission to the hospital, laboratory results revealed elevated HCO3, while serum levels of potassium, phosphorus, and albumin were within normal limits. Vitamin D (25-hydroxy) level was low, and vitamin A level was elevated. Extensive metabolic and hormonal workup for the etiology of the hypercalcemia revealed evidence of chronic renal insufficiency and elevated vitamin A levels. She had a renal ultrasound that revealed bilateral nephrocalciosis. Diagnosis of chronic hypervitaminosis A complicated by hypercalcemia was made and was managed by holding vitamin A supplements, aggressive diuresis, and prednisolone. This case emphasizes the importance of regular vitamin A monitoring in patients with CF. There is a wide variability for the lowest intake required to cause toxicity, and the lower limit to cause toxicity has not been determined.

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An 8-week behavioral intervention can improve weight gain in children with cystic fibrosis

The Journal of Pediatrics, May 1, 2006

282 Annual respiratory assessment and re-education of CF patients and families, a quality improvement project

Journal of Cystic Fibrosis, Jun 1, 2012

Goal: To develop annual standardized respiratory assessment tool to improve care. Methods: A resp... more Goal: To develop annual standardized respiratory assessment tool to improve care. Methods: A respiratory assessment (ARA) tool was developed. Patients/families were asked about CF knowledge, medication action, airway clearance and proper device cleaning. They were asked to bring all equipment to the assessment. It took about 40 minutes to complete. During the session, the Respiratory Therapist reviewed airway clearance techniques, equipment fit, settings and cleaning/use of equipment and inhaled medications. The family was assisted with replacement of any equipment, as indicated. Contact with homecare and DME suppliers was facilitated. A CF respiratory guidebook was developed and given to families at the end of the session. It includes basic information about day to day care, airway clearance techniques, nebulizers, disinfecting, and contact information. The ARA form is reviewed and signed by the pulmonologist and saved in patient's record. Results: 70 assessments were completed to date. 65% of those assessed reported doing prescribed airway clearance almost every day. Of those that were old enough to cough or "huff cough" 60% reported not knowing the importance of cough/ huff with airway clearance techniques. Only 52% had proper cleaning technique. 80% used the correct order of therapy, and 75% used the correct nebulizer for the correct medication. 70% were able to articulate the action of each medication. Discussion: Patients with CF have a complex treatment regimen. Knowledge gaps were identified and addressed in a standardized fashion for patients and families. The next step will be to measure satisfaction and change in knowledge and behavior on a regular basis.

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Correction of vitamin E deficiency with fat-soluble versus water-miscible preparations of vitamin E in patients with cystic fibrosis

The Journal of Pediatrics, May 1, 1993

Of 27 patients with cystic fibrosis, 17 had levels of alpha-tocopherol more than 2 SD below the m... more Of 27 patients with cystic fibrosis, 17 had levels of alpha-tocopherol more than 2 SD below the mean. Patients were randomly given either a water-miscible form of vitamin E or a fat-soluble form for 6 months. Either form was effective in achieving normal serum levels; the fat-soluble supplement has a significant cost advantage.

Appetite-Stimulant Use in the Palliative Care of Cystic Fibrosis

Bleeding diathesis due to vitamin K deficiency in an infant with cystic fibrosis

Respiratory Medicine Cme, 2010

With the wide implementation of newborn screening for Cystic Fibrosis, infants are being diagnose... more With the wide implementation of newborn screening for Cystic Fibrosis, infants are being diagnosed in the presymptomatic phase of the disease. Nutritional deficiencies (hypoalbuminemia) and fat soluble vitamins A, D and E deficiencies, due to pancreatic insufficiency and malabsorption, have been reported in the past at the time of diagnosis. 1,2 Rarely, infants with CF present with severe bleeding disorder, secondary to vitamin K deficiency, in the first months of life. 3 To our knowledge, this is the first case report illustrating bleeding diathesis in a one month old infant with CF. He was diagnosed by newborn screening and presented with a gastrointestinal bleeding due to vitamin K deficient coagulopathy.

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Disability and quality of life in cystic fibrosis from early age to adulthood

Journal of Adolescent Health, Dec 1, 1996

We evaluated the utility of the Social Security disability Insurance (SSD) program and the supple... more We evaluated the utility of the Social Security disability Insurance (SSD) program and the supplemental Security Income (SSI) program in cystic fibrosis (CF) patients with disability. Also, we evaluated the role of health care professionals in increasing the disabled patients awareness of the different resources available for them.

Adherence to treatment in children and adolescent patients with cystic fibrosis

Journal of Adolescent Health, 2006

The purpose of this study was to monitor medication adherence in cystic fibrosis (CF) patients an... more The purpose of this study was to monitor medication adherence in cystic fibrosis (CF) patients and its correlation with disease severity and patient age. Children less than 12 years of age (group 1) and adolescents 12 years of age and older (group 2) were recruited from the University of Michigan CF Center. The study duration was 3 months. A total of 22 patients per group were enrolled. Adherence to ADEKs, an oral multivitamin, and dornase alfa, a nebulized mucolytic medication, was monitored. Adherence to ADEKs was monitored by using the Medication Event Monitoring System (MEMS) SmartCaps (APREX, AARDx, Inc., Union City, California). Dornase alfa adherence rate was monitored by counting empty medication vials. Thirty-three patients completed the study, 15 patients in group 1 and 18 patients in group 2. The overall mean adherence rates for ADEKs and dornase alfa were (+/- SD) 63.6% +/- 24.0% and 66.5% +/- 31.2%, respectively. The median ADEKs and dornase alfa adherence rate for group 1 was 84.6% and 79.1%, respectively (p = .08); and for group 2 was 56.7% vs. 78.4%, respectively (p = .07). There was a trend toward significance, suggesting that the adherence rate for ADEKs was higher than for dornase alfa (p = .08) in group 1. Group 2 showed a trend toward adherence to dornase alfa than to ADEKs (p = .07). There was a trend for ADEKs adherence between groups 1 and 2 (p = .09), but not for dornase alfa (p = .93). Parental supervision and disease severity are likely to play a major role in adherence to medical management. Partnership with patients and families about the treatment plan might be important for improving adherence rate. The MEMS SmartCaps is an electronic monitoring technology that should be used to measure drug adherence objectively both in further larger clinical trials and in the outpatient setting.

379 Utilization and healthcare charges in an insured US cystic fibrosis (CF) patient population

Journal of Cystic Fibrosis, Jun 1, 2011

Objective: To compare biochemical parameters of saliva from CF patients and healthy individuals. ... more Objective: To compare biochemical parameters of saliva from CF patients and healthy individuals. Method: A cross sectional study including saliva samples of 42 CF patients from the Pediatric CF Reference Center of University Hospital of Campinas SP Brazil (UNICAMP) and 35 healthy individuals as controls. Saliva was collected with Salivet ® (Sardest-Germany) cotton roll, chewed for a minute. The amount of saliva was measured and pH and concentrations of bicarbonate, sodium, chloride, potassium, glucose, calcium and lactate, were determined by ABL mod 835 Radiometer ® (Denmark). Student's T and Mann Whitney tests were used for statistical analysis. The project was approved by the Research Ethics Committee. Results: the mean age of samples was 15.03 years (± 6.92 years). Among individuals with CF the mean age was 12.3 years (± 7.02 years) and in the controls, 18.2 (±5.24 years). Statistically significant difference between the two groups was observed for the variables: bicarbonate (p < 0.001), sodium (p = 0.000), chlorine (p = 0.03) and volume (p = 0.000). Averages of these variables in CF and control group, respectively, were: bicarbonate (4. 72 mmol/L; 1. 40 mmol/L) sodium (20.75 mmol/L; 10.50 mmol/L), chloride (30.41 mmol/L; 23.18 mmol/L) and volume (19.32 ml; 54.66 ml). No statistically significant difference was observed between the groups for pH, potassium, calcium, lactate and glucose. Conclusion: Some biochemical salivary parameters seem to be influenced by CF. Other studies are necessary to confirm the usefulness of saliva as complementary, fast and inexpensive tool for CF diagnosis.

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Colonic strictures in children with cystic fibrosis

Radiology, 1995

To determine the radiographic, clinical, surgical, and histologic findings in children with cysti... more To determine the radiographic, clinical, surgical, and histologic findings in children with cystic fibrosis who develop strictures of the colon. Ten children (five boys, five girls; age range, 2.5-9.0 years; mean age, 5.5 years), who were treated at the practices of the authors, were retrospectively identified and their medical records reviewed. Radiographic manifestations of the colonic disease included mucosal irregularity and spiculation with nodular thickening of the colonic wall and loss of normal colonic haustration. Luminal narrowing involved long segments of the colon. Longitudinal shortening of the colon was also a prominent feature. The decrease in caliber of the bowel ranged from mild narrowing to complete occlusion of the lumen. Histologic examination revealed severe submucosal fibrosis and fatty infiltration with transmural extension of the fibrosis to involve the serosa in some cases. Unlike in Crohn disease, however, acute inflammatory changes were minimal or absent. Colonic stricture in children with cystic fibrosis is due to irreversible and frequently progressive narrowing of the colonic lumen.

Development of an Interdisciplinary Telehealth Care Model in a Pediatric Cystic Fibrosis Center

Telemedicine reports, Oct 1, 2021

Background: People with cystic fibrosis (PCF) have unique physical and emotional needs, which are... more Background: People with cystic fibrosis (PCF) have unique physical and emotional needs, which are best met through interdisciplinary care (IDC). In the midst of the pandemic, our center aimed to begin a telehealth care model with an objective to increase successful care visits from baseline of 0-95% by June 26, 2020, including meeting cystic fibrosis (CF) care standards of IDC visits that are coproduced through agenda setting with PCF. Methods: Shifting IDC for pediatric CF patients to telehealth was part of a quality improvement initiative. Our team used asynchronous virtual visits (VVs), with the IDC team members' VVs done on different days than the physician's. Multiple plan-do-study-act cycles were completed to address evolving telehealth needs, including IDC team member flow logistics, communication with PCF, and surveying PCF for the patient perspective. Rates of IDC and agenda setting were measured from March 16, 2020 to June 26, 2020. Results: IDC VVs were at 86% in March 2020 with fluctuations until mid-May when we reached 100% and achieved sustainability. Agenda setting was reached at 100% and maintained. With continued effort, an additional 46.3% of PCF registered for the patient portal, totaling 90.6% with access. Our survey revealed 100% of PCF were able to see IDC team members that they needed to, with 87% ''extremely satisfied'' and 13% ''somewhat satisfied'' with their telehealth experience. Conclusions: Successful telehealth in pediatric CF IDC can be achieved through continuous communication, optimal utilization of available technologies, and may help foster unique opportunities to help improve health outcomes.

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231: Transition from pediatric to adult cystic fibrosis care: Adapting and maintaining using telemedicine

Journal of Cystic Fibrosis, Nov 1, 2021

Use of computerized tomography and chest X-rays in evaluating efficacy of aerosolized recombinant human DNase in cystic fibrosis patients younger than age 5 years: A preliminary study * Presented in part at the International Conference for the American Thoracic Society, April 23–28, 1999, and at ...

High resolution computerized tomography of the chest and pulmonary function testing in evaluating the effect of tobramycin solution for inhalation in cystic fibrosis patients Presented in part at the European Respiratory Society Annual Congress, Glasgow, Scotland from September 4 to 8, 2004, and ...

CF: There's an app for that!

Journal of Cystic Fibrosis, Mar 1, 2020

Addressing nutritional failure in a cystic fibrosis patient using nutritional and psychological intervention

Clinical nutrition ESPEN, Dec 1, 2020

BACKGROUND Malnutrition is associated with Cystic Fibrosis (CF) disease progression. That contrib... more BACKGROUND Malnutrition is associated with Cystic Fibrosis (CF) disease progression. That contributes to decreased lung function, poor quality of life, and increased morbidity and mortality. Appetite stimulants (AS) have been used to help increase caloric intake. When caloric intake is inadequate, gastrostomy tube (GT) feeding is offered and has shown to improve weight gain. In some patients, both AS and GT placement might not be adequate to insure weight gain. That could be due to the underlying psychopathological issues like anxiety and depression that are common in CF. AIM To address malnutrition in CF. METHOD Since increasing oral caloric intake was inadequate to improve weight gain. AS and GT feeding were discussed and offered. AS was started, but was not used regularly. However, GT was still needed to improve weight gain. Because of patient's hesitations, ongoing psychological counseling was done to help patient accept the intervention. RESULTS Addressing patient's depression and anxiety and the failure to gain adequate weight at the same time helped improving patient's nutritional status. CONCLUSION The presented case report demonstrates the improvement in weight gain and body mass index in a CF patient after addressing both the patient's psychological needs and increasing caloric intake through gastrostomy tube feeding.

Lung T1 mapping magnetic resonance imaging in the assessment of pulmonary disease in children with cystic fibrosis: a pilot study

Pediatric Radiology, Mar 11, 2020

Background Assessment tools for early cystic fibrosis (CF) lung disease are limited. Detecting ea... more Background Assessment tools for early cystic fibrosis (CF) lung disease are limited. Detecting early pulmonary disease is crucial to increasing life expectancy by starting interventions to slow the progression of the pulmonary disease with the many treatment options available. Objective To compare the utility of lung T1-mapping MRI with ultrashort echo time (UTE) MRI in children with cystic fibrosis in detecting early stage lung disease and monitoring pulmonary exacerbations. Materials and methods We performed a prospective study in 16 children between September 2017 and January 2018. In Phase 1, we compared five CF patients with normal spirometry (mean 11.2 years) to five age- and gender-matched healthy volunteers. In Phase 2, we longitudinally evaluated six CF patients (median 11 years) in acute pulmonary exacerbation. All children had non-contrast lung T1-mapping and UTE MRI and spirometry testing. We compared the mean normalized T1 value and percentage lung volume without T1 value in CF patients and healthy subjects in Phase 1 and during treatment in Phase 2. We also performed cystic fibrosis MRI scoring. We evaluated differences in continuous variables using standard statistical tests. Results In Phase 1, mean normalized T1 values of the lung were significantly lower in CF patients in comparison to healthy controls ( P =0.02) except in the right lower lobe ( P =0.29). The percentage lung volume without T1 value was also significantly higher in CF patients ( P =0.006). UTE MRI showed no significant differences between CF patients and healthy volunteers ( P =0.11). In Phase 2, excluding one outlier case who developed systemic disease in the course of treatment, the whole-lung T1 value increased ( P =0.001) and perfusion scoring improved ( P =0.02) following therapy. We observed no other significant changes in the MRI scoring. Conclusion Lung T1-mapping MRI can detect early regional pulmonary CF disease in children and might be helpful in the assessment of acute pulmonary exacerbations.

Cystic fibrosis detection in high-risk Egyptian children and CFTR mutation analysis

Journal of Cystic Fibrosis, Apr 1, 2007

Background: Knowledge about Cystic Fibrosis (CF) in Egypt is very limited. The objective of this ... more Background: Knowledge about Cystic Fibrosis (CF) in Egypt is very limited. The objective of this study was to screen for CF in Egyptian children with suggestive clinical features and to identify causative genetic mutations. Methods: Sixty-one patients from the Chest Unit, Cairo University Children's Hospital, Egypt, were included. Subjects presented with persistent or recurrent respiratory symptoms, failure to thrive, diarrhea and/or steatorrhea and unexplained persistent jaundice. Patients were screened using the CF Indicator™ sweat test system (PolyChrome Medical, Inc., Brooklyn Center, MN). A quantitative sweat testing was conducted on 10 of the 12 positive patients. Seven probands and one sibling underwent molecular analysis by direct DNA sequencing of the coding region and of the intronic sequences adjacent to the 27 exons of the CFTR gene. Results: Of 61 patients, 12 (20%) had positive sweat chloride screening. Ten of the 12 patients underwent quantitative sweat testing and were positive. Eight CFTR sequence changes were identified in seven affected probands and two were confirmed in one sibling by direct DNA sequencing. The study results suggest that CF is more common in Egypt than previously anticipated. Larger studies are warranted to identify the incidence, molecular basis and clinical pattern of CF in the Egyptian population.

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