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Quality of Life Among Males with Adrenoleukodystrophy Following Hematopoietic Stem Cell Transplant
Abstracts / Biol Blood Marrow Transplant 22 (2016) S19eS481
process of daily bathing using wipes, documentation, patient/family education, and ordering of discharge bath supplies. Outpatient staff required instruction in the process for protocol consent, skin swab collection, review of a daily bath diary, and a patient satisfaction survey. Findings & Interpretation: Evaluation of the protocol’s implementation process included monitoring of 12 study inpatients. Evaluation included 4 criteria: correct selection of bath wipe, maintaining blinding, correct process for bath administration, and proper documentation. Of the 12 patients, 7 met all 4 evaluation criteria. Of the 5 who did not, education was provided on site. Discussion & Implications: A critical component of research and maintaining internal validity of a study is ongoing monitoring to determine if the protocol is being implemented correctly. We plan to continue our evaluation on an ongoing basis and provide education as needed. We also plan to add the outpatient evaluation component.
TRANSPLANT NURSING-RESEARCH
738 Quality of Life Among Males with Adrenoleukodystrophy Following Hematopoietic Stem Cell Transplant Nicole Beckmann. Pediatric Blood and Marrow Transplant, University of Minnesota Masonic Children’s Hospital, Minneapolis, MN Topic Significance & Study Purpose/Background/ Rationale: Hematopoietic stem cell transplant (HSCT) with an HLA matched donor or umbilical cord blood is the only treatment for halting the progression of child-onset cerebral X-linked adrenoleukodystrophy (ALD). Following bone marrow transplant, some children with ALD remain asymptomatic or achieve stabilization of disease but have life-long challenges related to the neurological deterioration that occurred prior to stabilization. This study examined the health related quality of life (HRQoL) of children with ALD following HSCT. Methods, Intervention, & Analysis: Researchers conducted a retrospective study of HRQoL with 10 males diagnosed with ALD that underwent HSCT (7¼matched sibling, 3¼umbilical cord blood). Participants had a median age of 8 years at HSCT and 15 years of age at survey with a median of 6.5 years since HSCT. Children completed subscales from the Neuro-QoL and the PROMIS Pediatric Profile Instrument representing physical, mental, and social health domains. Parents assisted children or provided parent proxy reports for children unable to answer question independently. Spearman rho correlations were calculated to identify statistically significant associations between subscales. Findings & Interpretation: Of the 10 participants, 3 had significant auditory, visual, or motor deficits following HSCT. Statistically significant relationships were found between declining cognitive impairment and increasing symptoms of anxiety (rs¼ -.79, p¼ .014), stigma (rs¼ -.79, p¼ .014), anger (rs¼ -.79, p¼ .012), and decreased peer interaction. (rs¼ .70, p¼ .037). Increasing depressive symptoms were associated with higher levels of anxiety (rs¼ .83, p¼ .003), decreased peer interaction (rs¼ -.78, p¼ .007), and higher stigma (rs¼ .76, p¼ .010). All participants reported good physical mobility. Discussion & Implications: Although HSCT halts progression of the ALD disease, cognitive impairment increases risk of emotional and social problems that may impact the childrenÕs ability to adapt following HSCT. Further
S469
longitudinal monitoring of HRQoL is necessary to describe how HRQoL changes over time following HSCT and determine supportive care to minimize negative psychosocial outcomes.
739 TKO - Defining a Practice in the HPSC Population: A Prospective Cohort Study Jennifer Blaney 1, Donna Quiroz 2. 1 Bone Marrow Transplant, Childrens Hospital, Los Angeles, Porter Ranch, CA; 2 Children’s Hospital Los Angeles, Los Angeles, CA Topic Significance & Study Purpose/Background/ Rationale: The phrases “to keep open” (TKO) or “keep vein open” (KVO) are well known phrases throughout the medical and nursing in the literature citing a particular rate that is supported by evidence. In critically ill hematology/oncology patients, fluid balance management is an integral part of the process of care. A TKO rate of 10ml per hour on a CVC adds 480ml of fluid per day. Decreasing the volume of this fluid would be a benefit to patients whose fluid management is precarious and unnecessary doses of furosemide could be eliminated. Methods, Intervention, & Analysis: Between April 1, 2014 and September 30, 2014 a total of 80 admissions were in the control group. The median age was 7.5 years. In all, 42 (52.2%) were male and 38 were female (47.5%). The control group was compared to the intervention group of patients admitted between October 1, 2014 and March 31, 2015, for a total of 66 admissions. The median age in the intervention group was 6 years. In that group, 41 (59%) were male and 19 (41%) were female. The control group infused a TKO rate of 10ml per hour while the intervention group infused a TKO rate of 5ml per hour. A Chi-square test was used to calculate whether there was a significant difference between the two groups. Findings & Interpretation: The results of the statistical analysis were that there was no significant difference between infusing a TKO rate of 10ml per hour and 5ml per hour. The P value was 0.804202. There was no increase use of tPA when infusing a rate of 5ml per compared to 10ml per hour. Discussion & Implications: Along with GVHD, SOS is one of the most frequently encountered serious complications from HSCT. The incidence of SOS ranges from 5% to more than 60% in children (Barker, et al, 2003). Supportive treatment revolves around management of fluid using diuretics. Halving the rate of fluid infusing could prevent them from extra doses of diuretics and aid in fluid restriction.
740 A Study of Risk Factors Associated with High Doses of Ionizing Radiation Exposure from Diagnostic Computerized Tomographic Scans in Children Undergoing Allogeneic Hematopoietic Cell Transplant Paige Silverman 1, Courtney Briamonte 2, Komal Kothari 3, Sejal Bavishi 4, Manan Bavishi4, James So 5, Carrie Ruzal Shapiro 5, Xiaoyu Liu6, Zhezhen Jin 7, Prakash Satwani 2. 1 Division of Pediatric Blood and Marrow Transplantation, Morgan Stanley Children’s Hospital New York Presbyterian, New York, NY, New York, NY; 2 Division of Pediatric Hematology, Oncology, and Stem Cell Transplantation, Department of Pediatrics, Columbia University Medical Center, New York, NY; 3 Columbia University, NY, New York, NY; 4 Department of Pediatrics, Columbia University Medical Center, New York, NY; 5 Department of Radiology, Columbia University Medical Center, New York, NY; 6 Mailman School of Public Health, Columbia University, New York, NY; 7 Biostatistics, Columbia University, New York, NY
process of daily bathing using wipes, documentation, patient/family education, and ordering of discharge bath supplies. Outpatient staff required instruction in the process for protocol consent, skin swab collection, review of a daily bath diary, and a patient satisfaction survey. Findings & Interpretation: Evaluation of the protocol’s implementation process included monitoring of 12 study inpatients. Evaluation included 4 criteria: correct selection of bath wipe, maintaining blinding, correct process for bath administration, and proper documentation. Of the 12 patients, 7 met all 4 evaluation criteria. Of the 5 who did not, education was provided on site. Discussion & Implications: A critical component of research and maintaining internal validity of a study is ongoing monitoring to determine if the protocol is being implemented correctly. We plan to continue our evaluation on an ongoing basis and provide education as needed. We also plan to add the outpatient evaluation component.
TRANSPLANT NURSING-RESEARCH
738 Quality of Life Among Males with Adrenoleukodystrophy Following Hematopoietic Stem Cell Transplant Nicole Beckmann. Pediatric Blood and Marrow Transplant, University of Minnesota Masonic Children’s Hospital, Minneapolis, MN Topic Significance & Study Purpose/Background/ Rationale: Hematopoietic stem cell transplant (HSCT) with an HLA matched donor or umbilical cord blood is the only treatment for halting the progression of child-onset cerebral X-linked adrenoleukodystrophy (ALD). Following bone marrow transplant, some children with ALD remain asymptomatic or achieve stabilization of disease but have life-long challenges related to the neurological deterioration that occurred prior to stabilization. This study examined the health related quality of life (HRQoL) of children with ALD following HSCT. Methods, Intervention, & Analysis: Researchers conducted a retrospective study of HRQoL with 10 males diagnosed with ALD that underwent HSCT (7¼matched sibling, 3¼umbilical cord blood). Participants had a median age of 8 years at HSCT and 15 years of age at survey with a median of 6.5 years since HSCT. Children completed subscales from the Neuro-QoL and the PROMIS Pediatric Profile Instrument representing physical, mental, and social health domains. Parents assisted children or provided parent proxy reports for children unable to answer question independently. Spearman rho correlations were calculated to identify statistically significant associations between subscales. Findings & Interpretation: Of the 10 participants, 3 had significant auditory, visual, or motor deficits following HSCT. Statistically significant relationships were found between declining cognitive impairment and increasing symptoms of anxiety (rs¼ -.79, p¼ .014), stigma (rs¼ -.79, p¼ .014), anger (rs¼ -.79, p¼ .012), and decreased peer interaction. (rs¼ .70, p¼ .037). Increasing depressive symptoms were associated with higher levels of anxiety (rs¼ .83, p¼ .003), decreased peer interaction (rs¼ -.78, p¼ .007), and higher stigma (rs¼ .76, p¼ .010). All participants reported good physical mobility. Discussion & Implications: Although HSCT halts progression of the ALD disease, cognitive impairment increases risk of emotional and social problems that may impact the childrenÕs ability to adapt following HSCT. Further
S469
longitudinal monitoring of HRQoL is necessary to describe how HRQoL changes over time following HSCT and determine supportive care to minimize negative psychosocial outcomes.
739 TKO - Defining a Practice in the HPSC Population: A Prospective Cohort Study Jennifer Blaney 1, Donna Quiroz 2. 1 Bone Marrow Transplant, Childrens Hospital, Los Angeles, Porter Ranch, CA; 2 Children’s Hospital Los Angeles, Los Angeles, CA Topic Significance & Study Purpose/Background/ Rationale: The phrases “to keep open” (TKO) or “keep vein open” (KVO) are well known phrases throughout the medical and nursing in the literature citing a particular rate that is supported by evidence. In critically ill hematology/oncology patients, fluid balance management is an integral part of the process of care. A TKO rate of 10ml per hour on a CVC adds 480ml of fluid per day. Decreasing the volume of this fluid would be a benefit to patients whose fluid management is precarious and unnecessary doses of furosemide could be eliminated. Methods, Intervention, & Analysis: Between April 1, 2014 and September 30, 2014 a total of 80 admissions were in the control group. The median age was 7.5 years. In all, 42 (52.2%) were male and 38 were female (47.5%). The control group was compared to the intervention group of patients admitted between October 1, 2014 and March 31, 2015, for a total of 66 admissions. The median age in the intervention group was 6 years. In that group, 41 (59%) were male and 19 (41%) were female. The control group infused a TKO rate of 10ml per hour while the intervention group infused a TKO rate of 5ml per hour. A Chi-square test was used to calculate whether there was a significant difference between the two groups. Findings & Interpretation: The results of the statistical analysis were that there was no significant difference between infusing a TKO rate of 10ml per hour and 5ml per hour. The P value was 0.804202. There was no increase use of tPA when infusing a rate of 5ml per compared to 10ml per hour. Discussion & Implications: Along with GVHD, SOS is one of the most frequently encountered serious complications from HSCT. The incidence of SOS ranges from 5% to more than 60% in children (Barker, et al, 2003). Supportive treatment revolves around management of fluid using diuretics. Halving the rate of fluid infusing could prevent them from extra doses of diuretics and aid in fluid restriction.
740 A Study of Risk Factors Associated with High Doses of Ionizing Radiation Exposure from Diagnostic Computerized Tomographic Scans in Children Undergoing Allogeneic Hematopoietic Cell Transplant Paige Silverman 1, Courtney Briamonte 2, Komal Kothari 3, Sejal Bavishi 4, Manan Bavishi4, James So 5, Carrie Ruzal Shapiro 5, Xiaoyu Liu6, Zhezhen Jin 7, Prakash Satwani 2. 1 Division of Pediatric Blood and Marrow Transplantation, Morgan Stanley Children’s Hospital New York Presbyterian, New York, NY, New York, NY; 2 Division of Pediatric Hematology, Oncology, and Stem Cell Transplantation, Department of Pediatrics, Columbia University Medical Center, New York, NY; 3 Columbia University, NY, New York, NY; 4 Department of Pediatrics, Columbia University Medical Center, New York, NY; 5 Department of Radiology, Columbia University Medical Center, New York, NY; 6 Mailman School of Public Health, Columbia University, New York, NY; 7 Biostatistics, Columbia University, New York, NY