Hydroxyurea And Sickle Cell Anemia: Effect On Quality Of Life

Samir K Ballas; Franca B Barton; Myron A Waclawiw; Paul Swerdlow; James R Eckman; Charles H Pegelow; Mabel Koshy; Duane R Bonds

Disclosures

Conclusion

The quality of life of patients with sickle cell anemia is severely compromised similar to or even worse than patients with other chronic diseases such as arthritis. Patients who responded to hydroxyurea therapy had improvement in certain aspects of quality of life including social function, pain recall, and general health perception, in addition to the decrease in frequency of acute painful episodes, acute chest syndrome, and blood transfusion, responders to hydroxyurea with a pain score >5 achieved significant reduction in the tension scale compared to the placebo group and to non-responders.

Author Contributions

All authors participated in the design and coordination of the study. Samir K. Ballas and Franca B. Barton drafted the manuscript. Franca B. Barton, Myron A. Waclawiw, and Michael L. Terrin performed the statistical analyses and interpreted the results. James R. Eckman and Duane R. Bonds helped in editing the manuscript. All authors read and approved the final manuscript.

1 2 3 4 5

Next Section

Acknowledgments

The authors wish to thank the MSH Investigators listed in references^[8] and^[9] for enrolling patients in this study.

Funding information

Supported in part by NHBLI Cooperative Agreements: UO1 HL 45692, UO1 HL 45696, and Contract NO1-HB-67129.

Reprint Address

Samir K Ballas - samir.ballas@jefferson.edu

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Competing interests: The author(s) declare that they have no competing interests.

Cite this: Hydroxyurea And Sickle Cell Anemia: Effect On Quality Of Life - Medscape - Aug 31, 2006.

Table 1. Comparison of MSH Patients and Other Reference Patient Groups on SF-36 Measures
	Health Status Short Form 36 Scores
Patient Group	Physical Functioning	Role-Physical	Social Functioning	Mental Health	Health Perception	Bodily Pain Recall
No Chronic Conditions* (N = 2595)	86.0	87.2	92.3	77.6	72.6	74.2
Myocardial Infarction* N = 147	64.6	58.6	80.7	74.8	62.7	71.7
Arthritis* N = 2079	81.1	76.9	88.4	74.6	65.3	58.6
Sickle Cell Anemia ^† N = 143	63.6	45.3	65.1	70.0	42.4	44.8
MSH** N = 299	66.0	46.7	6.65.0	70.0	38.8	46.5

*Data from Stewart et. al (2), Data from Woods et. al (15), ** MSH: Multicenter Study of Hydroxyurea in Sickle Cell Anemia

Table 2. Multivariate Generalized Estimating Equations (GEE) Model to Assess the Effect of High HbF Response on QOL. The Effect of Independent Variables (Rows) on Each Outcome Measure (Columns) at 6, 12, 18 and 24 Months During Randomized Treatment to Hydroxyurea or Placebo is Shown. The Dependent Variables are: SF-36 Health Status Survey Variables (as a Percent Scores), Mood Profile, Ladder of Life.
Independent Variables	Quality of life Outcome Variables (Each Column is One Model Per Outcome)
	General Health Now	Physical Functioning	Social Functioning	Role- Physical	Role- Mental	Mental Health	Energy- Fatigue
High Response	0.0008	NS*	0.007	NS	NS	NS	NS
Baseline QOL Value	<0.0001	< 0.0001	< 0.0001	< 0.0001	< 0.0001	< 0.0001	< 0.0001
Baseline Daily Pain 0-3, 4-6, 7-9	<0.0001	NS	<0.0001	<0.0001	0.008	0.002	0.002
Baseline Fetal Hemoglobin <0. 5 vs > 0. 5	NS	NS	NS	NS	NS	NS	NS
Baseline Annual Crisis Rate <6 vs ≥ 6	0.002	NS	NS	0.0005	NS	NS	NS
	Pain Recall (4-week)	General Health Perception	Tension- Anxiety	Depression- Dejection	Vigor	Fatigue	Ladder of Life
High Response	0.0006	0.002	NS	NS	NS	NS	NS
Baseline QOL Value	< 0.0001	< 0.0001	< 0.0001	< 0.0001	<0.0001	<0.0001	< 0.0001
Baseline Daily Pain 0-3, 4-6, 7-9	< 0.0001	NS	0.001	<0.0001	NS	0.0004	0.007
Baseline Fetal Hemoglobin <0. 5 vs > 0. 5	NS	NS	NS	NS	NS	NS	NS
Baseline Annual Crisis Rate <6 vs ≥ 6	<0.0001	0.003	NS	NS	0.004	NS	0.008

* NS = p > .01

Table 1. Comparison of MSH Patients and Other Reference Patient Groups on SF-36 Measures
	Health Status Short Form 36 Scores
Patient Group	Physical Functioning	Role-Physical	Social Functioning	Mental Health	Health Perception	Bodily Pain Recall
No Chronic Conditions* (N = 2595)	86.0	87.2	92.3	77.6	72.6	74.2
Myocardial Infarction* N = 147	64.6	58.6	80.7	74.8	62.7	71.7
Arthritis* N = 2079	81.1	76.9	88.4	74.6	65.3	58.6
Sickle Cell Anemia ^† N = 143	63.6	45.3	65.1	70.0	42.4	44.8
MSH** N = 299	66.0	46.7	6.65.0	70.0	38.8	46.5

*Data from Stewart et. al (2), Data from Woods et. al (15), ** MSH: Multicenter Study of Hydroxyurea in Sickle Cell Anemia

Table 2. Multivariate Generalized Estimating Equations (GEE) Model to Assess the Effect of High HbF Response on QOL. The Effect of Independent Variables (Rows) on Each Outcome Measure (Columns) at 6, 12, 18 and 24 Months During Randomized Treatment to Hydroxyurea or Placebo is Shown. The Dependent Variables are: SF-36 Health Status Survey Variables (as a Percent Scores), Mood Profile, Ladder of Life.
Independent Variables	Quality of life Outcome Variables (Each Column is One Model Per Outcome)
	General Health Now	Physical Functioning	Social Functioning	Role- Physical	Role- Mental	Mental Health	Energy- Fatigue
High Response	0.0008	NS*	0.007	NS	NS	NS	NS
Baseline QOL Value	<0.0001	< 0.0001	< 0.0001	< 0.0001	< 0.0001	< 0.0001	< 0.0001
Baseline Daily Pain 0-3, 4-6, 7-9	<0.0001	NS	<0.0001	<0.0001	0.008	0.002	0.002
Baseline Fetal Hemoglobin <0. 5 vs > 0. 5	NS	NS	NS	NS	NS	NS	NS
Baseline Annual Crisis Rate <6 vs ≥ 6	0.002	NS	NS	0.0005	NS	NS	NS
	Pain Recall (4-week)	General Health Perception	Tension- Anxiety	Depression- Dejection	Vigor	Fatigue	Ladder of Life
High Response	0.0006	0.002	NS	NS	NS	NS	NS
Baseline QOL Value	< 0.0001	< 0.0001	< 0.0001	< 0.0001	<0.0001	<0.0001	< 0.0001
Baseline Daily Pain 0-3, 4-6, 7-9	< 0.0001	NS	0.001	<0.0001	NS	0.0004	0.007
Baseline Fetal Hemoglobin <0. 5 vs > 0. 5	NS	NS	NS	NS	NS	NS	NS
Baseline Annual Crisis Rate <6 vs ≥ 6	<0.0001	0.003	NS	NS	0.004	NS	0.008

* NS = p > .01

Comments

Commenting is limited to medical professionals. To comment please Log-in.

Comments on Medscape are moderated and should be professional in tone and on topic. You must declare any conflicts of interest related to your comments and responses. Please see our Commenting Guide for further information. We reserve the right to remove posts at our sole discretion.

processing....

Hydroxyurea And Sickle Cell Anemia: Effect On Quality Of Life

Conclusion

Author Contributions

Tables

Tables

Tables

Tables

References

Authors and Disclosures

Authors and Disclosures

Comments

Email This

Feedback