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Case Report Benefits of kinesiotherapy and aquatic rehabilitation on sickle cell anemia. A case report

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The process of hemoglobin polymerization and the consequent sickling of red blood cells that occurs in patients with sickle cell disease shortens the half-life of red blood cells. It causes vaso-occlusive complications, as well as pain and pulmonary and cardiovascular dysfunction. We evaluated an aquatic rehabilitation program used for patients with sickle cell anemia and examined the possible benefits that exercise in warm water has for the circulatory system, for relieving pain, and for increasing lung capacity. The patient was a 32-year-old female. The parameters that we used in this study include respiratory muscle strength (which was calculated by measuring maximum inspiratory pressures and maximum expiratory pressures), the McGill and Wisconsin pain questionnaires (in order to evaluate the patients' characterizations and descriptions of their pain), and the SF-36 Health Survey. The treatment included warm water exercises, stretching, aerobic exercise, and relaxation, during two sessions of 45 min per week for 5 weeks. The patient experienced a significant decrease in pain, a significant increase in the strength of respiratory muscles, and improved quality of life. We conclude that aquatic rehabilitation can be used to improve the clinical condition of sickle cell anemia patients, and we encourage more research on this new treatment regime, in comparison with other types of therapies.
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Genetics and Molecular Research 9 (1): 360-364 (2010)
Case Report
Benets of kinesiotherapy and aquatic
rehabilitation on sickle cell anemia. A case report
G. Tinti1, R. Somera Jr.1, F.M. Valente2 and C.R.B. Domingos3
1Departamento de Fisioterapia, Universidade Paulista,
São José do Rio Preto, SP, Brasil
2Departamento de Fisioterapia,
Universidade Federal Paulista, São Paulo, SP, Brasil
3Departamento de Biologia,
Universidade Estadual Paulista “Julio de Mesquita Filho”,
São José do Rio Preto, SP, Brasil
Corresponding author: F.M. Valente
E-mail: aviamvalente@hotmail.com
Genet. Mol. Res. 9 (1): 360-364 (2010)
Received November 14, 2009
Accepted December 14, 2009
Published March 2, 2010
ABSTRACT. The process of hemoglobin polymerization and the con-
sequent sickling of red blood cells that occurs in patients with sickle cell
disease shortens the half-life of red blood cells. It causes vaso-occlusive
complications, as well as pain and pulmonary and cardiovascular dys-
function. We evaluated an aquatic rehabilitation program used for pa-
tients with sickle cell anemia and examined the possible benets that
exercise in warm water has for the circulatory system, for relieving pain,
and for increasing lung capacity. The patient was a 32-year-old female.
The parameters that we used in this study include respiratory muscle
strength (which was calculated by measuring maximum inspiratory pres-
sures and maximum expiratory pressures), the McGill and Wisconsin
pain questionnaires (in order to evaluate the patients’ characterizations
and descriptions of their pain), and the SF-36 Health Survey. The treat-
ment included warm water exercises, stretching, aerobic exercise, and
relaxation, during two sessions of 45 min per week for 5 weeks. The
patient experienced a signicant decrease in pain, a signicant increase in
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Genetics and Molecular Research 9 (1): 360-364 (2010)
Aquatic rehabilitation in sickle cell anemia
the strength of respiratory muscles, and improved quality of life. We con-
clude that aquatic rehabilitation can be used to improve the clinical con-
dition of sickle cell anemia patients, and we encourage more research on
this new treatment regime, in comparison with other types of therapies.
Key words: Sickle cell anemia; Hemoglobinopathy; Kinesiotherapy;
Aquatic rehabilitation
INTRODUCTION
Sickle cell anemia is a serious genetic condition caused by the substitution of a glu-
tamic acid for avaline at position six of the beta globin chain (b 6 Glu-Val). It leads to polymer
formation when patients experience low oxygen levels, which results in sickling of the red
blood cells, causing health complications (Naoum and Naoum, 2004). These complications
include severe musculoskeletal and cardiovascular pain, which clinically manifests as palpita-
tions, dyspnea, and fatigue; these symptoms worsen during physical exercise. The primary
factors responsible for cardiovascular manifestations are vaso-occlusive events, which prevent
the transport of oxygen to the tissues (Ingram, 1989; Martins et al., 1998).
Kinesiotherapy (and, more specically, aquatic rehabilitation) is a treatment tech-
nique that causes a physiological response, including relaxed arterial and venous muscle tone,
lower blood pressure, a higher cardiac frequency and volume of ejection, an increased supply
of blood to the muscles, higher muscle metabolism, and more circulation in the limbs (Cam-
pion, 2000). All these changes, such as the redistribution of blood resulting from immersion
(hydrostatic pressure), lead to vasodilatation, venous return (Becker and Cole, 2000), and freer
movement of tissue uids, which aids in the removal of metabolites, improves tissue nutrition
and helps the healing process (Campion, 2000).
A preventative physiotherapeutic approach for the treatment of complications result-
ing from sickle cell anemia is rarely reported in the literature (Ingram, 1989; Martins et al.,
1998), but the literature does encourage the use of physiotherapy as part of an interdisciplin-
ary treatment program that offers the adequate attention needed by these patients (Naoum and
Naoum, 2004). We examined the effects of kinesiotherapy and aquatic rehabilitation on pain
levels and lung capacity in an adult woman with sickle cell anemia.
MATERIAL AND METHODS
A female patient, 32 years old, had been diagnosed to have sickle cell anemia at 8
years old; she was homozygous for the Bantu haplotype. Between 16 and 18 years old, the pa-
tient was repeatedly hospitalized for infections, leg ulcers, generalized pain, and anemia. The
patient reported being forced to leave her job approximately four years before, when pain was
the most frequent and intense. Because the patient was an adult, hydroxyurea was offered as
treatment, but the patient opted not to take this medication due to its adverse side effects. The
primary complaints reported during her clinical evaluation were apathy, depression, frequent
fatigue, and constant, generalized pain. The patient reported that she had not been engaging
in any physical activity after suffering from tachypnea and tachycardia during light exertion.
In order to improve the patient’s lung capacity and to relieve her pain, physiotherapy was pro-
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Genetics and Molecular Research 9 (1): 360-364 (2010)
G. Tinti et al.
posed (including a complete physiotherapeutic evaluation).
During her evaluation, the patient responded to the SF-36 Health Survey (Ware Jr. and
Sherbourne, 1992; Ciconelli et al., 1999), the McGill pain questionnaire (translated into Portuguese;
Pimenta and Teixeira, 1997), and the Wisconsin pain survey (Graham et al., 1980). An evaluation
was made of her respiratory muscle strength by measuring maximum inspiratory pressure and maxi-
mum expiratory pressure, with a spirometer (GERAR®). Physiotherapy was recommended in order
to improve the patient’s lung capacity and was initiated after the patients clinical evaluations and
excluding other possible cardiac problems that would have explained the patient’s condition.
RESULTS AND DISCUSSION
A kinesiotherapy/aquatic rehabilitation plan was developed; it consisted of exercise in
warm water, stretching, aerobics, and relaxation. The sessions were performed in a swimming
pool heated to a temperature of 34°C for 45 min, twice a week, for 10 sessions; the evaluation
methods described above were applied before the rst session and after the 10th session.
Changes in the composition of hemoglobin lead to complications characteristic of
sickle cell disease. These changes have signicant negative effects on the quality of life of the
patients, which we also observed in the responses to the questionnaires (Tables 1-3).
The pain experienced by the patient was reduced after the rst therapy session (Table 2).
The patient’s quality of life also improved (Table 1). This proves the benets of physical activity in
water, particularly the patient’s newfound ability to move in ways that she was initially unable to.
Among the pain index parameters from the McGill questionnaire, we also calculated
the percentages in the reduction of pain symptoms using the descriptors evaluative and mis-
cellaneous, which improved approximately 40% (Table 2). In the responses to the Wisconsin
questionnaire, relief from pain and relations with other people improved most (Table 3). Only
mood did not change from pre- to post-treatment.
Descriptors Pre-treatment Post-treatment
Mental health 40 60
Emotional role 0 33
Social role 25 88
Vitality 25 50
General health 5 42
Pain 50 93
Physical role 0 75
Physical functioning 45 50
A value of zero (0) corresponds to the most negative result (lowest quality of life), while a value of 100 corresponds
to the most positive result (highest quality of life).
Table 1. Pre- and post-treatment values from the SF-36 Health Survey.
Descriptors Pre-treatment pain index Post-treatment pain index
Sensory 33 30
Affective 11 9
Evaluative 5 3
Miscellaneous 7 4
Table 2. Results of the McGill Pain Questionnaire, with zero as the most positive score (the least pain) and 100 as the
most negative score (the most pain).
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Genetics and Molecular Research 9 (1): 360-364 (2010)
Aquatic rehabilitation in sickle cell anemia
The maximum inspiratory pressure was 76.7 and 79.2 cmH2O, before and after treat-
ment, respectively. The maximum expiratory pressure was 81.3 and 85.4 cmH2O, before and
after treatment, respectively. This level of improvement was important to the patient, who
reported that she could walk for longer distances without experiencing fatigue.
Intense physical exercise produces physiological changes that induce the formation of
hemoglobin polymers in sickle cell anemia patients, due to the presence of Hemoglobin S. This
process, which is caused by physical exertion, occurs as a result of dehydration, increased body
temperature, hypoxia, and acidosis, which are also evident at times of stress. Once this process
occurs, the chances of complications increase for sickle cell anemia patients (Klug et al., 1974;
Ingram, 1989). In light of this, the kinesiotherapeutic exercises that were proposed for this patient
were based on exercise physiology (Campion, 2000; Becker and Cole, 2000), with low intensity
and velocity and intermittent breaks. The patient reported a decrease in fatigue (with a reduction
in the frequency of the symptom) in both overt physical exertion and day-to-day activities.
Improvement in the social relation responses were also observed (Table 1). The pa-
tient reported more availability for events and activities. Initially, the patient had reported
feelings of embarrassment and fear of not being taken seriously when she complained of her
pain and limited movement, because there was no visible evidence for the cause of her lim-
ited movement and because she had previously experienced scolding and disregard when she
sought help for her suffering.
CONCLUSION
Based on the results with this sickle cell anemia patient, we suggest that kinesiothera-
py (more specically, aquatic rehabilitation), at low intensity and in heated pools, can improve
respiratory muscle strength and quality of life, and it can decrease pain.
ACKNOWLEDGMENTS
We thank the Brazilian foundation Conselho Nacional de Desenvolvimento Cientíco
e Tecnológico (CNPq) for nancial support.
REFERENCES
Becker BE and Cole AJ (2000). Terapia Aquática Moderna. Manole, São Paulo.
Descriptors Pre-treatment Post-treatment
Enjoyment of life 8 7
Sleep 5 4
Relations with other people 8 5
Work 7 5
Walking ability 8 7
Mood 6 6
General activity 7 4
Pain on the average 10 7
Pain at its least 4 1
Pain at its worst 10 8
Table 3. Results from the Wisconsin Pain Questionnaire, with zero (0) as the most positive score (the least pain)
and 10 as the most negative (the most pain).
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Genetics and Molecular Research 9 (1): 360-364 (2010)
G. Tinti et al.
Campion MR (2000). Hidroterapia: Princípios e Prática. Manole, São Paulo.
Ciconelli RM, Ferraz MB, Santos W, Meinão I, et al. (1999). Brazilian-Portuguese version of the SF-36. A reliable and
valid quality of life outcome measure. [Tradução para a língua portuguesa e validação do questionário genérico de
avaliação de qualidade de vida SF-36 (Brasil SF-36)]. Rev. Bras. Reumatol. 39: 143-150.
Graham C, Bond SS, Gerkovich MM and Cook MR (1980). Use of the McGill pain questionnaire in the assessment of
cancer pain: replicability and consistency. Pain 8: 377-387.
Klug PP, Lessin LS and Radice P (1974). Rheological aspects of sickle cell disease. Arch. Intern. Med. 133: 577-590.
Martins WA, Mesquita ET, Cunha DM, Ferrari AH, et al. (1998). Alterações cardiovasculares na anemia falciforme. Arq.
Bras. Cardiol. 70: 365-370.
Naoum PC and Naoum FA (2004). Doença das Células Falciformes. Sarvier, São Paulo.
Pimenta CAM and Teixeira MJ (1997). Avaliação da dor. Rev. Med. 76: 27-35.
Ingram VM (1989). A case of sickle-cell anaemia: a commentary on ‘Abnormal Human Haemoglobins. I. The Comparison
of Normal Human and Sickle-Cell Haemoglobins by ‘Fingerprinting” with II. The Chymotryptic Digestion of the
Trypsin-Resistant ‘Core’ of Haemoglobins A and S and III. The Chemical Difference Between Normal and Sickle Cell
Haemoglobins. Biochim. Biophys. Acta 1000: 147-150.
Ware JE Jr and Sherbourne CD (1992). The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and
item selection. Med. Care 30: 473-483.
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... Very few studies, and most of them time case studies with poorly controlled exercise intensities and volumes, investigated the effects of regular physical activity in SCA [46,47]. Tinti et al. [46] previously reported that two sessions of 45 min per week of aquatic rehabilitation decreased chronic pain, improved respiratory muscle strength and quality of life in a SCA patient. ...
... Very few studies, and most of them time case studies with poorly controlled exercise intensities and volumes, investigated the effects of regular physical activity in SCA [46,47]. Tinti et al. [46] previously reported that two sessions of 45 min per week of aquatic rehabilitation decreased chronic pain, improved respiratory muscle strength and quality of life in a SCA patient. Alcorn et al. [47] demonstrated that exercise therapy mixing moderate strength and endurance exercise, recreational gymnastics, stationary bicycle riding and games for 10-30 min duration decreased the length of hospitalization with vaso-occlusive crisis in SCA children. ...
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... For sickle-cell disease, this change was initiated by preliminary studies in humans examining the feasibility, safety, 26,27 and benefits of repeated moderateintensity exercise. [28][29][30] However, to our knowledge, no randomised controlled trial has been done until now. ...
... and researchers to conduct a pilot exercise-training study to provide new insights about exercise in sickle-cell disease. Several case reports 27,30 and one uncontrolled pilot study done in ten adults 38 suggested that patients' physical capacities could be improved by respiratory muscle training, physiotherapy, or aquatic rehabilitation. Liem and colleagues 26 proposed a home-based 12-week training programme for children and showed the feasibility of this intervention. ...
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A 36-item short-form (SF-36) was constructed to survey health status in the Medical Outcomes Study. The SF-36 was designed for use in clinical practice and research, health policy evaluations, and general population surveys. The SF-36 includes one multi-item scale that assesses eight health concepts: 1) limitations in physical activities because of health problems; 2) limitations in social activities because of physical or emotional problems; 3) limitations in usual role activities because of physical health problems; 4) bodily pain; 5) general mental health (psychological distress and well-being); 6) limitations in usual role activities because of emotional problems; 7) vitality (energy and fatigue); and 8) general health perceptions. The survey was constructed for self-administration by persons 14 years of age and older, and for administration by a trained interviewer in person or by telephone. The history of the development of the SF-36, the origin of specific items, and the logic underlying their selection are summarized. The content and features of the SF-36 are compared with the 20-item Medical Outcomes Study short-form.
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Full-text available
A 36-item short-form (SF-36) was constructed to survey health status in the Medical Outcomes Study. The SF-36 was designed for use in clinical practice and research, health policy evaluations, and general population surveys. The SF-36 includes one multi-item scale that assesses eight health concepts: 1) limitations in physical activities because of health problems; 2) limitations in social activities because of physical or emotional problems; 3) limitations in usual role activities because of physical health problems; 4) bodily pain; 5) general mental health (psychological distress and well-being); 6) limitations in usual role activities because of emotional problems; 7) vitality (energy and fatigue); and 8) general health perceptions. The survey was constructed for self-administration by persons 14 years of age and older, and for administration by a trained interviewer in person or by telephone. The history of the development of the SF-36, the origin of specific items, and the logic underlying their selection are summarized. The content and features of the SF-36 are compared with the 20-item Medical Outcomes Study short-form.
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The McGill Pain Questionnaire (MPQ) is a recent empirically derived instrument designed to provide quantitative information on major dimensions of pain. Although widely used as an outcome measure in clinical research, little attention has been directed specifically at the instrument itself. The present study addressed this need. Detailed findings were obtained for both single and multiple administrations of the MPQ in two subject samples, each composed of 18 cancer outpatients in pain. These data were compared to similar, but less extensive, data reported by Melzack [6]. MPQ indices proved highly replicable over the two subject samples tested and were remarkably similar to the findings reported by Melzack for a different cancer pain patient sample. No differences were found between the written form of MPQ administration used in the present study and the oral procedure followed by Melzack. The consistency of pain descriptor subclass choice in the present samples was high, ranging from 66% to 80.4% over 4 administrations, and these values compare well with the value of 70.3% reported earlier by Melzack. However, the present subjects selected a larger set of pain descriptor words compared to the word set reported to be characteristic of cancer pain by Dubuisson and Melzack [2]. Both individual and group analyses indicated the MPQ is best used as a measure of immediate pain, and not as a summary measure of past pain over a defined period of time. These findings support the use of the MPQ as a reliable, multi-dimensional measure of immediate pain, and suggest the potential value of future research aimed at refining the psychometric properties of the instrument.
Doença das Células Falciformes
  • P C Naoum
  • F A Naoum
Naoum PC and Naoum FA (2004). Doença das Células Falciformes. Sarvier, São Paulo.