Management of pain in sickle cell disease using biofeedback therapy: a preliminary study

Fifteen patients with a history of painful episodes of sickle cell disease were given training in progressive relaxation, thermal biofeedback, cognitive strategies, and self-hypnosis to help them develop self-management skills to relieve pain. Results show a 38.5% reduction in the number of emergency room visits, a 31% reduction in the number of hospitalizations, and a 50% reduction in the inpatient stay during the 6 months since the beginning of therapy compared to 6 months prior to therapy. Analgesic intake was reduced by 29% for those who were using it regularly. This is a preliminary study, and the results are considered only as suggestive of the potential use of biofeedback therapy and behavioral management in alleviating painful episodes in sickle cell disease.     

Acute admissions of patients with sickle cell disease who live in Britain.

All acute admissions of patients with sickle cell disease who lived in the London borough of Brent and attended this hospital were analysed for a period of one year. Sixty three of the 211 patients who were followed up by the haematology department required 161 acute admissions during the year. Most admissions (126) were for the 42 patients with homozygous sickle cell disease; 147 (91%) were for vaso-occlusive episodes, 142 of which were for painful crises, three for cerebrovascular accidents, and two for renal papillary necrosis. Preschool children with sickle cell disease were admitted predominantly with limb pain, whereas in schoolchildren and adults the incidence of trunk pain was higher. Twenty four of the 93 episodes of trunk pain culminated in an episode of severe visceral sequestration usually affecting the lungs, the liver, or the mesenteric circulation. Two patients died: an 18 month old baby with an acute splenic sequestration crisis and a 19 year old man with a severe girdle syndrome (sickling in the mesenteric circulation, liver, and lungs). Infective episodes were rare (11 episodes) but severe: one haemophilus meningitis, two salmonella infections, and three aplastic crises due to parvovirus infections. The average duration of the hospital stay was 7.4 days per admission. It is concluded that because sickle cell disease causes appreciable morbidity in older children, adolescents, and adults a systematic approach to management is needed to deal with acute episodes such as sequestration syndromes.     

Haptoglobin for the treatment of sickle cell disease

There are three haptoglobin phenotypes in humans designated: Hp1–1, Hp2–1, and Hp2–2. The Hp1–1 phenotype has been shown to be protective against certain diseases, and this has been suggested to be the result of better anti-inflammatory and antioxidative properties compared to haptoglobin polymers of the other phenotypes when clearing cell-free haemoglobin. We propose the use of haptoglobin for the treatment of sickle cell disease, where an oxidative state exists caused by a high level of cell-free haemoglobin. A significant number of sickle cell disease patients are severely affected and experience regular acute painful episodes resulting in hospitalisation.Therapeutic treatments for sickle cell disease are limited and therefore haptoglobin could represent a vital alternative therapy. A method has been developed as part of the commercial fractionation of plasma for preparing haptoglobin enriched for dimers. This is significant as it uses a mixture of plasma of all haptoglobin phenotypes, and allows annual production of hundreds of kilograms quantities of haptoglobin that may be required to allow treatment of thousands of sickle cell disease patients worldwide.     

Asthma is a risk factor for acute chest syndrome and cerebral vascular accidents in children with sickle cell disease

BACKGROUND: Asthma and sickle cell disease are common conditions that both may result in pulmonary complications. We hypothesized that children with sickle cell disease with concomitant asthma have an increased incidence of vaso-occlusive crises that are complicated by episodes of acute chest syndrome. METHODS: A 5-year retrospective chart analysis was performed investigating 48 children ages 3-18 years with asthma and sickle cell disease and 48 children with sickle cell disease alone. Children were matched for age, gender, and type of sickle cell defect. Hospital admissions were recorded for acute chest syndrome, cerebral vascular accident, vaso-occlusive pain crises, and blood transfusions (total, exchange and chronic). Mann-Whitney test and Chi square analysis were used to assess differences between the groups. RESULTS: Children with sickle cell disease and asthma had significantly more episodes of acute chest syndrome (p = 0.03) and cerebral vascular accidents (p = 0.05) compared to children with sickle cell disease without asthma. As expected, these children received more total blood transfusions (p = 0.01) and chronic transfusions (p = 0.04). Admissions for vasoocclusive pain crises and exchange transfusions were not statistically different between cases and controls. SS disease is more severe than SC disease. CONCLUSIONS: Children with concomitant asthma and sickle cell disease have increased episodes of acute chest syndrome, cerebral vascular accidents and the need for blood transfusions. Whether aggressive asthma therapy can reduce these complications in this subset of children is unknown and requires further studies.     

Triple alpha-genes (alpha alpha alpha anti3.7) in a patient with sickle cell anaemia.

This paper reports the case of a 17-year-old male student from the Jaizan area in south-western Saudi Arabia who had sickle cell anaemia and possessed three alpha-genes on one chromosome (alpha alpha alpha anti3.7) and two on the other. The clinical manifestations were severe, with frequent blood transfusion requirements and frequent episodes of painful crises, severe anaemia and tissue involvement. In comparison with age and sex-matched sickle cell anaemia patients with one alpha-gene deletion (-alpha/alpha alpha), or a normal alpha-gene arrangement (alpha alpha/alpha alpha), a more severe disease presentation was obvious in the propositus. It is suggested that with the surplus alpha-globin chains, more severe haematological and clinical abnormalities occur, these influence the phenotypic expression of sickle cell anaemia. However, more patients with this type of gene arrangement must be studied before a definite conclusion can be reached regarding the influence of excess alpha-globin chains on the presentation of sickle cell anaemia.     

EMG biofeedback for functional bladder-sphincter dyssynergia: A case study

The present study utilized EMG biofeedback in the treatment of functional bladder-sphincter dyssynergia, a learned incoordination of bladder and urethral sphincter activity during voiding. The condition is usually associated with a history of painful urination due to bladder infections, surgery, or harsh toilet training. The subject was an 8-year-old girl with chronic diurnal urinary frequency, urge incontinence, and nocturnal enuresis. Treatment consisted of intensive instruction in alternately tensing and relaxing her lower pelvic musculature, as well as relaxing during voiding. These exercises were accompanied by EMG biofeedback from perianal and perivaginal surface electrode sites. Home practice consisted of the tense-relax exercise, relaxation during voiding, and self-monitoring and record-keeping. There were 17 sessions over a period of 9 months. No medication was used. Marked reduction (to normal levels) in diurnal urgency and frequency occurred by the 3rd week of therapy, and complete recovery of normal function, including nocturnal continence without waking, occurred by the 13th therapy session, 5 months after therapy began. Follow-up 1 year after therapy revealed that these gains were being maintained. Pre- and posttherapy urodynamic studies corroborated the achievement of normal urinary function.     

Early deaths in Jamaican children with sickle cell disease.

In Jamaican children with homozygous sickle cell (SS) disease diagnosed at birth two-year survival was 87%, compared with 95% in children with sickle cell-haemoglobin C (CS) disease, and 99% in normal controls. Death among those with SS disease occurred most often between the ages of 6 and 12 months. Principal causes were acute splenic sequestration and pneumococcal infection. Neonatal diagnosis of haemoglobinopathies must be followed by close observation if mortality is to be reduced by early diagnosis and treatment of these complications.     

Simple electromyographic biofeedback treatment for chronic pediatric constipation/encopresis: Preliminary report

Pediatric constipation/encopresis is thought to be due, in part, to paradoxical constriction of the external anal sphincter (EAS) muscle during attempted defecation. This inappropriate contraction can lead to delayed, impacted, painful, and infrequent bowel movements. Standard Medical Care (SMC) involves disimpaction with enemas, followed by laxative therapy and diet modification, to maintain frequent soft stools. Using the case control method, the efficacy of SMC alone was compared with SMC plus EAS electromyographic biofeedback aimed at eliminating paradoxical contraction. Thirteen consecutive chronically constipated children received SMC plus biofeedback, and were compared with 13 age- and sex-matched children who received only SMC. Biofeedback subjects demonstrated post-treatment elimination of EAS paradoxical constriction. At 16 months follow-up parents of biofeedback children reported significantly greater improvement in constipation, encopresis, laxative use, and painful bowel movements compared to SMC.This research report was supported by the NIH under grant No. RO1 HD 28160.     

Some atypical and rare sickle cell gene haplotypes in populations of Andhra Pradesh, India.

We have investigated the clinical, hematological, and molecular genetic characteristics of sickle cell anemia patients from 6 populations of Andhra Pradesh, South India. Of 72 sickle cell chromosomes (HBB*S) 60 belong to characteristic Arab-Indian haplotypes, 6 to variant Arab-Indian haplotypes, 1 to a Bantu haplotype, 2 to a Cameroon haplotype, and 3 to rare haplotypes. This is the first report of a Bantu haplotype in an Indian population. Some information on haplotype characteristics of normal chromosomes (HBB*A) is also presented. The average hemoglobin level was 7.3 g% and mean fetal hemoglobin (HbF) level was 12.6%. The higher HbF levels corroborate earlier observations in sickle cell homozygotes from India. Clinical investigations have revealed splenomegaly and painful crises as the most common features in these patients.     

Stress reduction treatment of severe recurrent genital herpes virus

Four individuals with high-frequency recurrences of genital herpes virus of at least 2 years' duration were treated with two behavioral stress-reduction treatments. Subjects were given 10 weekly sessions of frontalis EMG biofeedback (2 subjects) or progressive muscle relaxation treatment (2 subjects). Presession and postsession frontalis EMG measures were recorded for all subjects across treatment. Outcome was measured by daily and weekly symptom charting mailed in weekly over 6 months, or by telephone interview after 6 months. Results demonstrated substantial improvement in reported symptoms with both treatments. Relaxation treatment resulted in a 66% and 100% reduction in frequency of recurrences. Frontalis EMG biofeedback resulted in a 72% and 7% reduction in frequency of recurrences. Follow-up at 1-year posttreatment showed that treatment effects were maintained by one subject, partially maintained by two, and reversed in one subject. The need for controlled investigation is emphasized.     

Painful crises in sickle cell disease--patients' perspectives.

One hundred and two patients returned structured questionnaires sent to clinics for sickle cell disease in the United Kingdom in order to gain greater insight into the patients'' perception of painful crises. Most patients who suffer pain crises experience a prodromal stage that should be investigated further to find out if prophylaxis is possible. Cold, exertion, and tiredness were the most important precipitating factors. Despite the increase in the amount of knowledge about sickle cell disease in recent years, and though 29 out of 88 patients (30-40%) believed that medical services were improving, 33 out of 88 (30-40%) were still experiencing long delays in being treated in hospital. A third of patients do not seem to receive adequate pain relief.     

Stress reduction treatment of severe recurrent genital herpes virus

Four individuals with high-frequency recurrences of genital herpes virus of at least 2 years' duration were treated with two behavioral stress-reduction treatments. Subjects were given 10 weekly sessions of frontalis EMG biofeedback (2 subjects) or progressive muscle relaxation treatment (2 subjects). Presession and postsession frontalis EMG measures were recorded for all subjects across treatment. Outcome was measured by daily and weekly symptom charting mailed in weekly over 6 months, or by telephone interview after 6 months. Results demonstrated substantial improvement in reported symptoms with both treatments. Relaxation treatment resulted in a 66% and 100% reduction in frequency of recurrences. Frontalis EMG biofeedback resulted in a 72% and 7% reduction in frequency of recurrences. Follow-up at 1-year posttreatment showed that treatment effects were maintained by one subject, partially maintained by two, and reversed in one subject. The need for controlled investigation is emphasized.The authors thank Stephen Ritz for his contribution to this study.     

The effectiveness of biofeedback-assisted relaxation in modifying sickle cell crises

Eight outpatients with sickle cell disease received six EMG and six thermal half-hour biofeedback training sessions. Statistically significant changes in the desired directions were obtained for the following variables: (a) frontalis muscle tension, (b) digital temperature, (c) frequency of headache as a crisis symptom, (d) frequency of analgesic use, (e) perceived pain intensity, (f) frequency of self-treated crises, and (g) state anxiety. Nonsignificant changes in hospital chart data were found. A 6-month posttreatment follow-up questionnaire revealed the continued effectiveness of the training received regarding headaches and mild pains.Portions of this paper were presented at the meeting of the Eastern Psychological Association, April 1984 and March 1985. The authors wish to publically thank the Ladies Auxiliary of St. Luke's-Roosevelt Hospital Center for donating tape recorders, home training relaxation tapes, and velcro thermistors to all participants. The authors are deeply grateful to Doris L. Wethers, M. D., director of the Sickle Cell Clinic at St. Luke's-Roosevelt Hospital Center for her cooperation with this study. She was especially helpful in clarifying the clinical manifestations of sickle cell disease. She was also helpful in enrolling patients and in obtaining the above-mentioned funding.     

The effectiveness of biofeedback-assisted relaxation in modifying sickle cell crises

Eight outpatients with sickle cell disease received six EMG and six thermal half-hour biofeedback training sessions. Statistically significant changes in the desired directions were obtained for the following variables: (a) frontalis muscle tension, (b) digital temperature, (c) frequency of headache as a crisis symptom, (d) frequency of analgesic use, (e) perceived pain intensity, (f) frequency of self-treated crises, and (g) state anxiety. Nonsignificant changes in hospital chart data were found. A 6-month posttreatment follow-up questionnaire revealed the continued effectiveness of the training received regarding headaches and mild pains.     

Profil lipidoprotéinique,isotopique et risque athérogène dans la drépanocytose en Côte d’Ivoire

The principal goal of this study was to assess the possible disturbances of lipids and lipoproteins in sickle cell disease and establish a relationship between painful crisis and atherogenic risks by the atherogenicity index in Ivoirian adults. We analysed serum plasma lipid and lipoprotein profiles of 126 subjects with sickle cell anemia, and 55 “Hb AA” healthy individuals. The lipid and lipoprotein parameters studied were total cholesterol, triglycerids, HDL, LDL, apoproteins A1 and B, electrophoresis of lipoproteins and haemoglobin. In painful crisis, levels of total cholesterol, HDL-cholesterol, LDL-cholesterol, apoproteins A1 (apo A1) in sickle cell anemia patients were shown to be significantly lower and levels of triglycerides higher than that of control group and steady state. The electrophoresis profile showed a significant fall of α lipoproteins while β lipoproteins were significantly high in period of crisis. The atherogenicity index (total cholesterol/HDL ratio) was also significantly high, just as LDL/HDL ratio in period of crisis. The overview of these results might hypothesize a high relatively atherogenic risk during the sickle-cell anemia crisis. A special monitoring of the patients in crisis is also necessary in order to prevent the risk of cardiovascular diseases.     

Pneumococcal bacteraemia: 325 episodes diagnosed at St Thomas's Hospital.

Three hundred and twenty five episodes of pneumococcal bacteraemia occurred at St Thomas''s Hospital during 1970-84, accounting for 13.3% of all episodes of bacteraemia. Twice as many cases occurred in male as in female patients, and common predisposing factors included chronic chest disease, alcoholism, haematological malignancies, cirrhosis, and sickle cell anaemia. Mortality was 28.6% overall but only 11.8% among patients who received antibiotic treatment for at least 24 hours. Most patients (261) had pneumonia, 26 had meningitis, and eight were children with occult bacteraemia. The commonest serotype of pneumococcus in adults was type 3 (39 episodes), and these strains were associated with a high mortality. Other factors determining a fatal outcome included underlying disease (such as cirrhosis, malignancy, and chronic chest disease) and extrapulmonary infection. Almost half the survivors were treated for 10 days or less and became afebrile within 48 hours.     

A retrospective,follow-up study of biofeedback-assisted relaxation therapy in patients with posttraumatic headache

Although biofeedback in the treatment of migraine and tension-type headache has been widely researched, there is little research examining biofeedback therapy in posttraumatic headache (PTH). In this retrospective study, 40 subjects with PTH who had received biofeedback-assisted relaxation at our headache clinic were questioned at least 3 months following the completion of therapy. Subjects were queried about improvements in headache, increases in ability to relax and cope with pain, and overall benefits, lasting effectiveness, and continued use of biofeedback in daily life. Results indicate 53% reported at least moderate improvement in headaches; 80% reported at least moderate improvement in ability to relax and cope with pain; 93% found biofeedback helpful to some degree; 85% felt headache relief achieved through biofeedback had continued at least somewhat; and 95% stated they were continuing to use biofeedback skills in daily life. A correlation analysis revealed a negative relationship between response to biofeedback and increased chronicity of the disorder. In other words, the more chronic the disorder, the poorer the response to treatment. A stepwise regression analysis found that chronicity of the disorder and number of treatment sessions significantly affected response to treatment. Data suggest that biofeedback-assisted relaxation should at least be considered when planning treatment strategies for posttraumatic headache.We wish to express our appreciation to Sandra Tomlinson Becky Kinloch, and C. M. Bundrick for their assistance in this project.     

Small fragment homologous replacement-mediated modification of genomic beta-globin sequences in human hematopoietic stem/progenitor cells

An ultimate goal of gene therapy is the development of a means to correct mutant genomic sequences in the cells that give rise to pathology. A number of oligonucleotide-based gene-targeting strategies have been developed to achieve this goal. One approach, small fragment homologous replacement (SFHR), has previously demonstrated disease-specific genotypic and phenotypic modification after introduction of small DNA fragments (SDFs) into somatic cells. To validate whether the gene responsible for sickle cell anemia (beta-globin) can be modified by SFHR, a series of studies were undertaken to introduce sickle globin sequences at the appropriate locus of human hematopoietic stem/progenitor cells (HSPCs). The characteristic A two head right arrow T transversion in codon 6 of the beta-globin gene was indicated by restriction fragment length polymorphic (RFLP) analysis of polymerase chain reaction (PCR) products generated by amplification of DNA and RNA. At the time of harvest, it was determined that the cells generally contained 相似文献   

Rheumatoid arthritis: A study of relaxation and temperature biofeedback training as an adjunctive therapy

Rheumatoid arthritis (RA) is a painful systemic disease and is believed to be exacerbated by stress. Relaxation and biofeedback strategies have demonstrated utility in alleviating both pain and stress-related symptomatology, and therefore were tested for efficacy with this disease in a two-phase study. First, 24 patients were taught a relaxation technique and then trained in either temperature elevation or reduction. Second, a group of 15 patients thus trained was compared with 8 others who received traditional physiotherapy modalities. Psychological tests, functional/physical evaluations, as well as measurements related to pain, sleep, and other activities were carried out. Results of the first study revealed significant and positive changes following treatment that were primarily related to pain, tension, and sleep patterns for both groups, but no differential effects were noted between temperature elevation or reduction conditions. This was attributed to both groups having maintained temperature above baseline during biofeedback training. The results of the second study consistently favored the relaxation and biofeedback over the physiotherapy group on the physical/functional indices. The psychological measures tended to remain constant throughout both studies, leading to the conclusion that the effectiveness of treatment was specific to physical functioning rather than to a psychological enhancement of well-being.This work was conducted in partial fulfillment of the doctoral requirements at North Texas State University by Phillip McGraw. This work was also performed pursuant to Institutional Grant No. 5-S07-RR05426-12 and NIAMDD No. 5 P60 AM20628-02.