Pain reports have become increasingly common and problematic in thalassemia. As patients are living longer, there is a growing need to study pain and explore its impact on patient lives. The Brief Pain Inventory (BPI) was used quarterly to assess pain and pain interference in North American thalassemia patients. The SF-36 and HADS were used to assess quality of life, anxiety, and depression. Of the 252 participants, 56% reported pain at least once over the course of this study, with 32% reporting severe pain (≥7/10); 16% reported pain at all 4 visits. Increased pain severity significantly interfered with daily life (p<0.001; regression analysis), and participants with more sites of pain showed an increase in the amount of daily activities affected by pain (p=0.001). Participants reporting more visits with pain reported a significantly higher impact on affective and physical function (p <0.001). Physical quality of life decreased with increasing numbers of visits with pain (p <0.001). Those who reported one or more sites of pain showed increased symptoms of both depression (p <0.001) and anxiety (p= 0.003). Participants reporting at least two visits with pain had higher symptoms of anxiety (p= 0.002), and those with at least three visits, higher symptoms of depression (p= 0.003). Pain in thalassemia is a common, often chronic condition that interferes with life. The study highlights the significance of pain in thalassemia and its impact should be considered in future research and treatments.
SummaryPain is not a symptom generally associated with thalassaemia. However, providers have noted increasing patient reports of pain, creating an impetus for this prospective, observational assessment of pain in thalassaemia patients. The primary study goals were to assess pain prevalence, severity, location, and potential risk factors. This was a multicentre, prospective study of thalassaemia patients receiving care at 12 Thalassaemia Clinical Research Network sites. Pain was assessed using the Brief Pain Inventory. Two hundred and fifty-two thalassaemia patients ranging in age from 12 to 71 years (mean 28Á8) were enrolled. Sixty-four per cent reported experiencing pain during the last 4 weeks, 22% of whom reported pain on a daily basis. Ordinal regression analysis of pain ratings demonstrated significant (P < 0Á001) correlation of increased age with increased pain, irrespective of diagnosis, transfusion status, gender, bone density, chelator type or iron overload. Eighty-one per cent reported having pain for 1 year or longer and 31% reported pain for five or more years. Pain is a major cause of morbidity and an unrecognized problem for patients with thalassaemia. Age is the strongest predictor of frequency and severity. Little else is known about the aetiology and predictors of this pain syndrome.
Aim To assess the feasibility of collecting electronic pain data in thalassemia patients, based on acceptability and convenience to participants and the study team. Methods Participants in the Thalassemia Clinical Research Network Assessment of Pain Study completed the Brief Pain Inventory (BPI) quarterly by paper or phone interview. Participants in a substudy completed the BPI-Short Form daily over three non-consecutive transfusion cycles through an automated telephone system. Results The consent rate for the main study was 93%, with 93% retention. The substudy had 75% retention, with more than 75% of scheduled calls completed. Regular monitoring of enrollment, missed calls, data quality, and the performance of the subcontractor for the automated system was crucial to fulfillment of study goals. Conclusions Use of electronic data collection for patient-reported outcomes was convenient for both patients and study personnel, but required human interactions beyond the automated system to maximize data quantity and optimize quality.
Continuous improvement in the life span of TM patients allows patients to address critical issues of specific organ function. Among these hypogonadotrophic-hypogonadism (HH) and infertility are common complications, having a significant adverse impact on quality of life (QOL). While pregnancies are reported in TM women, paternity is less common in TM men, with significant gaps in our understanding of the underlying pathogenesis of male reduced fertility. Growing evidence indicates that oxidative stress negatively affects sperm quality and function, playing a central role in certain types of male infertility. In addition, abnormal low trace elements, in particular zinc, are important causes for infertility. We hypothesized that deleterious effects of iron overload impair sperm quality beyond that of iron deposition in the pituitary and the resultant HH state, and thus aimed to evaluate potential mechanisms for infertility in TM men: Systemic and pituitary iron burden (liver iron concentration; LIC, pituitary iron by MRI, ferritin), reproductive hormones (LH, FSH) and semen fluid were measured in 7 TM men (median age 26, range 21-30 years). Semen fluid parameters included volume, sperm count, motility, DNA damage determined by DNA Fragmentation Index (DFI), concentration of glutathione (GSH), a major cellular antioxidant which plays a central role in the defense against oxidative stress, and that of pertinent metal/trace elements (iron, zinc, copper). Results were compared to normal reference and Pearson correlation analysis was performed to identify markers that best predict fertility potential and highlight pathogenesis.Sperm and semen plasma analysisLab resultsImagingPtVol. mlCount Mill/mlMotility %DFI %Zn mg/LFe mg/LGSH µmol/LLH/FSH mIU/mLFerritin ng/mLLICMRI R213.51787513141.41.189.54.9/7.96685.413.5± 0.222.500-54.42.732.20.3/0.9369625.216.4± 0.731.400-6.72.530.880.0/0.0428822.816.8± 1.442.314659.7163.02.218.661.6/2.710926.614.5± 0.951.51137---7.52.8/3.419248.4-64.594758.6120.407.54-37752016.7± 0.671.800-80.22.341.060.07/0.330002019.5± 1.2nl>2>20>50<151400.3±0.12030-60/ 0.9-15<300<39.5-13 Semen analysis showed that 5/7 (70%) had either low sperm count or complete azoospermia, and 4/7 exhibited reduced sperm motility (57%). The three patients with a measurable sperm count did not exhibit increased DNA fragmentation (normal DFI). Overall, patients had low seminal plasma GSH concentration, indicating increased oxidative stress. Seminal plasma had elevated iron concentration in 5/6 patients; zinc levels were low in 4/6, while copper was undetectable in all patients (nl 0.064 mg/L). Patients with highest pituitary iron (2,3,7) had the lowest LH/FSH levels and azoospermia, but pituitary iron (R2) did not correlate with LH/FSH, with sperm or systemic iron measures. Seminal fluid GSH (p=0.001), iron (p=0.06), and zinc (p=0.02) inversely correlated with sperm motility. Systemic iron status, as measured by LIC and ferritin, was inversely correlated with GSH (p=0.02; 0.04, respectively) and with zinc (p=0.04; 0.04, respectively). Our data suggest that increased free iron, iron-induced oxidative damage and low trace elements are associated with impaired semen integrity and may explain low fertility potential in TM men, beyond the expected effect of pituitary iron toxicity and HH. These abnormalities, however, did not result in sperm DNA damage, in a small analyzed sample size. Oxidative and metal profiles in seminal fluid may be a valuable tool to improve the reproductive evaluation of TM men. Anti-oxidant treatment will likely have an important role in increasing their reproductive potential. Disclosures: No relevant conflicts of interest to declare.
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