Pain in thalassaemia: the effects of age on pain frequency and severity

Abstract

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.

Thalassemia 2016: Modern medicine battles an ancient disease

“…Recently more attention has been paid to these types of issues. Among the areas receiving attention is the chronic bone pain and osteoporosis which can be quite debilitating in adult patients [47]. The pathogenesis is multifactorial, from bone expansion due to hyperactivity of the bone marrow, to hypogonadism [48], and even iron overload is contributory [49].…”

Assessment and treatment of pain in thalassemia

“…Previous studies show that hemoglobin level, bone density, iron status, and chelator use are not different between the groups with and without pain . The lack of identifiable risk factors is a hindrance to understanding the pathogenesis of pain in thalassemia.…”

“…A comprehensive assessment of pain in thalassemia was conducted in patients living in North America by the Thalassemia Clinical Research Network . This survey utilized the Brief Pain Inventory (BPI), an instrument that has demonstrated validity over multiple cultures, languages, and diagnoses . BPI assesses the pain perception (or sensory dimension) using questions on severity of pain and interference with daily life (reactive dimension), asking about general activities, mood, and sleep.…”

“…Although individuals 18–44 years of age had a lower prevalence of category 3 or 4 pain than those in older age groups, no difference was seen between those 45–64 years and those older than 65 years. It is notable that instead of the age threshold of 45 years in the general U.S. population at which prevalence of pain increases, this increase is observed at 35 years of age in thalassemia . In the national survey, it was observed that individuals with category 3 or 4 pain have worse health status, use more health care, and suffer from more disability than those with less severe pain .…”

“…Although average hemoglobin level is not significantly lower in individuals with pain, it may be very important in individual cases. A low hemoglobin, such as would occur at the end of a transfusion cycle, is the most frequently cited cause for worsening of pain (45%) that decreased after blood transfusion . Patients with delayed start of regular transfusions, and those with thalassemia intermedia, may be at higher risk for developing pain owing to continued hyperexpanded bone marrow .…”