Consensus Statement of the Indian Academy of Pediatrics in Diagnosis and Management of Hemophilia

Anupam, Sachdeva; Gunasekaran, Vinod; Ramya, H.N.; Dass, Jasmita; Kotwal, Jyoti; Seth, Tulika; Das, Satyaranjan; Garg, Kapil; Kalra, Manas; Sirisha, Rani S; Prakash, Anand; Diagnosis, Consensus in

doi:10.1007/s13312-018-1302-8

Cited by 17 publications

(13 citation statements)

References 25 publications

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“…A study in Southern Iran with no subjects on prophylaxis treatment showed that their total HRQoL was similar to our study 20 . Likewise, in India, where the implementation of the prophylaxis program is not routine, the total HRQoL is almost the same as our findings 11,21 . Meanwhile, the total score HRQoL of haemophilia children in Europe was 21.08, which means less impairment than HRQoL in our study 8 .…”

Section: Discussionsupporting

confidence: 84%

Health‐related quality of life of Indonesian children with hemophilia

Maimon,

Widjajanto,

Sitaresmi

2024

Haemophilia

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IntroductionMeasuring health‐related quality of life (HRQoL) in haemophilia patients provides a comprehensive patient's functional and the impact of the disease and its treatment.AimThis study aimed to assess the HRQoL of haemophilia children and related factors.MethodsWe conducted a cross‐sectional study from 6 April 2019 to 29 April 2019. We recruited children aged 8–16 years old who were members of the Yogyakarta Province Branch, Indonesian Haemophilia Society. Children filled in the Indonesian Haemo‐QoL short‐version questionnaire that consists of nine dimensions, and the score ranges from 0 to 100, in which a higher score shows a higher impartment.ResultsForty‐four children participated in this study. The mean age was 11.99 + 2.49. The median age of the first bleeding episode was 5 years, and the median frequency of spontaneous bleeding during the previous year was 13.5. All children received antihemophilic factor (AHF) on‐demand; most (81.8%) received AHF in the advanced symptoms. The mean total score of HRQoL was 42.7 ± 15.5, with the highest impairment in the family dimension, with a mean score of 68.0 ± 22.3. The severity of haemophilia was significantly correlated with the total score of HRQoL and dimensions of physical health, feeling, view, and family (effect size ranges from 0.30 to 0.42). The frequency of bleeding was significantly related to the total score of HRQoL and the feeling dimension (effect size of 0.47 and 0.34).ConclusionThe HRQoL of our study was lower than in countries where AHF prophylactic is provided. More severe haemophilia and more frequent bleeding significantly lower HRQoL.

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Section: Discussionsupporting

confidence: 84%

Health‐related quality of life of Indonesian children with hemophilia

Maimon,

Widjajanto,

Sitaresmi

2024

Haemophilia

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“…Data on LDP in lowresource settings has become available in recent years and shows that it can help reduce the number of bleeding episodes and the associated long-term issues with joint health experienced by PwH [22][23][24][25] . LDP is recommended by the Indian Academy of Pediatrics [26] and is also the standard of care for low resource settings recommended by the WFH [6] . Therapeutic care programmes involving LDP can help children with haemophilia in academic achievement by lowering bleeding complications and the number of days when they are from school and help them to actively participate in school and social functions.…”

Section: On Demandmentioning

confidence: 99%

Improvement in school absence after factor replacement in students with haemophilia in Upper Assam, India

Dutta

Boruah

Dutta

et al. 2020

The Journal of Haemophilia Practice

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BackgroundPatients with haemophilia who are not adequately treated experience a lifetime burden of joint complications and loss of functional ability due to repeated bleeding episodes caused by low levels of clotting factor VIII or IX in the blood. These complications can significantly impact day-to-day life, including active participation in school and academic study in children with haemophilia (CwH). Treatment with factor replacement therapy can help to prevent this, but access to factor has been challenging in low-resource settings such as Northeast India. This study shows the impact of factor replacement therapy on bleeding episodes, joint complications and school absence among CwH in this setting.MethodsA retrospective observational study was undertaken to examine the impact of receiving regular factor replacement therapy (prophylaxis or on demand) on school absences among CwH registered with the haemophilia treatment centre at Assam Medical College and Hospital. Annual bleed rate (ABR), Haemophilia Joint Health Score (HJHS) and Functional Independence Score in Haemophilia (FISH) were also assessed.ResultsThirty-eight CwH were eligible for the study; 26 (68.4%) were on prophylaxis therapy and 12 (31.5%) received on-demand therapy. In the year before starting regular treatment, the mean ABR was 37.8 (+20.0), HJHS was 31.1 (+18.1) and mean FISH score was 21.1 (+4.2). At the end of the study period the mean (+SD) ABR in prophylactic therapy was significantly lower at 5.8 (+4.6) (p<0.001) and the HJHS was significantly lower at 4.7 (+4.6). FISH score significantly improved to 27.9 (+3.3) (p<0.001). Prophylaxis showed better (but not significant) results in comparison to on-demand therapy.ConclusionsTreatment with factor replacement significantly reduces school absence in CwH and correlates strongly with joint health and functional improvement, with the effect slightly better with prophylaxis than on-demand therapy. Low dose prophylaxis is a good treatment option in low-resource settings, but improvements are also needed in rates of diagnosis.

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“…However, factor concentrates are costly and not easily affordable or accessible by all PwH, especially in developing countries like India. Although recommendations have been made for at least a low-dose regimen of primary prophylaxis in severe haemophilia [1] , high costs prevent this and PwH are generally treated on demand [2] .…”

Section: Abstract: Haemophilia External Injury Haemostatic Patch Indiamentioning

confidence: 99%

Haemostatic action of a topical foam-based patch (VELSEAL-T) in haemophiliac patients with external bleeding

Dutta

Das

et al. 2020

The Journal of Haemophilia Practice

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AbstractIntroductionHaemophilia is an X-linked congenital bleeding disorder due to deficiency of coagulation factor VIII (in haemophilia A) or factor IX (in haemophilia B) caused by mutations of the respective clotting factor genes. Treatment involves the administration of an appropriate dose of factor concentrate, as soon as possible, in the event of any bleeding episode. In low-resource settings, such as Northeast India, where factor concentrates are not widely available, people with haemophilia (PwH) may bleed profusely even from trivial external injuries, warranting transfusion of blood or blood products. We previously reported on the use of a low cost, foam-based haemostatic patch to treat an external bleed in a single patient. In this study, we investigated its use to treat a range of external injuries in PwH presenting at Assam Medical College and Hospital.MethodOver 24 months, eligible PwH with external injuries attending our haemophilia clinic were treated with a topical haemostatic patch (VELSEAL-T) at the target bleeding site. The time to cessation of bleeding was recorded and the wound sites evaluated after haemostasis to monitor efficacy and safety.ResultsOut of 72 individuals with bleeding disorders who volunteered to participate, 59 cases of external bleeding in 48 PwH were eligible for inclusion in the study. Nine (15.3%) had aberration wounds, 24 (40.7%) cut wounds, 21 (35.6%) tooth and/or gum bleeding and five (8.4%) bleeding from puncture wounds. The average time required for achievement of haemostasis was 9.9 (±4.7) minutes. Aberration wounds required the least amount of time for haemostasis at 7.3 (±4.4) minutes. Cut wounds required a mean time of 8.5 (±2.9) minutes; puncture wounds required 9.0 (±3.1) minutes; gum bleeding required the longest time to achieve haemostasis with a mean of 12.7 (±5.6) minutes.ConclusionThe use of this topical haemostatic patch has been shown to be beneficial in the treatment of external injuries in PwH, and provides a good treatment option in resource-constrained areas. A larger controlled study would be helpful to further investigate its efficacy and safety.

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Consensus Statement of the Indian Academy of Pediatrics in Diagnosis and Management of Hemophilia

Cited by 17 publications

References 25 publications

Health‐related quality of life of Indonesian children with hemophilia

Health‐related quality of life of Indonesian children with hemophilia

Improvement in school absence after factor replacement in students with haemophilia in Upper Assam, India

Haemostatic action of a topical foam-based patch (VELSEAL-T) in haemophiliac patients with external bleeding

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