Thalassemia major is characterized by the individual not being able to produce enough good hemoglobin. This is called ineffective erythropoiesis. This condition leads to a cascade of compensatory mechanisms. First it leads to massive bone marrow expansion, unusual organs become involved in attempting to make blood cellular components, the absorption of iron from the food intake increases and consequently there is severe organ damage.
The first and foremost step in proper thalassemia management is to shut down the compensatory mechanisms of the body to produce blood cells and thereby avoid a host of complications. This is done by ensuring that the patient has high enough haemoglobin levels supported by blood transfusions. Hypertransfusion is a widely accepted and effective mechanism which aims at keeping the pre-transfusion haemoglobin levels of the patients at 9 gm/dl.
At, Sankalp India Foundation, the program for thalassemia care and management has adequate blood transfusions is the key objective. Across several centers in which Sankalp is involved, all blood transfusions are free of charge (the product, consumables and the procedure all are free). Blood is sourced from voluntary non-remunerative blood donors exclusively and the parents are not involved in any way in the process of organizing for blood. The organization supports blood banking at large with blood donation camps especially at times of shortage. All this is aimed at ensuring that there is adequate supply of safe blood seamlessly and without the limitations of finances for each patient suffering from thalassemia.
The organization uses ThalCare to track every single transfusion, patient vitals, labs, chelation, complications, adherence, socio-economic issues etc. for about 750 patients. The regular tracking ensures that we have a detailed view on how our system is responding to the patient's clinical conditions.
Patient enrollment
Vast majority of patients from economically poor background.
Almost 50% of patients travel from outside the town where the centers are located.
Almost all patients join the centers in bad shape having received sub-optimal care and management for years.
IGICH receives a large number of patients at the time of first transfusion itself and thus has an opportunity to initiate early corrective action.
Patients with bad prognosis are being referred for better management and care to our centers.
Patients come predominantly from Karnataka and part of Tamil Nadu, Andhra Pradesh.
Our Centers
Thalassemia Day Care, Indira Gandhi Institute of Child Health, Bangalore - since November 2011
Project Samraksha, Rashtrotthana Parishat, Bangalore since August 2013
ai Shivshakti Centre for Thalassemia Management, JNMC and KLES Dr. Prabhakar Kore Hospital, Belgaum since May 2016
We evaluated the trends of pre-transfusion haemoglobin for the patients attending our centre over a period of time. We plotted them to show over a period of time what percentage of patient came with haemoglobin levels of 6gm/dl, then in steps of 1 up-to 10gm/dl. Here are the plots: How to interpret the charts
In any given time period, the larger the share of green color the better the performance of the centre.
From left to right, the reduction in the share of red and yellow means fewer children are coming with lower haemoglobin levels.
The right side of the chart shows the number of children who came during each time period plotted in the black line.
Children who are newly enrolled typically come with poorer haemoglobin levels and need months of therapy before their haemoglobin comes to stable levels.
Findings
Each centre has shown progressive decline in the number of patients visiting with lower hemoglobin levels even as the overall enrolment and number of patient visits is increasing.
Monthly meetings are being organised with the blood bank on scheduling, adequacy of blood and reactions associated with transfusions.
Indira Gandhi Institute of Child Health has thalassemia program for last 5 years with ongoing new enrollments several directly at diagnosis. The centre has demonstrated improving haemoglobin trends against several odds.
Project Samraksha is the standalone centre attached to a Blood Bank which manages toughest cases and get's referrals of highly mismanaged patients. The sustained hemoglobin level should be seen in light of high rate of ongoing new enrollments , intense use of hydroxyurea and occasionally super transfusion as clinically indicated.
Jawaharlal Nehru Medical College and KLE Hospitals, Belgaum is a Medical College and Hospital where transformation seen since May 2016 when organized thalassemia management program was initiated. The adoption of standard procedures and protocols seems to have worked very well.
Government, private as well as non-profit run institutions using common protocols have shown similar improvements.
How do we achieve progressively improving hemoglobin levels
Blood provisioning is not the patient's responsibility
Appointment for next transfusion given based upon patient specific transfusion history .
Precise system generated volume transfusion as opposed to number of bags enabling better predictability.
Weekly and monthly review of the pre-transfusion hemoglobin level.
Focus upon counseling and assistance to those who come repeatedly with low Hb to identify cause.
Several patients receiving financial support for transportation as well no excuse to miss transfusion appointment.
Intense planning for seasonal shortage of blood to ensure continued supply.
We measure progress, review periodically and respond to changes.
Conclusions
The pre-transfusion haemoglobin trends are very encouraging and confirm that organized, systematic approach towards thalassemia management with the aid of technology can ensure that the elusive goal of ensuring steady and safe haemoglobin levels is indeed reachable. Our sincere gratitude and appreciation for the numerous voluntary blood donors, the blood bank staff and blood bank managements and the partner institutions whose persistent and selfless contributions have made this possible. A word of sincere appreciation also for the financial contributors who support us in leaving no stone unturned to facilitating compliance to the planned therapy. And lastly, the relentless efforts of our doctors, nurses and coordinators who have worked painstakingly and tirelessly to put together the necessary elements needed to get fine results.
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