Sankalp is committed to the cause of ensuring no one dies due to lack of blood. We follow high standards of quality monitoring. We realized that we were receiving unusually high no of repeated blood requests from a ward in a Govt. Hospital. Additionally, the patients for whom blood was required were also the same. In order to understand the situation better, the volunteers visited that ward in 2010. They were confronted with children who had hemoglobin of 3-6 gm/dl. Someone explained to the volunteers that the children were suffering from thalassemia. The plight of these children was too much to be ignored.
After deliberations, planning and a lot of work, Sankalp supported the first thalassemia day care centre at Indira Gandhi Institute of Child Health on 14th November 2011. This was a time when the organisation was taking pedagogic steps into understanding the world of thalassemia management. The same pursuit of excellence and quality led the organisation to uncover various problems and solutions associated with effective management of Thalassemia. We would like to share with you 12 things that we learnt on our journey from the first 60 children being supported by the organisation to now – when we have 550 children taking transfusions at multiple thalassemia centres supported by us.
1. Follow up on patients who miss appointments is crucial
There are various complex social situations that a parent may be facing – especially in the case of genetic disorders which are considered largely incurable by the society. Many parents face problems related to their family, employment and finance which prevent them from coming for transfusions at the appointed time. Investing in understanding their problems enables making small changes which can dramatically improve their compliance.
Sankalp realised that some parents don’t come because they don’t have enough money to reach the centre. They were too shy to even admit this. A little effort was put in to resolve the situations and transport support was introduced. Of course the children are doing well now.
2. Option of cure is hope and enhanced compliance
The hope for cure is a very strong motivator for the families. Each thalassemia care centre must invest in exploring cure from the disorder through BMT. Even if one child is on the way to transplant, it radiates hope in the mind of other parents. This hope promotes compliance and helps overcome neglect.
Once Sankalp started offering HLA typing, dramatic improvement in attitude and compliance was observed. While just 5 families had a match, the HLA typing had a lasting effect on the centre as a whole. It was no longer fight against death – it was fight for a normal life.
3. Focus on prevention of complications
By the time the complications surface, a lot of damage is already done to the body. Several complications are irreversible. However, regular monitoring enables identification of complications at an early stage when they are manageable. Cost of screening of all children for complications may work-out to be a small fraction of the cost of management of the complications which otherwise would have happened. Serological, bio-chemistry, cardiac, endocrine and haematological tests done as a matter of routine have enabled us to early detect several complications. The interventions at an early stage are easy, effective and low cost.
4. Focus on preventing splenectomy
Splenectomy or removal of spleen is done routinely for thalassemia patients. It reduces the destruction of normal donor red cells in the patient’s circulation and helps reduce transfusion requirement. Once the child starts receiving more than 1 bag of blood a week, the doctors consider splenectomy. Once the spleen is removed the chances of mortality are much higher. Modern medicine offers alternatives to reduce transfusion requirements including use of hydroxyurea. All attempts should be made to preserve the spleen.
We did face the difficult time of seeing some of our children lose the fight. The most common underlying cause was severe septicemia. The common thread connecting these children was that they were splenectomised. With persistent effort – we have been able to prevent the children who otherwise were lined up for splenectomy to do well without it.
5. Focus on Quality of Life rather than manage transfusions and medicines
The centres giving care for thalassemia must emphasise on enabling better quality of life for these children as opposed to focussing only on giving transfusions and chelation. The message that thalassemia is a manageable blood disorder must reach the parents with adequate emphasis. Counselling should be done to ensure children are sent to school, treated at par with their siblings and made to feel normal.
We are glad to see children joining school again after counselling. We realise that the parents begin to think about education and future when appropriately counselled. The trauma of the past – when regular management was extremely difficult for the parents – leads to deep rooted negativity which must be removed with gentle and persistent counselling. If invested upon – it shows great results.
6. Structured data enables better management
Managing data through ThalCare – the world’s first web based application on comprehensive Thalassemia Management, enables frequent review of outcome and progress both on a per centre level and at the level of the individual patients. This ability greatly enables early detection of problems and timely interventions. The overall outcome of thalassemia clinic is greatly enhanced with minimal additional effort.
ThalCare has enabled extremely effective monitoring of the progress of the children as well as the centres enabling centres to treat more patients within the same setup while maintaining high standards of care in-line with the international protocols. Structured data allows predicting problems, highlighting issues and enables focussed attention.
7. Quality assessment and reporting saves time, money and prevents suffering
Assessment of essential quality parameters related to the delivery of services is crucial. Quality of nursing, lab investigations, blood products, blood bank services etc. should be carefully monitored. Poor quality not only has adverse impact on the overall outcome but also significantly impacts the time, money and effort that the centre, parent and the patient puts into disease management.
Weekly progress reports of the children on quality parameters enables early detection of organisational issues. Blood availability, staffing problems, problems with quality of investigations etc. are aggregated and made available which allow swift interventions and corrections. Hospitalisation is reduced, use of resources optimised and the delivery of care streamlined – all contributing to better care and management.
8. Reactions should not be masked – they should be addressed
The practice of giving prophylactic medicines to patients prior to transfusions may lead to suppressing the real problems associated with quality of blood products, suitability of blood products and development of antibodies – sometime worsening the situation. Reactions need to be noted, followed up and the underlying causes identified to enable proper care.
9. Simple double/triple saline wash is very effective
While leuko-depletion filters are good, there are financial constraints limiting their use. In this scenario double/triple saline wash of red cells is a cost effective and easy to organise first step into leuko-depletion of blood products. Done under proper environment with care, saline wash is very effective.
Several children at our centres who earlier complained of transfusion related reactions were much better off when they were given saline washed RBCs with fewer (sometime no more) reactions.
10. Focus on prevention of Thalassemia
Even asking a parent if there is anyone pregnant in the family could enable timely prevention. While cascade and population screening are great, when that is not happening, simple questions enable identifying risky pregnancies and targeted interventions.
We were able to offer antenatal screening to 12 mothers who were at risk to give birth to a thalassemiac child and 4 were detected carrying a thalassemia major.
11. Systematic centre management is crucial
Planning for appointments, availability of blood products and schedule of tests etc. must be done before the patient arrives at the day care. Creating a structured workflow of events starting from when a patient walks in, managing blood products for transfusion prior to visit, enabling speedy and timely delivery of tests, chelators etc. leads to minimised hospitalisation and restores normalcy of life.
We were able to limit hospitalisation from an average of 10 hours to 5-6 hours. At one centre we were able to run two shifts a day for transfusion thus maximising the utilisation of space. In-spite of these optimisations, the distribution of chelators and the scheduled lab investigations happened with greater adherence because of a dedicated staff undertaking systematic and pre-planned management.
12. Regular blood donation drives
For the success of the thalassemia day care program, there needs to be great emphasis in pre-scheduling and planning blood donation camps at regular intervals. Since the children need blood units collected in last 7 days, weekly blood donation drives are advisable. Unlike other scenarios of blood requirement, the day care centres need regular supply of fresh blood units and hence supply during holiday season and periods of shortage need to be carefully planned.