by Dr Reshma Shrinivas, Medical Officer In-charge, Project Samrkasha Thalassemia Day Care, Rashtrotthana Parishat, Bangalore
On 1st March 2016, when 13 year old Viren(name changed) was brought to Samraksha for the very first time, he was carried by his father on his back. The staff was surprised looking at a 13 yr old being carried by his father. The father explained to us saying Viren cannot walk even a furlong distance.
Viren was apparently normal till the age of 8yrs. He was not attaining growth and height like the other children of his age. Rather, he had thin legs and hands with huge protruded abdomen and projected upper jaw and was complaining of fatigue. Parents assumed that he was under nourished and started to feed him high calorie food .
However, his condition worsened. He was not able to digest the food and had loose motions very often. Also his fatigue increased day by day. He stopped going to school as he wasn't able to walk. One fine day, he suddenly fainted and was rushed to hospital. After relevant investigations, he was diagnosed to have thalassemia. They were told that he has to be on blood transfusions for the rest of his life. That point onwards he started on regular transfusions at a Government hospital in Bangalore. When he was transfused blood for third time, he developed allergic reactions with skin rashes and blood in urine. Immediately transfusion was stopped and his blood was sent for irregular antibody testing which revealed he had developed irregular antibodies. The fact that the child had O-ve blood group together with irregular antibodies made it extremely difficult to organize any blood units for him.
Thanks to the irregular antibodies, finding blood suitable for the child became extremely difficult. The child had frequent reactions. When the family met Dr.Stalin Ramprakash, haemato-oncologist at Sridevi Medical College and a visiting consultant at Sankalp-People Tree Centre for Pediatric Bone Marrow Transplantation, he felt that the child may receive better management at Samraksha and referred him to us.
On the very first examination we realized that the child had developed several complications. On examination he was pale, had breathing difficulty, showed signs of jaundice, chest retractions, heart sounds signifying failing heart, large liver and spleen (spleen was 10cm and liver was 8cm), malnourished and to top it all, he had the blood group which of O-ve. His white cells and platelets were also low because of large spleen size a consequence of chronic under-transfusion, and he was at the verge of de-compensation and downhill course.
In-spite of all the above complications, we took him up as a challenge to treat this smart little boy and give life to his dreams. Before we commenced with the treatment counseled father about high risk stage he is in and took consent from him to proceed with following proposed treatment.
We carried out a thorough evaluation and the following medical examinations were organized:
- 2D ECHO-revealed that failing heart was due to persistent anaemia (decreased haemoglobin)
- Ultrasound abdomen-to confirm liver and spleen size
- Serum ferritin to check iron load status
- Screening for transfusion transmitted infections
- Biochemistry panel which indicated high bilirubin levels.
- Complete blood count which showed a hemoglobin of 3 gm/dl
We decided to treat the child with bi-weekly packed red blood cell transfusions. We chose the most compatible from a large number of units at Rashtrotthana blood bank for transfusion. The blood product was to be triple saline washed each time and the transfusion of each bag was planned over five hours. We initiated chelation therapy but only after 4-5 transfusions supported by Sankalp India Foundation.
After rigorous transfusions and follow-up, now his hemoglobin is increased to 10.3 g/dl. His heart sounds are becoming better, the liver size has decreased to 2cm, spleen has decreased to 6cm,and he is able to do his daily activities without his parents support.
With a million dollar smile he tells us that he can not only walk but also can play with his friends. He does not feel easily fatigued and already told he has enrolled for admission to school for this academic year.
We hope to be able to undo the damage caused by years of inadequate management have hope to restore the normalcy and quality of his life and that of his family.