HLH is a rare immune deficiency disease. The experience of losing someone special to this vicious disease is at once heartbreaking and unfair…
In the afternoon of March 5, 2001, a tiny slip of a baby came into this world. We were ecstatic. It was a boy. No, there was no gender preference but growing up with three sisters and just one brother the idea of another male entity in the family circle was a cause for double celebration. And that is how my nephew Mihir Laishram came into our lives – as our first bundle of joy.
He grew up a very normal child. But as the only son to his parents and as our only nephew, Mihir was the centre of our universe. We doted on him; we wanted to be a part of his journey in life, at every step. Indeed we watched him grow from a toothless baby to a prankster to someone who liked his studies and enjoyed his sports.
I recall the annual school sports of St Anthony’s School of which he was a student in Shillong, the beautiful town of north east India. I had gone to watch him race the 100 metres and my eyes welled up in tears as his name was announced: “Mihir Laishram from Class VI B”. But no sooner had the gun-shot fired to start the race when someone next to him fell. Instead of racing ahead to win like he won the previous year, he stopped to help thus losing the race. In my eyes that day, he was clearly a champ destined for great things.
I am not trying to make a hero of my nephew. But, yes, if I can call him a hero, it is truly in his fight with his rare disease that ultimately took his life. He fought back from the very edge of death a few times. He did not give up till the end.
In 2011, Mihir turned 10 and fresh from his holiday in Australia he returned to school with excitement. He loved his school. Around April just two months into school, he started developing intermittent fevers. All along he had been a healthy child. The local doctors thought it was a urinary tract infection (UTI). Every time the fever returned he was given medicines for UTI. Later other doctors would tell us that UTI is more common in women as the bacteria can reach the bladder more easily.
By the third month when the fever came up again, he was admitted briefly in the Children’s Hospital for a week. After a strong course of antibiotics he was home. A month later the fever came up yet again. By this time, a decision was taken to take him to the big regional medical college in Guwahati, the nearest town which is 150 km away from Shillong. The head of the medical college Dr J N Sharma examined him thoroughly over days. Numerous tests and scans were done. All they found was an enlarged spleen. Dr Sharma then asked us to take Mihir to the All India Institute of Medical Sciences (AIIMS) in New Delhi for a bone marrow test. He believed in the accuracy of AIIMS’ findings.
AIIMS is India’s premier medical college and being a public institution, its facilities cater to about 1.5 million outpatients and 80,000 inpatients every year. Discouraged by the long queues and very long procedures, we went to the private wing of Gangaram Hospital instead in the capital where the bone marrow test showed everything was normal. A happy family came back to town and resumed life as normal.
But a difficult, long journey would begin few months later when the fever reared its ugly head again. This time we headed straight to AIIMS after consultations with Dr Sharma. It helped that Dr Sharma wrote a letter to the head of paediatrics AIIMS, Dr S K Kabra. Getting a seat is next to impossible given the long queues and volume of patients there. Mihir was admitted right away.
A battery of doctors including those from the department of infectious disease, general paediatrics, and immunology specialists examined him. The paediatric haematology and oncology team took the lead. We were asked questions about history of illnesses in the family, travel to foreign countries, possible exposure to harmful substances or diseases, or even dog bites. Every no answer led to more questions and confusion. One morning a conference was held where Mihir was wheeled in amid the presence of a huge team that discussed his case. The doctors eventually eliminated cancer as a possible diagnosis, which was a huge relief. It took about 13 days before he was finally diagnosed with HLH secondary, a rare immune deficiency disease. AIIMS had seen only a few cases prior to Mihir.
HLH is often caused by an inherited problem of the immune system, which is called “primary” HLH or “familial” HLH. In patients with primary HLH, cells of the immune system, principally T cells and NK cells, don’t work properly to destroy infected or damaged cells as they should. Because of this, the immune system becomes overstimulated and over activated. The immune system then begins to damage the patient’s own tissues and organs, including the bone marrow, the liver and the brain.
Some patients are diagnosed with what is called “secondary” HLH. This term is used when the doctor thinks the HLH may have occurred for a variety of different reasons, but not necessarily because of an inherited condition associated with abnormal function of the immune system.
Both are triggered via infection of some sort. In Mihir’s case it was the Epstein–Barr virus which caused it, although some doctors on condition of anonymity later told us that a genetic link could not be ruled out since hospitals in India do not have all the machines to test gene mutation.
Mihir’s immediate treatment was under the HLH-2004 Protocol followed worldwide. It consisted of a series of weekly treatments with dexamethasone and etoposide. Some patients have gone well into remission with this first line of treatment.
In the early days of his diagnosis, being hospitalised at AIIMS was not easy for Mihir. He was in constant pain from all the injections and medications. At one point, he even asked his mother, “Why am I suffering so much?” My sister told him, “Because you are the bravest of the brave.” A heart-warming incident that comes to mind was when a group of young children had come to pray for all the ailing kids in the ward. As they prayed, “Dear Lord, Please give Mihir the strength to fight his illness, make him alright so he can go to school and play with his friends….”, there were visible tears in his eyes. He told my sister, “Mama I felt so good to hear those words.”
When he came out of hospital he could hardly walk because of what they called muscle atrophy. But he slowly regained strength with all the support and love around him. I do remember taking him to a shopping mall in Delhi in a wheelchair but he was constantly stared at – the flip side of living in India – that I wanted to protect him from all the glares.
After being home for a few months, Mihir had a relapse. Since the first line of treatment did not work for him, stem cell transplant was the next line of treatment. So in August 2012, Mihir became the second person in India to undergo a stem cell transplant for HLH. He had an unrelated donor cord blood transplantation and during the three months’ stay in hospital he showed impressive results. He had little or no complications following the transplant. That year Mihir missed a year in school but he bounced back the next year and except for periodic visit to AIIMS where doctors monitored his progress, he had caught up with life.
Mihir went on to play his favourite game of badminton and last year was even selected for district level championships. He had also caught up remarkably well with his studies.
But HLH is an unforgiving disease. His journey was far from over. In May 2015, he had another relapse. Our world was ripped apart but there was a sunny optimism despite our darkest fears. We had heard patients in western countries undergo few bone marrow transplants before they finally got cured. We were not the one to give up either. But fate plays a cruel part. AIIMS did not give us a seat immediately for his second stem cell transplant (the only treatment for a relapse too). A date was given in August which was later deferred to Jan 22, 2016. It was a waiting period during which time Mihir had to undergo periodic chemo therapy, take immune suppressant drugs besides other medications. Life for the next 7-8 months was spent between hospital and school. But a resilient child that he was, he kept up with a lot of courage and hope. None of his friends knew he was ill because he never talked about his great fight with this monster disease.
Come middle of December 2015, Mihir had an aggressive flare of fever. The blood counts began to show erratic signs and at one time the platelets dropped to a dangerous low of 5,000. By the first week of January 2016, he was back at AIIMS weeks ahead of his scheduled date for transplant.
And we were unprepared for the things that lay ahead of us. AIIMS gave a blank no for further treatments. They said any strong medicines at this stage would have no effect. In other words, palliative care was what they wanted us to resort to as his disease had reached a stage with no curative option. But how can a father tell his child who is not actually bed ridden, who is walking and talking, albeit weak ,that there is no more treatment available? How can one humanly give up?
So in a last ditch effort his father went to meet a stem cell expert at Gangaram Hospital, who told him to bring Mihir there immediately. But it would be Mihir’s last week battling for dear life in the PICU (Paediatrics Intensive Care Unit) of Gangaram. On January 8, I managed to see him in the PICU landing at the hospital straight from a nine-hour flight from Paris in the wee hours of the morning. Our happiness at seeing each other was palpable. As he reached out his pain-ridden hand to touch mine, I told him everything was going to be OK.
The next few days were a blur. “Am I stable mama?” he asked again and again as he was privy to the discussion of doctors around him. Soon the speeches turned to slurs and he was no longer coherent. His major organs started to fail. He had unbearable pain, he constantly asked for water which was denied as he started to bleed from the guts as well. This demonic disease completely destroyed him. On January 15, Mihir was declared dead due to refractory shock as an immediate cause, Sepsis as an antecedent cause and HLH with relapse as an underlying cause. My little champ lost his big battle with HLH.
Our world has been torn apart but the consolation is that in death he is free of his pain. His doctor Samir Bakshi of AIIMS wrote to us: “Mihir was such a wonderful child and always positive in his thoughts.” Others would agree. In that week before he was admitted, Mihir had expressed a lot of desires. He wanted to take up computer science this year in school and he wanted to play more badminton. Positivity was his forte and he left an indelible impression in the sands of the short time he spent on earth.
This March, Mihir was to turn 15. Fifteen years is a long time in a man’s life but for us it was a life truly nipped in its bud. Nothing is more tragic than losing such a precious child.
A beautiful rainbow came into and affected all our lives, but he had to leave soon. He will never be forgotten…
By Indira Laisram