{"id":80740,"date":"2025-12-25T09:30:10","date_gmt":"2025-12-25T09:30:10","guid":{"rendered":"https:\/\/www.newsbeep.com\/us-ny\/80740\/"},"modified":"2025-12-25T09:30:10","modified_gmt":"2025-12-25T09:30:10","slug":"only-hope-for-infant-with-rare-new-genetic-disease-was-risky-tranplant-at-nyc-hospital","status":"publish","type":"post","link":"https:\/\/www.newsbeep.com\/us-ny\/80740\/","title":{"rendered":"Only hope for infant with rare new genetic disease was risky tranplant at NYC hospital"},"content":{"rendered":"

When little Dhruvan Gopi was taken by his parents to a New Jersey urgent care center, they never imagined he was suffering acute liver failure at just 7 weeks old. The shocking discovery set off a race to keep him alive long enough to receive a seldom-performed risky organ transplant<\/a> at Manhattan\u2019s Mount Sinai Hospital<\/a>.<\/p>\n

Over the next few days, he was rushed between hospitals in New Jersey and New York, his blood stopped clotting, his parents were warned he might not survive the night, and a large team of specialists converged around his crib. The doctors didn\u2019t yet even understand why a baby who had seemed perfectly healthy just a week earlier was suddenly fighting for his life.<\/p>\n

He would later be discovered to be suffering from an incredibly rare newly discovered genetic disease<\/a>.<\/p>\n

First signs of trouble<\/p>\n

Dhruvan was born on Oct. 15, 2024, to Gopi and Prabha Subramanian, who live in Edison, N.J. \u201cHe was fine for six weeks,\u201d recalled Gopi, a software engineer from Chennai in Southern India.<\/p>\n

Then one Tuesday in late November, things changed. His urine was dark in color and he began throwing up. His parents brought him to their local urgent care clinic, expecting to be back home in a few hours.<\/p>\n

\"TheThe Subramanian family. (Courtesy of Family)<\/p>\n

But staffers there sent them to a local emergency room, where doctors found the infant\u2019s liver numbers to be way off. He was transferred to St. Peter\u2019s University Hospital, where he had been born less than two months earlier. There he was treated for a urinary tract infection, but his liver tests kept looking worse. His blood stopped clotting properly. His platelets and red blood cells were dropping, forcing doctors to transfuse him just to keep him stable.<\/p>\n

That\u2019s when the parents got him to Mount Sinai hospital on the Upper East Side, recommended to them by a friend. Doctors there told them something unimaginable: their son very well might not survive the night.<\/p>\n

\u2018A day or so to live\u2019<\/p>\n

When Dhruvan arrived at Mount Sinai\u2019s neonatal intensive care unit<\/a> (NICU) on Dec. 2, he was in rapidly progressive liver failure.<\/p>\n

\u201cHe literally had only a day or so to live,\u201d said Dr. Sander Florman,\u00a0 director of the Recanati\/Miller Transplantation Institute, the transplant surgeon who would go on to perform a harrowing 11-hour surgery on the infant.<\/p>\n

\"DhruvanDhruvan Gopi at his home in New Jersey. (Courtesy of Family)<\/p>\n

The medical team raced to run every test they could: blood work, imaging, specialist consults. On Dec. 4, 2024, as they prepared to place him on a transplant waiting list, Florman\u2019s phone rang. It was a colleague at another New York transplant center.<\/p>\n

\u201cWould you ever use a 2-year-old liver for a \u2018domino?’\u201d she asked him, Florman recalled<\/p>\n

\u201cI said, \u2018Are you kidding me, what blood type?\u2019 And it was the same blood type [as Dhruvan].\u201d<\/p>\n

Most people have never heard of a domino liver transplant, in which one patient both gives up a liver, which goes to somebody else, and receives a liver from a third person. It\u2019s one of the rarest scenarios in medicine. Mount Sinai\u2019s team estimates they\u2019ve done just a dozen in 40 years.<\/p>\n

\u201cWe\u2019re going to take it out in 15 minutes,\u201d Florman\u2019s colleague told him about the 2-year-old\u2019s liver.<\/p>\n

When Florman got that call, he had still never met Dhruvan\u2019s parents. He walked straight to the NICU to meet the Subramanians for the first time.<\/p>\n

\u201cThe kid is absolutely going to die within 24 hours or so,\u201d he said of little Dhruvan.\u00a0 He had to explain, quickly but clearly, what a domino transplant was to the infant\u2019s shocked parents.<\/p>\n

\"SubramanianSubramanian family at Mount Sinai. (Courtesy of Mount Sinai Health System)<\/p>\n

A liver that is a problem for one child can in rare circumstances save the life of another. The 2-year-old was suffering from a rare metabolic disease called Maple Syrup Urine Disease<\/a> (MSUD), named for the sweet smell it can give a child\u2019s urine. A missing enzyme was letting certain substances build up to dangerous levels in that child\u2019s body.<\/p>\n

The 2-year-old\u2019s liver, one of the body\u2019s main producers of the missing enzyme, was not making the enzyme the way it should. But the liver was otherwise normal and doing its usual jobs of filtering blood, making bile and supporting metabolism.<\/p>\n

\u201cYou can take their liver and give it to somebody else who doesn\u2019t have that problem, because they make the enzyme somewhere else in their body,\u201d the surgeon explained.<\/p>\n

A 10-minute decision<\/p>\n

The parents were stunned. \u201cWe were very hesitant,\u201d Gopi said. \u201cYou\u2019re not mentally prepared for something like this.\u201d<\/p>\n

But the surgeon was blunt: For a baby this small getting a liver of this size is very rare, Gopi remembered him saying.<\/p>\n

\u201cThat\u2019s perfect size,\u201d Florman said. \u201cWe can split it, and we can still fit it under his belly.\u2019\u201d<\/p>\n

The Subramanians said yes.<\/p>\n

\"Dr.Dr. Courtney Juliano, MD, System Chief, Division of Newborn Medicine, Jack and Lucy Department of Pediatrics, Mount Sinai Kravis Children’s Hospital, and Dr. Sander Florman, MD, Director of the Recanati\/Miller Transplantation Institute, the transplant surgeon who operated on Dhruvan. (Mount Sinai Health System)<\/p>\n

The 2-year-old\u2019s liver was removed and rushed to Mount Sinai. While anesthesiologists put Dhruvan to sleep, the surgeon split the donor liver, trimming it down to a piece small enough to fit inside a 10-pound baby.<\/p>\n

\u201cIt\u2019s one of the most unbelievable, remarkable, serendipitous cases,\u201d Florman said. \u201cThese are very high-risk transplants and very few places in the country will take on children under 10 pounds.\u201d<\/p>\n

Dhruvan received the liver from the 2-year-old patient while that baby received a new liver from another patient and went on to do very well. But little Dhruvan still had a rocky road ahead.<\/p>\n

The surgery was only the beginning<\/p>\n

Even after the transplant, no one knew why a seemingly healthy newborn had plunged into liver failure after a simple infection. Mount Sinai\u2019s genetics team kept digging. Eventually, they found the answer in a scientific paper first published in 2023.<\/p>\n

\"DhruvanDhruvan smiling. (Courtesy of Family)<\/p>\n

It was a mutation in a gene called DOCK11, an incredibly rare genetic glitch<\/a> affecting how immune cells form and move.<\/p>\n

\u201cThese are rarest of the cases. In fact, there\u2019s no case of anybody being transplanted for this,\u201d Florman said. \u201cThese kids generally die \u2026 It\u2019s a brand-new class of genetic mutations that people are only just starting to understand.\u201d<\/p>\n

The DOCK11 mutation causes immune dysregulation, leaving patients vulnerable to overwhelming infections and runaway inflammation. In Dhruvan\u2019s case, the urinary tract infection was the spark that set off his immune system and destroyed his liver.<\/p>\n

The diagnosis also complicated his recovery. Transplant patients must take drugs that suppress the immune system to prevent rejection. The very system that, in his case, was already miswired.<\/p>\n

Still, there was no alternative. \u201cThis was the kid\u2019s only chance to be alive,\u201d Florman said.<\/p>\n

Months of setbacks and small victories<\/p>\n

After surgery, Dhruvan returned to the NICU, his abdomen initially left open because the new liver was still too big. Tubes and lines snaked from his tiny body.<\/p>\n

\"DhruvanDhruvan soon after transplant. (Courtesy of Family)<\/p>\n

He went on to face complications including blood clots, an infection in his belly requiring repeat surgeries, massive swelling that made him appear almost double his size.<\/p>\n

\u201cHe was very weak,\u201d said Dr. Courtney Juliano, the system chief of the division of newborn medicine at Mount Sinai Kravis Children\u2019s Hospital. \u201cHe required a breathing tube and a ventilator for a very prolonged period of time.\u201d<\/p>\n

Every morning, a large multidisciplinary team would crowd around his incubator.<\/p>\n

\u201cThe hospital staff and nurse took care of him like their own baby,\u201d Gopi said.<\/p>\n

Adjusting to post-hospital life<\/p>\n

After 4\u00bd months in the hospital, Dhruvan finally came home.<\/p>\n

\u201cWhen he was getting released, we were happy,\u201d his father said. \u201cOK, he is back home. But then it hits you hard. He\u2019ll be on a lot of medicines, a lot of care is required every single day. And then the lifelong immune suppression.\u201d<\/p>\n

\"DhruvanDhruvan Gopi after surgery. (Courtesy of Family)<\/p>\n

\u201cWe started with 11 medicines,\u201d he added. \u201cMorning and evening.\u201d<\/p>\n

Twice Dhruvan\u2019s parents rushed him to the emergency room for FPIES, a severe gut reaction where his body violently rejects certain foods. But each time, he bounced back, tolerating foods that once made him desperately ill.<\/p>\n

Stay in the present<\/p>\n

\u201cI\u2019m not a person who used to pray to God every single day. But now I do,\u201d Gopi said. \u201cIt changed his Mommy and definitely it changed us. We start having a lot of appreciation for what we already have.\u201d<\/p>\n

\u201cStay in the present \u2014 that\u2019s what I started doing more,\u201d he added. \u201cLive in the moment.\u201d<\/p>\n

Their older son, who is 8, dotes on his little brother. The house is noisy again.<\/p>\n

Dhruvan is already showing a stubborn streak. \u201c<\/p>\n

\"DhruvanDhruvan with his mother Prabha Subramanian at Mount Sinai Hospital earlier this month. (Courtesy of Mount Sinai Health System)<\/p>\n

If I go by his crib, I have to lift him,\u201d Gopi laughed. \u201cOtherwise, he\u2019ll shout, he\u2019ll do silly things, like try to bump his head into the railing to say, \u2018Hey, I\u2019m here.\u2019 He has his ways of getting things done.\u201d<\/p>\n

Back at Mount Sinai, the team that once fought to keep him alive now gets to enjoy the payoff.<\/p>\n

\u201cWhat we hope for is more than just survival. I want babies who are thriving, and he\u2019s a great example of that,\u201d said Juliano. \u201cEach time his parents bring him back, he\u2019s looking better, bigger, stronger, healthier, with less signs of what he went through.\u201d<\/p>\n

Eariler this month, Dhruvan, now 15 months old, was busy doing what toddlers do best when he made a return visit to Mount Sinai Hospital. Squirming, grabbing at his mother\u2019s shoulders and protesting whenever anyone tried to put him down.<\/p>\n

You wouldn\u2019t know that beneath his T-shirt a long scar runs down his tiny belly, a reminder of the night last year when doctors weren\u2019t sure he would live to see morning.<\/p>\n

\u201cCould there be anything more gratifying than seeing them a year later, running around like a normal kid?\u201d said Florman, a year after operating on him. \u201cToday, he looks like any normal 1-year-old kid.\u201d<\/p>\n","protected":false},"excerpt":{"rendered":"When little Dhruvan Gopi was taken by his parents to a New Jersey urgent care center, they never…\n","protected":false},"author":2,"featured_media":80741,"comment_status":"","ping_status":"","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[38],"tags":[128,267,75,9,24,264,10,12,63,129,131,130],"class_list":["post-80740","post","type-post","status-publish","format-standard","has-post-thumbnail","category-the-bronx","tag-bronx","tag-local-news","tag-manhattan","tag-new-york","tag-new-york-city","tag-new-york-county","tag-new-york-news","tag-news","tag-nyc","tag-the-bronx","tag-the-bronx-headlines","tag-the-bronx-news"],"_links":{"self":[{"href":"https:\/\/www.newsbeep.com\/us-ny\/wp-json\/wp\/v2\/posts\/80740","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.newsbeep.com\/us-ny\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.newsbeep.com\/us-ny\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.newsbeep.com\/us-ny\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/www.newsbeep.com\/us-ny\/wp-json\/wp\/v2\/comments?post=80740"}],"version-history":[{"count":0,"href":"https:\/\/www.newsbeep.com\/us-ny\/wp-json\/wp\/v2\/posts\/80740\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.newsbeep.com\/us-ny\/wp-json\/wp\/v2\/media\/80741"}],"wp:attachment":[{"href":"https:\/\/www.newsbeep.com\/us-ny\/wp-json\/wp\/v2\/media?parent=80740"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.newsbeep.com\/us-ny\/wp-json\/wp\/v2\/categories?post=80740"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.newsbeep.com\/us-ny\/wp-json\/wp\/v2\/tags?post=80740"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}