TEXAS TOWNSHIP, Mich. (WOOD) — A 4-month-old from the Kalamazoo area is one of only a few in the world with a rare condition similar to the one that recently took the life of British baby Charlie Gard. 
 
 Russell Cruzan III’s parents are fighting to find him treatment that could save his life. Even though doctors say Russell’s chances of survival are slim, his parents are hoping he’ll defy the odds. 
 
 Held by his mother in his Texas Township home Monday, Russell’s breathing was labored. 
 
 “It’s really hard to be told that your child’s chances of even making it to 2 are like 50 percent,” his mother, Michelle Budnick-Nap, said tearfully. 
 
 She and Russell’s father noticed something was wrong in early June. 
 
 “(We) kept asking the nurses why. Why is he not eating? Why isn’t he thriving?” Budnik-Nap recalled. 
 
 There were no answers. And Russell kept getting worse. Dehydrated and lethargic with a low body temperature, he was admitted to Bronson Children’s Hospital for a week. 
 
 Doctors treated the symptoms, but Russell’s parents say they weren’t ever given a reason why their child was sick. 
 
 Two days after Russell was released, his parents rushed him back to the hospital when he started having severe respiratory problems and then developed pneumonia. Russell was in the hospital for a month. 
 
 Still, no answers. 
 
 Until a test on his kidneys came back. 
 
 “When those labs came back the doctor called and she was like, ‘You need to bring him into the hospital right now. His labs are looking kind of crazy. We need to find out what’s going on,'” Budnik-Nap said. 
 
 The diagnosis was mitochondrial DNA depletion syndrome 13, which can cause a loss of muscle mobility and strength, decreased kidney function and an inability to swallow. 
 
 Charlie Gard also had mitochondrial depletion syndrome. 
 
 The baby in the United Kingdom was at the center of a legal battle over whether his parents should be allowed to bring him to the United States for experimental treatment. Doctors objected, saying it wouldn’t help and could in fact hurt Charlie. Eventually, his parents ended their fight when it became clear that the treatment could no longer save their son. 
 
 Charlie died last week. He would have turned 1 this week. 
 
 After Russell’s diagnosis, doctors told his parents the the odds were against him. 
 
 “‘We know the seriousness of this diagnosis. We know what the prognosis is. Do you want to keep fighting or do you want to give up and let nature take its course?'” Burdik-Nap said she was told. “Which is a really, really hard thing to hear. But I always tell them, ‘Bo, we want to do everything we can. We want to fight.'” 
 
 That’s why she and Russell’s father are working to get to Boston. 
 
 “If anything if they can’t save him or whatever — maybe he can be used to save kids in the future,” Russell Cruzan II said. 
 
 They say a doctor at Boston Children’s Hospital, who is one of the top mitochondrial physicians in the country, has agreed to treat Russell if a doctor at Bronson can get the insurance company will authorize it. If Russell goes to Boston, he would get a treatment aimed at helping his healthy mitochondria replicate. 
 
 “I don’t want to say that we’ve come to terms with it. We love him more than anything in the world,” Budnik-Nap said. 
 
 They may be able to get treatment for their son, but insurance isn’t covering the costs. A YouCaring page has been set up to help cover medical costs. 
 
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 Reddit ||||| Mr Justice Francis says it would take ‘drastic’ evidence for him to change decision not to allow critically ill baby to be flown to US 
 
 Charlie Gard’s parents have been given 48 hours to produce new evidence illustrating how experimental treatment could improve the condition of their critically ill 11-month-old son. 
 
 At an emotional high court hearing on Monday, punctuated by angry interventions by Charlie’s parents, Mr Justice Francis said he would be “delighted” to be able to change his mind from his decision in April not to allow the baby to be flown to the US but it would take “drastic” new evidence for him to do so. 
 
 Sitting at the high court, he said that by 2pm on Wednesday Connie Yates and Chris Gard must produce the evidence they claim to have, including “when it was published, when it became available to them”. There will then be a hearing on Thursday which could decide Charlie’s fate. 
 
 Quick guide Charlie Gard and mitochondrial disease Show Hide What is wrong with Charlie Gard? Charlie has a very rare mitochondrial disease caused by a genetic defect inherited from his parents. The official diagnosis is infantile onset encephalomyopathic mitochondrial DNA depletion syndrome, referred to generally as “MDDS”. What is mitochondrial disease? Mitochondria supply energy to the cells in the human body. Mitochondrial failure leads to cellular injury and cell death. When multiple cells fail, the body’s organs are damaged and shut down. These diseases are usually fatal and kill children, although sometimes they don’t show up until adulthood. Where they don’t kill, they cause serious permanent brain damage. What is the damage to Charlie? Charlie is in intensive care at Great Ormond Street hospital in London, the leading children’s hospital in the UK. Life support machines are keeping him alive. The hospital says that he has severe brain damage, cannot move or breathe by himself, is deaf and has epilepsy. It says his heart, liver and kidneys are also affected. His eyelids cannot stay open and because of the weakness of the muscles, his eyes point in different directions and the damage to the brain will not allow his sight to develop. 
 
 “I have to decide this case not on the basis of tweets, not on the basis of what might be said in the press, or to the press,” said Francis, acknowledging the huge public interest surrounding it. 
 
 As noisy protests in support of Charlie’s parents were held outside the court, lawyers for the two sides – his parents on one side and Great Ormond Street hospital (Gosh) on the other – engaged in heated debate over whether there was any new evidence. 
 
 Grant Armstrong, for Charlie’s parents, told the court a US doctor had suggested that, at a “conservative estimate”, the experimental treatment has a 10% chance of working for Charlie. 
 
 Yates, 31, intervened to ask the judge: “10%. You would if it was your son, wouldn’t you?” 
 
 In response, Francis said that if there was a 10% chance of recovery, including of brain damage, that would be significant and taken into account at Thursday’s hearing. 
 
 When the hospital’s lawyer, Katie Gollop QC, was speaking, Gard, 33, shouted: “When are you going to stop lying?” 
 
 Gosh previously won permission from the courts to turn off Charlie’s life support systems, on the advice of its own experts who said his condition was irreversible and that further treatment could cause him suffering. The decision of the UK courts was upheld by the European court of human rights. 
 
 But the hospital applied for a new hearing after receiving a letter from seven doctors urging the hospital to reconsider the possibility of treatment. Presented with the new evidence, the hospital decided to go back to the high court because it is bound by the earlier ruling, which expressly forbade staff from transferring Charlie for nucleoside therapy anywhere in the world. 
 
 Earlier, the judge rejected a suggestion by a lawyer representing the baby’s parents that he should remove himself from the case. 
 
 At the high court on Monday, Grant Armstrong suggested Francis’s previous ruling against the parents in April raised questions about his capability to hear the latest case fairly. 
 
 US pastor campaigning for Charlie Gard has history of publicity stunts Read more 
 
 The argument was given short shrift by Francis who said he had previously made a legal judgment on the facts and was best placed to review the new evidence. “Far from being evidence of bias, we have the antithesis of bias, which is that I did my job, and I will continue to do my job,” said the judge. 
 
 The hearing, in a packed courtroom in central London, is the latest in a long-running legal battle over the fate of Charlie, who has severe brain damage and cannot see or hear or breathe for himself because of a mitochondrial condition caused by a faulty gene he inherited. 
 
 
 
 Francis said: “I don’t think there’s anyone involved who wouldn’t want to save Charlie ... I am at one with the 350,000 [who signed the petition supporting Charlie’s parents].” 
 
 
 
 Gollop said she had seen nothing that could be called new evidence and that her client had only asked for a new hearing because of the letter from the seven doctors sent from the Vatican children’s hospital and also one from the parents’ solicitor threatening it with judicial review if it did not consider new evidence. 
 
 A statement on behalf of the hospital read in court said the legal proceedings had not only placed “extreme strain and stress on those that are treating him” but has been “desperately unfair to Charlie”. It said staff had been forced to take steps contrary to Charlie’s welfare. 
 
 Among the supporters outside the court on Monday was Reverend Jim Mahoney, a US pastor and pro-life campaigner who said he has flown to the UK to support Gard’s parents at their behest. 
 
 He held a prayer meeting in front of the Royal Courts of Justice, having already claimed that it was prayer that got the legal case reopened. 
 
 He denied that he and other third parties were contributing to a media circus around Charlie’s plight, insisting: “The situation is not just affecting Great Britain but the whole world.” He said he was not doing anything in relation to the case without first clearing it with Charlie’s parents. 
 
 Before Monday’s hearing, Mahoney announced that the US congress will vote on Tuesday on whether Charlie should be made a US citizen. Mahoney, who said he was not a spokesman for Charlie’s parents but had been asked by them to come to the UK, said the vote would, if passed, “in essence make Charlie a citizen of America for travel”. ||||| A 5-month-old local boy is fighting the same, rare life-threatening disease that took the life of baby Charlie Gard in London, despite a court fight over acquiring experimental treatment. 
 
 Local doctors say baby Russel Cruzan III might not live to see his second birthday. But his parents aren't giving up the fight. 
 
 
 
 Baby Russell has mitochondrial DNA depletion syndrome. But his parents told Newschannel 3 that they're hopeful experimental treatments could save their baby. 
 
 Baby Russell is a happy little boy who likes to coo and grunt to his parents. A sweet boy with a lot of strength-it says so on his onesie. 
 
 “As many times as he's been poked as many times as he's been messed with, he just smiles and carries on like nothing ever happened," said his father, Russell Cruzan II. 
 
 His mother, Michelle Budnik-Nap, said, “He does, he'll cry for a couple minutes, then he's back to his little happy self." 
 
 Mitochondrial DNA Depletion Syndrome affects energy in his cells. 
 
 
 
 “Ultimately, it affects the major organs because the major organs require the most energy," Budnik-Nap said. "So right now he's dealing with some kidney problems, some liver problems, heart problems, definitely some muscle weakness, although we're definitely working on getting that fixed." 
 
 His parents say 50 percent of children with the rare disease don't make it to age 2. 
 
 “When we came in to get the diagnosis they just said there's no treatment, there's no cure. We would encourage you to take Russell home and enjoy the time that you have with him,” Budnik-Nab, said. 
 
 They plan on doing that, but his parents also say they're working to get him experimental treatment from a doctor in Boston willing to help. 
 
 “It's time to fight we're not taking no for an answer,” Budnik-Nab, said. 
 
 And in their fight, it's Russell’s strength they say will help them live his life to the fullest. 
 
 “Happy and healthy as possible for as long as possible. And when that's not possible any longer we hope that we have done something to help other children with his condition,” Michelle said. 
 
 Next week, the family will see a neurologist to check how Russell's brain is doing. 
 
 They're planning a golf outing and spaghetti dinner to help fund treatments, travels, and lodging as they work to save their child. They've also started a crowdfunding page to help with some of the expenses. ||||| LONDON - Britain will become the first nation to legalize a "three-parent" IVF technique which doctors say can prevent some inherited incurable diseases but which critics fear will effectively lead to "designer babies". 
 
 After more than three hours of debate, lawmakers in parliament's upper house voted on Tuesday for a change in the law to allow the treatments, echoing a positive vote in the lower house earlier this month. 
 
 The treatment, called mitochondrial transfer, is known as "three-parent" in vitro fertilization (IVF) because the babies, born from genetically modified embryos, would have DNA from a mother, a father and from a female donor. 
 
 Although the techniques are still at the research stage in laboratories in Britain and the United States, experts say that now legal hurdles have been overcome, Britain's first 3-parent baby could be born as early as 2016. 
 
 Mitochondrial transfer involves intervening in the fertilization process to remove faulty mitochondrial DNA, which can cause inherited conditions such as heart problems, liver failure, brain disorders, blindness and muscular dystrophy. 
 
 Mitochondria act as tiny energy-generating batteries inside cells, and around 1 in 6,000 babies around the world are born with serious mitochondrial disorders. 
 
 Responding to the vote, Jeremy Farrar, director of the Wellcome Trust medical charity commended lawmakers for a "considered and compassionate decision". 
 
 "Families who know what it is like to care for a child with a devastating disease are the people best placed to decide whether mitochondrial donation is the right option," he said. 
 
 Mark Downs, chief executive of the Society of Biology, hailed "a great day for UK science" and said the landmark decision "will ensure mothers who carry faulty mitochondria can have healthy children free from the devastating conditions." 
 
 But Marcy Darnovsky, director of the campaign group The Center for Genetics and Society, called the move a "historic mistake" which turns children into biological experiments and will "forever alter the human germline". 
 
 "The techniques ... are relatively crude and will not in and of themselves create so-called designer babies," she said. 
 
 "However, they will result in children with DNA from three different people in every cell of their bodies, which will impact a large range of traits in unknowable ways and introduce genetic changes that will be passed down to future generations." 
 
 (Editing by Janet Lawrence) ||||| A promising technique to prevent mothers from passing on devastating mitochondrial diseases was thrown a biological curve ball this week: A paper published Wednesday in Nature shows that such diseases can come back to sicken a child, even when 99 percent of the mother’s own mitochondria are eliminated. 
 
 Mitochondria are the tiny power plants that provide the energy every cell needs to function. When a large percentage of these organelles malfunction, cells cannot do their jobs—and everything from weakness to death can result. Mothers with certain conditions may have such low levels of faulty mitochondria that they have no symptoms, but their kids can inherit a higher burden of these defective mitochondria, leading to devastating illness. 
 
 In attempt to combat this problem, researchers have essentially been—as one doctor described it—taking the “yellow part” of a mother’s egg and inserting it into the “white” of a donor’s egg. Because the child ends up with DNA from its mother, father and a donor, this mitochondrial replacement therapy has been nicknamed the “three-parent baby” technique. An apparently healthy boy was born using the approach in April in Mexico. He carries about 1 percent of his mother’s mitochondria, which if allowed to pass to him in full might have caused Leigh syndrome, a severe neurological disorder that is generally fatal in early childhood. His parents had two previous children who died of the disease, one at age 6 and one at 8 months, New Scientist reported. 
 
 But the new laboratory study shows that the mother’s mitochondria can sometimes replicate faster than the donor’s and come to dominate again, potentially bringing disease with it. Still, that effect is likely to be rare, says Shoukhrat Mitalipov, the developmental biologist who led the research and has used the technique on mice and monkeys, reportedly without seeing any health problems. Mitalipov and Paula Amato, a reproductive endocrinologist also at Oregon Health & Science University in Portland, said at a news conference that they think mitochondrial replacement therapy should be tested in a clinical trial—despite concerns about the mother’s defective mitochondrial DNA making a comeback. “Because these diseases are so debilitating and often fatal and there are no cures, we think it’s still worth trying to move this research forward to prevent those diseases if we can,” Amato says. Congress currently bans such three-parent procedures for clinical use in the United States, but the United Kingdom has approved a similar technique that would allow even more of the mother’s mitochondria to be passed on to the child and a trial could likely be conducted there, Mitalipov says. 
 
 Although mitochondrial diseases are fairly rare, affecting about one in 5,000 children, Mitalipov and Amato say the three-parent technique could theoretically also be used to treat infertility and help with some stem cell treatments. Mitochondria (which have their own DNA, separate from the DNA in a cell’s nucleus) are inherited solely from the mother. Some consider the three-parent procedure controversial because of this mix of DNA, and because scientists worldwide have agreed to avoid making changes to human genetic inheritance. 
 
 A number of experts have also argued that mitochondrial replacement therapy is too little understood to try in people. Klaus Reinhardt, an evolutionary biologist and zoologist at Germany’s Technische Universität Dresden, thinks the mismatch between the donor’s mitochondrial DNA and mother’s nuclear DNA could cause mitochondrial problems in about one in 50 children born using the three-parent process. Inherited together, the nuclear and mitochondrial DNA can resolve each other’s defects. But when they come from different people, this mismatch can create problems for the child, says Reinhardt, who was not involved in the new research. The human genome, he explains, has 6 billion pairs of DNA and mitochondria have about 16,000, making it very challenging to predict their interactions. “We have no idea who ‘talks’ with whom,” he says. 
 
 Dan Mishmar, a mitochondrial evolutionist and geneticist atBen-Gurion University in Israel, who was not involved in the new study, says his own research shows large differences between the mitochondrial genomes of Africans and Caucasians. When the parents’ DNA match their mitochondrial DNA, these gaps don’t matter, he says. But he worries about taking the mitochondrial DNA “out of context” when the donor and the mother have very different ethnic and racial backgrounds. Like Reinhardt, he worries that the DNA mixture itself could cause illness. “If you reduce the compatibility, you increase the susceptibility to complex disabilities, such as diabetes, Parkinson’s and … Alzheimer’s. That’s the thing we are afraid of.” But Mitalipov believes such problems can largely be avoided by matching the mitochondrial DNA of the mother with that of the donor, in much the same way that organ donors are matched to recipients by immune type. 
 
 Juan Carlos Izpisua Belmonte, a stem cell scientist at the Salk Institute in California who worked on the paper with Mitalipov, suggests an additional way to reduce the risks. He says his lab has developed a technique to cull mutant mitochondria from a mother’s egg before its nucleus is transferred to the donor’s. Reducing levels of the dangerous mitochondria should limit the number that reach the child, he adds. 
 
 For the new study, four women whose children had mitochondrial diseases underwent hormone therapy typically used for in vitro fertilization and provided eggs. Researchers took the DNA from the nucleus of these egg cells and transferred it into donor egg cells whose nuclei had been removed, a process called spindle transfer. The eggs with the mother’s nuclear DNA and the donor’s mitochondrial DNA were fertilized and allowed to divide and grow for five days. Then the embryos were turned into embryonic stem cells, ending their potential for development. These stem cells were transformed into brain, heart and other types of cells to see how their mitochondria would behave. In cells from one mother, the mitochondria reverted to the mutant type that she carried before the spindle transfer. 
 
 Although the new study raises questions about the safety of mitochondrial replacement therapy, Belmonte says it should also quell any fears that the research is moving too quickly. “We have to be convinced in the laboratory that we have done every possible experiment to make sure this is safe—as safe as we can [make it],” he says. “This is a wonderful way to avoid diseases, but we found a problem and we need to make sure we have ways to minimize or solve that problem before we go into the clinic.”
– Mitochondrial diseases afflict an estimated one in 4,000 people, most notably in the recent high-profile case of Charlie Gard in the UK, whose parents unsuccessfully sought to transport him to the US for experimental treatment. The fault lies in mitochondrial DNA, which is inherited from mothers and is responsible for powering every cell in the body, per Quartz. Now a 5-month-old baby in Michigan has been diagnosed with mitochondrial DNA depletion syndrome 13, which claimed the life of Gard last week. His parents will attempt experimental treatment in Boston, they tell NewsChannel3. With only a 50% chance of reaching his second birthday, they say at least their baby might "help other children with this condition." Russell Cruzan III, or "Bubs," first worried his parents in early June, when they wondered why he wasn't eating or thriving and was lethargic. After several hospital visits, respiratory problems, and eventually pneumonia, they got the grim diagnosis: His body lacked the energy to power his major organs. The couple doesn't have help from their insurance provider, per WOOD TV, so they've set up a YouCaring page to help with the initial test. This is the type of disease that geneticists hope to be able to stop in its tracks using so-called three-parent IVF, where a donor egg supplies a healthy set of mitochondrial DNA (37 genes) and the biological mother supplies her egg's nucleus (thousands of genes) without passing on mitochondrial diseases. (A baby was born in Mexico using this technique.)