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Jackson Health System
Angela's father Petrit Bushi, Angela and her mother Valbona, smile after the 6-year-old girl received a new liver, two kidneys and pancreas.
Valbona Bushi, 28, knew even before the doctors did: Her daughter Angela’s liver was failing, just as Angela’s younger sister’s had.
At first, emergency room doctors dismissed the kindergartener’s flu-like symptoms as nothing serious. But the next day the 6-year-old's abdomen was slightly swollen. Bushi, whose 18-month-old daughter Alicia had died of liver failure just a few months earlier, rushed Angela back to the hospital near their home in Jacksonville, Fla. After tests revealed the little girl needed a liver transplant and she was referred to Holtz Children’s Hospital, affiliated with the University of Miami Miller School of Medicine.
A new liver would save Angela from imminent death, but doctors wanted to do more -- to try to actually extend her life. Angela, whose parents had moved to Jacksonville from Albania seven years ago, had been newly diagnosed with a rare, fatal genetic disorder called Wolcott-Rallison Syndrome. Only about 60 cases of the disorder -- which causes infant-onset diabetes and liver failure, as well as bone fractures, intellectual impairment and frequent infections -- have been reported. Only one of those children lived into young adulthood. It had killed Angela’s younger sister and damaged Angela's organs.
On December 29, in an unprecedented multiple-organ transplant on a child, Angela received a liver, two kidneys and a pancreas, doctors said.
The operation -- the first time a transplant has been used to treat the disorder -- “might offer some hope for these children with this rare syndrome, that life can be prolonged, hopefully in a very meaningful way,” Dr. Andreas Tzakis, transplant surgeon, told msnbc.com Thursday. Tzakis is the chief of the liver and digestive tract program at Jackson Memorial Hospital.
A viral infection, the kind most kids easily bounce back from, caused Angela's liver failure, said Dr. Olaf Bodamer, director of the pediatric genetics program at Holtz Children’s Hospital. In children with Wolcott-Rallison, a protein doesn’t function normally. When patients contract a virus, their cells are unable to clear the resulting waste products, Bodamer explained. “Theoretically, any organ could be affected,” he says, but the liver, pancreas and kidneys are most vulnerable.
Angelad was diagnosed with Type 1 diabetes as as a baby, but, since her transplant, is no longer diabetic.
Her post-operative course “was tougher than expected,” says Tzakis. But she recovered and was discharged from the hospital on Feb. 23.
Now, Angela can’t wait to return to kindergarten which she had attended for only two weeks before becoming ill, her mother said.
“Every day she says, ‘I miss my school,’” Bushi says. Angela’s return to the germy world of elementary school will have to wait until she’s more stable, but Tzakis has “guarded optimism” that the transplant operation will halt or slow the normal course of her disorder.
“They saved my daughter’s life,” Angela’s mother Valbona told msnbc.com.
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God Bless Her and Keep Her in Your Care..
As long as her folks are paying for it and not society in the form of insurance it's a great idea. Our nation can no longer afford this sort of health care financial nightmare. Medicine is to prevent premature death and suffering, to promote quality of life, not promote immortality.
OK haters, pour it on.
norm903, Are you kidding me!?!?! I know you did not just say you hope they are paying fully for this! Medicine is to save lives? REALLY? This little girl is six years old! She may save this world, you have no idea what she may be capable of or who she may become, but if insurance isn't for the children, then who is it for then, and she is an American citizen, add please.... if her parents came here seven years ago, and she is six then she was born here, that makes her an entitled citizen to the same benefits you are entitled to. People with that attitude should just not speak at times before thinking.
It also may be possible that docotrs donated their efforts, and the organs were donated!
Wonderful story! What a bright future this little girl has.
No. The article failed to mention the immunosuppressive medications she will have to take for the remainder of her life. A common side-effectof many immunosuppressive drugs is decreased ability to fight infections, resulting in increased susceptibility to both common infections and very uncommon ones, such as various parasites and fungal infections. The drugs increase the incidents of certain forms of cancer. Other side-effects include hypertension, dyslipidemia, hyperglycemia, peptic ulcers, lipodystrophy, moon face, liver and kidney injury. Immunosuppressive drugs also interact with other medicines and affect their metabolism and action. One has to wonder whether this surgery will improve her life or only added to her misery.
well she would have been dead otherwise so at least now she HAS a future. the immunosuppressive medications are used to keep her new organs from failing again which could lead to her death. so YES she does have a bright future because without the transplant and the immunosuppressive drugs she wouldn't even have the option.
I agree with scales on this one. They never said how long her life expectancy was. According to the article, only one case has ever lived into young adulthood, so I can't expect that her life expectancy will be much longer than that, even with the transplants.
The immunosuppressive drugs don't stop the new organs from failing, they SUPPRESS the IMMUNE system, so the body doesn't kill the foreign organs. I'm speculating here, but since the disease she was affects a protein in the body, and can affect ANY organ, the transplants are just delaying the inevitable. As sad as that sounds.
Honestly, this "article" is lacking a ton of information past the sob story to get people to feel for this family. The thing is, this family had this happen once before. I don't know what the genetic percentage for this happening for them, but you would think they wouldn't want to risk it. Adopting would have made a lot of sense for this family.
Hey that's an excellent thought. So do you think everyone who's sick should just be left out in the pasture to rot or might you prefer to put a bullet in her head to put her out of her misery yourself?
Also, it's a god damned news article where they purposely have to dumb most of the science and genetics down so the grand majority of the fat fools in our country can read it without any extra information that might confuse them.
Medical technology is advancing at an amazing pace and maybe, just maybe, the extra five years or however long might be enough for a new treatment option or even a cure.
Transplantation medicine has come a long way. Anti-rejection drugs have been honed to the point where once the dose is established, the immune system functions at ~95%. The protein she lacks wouldn't affect the transplanted organs, because they have their own cells, their own DNA, and will produce the protein on their own. My question is - why did she need two kidneys?
P.S. It's inevitable for everyone; including you.
Wow! I hope she has an AMAZING life! You know, this is an inspiring story about a battle for a CHILD'S LIFE! God bless you, little one. You need it.
There are far more of these rare, genetic, metabolic diseases than people realize. They are a heart breaking cause of horrible suffering and sadly many children die for lack of any real treatment options. This family is the luckiest of the unlucky. I wish them the best. Thank goodness many brilliant people still go into medicine to help find answers and relieve suffering.
Coping With Illness. Sickness is a calamity that may befall a person even if he is materially rich. (Ec 5:16, 17; compare Mt 16:26.) Some individuals have delicious food in abundance but are unable to enjoy it because of some stomach or bowel disorder. (Ec 6:1, 2) Jesus Christ's spiritual brothers were also spoken of as being physically sick at times. (Mt 25:39, 40) Physical sickness was experienced by such Christians as Epaphroditus, Timothy, and Trophimus (Php 2:25-30; 1Ti 5:23; 2Ti 4:20), but the Bible reports no miraculous apostolic healing of these Christian men.
Nevertheless, when one of God's servants is physically ill, it is proper for him to pray to Jehovah for the fortitude needed to bear his malady and for spiritual strength to maintain integrity during this period of weakness in the flesh. "Jehovah himself will sustain [such a person] upon a divan of illness."—Ps 41:1-3; see also 1Ki 8:37-40.
Jehovah can remove sicknesses. (Ex 15:26; 23:25; De 7:15) Isaiah wrote of a time when "no resident will say: 'I am sick'" (Isa 33:24) and about spiritual healing of the blind, deaf, lame, and speechless, these prophecies also giving promise of physical cure. (Isa 35:5, 6) Jesus Christ, when on earth, accomplished both physical and spiritual healing of ailing ones by fulfilling the Messianic prophecy, "He himself took our sicknesses and carried our diseases." (Mt 8:14-17; Isa 53:4) The basis for these cures was the sacrifice of his human life, which would be the climax of the course he had been following ever since God's spirit came upon him at the Jordan River in 29 C.E. Christians thus have a basis for hope and abundant proof that through the resurrected Jesus Christ and by means of God's Kingdom, obedient mankind will receive, not merely temporary treatment of disease, but permanent release from sin, disease, and death traceable to Adam. For this all praise is due Jehovah, identified by David as the one "who is healing all your maladies."—Ps 103:1-3; Re 21:1-5.
If you know of anyone who can help the childen from these horable sufferings , make it known , if you care . I am trying .
I wish this girl the best and hope this is a cure for her. But like Scales67, I was wondering if this operation only prolongs her misery. Her parents seem like nice people and I'm sure they didn't know they carried this genetic disease. But after losing one daughter and almost losing another, I hope they do not have anymore children. Don't put another child through this.
"Woe to the Pregnant Women." When responding to the apostles' question about the conclusion of the system of things, Jesus spoke about fleeing from Judea and said: "Woe to the pregnant women and those suckling a baby in those days!" (Mt 24:19; Mr 13:17; Lu 21:23) The fulfillment and truthfulness of those words became apparent in the events prior to and during the destruction of Jerusalem in 70 C.E. Though reasonable activity and movement is usually possible for a woman during her pregnancy (Lu 1:39, 56; 2:5), extended flight on foot over mountainous country would be hard on her, and especially if her time for delivery was close. Extreme adversity befell pregnant women and those suckling babies when Roman forces laid siege to Jerusalem. Famine prevailed. During pregnancy it is important for a woman to have proper nourishment. If, for example, she does not get sufficient calcium, she might lose her teeth, as the body takes calcium to form the bones of the developing baby. Furthermore a woman's maternal protective instinct would increase her suffering as she saw infants starving and dying, all the while knowing that she would soon bring a child into such conditions. Josephus wrote about some starving men in besieged Jerusalem: "There was no compassion for hoary hairs or infancy: children were actually lifted up with the fragments to which they clung and dashed to the ground."—The Jewish War, V, 433 (x, 3); compare Lu 23:29.
The toture of children today , has increased to a propotion that can hardly be monitored . Your conclusion is recognized and prophycied . Do you think that a normal person would want to see anymore of this ? Thank you for your comment .
To scales and rado, there are many of us who received organs and have lived a very normal life even with the immunosuppressants. Yes, there are risks of cancer, infections, etc., but I doubt that very many transplant patients regret being given the extra chance at life. I had my transplant 12 years ago, and I am living a very full and enjoyable life. I do have arthrits from prednisone, but the pain from it will never compare with the chance to watch my two granddaughters being born or my sons graduate and become firemen and paramedics. Unless you have been there, don't be so ready to criticize others for wanting to live or helping their family survive. My life is great. You can't always believe what you read and all transplant survivors are different. Some may not have a good results, but there are many who live great lives. They work, play and do what many "normal" folks do. All medicines have warnings, but not all the results happen to everyone who takes that medicine. My meds are a very small part of my daily life, just like taking a shower or brushing my teeth. I can basically do anything I want. Everyone who wants it, deserves a chance at life.
I am so pleased to hear of the miracles Dr. Tazikas continues to be a part of..In 1987 I was working in a NICU in Richmond, VA and I first met Kamron Greene. He was 2 months old and had lost 90% of his intestines during a battle with Necrotizing Enterocoloitis. I was his nurse in his home as he received intravenous nutrition. At 10 years of age his liver was failing. After learning about Dr. Tazikas his Mother called Miami, got insurance approval and he was on a jet to Miami within 2 days. One month later he received his 5 organ multiple transplant under the care of Dr. Tazakis's team. It was a rough recovery but today Kamron is 24 years old and very healthy, on minimal immunosupressants, and none of the side effect mentioned by Scales67. Kamron is deaf from the high doses of antibiotics he received as an infant but still has a very good quality of life. You can connect with Kamron on facebook. I wish the Miami team all the best and to this little girl and her family I will keep them in my thoughts and prayers. There is always hope, Janelle-716476, keep the faith.
Thanks for the wonderful story. I wish continued health to Kamron!
God bless these doctors, she is lucky Albanian girl, wish her good health and long life with her parents !
Byl---Couldn't say it better for Angela, the doctors and parents.
Did anyone notice the words, "the doctors wanted to do more" and try to extend her life. Do I hear the word Experiment? While I don't agree 100% with Norm, as a society we can no longer afford to pay for experimental treatments. That takes dollars away from proven treatments that will save lives. I wouldn't even want to see the bills for this child, and this is child #2. I am not heartless, as a matter of fact, I am Board Executive of a charity that grants wishes for terminally ill and permanently disabled children. I feel for the children and the parents, but where do we stop. We are saving babies born too early at under 1 lb. to a life of disabilities and medical issues while people are dying of illnesses that have a cure but because they are not high profile or they don't have the ability to pay they are not getting. We are unfortunately going to have to make some hard choices in the future!
Health care is not a natural born right in this country, it's a privilege that costs money just like everything else. Focus on what is real, not ideal.
Rado- I just wanted to point out that the little girl that just had the operation is 6 years old; her little sister passed away a few months ago at 18 months old. So the parents in this article didn't choose to have another child after losing one to this disease; they lost their youngest daughter to it, and would have lost their oldest if the mom hadn't recognized the symptoms. I believe you missed that bit of information in the opening paragraph; so adoption wasn't an option as they already had both children before one passed on.
As for those saying (or implying) that this level of medical care is somehow wasteful because they can't promise this young girl a long life~ no one is promised tomorrow. You can walk out of your house and get hit by a bus; should you not receive medical care for any illness you have just because we can't promise that bus won't hit you tomorrow? We hope for a long life, therefore everyone is treated like that long life will happen. They replaced the organs most likely to be damaged by this disease, so this little girl could possibly outlive you (watch out for traffic ;) wouldn't want to get hurt!) and maybe in time they will find a cure. Or maybe they'll find this radical procedure IS the cure, who knows? What I do know is that many of the kids that have a hard start in life grow up with strong characters and want to give back all they have been given and more. And as a woman who has lost a child, I understand the parents agreeing to any possible way to keep their only surviving child alive. I don't know what hurts more- losing a child, or watching the other struggle. My heart goes out to this family, and I hope this is a story with a happy ending.
... what's done has already been done, but in my laymen opinion these organs could have been used more appropriately. We're already so limited in transplant-able organs, and while I hope she has great life, I can't help but think of the three other people who could have lived with significantly higher certainty. This wasn't an adult, or even a teenager, this was a very young child. From what it seems, a very low chance of getting through a half-decade. Speaking of costs..., using transplant-able organs has a cost as well, and that cost is far more valuable than US dollars. It could have been a teenager with genetic liver disorder, that would have lived many decades. It could have been two people with end-stage renal failure, living off dialysis machines. How much would you pay for more years with your husband or wife? We don't have the technology to save people like this girl, I'm sorry but we just don't at this moment in history! We do have the ability to save other people, and in this tragic case.. they were passed over.