Allograft Safety - Sydney Morgan Steinberg

Sydney Morgan Steinberg

BORN: February 20, 1995

PASSED AWAY: December 27, 2000

My name is Amy Steinberg and my husband’s name is Thom. This is the tragic story of our daughter Sydney’s battle with fungal endocarditis after she received a human heart valve allograft and how this terrible disease changed our family’s lives.

The Diagnosis

Our family moved to Albuquerque, NM in 1998 and soon sought out a pediatric cardiologist for our middle child, Sydney, who was born with Tetrology of Fallot. Sydney was only 4 years old when her father and I were told, by her new group of cardiologists that she needed to be implanted with a pulmonary valve allograft. We were confused because Sydney had just had a total repair for her Tetrology of Fallot when she was only two years old and were told by her former cardiologist in Florida that she should be fine until her pre-teen years when she would need her pulmonary valve replaced. Sydney’s father and I never thought to question why she should have another major surgery just two short years after her first surgery. We only knew that a pediatric cardiac surgeon was telling us that she needed the valve and we only wanted what was best for Sydney’s health. We never thought to question where the valve would come from, as we already knew that an allograft valve was from a cadaver. We assumed our child’s valve would be sterile because we thought only sterile conditions were used in surgeries. We never asked Sydney’s doctors about the valve in particular just general questions that a parent would ask a surgeon like surgery times, recovery times, we had questions about the heart-lung bypass procedure and the general risks involved with Sydney’s surgery.

The First Surgery

On December 6, 1999, Sydney had open heart surgery to replace her pulmonary valve. The valve showed very early signs of problems. During the valve replacement surgery, the surgeon had a difficult time controlling the bleeding at the suture site and the very first post-operative echocardiogram showed quite a bit of regurgitation of this new pulmonary valve. The regurgitation never got any better and as the months passed it actually got worse. At Sydney’s second post-operative check up in February 2000 her cardiologist discovered a mass inside the new pulmonary valve and immediately put Sydney into the hospital and on anticoagulant therapy because the mass was thought to be a blood clot. After a week in the hospital and countless blood draws Sydney was put on a two time a day shot for her anticoagulant therapy and I was to take her home and give her these shots every morning when she woke up and every night before she went to bed. After over three weeks of blood thinners, the mass never changed in size. During this time Sydney’s father got a great new job and we moved to Portland, OR with a referral to a pediatric cardiologist in our new city.

Signs of Fungal Endocarditis - The Nightmare Begins

After we moved to Oregon, I set up an appointment with Sydney’s new cardiologist and we waited for the day at the end of May that she would be seen, all the while thinking Sydney was doing fine. Just a couple of days before Sydney were to be seen by her new doctor, she started to get sick. Her younger brother had just gotten over the flu and we thought she must have picked up what he had been sick with. Sydney was extremely sick and the night before her appointment, she collapsed at home while vomiting in the bathroom. I called 911 and my new neighbors called my husband at work for me, all the while my other two children, Shelby, age 9 and Brady, age 9 months watched their sister and mom being taken away by paramedics in an ambulance.

Emergency Surgery

After a very scary couple of days, they were able to stabilize Sydney and we were told she had vegetation growing in her pulmonary valve and would need emergency open heart surgery to remove the growth and replace her pulmonary valve. She had this surgery on June 4, 2000. The surgeon was shocked to see the condition of Sydney’s heart. He said it was a mangled wreck and full of a creamy fungus, which would later be diagnosed as Arthrographis kalrae, a fungus found in soil and rotting vegetation and at the time had only ever been seen in one documented case of a skin infection. We were in complete shock and thanked God that she survived, yet we began to wonder how in the world fungus could be in our child’s heart and suspected the pulmonary valve allograft that was implanted in New Mexico. Sydney had a central line catheter inserted into her chest and was started on daily IV anti-fungal mediations (amphotericin and fluconazole) that I was taught to administer to her at home. She actually recovered quite well and we thought that once her round of IV drugs was done she would have her central line removed and she would only be left taking oral Diflucan for life, as the doctors couldn’t assure us that the fungus wouldn’t try to come back since fungus is so hard to kill. Our family was in shock and angry that this happened to Sydney, but we thought that everything was going to be alright and we looked forward to our upcoming vacation to Disneyland and to Sydney starting Kindergarten.

Third surgery - Trying to Cope

Our family had so much fun in Disneyland and we all thought that this was “Sydney’s trip” since she had just been through so much with her illness and surgery. On the way home from Disneyland Sydney started complaining that she felt sick and I thought she was just car sick. After way too many trips to her pediatrician to find out what was wrong, my husband took Sydney to the emergency room at the hospital where her surgery had been done and her cardiologist worked. She was given an echocardiogram that showed the fungus had returned and we were devastated. On September 13, 2000, we were told that she would need another surgery to remove the fungus and replace the pulmonary valve. Sydney was once again given a central line catheter and this time she was put on another anti-fungal called Itraconazole as well as the amphotericin. We didn’t know what would be in store for Sydney’s future but we knew that her infectious disease doctor told us that if this surgery and medication didn’t clean up the fungus Sydney would be left with a very grim outlook. This surgery was much harder on Sydney and having to go back on the amphotericin was devastating to her little body. She never really got any better and looking back we can see how we lost a little more of our daughter as each day passed.

Devastating Prognosis

On November 2, 2000 Sydney was home getting ready for me to start her IV medications when she collapsed. She was not breathing and she was purple as I dialed 911. I thought I was going to have to give my little girl CPR when all of a sudden she took in a deep breath and lay there on the floor looking at me in a dazed state. The paramedics took us back to the hospital and the doctors couldn’t quite figure out what happened to Sydney, but after my own research I truly feel that she had a pulmonary embolism and I knew in my heart that the fungus was not only in my baby’s heart but now also in her lungs.

On November 7, 2000 we were told that Sydney was not ever going to get better and that she was now deemed a terminal case. I know a huge part of me died that night, but I was determined to give Sydney the best last days I could possibly give her. At this point Sydney had been accepted into an investigational drug study with a new antifungal IV drug called Voriconazole and I had the doctors stop the administration of the Amphotericin, as that drug was taking so much out of her and she vomited daily while she was on it. The new drug wasn’t as harsh on Sydney and we prayed that it would be her miracle.

Our family tried to live our lives like everything were alright, but deep inside Sydney’s daddy and I knew we were losing our precious baby. She looked worse everyday and she tried so hard to be strong and live her life like “normal.” She was on around the clock oxygen and at age five was wearing diapers because she was too weak to ever make it to the potty. I would have taken care of Sydney forever in that state, but I know that God couldn’t allow this beautiful child to live life like this and I know that she was ready to go. On December 27, 2000 Sydney began to cough up blood and she died in her daddy’s arms of a pulmonary hemorrhage from reoccurring fungal endocarditis .

For the first time in a long time I know my baby didn’t hurt and for Sydney I was so happy, but for me my gut wrenching pain was just beginning. I hurt for a long time before Sydney died, but I was so busy trying to get her better that I didn’t allow myself much time to feel the pain. Now my baby was gone and all I had was time.

Important Message to Parents

If I had only one message I could give to parents, it would be to never, ever just hand your most precious gift over to the experts without researching and asking many, many questions. Sydney’s father and I always asked questions, but we always thought that the doctor is the educated one and that must mean they know what is best. Unfortunately this is not always the case. As we found out many cardiologists were totally in the dark about the safety and durability of allograft heart valves. If you feel something isn’t quite right or you are uneasy about the research you discover, do whatever you can even if that means screaming at the top of your lungs to anyone that will listen and make sure someone knows your feelings, fears and questions. It took Sydney’s tragic experience to teach me that, but I am a new mom because of what my little angel Sydney taught me.

Several years have passed since we lost Sydney to fungal endocarditis and after a lot of research and fighting we now know the terrible truth about human tissue for transplantation. Had we had the opportunity to know the details of how human tissue is processed, we never would have allowed Sydney to have that allograft valve on that day. Sydney was not in a life or death situation on December 6, 1999. Sydney was a beautiful, strong, happy, little girl with a heart defect and she deserved so much better than she was given. Thom and I should have been given all the pertinent information on tissue processing and the companies that process tissue. We should have been given other options to consider for Sydney. The Food and Drug Administration should have been keeping a closer eye on the tissue industry. There had been other red flag warnings about patient infections after receiving allograft heart valves and unfortunately for our family Sydney became yet another red flag warning that still to this day goes ignored.

Amy & Thom Steinberg


		Sydney Morgan Steinberg BORN: February 20, 1995 PASSED AWAY: December 27, 2000 My name is Amy Steinberg and my husband’s name is Thom. This is the tragic story of our daughter Sydney’s battle with fungal endocarditis after she received a human heart valve allograft and how this terrible disease changed our family’s lives. The Diagnosis Our family moved to Albuquerque, NM in 1998 and soon sought out a pediatric cardiologist for our middle child, Sydney, who was born with Tetrology of Fallot. Sydney was only 4 years old when her father and I were told, by her new group of cardiologists that she needed to be implanted with a pulmonary valve allograft. We were confused because Sydney had just had a total repair for her Tetrology of Fallot when she was only two years old and were told by her former cardiologist in Florida that she should be fine until her pre-teen years when she would need her pulmonary valve replaced. Sydney’s father and I never thought to question why she should have another major surgery just two short years after her first surgery. We only knew that a pediatric cardiac surgeon was telling us that she needed the valve and we only wanted what was best for Sydney’s health. We never thought to question where the valve would come from, as we already knew that an allograft valve was from a cadaver. We assumed our child’s valve would be sterile because we thought only sterile conditions were used in surgeries. We never asked Sydney’s doctors about the valve in particular just general questions that a parent would ask a surgeon like surgery times, recovery times, we had questions about the heart-lung bypass procedure and the general risks involved with Sydney’s surgery. The First Surgery On December 6, 1999, Sydney had open heart surgery to replace her pulmonary valve. The valve showed very early signs of problems. During the valve replacement surgery, the surgeon had a difficult time controlling the bleeding at the suture site and the very first post-operative echocardiogram showed quite a bit of regurgitation of this new pulmonary valve. The regurgitation never got any better and as the months passed it actually got worse. At Sydney’s second post-operative check up in February 2000 her cardiologist discovered a mass inside the new pulmonary valve and immediately put Sydney into the hospital and on anticoagulant therapy because the mass was thought to be a blood clot. After a week in the hospital and countless blood draws Sydney was put on a two time a day shot for her anticoagulant therapy and I was to take her home and give her these shots every morning when she woke up and every night before she went to bed. After over three weeks of blood thinners, the mass never changed in size. During this time Sydney’s father got a great new job and we moved to Portland, OR with a referral to a pediatric cardiologist in our new city. Signs of Fungal Endocarditis - The Nightmare Begins After we moved to Oregon, I set up an appointment with Sydney’s new cardiologist and we waited for the day at the end of May that she would be seen, all the while thinking Sydney was doing fine. Just a couple of days before Sydney were to be seen by her new doctor, she started to get sick. Her younger brother had just gotten over the flu and we thought she must have picked up what he had been sick with. Sydney was extremely sick and the night before her appointment, she collapsed at home while vomiting in the bathroom. I called 911 and my new neighbors called my husband at work for me, all the while my other two children, Shelby, age 9 and Brady, age 9 months watched their sister and mom being taken away by paramedics in an ambulance. Emergency Surgery After a very scary couple of days, they were able to stabilize Sydney and we were told she had vegetation growing in her pulmonary valve and would need emergency open heart surgery to remove the growth and replace her pulmonary valve. She had this surgery on June 4, 2000. The surgeon was shocked to see the condition of Sydney’s heart. He said it was a mangled wreck and full of a creamy fungus, which would later be diagnosed as Arthrographis kalrae, a fungus found in soil and rotting vegetation and at the time had only ever been seen in one documented case of a skin infection. We were in complete shock and thanked God that she survived, yet we began to wonder how in the world fungus could be in our child’s heart and suspected the pulmonary valve allograft that was implanted in New Mexico. Sydney had a central line catheter inserted into her chest and was started on daily IV anti-fungal mediations (amphotericin and fluconazole) that I was taught to administer to her at home. She actually recovered quite well and we thought that once her round of IV drugs was done she would have her central line removed and she would only be left taking oral Diflucan for life, as the doctors couldn’t assure us that the fungus wouldn’t try to come back since fungus is so hard to kill. Our family was in shock and angry that this happened to Sydney, but we thought that everything was going to be alright and we looked forward to our upcoming vacation to Disneyland and to Sydney starting Kindergarten. Third surgery - Trying to Cope Our family had so much fun in Disneyland and we all thought that this was “Sydney’s trip” since she had just been through so much with her illness and surgery. On the way home from Disneyland Sydney started complaining that she felt sick and I thought she was just car sick. After way too many trips to her pediatrician to find out what was wrong, my husband took Sydney to the emergency room at the hospital where her surgery had been done and her cardiologist worked. She was given an echocardiogram that showed the fungus had returned and we were devastated. On September 13, 2000, we were told that she would need another surgery to remove the fungus and replace the pulmonary valve. Sydney was once again given a central line catheter and this time she was put on another anti-fungal called Itraconazole as well as the amphotericin. We didn’t know what would be in store for Sydney’s future but we knew that her infectious disease doctor told us that if this surgery and medication didn’t clean up the fungus Sydney would be left with a very grim outlook. This surgery was much harder on Sydney and having to go back on the amphotericin was devastating to her little body. She never really got any better and looking back we can see how we lost a little more of our daughter as each day passed. Devastating Prognosis On November 2, 2000 Sydney was home getting ready for me to start her IV medications when she collapsed. She was not breathing and she was purple as I dialed 911. I thought I was going to have to give my little girl CPR when all of a sudden she took in a deep breath and lay there on the floor looking at me in a dazed state. The paramedics took us back to the hospital and the doctors couldn’t quite figure out what happened to Sydney, but after my own research I truly feel that she had a pulmonary embolism and I knew in my heart that the fungus was not only in my baby’s heart but now also in her lungs. On November 7, 2000 we were told that Sydney was not ever going to get better and that she was now deemed a terminal case. I know a huge part of me died that night, but I was determined to give Sydney the best last days I could possibly give her. At this point Sydney had been accepted into an investigational drug study with a new antifungal IV drug called Voriconazole and I had the doctors stop the administration of the Amphotericin, as that drug was taking so much out of her and she vomited daily while she was on it. The new drug wasn’t as harsh on Sydney and we prayed that it would be her miracle. Our family tried to live our lives like everything were alright, but deep inside Sydney’s daddy and I knew we were losing our precious baby. She looked worse everyday and she tried so hard to be strong and live her life like “normal.” She was on around the clock oxygen and at age five was wearing diapers because she was too weak to ever make it to the potty. I would have taken care of Sydney forever in that state, but I know that God couldn’t allow this beautiful child to live life like this and I know that she was ready to go. On December 27, 2000 Sydney began to cough up blood and she died in her daddy’s arms of a pulmonary hemorrhage from reoccurring fungal endocarditis . For the first time in a long time I know my baby didn’t hurt and for Sydney I was so happy, but for me my gut wrenching pain was just beginning. I hurt for a long time before Sydney died, but I was so busy trying to get her better that I didn’t allow myself much time to feel the pain. Now my baby was gone and all I had was time. Important Message to Parents If I had only one message I could give to parents, it would be to never, ever just hand your most precious gift over to the experts without researching and asking many, many questions. Sydney’s father and I always asked questions, but we always thought that the doctor is the educated one and that must mean they know what is best. Unfortunately this is not always the case. As we found out many cardiologists were totally in the dark about the safety and durability of allograft heart valves. If you feel something isn’t quite right or you are uneasy about the research you discover, do whatever you can even if that means screaming at the top of your lungs to anyone that will listen and make sure someone knows your feelings, fears and questions. It took Sydney’s tragic experience to teach me that, but I am a new mom because of what my little angel Sydney taught me. Several years have passed since we lost Sydney to fungal endocarditis and after a lot of research and fighting we now know the terrible truth about human tissue for transplantation. Had we had the opportunity to know the details of how human tissue is processed, we never would have allowed Sydney to have that allograft valve on that day. Sydney was not in a life or death situation on December 6, 1999. Sydney was a beautiful, strong, happy, little girl with a heart defect and she deserved so much better than she was given. Thom and I should have been given all the pertinent information on tissue processing and the companies that process tissue. We should have been given other options to consider for Sydney. The Food and Drug Administration should have been keeping a closer eye on the tissue industry. There had been other red flag warnings about patient infections after receiving allograft heart valves and unfortunately for our family Sydney became yet another red flag warning that still to this day goes ignored. Amy & Thom Steinberg