Thank you for the incredible opportunity given to Sofie.
Sofie, a lung transplant since 2020 at the age of three
Sofie was born on June 27, 2017 in Mannheim and had already had a long medical history. In the 22nd week of pregnancy, we received the diagnosis that Sofie had pulmonary cysts in her lungs. In the 29th week of pregnancy, we were advised to terminate the pregnancy late.
We were very lucky to find a fetal surgeon who operated on Sofie in the womb. After she was born by caesarean section in the 34th week of pregnancy, she underwent another lung operation on the first day of her life. Now we knew that she had made it and could lead a normal life.
Sofie developed splendidly. However, after a severe bout of pneumonia in April 2019, she was diagnosed with pulmonary hypertension, which is incurable.
"She had less and less strength to move, walk or eat. Her condition deteriorated rapidly."
We had Sophie's heart checked at the Großhadern Hospital and the physicians at the Hauner Children's Hospital looked after her lungs. However, the physicians couldn't explain what had caused the pulmonary hypertension. The heart was severely damaged because it could no longer work against the pressure in the lungs.
If patients are well controlled with medication, they can actually live with the disease for a few years. Unfortunately, this was not the case for Sofie. Within a few months, she needed oxygen 24 hours a day. She had less and less strength to move, walk or eat. Her condition deteriorated rapidly.
In April 2020, the first cardiac catheterization was performed in Großhadern. The diagnosis was devastating. Sofie didn't have much time left to live. I called the Hauner Children's Hospital, which immediately recommended that we contact the lung transplant team in Hanover.
"It became clear that only a transplant would prolong and improve Sophie's life and quality of life."
After a conversation with Dr. Julia Carlens, we had hope again. She gave us an inpatient appointment. Unfortunately, Sofie deteriorated rapidly and we had to go to the Hauner Children's Hospital as inpatients. The Hauner Children's Hospital was in contact with Hanover the whole time.
On 2 June 2020, we went to the Transplant Centre in Hanover, 610 kilometers away; we live just outside Munich. We received a warm welcome in Hanover. The physicians immediately took care of Sofie and examined her. Dr. Carlens explained to us that they were trying to help Sofie. At the same time, however, they would also carry out the examinations for a listing for transplantation as well as the educational and informational talks.
On June 4, Sofie was feeling worse. Dr. Dagmar Hohmann performed a heart ultrasound. After that, it was time to stop: any excitement could kill Sofie. It became clear that only a transplant would prolong and improve Sofie's life and quality of life.
The surgeons did not know whether Sofie could be listed. During the lung operation on the first day of life after birth, the lower left lobe of her lung was removed and her chest was deformed. The surgeons needed a recent CT scan. However, stress and excitement would have been life-threatening for Sofie.
"Unfortunately, time was running out. We didn't know whether Sofie would make it to the transplant."
Associate professor Dr. Nicolaus Schwerk made sure that a very quick CT scan was available. The pediatric pneumologist had a good rapport with Sofie. He calmed Sofie down and even sang to her. This meant that the CT scan could be carried out quickly, easily and without causing her too much stress.
PD Dr. Schwerk then worked with the surgeon Dr. Fabio Ius to get Sofie listed. After two weeks of fear and hope, she was listed.
PD Dr. Schwerk, Dr. Carsten Müller and Dr. Carlens explained the transplant to us in detail. They were able to allay our fears and give us hope of prolonging Sofie's life and improving her quality of life.
Sofie's condition got even worse. Prof. Georg Hansmann, who is a pulmonary hypertension specialist, was brought on board. He prescribed other medication for the heart and a special infusion therapy to ensure that the heart would hold out until the transplant. Unfortunately, time was running out. We didn't know whether Sofie would make it to the transplant.
The physicians, nurses and carers on ward 63 fought for Sofie. They were there for us and gave us hope. It was a very difficult time! Without the support of Nici, who became a good friend to me, and other parents of transplanted children and the pulmonary hypertension groups, we would not have made it through this time.
Dr. Hohmann checked Sofie's heart every week and gave her an infusion every two weeks in the intensive care unit. Sofie could only lie in bed or sit with help and could only drink, but not eat - she had no strength left.
"At that moment, I felt hot and cold. I still cry today when I think about that moment."
At the beginning of September, we had a wonderful day. For once, Sofie felt better that day and we had a lot of fun. When she fell asleep at 8pm in the evening, I thought today was the day. At around 9 p.m., Sofie's favorite nurse came into the room with a beaming face. I was told to answer the ward phone and PD Dr. Schwerk would have to talk to me.
PD Dr. Schwerk then told me on the phone that he had received an organ offer. The surgeons would check whether it could be transplanted.
At that moment, I felt hot and cold. I still cry today when I think about that moment. I called my husband and my parents. Sofie was taken to the intensive care unit to have her blood taken. Nobody wanted to take any risks in the last few meters. She was then allowed to continue sleeping - in her room on the normal ward.
I waited all night for the news as to whether they would be able to take the organ. It was unspeakable hours. I caused the Nursing staff and the nurse a few nerves. Nevertheless, they were totally friendly and reassured me. At 5 o'clock in the morning, the news came: Sofie would be transplanted at 7 o'clock. At that moment I was overjoyed and infinitely grateful.
Infinitely grateful to a family who, at their worst time, had agreed to donate their child's organs - and thus save other children. I think of them often and wish them much strength!
"When the anesthesiologist called to say that Sofie was in intensive care and we should come, we couldn't have been happier."
Sofie was taken to the operating room at 7 o'clock. She was cheerful because all the nurses and carers on the ward were standing in the corridor and were happy for Sofie. Four nurses and a physician took her to the operating theater. This positive energy made her so happy that it was easier to say goodbye; it was also a great support for me. Dr. Christine Beck, the anaesthetist, was totally sweet to Sofie. She fell asleep within a few seconds and was wheeled into the operating room.
Then the waiting began: my husband came to Hanover and we waited. Everyone encouraged us. When the anesthesiologist called and announced that Sofie was in intensive care and we should come, we couldn't have been happier.
Sofie was on circulatory support (ECMO) and was being ventilated. After three days she was extubated, after another three days the oxygen was taken away and the ECMO was turned down more and more. On the eighth day after the transplant, the ECMO was removed and then completely extubated. Unfortunately, Sophie's condition deteriorated within a few hours. She was intubated again. After a week, her condition improved and she was finally extubated. She still received oxygen and high-flow therapy. A few days later, she was transferred to the normal ward. There, Sofie's condition improved rapidly. Within a few days, Sofie was breathing independently and without help. After months, she started eating again and became more and more mobile. We were discharged after six weeks.
"We are infinitely grateful for this opportunity to spend time and have fun with Sofie."
Now, three months later, we as parents can say: "We finally have a child who moves and enjoys life." She is full of life and is developing better and better. Of course, she still has some deficits due to the 1.5-year serious illness. But she has a zest for life and is getting better day by day.
We are infinitely grateful for this chance to spend time and have fun with Sofie.
This would not be possible without
- the transplant team of the MHH Children's Hospital PD Dr. Schwerk, Dr. Müller and Dr. Carlens, Prof. Baumann, the physicians* and nursing staff of ward 63,
- the transplant team of surgeons, Dr. Bobylev and Dr. lus, who kept popping in to check on Sofie,
- the physicians and nurses in the intensive care unit,
- the pediatric cardiologists Prof. Hansmann and Dr. Hohmann and their team, and of course the donor family.
- and of course the donor family.
"Everyone fought for Sofie and without them we wouldn't be where we are now."
The mothers of the other TX children were also a great support; friendships were formed. The pulmonary hypertension groups also gave us massive support during this difficult time.
Everyone fought for Sofie and without them we wouldn't be where we are now. She is a lively little girl with a cheeky smile on her face.
When we come to Hanover for check-ups now, it's like a second home. The physicians, nurses and carers are incredibly great. You feel so comfortable and in good hands. You often meet the parents of other transplanted children - which is so nice.
We are incredibly grateful.
Thank you, thank you, thank you, thank you!
Submitted by Doreen Zieglmeier-Schedlbauer, mother of Sofie