“My Emmy touched so many lives and despite what she went through on a daily basis, she was positive and inspired others to be better people,” said Mary Peters Kramer on Thursday, the week after her daughter passed away. “I will continue to make sure people know who Emmy was and what she meant to everyone.”
Kramer added that while she was Emmy’s primary caregiver with the constant support and assistance of her other children, Mallory, Phillip, Kenzie, and Sydney, it was almost a role-reversal in terms of support.
“Somewhere along the way we realized that Emmy was actually pulling us along,” said Kramer. “She taught us to find a strength we didn’t know we had to do what needed to be done. The whole family stayed close, and it was such a pleasure to have her in our lives.”
Emily “Emmy” Christine Kramer, 19, Rich Fountain, started her first days of life at St. Louis Children’s hospital with a critical lifetime illness, and passed away Sunday, Nov. 29, 2020, at St. Louis Children’s Hospital in St. Louis, where she was well known and a favorite among doctors and staff.
“Emmy spent about 1,400 days of her life in the hospital,” said Kramer, noting there were 17 emergency airlifts, and more than 50 ambulance rides during Emmy’s treatment.
On average Emmy spent 4-12 weeks of every single year of her life inpatient at St. Louis Children’s Hospital.
“Every single year of her life,” Kramer reiterated. “We got in a groove of rushing Emmy to emergency rooms on our own, never waiting for ambulances, but the time came in her life when that was no longer the best choice. I didn’t really want to call the ambulance the first time. Honestly, I was afraid but it turned out to be a new sort of partnership in caring for my Emmy and they were so wonderful. They were so full of love and compassion for my child.”
After that first time, it became easier.
“The ambulance crews were the frontline guardians for Emmy,” said Kramer. “There was a lot of pressure on them to do the right thing and they handled it well. They have been the utmost professionals in helping me get Emmy stabilized but they also developed a very strong relationship with Emmy and the whole family — a relationship of friendship beyond the job that they had to do.”
Emmy was the first patient enrolled in the Special Needs Tracking Response System (STARS), which was developed at Cardinal Glennon Children's Medical Center in St. Louis. A look at that program and how it benefitted the Kramer family appears separately in this week’s paper.
When Emmy was born and diagnosed with DiGeorge Syndrome, she underwent a massive open-heart surgery at nine days of age. None of her medical team was sure she could even survive that surgery, and it was a 100-day struggle of clinging to life before it looked like she might. That is when the family was told by Dr. Andy White, an immunologist, that “what Emmy just went through —that will not be the mountain in her life,” and then explained that DiGeorge Syndrome includes immune deficiency, with Emmy having only about 10% of normal immunity against infections. Further complicating the situation is the fact that what immunity there is gets confused, and attacks healthy cells and tissues.
“So our Emmy basically had no immune system and her life would be plagued with life-threatening infection after infection,” Kramer explained. “Her team just did not feel it would be possible for Emmy to survive even two years if that.”
DiGeorge Syndrome can also include birth defects such as congenital heart disease, which was present in Emmy, who has had two open-heart surgeries and a special valve placement over the years. As time went on, it was clear that Emmy had a worst-case presentation of DiGeorge Syndrome, along with her body’s other unique illnesses and conditions. Many times Emmy’s illnesses were a source of mystery to the team of specialists that treated her.
"It was like a build-your-own game," said Kramer. "They just kept adding squares. This syndrome created unique diseases that doctors didn't know how to treat. Most of the diseases she had are incurable and many times her medical team would say ‘She is writing her own book,’ in regard to treating her.”
Shortly after being diagnosed with DiGeorge Syndrome and finding out the severity of the range of that syndrome that she was burdened with, Emmy was one of 10 or 12 children in the entire world chosen to be evaluated to receive a thymus tissue transplant by a world-renowned specialist at Duke University. Unfortunately, after months of testing and a two-month stay at Duke Medical Center in North Carolina, Emmy was not given the transplant due to some FDA regulations.
Luckily her physician there, Dr. Louise Markert, also possessed deep ethics and would not do this procedure on Emmy because she had no confidence that it could even help her after all the testing was done, Kramer noted.
"Emmy was a high celebrity soon after she was born, and it amazed me how many people showed their concern for her and constantly rallied around her showing support," Kramer added.
Kramer noted that Emmy’s constant life-threatening illnesses made it virtually impossible for her to have any socialization outside the home, and it was especially difficult to even consider attending school, though Emmy did achieve some milestones on that front.
"It was always a real challenge for her to go to school because of her health burdens and the extra layer of danger being around others posed for her," said Kramer. “Through her entire life, the most she was able to handle physically is a very, very part-time schedule if even that.”
As the years went by, and Emmy continued to overcome ridiculous odds, it was also a reality that she continued to acquire more and more disease and health burdens. When Emmy was 9, St. Louis Children’s Hospital established a Palliative Care/Advanced Care team to support kids with no cures.
The Palliative Care Team operates at a level just below hospice and tries to help patients enjoy their lives despite the burden of intense, incurable illnesses.
“They teach you to reclaim little parts of your life when you can,” said Kramer. “You have to maintain your identity so you can help others. We focused on quality of life and learned to make the best of any situation and just make absolutely sure to ‘Live each day well.”
They helped both Emmy and her mother try to find a balance between “being alive” and “living.” At one point, Emmy spent yet another year battling a devastating disease that gave her little hope of survival — again — but as she seemed to stabilize, Kramer searched for a new sense of selflessness, realizing Emmy’s life was her own.
She engaged the help of the Palliative Care Team, which provides advanced care for quality of life, which is something Kramer took very seriously.
"What could we do to make her life better? I'm talking about emotionally, not just physically," said Kramer. "Where can she go and what can she do? What does Emmy need to add joy to her life so that she might find the strength to keep plodding? There's a risk involved with everything she does, but we asked her what she wanted to do."
With little time spent at school, it was one of Emmy’s greatest wishes to return and participate in daily activities at Fatima.
"It's really hard to not go back to school," said Emmy in 2015. "I feel like I'm not part of things."
"Like for any teen girl, it was emotionally nerve-racking at age 14 to go back to school after basically missing an entire year because she has never had a chance to build a lot of relationships, but she was very excited, and it was a great day,” said Kramer. “It was a real ‘win’ for Emmy to be able to have a tiny bit of control in her life."
That was in 2015, at the start of Emmy’s freshman year at Fatima. The most she attended in one year was maybe 19 days, which Kramer called a victory.
Emmy was able to attend school for a short time during Red Ribbon Week. “She had wanted to be at school for Pajama Day since the fifth grade, and she was finally able to do that,” said Kramer. “Her wants were so simple. Just knowing she had her own locker at school, even if she wasn’t there to use it much, was very exciting for her. She bubbled over It was her taste of normal.”
In 2013, as part of the Make a Wish Foundation program, Emmy was able to visit the cast and crew of "Cake Boss," a show on TLC. “That was a very special occasion but it almost didn’t happen,” said Kramer. “Emmy had a bad turn and was in the hospital for a couple of weeks. The Foundation gave her until Wednesday to commit to the trip and on that Wednesday she had recovered enough to be discharged and we left a couple of days later on Saturday for the trip.”
While she was enrolled in grades 7-12, Emmy’s Special Education teacher at Fatima was Stephanie Hoffman, who said this young woman was a kind and loving individual.
“She had an aura about her that would turn anyone's day around,” said Hoffman. “You couldn't help but love being around her. Emmy was my student for six and a half years. I loved every minute of her coming to school when she was able, going over to her house to tutor, and most recently our Zoom meetings and reading the ‘Little House on the Prairie’ series. Emmy put her all into everything she did. She would read until her lungs would no longer allow her to. She would write her sentences when her hands were tired and finish the math problems even if she wasn't feeling the best. I couldn't have asked for a better student.”
Emmy’s family constantly thought of how special life events could be modified so that she could get her chance to enjoy them. One example was being glammed up by her sisters just so she could be able to participate in photos for prom, even though her health prevented her from actually attending the event.
Her brother even put on a shirt and tie and surprised her with flowers and was part of her photoshoot.
One of her greatest life wishes was to be part of her sister’s wedding this past fall. She was taken to have a dress-fitting and she finally even got permission from her immunologist to get her ears pierced in preparation for the wedding but that was only part of it.
“Emmy was very nervous around large groups of people and decided she would maybe feel most comfortable walking down the aisle with her soon-to-be brother-in-law, the groom. They were very close,” said Kramer. “They almost did it, too, but in the end, she walked with her nephew, one of her very best friends. It was very sweet.”
It also turned she wanted to not only be part of the wedding but to make a toast. Most people don’t think twice about jotting a few lines, but for Emmy, it was a tall order.
“She worked very hard with her speech therapist on her toast, tediously pushing through to write it out on paper despite her weak, trembling hands, and then found the courage to stand with a microphone and deliver it in front of 200 people,” said Kramer. “She was an incredible kid.”
Emmy also enjoyed playing cards and going for a ride to get sweet tea from McDonald’s. “Everyone knew she loved that tea, and every day, someone would bring her one,” said Kramer. “There was a network of people who just wanted to make sure she had it, and Emmy would send out Snapchats or other messages letting people know ‘look what I got again today,’ with a picture of her with her tea.”
After she turned 18 — a modern miracle for someone who was not expected to celebrate her third birthday — Emmy needed an official identification card. “Emmy didn’t do well around camera flashes, so we had some trouble getting a picture for the ID,” said Kramer. “It took weeks of practice to build the nerve but she got it done. Turns out that and her eyes were closed, but she was so happy to have that piece of adulthood right there in her wallet.”
To celebrate the victory of turning of Emmy turning 18, Kramer and her other daughters took Emmy to Oklahoma, where she was able to gamble at an Indian casino. “We had a blast,” Kramer said. “We knew there would be a risk but everything in life has a risk. Sometimes you have to challenge that. Life is to be lived, not endured.”
The ultimate achievement came last May when Emmy walked across the stage to receive her diploma with classmates at Fatima.
“With a lot of preparation she was able to deal with the large crowd,” said Kramer. “We knew that would be a challenge because she has been so isolated all her life, along with physically walking, but she did it, and we were so proud of her.”
“When graduation time came around my heart exploded with happiness when I saw her walk across that stage,” said Hoffman. “She worked so hard for that one single moment. This teacher-student relationship came with a bonus. I was lucky enough to earn friendship status with Emmy and her family. This is a friendship that will always stay with me. Emmy will always hold a very special place in my heart. She is truly one of a kind and will never be forgotten.”
A moment of silence in Emmy’s honor was observed at Fatima High School upon learning of the loss of Emmy. “Our family was so immensely touched by this show of respect for Emmy’s life,” said Kramer.
Emmy’s remarkable journey was made so not just because she defied the odds and lived longer than anyone expected, but because she lived with an attitude that inspired others.
“She carried her load so gracefully,” said Kramer. “Her life was horrible. She suffered daily and yet she chose to get through it and get on with the day. She was very conscious of her suffering but rose above it, carried it gracefully, just focusing on all the blessings of life. She taught us all that joy could be found under any circumstances — we just had to choose to find it.”
Early in Emmy’s life, Kramer said there was a real connection with doctors who had a genuine interest in her well-being. “She grew up with them and they grew up with her,” said Kramer. “We had an intense relationship in the medical world — these are the people who stood beside us during the worst of the worst events for Emmy. They have been kind, dedicated, and understanding. And beyond that, they had a real love for my child. I think they kind of clung to Emmy as a sign of the importance of their work and that helped them push to be even better physicians. On Emmy’s last day with us, even though it was a Sunday, her closest doctors came running to the hospital to kiss our Emmy on the head, to join us in feeling the loss of someone so special. To sum it all up, St. Louis Children’s Hospital was like a second hometown to both Emmy and me.”
As parents with young children will understand, Kramer said she was driven by a need to make sure Emmy had whatever she needed. “When the baby is young, you do whatever it takes,” she said. “All you want to do is take away the pain, hold out for things to just be ‘fixed’ and do whatever you can muster to just make things better, but with Emmy, that just wasn’t possible. So eventually we had to gain an understanding of how to just accept what our Emmy was asked to carry in life and focus on every little thing we could to make her comfortable, and to bring joy to her life. She reached incredible, unexpected milestones in her life and no one will ever forget the first time she took eight or 10 steps on her own when she was 5 years old. That event drew a crowd.”
Family was very important to Emmy, and her family put her wants and needs above their own. One year, despite an extreme situation of serious illness starting on Christmas Eve, Emmy begged to stay home so everyone could be together on Christmas. She had missed Christmas at home the two preceding years and this was so important to her.
“Honestly it was petrifying, with the terrible shape she was in due to illness, but we straightened our backs and just gave it a try, knowing at any moment we may have to call our ambulance friends to save her,” said Kramer. “So at four or five in the morning we all just came together opened gifts — very quickly — and then I rushed her to the hospital,” said Kramer. “Somehow, she barely escaped completely going into septic shock again but fulfilled her wish — she was amazing like that.”
By the end of her life, Emmy had a team of 16 different specialty physicians in addition to her lifelong primary care physician, Dr. Jack Dodson, of Jefferson City Medical Group, who had treated her since birth.
“He took on the enormous challenge of caring for our Emmy and went out of his way to help,” said Kramer. “He is an amazing doctor who has treated all my kids, and I could count on him for anything at just a moment’s notice.”
Four nurses worked with Dr. Dodson’s office over the years and all bonded greatly with Emmy. Also several years ago, Emmy was given the ability to have a part-time personal aide. A very special friendship developed with Kirsten Steinbeck, of Linn, who was in nursing school when she met the young lady. Her care for and relationship with Emmy immediately developed beyond the job.
“She was so good to Emmy,” said Kramer. “They were best friends, with a lot of chatting that brought Emmy to a whole new level. Emmy hadn’t experienced that before. Kirsten is really only a couple of years older than Emmy and their friendship helped Emmy become more adult-like, more exposed to what friendship can mean in the life of a teenage girl.”
Steinbeck said that at first, it was just a great opportunity.
“It was a job where I showed up, did what I needed to do, and then socialized with Emmy by getting my butt kicked at Disney Scene-It,” said Steinbeck. “Quickly, this opportunity and job came to mean much more than that to me. Emmy and I became friends, which lead to a close bond between us, and in Emmy's words, I am her 'very own best friend.’ We enjoyed spending time together whether that included reading a chapter book, learning a new card game, writing letters to Emmy's pen-pals, or listening to Christmas music all year long.”
Emmy would want to go out and do things like going to shop for a gift for someone at Target and Steinbeck said she really enjoyed that activity. “So in strength, Emmy tucked her medical burdens in her purse, not allowing them to stop her, and would go shopping with her best friend. Emmy was able to do this so often; she would overcome whatever medical burden that was present by tucking it away and off we would go to enjoy life. Together, Emmy and I liked to make trips to Bob’s Frozen Custard in Belle, Starbucks, inviting a friend over to play games with us, Dave's Pizza and Wings in Linn, and for my birthday, Emmy was able to come to a night out with 'the girls' for Mexican. Emmy and I loved to share each other’s company during all life adventures and we laughed a little too hard together sometimes. My goal was to help her achieve what she wanted to achieve and enjoy life while doing it. Without her knowing it, Emmy taught me so many life lessons, including how to enjoy the little things in life. Because Emmy did just that, despite what the burden was, she was determined to overcome it, to be able to enjoy the little things. While I will miss Emmy so dearly, I will forever cherish our memories together, as we've created many over the last three years. I will forever be grateful to the Kramer family, but especially Mary, for allowing me in her home and treating me as if I was one of their own. Emmy has left her mark on this world but for those of us close to her, she lefts a bigger mark on our hearts. It was just simply who she was that touched you.”
Nurse Mallory Goodman worked with Dr. Jack Dodson in caring for Emmy for the last four years. In a moving post on Facebook, Goodman said that while it would have been nice to enjoy Emmy’s company under different circumstances, “She always brought joy and light to the room. Even when she wasn't feeling well, she always pushed through and found a way to make me smile or laugh. Over the years Emmy has gifted me with a McDonald's sweet tea koozie, cupcakes, and flowers, but most importantly love and happiness. Her spunk and sass will forever be my favorite memories of her. Emmy lived life to the fullest and a huge credit goes out to the Kramer family for allowing her to do so. Fly high Emmy, you are so loved.”
Kramer had to learn a lot of things as the primary caregiver.
“It was vital that I understood how everything worked, and what to watch for when Emmy wasn’t feeling well,” said Kramer. “I kind of had to master a basic understanding of all body systems, which isn’t easy when you are ‘just somebody’s mom.’ It was daunting, especially in the beginning, but I had a lot of help from my other kids. I felt called to be her caregiver. It was exhausting but worth every minute, and caring for my Emmy was the absolute joy of my life. Her example made me such a better human being and her love carried me through the hard days.”
When Emmy needed to be hospitalized, her doctors were eager to help.
“I know the specialists in St. Louis loved Emmy,” said Kramer. “They saw her not only as a medically-special patient but as a human being. I think working with Emmy all these years made them better doctors because of all the challenges her condition presented.”
When the end came, Kramer said she was devastated as she watched the doctors work on Emmy.
An ICU doctor who had only known Emmy for seven days was just one of the many physicians to enter the room and kiss Emmy on the forehead in a gesture of respect. “In just one short week of life my Emmy made a life-altering impression on this woman,” Kramer said.
Kramer was understandably emotional as she spoke of Emmy last week.
“I don’t know what I’m going to do, I have to rediscover who I am,” she said. “Emmy was the other half of my very heart, and I feel a little lost now.”
A simple walk is something new for Kramer. “I can just take a walk if I want, down to the neighbor’s house and back, or I can run to the store or run errands, but it doesn’t feel right, it is not a feeling of freedom, so to speak,” she said. “Before, I might have been able to walk to the mailbox but then I rushed right back to check on Emmy. It was just part of life, and I accepted it. Now, I keep looking at her room and listening for her, longing for her.”
Emmy is not truly gone as long as she is remembered, and Kramer plans to make sure everyone remembers her daughter and her special life journey.
“Emmy squeezed every last drop out of life every day,” she said. “What we do, how we act, what we say, it all matters in the world. Emmy understood that and somehow managed to be positive in the face of the most adverse conditions. I will carry that with me for the rest of my life.”
Kramer added that she and her family have survived through the support of those in the community.
“I can’t begin to express my appreciation,” she said. “It has been a difficult journey, and especially difficult at this time with the loss of our beloved Emmy, but we have been shown love and support beyond anything I could have imagined. We have always been so touched that our Emmy inspired so many others.”
Memorial contributions suggested to Emmy Kramer Memorial Fund in care of Mid America Bank. Condolences can be mailed to the Kramer Family, P.O. Box 234, Westphalia, MO, 65085.