Wednesday, March 20, 2013

Emma Hope

Emma Hope
I asked Melissa if she would let me tell her story, a journey that began over a year ago. She answered, “That's not a problem; I would love to share our story :) I am very proud of Miss Emma and would love to share how miracles can happen.
            This is a true story about one of the most difficult but also one of the most moving times I have ever shared with any family.
            I received a phone call a little over a year ago. Dear friends of ours had retired and moved to Arizona after having had enough of our Minnesota winters. They were calling because their own daughter was in a hospital near her home in Wisconsin with a brain aneurysm. Emergency surgery was scheduled for later that day. Her prognosis did not look promising. My friends were still reeling from the news, but that was not all. Their daughter had been caring for her own adult daughter who had her own challenges, including mental health issues and a toddler that her mother felt Melissa was unable to care for by herself. Now that her mother was in the hospital, Melissa was essentially homeless and without a caregiver. And she was pregnant.
            My friends were desperate. Could I help find Melissa a safe place to stay? Would she be eligible for a shelter? She was still staying in Wisconsin and I lived in Minneapolis. Would she qualify for benefits and help here before establishing residency? I couldn’t answer any of their questions without doing my homework, but said I would do all that I could.
            My first day of phone calls dredged up nothing. There were long waiting lists for all of the shelters. Melissa was not technically eligible for most of them because she was not a victim of domestic abuse, nor was she receiving Minnesota Social Security Disability benefits, and she had not been a state resident for the required 60 days. The next day I posted urgent requests on our local childbirth collective resource bulletin board for any help or suggestions. The birth professionals throughout the community here rose to the occasion; I soon had lists of names and agencies to call. Finally, one connection led to another and the grandparents off in Arizona discovered a tiny shelter here – only 7 beds—right here in Minneapolis run by 4 Indian nuns who were members of the Missionaries of Charity of Calcutta, the order of sisters that Mother Teresa founded in 1950. Their only stipulations were that the mother was homeless and pregnant and agreed to abide by their rules. BINGO! Melissa could go there. She could then apply to the local social services and begin the process of finding an appropriate permanent living situation. Being near where I live also helped. I could visit her often and help her negotiate both the welfare system in our county and the medical clinics where she would get optimum prenatal care.
            Her uncle drove her to Minneapolis the very next day and I met her at the shelter with a fresh bouquet of flowers for her new room and to help her get settled. The relatives in Wisconsin, though they were not in a position to house her, did arrange for her to see a doctor before she left. An ultrasound, a routine procedure to help assess the age of the baby and establish a guess- or due-date was ordered but revealed some disturbing results. Within hours, she was being referred to the neonatologists at a major medical center in St. Paul. There were no advanced diagnostics available in that part of Wisconsin so they wanted further tests done in Minnesota. The results were shattering: the baby had inherited a genetic chromosomal deficiency and had a condition termed fetal or lethal anomaly. That meant that this baby may not live, either to term, or if she did make it that far, there was no guarantee that she would live after that.
            The family was shattered by this latest development. They were still not out of the woods with Melissa’s mom, and were focusing all their energy on her. I assured them that Melissa was doing OK. The shelter was able to give Melissa her own room and she seemed to be settling in just fine.
            During the following week we heard that Melissa’s mother was doing amazingly well, which was a huge relief. In the meantime I had lined up appointments for Melissa: WIC, food stamps, social worker, etc., and we had her medical records forwarded to a neonatologist at a high risk clinic that would be able to let us know what her options were, and what the possibilities might be for this baby. We knew by now that it was a girl, and Melissa promptly named her Emma Hope.
            While Melissa and I were getting to know each other and the doctors were putting together a plan of care for her, her family members were discussing their opinions, unbeknownst to us. Some felt that an abortion was the only route for Melissa given what they now knew. Others felt that such a choice would harm both Melissa’s fragile spirit and her future. Angry words were flying back and forth between the two camps.
            About this time I learned that Melissa was married. When she was pregnant the first time with Lexi who was now a toddler and living with an aunt, she and Mike had married. The stress of raising and supporting a family had become too much for Mike who had his own set of challenges, and he had left, though he stayed in touch with Melissa. When he heard about Melissa’s present situation, he again showed up, offering his sincere support and concern, though he wasn’t financially stable enough to offer her much else than that. Even his living situation was tentative – a couch at a relative’s house.  
            During the ensuing weeks we learned all that we could about her baby’s condition which was now confirmed: hypoplastic left heart syndrome or HLHS.*
            Melissa’s grandparents had also been researching HLHS and were sending me emails with their findings several times a day. They concluded early on that perhaps it would be better to bring this baby home after birth and just love her for as long as they had her until Nature took its course. Melissa and I talked about these options and explored every possible scenario. I found her to be fully capable of understanding the seriousness of the prognosis and certainly able to make choices, in spite of all her challenges. She decided right from the start to carry her baby to term. She was barely 6 months along when we first met, and the hospital had presented her with the option of terminating the pregnancy. She was quite aware of the finality of death having recently lost a beloved grandmother.
            Her family was still very absorbed with Melissa’s mother and helping her recover, so Melissa was still very much my constant companion when I wasn’t working. She had never lived in a city before, had never been on a bus or train. She had literally come out of the woods of Wisconsin. She didn’t know how to find out where things were. We had a great time learning all these things those early months. I suggested she pull the bus stop cord on our first bus ride. She hesitated and then gently tugged on the pull rope until a buzzer went off and then giggled and blushed. She had done it! We went out to the Mall Of America by train for the first time. We ate Chinese phÓ, Mexican burritos and her first-ever chai lattes. I brought her to my apartment and we cooked things she had never heard of. She still teases me about making her eat seaweed – sushi – (I actually only made her taste it) and avocados (guacamole) and artichokes. Melissa went from being petrified of the city to being quite street savvy in a very short time. All of a sudden I was getting texts on my phone that she was at a beauty parlor getting a haircut, or out somewhere at a Chipolte restaurant. A friend of mine picked us both up one day and bought Melissa several maternity outfits before treating us to lunch. By now she was bursting out of her men’s cargo jeans and jerseys.
            The neonatal clinic visits were another story. At the beginning of each appointment, the clinic would do an ultrasound. We could see her baby in 3-D, moving and looking terribly cute and very much alive, but of course we had to remind ourselves that Emma was living off of her Mommy’s heart at this point and wouldn’t do as well once she had only her own defective one to depend on. Melissa was presented with all the statistics and possible outcomes of all the procedures. I personally felt that it would be better to have her baby close for all of her short life than be subjected to numerous surgeries and long hospital stays hooked up to all sorts of monitors and not being able to be held outside of her isolette most of the time. What would bonding look like? I told Melissa exactly how I felt and so did her grandparents who were also leaning toward compassionate care while rejecting the surgical route. This also made sense to Melissa at the time. Then came the appointment with the two surgeons.
            Some of the most eminent surgeons in the country who are treating HLHS happen to practice in Minnesota at both the Mayo Clinic and Minneapolis Children’s Hospital. They met with Melissa and her husband and talked to them about “Giving her their best shot!” and, “We want to give her a chance….” Nothing about her having less chance than most babies with HLHS because of the addition of the genetic problems. I don’t know if it is because they are surgeons, that they hoped to hone their skills and hoped too, to eventually perfect the surgery for these babies, but what came across in the end was, to Melissa and Mike at least, a chance to grab at hope and possibly a cure. 
          I tried to explain what that would entail and that no guarantee was being given that Emma would even survive each surgery or the recovery phase after each one. But I also had to trust that Melissa was Emma Hope’s mom, in spite of everything else, and could and should be able to decide whatever choices she had to live with for the rest of her life. In the meantime while the rest of the family decided together that compassionate or only palliative care was the best option in their eyes, Melissa finally decided otherwise. She would give her baby every chance she could… and the differing opinions among the relatives continued.
           About this time I discovered a beautiful book called Waiting with Gabriel: A Story of Cherishing a Baby's Brief Life by Amy Kuebelbeck, (Loyola Press, 2003). Gabriel was also born in Minnesota with HLHS. His parents chose compassionate care and stayed with him for his entire, though short life. He died shortly after birth in his mother’s arms. With my encouragement most of her family read the book. So did Melissa.
            Then then emails started once again. Couldn’t I persuade Melissa and Mike to change their minds? Some felt that she couldn’t possibly decide these things for herself, that she was too handicapped to comprehend the consequences.
            I felt otherwise. As part of my own research I even arranged to spend time at Alexandra’s House, an infant hospice in Kansas City, Missouri and peppered them with questions about how I could best support Melissa and her family. I spoke with providers, exploring everything I thought would need doing: permission from the nuns to bring Emma home to the shelter and explaining how the hospice nurses and I would take care of Emma and Melissa for as long as was needed. The nuns offered to have the wake in their little chapel when the time came, and a priest from the family’s parish in Wisconsin was notified and agreed to be on call to come whenever he was needed. A parishioner lovingly built a beautiful little coffin. So, for Melissa to change her mind and decide she just couldn’t do nothing, which was also a choice, was very difficult for some of her family to accept.
            Finally I added my opinion to the discussion by writing an open letter to Melissa’s family explaining how I felt: that I didn’t have to live with her decision for the rest of my life, nor did they. Only Melissa could ultimately decide what was best for her and her baby and we must not only respect that choice, but we have to continue to surround her with love and support her.
            Emma decided to arrive just before her due date on June 14th. After a short labor she was delivered vaginally and needed little help starting up on her own. She weighed in at a whopping 8 pounds and 3 ounces. We got to see her briefly as she was being whisked off to the NICU where she would be prepped for surgery during the coming days. Melissa’s mother was even there, having made an amazing recovery herself in time to see her little granddaughter for the first time.
            The next six weeks were a blur of doctor appointments, tests, surgeries, and ups and downs while constantly holding our breaths hoping that things would continue to go forward for beautiful little Emma. Melissa was able to stay at the Ronald McDonald House near the hospital and visit Emma. She pumped her milk and delivered it daily to the NICU. Emma just surprised us all and kept growing with very few setbacks during those early weeks. While all this was going on, Mike started looking for an apartment to bring his little family home to. When Emma was finally discharged they moved to a town in southern Minnesota. 
            Part of our concern was if Melissa and Mike would actually manage to learn and demonstrate that they could perform all the various care and procedures that Emma would require. During all of this time Melissa had often neglected her own battle with diabetes, and that alone left us wondering if she could manage that along with attending to Emma’s needs. But Melissa surprised us all. At one point she suddenly rose to the occasion. Not only was she managing to follow her own scrupulous diet and keeping her blood sugar levels within range, but she learned how to suction, tube feed and maintain Emma’s equipment in time to bring her home.
            The second surgery went as well as could be expected when Emma was 4 months old and Melissa moved back to the Ronald McDonald House once again. And again, Mike and Melissa were able to take Emma home after another month in the hospital. Emma continues to do well. She can sit up, reaches for things, coos and laughs and enjoys her big sister Lexi whenever they can visit. Emma is 9 months old now and weighs in at 17 pounds! She is a miracle. She’s gone beyond any one’s wildest dreams for her.
            I have learned from Melissa that I can never say what might be best for anyone else. I have learned to stand in awe of the power of a mother’s love. Mike and Melissa know they might not have Emma forever, but they have learned how to live only one day at a time. I have learned a lot from all of them: Melissa, Mike and Emma. Thank you. Forgive us all for ever doubting you.

*More on HLHS:

Hypoplastic left heart syndrome occurs when parts of the left side of the heart (mitral valve, left ventricle, aortic valve, and aorta) do not develop completely, meaning that the left ventricle of the heart is severely underdeveloped. The condition is present at birth (congenital). Modern technology is actually addressing this with staged surgeries to first close off parts of the heart using what is called the Norwood Procedure which is usually done shortly after birth, and then two further open heart surgeries are done to attempt to reconstruct the heart in order to allow for better circulation. Without life-prolonging interventions, HLHS is fatal, but with intervention, an infant may live with a five-year survival of about 65 % even after surgical repair. Hypoplastic left heart syndrome accounts for 2 to 3 % of all congenital heart disease with a prevalence rate of two to three cases per 10,000 live births in the United States.
            A pediatric cardiothoracic surgeon may perform a series of operations or a full heart transplant. In the meantime, the ductus must be kept open to allow blood-flow using medication containing prostaglandin. Sub-atmospheric concentrations of oxygen are used in the preoperative and postoperative care of infants with HLHS. The Norwood Procedure is done almost immediately after birth, the Glenn Procedure at 3 to 6 months of age, and the Fontan Procedure at 3 to 5 years of age. Those babies who undergo cardiac transplantation have a 5-year survival of approximately 50-60% as a group. However, it is becoming clear that there are higher risk subsets where 5 year survival is lower including those with lower birth weight, additional congenital anomalies, a genetic syndrome or those with a highly restrictive atrial septum. For patients without these additional risk factors, 5 year survival now approaches 80%. Further, studies show that about 50% of those children who survive surgery in the early development of staged reconstruction have developmental delay or need special education; about 25% of these surgical survivors have severe disabilities. There is growing evidence that while the incidence of developmental and behavioral disabilities continues to be higher than that in the general population, children operated upon in the more current era have shown significantly better neurological outcomes.
            Some parents choose an option referred to as compassionate care, and these infants receive treatment aimed at relieving suffering without extending life. Compassionate care is overseen by a physician, and may be carried out either in the hospital or at home. Studies show that when presented with all options, about half of parents choose life-prolonging interventions and half choose compassionate care, although when I began questioning our hospital’s neonatal cardiologists, they told me that they had actually never had a family choose compassionate care and that over 99% here in Minnesota at least went ahead with the surgeries. A study in 2003 concluded that a selection of physicians who are experts in the care of children with HLHS were evenly split when asked what they would do if their own children were born with HLHS, with 1/3 stating that they would choose surgery, 1/3 stating that they would choose palliative or compassionate treatment without surgery, and 1/3 stating that they are uncertain which choice they would make. The three-stage procedure is a palliative procedure (not a cure), as the child's circulation is made to work with only two of the heart's four chambers.
            While infants successfully treated for HLHS have a good chance of survival, they may experience chronic health problems for the rest of their lives. The 3-stage surgeries were developed in the early 1980s with no survivors prior to that time. Therefore, the earliest survivors are in their early 30s today and the long term prognosis is unknown.  However, the advances in surgical and medical techniques have helped increase the survival rate dramatically since the surgeries were first developed.
            As is true for patients with other types of heart defects involving malformed valves, HLHS patients run a high risk of endocarditis, and must be monitored by a cardiologist for the rest of their lives to check on their heart function. The three stage Norwood Procedure only increases the life span of the heart as HLHS cannot be cured. A heart transplant may be needed at some point in the child's lifetime. By the time the child is 3 or 4 years old, they may become a good candidate for a heart transplant, though of the over 1,000 children a year in the U.S. waiting for a donor heart, only about 70 actually receive one. Of those, there is still the possibility of the body rejecting the new organ, or of developing other side effects from the anti-rejection drugs, cancer being one of them.

©Stephanie Sorensen 2013

1 comment:

  1. this is beautiful steph. Thank you so much for sharing our story. I had doubts myself about raising emma but in my heart i knew it was the right thing in the end. We can only live one day at a time.