Read all about it

Scott and I were talking today about how many people are reading this journal that we’ve never met, and we wonder what they know about Ben. I thought that it might be worth the time to sort of write out how we got where we are, and the time line of what has happened in Ben’s short life so that you can all know where we have been and how we got here.

In late September of last year, I had an elective blood test done during my pregnancy. It is called a quad screen test, and it will test for certain fetal proteins in the bloodstream…higher or lower levels of these proteins can indicate a neural tube defect in the fetus, or a trisomy (tri = three, somy = relating to the chromosomes, so an extra chromosome in one of the pairs in the body….). Down Syndrome is trisomy 21, which means that people with Down Syndrome have an extra twenty first chromosome. There are other trisomies too, trisomy thirteen and trisomy eighteen are both conditions that are fatal in the first year of life. After the results of the blood test came back showing that the baby may have an increased risk of having Down Syndrome, we had the chance to have an ultrasound where they would look for “soft” markers…physical characteristics that may show if the risk for Down Syndrome was indeed there. After that we would have the option to have an Amniocentesis where they could count chromosomes and actually determine if the baby had Down Syndrome. Originally we did NOT want to have the amnio because there is a risk of miscarriage and we had decided years before that we would raise any baby we have with Down Syndrome.

We went in for an ultrasound in early October, and the doctor at the hospital informed us that the heart looked irregular. At the time, he told us that the baby had a ventricular septal defect (VSD), which is a type of hole in the heart. If you draw a box, and put a plus sign in the middle (kind of like how little kids draw a window, a box with four quadrants) you have a “stick figure” heart: the top two boxes are atria, the bottom two boxes are ventricles. A ventricular septal defect is a hole in the wall between the ventricles. The doctor strongly recommended the amnio, because almost half of all children born with Down Syndrome have VSD’s…it is quite common. We were also told that this condition was completely operable, and easily fixed. We anticipated having surgery within the first six months (or around six months) of life.

We wound up deciding to have the amniocentesis, because we wanted to know if it was Down Syndrome, and we wanted to rule out either of the other two trisomies. In a cruel twist of what I now recognize as irony, I made this statement to my mom, to Scott and to the doctor: “I could never bring a baby home and know it would not see it’s first birthday, and watch it die in the first year of life”. Don’t ever make statements like this; they come back to you later.

We went down to Detroit to Hutzel Women’s Hospital to have the amnio. Hutzel is associated with Wayne State University School of Medicine, and they deal with a lot of high-risk issues with pregnancies. We met with a genetic counselor who explained what would happen, did a full family history, and explained to us what Down Syndrome is. One advantage I am very grateful for is that Scott and I both have Science backgrounds…. we understand the biology, how it is caused, and what happens to create this condition. We are definitely at an advantage when having these things explained to us. We also arranged to have a fetal echocardiogram done to assess the heart defect. After these two procedures, it was determined that 1) we were having a Boy…2) He would have Down Syndrome and 3) He had a congenital heart defect. They recommended that we follow up with a pediatric cardiologist at Children’s Hospital.

All of this happened in the month of October. We figured that the appointment with the cardiologist was not pressing…. we started to do some research on Downs, read lots of books, talked to parents, and started to make a plan. We actually got excited about as much of the time as we were scared…. and we were definitely scared. But overall, we were ready for the challenge.

As October turned into November, we started to get referrals and I talked to my Doctor about getting a Pediatric Cardiologist. Then the holidays hit…by the time we had an appointment, it was January 27 and my due date was only about a month and a half away. The cardiologist looked very carefully at Ben’s heart, and it took a long time. At the end of the echocardiogram (a fetal echocardiogram is basically a detailed ultrasound) she invited us into a conference room and explained that the baby had a second heart defect known as a Truncus Arteriosis. Go back to your “stick figure heart”. Draw two tubes that leave the heart, one from the top right box, and one from the top left box. The one on the right exits the heart, curves to the left and goes to the lungs. This is the pulmonary artery. The one on the left exits the heart and curves to the right, this is the aorta, it carries oxygenated blood to the rest of the body. In a heart with Truncus, these two arteries are fused so that instead of two arteries, there is only one. The cardiologist explained to us that surgery to fix these two heart defects could be done, and we thought that she was telling us that it was completely reasonable; tricky, but reasonable and doable. We kept hope that everything would be okay.

My pregnancy was now officially classified as “high risk”, and both the cardiologist and my doctor recommended that I transfer my care to Hutzel Hospital for the remainder of the pregnancy. They said I could give birth at Huron Valley Sinai hospital if I wanted, but Ben would have to be transferred to Children’s Hospital Neonatal intensive care within twelve hours of birth. There was no way I was going to remain at Huron Valley while Ben was in Detroit. If I was at Hutzel, I could go and see him at any time. Hutzel, Harper, Detroit Receiving and Children’s Hospitals are all connected via an underground tunnel system, so I would be able to visit him even as I was recovering from delivery in the hospital. My Doctor (who, it should be stated is the best doctor on the planet…) arranged for me to be seen by a group of doctors associated with Hutzel who specialize in high-risk pregnancy and deliveries. Less than a month before I would give birth, I started seeing a new OB.

While this was going on, actually back in December, it was recommended that we start monitoring the baby’s heart so that as he grew in the third trimester we knew that there was no stress on the heart. The week after Christmas I started going to Huron Valley twice a week for “Non Stress Tests”…I sit in a recliner, they hook up a fetal monitor to my belly, and I watch TV for a half an hour. Every time the baby moves, I push a button and a little machine records the movement. A doctor then reads this, and makes sure that the heart rate responds to movement. When I started seeing the new Docs down in Detroit, I was going once a week. Scott and I also made an appointment to meet with the team of Doctors who would be on call around my due date, one of them from Children’s, one from the high risk group, and a third from Hutzel…they would assemble an Intensive care team to care for Ben directly after birth. In addition to this, I was having more ultrasounds done…one a month for the duration of my pregnancy. By the last few weeks before I delivered, I was in a doctor’s office or a hospital four to five days a week for some reason or another.

I went into labor on the night of February 20…I’ll spare the details, but it was a beautiful, easy, and amazing labor and delivery. I am lucky to be one of those healthy Polish girls who can have babies with ease. Benjamin Scott Randall entered the world on February 21 at 5:27 PM. He was whisked away to the intensive care unit at Hutzel immediately, and once he was stable, a transport team from Children’s took him over there…we did get to see him and hold him before he left. By ten in the evening, I was in a recovery room, and a call from the NICU nurses at Children’s Hospital told us that Ben was doing very well.

The next morning the first thing Scott and I did after breakfast was go to see Ben. The nurses brought us a wheelchair, and I climbed in for the ride. As we were greeting Ben and talking to him, the cardiologist on call, a different one than we had seen before, came into the NICU and introduced himself to us. He suggested we go and talk about Ben and the results of the echocardiogram that had been done after birth. He said to us that not only were the two defects we knew about present, but a third defect was identified as well. Remember your drawing of the heart? Find the Aorta…after it crosses over the pulmonary artery, it divides in two. One half supplies blood to the upper part of the body, the other part supplies blood to the lower extremities. There is a condition called a Coarctation of the Aorta, which means that there is a constriction in the aorta, like someone tied a string around it. Ben’s is in the part that supplies blood to the lower part of the body. The cardiologist said that the first two defects (The Truncus and the Septal defect) had only been seen TOGETHER a total of about fourteen times…ever…in the world. Adding in the coarctation created a heart that had never been seen ever, in any body, anywhere in the world to his knowledge. This is all before we add in Down Syndrome. Ben was one of a kind.

Fortunately for us, we didn’t have a rushed decision to make. Ben was on a hormone called Prostaglandin when he was in the NICU…the reason for this had to do with the heart and its anatomy. When we are in the womb, we obviously don’t breathe air; we get oxygen from the mother’s blood. There is a small tube in the heart called a “Ductus Arteriosis” which bypasses the route to the lungs. This duct closes shortly after birth, since the baby starts breathing air, and the lungs provide oxygen to the body. The Prostaglandins keep that duct open, and Ben was doing great…he wasn’t on a ventilator, and we could see him whenever we wanted. Obviously he couldn’t stay there forever, but we had time to make a decision.

We were given three surgical options. One was to fix all of the defects totally and completely, just as if they had all occurred individually. The Second was to perform an operation called a Fontan procedure…it turns the heart from a four chambered pump to a two chambered pump. The third was to put Ben on the list for a heart transplant. Our fourth option was to take Ben home and make him comfortable, involve hospice, and love him for whatever time he had.

The first option, complete reconstruction, was not a good option for a few reasons. First, it is a very long and complicated surgery to begin with, involving putting Ben on a heart and lung machine, and doing a very lengthy and complex surgery on a heart the size of a golf ball. Secondly, it turns out that Ben’s heart has another issue: the left ventricle is proportionally smaller than it should be. Some of the space in this ventricle would be taken up by a tube that would have to be put in to fix the Truncus, meaning that not enough space would be available for proper function of the heart. The cardiologist consulted with several surgeons who were initially very interested and confident in the reconstruction, until they saw pictures of Ben’s heart. Then they stated, too, that his specific heart anatomy would not support the surgery well. The chances he would make it through the surgery were very, very small. If he did survive this surgery, he probably would have other surgeries later in life, because he would outgrow the “fixes” they did in the heart, or some of these would leak. We were looking at multiple open-heart surgeries over the course of his life.

The second option, a Fontan Procedure, has some specific requirements for blood flow through the lungs, most of which are not typically fulfilled by patients with Down Syndrome. In addition to this, it is a set of three surgeries, all before the age of two or three. This procedure had been tried on a few Children with Downs, and all of them had died after the surgery…it was clearly not an option for Ben.

Transplant was the only true option we had, and it has it’s own set of issues. First, there is the wait for a heart. Then there is the issue of making sure Ben is a good candidate for a heart. After the Transplant, patients are on immunosuppressive drugs to make sure the body does not reject the heart. These drugs are a life-long necessity; transplant patients are on them forever. There are a couple of issues with this: first, many people with Down Syndrome have increased occurrence of respiratory infections and many other diseases, including leukemia. Leukemia is a cancer of the blood that affects the immune system. So we would be taking a kid who was already going to potentially have lots of issues with illness and his immune system and put him on drugs to further suppress his immune system. In addition to this, there is the issue of self-care. We have no way of knowing the degree to which Ben’s Down Syndrome will manifest itself, so we don’t really know if he will be able to live on his own one day (even though the chances of this are good), much less keep track of drugs that are imperative for his survival.

We were faced with a major ethical decision that I hope I never have to make again. Do we go the whole nine yards for my kid, knowing he may not make it through the surgery, and if he does, the after effects will severely restrict his quality of life, or do we live whatever life his has with him and make sure it is the best we can give him for however long he is here? Over the next four days Scott and I had both sets of parents meet with the cardiologist so that they could get all the information first hand, and ask any questions they had. Two very important questions that stick out in my mind were asked by our parents. One was from Scott’s dad, that was to ask the Cardiologist how many babies he had sent home to be cared for by hospice. In other words, how many times had he suggested non-intervention. The Doctor said he had only done this one other time. Both of our sets of parents asked the doctor what he would do if it was his kid. Dr. Petterson said he would think very strongly about not intervening.

We initially thought we would consult with our parents and then make the final decision on our own. After talking with them and hearing what the Doctor had to say, however, we all came to the same conclusion: we were going to take Ben home.

After making this decision, things moved very fast. We met with Hospice the next day. It was explained to us what we could expect to see in Ben. Dr. Petterson let us know that it was impossible to know exactly what would happen, but that within a few days, the fetal duct in the heart should close, and after that we would start to see signs of cardiac failure. We made arrangements with hospice for what would happen in the event of Ben’s death, and talked about what would happen at home. The next day we brought him home.

After the first two or three days at home, we did see changes in Ben. His breathing is definitely more labored, and he occasionally turns blue. He always has kind of a bluish tint around his nose and eyes, and after he eats, around his nose and mouth. This blueness varies from unnoticeable to very pronounced. He also has variations in his breathing. Sometimes his breathing is very comfortable, other times it can be very fast and shallow, or very very labored and deep. We never know what will happen. Also, since Ben’s circulation is not completely normal, his hands and feet will turn purple if he is not kept very very warm; we keep him wrapped in a blanket very often.

We have had over a month with Ben, and each new day is amazing. We feel that he IS a miracle and we HAVE experienced a miracle, just because he is still here. We were told initially that we would have maybe six days. A month is a miracle. Even though we don’t know what will happen next, we know that Ben is special, and everything we have been through has been worth it for him.

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15 Responses to “Read all about it”

  1. dc_chick06 Says:

    We’re all very glad that Ben is still here! I’ve got to agree, he is living proof of a miracle. I look forward to the updates, and hope that all to come are good ones!

    Brandy Van Camp

  2. beautiful_lady Says:

    Wow. Just…wow.
    I’m a friend of Karen’s from high school and I always scan her LJ to find out new news on her nephew. She speaks with such tremendous love for him that it’s hard not to want to know what is going on with this child.
    I commend you both on being such strong people. After reading the full experience, it’s easy to see that none of this has been easy. But you have chosen to remain optimistic. That’s very admirable and if anything, I’ll take your attitude towards such a difficult thing in life away from this story.
    Thank you so much for sharing this…it truly is a story to be told, even if I don’t know who you are.
    Stay strong.

  3. subtlehero Says:

    Amazing story

    My name is Stefanie and I found the link to this journal through Karen, who I only know through some friends and have never even met in person.

    I wanted to tell you that this is a truly amazing story. I think the both of you, all of your parents, and definitely your son are all just spectacular people. This story is completely touching, and I really think you did the right thing(though I’m sure you don’t have any doubts).

    I wish you the best of luck, and you’ll be in my prayers.

    P.S. I come from a strong science background as well, and thoroughly enjoyed your explanation of the conditions… I don’t think most people could have made it that easy to understand.

  4. Anonymous Says:

    You are incredible

    Scott & Kym,
    Thank you for sharing your journey with us. Thank you for sharing Ben with us, too. Although I am not able to see him in person, I feel like I’m there with you. I am enjoying getting to know Ben and rejoice in his progress. I am glad your families are there for you in what has to be the toughest time you’ve ever gone through. Please know that I am here for you, too. What you are doing by sharing your story is such a loving act. Ben is touching people’s lives in ways that folks who live to be 100 years may never.
    I think of you often and send good thoughts your way.
    Sincerely,
    Heather Wade

  5. Anonymous Says:

    Thank you so much for sharing Ben’s history. I have such deep respect for you both, and your families, for making an incredibly difficult decision with Ben’s best interests in mind. Our culture is often so quick to move toward intervention–we just want to DO something–it is very challenging to let life unfold. While Ben is truly a gift for you, you are also a miraculous gift for him. Imagine! Love flowing his way constantly, every day. A miracle, indeed. Warm regards for you all–Nancy Quay

  6. Anonymous Says:

    Mrs. Randall you are amazing.

    Mrs. Randall, you are my hero. You are my role model, you are amazing. you have been so strong through all of this and i am proud that i even know you. i look up to you. you are the most amazing person i know. i dont know anybody who could be as strong as you. i love you mrs. randall.

  7. zoobily_zoo Says:

    I just cannot wrap my mind around it. Being a parent, it is inconceivable. I knew when Tristan was in special care that he just had to get over a small hump and that there would not be a lasting effect. But I still came unglued each time I had to leave him. This is why I just can’t understand how your attitude is possible. I know that you’ve heard it numerous times, but it truly is admirable. I will also echo an above comment in saying that you are not the only lucky ones, Ben is also incredibly blessed. You have given Ben a chance that a lot of couples wouldn’t have considered. Bless your hearts.

  8. nivahunter Says:

    Mrs Randall- You’ve had a hard go with Ben so far, and probably an even tougher one to come. From what I hear through you and through Mr. Mantas, you’re doing the same as Ben is-Awesome. Don’t forget that we’re (the students) are praying for you and that we wish you guys well.
    -Virginia S.

    • Anonymous Says:

      Amazing

      Thanks so much for keeping us all “up to speed” on Ben. He (and ya’ll) have been in our prayers daily here in Texas. Ben is lucky to have such great parents. Keep up the great work!

      matt bell

  9. Anonymous Says:

    I haven’t posted anything yet because I was seeing you three times a week! I miss all of you, and hope that this weekend I can come visit. With conferences, this week has been crazy, but I will try to call tomorrow (Thurs)
    Love to all… Kim

  10. milanase24 Says:

    la vita bella

    “life is beatiful” hi im karens friend from east lansing and she told me about little benjamin and i dont know if you know it or not i had my mom put his name on the praying list in vatican city and they prayed for him last sunday i read the story of ben and i was really touched thank you for sharing the story with us i wish your family and ben the best of luck and health for ben i hope he gets better and give ben a hug for me

  11. Anonymous Says:

    Ben

    I’m glad that this miracle can not only be witnessed by you, but by all of us who care about your family. You are so kind to share these private thoughts with us.

    Love and Prayers,
    Georgeann

    • Anonymous Says:

      Re: Ben

      Dear Scott and Kym,
      What an amazing story!! We are all praying so hard for you and especially for Ben! We’ll keep reading the web site for updates but know that we are always thinking of you and praying for you. Love, Jim and Ruth Brueck

  12. Anonymous Says:

    Ben

    I am so touched by your strength. I was just reading a blog my mother wrote on this web site and I decided to type in Truncus Arteriosis just to see what I would find. Well, I found your fabulous story and your sweet baby, Ben. I have a 5 1/2 year old daughter who has had 3 open heart surgeries to repair her Truncus Arteriosis. Just reading your story was somewhat similiar to some of the problems that Isabel had. She is doing very well. She just started Kindergarten this fall and one would never know she went through so much. Keep up all of your wonderful mothering and love to Ben that is just what he needs.
    God is looking after the rest.
    Blessings to your family,
    Jill

    • coffeemomma Says:

      Re: Ben

      I would love to talk to you more! If you read this and are interested, please leave me your Email (I’ll leave it screened so it won’t be available). Thank you for your comment!!!

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