The Details of Judah's Condition and Care

Since Charity Air Ambulance posted Judah's story, we've gotten a lot of messages, especially from other parents of Potter's Sequence children, asking how Judah is being cared for. I thought I would post something then that explains it in detail so we can hopefully answer all the questions.

Firstly, Judah's condition is called Potter's Sequence, previously called Potter's Syndrome, with the specific type being bilateral renal agenesis (BRA), or the absence of kidneys. The building blocks for kidneys are developed in the first 28 days of gestation and begin working during the 14th week and take over the production of amniotic fluid from the placenta. This is where Potter's turns deadly. Without the amniotic fluid, the babies suffer deformities and their lungs fail to develop, making it impossible for them properly breathe and oxygenate their bodies once the umbilical cord is cut. They die of respiratory failure before they die of kidney failure. Potter's Sequence can come in various different forms, including having kidneys but the kidneys aren't functional for one reason or another. Potter's occurs in 1 in 4000 births and is usually 100% lethal. Up until 3 years ago, there were no survivors. Then one set of courageous parents decided to try and do something about it and set in motion the treatment that led to our Judah surviving so far.

The first step in Judah's care was the placement of an amnioport in my abdomen to deliver normal saline into the amniotic sac three times a week to help him grow and develop his lungs. We had to travel to Cincinnati for this because, to my knowledge, only three hospitals in the U.S. will do amnioinfusions willingly without being pushed. Because they puncture the sac, I leaked fluid throughout my entire pregnancy following the surgery, had contractions if I got out of bed for more than using the bathroom or going to appointments, and went into premature labor twice. His prenatal care, in relation to his postnatal care, was relatively simple. He was born at 33 weeks and 3 days gestation after my water broke and we could no longer do infusions or stop labor.

Judah being intubated right after birth

When Judah was born he didn't cry but he did try to breathe. They intubated him when he was five minutes old and managed to get him stable enough to take him from the operating room (he was a C-section) to the NICU. Following his birth he was given a chest tube for a pneumothorax and then transferred to Cincinnati Children's Hospital (he was born at a different hospital since CCH currently doesn't have a labor and delivery floor). His blood pressure was extremely low when he was born and the doctors said they "threw everything and the kitchen sink through his UVC line" to get his blood pressure up. Once they considered him a pulmonary survivor (at three days old), they did surgery at his bedside (because he was too unstable to move to the OR) to place a hemodialysis (HD) catheter in his neck and a peritoneal dialysis (PD) catheter in his abdomen. They tried a gentler form of hemodialysis called aquapheresis first but his blood pressure immediately crashed and they had to take him off of it. They waited another day or so and then started him on PD. This gave him enough time to come down off his blood pressure medications (he had been maxed out) enough that when his PD catheter ultimately became ineffective because of leaking, they were able to do aquapheresis again and give him good enough nutrition to heal his PD surgery site.

Judah's problem list at birth included bilateral renal agenesis, small lungs, low blood pressure, pulmonary hypertension (high blood pressure only in the lungs), two hernias (common in preemies), and a pneumothorax. He was on dialysis, nitric oxide therapy (pulmonary hypertension), flolan (pulmonary hypertension), epinephrine and vasopressin (low blood pressure), a ventilator, a chest tube, and TPN (total parenteral nutrition - basically IV nutrition) and lipids for his food. He was extubated at 2 1/2 months and off all the IV meds except the nutrition over that period of time as well.

Judah on his due date

He remained on PD for around 3 1/2 months until his PD catheter became clogged and they had to do surgery to fix it and then surgery again 2 days later when it stopped working again and they had to replace it with a bigger one. Following those two surgeries they put him on hemodialysis for 3 days (surgery recommended 2 weeks) and he didn't tolerate it well so we had to go back to PD. Just before Christmas he was transferred to Texas Children's Hospital which was back home for us. Following his transfer home, he developed a hernia at one of the surgery sites and began leaking PD fluid. He was then put back on hemodialysis for 2 weeks until he developed peritonitis (assumed - we never found anything on the cultures) at which point his blood pressure got so low that he had to be put back on medication for it - this time dopamine and epinephrine. He also had to be re-intubated because he was breathing too fast and hard and they were worried he would wear himself out. They put him on another form of dialysis called continual renal replacement therapy (CRRT) which is a gentler form of hemodialysis that runs 24/7. He is staying on that currently until they can get him back off the blood pressure medications and get him more stable. The issue with CRRT is it can be very dangerous to start them on the machine (example - Judah coded by dropping his heart rate the 3rd time they tried to start him on it) but once they are on it is very safe and gentle.

Judah's first Iron Bowl (We shout War Eagle)

Judah's current problem list is bilateral renal agenesis, small lungs, low and high blood pressure, peritonitis, respiratory failure, and resistance to sedation. He is on CRRT, dopamine and hydrocortisol (low blood pressure), fentanyl and versed (sedation and pain management), antibiotics, antivirals, and antifungals (covering their infection bases), TPN and lipids, a ventilator, saline mixed with heparin to keep one of his PICC lines open, and a warmer (CRRT drops the body temperature). Before getting sick, he was only on TPN, lipids, fortified breast milk (for more calories), hemodialysis, and 7L high flow nasal cannula.

His ultimate goal is to get him onto a dialysis cycler that he can go home on. He will then have to be pretty isolated (renal babies are extremely susceptible to infection) until he reaches 20lbs (around 2 years of age - it's hard for renal babies to gain weight because of fluid restriction) at which point he can receive a kidney transplant. He will then be on immunosuppressants for the rest of his life to help keep him from rejecting the kidney and will need another transplant in 15-20 years under current technology.

How It Works: Peritoneal Dialysis

There has also been some confusion as to how it is exactly that peritoneal dialysis works. Without getting too technical it's basically the following:
A catheter is surgically inserted into the peritoneal cavity, a spot between your abdominal wall and your organs that contains many, many blood vessels. Once the site has been allowed to heal (which isn't usually the case with babies) for 1-2 weeks per the surgeon's recommendation, it can be used for dialysis. With this type of dialysis no blood has to leave the body so there is less risk for infection (of the blood at least) and it is less draining and stressful to the body. PD runs in cycles - fill, dwell, and drain. I am not aware how long it takes to dwell in adults, but with Judah it would be 30-45 minutes to dwell with 10 minutes to drain and typically 2-5 minutes to fill. The fluid used for PD is a special type of sugar and salt water specially prescribed by the nephrologists based on the electrolyte and fluid balance needs of the patient. The peritoneal cavity is then filled with dialysate using an amount prescribed. Judah started at 20mL because of how tiny he was and we had worked our way up to 50mL before his most recent leaking. The goal for him is 150mL before he can go on a cycler, move from the NICU to the renal floor and from there work on going home. Once the cavity is filled, it dwells for 30-45 minutes, depending on how many cycles you want to get in per day. Since the fluid put in has no toxins in it and the blood does, the toxins and extra electrolytes and fluid are pulled into the peritoneal cavity through diffusion (toxins) and ultrafiltration (water). When the cycle is up, the stopcock on the tubing is turned to drain and the fluid drains out over 10 minutes. You want to get out the same amount or more than you put in and output is carefully measured. The cycle is then repeated. This is done manually by a nurse 24/7 until a high enough volume is reached for the cycler to work at which point dialysis can be done over 12 hours and the patient can live a relatively normal life during the other 12 hours.

Hopefully this helps explain things for anyone who is curious and helps anyone who may be looking for help for their recent diagnosis of Potter's in their baby. This post will become updated with his treatment as time goes on.


Judah passed away at 11:04am on February 25, 2017 after taking a sudden turn for the worst. We're still unsure what caused him to get so sick so quickly but we know he is healed and happy in Jesus' arms and we are looking forward to the day that we see him again.

Behind Our Baby's Name

I've had a lot of people ask me why it is we chose our son, Judah's, name, and also why we gave him such a long name. Before we knew he was going to have problems, we had such a hard time picking a name for him. I was really hoping for a girl for two reasons - 1) I grew up with only little sisters and didn't have a lot of experience with boys and 2) I had the perfect name picked out for a little girl that I was absolutely in love with. When we found out we were having a boy it started a long running disagreement between me and my husband about what name to give our baby. We went through literally hundreds of them. I wanted something softer like Liam or Oliver and he wanted something strong and manly (insert tons of unpronounceable Scottish names here). While we were visiting my husband's family in Georgia when I was around 18 weeks pregnant, his sisters were reading off names out of a baby book that his mom had used before he was born and two names finally gave me pause - Asher and Judah. Asher was more of a middle name to my husband so Judah became our frontrunner. However, a couple of weeks later, we were given the bad news that something was wrong with our son and naming him went on the back burner and we just kept calling him by his nickname that we had been using for him from the beginning - Baby Bean.

Fast forward 13 weeks and we were told on an August morning that they could no longer stop my labor and we were having a baby that day. We had four names that we loved but hadn't narrowed down to two so when I asked my husband what were going to name our baby he said without hesitation, "Judah Nathaniel Oliver Beorn." And it just felt right. The nurse came in a few minutes later and said she was trying to make name bands for the baby and asked his name. Again, without hesitation, we answered, "Judah." And each of his names has a special meaning behind it.

Judah
In Genesis 29, Jacob marries two wives - Leah, after being tricked into it by her father, and Rachel whom he actually loved. We are told that God sees that Leah is unloved and takes pity on her by giving her sons while her sister remained childless. With her first three sons, Leah gives them names that reflect how God has given her children and not her sister. But when she gives birth to her fourth son she says, "This time I will praise the Lord." And she names him Judah, which means "praise." Even though I just liked the sound of the name Judah, this verse was what solidified it for me. Judah is actually our second child, with us having lost the first to a miscarriage at 8 weeks around three years ago. After my miscarriage I was very angry with God for taking my child away from me and for my life seemingly falling about afterwards. It took me a long time to lose that anger and even longer to get back to a point where I could trust God. So when I found out I was pregnant again I decided I was going to praise God for a second gift no matter what the outcome and while that has been so hard throughout this journey we've gone on so far, I just have to think about what Judah means and I am reminded of how good God has been to us so far.

Nathaniel
This is a name that is Greek but comes from a Hebrew word meaning "God has given." After I had my miscarriage I almost immediately wanted to try and have another child to try and heal my heart as well as "replace" what was lost (which is not the right thing to do when dealing with grief). However months and then years passed without us being able to have a child. We went to see a fertility specialist after two years of no luck. They weren't able to find anything that was obviously wrong so they wrote up a plan for us to start Clomid, a drug to help. We decided to wait a few months before starting, mostly because I was in nursing school and we wanted to make sure I would graduate before having a baby so we wouldn't have any conflicts but also because it wouldn't be covered by insurance and would cost $500 for every round we had to do so we wanted some time to save up for that specifically. However, two months before we were going to start, I found out I was pregnant all on our own and completely by accident. After 898 days, God had finally answered our prayers for a child so the name seemed appropriate to celebrate our journey to him and all the emotional ups and downs we had gone through to get there.

Oliver

This is a French baby name meaning "the olive tree." Of course because it's French my husband thought it was too girly and soft of a name. He also didn't want his son to have the same name as, according to him, "the grumpy Green Arrow." However, despite the fact that Oliver Queen is often brooding and going back and forth on his morals and direction in life, he is resilient and no matter how bad things get he continues to fight and keep moving forward no matter the situation which is something our baby desperately needs, especially during his harrowing first few weeks of life. In the Bible the olive tree also signifies fruitfulness and beauty, which to me is significant because of our infertility issues but also because a lot of times I need to be reminded of the beauty in the everyday things that come with having a baby in the NICU for an extended period of time.

Beorn
I remember a particular date five and half years ago when my husband and I were dating. We were in a long-distance relationship and I had traveled to Auburn, AL to attend my husband's graduation from college. We had gotten milkshakes from Chick-fil-A and were going for a walk in a nearby park and just talking about the future which of course, for both us, included children. My husband, half joking and half serious, mentioned how he wanted to name his children after Lord of the Rings characters. This mortified me, however, because of how, despite my mom's best efforts to give us names that no one could make fun of, I was still bullied in middle school for other things about my personality and lifestyle. This left a lasting mark for me and made me not want to make my children easy targets for other kids, which I felt one of those names could do. After a discussion about it, we came to our first compromise (that I can at least remember) of our relationship - I would have the final say on first names and he would have the final say on middle names. Because of this we decided we wanted our first boy's middle name to be Beorn, a character in J.R.R. Tolkien's The Hobbit who could turn into a bear. Beorn is also an Old English word for "a warrior, a hero, a man of valour" which, unknown to us, would be something our son would need to be to make it through life. And, from this middle name, also came my nickname for him, Judah Bear.

Overall we felt he needed some strong names to get him through what promised to be, if he survived birth, a harder life than any person should have to have. We also wanted it to reflect, however, God's love and promises and overall, his miracles, as our sweet boy is definitely one (only the fourth baby with his condition to survive and the first boy). Even though his name is long and kind of a mouthful, we believe it is the name he was meant to have and also has helped remind us that God requires our praise no matter the situation as well as his grace and
provision in this situation.

It takes a literal village to raise a NICU child and Judah has been no different with all his nurses and doctors and all the people who have prayed for him and sent him gifts and well wishes. We would not be where we are today without our village and ask that you continue to support him as we face our day-to-day challenges of not only keeping him alive but helping him to thrive.

How We Got Here: Baby Morgan's Story

As I changed into my scrubs that morning, I figured my life wouldn’t be any different. I would back up my backpack, head on campus, sit through a med-surg lecture, and then head to the campus library to study for the afternoon. But then my mind went back to that test sitting on my bathroom sink and my heart started pounding again. I wasn’t expecting it to be positive – for close to three years they had all been negative, leading us to head to doctor after doctor without much luck. I walked into the bathroom and picked up the test. Two lines. I couldn’t believe it. My husband and I, it seemed, were finally going to get the baby we had been praying for.

Not more than a week later, I was headed to the ER, crying and praying, hoping that I wasn’t having a second miscarriage. For the past several weeks I had been tired and nauseous and then that morning I had woken up with severe cramping and spotting. So instead of going to class that morning, I called my husband, who was on his way to work, and went on my way. At an ultrasound a few hours later, I saw my little tiny baby on the screen and heard his wonderful little heartbeat for the first time. Relief flooded over me. Later in the day we were told I had a subchorionic hematoma which was surrounding the baby, most likely from the baby suddenly becoming jolted and tearing away from the lining of the uterus. I was put on modified bed rest and told to follow up with my OB/GYN in a few days. When the OB confirmed it at my appointment the next week, I knew I would have to drop out of nursing school, but I didn’t care – I was more concerned with having a healthy baby. Unfortunately, I had no idea that that could never be the case.

A month passed and the hematoma cleared up, getting me off of bed rest. During that time my nausea had gotten worse and worse, leading to hyperemesis gravidarum, or severe morning sickness. At this point in my pregnancy I had now ended up with two conditions that only occur in 1-2% of pregnancies but the baby’s heartbeat was strong and each wave of nausea and bout of vomiting just made me even more reassured that my baby was okay and growing stronger and stronger. Another month passed and we held a gender reveal party that revealed we were having a boy. This wasn’t the outcome I was totally hoping for but mostly because I had grown up with younger sisters and didn’t have much experience with baby boys. But after the initial shock wore off, I got excited. I was in my second trimester and passed the threat of miscarriage and I felt like I could finally start preparing for our little guy’s arrival. We started buying baby clothes, including an Auburn University jersey when we visited my sister and her daughter in Auburn, AL (my husband’s alma mater). I started to feel him kick and bump around, with his favorite activity being to punch my bladder.

After visiting my sister, we drove a couple hours away to visit Brendan’s parents in Georgia. While we were there I got a call from my OB/GYN saying one of my lab tests was abnormal. The baby had been tested for spinal bifida via a blood test a few days before we left and it had come back with high alpha fetal protein, giving him a 1/110 chance of having a spinal cord defect. We set up an appointment with a maternal fetal specialist for the next week and my months of anxiety (unknown to me) began. We finally made it our 20 week ultrasound with the specialist. For those who don’t know, the 20 week ultrasound is much more in depth than other ultrasounds done previously to check on all the baby’s systems to make sure they are developing correctly. We sat with a genetic counselor first who asked us about our family histories and talked to us about how the high AFPs was most likely due to the bleed at the beginning of my pregnancy and, if not, we only had less than 1% of a chance that there was anything actually wrong with him.

As the ultrasound tech looked at our baby, I relaxed and just enjoyed seeing my baby. I didn’t suspect a thing. Then the doctor came in and told us she was very concerned about our baby. My heart started racing again. She said the baby had almost no amniotic fluid and she thought the baby didn’t have any kidneys. Then she found something that looked like a kidney and two renal arteries so she thought the problem most likely lay with the placenta but the lack of fluid made it hard to tell. She put me back on modified bedrest and scheduled an appointment for two weeks later, hoping that there would be more fluid and they could get better visualization to see what was going on.

Baby Morgan after his first fluid injection.
His facial features are flattened - a common indicator of Potter's

So began another two weeks of anxiety and desperate prayer on the part of my husband and me. When we got to our second appointment, my anxiety was through the roof. I couldn’t sit still and I felt constantly on the verge of tears. When the tech began his examination, all the hope I had been holding onto for the past two weeks disappeared. There was still no fluid and the baby was very compressed and hardly moving. When the doctor came in, she said as far as she could tell there was nothing wrong with the placenta and they couldn’t find any kidneys or renal arteries. She recommended that we prepare ourselves to lose our baby. I couldn’t believe it. After almost three years of prayer and believing we couldn’t have children on our own and being only a few months away from starting fertility treatment, we had gotten pregnant on our own. Now they were telling us that the pregnancy had been doomed from almost the very beginning and we hadn’t had any idea. I felt foolish for getting my hopes and buying baby clothes and a diaper bag. My pregnancy was only half over and it now felt like it had ended abruptly. The baby was still alive and I could feel him moving around but even that felt like a cruel reminder that I could never bring him home. Instead I would once again be childless, and the majority of the world would have no idea that I was a mother two times over because there would be no baby in my arms.

Later in the day, a close friend of mine, after hearing the news, sent me an article about the first baby to survive with no kidneys thanks to a couple of parents who were unwilling to give up on her and the willingness of several doctors to try some risky, experimental procedures in an attempt to save the little girl’s life. So we began to look into it. Johns Hopkins and the doctor who had worked with the first baby, got back to us and told us that they were no longer doing to the procedure. We had also talked to our doctor who gave us the name of another doctor who suggested we try Cincinnati Children’s Hospital, Stanford, and Colorado Fetal Center.  Cincinnati is actively running studies on amnioinfusion and babies with kidney abnormalities and told us they would try and set us up with appointments for an echocardiogram, an ultrasound, and an MRI to see if we were eligible for the treatment. Stanford told us they were working with Texas Children’s Hospital to get board approval to do amnioinfusion to begin their own version of the study and they would contact Texas Children’s to see if they would help. They said they would at least run the tests but had no guarantee that they could do the amnioinfusion.

Either way, if there wasn’t too much damage to the baby, they would then surgically implant an amnioport in my abdomen to deliver normal saline or lactated ringers (an IV solution) into the uterus once to three times a week to try and stimulate the baby’s lung development and keep them from developing too many skeletal and muscular deformities related to the lack of room babies with this condition, called Potter’s Sequence (specifically, in our case, bilateral renal agenesis), usually develop. Without the lung function, no care after birth is possible, since, of course, they can’t breathe. Most Potter babies die from respiratory failure within hours, if they survive birth at all. The goal then would be for the baby to remain in the womb until full term at which point they would deliver the baby and, assuming he had adequate lung function, start him on dialysis. This would require him to stay in the hospital after birth for at least six months to stabilize him on dialysis at which point he could finally come home and have nightly dialysis for the next year and a half, or until he reached a certain weight, at which point he could then receive a kidney transplant. Then, depending on the condition of his bladder, he would need a ureterostomy (where the ureters are attached to the abdominal wall and drain into a bag on the outside of the stomach) until he could have reconstructive surgery on his bladder at age 5.

Baby Morgan after 3 weeks of injections. He's happily
settled with his feet and hands covering his face.

Texas Children’s wasn’t able to get the approval because the board didn’t believe they had the resources to take care of a baby with no bladder. So, very much at the last minute, we bought plane tickets and flew to Cincinnati to begin the testing on the baby. We then spent the next few days getting an electrocardiogram, an ultrasound, an MRI and meeting with the many doctors it would take to care for our baby. Every other system checked out fine, with the exception of a slightly enlarged heart and a small cyst on his lung, both of which the doctors said weren’t really of any concern. After a lot of back and forth on our decision, we decided to go through with the surgery, which took place the next week. I then spent about a week and a half in the hospital in recovery and am now being seen three times a week on an outpatient basis.

We realize this is a long shot. We realize there’s not much hope for our son. But we are refusing to give up. We are believing that God can work a miracle with our little boy and bring him into the world where he will survive and flourish.