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.