Meryck

Prune Belly Syndrome Network, Inc.

~ Meryck ~
as written by Mom Stefanie

I have 3 children, ages 15, 5 and 3. My oldest son (Tim) came along the day before hurricane Andrew hit in 1992. Tim and I lived in Miami with my mother and my grandparents. I spent my first night home with him in the hallway waiting for the hurricane to pass through. We went through some rough times after that, but he was a healthy child.

About 9 years later, I met my husband. Before we even got married, we found out that I needed surgery to remove a large portion of my ovaries. After the surgery I was told it would be a miracle if I had any more children. 1 ½ years later, I had Kyle (now 5) who was also a healthy child. I called him my “miracle baby.” We, as a family went through some hard times and my husband and I divorced.

In 2003, I got pregnant with my 3^rd son, Meryck. I was even more shocked that I conceived again. I remember when he was born -- looking into his eyes and seeing an “old soul.” The day after his birth, I had my tubes tied and he had a circumcision. When he came back into my room, he was crying in pain, but at the time, I couldn’t figure out why. When we were getting discharged, the pediatrician checked him out and told me that Meryck had a broken right clavicle and a right undescended testicle (a symptom of PBS). I had already noticed the testicle.

This brings me to my story of my son’s diagnosis of Prune Belly Syndrome (PBS). I noticed immediately after he was born that his stomach was very large and wide while lying down and wider when he sat up (sort of like a frog’s stomach or “pot belly” appearance). I asked his pediatrician about it and was initially told that he had a “floppy bladder.” The pediatrician also told me to wait about a year to see if his testicle would descend on its own. That was when I started doing a lot of research on the internet -- all signs pointed to Prune Belly Syndrome.

Since Meryck had a broken right clavicle, the pediatrician sent us to an orthopedic doctor who in turn, referred us to physical therapy because Meryck’s head was flattening since he favored facing left when lying down. Meryck began physical therapy for his neck at about 4 weeks old and was in daycare at 6 weeks old because I had to work, especially since I was a single parent of three children at that time. As physical therapy went on and Meryck got older, the daycare teachers and I noticed that he didn’t use his stomach muscles well (another sign of PBS). Instead of crawling, he would use his arms to pull himself around on his belly. The therapy continued for his neck but began to included working on his stomach muscles as well. He didn’t start walking until he was 1 ½ years old (which I thought was odd because my other 2 children walked at 10 and 11 months).

After about a year I decided to have his testicle descended and saw a urologist around November 2005. At our first appointment, I questioned the urologist whether Meryck could have PBS and again was told no and don’t worry about it. Meryck had his right testicle descended in April 2006. While the urologist was doing the descencion, he found an inguinal hernia, repaired that and also did a meatomy (made his pee hole bigger).

Between February 2005 and September 2006, I had gone through several pediatricians for various reasons. Then, in September 2006, Meryck had blood in his stool so I took him to the pediatrician I had at that time and they sent him for an ultrasound of his lower abdomen. When the ultrasound was done, it showed that his right kidney was severely damaged, Meryck had grade IV-V bilateral hydronephrosis (yet another sign of PBS), a large ureterocele (bubble) extending from the ureter attached to his “bad” right kidney, and a “grossly enlarged” bladder.

With this new information, I immediately brought it to the urologist that had descended Meryck’s testicle. More tests were ordered by the urologist and I brought the results of those tests to the urologist to try to determine a plan of action (mind you, I had also approached him about PBS, but it fell on deaf ears). At that appointment, the urologist explained how the urinary system worked (which I already knew) and said that he didn’t think that Meryck’s right kidney was functioning as much as the test said. The urologist didn’t look at many of the films I brought him and told me he would look at them later and come up with some type of plan for Meryck. It made me feel like Meryck’s health was not very important. As I was leaving the office, I told the nurse I wanted the films back and I was advised that the doctor usually didn’t give them back. Since I was so upset, I told her that he could keep them to see where he went wrong. Not to my surprise, I got a call on my cell phone about 15 minutes after leaving the office from the doctor and he was talking something about reconstructing Meryck’s kidney (which I knew wasn’t possible) and I just sat there listening and knowing that I wouldn’t go back to him again.

September 20, 2006, I drove Meryck to the Shand’s Hospital Emergency Room in Gainesville (2 hours away from our home). We ended up there for five days. Since he had been catheterized 3 different times within 4 days from the tests the urologist had ordered, it had caused a severe kidney infection called pylonephritis. The hospital referred me to a nephrologist in Gainesville where we went as soon as Meryck was discharged. When we saw the nephrologist, I also asked him whether Meryck could have PBS and was again told no. This nephrologist referred me to a different urologist in Jacksonville. Unfortunately, he was in the same group as my prior urologist.

I made appointments with this new urologist and also asked him whether Meryck could have PBS and was told no again (surprise!). The doctor scheduled surgery for Meryck in the middle of October 2006 to have his right kidney removed and the ureterocele incised. Prior to his right kidney being removed, Meryck’s left kidney had very minimal damage to it. Once this urologist removed his right kidney, Meryck’s left kidney got progressively worse and quickly. The only plan this urologist had was to monitor the progression of the damage to Meryck’s solitary kidney. At our last visit to this urologist, the doctor had sent in an intern to talk to me. The urologist didn’t even come in to see Meryck. I was very upset and let his nurse know and asked for all my films back and left. That was the last time we went to that urologist’s office.

In February 2007, I re-married my husband who had, since our divorce, joined the Air Force. In March of this year, I made appointments with the base doctors who had ultrasounds run immediately. They gave me a referral to have Meryck seen by a urologist immediately. The case manager that does the referrals sent me to several urologists in the area, but none would touch Meryck’s case. I was told his medical problem was too extensive for their practice (even though they were pediatric urologists). I was eventually referred to the urologist group who are his current doctors. Their office is 3 hours away from our home, on the other coast. At our first appointment, I asked whether this urologist thought Meryck had PBS and for the first time, the idea wasn’t dismissed. He referred me to a nephrologist who was also in Tampa. We made an appointment with her immediately. When she walked into the room, I asked if Meryck could have PBS. She took one look at the baby pictures I had shown to so many of the previous doctors, the films and another look at his belly, and said that he absolutely had mild variant Prune Belly Syndrome. I was so happy and so sad at the same time. Finally, a doctor that listened to me and cared about my son after fighting for 3 years! She even brought in one of her colleagues to look at the films, Meryck’s belly and the baby pictures to re-confirm her diagnosis. We had a diagnosis!

Because of Meryck’s PBS, his bladder is grossly enlarged. For instance, June 7, 2007, he had surgery for a re-incision of the ureterocele; the next day Meryck stopped peeing. He went 14 hours without voiding, so I rushed him to the Tampa hospital emergency room. When the hospital put a catheter in him, 700 cc’s came out of his bladder. A grown person with that much urine would have been doubled over in pain, where Meryck slept all the way there and did the “pee pee dance” while waiting for them to catheterize him. He was kept overnight for observation and we went home the next day with a catheter in place. June 18, 2007, the catheter came out and more ultrasounds were run. There was still slow deterioration within his single kidney.

On August 3, 2006, we went to our urologist and a catheter was placed in Meryck again. He had it in for 6 weeks until recently when he had surgery for a suprapubic tube insertion, dissection of the ureterocele and fixing of the stump that was left when the right kidney was removed. Hopefully this will take the pressure off his solitary kidney so that no more damage will occur. If this doesn’t work, then Meryck may have to have another surgery where they take his ureter and basically poke it through his stomach so that the urine will completely bypass his bladder and drain directly into a bag taped to his stomach.

Living with Meryck’s PBS isn’t an easy thing. I have to take care of Meryck full time right now. Meryck takes antibiotics daily to try to prevent urinary tract infections, but that doesn’t mean that he doesn’t get them. Meryck also has to take a stool softening medicine daily because constipation can put pressure on his kidney and create more damage. While Meryck has the catheter, I have to sponge bathe him. I have to take special care of the catheter tube. I have to document his fluid intake and output. I keep spreadsheets of his blood work and have every film from every test that has been run on him. I bring them with me to all doctor’s appointments in Tampa which can vary between 2 or more times per month.

Meryck is such a loving child. He has a zest for life and even through the hardest times, seemed to manage to have a smile on his face. When he sees people, he tells them he has a tube in his belly and it helps him pee. He is always so brave and strong, sometimes I wish I had his strength. He loves to play, spend time with his brothers and is content to just sit on the couch watching TV with Mom or Dad. He has taught me not to take little things for granted and has given me comfort without even knowing it.