“I have told you this so that you might have peace in Me. In the world you will have trouble, but take courage, I have conquered the world.” John 16:33
Our beautiful daughter, Remi Elaine Goodall, was born February 2, 2006 with Autosomal Recessive Polycystic Kidney Disease (ARPKD) and Congenital Hepatic Fibrosis (CHF). While pregnant with our second child, we didn’t notice anything different and were preparing for the new addition like any other family. All appeared fine until an appointment around 32-34 weeks gestation, when the doctor said I was “measuring small.” After a few ultrasounds and appointments with specialists, it was found that our baby had enlarged, non-functioning kidneys. We met with a high risk OBGYN who advised that Remi’s outcome was bleak. After taking an ultrasound, he talked to us about Polycystic Kidney Disease, in particular he told us of a rare genetic form of the disease called “ARPKD” or autosomal recessive polycystic kidney disease. After about ten minutes into our consultation, we realized what he was trying to tell us. He forwarded his findings to the Nephrology Department at Children’s Hospital (Columbus, OH). Meanwhile, like any other parents, we went home and mourned what we thought was the loss of our child. In the next week, we met with the Chief of Pediatric Nephrology at Children’s hospital and were told that there was hope. Considering we are only dealing with one diagnosis, ARPKD, she might be a candidate for peritoneal dialysis for a year or two and then could receive a kidney transplant. Though ARPKD is a rare thing, there are options and treatments that can work. Babies like Remi have a 10% chance of making it past birth and then a 50% chance to make it to transplant. Typically the 50% is due to “overwhelming” infection. Both Remi and I were monitored closely those final weeks of pregnancy, as I had little to no amniotic fluid. I was visiting the OBGYN every two days for non-stress tests to see how she was doing. On Feb. 2,2006 Remi Elaine was born at 12:30 am – I was to be induced the next day, however, she decided to come of her own free will. It was as a true blessing from God that Remi had some lung function at birth, as most infants diagnosed prenatally have minimal lung growth due to large kidneys taking up the room in their abdomen and the lack of amniotic fluid during the pregnancy. Remi was baptized & received anointing of the sick when she was around 4 or 5 days old. Her kidneys were extremely large, (the same size as mine), and at 7 days of age she underwent a double nephrectomy – the removal of both kidneys. A peritoneal dialysis catheter was also placed in her belly for her to begin dialysis treatments that started immediately because she had bad BUN and creatinine clearance levels. Remi was intubated (put on the breathing machine) immediately after birth and remained on the “vent” for about 4 weeks. She came off of the vent and went to “room air” for one day. That night one of the lower lobes in her lungs began to collapse so they put her on a C-PAP machine for a bit and then onto the canula to assist with her breathing. By a month and a half of age Remi was breathing on her own with no assistance! Multiple testes were run and they said she seemed to only have ARPKD & CHF, no other syndromes or birth defects that are sometimes found in these children. After a couple weeks on the peritoneal dialysis Remi developed a leak around the peritoneal dialysis catheter in her abdomen. The dialysis fluid was coming out from around the catheter and this isn’t good because it is an area where infection can be introduced (main concern) and that dialysis treatments wouldn’t be effective. Remi had her first bout of peritonitis (infection in the peritoneal cavity) within the first month. After vigorous antibiotic treatment and anti-fungal treatments she had negative cultures and overcame that infection. About the same time the doctors began feeding Remi some formula. It was discovered that she had reflux that wasn’t allowing her to properly hold down or digest the right amount of food. Children with ARPKD have a hard time growing because the body doesn’t know what to do with the growth hormones put out by the pituitary gland. Typically children with ARPKD are smaller than others and developmentally behind until about three months after their kidney transplants. So for all of these reasons the feedings were a very important part of her growth because she could fall way behind where we needed her to be in order to receive a kidney transplant. We found that her feedings via bottle weren’t going so well so they started feeding her thru a NG tube that is placed down into the upper portion of her intestine. Fairly soon the doctors noticed she had a lot of additional fluid in her abdomen or a “positive output” when they would drain her peritoneal dialysis fluid. Her dialysis “output” became very cloudy (it should be clear like urine) and they thought she had another round of peritonitis. Further testing proved that Remi had something rare thing called chylous ascites (a lymphatic vessel that was leaking into her abdomen). This is sometimes occurs after surgery because the lymphatic vessels are cut during a procedure, however, they generally clear up on their own. The last documented case, we were told, that was similar was in 1995. Everyone seemed confused because, typically, this clears up on it’s own. We had a care conference with the doctors & surgeons to discuss our plan of care. The surgeon said that the first priority was to get the ascities to clear up by medicine or surgically. The second issue was to get her peritoneal dialysis catheter to stop leaking, third was to get a g-tube in (to feed her thru the abdomen) and do a Nissen fundoplication (procedure to wrap the top of her stomach around the esophagus to help eliminate her reflux). The doctors implemented a machine called a “wound vac” that went overtop of the peritoneal dialysis catheter (a sponge like material) and was hooked up to a vacuum-type machine. The purpose is to promote tissue growth, heal a wound over and to keep a sterile area. This worked as far as keeping things somewhat sterile, however the leak was not slowing down and the chylous ascities wasn’t clearing up from the medicine that she had been on. Tests were ordered from the GI unit to see if Remi possibly had a disease (that goes hand in hand with ARPKD) called Caroli’s disease. It is a back of the liver/gall-bladder system and can sometimes cause an ascites. The testing was done and the doctor said that she definitely was “in the category or sub-category” to have Caroli’s disease, however, it’s a difficult diagnosis to make and he didn’t want to do that until she had some of the classic symptoms: liver back-up, fever, gall-bladder back up, etc. He said that they did notice she that she had mild cirrhosis of the liver (which we knew was the CHF – congenital hepatic fibrosis) but otherwise things looked pretty normal with those organs. Typically babies with ARPKD also have CHF – she did have this but it was very mild and, in the beginning, didn’t affect her liver function at all. A couple weeks later it was found that Remi had another case of peritonitis. They immediately put her back on antibiotics and anti-fungal medications. This seemed to clear up the symptoms however she came down with another type of bacteria (while on antibiotics) that was creating a new infection. The doctors said that they thought she just couldn’t shake the peritonitis and that the next step would be surgery to remove the old peritoneal dialysis catheter that was leaking and to put in a hemo dialysis catheter in her neck. This was done so that the peritoneal lining might be saved as sometimes when they have recurring bouts of infection it will ruin the lining on the peritoneum and that type of dialysis will not be an option. They also thought that the bacteria could be “hanging onto” the catheter that was originally placed. It was late April 2006 and she had her second surgery to remove the old cath & place the hemo dialysis catheter. The first time they attempted hemo dialysis all things went pretty well. Her blood pressure didn’t drop significantly (her “normal” blood pressures ran very low on average) and her BUN/creatinine clearance levels were pretty good. They were doing a traditional type of hemo on her at that time. The second time they attempted hemo dialysis with her (two days later) things were a bit different. Her blood pressures were unstable so they put her back on some medication to keep her pressure up. Her clearance levels were looking okay, however, they weren’t able to pull the excess fluid off of her body like they could with peritoneal dialysis. The doctors waited three days and then did another round of hemo dialysis. This time it was completely different. They couldn’t get the machine to run very smooth and then when they did they would have problems with her catheter clotting (they couldn’t pull blood out or put any in). It was decided that we would try a different type of hemo dialysis called CVVH, which worked well with infants and people who had blood pressure problems. They started this mid-afternoon and by 3:00 am my husband came home and they were still having problems getting things to run smoothly for her. At this point she hadn’t been dialyzed in days and they needed to get her levels back on track and the extra fluid off of her body. They had to re-intubate her (put her back on the breathing ventilator) due to excess fluid and sedation as they needed her to be completely still for the CVVH dialysis. The site where the old catheter was had been left open to drain the asctites (fluid in the abdomen) out. She started putting out mass amounts of fluid from her abdomen and they weren’t sure why. The nurses began to collect the amounts in hopes of having it analyzed to see exactly what it was from. The area where the old catheter was had been blocked so the ascities wasn’t draining from that site anymore. Her abdomen began to get bigger and bigger and one of the surgeons said that this might be when the lymphatic vessel will “clot” off because of all the pressure inside. Remi also had a fever, two days in a row, for the first time in her life. Records say they thought she might have been septic however this was never confirmed. Thursday May 4,2006 I went up pretty early as I was expecting some type of hemo dialysis to be run that day. The surgeons came in and told me that she had fluid in her lungs and possibly her heart (she was on heart medication for the first time ever and was on medicine to keep her blood pressure up). They had to “up” the vent because she was having a more difficult time breathing. It was decided that the surgeons were going to go in there & try to find/tie off all lymphatic vessels they could find to try to get this ascites to stop. They were also going to close up the old peritoneal catheter site, place a new pd catheter on the left of her abdomen and try to place/re-position the hemo dialysis catheter that was previously placed on the right of her neck. The theory was that we would again try hemo and allow a new pd catheter time to heal before using it this time so that we wouldn’t have problems with leakage again. After surgery the surgeon came out to talk to us about what they found. He took us into a room off of the main surgery waiting area of Children’s hospital. The surgeon said that she was doing okay, however, they found something very unexpected. Her liver, which had been seen as normal 7 days earlier in surgery, was almost double in size, hard and backed up. Basically it was not functioning at all and this was causing a back up into the ducts, which, in turn, caused a new type of ascites. They said they didn’t know how or why this happened and it wasn’t something they had seen. When this type of failure happens, given her double nephrectomy (removal of both kidneys) and her age, there is really nothing that can be done. The nephrologist called various doctors and hospitals to gather info – he advised that liver transplants in children her with no kidneys were unsuccessful. They said that we could keep her on dialysis, however she would pass away within a few weeks from the liver disease. We decided to not keep her on dialysis and to let her go peacefully. That night we had our priest come up to visit – he also delivered the Last Rites for Remi. We had some friends & family visit and love our little Remi over the next two days. She was still on the vent & not very alert but tried to open her puffy little eyes while family was there. Friday May 5,2006 at 6:30 pm we removed Remi’s breathing support. The neonatologist (baby doctor) gave her a lot of morphine and another medicine (that is a hypnotic) to ensure that she wouldn’t be aware at all. Remi was so peaceful and beautiful. Jim and I held her, turned the lights down low, played lullabies for her & danced with her. At 7:10 pm Remi stopped breathing for about five minutes. We called the doctors in, put her in the crib and began to bath her. At 7:15 she started breathing again so we picked her up and held her the rest of the night. Jim and I both felt very strongly that her spirit had left at 7:10. After that point things were different – can’t really explain why or how but they were. Around 12:30 am on May 6,2006 Remi passed away on daddy’s chest. I can’t even explain the feeling in words. It truly was a connection with her and God that we have never felt in our lives. This act, helping her leave the world in a loving and peaceful way, was more spiritual and amazing than childbirth. It was a connection with her spirit that I can’t explain. For the first time we saw our daughter at peace with no intrusive medical devises – we were able to swaddle her and walk around with her in our arms free from breathing tubes, catheters & IV’s. I wanted to skip up and down the halls of the hospital – I wanted everyone to see what a blessing she was. The relief & peace for her was so overwhelming – what a true gift God has given to us! I’m so thankful that God allowed us to bring her into this world and also to help her go. We never could have imagined that we would feel joy and peace at such a time. What a soul to have lived for three months.