This is the first in depth update we have made in a couple of months, most of the latest updates regarding William we have posted directly to Facebook. He was doing fairly well until the end of April when he substantially decreased eating and became dehydrated. We spent 8 days at Children’s Hospital giving him fluids, running tests and trying to get his blood pressure under control. The first test that was run was called a pH probe. The purpose of this test is to measure the existence of, and acidity of reflux. The results of the test showed that while William does reflux some, the acidity of the reflux is well within normal ranges, and most likely did not cause his feeding problem. The second test that was performed while we were at Children’s was a swallow study. During this test a doctor watches William, under x-ray, drink a bottle to check for aspiration and swallowing issues. This test too, came back negative. While there we worked on trying to figure out why he does not like to eat with a speech therapist, and how we can improve feeds. We began using a bottle designed for babies with cleft palates that has improved his comfort during feeding times, as it does not require him to work as hard. At this point we still are not sure what causes him to not want a bottle, but we continue to try to improve his feeding. One cause that cannot be tested in the hospital, but can be caused by high creatinine, is that William just isn’t ever hungry. Currently, William has an NG tube to supplement what he will not eat himself. The NG (nasogastric) tube is a short term feeding tube inserted through the nose. If he doesn’t make any progress over the next 2 months, he will most likely need a G (gastrostomy) tube, which is a more long term solution.

While we were in the hospital, after William had been rehydrated with IV fluids rather quickly, his blood pressure spiked into dangerously high ranges. This was the main reason that we had to stay for 8 days. The doctors continued to experiment with different doses and types of blood pressure medicine. Toward the end of the week, his blood pressure was making steady progress in the right direction, so we were discharged. The following Wednesday we went to our Nephrologist who checked his blood pressure, and confirmed that it had gone down to a more normal range. However, today when we went, his blood pressure had spiked again. We are going to change medicines again in an attempt to get it consistently under control. Also, his hemoglobin, the oxygen carrying component of your red blood cells, was abnormally low at our visit today. If this doesn’t increase by our visit in 2 weeks, we will have to begin daily/weekly shots to help his body produce the required levels.

Until the our visit to Children’s Hospital, William’s diagnosis was largely unknown, and lumped into Childhood Kidney Disease. However, the dehydration, along with his high blood pressure, large kidneys and blood markers have led to a fairly firm diagnosis of Autosomal Recessive Polycystic Kidney Disease (ARPKD). ARPKD is an inherited recessive genetic disease that affects roughly 1 in 20,000 people. This is a big surprise to Kaylee and me, as it has never been an issue in either of our families. A link to a more comprehensive definition is below:

Autosomal Recessive Polycystic Kidney Disease

In the past 2 weeks since we got out of Children’s, we have had a lot of fun with William. The NG tube has taken a lot of stress off all three of us, and allowed us to do a lot more than we could before. We have been to the park, the zoo, the lake, and Kaylee and William have been going on a lot of social visits. And, William has even made a couple of friends. One, Walker, is more than twice his age, but they both have red hair. Also, we have discovered that William loves aerobics, and may be starting up a class for babies at the Mountain Brook YMCA any day now.

We appreciate all of the love and support that we have received from all of our friends and family. We couldn’t have made it this far without your prayers. Thank you all from the bottom of our hearts. Please continue to pray for:

• William’s blood pressure to go down, and to stabilize.
• William’s electrolytes would stabilize.
• William’s hemoglobin would increase and stabilize.
• William would begin to eat most of his food by mouth.

Kindest Regards,

Kaylee and Matchett Gunn