Aubree’s Journey

November 26, 2014 / admin / 3 Comments / ARPKD/CHF Alliance, Living with ARPKD/CHF

by Susie Hoguet

This is a story of my sweet Aubree, and her story begins in utero. When Aubree was 24 weeks gestation, it was discovered at a routine check-up that I had little to no amniotic fluid, so I was hospitalized and monitored. While I was in the hospital, my husband and I consulted with multiple doctors and were told many devastating and conflicting things.  Since about 20 weeks gestation the amniotic fluid is mostly baby’s urine, they were unsure of the baby’s kidney function.  Statements that stood out most included an uncertainty regarding what exactly was wrong with the baby’s kidneys, they appeared echogenic and were unlike any the doctors had seen before. Some doctors were also concerned how critically her lungs were compromised, since I basically had no amniotic fluid. Another doctor told us there was nothing they could do for us and our unborn baby so we should go home – no one would know what condition my baby would be in until she was born.

Thankfully, some of the doctors and myself had hope for our unborn child, and we stayed in the hospital. During 17 days of uncertainty, we prayed and stayed positive for our baby. On December 3rd, at 29 weeks gestation, the doctors said today was the day the baby needed to be delivered via c-section.After every scary scenario that was thrown our way pre-birth, the greatest sound we have ever heard was the sound of Aubree’s first cry. Finding out we had our first little girl, her cry was heavenly. She was whisked away to the Neonatal Intensive Care Unit (NICU).  When we went to see her I got to hold her little finger for the first time.  We questioned the doctors regarding her kidney function and were told Aubree was urinating so her kidneys were fine.  We asked if an ultrasound should be performed and were told it was not necessary. It was hard to believe Aubree was doing so great after everything we were told. However, in the back of our minds we just knew everything was not fine, but listened to the experts.

On December 6, I was finally able to hold my little miracle. During Aubree’s 80-day stay she developed high blood pressure. Again, the doctors said with an infant this usually has something to do with the kidneys so an ultrasound was finally completed. I was told the results needed to be reviewed because the doctors did not agree with the radiologist’s findings – which were that Aubree had kidney disease. Even though the doctor said he didn’t feel she had kidney disease, it still felt like our hearts were breaking.  A couple days later they said her kidneys still appeared echogenic; however, she did NOT have kidney disease.  Two weeks later another ultrasound was completed and the results came back that her kidneys were fine. We finally were able to bring Aubree home on February 20th, with unexplained high blood pressure in the 150/90’s range.

When Aubree was 6 months old she had an ultrasound of her spine for a sacral dimple. Although the sacral dimple is unrelated to ARPKD, they also perform incidental scans and take measurements of the baby’s organs.  Once again we were not contacted by the doctors and did not receive the results of the ultrasound, so we went to pick up the report in person. The doctor handed me the report, roughly scanned it and said everything is fine with her spine, however her kidneys appear echogenic and they may appear that way since she was born premature. I of course went home and read the results and was shocked to read Aubree’s kidneys appeared echogenic with the suggestion of small cysts. My heart sank and I burst into tears. I could not believe what I was reading. No one ever mentioned Aubree had cysts in her kidneys. I immediately called all of her doctors and Aubree had another ultrasound, which confirmed, in fact, she had kidney disease and that was the cause of her high blood pressure.  Aubree was officially diagnosed with Autosomal Recessive Polycystic Kidney Disease (ARPKD).

Currently Aubree’s treatment plan is to monitor her blood pressure and kidney function.  She takes a diuretic and a blood pressure medication to control her high blood pressure. One day she will require dialysis or a kidney transplant. I wish I could take away the disease and give it to myself, but I can’t.  I wish I could take away the monthly blood draws, but I can’t.  I wish I could take away the uncertainty of the disease, but I can’t.  What I can do is bring awareness and hope to other families of children who are diagnosed with ARPKD/CHF.

A year after her diagnoses I realize how we came to find out Aubree has ARPKD does not matter.  What does matter is we are blessed and fortunate to have Aubree in our lives. We will continue to fight for her and are thankful to spend every moment of every day with our little hero.

Today, Aubree is a loving, sweet, energetic toddler. She enjoys singing, dancing, and running around with her two big brothers.  Hearing her laugh and giving us all hugs and kisses are the best moments of our day. Yes, she has ARPKD; however, I like to think of her as our sweet little miracle that has big plans in life.