Beating the Odds, Ezra’s Life with ARPKD/CHF
Our son Ezra was born with Autosomal Recessive Polycystic Kidney Disease (ARPKD) on September 12th, 2001. As most of the stories go with ARPKD children, we were given no hope for his survival. We prepared for his passing, mourned him and like many, listened to the doctors and believed there was no hope for him. Finding out the baby you have loved and carried for 8 months is going to pass away shortly after you give birth to him is nothing short of devastating. Luckily, my husband and I had family and friends surrounding us with love and support and we could not have gotten through those heart wrenching days without them.
I was induced on September 11th, 2001 on the very same day the Twin Towers were attacked by terrorists and went down. On top of what we were facing with losing our baby, seeing the devastation across the country was unbelievable. Despite all of the tragedy around us we had our sweet baby, Ezra Daniel Quail in the afternoon the following day. He was breathtaking. We had several doctors and nurses in the room during delivery expecting the worst. He was checked over and placed on my chest. He was breathing, he was crying, he was beautiful. We had him baptized 10 minutes after his birth; we were surrounded by our dear friends and family. The waiting began…..
We were told by the doctors that he would not survive for more than a few minutes to hours after his birth. The nurses told me I could take his first bath with him as soon as my epidural wore off. That was my new goal, to take a bath with my sweet Ezra before he left us. I was granted my wish and it was wonderful.
We were in the hospital the standard two days, the second day, we were given false hope that what Ezra had was not ARPKD, it could have been a different kidney disorder and they were taking him to get and ultrasound to check it out. He was brought back to us and it was explained that it did look as if he had ARPKD and of course, the outlook was not good. They gave us the choice to let him pass in the hospital while we went home, or we could take him home with us and wait for his passing. To me, my baby was alive and doing well, there was no way I was going to leave him.
Ezra showed signs of hunger his second day. My milk had not come in and no one had shown me how to breastfeed so I did not know how to feed him. I asked the doctors to please help me give him a bottle and they said they did not think feeding him was a good idea. They said it would just prolong his pain and suffering. My “Mother’s Instinct” kicked in, I refused to starve my baby to death. With the help of our amazing nurse, we were able to start feeding him sugar water from a syringe. Ezra was too premature to take a bottle. We did this throughout the rest of our stay until our nurse found us a special bottle for preemies. Ezra was then taking formula through this bottle and he was having wet diapers like a normal newborn. The doctors still gave us no hope.
We took our baby boy home and like many new, young parents we weren’t sure what we were doing. Ezra was drinking and wetting his diapers normally. His belly was quite extended but he did not seem to be in pain. He was a great baby and didn’t cry unless he was hungry. I was so in love with him. I was sitting on my couch a week later holding Ezra when my phone rang. It was a heart specialist that had seen Ezra in the hospital and he said he could not get him out of his thoughts, so he did some research. He told me statistics of ARPKD that we had never heard. He said some babies do survive! He said he thought Ezra was going to be ok. He said he thought Ezra would be in our lives for a very long time. I will never forget those words. He talked to me for a while explaining what he had found out about children born with this disease. He said he was going to have a pediatric Nephrologist call me later that day to explain some more about this disease. I couldn’t wait.
Later that night we received a phone call from Dr. Kim from the University of Minnesota. He told me he wanted to see Ezra and run some tests. We went a few days later and met with the specialists. Dr. Conair, who was a colleague of Dr. Kim’s met with us that day and gave us the results of the tests. He came into the room with a huge smile and said he had fantastic news; Ezra’s kidney function was good! He said we would battle certain aspects of this disease and that sometime down the line; he would more than likely need a kidney transplant. We were astounded. Ezra was going to live!! We couldn’t wait to tell our friends and family who loved him so much.
The first year was our biggest challenge. Ezra couldn’t drink too much of his bottle at once because due to his enlarged kidneys there wasn’t much space in his tummy, so he would projectile vomit if he had more than 2 ounces at a time. When he was 2 months old he was hospitalized for extremely high blood pressure running 200/100. Due to the high blood pressure, his heart was enlarged from working so hard. It was scary, but it went back to normal after his blood pressure was regulated. After a week’s stay in the hospital to get his blood pressure under control we were sent home with a blood pressure machine. He was on several blood pressure medications and also supplements to help regulate his electrolytes. On top of all of that, his head looked rather large so the doctors did a CAT scan on him, which showed us he had extra fluid around his brain. Luckily, that went away after a few months, but it was scary to think something was wrong with his brain as well.
At seven months he was hospitalized again for 200/100 blood pressure readings. After changing his medications around, we finally got him stable. He fought bronchitis and ear infections his entire first year. On top of that he just wasn’t growing or putting on much weight.
We got through that first year and everything started to get a little bit easier. Ezra was doing better and was starting to get older. He started walking at 18 months, and genuinely was a happy child. When he was 3 he was hospitalized for the third time for 200/100 blood pressure, every stay was a week’s long stay. This time we think we finally got his cocktail of medications right.
Ezra is 11 years old now and doing wonderfully. He is at about 50% function and has been for several years. He sees the Nephrologist every six months unless his labs are abnormal, then he goes more often. He sees his Endocrinologist every 3 months to track his growth. We have seen his kidney function go up and down throughout the years, but for now he is still doing well. He is not on a kidney transplant list because he is not in need of one just yet. When they do take his kidneys, they will leave in his diseased kidneys and place the new one above his bladder. We are hoping he will not cross that bridge for many more years.
There are things Ezra cannot do such as, playing contact sports. His kidneys and spleen are very large and cannot be ruptured. We could get him a spleen guard if he truly wanted to try a sport that was not too dangerous but he has not requested that yet. We have never told him he cannot do something, we simply suggest something he can do instead. He loves to swim, play Legos and video games. He does not eat meat or drink milk; he says many types of food taste terrible to him. He drinks a ton of water every day. He has been on growth hormone therapy since he was 6 years old and is almost 5 feet tall. He currently takes 4 blood pressure medications twice a day and has his growth hormone shot at night. He is a normal 11 year old boy who has a normal life. It is the greatest gift we could have given him. We have never made him feel special or different. He does not feel sorry for himself. We do not surround our lives with this disease. It is what it is and we go day to day as normally as we can.
I have been a huge supporter of the ARPKD/CHF Alliance since Ezra was very little. We had no idea what was happening to our child and the Alliance was our source of information and support. We have hosted three walks in Ezra’s honor for the ARPKD/CHF Alliance and have had two on-line fundraisers. I am now on the Board of Directors for the ARPKD/CHF Alliance and couldn’t be prouder to dedicate some of my time to help support the people who supported us in a desperate time of need.
Denelle Quail Mom to Ezra (11) affected by ARPKD/CHF and Eben (3) unaffected
5 Comments On “Beating the Odds, Ezra’s Life with ARPKD/CHF”
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I would like to know where my daughter can get more information on this disease. She has seen 3 doctors. Two of them are from Pittsburgh and one from John Hopkins Hospital in Baltimore. The two from Pittsburgh are giving her no hope. Said the baby will pass away a few minutes after he is born. But the specialist in Boston was a little more hopeful in that he feels since she had fluid until 224 or 25 weeks that the baby would have developed lung function and should be able to breath after he is born.We are going to call tomorrow and switc doctors and see what they feel we can do. This is so sad and most web-sites are negative about the outcome. We need our miracle! thanks for any info or comments.
hello im Ezra quail now I live in South Dakota I have many friends here at my school Harrisburg north middle school. thank you so much for donating for my condition.
My name is Gina and my son’s name is Anthony. He was born in 1990 with arpkd but was not diagnosed until 2 years later. Anthony is now 22 almost 23 years old and has lived a very normal life with arpkd. Sounds like Ezra is going down the same path! Lots of luck to ur family!!!
Hi, I enjoyed reading your uplifting story about Ezra. My son, Benjamin, was diagnosed with ARPKD when he was 2 days old and had been followed by NIH until a few years ago…NIH claims that the slow progress of the disease was cause for the study to be suspended, but I suspect it is for financial reasons as well.
Anyway, my son will be 7 years old in a few weeks and is doing well on blood pressure medication. He is short for his age, however. We have thought about growth hormones but want to be sure that it won’t speed up the ARPKD and cause his kidneys to fail sooner than they would. Do you know anything about this? I read that your son is on growth hormones and hope you have some insight on this. NIH hasn’t returned my calls and my nephrologist is not sure. I would love to hear if anyone has any info on this.
There is definitely hope – my daughter is 27 and has the disease – they did a renal /splenal shunt when she was maybe 8 as she had GI bleeding, and then a gortex graft in her arm to get her ready for when she would need dialysis but her kidneys kept going until she was about 16 and had her first transplant. She is on her second transplant at 19 and 8 years out now – but she needed a liver, too and that was the magic to make it all great!