Thank you for visiting my fundraising page. This page is in memory of my baby half sister Nicole, our daughter Nora Evelyn, and in celebration of our son, Nathan. All were born with ARPKD/CHF (Autosomal Recessive Polycystic Kidney Disease/Congenital Hepatic Fibrosis). A single gene is responsible for each, and my husband and I are carriers.
When my half sister Nicole was born in 1978 I was four years old, and her loss touched all of our hearts forever. All of my life I loved her and counted her among my siblings. I knew that there was a possibility that I was a carrier of the disease that took her from us at the age of two months.
Our son was born in August of 2007 after a difficult pregnancy. He was not diagnosed until he was two months old, after 6 weeks of screaming. One of our happiest moments was the first time he laughed, in the hospital, when he was two and a half months old and we were finally able to get to know him. He is the light of our lives and a total ham! He goes to Pre-K and has lots of friends. We try to keep life as normal as it can be. He spent the first year and a half of his life in and out of hospitals, often in for weeks at a time.
When Nathan was one and a half, and still spending much of his life in the hospital, we learned I was pregnant again. We were thrilled and terrified. At 18 weeks we learned that my amniotic fluid was very low, and we knew that this baby, too, had ARPKD/CHF. I was hospitalized for much of the pregnancy, leaving my husband going from the Children's Hospital to the adult wing, caring for our son and for me. Thank goodness for my Mom- Grammy is our hero!
Our daughter, Nora Evelyn, was born at 35 weeks gestation, and when we heard her cry our hearts soared.... only to come crashing down as we learned the severity of her lack of lung development. We were able to have her with us for 25 hours, and the family was able to take turns holding her and saying hello and good-bye to our angel before we had to let her go.
Nathan was two years old when his sister was born and died. When people ask him if he has any siblings he answers "Yes, baby Nora. But she didn't get put together right in my Mommy's belly." We know now through our research that more could have been done for our daughter. She could have been given a chance to live, but we did not know what we needed our doctors to know. We wanted our daughter to have a chance. We now know that had we been in a location with specialists in ARPKD/CHF, our daughter may well have had that chance.
There are too many babies lost due to a lack of knowledge about this disease. We have come so far since 1978, and babies born with ARPKD/CHF should be given their best chance. One of the main goals of the ARPKD/CHF Alliance is education- not only for families, but for the medical profession as well. There are still doctors who tell families that their babies cannot live with ARPKD/CHF. Our son would beg to differ!
We recently took our son to an ARPKD/CHF specialist (another hero), and were shocked to learn that our son's disease is way further progressed than we knew. He has about 20% kidney function, and his liver is more severely affected than we knew. He will need a dual kidney and liver transplant from a deceased donor, and will probably be listed within a year. We will be moving temporarily from Tulsa, OK to Boston, MA, when Nathan is listed. We also learned that a drug to stop the progression of the disease may be available within the next 5 years... but that will be too late for Nathan. He will need his transplant soon. It will not be too late for all of the other children and babies living with ARPKD/CHF, or those yet to be born with the disease. For them this will be a miracle, just as a transplant will be a miracle for Nathan and others.
For now, we play and snuggle, and do all of the things a family with a 4 year old does. And we travel many hours to see specialists, take him for shots every week, feed him through a G-tube, and will soon be giving growth hormone injections nightly. He is on many medications to control high blood pressure and other conditions caused by chronic renal failure. And none of it keeps him from being a happy, smart, loving, and goofy little boy.
The ARPKD/CHF Alliance is the only organization dedicated solely to research and education for ARPKD. If you are able, please help us to raise money for this important research, and for education so that babies like our daughter Nora may have the best chance to live, and children like Nathan can get the best care possible. No amount is too small to make a difference!
Donating through this website is simple, fast and totally secure. It is also the most efficient way to support my fundraising efforts. Many thanks for your support.
Holly, Kirk, and Nathan Smith