Let me (and my mommy) tell you about our family. My name is Kristi and for the past 3 years, I have been a stay at home mom, caring for my children (Molly age 9, Patrick age 7, and Matthew age 1). My husband, Kelly, is a police officer and currently works the night shift. I also have four amazing step daughters (Brittany, Jordan, Jessica and Olivia). When Molly was 3 years old she started getting kidney stones, many were so large she had to have them surgically removed. She has had several surgical procedures in her young life and it was not until she was 5 years old that we had a diagnosis explaining what was happening to her. Molly was diagnosed with Primary Hyperoxyluria Type 1. It's a genetic disorder that her father and I are carriers for. We had never heard of this condition and to our knowledge, no one in either of our families ever suffered from it. Since we are both carriers, there is a 25% chance of our children getting this disease. We had our son Patrick tested and he does not have the disease, nor is he a carrier. When I found out I was pregnant with Matthew, we had a special test done and it was discovered that he also had the disease.
We assumed Matthew would react the same way Molly did, but his case was much more advanced. At 4 months old, he started throwing up a lot and not acting himself. Doctors thought it was reflux and we tried medicine. It didn't work. After being told several times it would pass, I insisted they check further and do more tests. A test confirmed he was in end stage renal failure and no longer a patient our doctor knew how to treat. He would have to go immediately to University of California San Francisco Hospital by ambulance. We were so scared, we had our priest come baptize him in the ER. That was July 12, 2011. We arrived at UCSF shortly after midnight on July 13th and stayed in the PICU for 13 days. He had so many surgical procedures. He had tubes and monitors hooked up to every part of him. It was so hard to see our baby like this. After several days they were finally able to do dialysis through a catheter that was placed in his neck. Slowly, he started coming back to us and was once again our sweet little boy with the amazing personality and charm. We were released from the hospital but had to come back 6 days a week to the outpatient dialysis unit. That means commuting everyday from Antioch to San Francisco.
Here is a typical day for us: I get up at 3:30 A.M. and get meds and formula ready for the day. The car is packed with everything we need for our trip. Matthew gets his first dose of meds at 4:00 A.M. through his GTube. We load up in the car by 5:00 A.M. We pick up whoever is going with us that day (Monday & Tuesday my dad, Wednesday my mom, Thursday & Friday my husband and everyone takes turns on Saturday). We arrive at UCSF around 6:15 A.M. and on the machine by 7:00 A.M. Dialysis is 4 hours hooked up to a machine that cleans Matthew's blood. During this time we entertain Matthew by watching movies, reading books, playing with toys and I nurse him. We are usually done by 11:00 A.M. and leave by 11:30 A.M. to head home in Antioch.
Matthew gets medicine and supplement formula several times throughout the day. We play when we get home for a few hours and visit with Matthew's brother and sister when they get out of school. I help the kids get homework done and take Molly to soccer practice 2 times a week. Matthew and I get home and prepare for our evening routine. When Matthew goes to sleep, I hook him up to another machine for a different kind of dialysis called peritoneal dialysis. Matthew gets this type of dialysis when he is sleeping every night for 2 hours. Peritoneal dialysis helps stretch his abdomen so it will be large enough to receive a transplant. We are usually done with everything around 11:00 P.M. Matthew sleeps with me so that I can nurse him if needed, but mainly so that I can monitor him as he is hooked up to a feeding machine and does not pull out any of his catheters or tubes. Most babies with kidney failure also lose their appetite and forget how to eat (swallow) so if they are still nursing, it is encouraged they continue so they get nourished and continue to swallow.
Matthew's condition has no cure. It is a liver disease that effects the kidneys. Basically, Matthew's liver does not have an enzyme that eliminates the oxylate in his system so the oxylate deposits in other organs, destroying them. The kidneys are the first organs affected. Oxylate attaches to calcium and forms the most common type of kidney stone (like in Molly's case) and can attack and destroy the kidney, as in Matthew's case. Dialysis is needed to rid the body of waste and fluid since the kidneys dont work. The only treatment is a kidney and liver transplant. Since it is a double organ transplant, it has to be a deceased donor. Doctors will not take two organs from a living donor. Matthew had to get big enough before he was even listed on the donor list. He finally got big enough in April of 2012 and has been listed nationwide ever since. It is crucial we keep Matthew healthy so that he remains on the list. If he were to get sick he would have to be hospitalized and he would be suspended from the donor list. Matthew gets regular checks on his eyes and heart as well. Oxylate can also deposit and destroy his heart and eyes so it is important he get a transplant before that happens. Matthew needs organs from someone aged 12 to 24 and has a small build. He cannot receive another baby's organs because once transplanted, the organs will not grow. Because the donors organs are bigger, we need to stretch Matthew's abdomen so that it can fit the donated kidney and liver. After Matthew receives his transplant, he will continue to have dialysis another month or two to rid the body of the oxylate in the system.
We realize talking to others about becoming organ donors is not a typical conversation but feel the need of awareness should be made. It is very difficult to consider anything happening to a child, especially your own, however we hope people will choose to donate their child's organs should something tragic happen. Thank you for reading and sharing Matthew's story. Please talk to your family about becoming an organ and tissue donor. The more people who register as donors, the more lives we can save!
Please visit www.ohf.org for more information about Matthew's disease. For more information about becoming an organ donor please visit www.organdonor.gov.