OHF Stories is a new initiative from the Oxalosis & Hyperoxaluria Foundation intended to heighten awareness of our rare disease through the stories of families living with it. Our first author is Kristi Ouimet, who many of you may know, mother of two children with PH1.
We invite any family with a story to tell to contact us. The more information available to the public, the scientific community & legislators, the better chance we have for more research, more understanding and, eventually, a cure.
By Kristi Ouimet:
It was just past midnight on 7/13/2011 as I walked behind the gurney through what seemed like an endless maze of hallways and elevators and up to the 6th floor. We found a temporary home in a curtained section of 6 north. As I met with the nephrology doctor, I was preparing myself on how I was going to explain the disease my son had and what it had done to him. I was so used to explaining to doctors what Primary Hyperoxaluria was, that I didn’t even think he would be familiar with it. He politely interrupted me and said he was not only familiar with Primary Hyperoxaluria, they have successfully treated patients with it and currently have other patients with it that were doing well. Finally, a hospital and doctors who understand and know what to do.
The nephrologist said the first thing we had to do was stabilize Matthew and get a hemodialysis catheter surgically placed. They explained he had toxins in his body that his kidneys were unable to flush because they no longer worked. The hemodialysis catheter would allow a machine to clean his blood for him and help him feel better. They were surprised he didn’t have seizures since his sodium levels were so low. They told me once his sodium levels came up, Matthew would be more lucid and his personality would come back. The night consisted of meeting teams of doctors and lots of paperwork and questions and more questions and lots of reassurance from everyone.
The next day we were moved to the pediatric intensive care unit (PICU). We struggled with understanding all the terms and procedures and treatment plans. One nephrologist told me not to search out a lot of information online because it might not be accurate. He said if I wanted to find factual information about this disease, OxalosisHyperoxaluria Foundation and Mayo Clinic were my best source of accurate information. If I had any other questions to ask the doctors at UCSF and they would explain things best they can. I told him I was confused how my son could be so sick when my daughter who has had this same disease for so long, wasnt as bad. Did I do something wrong or miss something? He said in his experience there are no two patients who respond the exact same way. There are lots of similarities but each have differences as well. Treatment plans may be the same, but each patient reacts their own way and it was no ones fault. Even if Matthew was monitored daily, it might not have prevented this.
Matthew had lots of “hiccups” in his recovery. They struggled getting a PICC line in, but once it was established he was able to get labs done without all the attempted pokes. His IV was giving him much needed fluid, but since he couldn’t pee, he was swelling so much he couldn’t open his eyes anymore. The attempts at placing a temporary hemodialysis catheter in his femoral arteries failed on both legs. They were finally able to get a temporary hemodialysis catheter placed in his neck.
I met two incredible dialysis nurses as they wheeled a huge machine into our tiny room. They explained everything they were doing as they hooked him up for his first hemodialysis session. He was only on the machine for about an hour, but it was a start. Over the next couple days he was able to handle a little more each time and the swelling started to go down and he was starting to feel better.
Other problems presented themselves. Matthew was having difficulties with excessive bleeding and he was tested for blood disorders. His skin was having a bad reaction to the adhesive tape. UCSF is a teaching hospital so every doctor on our service was followed by a team of other doctors and students. I was struggling with keeping everything straight so I asked them to limit telling me about all the new “problems” that they were working through and possibilities of their causes until it was absolutely necessary to let me know. I really wanted to be part of rounds and be involved in all the decisions regarding Matthew’s care, but I realized there were times that I was in the way and overwhelmed. They were very accommodating and would do their rounds and examinations then I would speak to one person per team. Matthew’s excessive bleeding resolved when his vitamin K was stabilized and it was discovered he had a severe allergic reaction to adhesive tape.
Everyday, things got a little better. His nephrologist met with us and said Matthew would be able to go home, but there was a twist. Matthew would need hemodialysis 6 days a week and it could only be done at their outpatient facility across the street. He explained that pediatric hemodialysis patients need extra care and it can’t be done at any other facility outpatient facility. UCSF was the closest facility to us. He arranged for a tour of the outpatient facility and explained Matthew would need to start going the day after he was released and he would need to go everyday except Sunday. Before we were discharged, Matthew underwent another procedure where he had a hemodialysis catheter surgically placed in his chest and the temporary one removed from his neck. After two weeks of being in the hospital, we were finally able to go home, even if it was only for a few hours.