Ryan William Kulik
Ryan William Kulik
August 22, 1998 - August 7, 1999
Probable Mitochondrial Encephalomyopathy - Complex 1 & 3
Our son, Ryan William Kulik, was born on August 22, 1998 after a normal, full-term pregnancy and natural delivery, just as his mother, Lisa, had with Ryan's two sisters, Ashley and Alexandria. As Dad, I was "on hand" for all three events, and felt that the birth of Ryan seemed as smooth as his sisters' had been. Family, nurses, and doctors complemented on the new member of our crew. Ryan was a very healthy and happy baby and went home a day after entering this world.
Ryan's first few weeks are quite normal - a typical new born who does not sleep through the night, but wanted food every three or four hours. After about three weeks Ryan became very cranky and appeared to be constipated, his stomach tightened up and he was very irritable. We took him to his Pediatrician, who changed Ryan from Carnation GoodStart formula to Similac. After about two weeks, Ryan continued to show signs of constipation, and again the Pediatrician changed his formula.
Ryan was now about two months of age and about this time we noticed that his eyes didn't seem quite right. He didn't seem to be able focus both eyes on one thing. We knew that kids his age can't necessarily see clearly, but there was something different about the way that Ryan looked at things. We brought that to the attention of his Pediatrician, who said that Ryan would grow out of it.
At three months old, in November of 1998, Ryan was still having problems with his digestive system, and his Pediatrician directed us to a fourth type of formula - the third was Infamel, the fourth was Similac Low Iron. Ryan sometimes seemed comfortable, especially after changing formulas... and for a while we thought things were going OK.
On Thanksgiving Day, Ryan cried all day. Late in the afternoon, after Lisa and I exchanged duties of comforting Ryan and cooking the family dinner, we decided it was time to call the doctor. The doctor gave Ryan something to calm him down so he would sleep through the night. "There's a bug going around" he tells us.
A few more days passed and still Ryan had problems with his formula. He also was leaner than most kids his age. We brought him to his Pediatrician and Ryan is given yet another formula - Alementum. He has a routine checkup at about 15 weeks and Ryan is found to be well below the average weight for a baby his age. We again bring up the matter of his eyes, and the Pediatrician again suggests that he'll grow out of it.
At five months of age Ryan was not acknowledging, reaching or responding to things. He is now on his sixth type of formula... Isomil. At his six-month routine check-up we again voiced our concern about Ryan's eyes, that he has trouble focusing and doesn't grab for things. He doesn't roll over, sit up or even speak the little goo-goos that babies do. By this time, we have spent more time in the doctor's office for Ryan than we have collectively, for the four of us together.
The Pediatrician set up an appointment with a pediatric optometrist. On our way to the eye doctor I cry, thinking that our son might be blind. Ryan eyes were tested and, although his eyes and their components seem to function ok, the doctor says Ryan may have exotropia, where his eyes are pointed outward, the opposite of cross-eyed. The doctor tells us that it can be corrected with a minor surgical process... man, are we excited... nothing too serious.
However, the optometrist says that Ryan should see a neurologist to make certain "nothing else is going on"... like a tumor or something. We were sent to the Connecticut Children's Hospitals Neurology Department. During our first visit, several things - Ryan's size and weight, his eyes, his high muscle tone and his continued digestive problems, immediately alarm the neurologist. She is concerned that he can't crawl, sit up, and that he chews on his hands for stimulation. She orders blood and urine tests.
It is now the end of March, two weeks after the tests and we're anxiously waiting, calling every other day inquiring about the results. I am in my office at work when I receive a call from the neurologist. Ryan has elevated alanine and lactate levels. That coupled with his global developmental delay suggests a type of mitochondrial disorder. I sink down into my seat; a single tear comes to my eye, and then many, many more. I call Lisa.
At about seven months of age, Ryan begins to have what appear to be seizures. He gets very excited, his eyes roll back, and he stiffens up... similar to the way he did when he was having problems with his infant formulas. He also seems constipated. We alert the neurologist and Ryan's Pediatrician.
Our neurologist orders a muscle biopsy. We set up the next available appointment, June 6 & 7, to fly to Atlanta to Scottish Rite Children's Hospital for a muscle biopsy, the best test to diagnose a mitochondrial disorder. We are now on the Internet daily looking for information and sharing experiences with other people. We are learning a lot, on our own, but it is stuff we don't necessarily like to hear.
We basically use our Pediatrician as an insurance coordinator, and have taken actions into our hands. Reality started to bear in. At the suggestion of Ryan's neurologist, we contacted "Birth-to-Three" to get physical therapy help for Ryan. They begin to come in twice a week to work with Ryan, and continue to do so for the coming months.
In late May, and with Ryan at nine months old, his neurologist recommends a MRI. After a few scheduling conflicts between the Pediatrician's office and the lab, Ryan gets to travel into the big tube. While reports indicate slight abnormalities towards the front hemisphere of his brain, things look somewhat normal. But at home Ryan continues a pattern of unresponsiveness and irritability.
It is June 5, and Ryan, Lisa, his sisters and I board an airplane to Atlanta for a visit with Dr. Nicolas Krawiecki and Dr. John Shoffner of Emory University School of Medicine and the Scottish Rite Children's Hospital. Ryan's visit there is special, getting to fly in an airplane for the first time, and then visiting his cousins from Atlanta. As for the doctors, nurses and such, while Ryan may not have appreciated them much, his parents were comfortable with their attention and professionalism. They are frank, honest, sincere and sympathetic. Ryan had the routine blood and urine work, and then the main reason we were there... a muscle biopsy and spinal tap. We left Atlanta, glad to go home, anxiously awaiting results.
A few weeks pass, it is now near the end of June, and we tell our Pediatrician and Neurologist about some things that we've read about Carnitine and Co-enzyme Q-10 supplements. After some time, they put Ryan on both, and Vitamin B50 complex, and vitamin E, as well. We also mention NADH, but they are not sure of what that might accomplish.
In late June, Ryan begins to do something very different. At night he wakes up, and while not yelling or screaming, he says "whoooo, whoooo, whoooo". It is not a cry. He almost seems scared of something. We also notice that every once in a while he takes a deep breath, like a giant sigh. He breathes normally, but then suddenly takes a deep breath.
In early July, Ryan is almost ten months old and I'm holding him and walking under the big majestic maple tree in the side yard of our home. The kitchen window is open and inside we can hear Ryan's mom, Lisa, and his two sisters, Ashley and Alexandria, making dinner, chatting like mothers and daughters do. For a second, Ryan looks at me and all his senses seem to become alive, like never before. He smiles and coos as if trying to say something, so intentional... so deliberate. It is a beautiful moment.
The middle of July comes, and Ryan's constipation continues. He has not been passing stools normally and he continues to take these random deep breaths. We take him to the Pediatrician with our concerns, and Ryan is prescribed Calo syrup and a suppository to help him. Ryan poops. They tell us his lungs are clear.
A few days pass though, and again, Ryan can't pass a stool. We call the Pediatrician, and they tell us to increase the Calo Syrup and try another suppository. And he poops.
It is late July and Ryan has gone almost five full days without a bowel movement. And today he hasn't urinated in twelve hours. He also appears to have had a few seizure episodes. Very frustrated, I call the Pediatrician's office and tell them they need to look at the whole picture: not passing stools, no urination, seizure like activity, possible mitochondrial disorder (we hadn't gotten the test results back from Atlanta.) We find that we need to explain Ryan's medical history each time that we call the Pediatrician. The nurse on call recommends we take him to the emergency room. We do.
They put Ryan on an IV to get fluids into him. While we do not admit publicly to his possible fate, we have Ryan baptized in the hospital. Ryan bounces back pretty well and we are able to take him home after being in the hospital for two days.
On Tuesday, August 3, 1999, Lisa calls me at work with alarming news. Ryan's nanny thinks that Ryan has stopped breathing and has called the ambulance. I rushed home to arrive at the same time as the ambulance, and to ride with Ryan to the hospital. He is breathing and everything seems to be OK... but just OK.
They put Ryan on oxygen, which they kept him on for a day and a half. They also put an IV and a feeding tube in him. The doctors in the ICU explained the process that they were going to use with Ryan to us. They will take the oxygen tube out on Thursday (the 5th) to will try to get Ryan breathing on his own again. If they don't do this, because of the mitochondrial disorder, Ryan could lose all muscle strength required to breath. They try to take the tube out, Ryan does OK for a few hours, but then CO begins to build in his blood. They put the oxygen tube back in, but take it out again when the CO levels drop back down. Now reality is looking us right in the face.
It is Saturday morning, August 7, 1999, and Ryan has made it through the night. Ryan's aunts and uncles, grand parents, a few close friends, and most of his cousins have come in to see him. Most know they are saying goodbye. At about 1:00 p.m. Ryan waits for the last of his cousins to return from their vacation on the Cape. His uncle and cousins come in to say hi, but we know deep inside that he is saying goodbye. They leave the room.
Ryan is tired. He has been fighting something for almost one year that is unlike any other disease. If it were a tumor, they might try to cut it out...or cancer, maybe radiation. But this is different. It is the mitochondrial system, the very fundamental function of life, and the mechanism that dictates energy production within a cell.
It is Saturday afternoon, a few minutes before five o'clock. Lisa and I sit next to Ryan, watching the monitors, caressing his little body and telling him everything will be OK, just like we had a little less than a year ago when he was born. It is OK for him to go now. I sing to him the song that I have sung to him since his birth: "Oh Ryan, Oh Ryan, you're such a big, brave lion." We assure him that his sisters love him, and always will. He takes a deep breath and his strong, yet tired heart beats on for a moment...
... And then he is gone.
[This tribute to Ryan was written by his father, Gary Kulik.]
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