Von Playing at the Park

While pregnant with Von, there were signs things weren't right but nothing we could put our finger on. He had shorter than average legs, a larger than normal head and a long torso. We had made the decision in the 20th week to forgo all extensive fetus testing as it didn't matter to us if something was wrong. We believed he was our child for better or worse and no testing would change it. After a very difficult pregnancy, Von came in to the world early at 35 weeks. He was troubling for doctors. His head was large, he was shorter than normal, he had several heart murmurs, pulmonary issues, digestive issues, unexplained hypoglycemia, and jaundice that would not clear. We spent many weeks at the NICU in Children's at the Minneapolis Campus. Finally on day 39 we were released from the NICU with a protocol to follow up with GI for a liver that wasn't functioning normally.

We were home for a few weeks, and Von was not gaining any weight. We were having difficulty with eating, severe reflux, vomiting and he was a very quiet baby. Looking back he was too quiet. He never cried. He didn't even cry when he was born. I was just getting ready to go back to work after more than 4 months off of work. I had been on bed rest a portion of my pregnancy, and then because of his hospitalization my employer gave me extended leave. He caught his first cold, and we thought we could just manage at home. On my second day back to work, my husband flew up the stairs and said he had stopped breathing. His lips were blue but he was breathing again. This apnea continued and we rushed him to the clinic. In the clinic, he stopped breathing again and went completely limp in my hands. We were rushed via ambulance to Children's Minneapolis. We arrived at Children's and were admitted to ER. There were scans done, blood work, he was put on oxygen, and as we were rolled out to the X-ray I noticed he was foaming at the mouth. Something was not right with my child. He got progressively worse and was rushed in to Trauma. They place him on a ventilator, and started pumping him full of fluids.

We were admitted to the PICU with no known reason or diagnosis for what was going on. He was negative for every virus including a spinal tap to rule out meningitis. At the time of his admission to the PICU his stats we a body temp of 91.4, blood sugar of 7, he had respiratory failure, was in the beginning stages of cardiac failure, and he was in liver failure. His body was shutting down and they didn't know why. We saw more than 10 specialists over the next few days as they tried to figure out what was going on. By day 2 there were still no answers, and he was progressively getting worse. They didn't know if he was going to make it and they told us to be prepared for it. The evening of day 2 they started giving him massive amounts of antibiotics and a steroid call Solu-Cortef. Within 12 hours his blood sugar started to stabilize, his body temperature started to improve, his liver function was still in failure but his other vitals were improving. They brought in the Endocrine team. They pulled more labs, and within another 2 days we did an MRI.

On Wednesday January 16, 2013 after 6 days on life support we were given our diagnosis of Panhypopituitarism. Von was born with no pituitary gland. He was making no life essential hormones including cortisol, thyroid, growth hormone or testosterone. We were told that he likely also suffered a brain injury from the illness. Doctors brought in pamphlets and sat us down and shared what it would mean for his life. Von would live on steroids and hormone replacements for his entire life. He would never be able to play contact sports, and because of the Adrenal Insufficiency he would life with a life threatening condition for the rest of his life. There was no cure, and the only thing we could do was manage it with medication and be vigilant to help him manage any stress in his life. Any stress he faces he will always need extra monitoring and medication to make it through.

We were shocked and grateful for a diagnosis, but we had no idea what it meant. In addition to all of this, Von's lungs were weak after being premature and a week on a ventilator. He was also diagnosed with severe asthma.

Since his diagnosis, it's been a lot of doctor's appointments as we try to figure out why this happened. Doctors still can't figure out why the gland never formed. They said the chances of it happening was 1 in 500,000. We are still searching for a reason for why it happened, and even when we do, if we do, his long term prognosis does not change. He is missing the master gland of the endocrine system. He's had a lot of developmental issues to go along with the diagnosis. He has expressive speech delays, motor processing issues that effect nearly every part of his body, and he is cognitively delayed. Despite all of it, it is the happiest child I have ever met. He smiles and laughs all the time. He loves his daddy, playing with his nanny, and trying his hardest to be a little boy. It's not easy for him, but he finds a way through adversity. He will be in Speech, Occupational and Physical therapy and that will likely go on for years. We have created a team of dozens of people that help us manage his progress, medications and development. It's not the life we imagined but I would not trade it for the world.

Von will not be a typical kid, but I think typical and normal is boring! As he grows I know we will continue to face obstacles, but I know he will do it all with a smile on his face.

 

Back to Other Stories

-The support, information and encouragement provided by the PPFL parents is not meant to take the place of medical advice by a medical professional. Any specific questions about care should be directed to a health care professional familiar with the situation.