Mr. Ben came into our lives a happy and hungry boy on December 15th, 2007. Other than a case of jaundice that required extra hospitalization, he was seemingly healthy and was the perfect fit into our family. He cried very little, loved when his big sister Anna would give him kisses and watching football games with dad. Everything was perfect! We were loving life and looking forward to what the future held for our sweet little man.
When Ben was almost 2 months old, we began to notice that something was wrong. He wasn't smiling, nor was he making any real eye contact with us. We thought he had some sort of vision problem. We brought our concerns to our pediatrician, who reassured us that Ben was fine and that all children develop at different rates. We took him home, but we were still very uneasy.
About a month later, Ben had made very little progress in his development. He was not smiling at all, and was still not making eye contact. Again, we were reassured by our pediatrician that things were fine. After a few weeks of still no progress, our pediatrician agreed that Ben probably had a vision problem and scheduled an eye exam at Arkansas Children's Hospital. He also decided to schedule a brain ultrasound, just to rule out any neurological issues. We were of course very anxious, but still didn't think that there was anything too seriously wrong.
We got Ben's brain ultrasound done, and the results were normal. When we went to get his vision checked, however, we discovered that there had been some miscommunication somewhere, because instead of an eye exam, he was scheduled for a hearing exam! We were frustrated, and of course his hearing was fine. It would be almost 9 months before we would be able to get into see the eye doctor where we would find out that Ben was legally blind, but we had no choice but to wait patiently. In the meantime, Ben got very sick. He ended up in the hospital with a mild case of pneumonia and a severe UTI. They did some tests, and we found out that Ben's left kidney was too small. We were very concerned with this news, but were reassured that it wasn't a very serious problem. Ben was almost 5 months old by now, and still not even holding his own head up, so his doctor referred him to a physical therapist. We started therapy and Ben hated it!! He would always cry for the whole session, but his therapist was so patient and sweet that Ben eventually 'tolerated' her. She even brought up Ben's coarse hair in one of her assessments, and when we asked the doctor about it he told us that there was a disease called Menkes kinky hair syndrome, but that Ben didn't have all the symptoms and he was just sure he didn't have it. We tried to convince ourselves that he was a doctor, he knew what he was talking about and that he had to be right, but it was always in the back of our minds.The doctor did agree, however, that there was some sort of problem and decided to order an MRI. We found out that Ben's cerebellum was too small. Shortly after we got the results of the MRI back, Ben had his first seizure on father's day of 2008, and by September he was aspirating so severely that a g-tube had to be placed. With all of this information, his pediatrician decided that it was time to see a geneticist.
In October of 2008 we had our first genetics appointment, and as soon as his doctor walked into the room he knew what was wrong. He explained to us that he did think Ben had Menkes disease, and ordered bloodwork. One week later on October 17th, 2008 we were devastated when we learned that Ben did in fact have Menkes disease. We were shown how to give copper injections that day, which was so scary! After about a week of copper injections, Ben's seizues had disappeared, he was smiling all the time, and things were really looking up. We even found out that Ben's form of Menkes had the potential to be milder than some of the cases we had read about. We were so excited! It was very scary to know what could happen, but Ben was doing so well we just knew we would have him for years to come.
Other than frequent UTIs, Ben continued to do well. In May of 2009 however, he started getting sick. He had a UTI almost constantly, and had fever all the time as well. He was hospitalized for a UTI, and during his stay developed extreme feeding issues, and was throwing up all the food he was taking in. Tests were run and we found out that Ben's gut was very slow moving, so it was decided that a g-j tube should be placed. It would go directly into his intestines, and by bypassing the stomach they thought all his problems would be solved. 1 week after being released from the hospital, however, Ben had a 103 degree temperature. We were going to make him a doctor's appointment, but before we could do that he started vomiting blood. We took him to the emergency room and found out that he had blood in his stomach bile and stool as well. They did a scope study and found that his g-j tube had coiled up into his stomach and irritated is stomach, and they believed that this was causing all his problems. The tube was repositioned and we were sent home a couple of days later. Four days after arriving home, Ben started vomiting blood again, and we automatically assumed it was due to his g-j recoiling. However, his tube had not moved, so we were kept for a few days and sent home. We were told that there was really nothing they could do about the bloody vomit, and that we should just try to make Ben as comfortable as possible. After 2 more hospitalizations, they concluded that his digestive tract was failing and a PICC line was placed. He received his nutrients through it, and was doing very well. He was still vomiting blood almost daily, but we were just kind of learning to live with it.
On the 1st of August, 2009, Ben was admitted to Arkansas Children's Hospital with a mild respiratory infection. He was observed for a couple of days and released on August 4th. He was doing well and even gave me a couple of grins that day, which was something we hadn't seen in a very long while. Everything was going normally that night, I put his sister to bed and Ben and I were just hanging out. At 10:30 that night, Ben suddenly stopped breathing, and was never able to be revived.
Ben was and will always be such a blessing to us. Every day since he came into our lives, we have thanked God that he thought we were good enough to care for such a special little boy. He brought us so much joy during his short 19 months, and we wouldn't trade the time we got to spend with him for anything in the world. It gives us so much comfort to know that our sweet Ben is and Angel now, and that he is laughing and running and playing, free from all the paing he knew while here on Earth. He is still very much a part of our everyday lives, and we talk about him every day. We count ourselves so very lucky and blessed to be his family, and we can't wait until the day we see him again!
Jamie & Travis
Posted: October, 2009