Home for the Holiday's!

We were so happy to be in our home, back with the rset of the men in my life. Ethan, Logan, and Landon were so happy that Evan was back home. Evan reached his 100th day post transplant on Christmas day! That is a very great day, they say the most critical time is the first 100 days after transplant.

We have had so many up and downs in this journey, you think you are taking all the right steps in the right directions, then BOOM, your taking another step back. On Jan. 11th, Evan was put back in the hospital for a fever. We knew when you came in with fever they admit you for at least 48 hours, so we were hoping thats all it would be. Well, it never seems to turn out that way, I ended up with pnuemonia, I was in the hospital for another 5 weeks. Ethan, my oldest, took us being back in the hospital really rough this time, he would call me every night in tears, begging me to come home. This is one of the toughest parts of this, I have four boys that I adore, but I have one that needs me more.

We spent our days in the hospital with PT, speech and OT therapy's, playing, and sleeping. It is hard to explain living in a hospital, it is very emtionaly exhausting. Every day you wake up praying that he doesn't get another fever, that his oxygen would go up, that he will stop coughing. There have been days when all I do is question God, even when I know He is taking care of him. I guess that's just our human nature.

Evan was finally released from the hospital on Feb. 16th..............

Can we leave yet?

Evan was able to get out of the hospital on a dat pass for Thanksgiving. We went to the apartment and spent the day with the boys, my Mom, and Anthony's Mom and Dad. It was wonderful to be out and have the family time I had been longing for. But, let me tell you, I can't wait until next years Thanksgiving dinner, Kroger does not fix a good one! It was so great to see Evan happy to be with his brother's for the day, but it broke my heart when he and I went back to the hospital that night, and he just sat and cried for them. He has been through more than anyone, especially a child, should ever have to go through. I have to say, it is a different world here, your home is your hospital room, you have to have someone come in and sit while you take just a minute to go to the bathroom or take the fastest shower you've ever taken, you share two washers and two dryers with about 60 other families, you eat hospital food every day, you are awakened every couple hours by beeps or by a nurse coming in, you wash your hands so much they bleed.....But, on the other hand, you meet some people you will always be friends with, you learn to care deeply for those around you going through the same struggles, and I guess most of all, you learn to fully rely on God.

It is truly unexplainable the way a Mother and Father's heart feels when they see they're child go through something like this, there is no way for anyone to know unless they have expierienced it with their own child. The worst pain imaginable.

Evan was finally released shortly before Christmas, and we were given the ok to go home, home! We were so excited to be in our own house for the holidays! But, our excitement was unfortunately short lived..............

Time together

Our days in the apartment were spent playing together. Evan was able to scoot around on the floor, and he would walk around the furniture. I was constantly sanitizing everything he could touch, the tables, his toys, the bed rails, his high chair. We would take long afternoon naps together, which was very nice being that I was up half the night changing sheets, putting more feeds in the pump. Evan has been sleeping in the bed with me for a while now, I can't bear for him to sleep alone, and it also calms my fears.

Anthony and the boys were suppose to join us at the apartment when Evan was released, but due to a vaccine they recieved, they had to stay away for another couple weeks. On pretty days, Evan and I would go for rides, taking in the sights of Cincinnati, he usually slept almost the whole time. My Mom came up to stay with us some, it was so nice to have someone else there with us. I missed Anthony so bad, just having him there to lean on when I was afraid, to talk to when I couldn't sleep. And oh how my heart ached from being away from my boys. I have always been fortunate enough to stay home with my children, I could count the times on one hand that they stayed with a sitter, and at that it was family. So, this was like torture. I knew Evan missed them as well, most days he would just lay on my chest, I could feel his lonliness, as I'm sure he felt mine.

One afternoon as I was hooking up Evan's feeds, his tube got stuck down in the side of the couch, when I picked him up, it shot right up and hit him in the eye. I called the Dr. on call and they wanted me to bring him to the ER. I couldn't stand the thought of taking him to that germ ridden place. I was fortunate to meet a wonderful friend while staying in Cincinnati, and she went to the ER with us that night. I was glad we took him after we found out that he did scratch his retena, he was put on eyedrops and they said it should heal in a few days.

Just a few days later, he ended up with a fever, just at the 100.5 mark, and we had to go back to the ER. We were in there all night, they addmitted him and drew cultures to find out the source of the fever. It ended up being an infection in the central line, E coli, so we were in the hospital for another week. The day after Evan was addmitted was my 30th birthday, so Anthony came up and relieved me so I could go get my hair done and spend a night relaxing. It was not the way I pictured my 30th, I missed my family so bad.

Evan was finally reuninted with his brother's after a very long two months. It was very emotional, Landon kissed him over and over, and Ethan and Logan just wanted to hold him. They didn't seem to notice his drastic change in appearance, all they could say was how cute he was.

He was only able to be back with his Daddy and brother's for one week. He got a fever in the night, we once again made the call to the hospital. They told us we could wait and bring him to clinic in the morning. When he woke up that morning I was in complete shock as I looked at him. His eyes were swollen shut, his mouth was full of sores, and the skin on his face was peeling off. GVH (graft versus host disease). I remembered them telling us this could happen, but I never imagined it would look this bad. Anthony had to stay at the apartment with the boys, so I was off to the ER with him again, alone. I cannot even brgin to tell you how afraid I was. The Dr.'s were in and out, drawing blood, checking blood pressure and temp. He was in so much pain, he cried and cried. His heart rate was 220, and he has a temp of 103. He was addmitted and started back on high dose steroids to try and fight it off. GVH is the body trying to fight off the new cells, but a tiny bit of it is actually a good thing, it shows that the body is doing what it's suppose to. Evan was diagnosed with stage 4 skin GVH and he remained inpatient for another three weeks while his body healed. When I looked at him, I couldn't imagine his skin ever looking normal again, he looked like a burn victim. He eventually started scabbing up, lost all the dead skin, and beautiful new skin was underneathe.

I got really down when he was in with the GVH, I felt I was praying with no response. I missed my kids, my marriage was on the rocks. It's crazy what stress can do to a person. I would lie awake watching him sleep, praying God would heal him. Asking God to hold me in His arms so I could close my eyes, just for a little while. It's amazing how, even when you doubt God, He knows your heart, and He never leaves your side, He was holding Evan all along. I have been asked the question many times, "As a christian, why do you think this is happening to your child". I guess my response would have to be, if Evan's story has touched even one person and brought them to Christ, then that's reason enough. I have been told how strong I am, I am only strong through Jesus holding me up, and there are days when I cry all day long, and feel as if I can't do it any longer, but then, He puts me back on my feet, wipes my tears, and tells me I am not alone, and I never will be.

Transplant

Evan recieved enzyme treatments throughout August and had his Central line placed on August 20th '09. He was put to sleep for this, he did really well. With Hurler babies, there is always a big risk with sedation, so they try to minimize the times they have to be. We had to spend the night in the hospital, this was our first stay. He was admitted on August 26th for the transplant process to begin.

The first four days he recieved chemo, the reason for the chemo is to knock out his immune system, if he has an immune system when he recieved his transplant, it would just kill the new cells. They told us the most likely side effects would be fever and vomitting, and he would probably slow down on eating. It was so hard to watch this beautiful baby, who was full of life, having to be completely broken down, just to be well. That first chemo shot was so hard on me to watch, knowing they were sending poison into his little body. I remember everything about that night, I sat in the rocking chair in room 507, rocking him as he felt the sting of the shot. The nurse was telling him how sorry she was, that she just hated to hurt him. He handled everything with a smile on his face, greeting each nurse with a smile, and blowing kisses as they left.

Evan has always been the happiest baby, so laid back. From what we have learned and wittnessed, all Hurler babies seem to be this way. The Dr.'s even told us, it is just their temperment. After staying a week inpatient, Evan was doing well enough that they allowed us to leave for seven days. We were to stay in the apartment we were renting in Crestview Hills, Ky. And he was only to be around Anthony, myself, and grandparents, as his counts were already falling. That week at the apartment was scary. It was just he and I because Anthony was in Versailles with the other boys who weren't aloud to be around him. He began getting sick every time he took a bottle, and didn't want to eat. We were readmitted that next week for more chemo, a stronger dose this time. He did pretty well, he was a little weak, but for the most part he seemed himself. He was able to start eating again, and he continued to play.

On September 18th '09 Evan recieved his new cells. They gave him pre meds in case of an allergic reaction, therefore he slept right through it. It was amazing just watching those bright red cells go in. Something that seems so simple, yet, it is so complex. The cells went in over about two hours. When he woke up he was pretty puffy from the volume of blood he recieved, it took a couple days for that to subside. We knew that after transplant was when all the real sickness began. We prayed that his wouldn't be too bad, but we knew God would see him through. About a week after transplant I believe, is when he got an NG tube placed in his nose for feeds. He was still eating a little bit at this point, but at around the two week post transplant point, they can develop something called Mucositis. This is really bad sores that start in your mouth, and go all through your GI tract. So, they want to place the tube without any sores. Evan actually did really well with the tube, he tried only one time to pull it out, from then on, he never even acted like he knew it was there. The NG tube ended up having to be advanced to an NJ, which goes on past the stomach into the small intestine, he was vomitting everything in his stomach up.

Shortly after transplant he started sounding funny when he cried, like he had a mouth full of cotton, they said this was the start of the Mucositis. He ended up getting it pretty bad, blisters all inside his mouth. He stopped all eating and drinking, and was put on a Morphine pain pump. He also had it all through his GI tract as well. He was placed on feeds and TPN and lipids for nutrition. He slept almost all the time, which was a blessing because if he was awake, he was in pain. The Mucositis lasted around two weeks.

Almost exactly two weeks after transplant Evan's hair started to fall out, it would be all over his face, on his pillow and clothes. When it starts doing this, the best thing to do is buzz it down. I couldn't help but cry as I ran the shaver over his beautiful white hair. Not because he would be bald, just because he looked, "sick". Of course I fell in love with that little bald head before the night was over. He was feeling quite a bit better at this point, we would sit together and watch movies and play. There were definately times of deep lonliness for me, I had never actually been alone before. Yes, Evan was there with me, but I was in another state, away from my husband, my other boys.

Evan's counts started to rise and they were watching for engraftment around 20 days post transplant. The first engraftment study they do, they always hope for a 100% engraftment, that would mean that all Evan's cells were gone, and he only had donor cells. We were so excited and nervous all at the same time to get the news! We found out around that day 20 mark that he was 100% donor!! He was able to be released from the hospital in early October to go to the apartment. We were then to start clinic appoinments three days a week, and home health care would start coming too. Evan was released on feeds and I had to learn how to do everything a nurse would do. That first night at the apartment was wonderful, life, a tiny bit back to normal.

What now?

Tears streamed down my face. Dear Lord, we begged for Your healing for Evan, why is this happening? All I could think was, why? All I wanted to do was sleep, wishing when I woke up, this nightmare would be over. We went back for his follow up appoinment, several family members went with us to hear the details of what was to happen next. The Dr. 's tried to explain things to us, we were hearing words we had never heard, terms that only a Dr. would understand. I remember sitting there, just crying, listening to our "options". They explained that with a bone marrow transplant, Evan would have the oppertunity at a "normal" life. Without it, he would recieve weekly enzyme treatments, the bad thing about the enzyme treatments is that they don't pass the blood brain barrier, therefore he would become mentally retarded fairly quickly, then as the brain shut down, so would the rest of the body. Average life span without BMT, around 13 years old. So, to us, there was no option, we would risk the bone marrow transplant. We all went for blood work that day to see if any of us would be a possible match for Evan. The best chance for a match would be one of his brothers, a 25% chance. Only a 1% chance for Anthony and I to match.

We were told about the reason this happened. Anthony and I are both carriers of this gene, therefore, each of our children had a 25% chance of being born with MPS, statistics were exactly right, 1 in 4. Evan started enzyme tratments the week after diagnosis while waiting for a match. These treatments were 4 hours long. He had to recieve an IV every week, with several sticks before getting a good vein. He usually slept through at least half of it, due to Benadryl and Tylenol as pre meds. We found out soon after that none of us were matches for him, he was put on The National Marrow Registry waiting list. They told us it usually takes two to three months to get a potential match.

In the meantime we decided to do a Bone Marrow drive in our town, knowing the chance of getting a match was slim to none, it made us all feel better just to be trying. What a blessing this was, seeing people coming in and signing up to be potential donors, if not for Evan, for someone else. There were people we didn't even know, people that just saw the sign and were touched to stop. It is truly amazing the good that comes out in people when you need them. The drive was a huge succes, bringing in around 250 people.

We were contacted by Children's about two months later, they told us they had not one, but three full matches for Evan! They would decide which one was the very best fit for him. And if something went wrong, we also had a backup. Evan's donor is a 21 year old male, that is all we were told. Wow, that is all I could think, this boy, just a young man, he is doing something that will bring him pain, to give our Evan life. Oh thank You Jesus for this man.

Evan was put through several tests to make sure he was stable enough to go through transplant, a CT scan of the brain, an echo and EKG of the heart, a Broncoscipy of the lungs, and numerous blood tests. He passed all of them with flying colors. We were more than pleased to find out that he had no damage from the MPS. Alot of kids are already having irreversible symptoms by this age, including hearing loss. We were told is was caught early enough. After they knew he was healthy, we met Evan's amazing Dr. She is a Hurler specialist, and is one of the top BMT Dr.'s in the world. We were very pleased to have her as Evan's primary Dr. The first thing she told us is she would give us the scary statistics only once, and unless any problems were to arise, everything from that moment on would be only positive talking. This is one of the things I have grown to love and respect her most for. We were told that there was a 10% chance of fatality due to the actual transplant process, when I thought of this I was filled with fear, one in 10 children, that sounded like way too big a risk. She said, let's say 100 out of 1,000, somehow, that did make me feel slightly more comfortable. I began to cry, telling her, I cannot lose my child, I can't live without him, her reply, if you don't do this, you will be living without him. We put him in the hands of our Lord that day, and signed the consent.

On July 1st 2008, we were blessed with beautiful twin boys, Landon and Evan. Everything was typical through my pregnancy, just as the two before. Morning sickness, cravings, not to mention swelling, heartburn, fatigue. I have always loved being pregnant, everything about it, even the labor. Such an amazing thing, knowing you are bringing a life into the world.

I lost a baby in November '06, so what a blessing to hear "it's twins"!! As I lay there waiting to hear the cry of two healthy boys, my Mother and my husband by my side, I am praying. Lord, please help me do this, I need Your strength. Landon was layed on my chest, 6lb. 1oz. screaming loudly! Seven minutes later, Evan Ray, 6lb. 1/2 oz. They didn't leave him on my chest as long, he wasn't screaming the way Landon was either, was something wrong? Was there something they weren't telling us? They wisked them to the nursery, they passes all the usual tests and were brought back to my arms with a clean bill of health. They were soon joined ny their big brother's, Ethan 8, and Logan 5, who couldn't get enough of them.

The weeks to follow were just as I had remembered with my first two, sleepless nights, breastfeeding struggles, and just pure bliss. When Evan was about six weeks old, we starting noticing some small differences between he and Landon. Some things we could write off as just "all kids are different", and some things brought more concern. Evan and Landon both ended up with bilateral inguanal hernia's at just 5 weeks old, and were operated on to have them repaired. About a week later, I noticed Evan's soft spot looked puffy, I took him to the Dr. expecting to hear, he is fine, your worrying too much. Well, he says take him to the lab, he needs to be tested for Meningitis. Of course I was hysterical, only to hear he is fine, sometimes this just happens.

As time went on, as Landon started smiling, rolling over, sitting up, and eventually crawling, Evan was always two steps behind. I began thinking more every day that something wasn't quite right. Here are some things I was noticeing: From the time Evan was born, he had a sunk in chest, we were told this was Pectis. He also sounded very loud when he breathed, we were told this was Tracialmalagia (sp?). Also, one of his little feet was turned in, we were first told this was club foot, then refered to Shriner's Hospital to have it looked at. He also had weak muscle tone, some gagging when eating and drinking, and a very narrow palet. At Shriner's he was mis-diagnosed with a mild Cerebal Palsy. Oh, my heart sank, how, why? Was it something I did when I was pregnant, or something during the delivery?

I immediately called the Pediatrician, trying to talk through my tears. He was convinced that Evan was fine, and he was just slightly behind, he told us not to worry, that he believed these things he was expieriencing were to be outgrown. My heart told me different, we went to several specialists, all said the same thing, he is fine. With much persistance, I got the Pediatrician to give us a referal to Cincinnati Children's Hospital Neurology Dept. We saw the Dr. in April '08. When she looked him over, she said it was not Neurological, but she wanted to run some special tests. They took some urine for something they called a GAG test, said we would have the results in a few days, not to worry even if they came back positive because sometimes they get false positive results. We get the phone call, positive. So what does this mean? We were instructed to make an appointment at the Genetics department. We sat in that room wondering what all of this meant, completely clueless. They were asking us all kinds of questions, family history, is there anyone with genetic problems in your family. The whole time we are thinking, it's just a false positive result, he is ok. The Dr. walks in, looks at Evan, and she immediately knows. She starts telling us about MPS1 and MPS2. She says she is certain that Evan has one of the two. Her explanation, "MPS is an enzyme defficiency, without this enzyme, sugars build up in all the vital organs eventually causing all of them to shut down." Ok, is there a light somewhere in this dark tunnel you are taking us down? Our first question, "Is it fatal?" Yes. But, with MPS1, he has the oppertunity to recieve a Bone Marrow Transplant, this is very risky, but he could go on to live a normal life with a succesful transplant. With MPS2, he would recieve weekly enzyme replacements, for life, as long as he would live. So, of the two, our thought was that MPS1 was the one we were hoping for. They drew more labs, and told us we wouldn't have the results for another two weeks.


I will never forget leaving the office that day, putting my perfect babies in the car, and thinking, I can't do this. Asking God why, why have you let this happen? Not my baby, I can't live without my child Lord. Please let the test come back ok, let them be wrong, please God! We prayed healing for Evan every day for that two weeks, I honestly felt like my whole world was crashing in around me. Two weeks came, I called to see if they had the results, MPS1. Now, where do we go from here.

Heavy hearted

My heart is heavy as I sit in a dark hospital room, my sleeping child on my chest. In a million years, I would never have imagined being were I am today. I remember thinking as I passed by a child with what seemed to be a very serious illness, pale, covering her head with a pretty silk scarf, look the other way. It was too scary to imagine what her mother's heart was feeling, will I see her graduate kindergarten, go to her first dance, wipe away her tears on her wedding day? It was easier for me not to even think about it, ignore the Saint Jude's commercials, simply out of fear of the unknown, fear that, "what if" it was my baby.

As I think back now, tears fill my eyes, just the thought of being "normal" again, seems so far from my grasp. When you see your life as a dream, of course everything looks perfect, big house, prince charming, healthy children. Of course, life in reality is only pieces of your dreams. But, the ones you prayed for, sometimes don't come true. This is a story of a Mother's journey, a journey into the unknown, with her beautiful baby.