G-tube Madness

Day of surgery

Zac went down to Primary Children's Hospital in Salt Lake City on his birthday Oct. 13th, to get his G-tube placed. He did fairly well, until we got home and the following 10 days were filled with painful cries. I called several times to get pain medication for him. (They said give him Tylenol and Ibuprofen as needed for pain.) We did as they told us, but it seemed he was always in pain. I finally got a hold of the GI Dr. who performed the surgery, and she said that he shouldn't be in pain, but just in case she wanted to see if the tube was placed correctly. BUT she was going out of town that day. So the next day we went back down to Primary's for the scan, the tube appeared to be where it was suppose to be. The Radiologist who did the scan said there was nothing wrong, then that evening someone who looked at the results of the scan called and said there was nothing wrong. The next day after Zac had cried and cried all day, I called again, and another doctor looked at the scan results and discovered that even though the tube was in the correct place, it was too tight and pinching his stomach, he asked if we could be there by 5 (It was after 4). Ummm no, we are over an hour away and longer if there is traffic. Brett and I had a date night planned and babysitters arranged. We asked if there was something we could do from home, so we took Zac in on the bed and loosened the tube. Brett should have been a doctor, he is good at that kind of stuff.

In the ER the 1st time

After that things were ok for a couple weeks, he seemed to be doing better. Until he had the G-tube for 4 weeks and things started to smell bad, his breath and his G-tube site. I was very concerned that Zac had developed an infection. Also his chest had sounded bad. I was concerned that he might have aspirated and it caused pneumonia. So what do I do? go to the local ER on Sunday evening (bad idea). It was so busy that I don't feel like they did a good job looking at Zac, or taking the time to examine him properly. We waited a while to be seen, but was in the room just a couple minutes before they sent us on our way. The next day he was seen by his Physical Therapist and Occupational Therapist, they both agreed that his lungs were not clear, and that the smell was bad. Brett has a friend/coworker at the jail that is a nurse and he gave us some ideas of things to help with the smell and the lungs. We did but a couple day's later things were still bad, and this last Saturday Brett took Zac down to Primary's ER. After speaking with 3 different doctors, a surgeon and the GI Specialist on the phone they discovered that Zac's skin was growing up the tube and not healing properly. Which is why it was smelling so bad. They took 5 hours to decide whether to cut off the extra skin, cauterize, or finish the procedure and place the peg. They ended up just cauterizing it and sending them home. Zac is now scheduled for the 2nd part of the G-tube procedure on December 9th. Pray that this goes well for him. He has already been through so much, it hurts my heart to watch him suffer more because his doctors keep making mistakes, and over looking things.

They decorated his room for his birthday!

Ace is such a good helper.

Change!

Our family has gone through a lot of changes this last month and a half since I wrote last.  We put our house up for sale and it sold in 3 weeks and closed 3 weeks later. We moved back to one of our rental houses. After a lot of cleaning and work on the house we finally moved in. We had so many wonderful people help us. We are lucky to know so many people who are willing to give their time and energy to us.
Within a week of moving we took a family vacation to Hawaii!!! When we got Zac's diagnosis and we learned that our time with him would be short, and that he would go down hill pretty fast, we knew we wanted to take one more trip to make some special memories.
Between the week of moving and Hawaii we decided to get Zac the NG-tube, which is the feeding tube that goes down his nose into his stomach. We wanted to have the knowledge of using it just-in-case Zac needed it while we were in Hawaii. We didn't want to take the risk of him not eating and getting dehydrated while we were there. We brought all of the equipment with us, but luckily we haven't had to use it. But Zac is still loosing weight and we are going in to get the G-tube on Monday the 13th, Zac's 2nd birthday.
Before we left for Hawaii, Zac was having a very hard time sitting on his own, and wasn't crawling at all, but after his vacation he was doing really well, sitting by himself, and crawling around a bunch. This week he is loosing those skills again, but it was nice to see him moving around, at least for a while.
We continue to enjoy our time with Zac, we are starting to figure out his care plan. At every doctor appointment they ask us about it. It is really hard to decide what measures to take to keep Zac alive. We also are going to be meeting with the mortuary and make funeral arrangements, we feel like it is going to be easier now, then after he passes. One thing is that my Dad is going to build Zac a casket. We know that quality of life is very important to us, so we will have to figure out where we think he has no quality of life. Ugh! I have been putting it off for a while because how do you decide when and at what point you let your child die? There are no rules or instructions, and the doctors just say, It's up to you...This will definitely be the most difficult part of this journey. One thing is for sure, every time Zac smiles, it makes you forget about his battle. Good thing he smiles a lot.

Zac Gave Me a Fright!

Yesterday Zac and I were on our way up to Primary Children's Rehab of Ogden for physical therapy, and on the way Zac started to cough, (He doesn't have a lot of power behind his cough.) Up comes some mucus and it must have gotten caught in his throat and closed off his airway because he stopped breathing and his lips started to turn blue. I of course pulled over to the side of the road, jumped out of the car and went to assist him in the back. Well, he got it cleared and I realized that I was helpless to do anything for him. I tried to remember back to those 1st Aid classes I had taken. And when someone is choking on food, you insert your finger inside and try to clear the airway, mucus isn't solid like food. That wouldn't work. After I returned home Brett advised me to pull him out of his car seat and turn him face down and see if gravity would help. Who knows, I hope I never have to find out. Anyways, I get back in the car and away we go for his appointment, I keep looking in my rear view mirror to watch him and he is acting really off, his eyes keep closing and his head is more floppy than usual, and his color is off and his breathing is abnormal. But then just as we were pulling into the parking lot the coughing/choking/not breathing happens again. At this time I decide to drive straight to the ER, since we are only a couple blocks away. I get there and he seems fine, his STATS are normal. We meet with the ER Dr. and I have to educate him on Zac's condition, which is hard for me to do without being an emotional wreck. Then he asks me what I want him to do... That was frustrating because I do not have any sort of medical knowledge. So I ask if he will look at his lungs. I don't know???  So they do an X-ray where they strap him inside a tube looking thing. This was our first encounter with this equipment, it was miserable to watch, and miserable for him, but very effective. The last time he had X-rays was at Primary Children's Hospital, and they didn't use anything like that, we had to hold him down and keep him still, they had to keep trying to get it right, but yesterday, the tech got it done in one shot. Zac does have the beginnings of a virus infection in his lungs which caused the mucus. We have a suction machine coming in the morning to help with this.

With this eventful, scary morning I realized that I am still very far away emotionally and mentally for him to die. I just kept thinking, no, no, no, they said 3 years old, he's not even 2 yet. I am not ready for this... Now, I don't know if you can ever really be ready to lose your baby, but I know that yesterday I was defiantly not ready. Glad he came through the victor!

After spending the morning at McKay Dee Hospital's ER, we went straight down to Primary Children's Hospital and first met with the Palliative Care Group called Rainbow Kids. There again we have to educate them about his condition, but this time I let Brett be the voice and I tried not to listen. This group has a pediatrician, social worker, chaplain, 2 nurse practitioners, and a few more people that I can't remember. We talked about SSI, Medicaid and Hospice. They want to be part of the team, they help find resources, and will attend appointments with us, and when Zac gets admitted to the hospital they will visit us and be involved.

Our next appointment at Primary's was at the Dysphasia Clinic where we met with a GI Dr and a Nutritionist. Zac is loosing weight, he is down to 22 lbs. and 30 inches in length. Which in comparison to others his age, he is 5% for weight and 0% for height. We are going to have a NG tube placed, Brett and I will both learn how to insert and remove this from his nose. They think we should tape it to his face, but knowing Zac, I don't think that will fly. They also said we can see how the day goes and insert it for a feeding if he didn't eat enough that day, then we would remove it. We will do this next week, this will require him to be admitted to Primary Children's and stay over night.  This NG-tube is a temporary option for him. We will have the G-tube placed directly to his stomach in the middle of September. Since the G-tube takes about 8 weeks to heal, we are waiting until after our beach vacation.

Brett and I are so grateful for the goodness in people, since we sold our house in 3 weeks, and are moving into a rental house that was trashed by our last tenants, we have had so many people from our ward, family, and friends come to help us with the house. I knew we couldn't do it alone on the short time frame that we were under. But people are amazing and gracious to come over and help. We have also had our Ward (church group) bring in meals, and help with the boys. There is also many of them who have participated in Zac's fundraisers. We are sad to be moving out of such a great Ward and neighborhood. I will remember and always be grateful for your kindness. I have learned so much from your example.

My co-workers aka friends have really out done themselves, they arranged 3 fundraisers and a drawing, (raffle). Several of them worked behind the scenes to pick up donated items, and ask local businesses for items for the drawing. I have really learned humility and friendship from them. I feel my life is normal being around you at work, that is a blessing.

Now our family, we couldn't do this without you. All of the support and help from both Brett and my parents and siblings, I will love you forever. I am glad we have each other. You are our emotional buffers, your loss is the same as ours. We need each other in these hard times, and I am grateful for each of you. P.S I really miss my little brothers Ben and Jake. I could really use them right now. :(

Thank you will never be enough, but it is what I have. I have the rest of my life to pay it forward. Just know that we are grateful for your support! I know there are many who don't live close by, but that are praying and encouraging us from a distance, I am grateful for you as well. Please continue to pray because I know it is helping.

Results

Swimming With Brothers

Today after a month and a half I finally got the results of the blood work of Max and Ace, unfortunately both boys tested in the carrier range for GM1 Gangliosidosis. This will only affect them when they decide whether or not to have children, there is a 1/250 chance of marrying another who is also a carrier. Brett and I were just lucky enough to have found each other.

Zac has regressed so far that if you judged him on an age level, I would say he is somewhere between 4 and 6 months old. He is unable to hold himself up, sit up, or swallow solids very well. He cries much of the day. We have a bumbo that he sits in for a couple minutes at a time. He struggles in his car seat and in the high chair. So the only option we have now is to carry him around all day. This is tiresome for Brett and I, and it is hard to accomplish anything while holding him. Our most productive time is during nap time. I received a phone call yesterday from Primary Children's Rehab and they just had a positioning chair donated to them and called and asked if we wanted it. I am not sure I know what it is, but if it means I can set him down for a while I am willing to give it a try.

He actually gets strapped into the gait trainer even though he can't move himself around. He gets pushed around the kitchen and living room by his brothers. This lets them play together, and I get to take a shower. This is fun for him and Max and Ace. I admit that I enjoy watching it a great deal too!

We have taken therapy off of our priority list, we felt like it was time consuming, and wasn't helping him. If we thought it would have made a difference in his abilities we would have continued with each (PT, OT, and Speech) weekly. He cries almost the whole appointment. It just wasn't worth it. So now we are doing one appointment a week with Primary Children's Rehab. (Instead of 3.) We are continuing with Early Intervention twice a month for PT, OT and Speech. That is nice because they come out to the house. He has gotten used to the therapists that come and he lets them work with him.

This coming week will be a hard one for us, we will be deciding on the best time for the placement of the G-tube. (The sooner the better.) We are meeting with Hospice and Rainbow Kids through Primary Children's. Rainbow Kids is palliative care, I don't know anything about that, I guess I will find out. Please continue to pray for us, we need all the strength we can get, also we need Heavenly Fathers help with the decisions we are forced to make.

Zac LOVES Sara

These last few months we have tried to do fun things and make of memories with Zac, we also take a lot of pictures and videos. After he passes we want to make sure we have no regrets. We have had lots of fun, but don't get me wrong this has been the hardest time of my life. Everyday that I watch Zac's little life take another step backwards, it tears me up inside. I struggle with myself, I am constantly fighting depression, I have a hard time focusing, and my mind feels fuzzy. I try to swallow the constant lump in my throat every time someone talks about Zac to me. I try so hard not to be emotional and cry. But I am no good at it. I just can't imagine life without my little Zac, he is MY baby and I want to keep him. I KNOW the plan, I KNOW where he is going, and why he is going through this, but I am selfish and human. I wish things were different. But having the knowledge of my forever family keeps me going. This is just a short time, but very hard.

"The Lord takes many away, even in infancy, that they may escape the envy of man, and the sorrows and evils of this present world; they were too pure, too lovely, to live on earth; therefore, if rightly considered, instead of mourning we have reason to rejoice as they are delivered from evil, and we shall soon have them again." Joseph Smith

The hardest things.

These past couple weeks have been filled with emotion. We have been asked some very hard questions regarding Zac's care. They said we can be aggressive with his care, or do as little as we would feel like. Now that we have met with most of the specialist doctors, we decide if we want there involvement. My Pediatrician said that they can keep Zac alive for a long time living on machines. But Brett and I know that no matter what happens or how good of care he receives it won't change the fact that he has a terminal illness. There is no cure, there is no treatment.  We have decided that quality of life is more important than quantity. BUT I still am upset that we as Zac's parents have to make that decision. No parent should have to decide whether to let there kid live or die. It breaks my heart!

We also learned today that Zac is being classified as GM1 Type 1, and with the aggressiveness of the disease it makes more since. They have seen more of the physical features of Type 1 shown in his bones. And on a daily basis we are seeing regression.   He has been sleeping more throughout the day. His awake time is often 6 hours. But sometimes we try to push it a bit longer. He is getting more tired and weak everyday. He is no longer able to hold his body weight up with his arms and face plants as he tries to crawl. Yesterday he tried to get in the position to crawl and he couldn't get his legs to cooperate. Poor little dude. He gets frustrated and upset, and I get a broken heart. But with that said the pediatrician thinks that he will be lucky to live to his 3rd birthday. This makes me very sad, I am selfish and want to keep him. I know he will go to a better place, I just feel bad for us.

We are expecting to get a package in the mail tomorrow. It's "The Vest!" This will break up the mucus on his lungs and help it to drain. This will prevent early onset of pneumonia. He is also suppose to start breathing treatments of Albuterol and Flovent. But our insurance has denied the claim. This insurance stuff is a mess. We are trying to figure out a better more comfortable way for Zac to get around. We are thinking of a car bed, and a better kind of stroller. He is 24lbs of dead weight. I need to get something to pack him in. While in the car he struggles to keep his head up, this stinks because we are always in the car going to doctor appointments. We are concerned about his airway.

We picked up a pulse oximeter today that Zac is suppose to wear off and on while he sleeps and while awake, that didn't go over very well he cried for hours. He didn't think it was very nice of us to make him keep it on. We finally took it off so he could get some sleep. If it starts to get low that is a sign of him getting sick, or not having enough oxygen. Therefore, we monitor it daily and if it is low on a particular day we call or go to the hospital. If Zac gets sick at all it will mean pneumonia..hospitalization..and maybe death. So we have got to stay healthy as a family. PLEASE don't come over if you are sick.

Zac is maintaining his weight but is not eating consistently, we feel we are getting to the point where a G-tube should be placed in his stomach. We are meeting with the dysphasia clinic in 3 weeks to figure that out. The Pediatrician said that Zac would handle the sedation better now, than he will later. This will also take away the worry I have of him aspirating. But even with having a G-tube, he will still be able to eat the normal way. This will just make it so he doesn't have to work so hard for his calories.

We love Zac so much, we can't imagine our lives without him. This is a very hard time for our family and we appreciate everything that is being done to help lift our burden. All of the financial donations have been amazing, it is nice to know that we don't have that additional stress of medical bills, and instead we can enjoy the short life of handsome little man. We have been blessed with people helping out with meals, that is a blessing all the time (I hate to cook). After pulling an all day hospital visit it is so nice to have a meal already prepared for us. Thank you! We are very grateful for that as well. There has been family, friends and neighbors who help with Max and Ace. They are having a great time instead of being dragged to Zac's appointments. That also helps the appointment go smoother too, nothing like sitting there with 2 extra kids who don't want to be there, for several hours. Last but not least we are very grateful for all of the prayers, we draw strength from them. This is hard to explain, but I know that every pray is helping my family. Keep them coming, please and thank you!!

Bonded

Zac thinks that he and I are one person. It is interesting how sensitive to emotion he is. If I laugh, he laughs, even if he is not by me. For this reason we try very hard to keep positive emotions. A couple days ago I say to Brett "watch this," and I started laughing and Zac followed suit. Then Brett said "okay, I can do that too," then he laughed...nothing happened. Zac and I have a very special unique bond. He is mine and I am his.

Zac has his favorite people and favorite activities. He LOVES to wrestle with his daddy and brothers. He enjoys bike rides and stroller rides. He basically likes to do whatever his brothers are doing, he likes to be in the middle of things. Zac has a daily routine of making a mess, (there is only a small amount time where he will get down and "plays") He starts in the living room and pulls out the DVDs, then moves to the diaper cupboard, goes over to the games, moves to the kitchen and takes out the Tupperware, then goes to the bottom drawer and takes out the baggies, moves to the stove and takes out the pans, then he is done. This happens everyday and takes him about 45 minutes. During this time I have to stay on the couch and watch him. If I attempt to get up and do anything he cries. Then it is nap time, he goes to bed and I clean up the mess. Good times!

We had a good week, we went to the Weber State Fireworks, spent Monday morning at Willard Bay (Zac loves to swim), took a bike ride, and had a couple doctor appointments. We are trying to make the most of the little time we are given with him. Especially since he is sleeping so much and is only awake around 6 hours a day.

We learned that Zac has lost his peripheral vision, and is having inner ear problems. This makes it hard for Zac to lay on his back, it causes him to feel dizzy and feel like he is falling into nothing. This makes me sad because I feel helpless to do anything. We are trying out new ways change diapers and dress the baby that doesn't require him to lay down on his back. Ideas??

Because Zac is sleeping so often and not eating much, I am getting concerned that he isn't getting the nutrients that he needs. I am researching the most nutrient foods and planning a special diet for him. I am also going to try to meet with a Dietitian in the near future (if I can get in). We also went to see an Occupational Therapist today for feeding. Her biggest concern is that he will get too tired while he is eating and aspirate. After he ate 3 Gerber meat sticks he was already tired and started to struggle swallowing. She wants us to feed him more pureed foods, and run everything through a food processor. We are trying to hold off as long as possible on the Gtube.

I spoke with the GI specialist today and she said that the results of the swallow study were in and that it didn't appear that he was aspirating while he was eating. But it was not a good study. (He screamed the whole time.) She has put him on Myra lax because his GI system is weak and is moving very slowly, she also thinks this is the reason he doesn't want to eat much.

I also spoke with the Geneticist this evening, he said that Zac's organs are the correct size, but his bones show signs of GM1 type 1, but it isn't real severe. He is still trying to get approval from the insurance company to do a full on genetics test, and the way I understand it, it will explain more about the time frame that it onset. He said it will answer more of my questions. I asked about longevity, he said that this would help answer that, I have no idea how that could be. I also asked about Max and Ace's blood work results, the computer says that is it still pending. I am going to call him back next week.

There are 2 clinical trials that we are going to sign up for, one is for the NIH (National Institutes of Health) in Washington DC, they will observe the disease (since it is so rare, they don't know much about it). They will have us come out every 6 months for 4 or 5 days. And then there is another one in Minnesota, and they are trying to control seizures. They would put him on a Ketogenic Diet and prescribe him a drug. But as of today Zac is not having seizures, so the Geneticist is going to call and see if they still want him in the trial.

Sorry if I have repeated myself. I am very grateful for all of the love and support from all of you. I feel strengthened by your prayers for my family. I know that we couldn't do this without you! Please keep praying, this is going to be a long hard road for us. The emotional strain is wearing on us. But with our faith and hope in the Lord we can get through this.

 Please feel free to leave a comment!

The Pursuit of Happiness

As Brett and I are in the kitchen, (he is cooking and I am cleaning, which is pretty much the roles we have taken) we are talking and laughing about things, and Max asked, "why are you guys always so happy?" With all of this doom and gloom that is staring us in the face every minute, we have decided to make the most of life and cherish every moment. Are things perfect, no way! Knowing that we have little time with Zac it didn't automatically turn the kids into perfect angels. But we know that happiness is a choice. Even though we are watching our son literally die before our eyes, we are going to enjoy this journey. We hate the prognosis, but we love him so much. We believe that there is a purpose and reason why we are going through this and we hope to come out the other side stronger. But for now we still really need your prayers!

He thinks he is so big just like his brothers.

Update on Zac: He is getting more tired and irritable. He can remember things he used to do and gets frustrated because he can't do them anymore. He only wants to be held. And when I hold him he rests his head on my shoulder. Zac has always been weird about laying on changing tables and exam tables. So we adjusted and used the floor. But in the last couple days he has developed a fear of laying on the floor too. He is not happy getting a diaper change or getting dressed. With the other boys I would change diapers while they stood up, but Zac isn't strong enough to hold up his body weight for long. 

Because he is getting more tired and weak we have decided to go back to two naps a day instead of one. But that doesn't leave much time in the day to be awake and play, especially since it takes a very long time for him to eat, and they want him to eat 6 times a day. Today his awake time was 6 hours. That just doesn't seem like enough time spent with him. But if he is tired he isn't fun to be around either. We will just make the most of the happy boy we love.

Happy to be outside.

Since we received the diagnosis we have been going from doctor to doctor, specialist to specialist, hospital to hospital. Every time we get in the car now Zac starts to cry thinking we are going to the doctor.

We have been seeing the Pediatric Neurologist, and she was the one who told us the diagnosis. We will not be seeing her again until Zac starts having seizures. I have been praying everyday that he skips that part of it. They scare me to death.

We have an amazing Pediatrician who has mirrored the urgency I have felt since the day of the tremors. She has an assistant lady who has been making all the specialist appointments, filling out paperwork and appealing things through our insurance company. The day we met with her was the day I felt like I could breath again. It has taken much of the stress and leg work off of me.

We have been to the Geneticist at Primary's and he ordered a full skeletal x-ray, ultrasounds of his organs and then more labs and a urine sample form Zac. We went straight across the hall and did all of them while we were there. It was a long day. He also ordered blood work for Max and Ace so we know whether or not they have the disease or are carriers of it. We have not heard back.

So tired

 We went to the Pediatric Gastrointestinal specialist in Riverton who will be monitoring Zac's eating and digestion. She will be the one to put in his G-tube (feeding tube directly to his tummy) when the time comes. She also ordered a swallow study to be done at Primary Children's with a Speech Therapist. Which we did yesterday, it was at nap time and he doesn't like to be set down. So to get him to cooperate was impossible. The therapist said that she wants all his food to be pureed. Also that he is not aspirating while he eats. Very Good.

We met with a Cardiologist, she did an EKG and it came back normal. But she said the things that they worry about with kids like Zac don't show up on an EKG and that we need to go back and have him sedated and get an ultrasound of his heart. I still need to make that appointment. Ugh! I don't know why we didn't do it all at the same time.

Fun on Grandpa's tractor.

We are scheduled to go to an Opthamologist and Pulmonologist in the near future. He is still having physical therapy weekly, I just got a call today saying that the appeal went through my insurance company and Zac will be starting weekly speech therapy and occupational therapy through Primary Children's Rehab of Ogden. He also is involved in Early Intervention and all 3 therapists come out twice a month each. 

Even though Brett's medical issues sucked it has been very nice to have him off of work for the last month. I don't know how I would have done it without him. And also for all those who have watched Max and Ace so they didn't have to sit in a boring hospital day after day, I am grateful for you too. 

He is starting to squint his eyes all the time.

With all of these appointments we have been getting medical bills pouring in through the mail and we are very grateful for those friends and family who have donated to help ease our burden. Thank you, and God Bless you!!! If you have any desire to donate there is a link on the right side of our blog. Also, if you want to leave comments or have any fundraiser ideas, please let me know.