10/26/2011

Liam is going in for surgery tomarrow around 1pm. He is getting a G-tube and a Nessen Fundoplication. Hopefully this will fix the problem and Liam will be able to hold down his foods. He got his first dose of Biotin tonight for his Biotinidase Deficiency and will continue to get it until we get the results back from his test. Theyre going to intubate Liam for surgery. Im not sure how Im going to react seeing a breathing tube in my son again. Too many memories of UCSF ICN. If they have problems excubating Liam then he will have to have it for a few days. It can also take up to 4 hours if there isnt any complications in surgery. If theres not a lot of scar tissue they will be able to do surgery laproscopically. Thats what Im hoping for. Praying little Liam is strong enough to survive this. Hes a fighter, even if he is weaker right now. All I want is for my baby boy to be good. I want him fixed. I want to take him home and enjoy the holidays without having to be in a hospital. Im trying really hard not to worry. I have no idea what Im going to do with myself for 4 hours while Liam is in surgery. I know right out of surgery I wont be able to hold him and that going to be hard. Im trying to keep myself from sleeping in the rocking chair all night with Liam in my arms. He needs a really good nights sleep so he can be strong. Were not exactly sure how much longer were going to be here. We know at least a week. I miss my cats, my house, my couch and my bed. You would think that this would get easier over time but its not. It seems to get worse with every taste of having my son home. I pray to God that we wont have to spend Thanksgiving or Christmas in the hospital. I know things could always be worse. Please pray for Liam.

10/24/2011

Ok we were misinformed it wasnt cystic fibrosis that Liam was positive for at birth like we were lead to believe. His retest for it was neg :) What Liam had at birth was called Biotinidase deficiency. Its very rare. Only 1 of every 110,000 births.Biotinidase deficiency is an inherited metabolic disorder of biotin (vitamin B) recycling that leads to multiple carboxylase deficiencies. The mos...t common early symptoms include seizure activity of various types (myoclonic, grand mal, and focal or infantile spasms) and hypotonia. Other early symptoms include breathing problems (tachypnea, hyperventilation, stridor, apnea), skin rashes and alopecia. Later developmental delays, speech problems, ataxia, and vision and hearing problems may occur. Less frequent findings include feeding difficulties, vomiting/diarrhea, fungal infections, hepatomegaly and splenomegaly. This disorder is inherited in an autosomal recessive pattern. As an autosomal recessive disorder, the parents of a child with biotinidase deficiency are unaffected, healthy carriers of the condition and have one normal gene and one abnormal gene. With each pregnancy, carrier parents have a 25 percent chance of having a child with two copies of the abnormal gene, which results in biotinidase deficiency. Carrier parents have a 50 percent chance of having a child who is an unaffected carrier and a 25 percent chance of having an unaffected, non-carrier child. These risks hold true for each pregnancy. All siblings of infants diagnosed with biotinidase deficiency should be tested. Biotinidase is an essential enzyme that liberates biotin from proteins in foods for utilization by the body. It also enables the body to recycle biotin, which is necessary for proper functioning of carboxylase enzymes. Without biotinidase to release free biotin, the ability of the body to alter fats and to metabolize proteins and carbohydrates is impaired. Complications including metabolic acidosis, coma and death can occur unless supplements of biotin are provided.

The good news is that this is something that can be treated with meds. Yes if he is still positive then he will have to take them for the rest of his life. We will have to have Lanie tested for it because they say all simblings need to be tested because if one kid has it its possible another has it as well. He will also have to see a geneticist regularly. I feel better with this diagnosis than with cystic fibrosis. No Im not happy but it is what it is. Ive done some research and Im sure theres more to it than what I've read online. Childrens hospital is waiting on paperwork they need from UCSF before they can re-test Liam. Apparently we are about 2 days over due in retesting Liam but they didn't know about it and we didn't either. The doctor found all this out when he finnaly got ahold of the results of Liam newborn screaning test. Theyre going to talk to the surgeons and hopefully this test wont delay Liams surgery. Were hoping to keep things on track.

On a good note, Liam had some visitors tonight. His Tia, Uncle, Cousins, and Auntie Jolene came to visit It was a great visit and I got some pretty cool pics :)

10/23/2011

Just talked to the surgeon. They want to run some test before they do surgery. Another ECO of his heart, blood work, re-test for cystic fibrosis since he was positive at birth and a few studies on his lungs and airway. They said they'll try to do it microscopically but if theres too much scartissue theyll have to open him up. They might have to re-intubate him and she said it could be hard to excabate him so he might stay on it for awhile. Itll be hard to see but they have to do what they have to do. They just want all the ducks in a row and have no suprises like hyper tention or anything else. :( They said they might have to put off the surgery for 2 weeks if he is still sick. The first time I got to see my son he was intubated. It was the image that poped into my head when the surgeon mentioned it as a possibility. I felt like we were taking atleast 3 steps back just with his respitory problems. If it came down to intubating Liam and having him crash suddenly during surgery its an obvious choice. Situations like this just don't help my PTSD. I know I need to be strong for Liam and for Lanie and for Justin so I have to keep it together. I know eventually we will get to the point that this will all just seem like it was a nightmare. It feels like a dream that we even had him home, like it didn't even happen. We were blessed that we got to take him home though. We are blessed to have every day we have with him. Liam is a fighter and he will fight to the very end. The statistics for people with cystic fibrosis suck. They're life expactantcy is 30-40 years and 95% of males with cystic fibrosis are infertile. Then again there's no telling how long a CDH baby will live. Theres no reason to think they can't live a long happy life but there are too many unknowns. Again Liam is a fighter and it doesn't matter what he has. He won't give up and neither will we. We will love him his entire life and hopefully, if God willing he will outlive us. I am happy we have answers. I am happy we have a "solution". With the world of technology these days theres no reason to think the worst. I know that we're going to celebrate everyday and every smile. We're going to make sure he lives as normal as a life he can and that he is happy and painless. I know I will continue to tell his story in hopes that it helps someone else and get out awareness. Being a mother of a baby with Congenital Diaphragmatic Hernia has changed everything for me. It's not an easy road. It's a long, hard, dark road we have ahead of us but Liam's smile gives me the light I need to see the end.

10/22/2011

I find myself completely pissed off. We just found out from Liams specialist doctor at Childrens Hospital that in her conversation with UCSF Pedi-Surg that UCSF said they thought he needed a Gtube that they were going to talk to us about it on our next CDH clinic in November. Basically they werent going to give us a heads-up; they were just going to ambush us. The doctors I thought I had an open communication and good relationship with and were supposed to trust. I feel like that trust has been broken. We told them several times before they released Liam the last time to do whatever they had to do to fix Liam so we didnt have to put him through this again. They just went ahead and released him already suspecting that he needed a Gtube. What makes it worse is that if they would have just kept Liam 2 more days then this would have already been done. We wouldnt have wasted 10 days at Childrens trying to figure out what was wrong. With a critically ill infant you dont have time. Liam was already way under weight and length that he is failure to thrive. Liam doesnt have any weight he can loose. I feel like Liam is a guinea pig for UCSF that they just want him for research and instead of doing whatever they had to to make him better theyre experimenting instead of jumping straight to the problem solver. Keep in mind I absolutely love UCSF. They know what their doing, they know his case. My problem is with the communication. All Ive asked over and over again was that they keep us updated and they do whatever they have to fix him. So maybe Im not mad but more disappointed. We also found out today that Liam needs a fundoplication. They take the top of the stomach and wrap it around the esophagus to keep him from being able to reflux his food. Theyre going to do this the same time they do the Gtube procedure that way they only have to put him out once and only recover once. I know its whats best for him. I know he needs it. Ive done the research and talked to the doctors. The mommy in me just wants to cuddle him close and cry with him. The analytical part of me wants to hand him over and say fix him. Its a good thing he can have the best of both because not only do I get to cuddle him and love him but I get to make them fix him.

About fundoplication:
In a fundoplication, the gastric fundus (upper part) of the stomach is wrapped, or plicated, around the lower end of the esophagus and stitched in place, reinforcing the closing function of the lower esophageal sphincter. The esophageal hiatus is also narrowed down by sutures to prevent or treat concurrent hiatal hernia, in which the fundus slides up through the enlarged esophageal hiatus of the diaphragm.
In a Nissen fundoplication, also called a complete fundoplication, the fundus is wrapped all the way 360 degrees around the esophagus. In contrast, surgery for achalasia is generally accompanied by either a Dor or Toupet partial fundoplication, which is less likely than a Nissen wrap to aggravate the dysphagia that characterizes achalasia. In a Dor (anterior) fundoplication, the fundus is laid over the top of the esophagus; while in a Toupet (posterior) fundoplication, the fundus is wrapped around the back of the esophagus.
The procedure is now routinely performed laparoscopically. When used to alleviate gastroesophageal reflux symptoms in patients with delayed gastric emptying, it is frequently combined with modification of the pylorus via pyloromyotomy or pyloroplasty.

The mortality rate is less than 1% which is the best odds Liam has encountered in his life. I am still very scared because I know how fast things can go wrong. I am trusting in God that he will be right there with Liam every step of the way and keep things on track. I am still requesting prayers for Liam. I am a firm believer that prayer works and there is never too much prayer. Please find it in your hearts to just send up a little prayer for Liam.

10/21/2011

Childrens's Hospital doctors consulted with UCSF Surgeons and they decided Liam needs a Gtube. A Gtube has the same concept as an ngtube. it goes straight to the stomach. The difference is it is surgically inserted through the abdomen wall into the stomach. The reason were doing this and not sticking with the ngtube is because with the ngtube there is a chance of his tube migrating up and slipping back down and going into the lungs. That would be very bad because then his feedings go into the lungs drowning him. He needs to be on continuos feeds 20 hours out of 24 hours in the day because of his gastritous and reflux. Its alot safer and allows us to give him the callories and volume he needs to grow. Please pray for Liam he will be having surgery sometime next week. We thank you all for your prayers and support.
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Dear Santa,
Its been a rough year and I feel Ive been good. Everything seems to be breaking around me. My truck, my dryer and my son (to some extent) are just a few examples. I feel Ive handled it all very well so I should be rewarded. Santa this year for Christmas I just want something that works. If I could choose one thing to work I would without a doubt or second thought choose my son. I will gladly forfeit all future Christmas, birthday, mothers day, anniversary and any other gifts for the rest of my life for this one gift. Being said, I feel that such a selfless act should not go without reward and feel I should be able to choose that reward. I choose my son. I dont need any fancy wrapping, no bows, or streamers. No bling or glitter. I dont even need it to be under the tree when I wake up Christmas morning. So please Santa, just this once, grant me my wish.
-Liams Mommy

P.S.
If I promise to be good every year would you find a cure and answers to CDH?

10/20/2011

1am I just read this ariticle about a mother who is loosing her son to Tay-Sa chs disease amd it brought to light so many things. I realized that with Liam I am trying to make the best of every day I have with him because I have seen that with CDH kids you never know what the future holds. I am thankful that there still is a future involved with Liam that he doesn't have a death sentance like Ronan from the article. Because of Liam's uncertain future I realize that I parent him differently than I do my perfectly healthy 3 year old. I find myself more tolerant and easy going with Liam and more stern with Lanie. I realize now that noticing what I am doing that this is not fair for either child. Lanie will end up resenting Liam for us being easier on him and for spending so much time away from him since his birth. Liam will end up hating his sister for resenting him and therefor being mean to him. It's something that needs to be addressed and changed. No more can Lanie loose her parents just because Liam is sick and admitted into the hospital again. I need to be more patient with her and understanding. As hard as this is on my it must be ten times harder on her. If something should happen to Liam. God forbide, I want Lanie to have been able to spend as much time as she could with Liam. I want our family to spend time as a family and make as many memories as we can. We have witnessed how quickly the health of a CDH baby can go downhill so quickly so we don't take for grantit how well he is doing now because things can always be worse. Still when thinking of Liam there is still a future, there is hope. So he won't be able to play football or be a boxer, the great thing is life will still go on. He might not even be able to be a race car driver but he can still enjoy the sport. The wonderful thing is Liam has a fighting chance. We have spent alot of time in hospitals and undergone many procedures but we still have him. We can still hold him and tell him how much we love him. Children are blessing sent from God. Liam just happens to have to work harder than most. But still things could be worse. I encourage all to read this article called "Notes from a Dragon Mom". It'll make you rethink your perspective. If you think you have it bad just remember there is always someone else out there that has it worse. You should be thankful for what you have not regretful for what you don't have. I see this moms point of view and to an extent do the same things. I am always defending my child. When someone refuses to see that Liam is different I pound it into their heads that he is and that I almost lost him before I ever got to hold him. When I see pregnant women out there smoking or drinking or doing drugs is makes me angry to no end. They don't understand what that does to a child. They take for grantit that children are born healthy and happy that you don't have to do anything differently for it to be so. I did everything right during my pregnancy. Took what I was supposed to , stay away from what was harmful and still my son was born with a defect that almost killed him. Read this article. Maybe cry alittle, I know I cried for this family, then prayed for them. Then hug your children close and tell them everyday how much you love them. Even if your mad at them, if their in trouble, it doesn't matter. When your done yelling at them don't forget to tell them you love them. Stop taking for grantit today because you never know about tomarrow. http://www.nytimes.com/2011/10/16/opinion/sunday/notes-from-a-dragon-mom.html?_r=2

10/20/11 9pm
We had a meeting with the doctors this morning. I'm sick and tired of fighting for my son to get the care he deserves. I'm tired of the doctors not doing anything until they are forced too.I'm at my wits end. Unfortunately Justin hasn't reached the point I'm at. One minute he thinks he needs to be transfered to UCSF the next he's fine with the way things are going here. All the doctors are doing is passifing us and they can tell its working on him so they keep doing it. It's the only reason we got them to do more. The doctor tried to tell us today that we asked for things to be done slowly with Liam. I just staired at him because not once did we say they were moving too fast or pushing too hard. Every step of the way weve told them their not doing enough. He tried to pass the blame onto us. They tried to discharge Liam while he was still vommiting and now they want to blame us because we have a grievance against them. Not only am I fighting the doctors Im fighting Justin and somewhere someone has to give. I wish I had all the answers, have tried to get all the answers but they just aren't there. I feel lost and confussed. I want to scream at the doctors to just fix Liam. How hard can it be to just fix him? They went from thinking it had nothing to do with CDH to thinking it could be linked to his high floppy right side diaphragm. I just don't know. I'm loosing sleep over this. I can't sleep, I'm never hungry, Im terminally pissed off. I have so much pent up anger and all it's going to take is one of these doctors or nurses popping off at the mouth with some stupid comment for me to blow up. I know things will get better but right now it feels like we're stuck in a hole. Life will never be the same again. I knew having another child would change everything I just never exoected things would change so drastically. I pray every night for guidance and answers but have yet to find any.

10/19/2011

It's been a hard struggle this time around. It seems we're doing more fighting with the hospital than Liam is getting care. Their just trying to treat him like a normal kid and pretend he doesn't have CDH. It's B.S.. They just keep shouving food down him, putting him in pain. He spends all night during his continuos feeds fussy and cranky. I spend that time holding him and comforting him. He vommited again tonight. We were only half through his feed when he lost it. It looked like old curddled milk, even the PCT comitted that it looked wrong. That was arpund 4:30pm. His doctors here at Childrens never came in to talk to us. We can't get any doctor to sit down and talk to us to brainstorm or come up with a game plan. They wanted to discharge us today but we refused to let them after he vommited last night. We told them that we weren't taking him home until he was on full feeds and held them down for 24 hours. Suddenly after that converstation they were forcing 70mls down him when he was only taking 60mls before. They're trying to rush us out and not fix the problem that Liam has. I've been arguing with Justin over what I think is best for his health care. We're trying to do everything we can to do whats in the best interest for Liam and sometimes that means comprimissing. I aggreed to let Valley Childrens try to fix Liam. Now were trying to figure out what our next move is. We've talked to the charge nurse, on call doctor and a social worker tonight about our concerns and it seems were wasting our breathe. I pray tomarrow will yeild more answers and a solution. Liam is in God's hands and I pray that God leads us to make the decisions that are right for him. To think this all started because Liam cought a cold while at UCSF. I think thats why it's been a hard decision to make on wether or not to take him back there. Please keep Liam in your prayers.