Day 9: When all else fails, try new things

Since Liam failed tolerate higher calorie formulas we've been left with no other options but to try a caloric addative. Duocalorie is a powder that adds calories to just about anything. We started adding it to his formula around 1pm today. We are starting with making the 20 calorie formula into 24 calorie. We started off lower just to make sure he tolerates this. Using this adative has his benefits: Liam will get the calories he needs without thickining the formula or going up on volume Its a quick way to allow him to grow faster (if it takes) But everything has its risks: Caloric adatives can slow down the digestive system and Liam is already slow. If not watched carefully Liam could get too many calories resulting in him getting fat. Allowing Liam to get too fat will cause pressure on his lungs and heart. Which would make it harder for him to breathe and we want tto make it easier. Liam will have weekly weight checks for awhile, then bi-weekly. His growth is and will continue to be closely monitored. The doctors like to just tell me how his weight gain is but stray from talking about his size compared to others his age. Today I was reading through facebook post when saw a FB buddys 4 month old son was right where he was supposed to be in weight and length. It really put things into prospective for me. I wasnt aware he was so far behind until I saw this. My 10 1/2 month old son is the size of an average 4 month old. Thats 6 1/2 months behind. Im not freaking out, theres nothing I can do that im not already doing. I just like having all the facts. Besides I was always on the small size growing up, born 4 pounds. Liam was 2 pounds 4 ounces at 37.5 weeks. He's come a long way and doing pretty darn good. He is truely the under dog, and I always root for the under dog. One day little Liam will sprout up and no one will ever be able to guess he had been so small. If not, he'll just be short and win the girls with his eyes lol. Another new thing Liam yried today was peaches. They didn't go over well. He is not a peach kind of guy. Is it bad that I want to have him try everything, even chocolate cake, in hopes that there is something out there he likes and is enthusiastic to eat? Im just going to pretend you all said "no". I talked to Tablezo, the pulmonoligist, first thing this morning. Finnaly after explaining where we live ane the air conditions AGAIN, she gets it. She said on bad air quality days we will have to turn up the oxygen for that day. When Liam is having his asthma attacks we can go up to 2 litters to help him out. She wants his base line to be half a litter if he tolerates it and for him to have time off turning the day but if I see any signs hes not tolering it go back up. This is in hopes that it will keep him out of the hospital. Her other recommendation is that we move. Its something we are working on. I explained that to her as well as the fact that the trip to UCSF drained us of our savings which we were useing to move. I talked to the nutritionist as well. He was the one that suggested Duocalorie over other adatives. Dr Lageate talked to case management who is working on getting Liam a pulse ox monitor but isn't sure if they will be able to get insurance to cover it. She also explained that we are back at the point of him tolerating everything we were doing at home so she wants to discharge him tomarrow. Even if he doesnt tolerate tbe duocalorie because at home he was only on 20 calorie while he tried to figure out what else to do. Unless something massive happens then we get to go home tomarrow. That makes me happy. Unfortunately ill be taking Liam back to a sick daddy and sister. I gave strict instructions for daddy to spray the air freshner/germ killer through out the house both tonight and tomarrow morning before we get home in hopes to kill off anything airborn and so the spray wont affect liam. A few things you can pray for: Liam tolerates Duocalorie That insurance pays for Duocalories as well as a pulse ox Pray daddy and sissy wake up in the morning not sick That Liam doesnt catch whatever it is they have That we find a way to move back into town for Liams sake That this will be our last hospital stay until after Liams first birthday Sent from Yahoo! Mail on Android

Day #8 Mommy was right

We had a really long night last night. At 4:45pm yesterday they weined Liam's oxygen down to .75 litters. Within half an hour I started seeing it effect him. He became extremely tired but restless. He tossed and turned all night long, cried out as if in pain. Within 30 minutes he seemed to no longer be able to breathe from his nose. It sounded congested so I had them suction him. Nothing came out. He's not congested his nasal passage is swollen. This is another thing that happens when we try to wein. I had fed him solids at 6:30pm. He ate 1/4 a tub of bananas, 1 bite of mashed potatoes, and 3 bites of peas. 20 minutes later he threw it all back up. I warned the doctor that weining wouldnt go well. I told her all this would happen. Ofcourse I didn't want to be right and in a situation like this being right feels bad. So DR Ligeate said to turn his oxygen back up to 1 litter, but not before she got her two cents in. "You know he shouldn't react this way right?" She asked me. "Ofcourse he shouldn't but the fact is he does" I responded. "I understand the ultimate goal is to conpletly wein him off oxygen, but I cant stand here and let it be done at his expense" ˝thats not my goal. My goal is to give him the healthiest lungs possible. Oxygen is toxic, albuterol isnt˝ she explained. "Albuterol treatments every 5 minute's wasn't working. We have to do whay works. Oxygen is working, albuterol isnt. If you want him off oxygen your going to have yo find a way to keep the pressure going into his lungs. We know he needs the pressure. UCSF was trying to do pressure at home without oxygen when he had his big desatting episode." I told her. "Turn his oxygen back to one. Ill write the orders to not wein and have Tablezo (pulmonologist) come talk to you tomarrow" with that she was gone. She did throw in a shot at me before she left. "Does he sit?" Lageate "Yes but hes wobbly and not really on his own" me "Its jusg everytime im in here he's on his back. Do you work with him?" Lageate "Everyday most the day. You ever notice everytime you walk in im eating lunch?" Me I think her pride was hit that she was proved wrong and that I called her on it. Not that I blame her but come one. We have bigger issues than me doing physical therapy with Liam. I can't work on his sitting while im eating. Im have to eat and am allowed a break. I dont leave him and go off walking, shopping, getting my nails done (even though I probably should take some time for myself). I sit here and play with him, soothe him, make him feel better anyway I can. I do take a little offense to what she said. I tried not too, but Im only human. Liams oxygen went back up around 1pm. Within the first hour of increase, his nosal passage started opening back up. He became happier and less whinny. By the end of the second hour, he was breathing through his nose again. Started rolling around the crib playing and jabbering. He got up onto his knees like he was going to crawl. And even fell asleep for a nap without fighting it. Tomarrow is another day. Ill be making my case with pulmonology, praying they see reason. If they would just work with me, together we could figure out whats best for Liam. Tomarrow Lageate will talking to case management about getting a monitor for Liam as well as talking to nutrition about getting the right caloric adative for Liam. Which means I will also have to talk to case management and nutrition. We wont be going home tomarrow. We probably wont be going home on thursday. If we're lucky we will get to go home by monday. But when I take him home I want to know we did everything we could this admittance. I text Liams pediatrician this morning to let him know that Liams been in the hospital. He said he'd call them for info and told me not to give up no matter how frustrating this gets. That we will find answers. He even sent me a pic saying that it "always got me through hard times and adversity". It made me smile, and gave me more courage to put my foot down today and stand up for what I believe in and give my son a voice. Even though that frog is being swollowed, he's still fighting back and will continue until his last breath. Its inspiring. As far as changes go, increasing his oxygen back to 1 litter is all thats being done today. "Slow and steady wins tbe race" snd we will win. We're going to kick CDH's @$$ and come out victors. We are declaring war and intend to win. To all the families out there affected by CDH, stay strong, stay possitive. Sent from Yahoo! Mail on Android

Journey through pictures: hospitalization #9 days 1-7

Day 1: liam self impossed tummy time Day 3: practicing sitting on his own Day 5: visit from daddy and sissy Day 7: eating soluds and hanging out with mom Sent from Yahoo! Mail on Android

Oxygen/Albuterol toxicity

If you would have told me a year ago my son would be born with CDH, I wouldn't have believed you. If you would have told me id be fighting with doctors I would have said you were lying. If you had predicted that Liams birth as well as his first year would have put our family through trials and tribulations and that it was only the begining I would have told you to shut up. If you would have predicted all those things and I was sitting here now, Id ask you to give me the winning lotto numbers. I never thought this could happen or would happen. And I never thought Id be willing to record it for the whole world to read. Circumstance changes you. Its changed me to the very core of my being. Before I never would have argued with a doctor. Now it seems thats all I do. Im no longer the girl who avoids confrontation and keeps to herself. I am now the woman who isnt afraid to stick up for her child, even if it means yelling. Im still quiet and a people watcher, but that veil lifts when the need arrises. I state my complaints and frustrations. I am forever changed. They just lowered Liams oxygen from 1 litter to .75. I agreed to let them go down to a half a litter. I fought and fought with them. Liam is still desatting, why would you lower the oxygen? I had to do research to understand what they were saying about oxygen being toxic ( http://en.wikipedia.org/wiki/Oxygen_toxicity ) . Its toxic at high levels for extended periods of time. I get that now. What I dont get is weining while hes still desating. They actually told me that albuterol isnt toxic. I researched online and albuterol is meant to be a short term use drug because if used chronically it can make symptoms worse instead of better having a paradoxical effect. Not good because liam uses every 4 hours every day. ( http://www.livestrong.com/article/113319-albuterol-side-effects-children/ ). Albuterol toxicity includes tachycardia and flushing of the skin. The doctors claim that albuterol not being toxic is a lie. I personally can't wait for our pediatrician to Get back in the office tomarrow so I can catch him up on whats been going on. He doesnt even know liam is in the hospital because I didnt want to ruin his vacation. We also found out that Liam should never have been discharged on oxygen and trying to wein without a pulse ox monitor. She also doesnt want to discharge him until they can get us one. So please pray our insurance approves it because I have no clue what we're going to do if not. It seems like at every turn we get stuck between a rock and a hard place, never getting a break. If theyre refusing to pay for 8 tube extention and only doing 6 then I doubt theyll approve the monitor that we keep getting told he needs. They expect us just to be able to go out and buy these things but they dont understand tbe situation of having 2 unemployed parents trying provide for a family of 4. WIC stops giving formula the month Liam turns 1. As it is were not getting enough formula through them and I have no idea what were going to do for his formula then since he isnt eating by mouth but a few bites. If only we could stop all these hospital stays so I could stay home with Lanie and Liam and Justin could go back to work. This is being hit by circumstance. Some people never have to know what it means to struggle. Those who struggle, end up stronger in the long run. Our family wasnt even hit the hardest by CDH. There are other families out there whose marriages don't survive the destruction CDH causes. I pray for strength for families stuck in this tornado. Its all I can give for now. I see alot of parents here at the hospital. A mom sitting on a bench trying to feed two small children while a third is somewhere in the hospital sick. Another mom struggling to smile for her son on the playground while worrying for her baby. Others crying thinking theyre alone. I pray for then all. I dont know theyre situations but I do know how it feels to watch your kids be sick and in the hospital. No matter what their reaso for being here is, it still hurts. They only thing we can do is pray and stay strong for our kids. I dont know if ill ever get over the pain the last year has brought me. I dont know if ill be able to laugh about all this one day. I just pray we get through it with as little damage as possible. For now this is our lives, our rollercoastering tornado. On a lighter note, there is only 48 days until our little Liams first birthday! Its not just a milestone for us, its a huge event. Liam has survived so many odds. Its a true miracle. I was in labor from before easter to july 14 with this little man. It was painful and I thought id go crazy from pain and bedrest. I remenber it like it happened yesterday. Me sitting onvthe edge of the bed rocking myself and takinga deep breathes trying not to cry my eyes out. I can still feel the pain when I think about it. Boy was I happy when they said it was time. I dont think I could have gone another day. Every pain only secured the bond between liam and I making ig stronger. I love you little man. You already have us at your beaking call with one bat of those eye lashes and big eyes. Sent from Yahoo! Mail on Android

Calories didnt take/pulmonologys stupidity

Today is day #6 of hospital admittion #9. We tried 22 calorie formula on Liam today. He didnt tolerate it. When Dr Ligeate came by she had told me that pulmonology had called her saying they wanted Liams oxygen weined. Liam has been desatting twice a day so why on earth would you wein a desatting babies oxygen? Ive gone around and round with them over this. They didnt even have the gull to come talk to me face to face but instead told another doctor to do it. I will be requesting a meeting on monday to discuss these issues along with the fact that he got admitted monday and we havent heard from them until sat. Ligeate gets Liam. She understands that you have to take it super slow with him because everything little thing we do he reacts in a big way. We stopped the biotin and liam hasnt had an emesus in 2days. Thats one problem fixed, for now. Liam has lost half a pound since monday when he was admitted. Im sure tomarrow we will address using a caloric additive. Still no sign of Liam rash returning. His cough is still pretty bad. Really theres judt a few things left to do before we can go home: Get liam gaining weight Straighten out the oxygen situation Pray that insurance approves a pulse ox monitor because aparently we should never have been sent home without one since were supposed to try and wein him off. I know we wont be released before wensday. Im not rushing them or even asking a timeline. It was mentioned because case management wont be back before Tuesday. When we leave this time I want to stay home. Atleast for a month, well until after liams first birthday. The way everthings been going only points to us most likely being admitted for his birthday. Its hard to make it special if your in a hospital. If youd like to send liam birthday wishes you can email him at cherubliam@yahoo.com and ill read them to him. Thank you all for your continued support and prayer and sticking with us through this crazy hellish journey CDH is taking us on. Sent from Yahoo! Mail on Android

Taking it slow...Liam update

Liam is back on 20 cal. Weve officially tried every other higher cal and he cant handle anything higher. His prevacid has been increased. Theyve ordered a stool study to make sure he has no infection there, if he poops cause he hasnt gone since yesterday around noon. Liam did desat a few times today but hasnt in several hours. He vommited alittle bit, not even 1ml, after meds, great news. We DCed biotin and that looks like a key to his vommiting so yay for that. Hes even sleeping more comfortably. They do think its odd that liam seems to be allergic to biotin that his body needs, and suddenly at that, but given liams past on never following the normal paterns for anything they arent too concerned. Still theres no talk of going home. We have a doc whos has liam before so she knows him and is taking it slow knowing that for every one small thing they do, he reacts big. Shes trying to figure out the puzzle but seems a bit baffled and taking all my suggestions to heart. Buts thats also because she knows i know my son better than anyone. We know hes going to have to have a calorie additive because hes not gaining weight but we dont want to rock the boat with thst yet. That doesnt mean she wont decide to do it tomarow though lol. Please keep our little lamb in your prayers. Sent from Yahoo! Mail on Android

Biotin issues

I hate hospitals. More problems always occur during admitions. They smell weird. You have to sleep on hard surfaces with blankets that are scratchy. You get to see a doctor once a day for less than 5 minutes. Liam gets easily bored and cranky. When he finnaly falls asleep they wake him up. What makes this on the verge of unbearable is that I dont get to watch my baby girl grow up. I dont get to kiss her goodnight or make her feel better when she gets hurt. Im not there to make sure shes eating right or to read her stories. I dont dont get to play tea party or lalaloopsies with her. Not when im here at the hospital. And everytime I have to go away with Liam, i come back to an angry little girl. A hurt and angry little who. It breaks my heart. The question I get asked ALL the time: how do you do it? I always answer: its hard but we manage. The truth: its extremely painful and heart wrenching and isnt going without causing damage. I have meds that are supposed to keep me from being depressed but really only keep the emotions under the surface. What helps the most? This. Blogging, journaling, writing poetry. My sweet little girl is at home getting tucked into bed and im wishing I was there to cuddle with her. I know what your thinking and ive heard it all before. Ghe hospital is only an hour away. I could easily drive back and forth every night. I guess for some people thats an option. Not for us. We dont have the money for gas nor do we have a vehicle that could handle daily trips back and forth. We are working class family who have been hit by one bad circumstance after another leaving us both unemployed. That makes the stress a whole lot worse. Its still not a complaint. Its just life. We are a family that survives and have stuck together through "hell". Yes I wish things were better, easier, but who doesn't. The doc is now thinking Liam has an allergy to biotin. For a normal person, youd just carry around an epi pen and try to avoid foods that have biotin in them. For some with Biotinadase deficiency, like Liam, this is bad. His deficiency is only a partial so were not exactly sure how this is going to play out. We do know he needs to take the biotin in order to break down and obsorb foods to grow and we also know that missing a few doses won't really hurt him. What we dont know is how long he can ho without it and if he truely is allergic what were going to do. So were waiting on genetics to give us some insight. Liam is still doing basically the same, except for vommiting up his biotin. And this could turn out to be a coincidents, one really huge one. Im waiting and trying to do so patiently. I dont want them jumping to conclusions. If you could, please pray: 1. That Liams is not allergic to biotin 2. That he stops vomitting 3. That he has no more asthma attacks 4. That his rash and cough go away 5. That we find out whats causing the rash, cough, vommiting, asthma attacks 6. That liam gets a medi port soon so he doesnt have to be poked anymore 7 that we get to stay home for longer than a week before his next admittance and that he doesnt have to spend his first birthday in the hospital Thank you for your continued support and prayers. Sent from Yahoo! Mail on Android

Hospital stay #9 underway...liam update :(

Have you ever wanted to move on past something but are literally stuck? Thats us. I kept holding out hope that all the "rumors" about how things would get better after Liam turned a year, how things would be more "normal" with less hospital stays, would be true. Ive prayed and prayed until ive fallen asleep crying for this. I want it so bad it hurts. It hurts to breathe. Liam is 10months 8 days old and there is no normal in sight. We took Liam to a park on saturday. No one else was there. It was later in the evening, cooler, no wind. I decided to take his 10 month old pics andhis very first pics outside. This was a huge event for him. I was so excited. For once he could act like a normal boy. I laid down a blanket on the grass for him. He laid there looking at everything as I snapped pic after pic. He managed to get a handful of grass. He didnt know what to do with it but stare at it then drop it on himself. I thought to myself "i cant believe were here. I cant believe were finnaly getting to be semi normal". I didnt know how wrong i was at the time. Later that night he started having asthma attacks. He wasnt even moving air within his lungs. I did chest pt and treatments and he seemed fine. I had been 2min away from calling 911 when he recouvered. The only reason I didnt rush him to Children's was because I was warned he was asthmatic and this could potentially happen. He seemed fine all day sunday except he slept all day. When night came he started having asthma attacks again. I was up all night giving pt and treatments. At 6:30a mon he got worse. Headed up to children's around 8:30a. By the time we got there at 10a he had had 8 breathing treatments. When we took him out of the car, he was covered in a rash. From ER he was admitted. They think he has a virus but its speculation. They think that the fact that this happened the same day we took him to the park for the first time was a coincedence. Personally I think theyre wrong. Even though I loved every milla-second of our time there, im blaming myself. I should have known better, thought harder. I captered many wonderful pics of liam enjoying himself, and im glad I did because it wont be happening again anytime soon. His birthday that I wanted to have at a park because we were going to invite alot of people will have to be held somewhere else, possibly even canceled. Another sword in my heart. I wish we could get passed this point of having to issolate and stuck at a hospital every other week. Instead of growing bigger and healthier were encountering more medical issues. The benadryl has worked amazing for the rash and cough, another reason im sure its no virus. Now we have yet another problem. Liam is Biotinadase defficient and needs biotin supplimants. Everytime we give him biotin, he vommits. And some signs of an allergic reaction are respitory issues and rash, which could be coincedence. It scares me because i have no clue what ws can do if hes allergic to biotin. Ive searched the net but theres nothing for a biotinadase defficient person whos allergic to biotin. If a normal person is allergic you just avoid food that have biotin in them and carry an eppy pen around. We cang draw blood for his allergy test because Liam has no veins left to poke. Hes been poked so much his veins dont bleed or hold ivs. He needs a medaport but cant get one until hes not sick and liam seems to never be not sick. My hands seem tied. A few hours of "normal" family outing to a park has lead to this, liams 9th hospital admition, and the unveiling of yet more medical issues. When will it be enough? When will Liam get to do all the things other little boys and their families get to do? I know God has a reason for everything and we have to trust in him. We need a life outside of hospital stays and doctor visits. I wont lie, this is hard. Its taking its tole on me, liam and the fam. I blame myself for everything. Liams cdh, all the hospital stays, evrrything. I have to believe God hasnt done this to be cruel that im at fault otherwise God would just be cruel and ugly instead of forgiving, loving, savior. Whatever I did I wish I could change, take back. Im in an awful place right now, struggling with it all. My son needs a break. He deserves it. Ive gone from praying to begging for liam. Its hatd to have the doctors shrug theyre shoulders and say "i dont know" all the time. Is my son really the only cdher out there baffling the doctors? They act like hes a rubics cubs tbyre trying to solve only its missing all its stickers. I lay everything out for them on a silver platter, its no holds bar when it comes to liams symptoms and still they judt dont know. Im frustrated. Angry. Depressed. Sent from Yahoo! Mail on Android

Phnemonia, compromised lungs and more

Liam had a bad night last night t. Had a fever then vommitted because a nurse put his feed in gtube instead of jtube. Docs drew blood to check his immune system. Cultures from his bronch are growing something but we wont know until mon exactly what. We are starting antibiotics. His bronch also showed upper airway is floppy. Ct showed that his left lung is way under developed. His right lung is compromised by the diaphragm being so high and not allowing the lung to fully expand. Because of this secretions arent able to be moved and stuck there leaving him very subseptable to infection and phnemonia. There is phnemonia in his right lung. The cf test was also done this morning and hopefully results will be in by 2p. We are getting discharged so we might not be here to get the results. Everything that theyd do here for liam can be done at home because he has home oxygen. Kinda scared about the cultures from his airway. Pulmonary hypertension was negative thank God! Pulmonology said basically liams working off 1 lung and we just need to make him grow fast. Were also going to start short periods of time off oxygen to strengthen his lungs. They know his immune system is compromised because of all the infections but we wont know more until the lab work comes back. So now its even more important that everyone washes and sanatizes their hands before touching liam. And if you have a sore throat, cold, flu, allergies, even itchy eyes or so much as sneezes you cant be around liam. I just had a doc tell me that. Ofcourse justin, lanie and I because we live with him have to wear mask if were sick. So fair warning if you show up on my door step with any symptoms ill probably bite your head of and send you on your way. Just thought you should be warned. The doctors were very serious about this. Sent from Yahoo! Mail on Android

I'm leaving on a jet plane....

Or rather: we're leaving in a packed down yukon lol. Liam and I headed off to Childrens this morning. We only had to wait a total of an hour and a half to get in a room. Thats gotta be a record. Before we left I got in some cuddle time with Lanie then we gave kisses and headed off. Ofcourse this would be more interesting with all the pics. We stopped by uncle and tias so they could give kisses, and I needed a pit stop, then we were off. We got into the room around 1:30 and its been busy since. So many questions to answer.....again and again and again, supervision on my part so they dont put the wrong meds in the wrong tube and so forth. And low and be hold one of the docs doing his admittance is an old friend. How old? We use to play barbie dolls together. Pays to be nice to people. Not much happened. They didnt find a good vain at glance today so they decided to wait on the iv. I was approached with them asking about doing a medi-cath so its stays in and he doesnt get poked. I dont know what to do about that. I need guidance and prayers for that one. CT is scheduled for wensday because they didnt have an opening for tomarrow. Looks like im going to have to put my foot down and demand they do the CT and bronch at the same time so he only has to be intubated and sedated once. Liams J tube is acting up and getting clogged then backing up into his stomach. He was supposed to have an  xray but they never showed. Then when we vented his gtube really think green mucus came out and the nurse is thinking CF too now. Then liam decided to throw up on us. Now hes fast asleep lol. Glad today's over. Sent from Yahoo! Mail on Android

Let the testing commence

Liam was discharged Friday April 27th from UCSF. The ONLY thing they did was send him home on oxygen. He was on half a litter when we left but since has been uped to 1 litter. Half a litter kept him from desatting but didn't help him much otherwise. 1 litter has taken away most of his "asthmatic episodes" of coughing and struggling. Monday he will be admitted to Valloey Childrens, again, where we will run several test:

CT scan of his lungs
Bronchioscope of his ariway
Sweat Test for Cystic Fibrosis
ECHO for pulmnary hypertention

On an xray that was taken of Liams lungs about 2 months ago or so, scar tissue was reportedly seen on the bottom of his right lung. Hopefully the CT will show more and be able to tell us more about this. Were praying that there is nothing wrong with his airway because if there is, he might need a trach. Liam has alot of symptoms of Pulmonary hypertention. We pray he does not have PH but if he does were praying they catch it before its critical and untreatable. Pulmonary Hypertention is something that kills alot of CDH babied before they even get a chance to get their repair. PH is also something that sneaks up on some CDH babies when we all think that theyre in the clear. We were forwarned to keep an eye out for PH. We are also testing for Cystic Fibrosis because our Pediatrician wants to cover all the basis. Even when Liams breathing is under control he is still very slow at putting on weight and CF is the last thing our Ped can think of that would cause slow weight gain.

What sucks is that Liams symptoms match both PH and CF. And patients with CF generally have PH as well. Liam does have alot of mucus in his lungs and airway (like CF) and when weve been qble to suction it out its been very thick (just like CF).

We, I, am asking that you keep our little Liam in your prayers. He's a fighter and has given us such hope that he will continue to fight. Things are hard on our family, being split up most of the time with Liam and I at the hospital and Justin and Lanie at home. It's hard for Lanie to understand and has told me during our recent stay at UCSF that she just wants me to leave him there and come home to be with her. Because of the strain on our family I am also asking that you keep us in your prayers and pray for strength and understanding as well as patience. God answers prayer and Liam is proof of that. Prayer has gotten us through the worst times of our lives and I have faith that prayers will continue to be answered. We sencerly thank you for your love and support of our little Liam.

                               (Nurse Jen is the best nurse on the 7th
                                floor at UCSF. She quickly became like
                                family.)

                                    (Nurse Jen cuddled as often as she could)

                                 Liam is a typical boy picking on his sister

A glimpse into the future? Dr Liam has a nice ring to it

Playing with his food not actually eating it.

Sissy pretending to have a nasal canula like Liam and copying him.

                                    



More info on the conditions mentioned above:

Pulmonary Hyertention is rare and can be confused for asthma and COPD. (Those of you that have followed Liams story know that he has been diagnossed as asthmatic just because he has asthma like symptoms and asthma runs in the family. Asthma has since been basically cast aside)

In medicine, pulmonary hypertension (PH) is an increase in blood pressure in the pulmonary artery, pulmonary vein, or pulmonary capillaries, together known as the lung vasculature, leading to shortness of breath, dizziness, fainting, and other symptoms, all of which are exacerbated by exertion. Pulmonary hypertension can be a severe disease with a markedly decreased exercise tolerance and heart failure. It was first identified by Dr. Ernst von Romberg in 1891.^[1] According to the most recent classification, it can be one of five different types: arterial, venous, hypoxic, thromboembolic or miscellaneous.

Because symptoms may develop very gradually, patients may delay seeing a physician for years. Common symptoms are shortness of breath, fatigue, non-productive cough, angina pectoris, fainting or syncope, peripheral edema (swelling around the ankles and feet), and rarely hemoptysis (coughing up blood).
Pulmonary venous hypertension typically presents with shortness of breath while lying flat or sleeping (orthopnea or paroxysmal nocturnal dyspnea), while pulmonary arterial hypertension (PAH) typically does not.
A detailed family history is established to determine whether the disease might be familial. A history of exposure to drugs such as cocaine, methamphetamine, alcohol leading to cirrhosis, and tobacco leading to emphysema are considered significant. A physical examination is performed to look for typical signs of pulmonary hypertension, including a loud S2 (pulmonic valve closure sound), (para)sternal heave, jugular venous distension, pedal edema, ascites, hepatojugular reflux, clubbing etc. Evidence of tricuspid insufficiency is also sought and, if present, is consistent with the presence of pulmonary hypertension.

All info listed was from Wikipedia. If you'd like more info just click here.



Cystic Fibrosis:

Cystic fibrosis (also known as CF or mucoviscidosis) is an autosomal recessive genetic disorder affecting most critically the lungs, and also the pancreas, liver, and intestine. It is characterized by abnormal transport of chloride and sodium across an epithelium, leading to thick, viscous secretions.^[1]
The name cystic fibrosis refers to the characteristic scarring (fibrosis) and cyst formation within the pancreas, first recognized in the 1930s.^[2] Difficulty breathing is the most serious symptom and results from frequent lung infections that are treated with antibiotics and other medications. Other symptoms, including sinus infections, poor growth, and infertility affect other parts of the body.
CF is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR). This protein is required to regulate the components of sweat, digestive juices, and mucus. CFTR regulates the movement of chloride and sodium ions across epithelial membranes, such as the alveolar epithelia located in the lungs. Although most people without CF have two working copies of the CFTR gene, only one is needed to prevent cystic fibrosis due to the disorder's recessive nature. CF develops when neither gene works normally (as a result of mutation) and therefore has autosomal recessive inheritance.
CF is most common among Caucasians; one in 25 people of European descent carries one allele for CF.^[3][4] The World Health Organization states that "In the European Union, 1 in 2000-3000 newborns is found to be affected by CF".^[5] Individuals with cystic fibrosis can be diagnosed before birth by genetic testing, or by a sweat test in early childhood. Ultimately, lung transplantation is often necessary as CF worsens.

The hallmark symptoms of cystic fibrosis are salty tasting skin,^[7] poor growth and poor weight gain despite a normal food intake,^[8] accumulation of thick, sticky mucus,^[9] frequent chest infections and coughing or shortness of breath.^[10] Males can be infertile due to congenital absence of the vas deferens.^[11] Symptoms often appear in infancy and childhood, such as bowel obstruction due to meconium ileus in newborn babies.^[12] As the children grow, they must exercise to release mucus in the alveoli.^[13] Ciliated epithelial cells in the patient have a mutated protein that leads to abnormally viscous mucus production.^[9] The poor growth in children typically presents as an inability to gain weight or height at the same rate as their peers and is occasionally not diagnosed until investigation is initiated for poor growth. The causes of growth failure are multifactorial and include chronic lung infection, poor absorption of nutrients through the gastrointestinal tract, and increased metabolic demand due to chronic illness.^[8]
In rare cases, cystic fibrosis can manifest itself as a coagulation disorder. A double recessive allele is needed for cystic fibrosis to be apparent. Young children are especially sensitive to vitamin K malabsorptive disorders because only a very small amount of vitamin K crosses the placenta, leaving the child with very low reserves. Because factors II, VII, IX, and X (clotting factors) are vitamin K–dependent, low levels of vitamin K can result in coagulation problems. Consequently, when a child presents with unexplained bruising, a coagulation evaluation may be warranted to determine whether there is an underlying disease.^[14]

Lung transplantation often becomes necessary for individuals with cystic fibrosis as lung function and exercise tolerance declines. Although single lung transplantation is possible in other diseases, individuals with CF must have both lungs replaced because the remaining lung might contain bacteria that could infect the transplanted lung. A pancreatic or liver transplant may be performed at the same time in order to alleviate liver disease and/or diabetes.^[76] Lung transplantation is considered when lung function declines to the point where assistance from mechanical devices is required or patient survival is threatened.^[

Quality of life
Chronic illnesses are very difficult to manage. According to Schmitz and Goldbeck (2006), the fact that Cystic Fibrosis significantly increases emotional stress on both the individual and the family, "and the necessary time-consuming daily treatment routine may have further negative effects on quality of life (QOL)".^[92] However, Havermans and colleagues (2006) have shown that young outpatients with CF that have participated in the CFQ-R (Cystic Fibrosis Questionnaire-Revised) "rated some QOL domains higher than did their parents".^[93] Consequently, outpatients with CF have a more positive outlook for themselves. Furthermore, there are many ways to improve the QOL in CF patients. Exercise is promoted to increase lung function. The fact of integrating an exercise regimen into the CF patient’s daily routine can significantly improve the quality of life.^[94] There is no definitive cure for Cystic Fibrosis. However, there are diverse medications used such as, mucolytics, bronchodilators, steroids and antibiotics that have the purpose of loosening mucus, expanding airways, decreasing inflammation and fighting lung infections.^[95]

In addition CF patients cant be around others with CF because the bacteria in one patient can be spread to another causing death.

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