Friday, December 31, 2010

New Year's Eve and the terrible, horrible, no good, very bad day

We had a bad day yesterday but today has not been so great either..looking at the positive though.. he is stable most of the time today and is not on the o2 and is sleeping at the moment.. or actually all day really.



Starting with yesterday.. He had a picc line placed in the morning and of course I knew going in that there would be complications..there always are. Sam is never predictable except in that I CAN ALWAYS count on him being unpredictable and suprising everyone by what we have begun to call "misbehavior". For those reading who are new to Sam..misbehavior is when Sam decides to stop breathing or his heart rate plummets out of the blue (no pun intended). This is called Bradycardia and for Sam who normally has a heart rate in the 120-140 range (normal for babies), bradys are times when his heart falls dangerously low..officially below 100 but not symptomatic on him till in 70 to 50 range. Picture below is example of him dropping from 130s..it eventually stopped this time around 70s and then headed back up so not a biggie. He does this fast and with no warning and sometimes he does not turn back up too well. This is bad in that his heart has stopped before for short periods which is called asystole. I know you were not planning on MED101 class but Jason teases me that I have gotten so comfortable with the terms that I don't take in the general public's learning curve on this stuff.




So yesterday I counted on more "bradys" during the picc line placement procedure down in interventional radiology. They do it with basic anesthesia and they usually only require an LMA tube placement which just keeps his airway open at the mouth and top of the throat. I made sure they knew with mito kids they have to take malignant hypothermia precautions. As usual, when Sam is given meds to relax...he really relaxes. So, he had to be intubated and that was our first unplanned aspect of the procedure. Then they found what was thought to be pneumonia in the top of the left lung and then to make things more fun, the usually easy insertion of the PICC Line was complicated by him not clotting well. This Picc line is a central line to an artery near his heart which will allow easier and less damaging access for giving him medication and TPN (liquid food). The radiology Dr said he had to put pressure on it for a while to stop the bleeding and it was not an anticipated situation but he kept telling me he could see how complex Sam was and how sorry he was for our anguish. Very kind man. What situation with Sam is EVER an anticipated one?? Maybe the blood clot issue is related to his dysautonomia or one of his many meds.

They did get his blood to finally clot and then were able to extubate him but he required 02 through the night. He has been off it and only has occasional bradys and desaturations of his oxygen levels at this point.




Part 2 of the terrible, horrible, no good, very bad day (I love that book..) was a not so thoughtful Dr who needs some "sensitivity training", as the social worker gal put it who always checks in on us. This guy was consulted for some reason and I have no idea which specialty but he came in and was having me fill out an insurance claim form he needed when he asked me about Sam's new diagnosis about the mito disease stuff and while here went on to say, "do you have other kids?" yes I said..two daughters 5 and 4. "are they healthy?" yes.. we are very blessed that they are very healthy. He then proceeded in a flat and rude manner to say, "good..cause it does not look good for him." and with that turned and exited our room....AHHHHH. WHO DOES THAT????

Lord, thank you for giving me strength to control my anger at that moment. Before Sam's birth I never experienced such crazy interactions as I have this year. I know I am the LAST person in the world to make judgement about what people sometimes say or do thoughtlessly as I am the one who often puts my foot in my mouth during awkward moments but I sure hope I am never guilty of knowingly walking into a critical child's room and wreaking unnecessary havoc on a beaten up family's emotions. I wasn't born yesterday and I KNOW Sam's situation is serious and he certainly could have a bad prognosis. NO - I would not want a Dr sugar coating anything but my goodness..I don't need that kind of negative attitude from someone clearly NOT that involved in his care or even looking for solutions. Which.. brings me to my last issue with that interaction. I had the social worker looking up who this guy was and they could not figure out from his signature who he is.. should I be concerned that this random guy in a badge made me fill out insurance forms and signed intials KM in the Dr. field of the consult spot on the chart?? There are soo many in and out of here that I often can't make hide or hair of who is with what service. I usually ask but I was so tired and then he was gone in a flash. I guess I just needed to vent about that and let it go..

Today we also had another chest xray and rediology told us it was pneumonia but the pediatrician thinks it is atelectasis?? which is when the bronchi are not being fully expanded with his breathing and so condenses in and that causes the cloudiness on the x rays? either way she said antibiotics are on board.

Big part of today was the Gastric Emptying Study under flouroscopy. He failed. It took him 121 minutes to move less than 2 ozs (6 ozs is a regular feed for him)from the stomach to the intestines by g tube. UGH. He will likely require a G/J tube placed to bypass the tummy and go on to the intestines. But my issue is also that the intestines are always really slow too so will it solve anything? He is on laxatives 3 times a day and colace stool softener and still sometimes goes like 9 days before he stooles. UGh. They can do a pyloro plasty but that is permanent and I so hate permanent stuff plus if this is an energy muscle issue.. will it solve anything to be just opening that up more so it can sit in traffic in the intestines? Plus he might not have good energy stores to get through another big surgery. He coded a bunch of times after the last one and then stopped tracking for two months..way too stressful for our little man.

On a sweet note..Sam does not laugh out loud but he has the sweetest impish squicky laugh that can only be heard with strong ears but has the biggest smile you ever saw when you play the animal finger puppets a friend sent us..Thank you Roake Clan! He loves them! His nurses have even jumped in on the act. We can't thank all those praying for Sam enough for all the prayer..we feel it and know you are the reason Sam stays strong. He is teaching me everyday about joy and God's gifts.. real gifts are love and family and friends and connections..all the other junk can just go out the door. If you don't have love and build bonds and enjoy the moment and learning from eachother and God..you have nothing. But through God... we can do all things! So that is where we are today.. terrible day or week as it is right now..we can get through it and onto bigger and better days.

God works in mysterious ways. First, He brought a kind friend to my rescue yesterday right after the not-so-nice Dr left..just as I was needing to get us checked into the ron Mc donald house here so that when Jason arrived with the girls we would have a resting place for the weekend. I needed a ride over there and was a little down and flustered. She called at just that moment and said.."guess what, I am right across the street." I can't believe she drove an hour with her family of 5 to have like 10 minutes with me but it was such a blessing and her family watched the girls while Jason and I got to pray with Sam at his cribside last night. It is rather rare for us to be able to do this as siblings can't be in the PICU. SO I thank God for her friendship and for all my friends who have been such a HUGE support.

Secondly, we ate dinner together as a family at the house as we were unable to reenter during shift change. That is a nervous time for me to leave Sam alone and then in walked Richard Lamar, a friend from Highschool whose baby was born 11 weeks early when his wife was forced to deliver due to preeclampsia and HELP syndrome. Their baby is like 2 lbs and in the NICU here. She is doing ok for now but Lord, please watch over baby evelyn and all those innocent little ones in the NICU. I hope we can be of support to them when they need it as we have become pros at some aspects of this experience. Their family needs our prayers and I know as this is their first child, that they are probably really overwhelmed.

The last little info I am trying to figure out is the plan for moving forward..should we be transfering to shands or atlanta and can we do that with the new insurance not in place and the old ending today? His GI surgeon is at UF and we loved him but hated neuro there for delaying the biopsy and dismissing him as a newborn who would probably "grow out of this" plus they kinda got to a stuck point and left us with the tracheomalacia diagnosis and no other ideas. They left me with the impression that likely the airway issues were caused in utero, at birth or after and this led to neuro damage leading to more systematic damage which could still be how the mitochondria started dysfunctioning but seemed they gave up on more investigation at some point. For cardio surgery if pacemaker was needed and for the mito GOD we loved atlanta...and so that is another good option. They only have one surgeon and a handful of outpatient type GI guys and so resources are limited. They can't even place the GJ till the surgeon returns from vacation next week...boy we live in a local yocal area if the only surgeon at the biggest hospital we have an hour from home is not even on-call!!

I need to see about how insurance issues would work with the agent.. He called today to say maybe we should sign up for cobra as a 3rd level of surity that we don't have a gap in coverage while we wait for independent insurance coverage.. I am sooo nervous. We can't afford ambulance again as we have been slammed this year in paying for those each time so would that mean holding our breath while we drive 6 hrs with IV poles out the back window and tanks in tow? yikes. What to do.. What to do.. well I will sign off for now..God Bless.

Thursday, December 30, 2010

Tank is empty and Sammy is smiling

If you are not up to date on where we are now with everything..scan on down to last night's blog and info about the news from the diagnosis.

Update this A.M.:
Sam had a MUCH easier night with nothing in the Tum Tum. He was even smiling a bit. We got to come off isolation status as all the viral tests came back normal. Not a suprise to me. I don't really think he had a big bug. I think the slight fever from the vaccine put him in a weak state and the mito issues led to a total breakdown, the GI of which is usually last to hit. So we will have to figure out a new protocol for vaccines..I want to say no to them altogether but all these mito docs say for all the problems mito kids seem to have with them it would be FAR worse if they got exposed to one of the diseases covered by those vaccines. EEECH..need help on weighing out that one.

We get the picc line this morning and they said he will be getting some gas for the procedure but hopefully that does not put him over the edge as in the past. That should be in the next hour or so and then he can get much needed TPN. They said a permanent medport can be placed outpatient under the skin for long term central line use but a Picc line can be kept for up to a year I believe..one more thing to service at home though.

No reflexes in his right leg today. I know not to focus on the neuro stuff when I am already so worried about the gut issues, the bradys and the resp distress but I always worry the brain will loose precious energy every time we get sick. For all his limitations he seems very smart to us. I read an article that said these kids can have neuro decline in the 2 weeks following an illness because the body is sending whatever energy it can make to the illness, diverting it away from the organs that demand the most and so that is when his body starts breaking down. Gotta have some fuel and when your body does not make fuel well, then you end up stuck on side of the road.. or in our case this year.. the pediatric ICU.

My understanding is his brain is always starved for energy which is why he has had diffused encepholopathy readings on the EEG. Maybe that can turn around once we start the new mito cocktail that was ordered. Those meds are intended to improve the bodies ability to produce energy on a cellular level.

NEED YOUR PRAYERS.. and not just for Sam to get better but on a different level for a huge issue that is hitting us hard. Our very fancy $1650/mth group insurance policy is terming tomorrow and we have applied for a new independent insurance policy also through BCBS and hopefully will be a more reasonable cost and not more than a mortgage payment as is the current policy. We left our current agent when he negatively told us that even though the law says Sam can not be denied for prexisting conditions, that the insurance company will find a way to deny us..that would be fraud in our assessment so we went with someone who will fight for Sam's coverage.

We have been slammed financially from every direction this year and we need some prayers for peace and control. If we could obtain nursing aid at home I would able to help more with Jason's business and so we are praying for a solution on that level as well. Thank you for all the prayers. Forgive me for the long posts..I am missing my mother so badly as she is way down south visiting my sister. She is deaf I can't call her so this is the only way to communicate. God Bless.

Wednesday, December 29, 2010

What a way to start the New Year..

Sorry in advance..this is going to be a long one..



Yesterday may have been the hardest day ever for me..and let me tell you.. I have had a few by now :). But I also have had some wonderful life experiences and gifts to be thankful for and for which offset the worst times by huge measure.. I am emotional at the moment so I will probably end up saving this and waiting to send it out when I can remove any words of anger and "clean it up" for sake of the innocent.. We were already dealing with Sam's chronic return of illness in the past week. Basically a repeat behavior of the illness from 2 weeks ago and also of the one from before Thanksgiving. We worried we might be back in the hospital during Christmas but somehow we narrowly avoided it but are here now. Then on Monday, I checked my email and had a big whammy of news in the form of Sam's 79 page report of biopsy results ..I know.. I know..we knew it was coming.

I should have been better prepared. I had actually called to check about it on Thursday and they said all reports were running late and so I dismissed it and moved on to worrying about his worsening illness. The report was mailed to us on Christmas Eve - Thank you Lord for redirecting me away from my email at that time so I could clear my head and give the girls some joy without crying through our birthday party for Jesus.

I saw the email and almost instantly called Jason knowing what it was. He would not stay on the phone while we read it and so I called a dear friend to stay on the line while I went through everything. I did not want to read it alone..I guess that is how Jason and I differ most..he deals well in solitude and I am a need-some-support kinda girl. I was so nervous as I opened this 79 page report of mumbo jumbo that only the brightest Harvard chem grad could decipher, that I nearly missed the whole point. Sam has defects of Complex 1 and 3 (maybe something wrong with 4 too) in the mitochondrial enzymology chain. Probable Mitochondrial Disease. UGH. Tough to swallow but we had known it was suspected. Why is it so hard to see it in print? It was insane how much this report entailed in its 79 pages.. I barely got two paragraphs in and I knew scarey stuff was lurking..I have since read it all and realize that there is too much for me to even begin to understand. The results mention other issues as well but all is well over my head. He describes lab finding after lab finding and more data than I could even comprehend. One biggie is that most who have this do NOT get it from their mother as many believe. Most get it in an autosomal recessive fashion or as some form of mutation or gene damage.

There is still pending research on some aspects of his workup and the disease confirmation is listed under probable because they basically need to find the bad boy gene for final confirmation of primary mito disease..This mito dysfunction could all still be secondary from some insult or other pathology. They have a mutation they are investigating. I have about a million questions.. So we will hit on those when we go to do our follow up in person with Dr S or his nurse on Jan 10th in Atlanta.

The nurse in their office says it is probably good that we don't fit into the 5% crowd that get an exact "syndrome" diagnosis with all the gene confirmations and linked info because that can mean we are farther along in negatory territory..but I feel already in the few months since we did the biopsy we are already in far worse territory than when he was clinically evaluated by Dr.S for the biopsy. We did not have the major GI shutdown going on back then..uugh. which brings us to why we are back in the hospital ICU again today.

Sam's illness of last week started after 2 vaccines Monday (our first since age 4 months). We made a plan to spread them out slowly (much more on this in a later post). He had low grade fever and then no fun at speech therapy and sounded junky, labored breathing and this got worse over next 2 days with higher fever of 102 which went away but he was still laboring and weak. He got started on antibiotics again for an infected ear and then all of a sudden GI started going capute again on friday/ saturday. By sunday morning we were having bradys and turning blue in my arms and he had a seizure which I think was likely due to his meds not staying down. When I say his Gi goes capute basically I mean that you either can't get it to go in the g tube or you get it in and he writhes in pain and pushes it all back out like vomit but through the gtube syringe used for venting. He had gas and not much stoole (which is usual issue for him) but what he had was loose. He is already on laxatives 3 times per day and colace. It got better when feeds were on hold for a while but then he would go into crisis when anything(even meds) were pushed in. He had bradyed (dangerous low HR) all the way home from an opthamology appt on monday in pensacola that monday afternoon. I was pulling over the car and giving oxygen and I guess in retrospect that was due to his bradycardia meds not staying in with these feeds.

I got worried about repeating the dehydration and dangerous bradys we had before where we got to a point that I could not even get little amounts of pedialyte in for days. So I called the GI doctor and he said we needed to be admitted.



So luckily, Tuesday the nurse was at my house for the last insurance allowed days and helped me get Sam packed and meds and records ready etc. I was graced by a sweet friend taking the girls. My family also agreed to take them over from her later. We peeled out of there and got down to the hospital in what seemed like took hours.. Of course with no feeds going he started perking up and looked down right calm when we arrived. They gave him fluids, did x rays and rectal exam all the while saying we would have to stay regardless of findings because of his crazy history and dsats he kept having. They then made me give him a feed just so they could witness his behavior..seemed like torture but we did it and boy he showed his true colors. He did not tolerate the food and furthermore started going into painful contortions and respiratory distress. He had a blue dsat episode and went back on the o2. I will try to post a quick video under my afewnegron youtube.com account as sam12.28.10 if possible.

We were first admitted to the floor and are now in the ICU because he decided to misbehave this morning with another blue dsat episode. He had been dipping sats into the 80s but on this one he stayed down at 79/80 too long. The bad part about the floor was I called the nurse on the call button and said he is blue and I need help but noone came for like 5 minutes. By then he luckily had recovered but this has happened to us before on the floor and I got pretty mad. I think it was the meds they put in his tummy that put him over the edge. He is off all other food in the tummy. I had a sweet visit from my friend Jenni and we shared coffee and a change of scenery down in the cafe by the Christmas tree. Hate that our reunion had to be so short and in such a place. The last time I saw her was in Atlanta under similar circumstances.

So for now they are planning for Sam to get TPN (liquid nutrition basically) by internal port or picc line as they have stopped doing peripheral IVs for TPN at this hospital as it strips the veins..so this may actually mean we would leave with a port which could be helpful at giving IV fluids at home or meds in emergency but not having nursing at home with a port seems impossible for me to manage alone. To place the port he will have to go under and this brings more issues as he often misbehaves while under anesthesia, sometimes requiring intubation. Hope that is not the case this time..at least now they do have a pediatric anesthesiologist on staff. I don't know at what stage we have to consider other hospitals. They have discussed pyloroplasty (sp?) if a gastric emptying study shows warranted delay but right now they are holding off on doing the study to see if he rebounds first. They think his gut just shuts down when it gets low on the energy fuel ATp what have you from bad mitochondria. I think in that scenerio I would prefer a G/J tube placement which would allow for feeds to bypass the stomach during those times when his tummy just wants to shut down operations. I hope we are not experiencing a worsening of his condition and that these last few illnesses have been just that..winter illnesses that he just can't handle like other kids. His little body decompensates or "crashes" as I often call it.

I have prayers tonight for several families. My friend Robin's father is being transferred to an LPAC in Birmingham today in a critical medical state and they are just trying to ensure a safe place for him of convalescence. I believe they feel it is just a matter of time and I know they are enduring great strain and grief. Her father is greatly loved by all who know him. My prayer is for peace and rest for him in a safe and caring environment.

I am also praying for another sweet mito child of a mom whose blog (www.terratalking.com) I often follow, back in the hospital as well with very similar symptoms/ circumstances as Sam's and even though I don't really know them, I pray for them often. Somehow I relate quite a bit with this mom. She was also a MOPS leader and Christian mom of 4 daughters.. I thought I had it crazy with my 3 munchkins! There are so many other sweet babies out there fighting this disease and it is such an aid to me to connect with them and learn from their experiences..how they coped etc.. There is a United Mitochondrial Disease Foundation site(www.UMDF.org)that has also helped me in understanding more about this disease as it has such a wide range of symptoms. Most of these kids, like Sam, look so normal and it would be hard if you saw them to know what serious challenges they face daily. I certainly feel naive to only now understand more about the medically complex kids out there. I will probably pull together some of my research to post here eventually..in the meantime we are focusing on treating his symptoms and thinking positively for the future! God Bless and thank you for those of you praying for our Samuel!

Sunday, December 26, 2010

A New and Glorious Morn..

On Christmas Eve, carols such as "Oh Holy Night" usually stir me to tears while singing with our church choir or listening amidst candle light in quiet prayer or worship. I have been unable to sing with my church choir due to Samuel's chronic health concerns and needs. Furthermore I was unable to go hear the beautiful sounds of our church choir for Christmas Eve services because Samuel fell ill Wednesday after I had worked so hard to maintain his health since our most recent hospital discharge only a week and a half before. But even though I was home bound for the holidays, I played Christmas CDs as usual to get into the spirit. Never before had I heard the lyrics and felt their meaning like this year.

"The thrill of hope the weary world rejoices, for yonder breaks a new and glorious morn. Fall on your knees and hear the angels' voices! Oh night divine.. Oh night that Christ was born."

I am on my knees and so thankful God sent his son to help guide us in living our lives His Way. I think of Mary on that night of His birth..in many ways not unlike any new mother. She could not fully know the role God had chosen for her life or how this one baby boy would change the world. I think of that night of light. Light from one northern star guiding the kings and shepherds to the One who would lead us through dark nights and show us the path. Our family is on a path right now of which we can't see enough far ahead to feel safe or secure but with Jesus at our side I know we have to trust Him and let him guide us.. and that has been the light shining through our Christmas season.. We know he has a path planned and will lead us. We just have to follow Him.

The last week or so was unique in that I had several more days of nurse aid at the house with Samuel and that was a blessing for sure...can't even put it into words. It is indescribable what it means to let go of some of those fears and all the medical data and duties and logistics for even a short time. She is incredibly capable and experienced with medically complex kids. She is detailed and very purposeful in her manner and care of Samuel along with her gentle and compassionate way of approaching him. On top of her experience she recently overcame pancreatic cancer in a most miraculous fashion and so certainly understands the side of being on the other side.

On Monday, I took Samuel for his 9 month well baby appt. We had skipped the 6 month vaccines while in the hospital and then had delayed out of concern over some conflicting research about the safety of administering vaccines to children that have mitochondrial disease. Without knowing if that is what Samuel's diagnosis will be, I was nervous to plunge forward without further research. What I found was most Drs agree that the risk of diseases that could have been avoided by taking the vaccine may be more dangerous than the suspected worsening of conditions or onset of autism reported by the families of these compromised mito children. It seems reasonable that the views of these families are correct, in that the already at-risk nature of their child's condition may make them vulnerable to the trigger and chain reaction effect started by being "down" on some bio- level due to the vaccines. So weighing all this out I decided maybe we would go slowly, start with only one or two vaccines at a time spread apart and possibly avoid the MMR for a longer period. That was before Sam got sick.. now I am thinking.. hmm maybe vaccines can wait longer.

Samuel had low grade temp monday afternoon and then a little heat at the site on his leg Tuesday morning. We did speech therapy Monday and Tuesday working with oral feeding and I always worry about aspiration when he sounds wet afterwords as he did with these sessions. But he seemed clear after short period upright. By Wednesday morning, he had a 101 fever, red cheeks and labored breathing. By that afternoon he was really having a tough time breathing and was running up to 102 fever or so which is very high for him. Amidst all his other medical issues, high fever is actually unusual for him. The Drs told us to treat Sam a little differently when it comes to treating pain and fever as with his dysautonomia symptoms, they are not entirely sure a temp reading is accurate to what is going on internally. By Thursday morning, he was struggling and the nurse was mainly holding him constantly as he was not able to sleep or come off the O2. I had done all of Jason's Christmas marketing stops on Wednesday with the girls all day in and out of offices and had promised a beach play date if they were good, so Thursday morning we went to the beach to trade Christmas gifts with their close friends. It was hard leaving Sam but I knew we only had the nurse for this finite time and the girls were starved for one on one time. It was beautiful weather and they had a nice time but by the time I got home Samuel was seemingly worse. The nurse had called his pediatrician and they wanted me to bring him in for a status check.

At that appt, she discovered more retracting and respiratory distress and his ear was infected as well again. More antibiotics and orders to keep him hydrated. He was tolerating feeds so I was hopeful that we could avoid another hospitalization, especially for Christmas. Our nurse offered to come Christmas morning and watch him so the girls could have some undivided attention and "normalcy". We finally took her up on it and that ended up being such a blessing. Samuel was ill to the point of moaning and struggling for air through most of Christmas Eve but seemed somewhat better Christmas morning, catching a little nap between periods of stress. The nurse left and we had to decide whether to join his parents for dinner down the road. We decided to do it because he seemed pretty stable but once we were over there a short time, he took a turn for the worst and it was not a comfortable visit for him.

I got really emotional as the girls jumped around enjoying presents from grandparents. They were so carefree. I remembered them at Sam's age on Christmas..completely different than his first Christmas experience. He was limp and moaning, gasping and weak. He was on O2 and pretty oblivious of anything. Then he stopped tolerating his feeding and was arching in agony. His GI system had finally gotten the message that the respiratory and ears were already feeling..this is the way of things for him as I am learning. Everything just shuts down like on the highway when one little bumper accident leads to a whole chain of accidents and traffic jams. I just want to get him on the shoulder of the road so he can drive on past to the next exit.. past all this mess.

We went home and he seemed a little better. I even tried to shoot a first Christmas picture. He was not much into that plan. We tried to make him comfortable and he finally fell asleep. It was a most difficult night however with several bradycardia events. In the morning, he needed O2 again and was having drastic color changes. He was in pain and then when trying to hold him, his face twitched and his eyes shifted back and forth in such a way that I was pretty sure it was a seizure. It was a tough morning and we were about to depart for the hospital when he fell asleep. We decided to back off on feeds and substitute with pedialyte. This seemed to help..he started breathing more evenly and actually had a good nap. We are still not sure what to do but I will definitely be paying a visit back to his Dr in the morning. One day at a time..for now I am just thankful the girls had a nice Christmas playing and singing and listening to beautiful carols filled with hope and promise for a new and glorious morn. God bless all the families struggling with grief or distance or illness this season. There is a beacon of light there to lead all of you as it is leading us each day.

Wednesday, December 15, 2010

There is NO place like home..

I did not have to click my ruby slippers together but somehow we did get back home on Sunday. I was beginning to feel sick being in that little room for so long. I could not use the shower in Sam's room as it was hardly a trickle of water that came out plus it did not feel especially clean. Sleeping on the pullout chair/ couch was NOT much fun as usual. The ICU is a different story.. much cleaner shower and not too bad recliner but then you often don't want to use either because you have more doctors rounding through constantly and things are more critical so you are feeling like you need to stay "on" with bated breath about his status and any treatments or tests...not a time to get much shut eye.

Anyways, before we left Sam pulled out his gtube and the tech just stood there blankly while I hollered for supplies etc..by then I was ready to go home. He did it again at home and this time the balloon had popped on it and we had to use our back up button..leaving me without a backup so until one comes..he better not mess with it. In the hospital they slowly inched up the rate of his feeds again with no particular understanding of why his gut shut down these past few illnesses. Seems as though if he is stressed in the smallest way, everything shuts down. He was looking bright eyed and bushy tailed though after the IV fluids and some rest so we hit the road sunday. He has been sooo happy to see his sisters and they were pleased as punch to see him. They have however been more difficult to handle in the "pushing my buttons" category.

At home Sam has had a few difficult periods tolerating feeds at a regular rate but overall he has been fine. Tuesday I had a visit from the nursing company about the details for setting up the only nursing care that insurance will cover which is NOT much. They will cover about 60 hours total per year so we are getting about 8 full days between now and end of the year. While in the hospital we agonized over how to obtain the care he needs but I am still completely blocked on this. If we stay with insurance they won't pay anything and now that we are having to change from our ultra expensive group plan..we will be lucky to be approved by a new independent plan. Kids are legally now not allowed to be turned down for preexisting but our current agent was pretty straight forward about his feeling that they would find some kind of bogus reason to not take Sam. Our group is going to be even smaller now so we may be looking at an even more expensive group policy as well and that may be impossible for us right now. We still won't have the nursing home care either way.

I have been researching a secondary medicaid waivor which is based on his disability and not on income etc..It comes under something called the katie beckett law which is specifically for kids that require medical equipment and involved care such as Sam does. It is apparently more readily available in other states but nearly impossible to access in Florida.

Today we had the nurse for 7 hours and BOY... what a day of clarity of mind and spirit it was for me. I was a little sick to my stomach leaving him when he has hardly ever been out of my grasp but I needed to trust someone to take some of this off my shoulders a little for just that short time. I actually left the house without lugging tons of medical equipment... wow what a feeling! I did not have to think ahead about every med that would need to go along or how much food should be primed in his pump or whether oxygen should be brought along just in case.. or whether Sam would be comprimised by my need to get groceries.

I was able to watch Sofie in her musical program at church and run something into Bella's class that was forgotten this morning. I can actually see how I would have a life again if I had this kind of support.BUt it is not like finding a babysitter..nursing is very expensive. I could work and contribute to finances though if we had this help. I could be fresh for the girls. But all we get is these 8 days so it is a bit of a tease. I am thankful God has given us this small break and I hate to be greedy but please..if you are listening Lord... we need the extra support. Show us how to make it work so that Sam can thrive and grow safely in our home without pulling everything we have built to pieces. For now I am just going to keep praying and doing my research. I am sure it will all work out. Bless him..he is worth every agonizing moment. He is so sweet and innocent. He looks at me like..I am sorry mom that I have my own set of instructions and don't fit the mold. He is absolutely perfect to me.

Friday, December 10, 2010

Home sick

We are sooo ready to be back home. I miss my girls and they tug at my heart every time I talk to them. They beg me to be home and ask why Sammy and I always go away to the hospital. I blogged yesterday and did not publish it because it was a little raw..it was the 15 yr anniversary of my dad's death. I was only 16 when I lost him..half my life ago. Yesterday was maybe the hardest anniversary yet because it was lonely sitting with my memories. I wish I could go to him with some of this, but in some ways I guess I feel his presence and him weighing in when I go to God in prayer. I would give anything to just have one hour with him again.

I think my day took a turn yesterday when I passed a chaplain who had prayed at our bedside with Sam and I the day before. She was walking with a family in tears who looked rather shocked. I remembered that feeling..that moment when they told us he was gone..Only that morning he had asked me to drive him to the base for some Christmas shopping. I had my learner's permit and normally would have lept at a chance behind the wheel, but Jason had called and I chose a trip to the mall with him instead. Dad was crushed in his car that day. They fought to save him while we waited in a room nearby with our pastors. Everything changed that day but he is still with me and I know he watching over us. I remember his face from our last exchange as I did my hair in the bedroom mirror. "You sure you don't want to go? I love you," he said..and then he was gone.

It was one year ago yesterday that we also said goodbye to Jason's grandma, our mimi. She was quite a force in our family. It is not the easiest time of year for anyone who has experienced a loss. I think it is because of the heightened emotions and memories. I usually find personal strength and overcome those feelings by focusing on all the joy of the season. I think of all the blessings and the fact that we DID get to share beautiful memories with those we have lost. By the end of the day, I felt better and I loved all the more on my sweet baby boy. He is such an angel.

Sam has begun to tolerate small feeds a little better and is inching up in rate at 5 then 15 ml/hr to now 20 mls per hour..a long ways away from a normal feeding rate but we don't want to overwhelm him. He was moved from ICU to the floor today and we hope to go home soon..ICU, FLOOR and OUT THE DOOR!!

Sam met Santa for the first time while here and has had many sweet visitors from local churches and organizations. Tonight he heard carolers and it reminded me of how much I enjoyed caroling last year. Some of my MOPS moms went around to those of our church unable to leave home and at the local retirement home. It was the highlight of the Christmas season for us and I am touched that people are doing that here for all the sick children. It is sad to think of children here on Christmas..I hope if we get out of here soon I will get a chance to pass on the cheer and joy of Christmas as it was shown to Samuel this week.

Wednesday, December 8, 2010

magical fluids on ice

Since arriving late Monday night, Sam has been on IV fluids with no tummy feeds as he had not been tolerating those at home and it had been pushing him into major respiatory and cardiac distress. There could be any one of a bunch of varying opinions of our doctors who all seem to have beducated in the carribean or some other medical university I have never heard of before..not that they are not determined and compassionate..they really are but Sam may just be a little complex for tthem here. So here are some theories:
1. He could just have a virus they have not found in bloodwork with multisystem involvement.
2. He could have had another "crash" of sorts which would indicate a metabolic breakdown like that of mitochondrial disease.
3 He could have had some GI block or issues that are causing tummy to press on diaphragm and stress autonomic nerves which then slow down his heart.
4. He could have some transitionary weakness of all systems..
The bulbar, stomach and intestinal muscles from some neuro or myopathy origin.

One big thing that happened yesterday was hearing news from the Dr here that they madecontact with Dr.S and found out that another test result from atlanta came back with concerning results of. Some nature..unknown to docs here or myself. Certain acids were at bad levels or something and so they have requested that urine and plasma be collected here and overnighted on ice to a lab in. Colorado where some of his stuff has been being analyzed..nervous nervous..whatever glutamate alpha whatever is off..will that lead to answers? Praying..Dr. S also told my. Dr. Here that we may not have answers from biopsy in the 6 to 8 week time frame..it may take more like 12 weeks..ugh!

I hate to make light of a scary situation but I keep thinking of that song for a checkers burger commercial .."You gotta eat..no matter who you think you are..you won't get far..you gotta eat"..so the mission for today is EAT Little Guy..Let food in your tumm tumm!

HUGE AMOUNT of Prayer going out to my friend Robin today..and it is her bday too.

Tuesday, December 7, 2010

Back in the joint...

That's right..here we go again..of course I knew we would end up back in the ICU eventually but I was hoping not again in 2010..never a dull moment. He became sick this weekend and we could not get him to tolerate food so we kept trying pedialyte but it would make him writhe and scream out in pain. We began to worry about dehydration and his bradys were happening so much more frequently that I had him on o2 more often than off it.                  So we  we finally reached a point last night where it felt unsafe to stay at home any longer..and after 3 days of no sleep I could not figure out whether it was safe to try to drive us the hour to the childrens hospital or go to local ER and be transported..can you guess which one I went with?  Did I mention..no sleep in 3 days..well needless to say it was the scariest drive ever at midnight on dark interstate with  Sam struggling in the back and periodically tummy emptying into his open syringe venting his tummy..yucky horrible drive but thank you Lord for guardian angels as my good friend Jen helped me immensely with her calming advice and directional aid as my phones GPS would have dumped us out in the red light district..yes..in all my no sleep frantic momma wisdom i took the road less traveled by..and it almost made all the difference. yikes...                                                                                                                            So we made it here and found out my Dr had set up  a direct admission to ICU which was a saving grace and then we had to do that agonizing history run through  all though the attending knows sam well and has cared for us since birth..then the most miraculous thing  happened...sam went to sleep..nov bradys..no agony and less struggle breathing..of course sating at 100..they must think i am loosing it..no..the nurse says clearly going NPO and stopping all trys at tummy feeding must have made the difference but now he has risen and still no episodes of bradycardia but he is junky..he is getting iv fluids and so hopefully after that goes in he will get stronger so we can go home soon..they said no viruses showed on the labs and yet..he cant seem to handle anything in his tummy without total crashing failure to breathing..heart rate..etc..for now we will just wait and seee..they said they may ultrasound his liveer..one more test..praying for girls and jason to stay the course at home with little upset..sure it was another shock to them that i am gone..hate that they always have to find out i have left after waking up. if my grammer is amess it is because i am doing blo on cell phone..hate tiny buttonss..signing off for now.

Sunday, December 5, 2010

Out with Scrooge and in with Christmas Spirit

Sam took a bad turn last night and is very sick again. It was a shock as he was great yesterday morning and even saw the Dr when we took the girls for flu shots. I should have known when he sounded kinda junky and was having more and more bradys that we were on a downward spiral. So..we were up all night as he was not breathing well, turning blue in spells and struggling and then also not tolerating his feeds. We are exhausted and maybe a little slap happy at this point. I must have prepped for the ER 10 times but Jason is convinced that there is NO point in going to our local ER with Sam.. and I hate the idea of another ICU stint..can't we just help him get past this here at home.. Having a nurse here would be GOLDEN. Our Dr said to go ahead and bring in the nurse for whatever the insurance would cover which I imagine will be like 1 week and so I called them this morning..the woman must have been thinking.. "why is this woman calling on a sunday?" Well.. because I am over tired, can't remember the day of the week and just plain desperate for aid!

Sam had a fever but I think I have it under control and he is resting for the first time since nap yesterday..Jason has crashed too and I am staring around in horror at all that the girls did to pull the house apart last night and this morning in our distraction of dealing with Sammy.. well no biggie..I had more decorating to finish today anyway and was bound to get messier before it all came together. The problem is when Sam is sick all you can do is hold him and try to make him comfortable as he gasps and pulls at every breath. He is so miserable. Getting emotional is not going to turn it around so we will focus on positive Christmas feelings and pray for stronger days.

Going into this magical time of Christmas and all the changes of transforming our home over this past week.. I have decided I am redecorating my mind and spirit as well... OUT with Scrooge and all the baggage and negativity and self doubt and worrying and fear....and IN with the Christmas Spirit and laughing over the things we just can't control. If I don't learn to laugh about it and accept it and trust God every day..(and not just when it is convenient).. then I really will go bonkers.. so with that new frame of mind I am going to find my inner peace and joy and shut the door on the rest ..at whatever cost!!!

"On the 1st Day of Christmas my true love came to me..." and said:
BIG changes are underway.. Jason made a decision to branch off from his partnership at the firm and fly solo. Overhead was way too high and with all Sam's medical bills and so far no luck at securing the nursing aid that would free me up more to aid in finances..we have to be able to cut corners and make our own budget decisions. He is planning to stay in the same location and most everything will be the same except now we can control the figures a bit more. It was a scary proclamation for me to swallow at first because I feared loosing our insurance in the transition but I know it will all work out. Praying our new insurance does not go up much higher than the $1600 we are already paying each month.. but I am sure it will all work out. This year has humbled us a bit after being so stable at a young age only to realize one event can totally rock your world.. but we are very joyful and thankful for our blessings.. so any changes don't have to be scary.. it is all just an adventure right?

The reason the 12 days of Christmas song came to mind above was I read a blog recently that really had me in tears relating with this other mom as she blogs each of the days of Christmas and did a 12 days list of their 12 specialists who work with their daughter who has mito disease (the disease we are awaiting our results on regarding Sammy).. Their story has seemed eerily similar to ours in many ways except they are farther along in the journey and seemingly more adjusted. I am inspired by her humor about life and I found myself laughing over what I would have thought if you had told me last year what was about to happen or even shown me a peak at her world... boy was I clueless!! SO here are my 12 days of Christmas for all that craziness that the last 8 months have brought anew into our lives after the traumatic arrival of our beautiful son.. the thing is that it also brought more love and closeness in our family than ever before.

"On the first day..."
1 G tube button, 2 invasive surgeries (gtube with fundoplication and the muscle biopsy), 3 ambulance trips, 4 wonderful therapists (speech, physical, developmental, occupational), 5 continuous EEGs, 6 concerning seizures (not caught on EEG), 7 hospitalizations totaling 11 weeks this year, 8 weeks of ron mcd house stays at 4 different houses 9 different medications (caffeine, prevacid, many antibiotics, Coenzyme P5P, Tegretol, Colace, Milk of Mag, Miralax, and Robinol), 10 pieces of medical equipment (heart moniter, feeding pump and bags, 02 compressor, 02 tanks, various size syringes, needles, Mic Key extension kits, nebulizer, handmade pulmo back beater, and the all important breast pump), 11 Various medical tests done in triplicate (Cat scans, MRIs, Sleep study, EKGs, swallow studies, radio nucleid reflux studies, a million X rays, laryngoscopy, bronchoscopy, halter monitering and more blood work than I have ever seen..), 12 different types of Physicians (obgyns, neonatologists, pediatricians, ICU intensivists, neurologists,
pulmonologists, cardiologists, geneticists, Mito specialist/neurologist/ geneticist/ biological scientist = total brainiac, gastroenterologists, anesthesiologists, and general surgeons)...
Fa La La Laa...    and that was all just the tip of the iceberg!

Thank you Lord for humor and for only allowing us to see this list in review and not before we needed to know what lie ahead. Lord help our Sam to get well again..and stay well.


  

Saturday, November 27, 2010

Gobble Gobble

Thanksgiving has never been such a time of reflection and thanks for me as it was this year. I am so blessed by wonderful children and a most loving husband. I was particularly thankful thursday for Sam turning the corner on his illness and making a rebound. He slept all morning while we scurried around prepping for 14 person dinner and awoke as everyone arrived. At one point my mom and I thought he might have had a seizure but it was just because we could not get his attention as he was laying in his crib. Visiting with family, he was in great spirits and had tolerated a couple normal feedings of milk.. finally moving off the pedialyte to the millk. He spent about an hour of full busy activity and then basically crashed for the rest of the day.. it took alot of energy for him but he loved seeing all the relatives. I was further blessed  not to have to cook the bird this year.. my mother in law did the honors on that one. We had a great visit with everyone!



Yesterday afternoon was spent decorating with my mom with Alabama football on the TV and chili on the stove. A cold front had come through and so all in one day we went from 70s and 80s to the 30s which made it feel all the more fitting to do our Christmas decorating! The girls were fully of silliness as they could not wait to find the nutcracker box. Nutcrackers are their absolute favorite decoration at Christmas. As I drove my mom home last night I thought about how very very blessed we are that she is here with us... she has overcome soo much. I could not have gotten through this year without her.

Late afternoon we had a call that nearly threatened those good feelings as it was the home health company calling to say that they found out our insurance policy only covers up to $2500 per year of home health. Even as our policy has a $5 million lifetime limit.. not much covers help at home with an involved child. I had been praying so hard about this and it just feels like we can't find a way to make anything work in our favor. The company said full time home health would be about $180k per year which is nothing in relation to what is spent in an ICU at about $50 -100k per week and Sam has been in the ICU or NICU 5 times and for up to 3 months of his little life. They have tons of other infant patients that get full time nursing fully paid by medicaid. I just feel sick about everything. I don't know what to do differently to find the solutions. I am out of ideas and just plain tired. I need to work as we have been hit like a truck by this medical stuff and my not being able to aid in bringing in additional income. I just have to trust God to help us find the answers.

That evening we also had a wonderful visit from our good friends, the Riddle family. Greg was one of my closest friends from high school and helped bring Jason and I together in 9th grade... ahh that was soo long ago. They have a new baby girl and she was absolutely adorable. The babies got to meet each other and Sam was quickly entranced by lovely little Lillian. He wanted to touch her face and pull her to him. It was so sweet! He really has met few babies and it was so neat seeing his eyes dance at watching her up close. I am so thankful for good friends!



So today we are finishing up the decorations and hopefully Sam has a low key day so he has energy for tonight. We are planning to have some good friends over for a little potluck get together. Should be a good time. I can't recall the last time we had anyone over besides family before this week. It has been a while as we have been hermit crabs I guess. Prayers for all those families experiencing illness or a loss in their families during these holidays. Even with all the reasons to find joy, it can be a very hard time of year for many people.    
      

Tuesday, November 23, 2010

Getting some help..

This is obviously the week of blogging as I usually don't blog but once a week but this has been a busy and draining week for us. Today was especially notable though because it is the first day in quite awhile that I felt something good might happen to help us care for Sam. I met with a company about getting full time nursing care for Sam. YAY!!! I hate to have to bring someone into our home but I think I am reaching a point where I am spread far too thin and frankly emotionally and physically exhausted. I look in the mirror and don't recognize myself anymore..I look the way I feel for sure! I can't believe we have gone 8 months without getting this kind of care arranged before. As it is not yet approved by insurance I am not wanting to jinx us but I may soon actually be able to concentrate on one of a million things that have been set aside in doing all the daily tasks that independently seem not so bad but all together are rather overwhelming. Here are pics of some of Sam's equipment..just knowing how and when to use them require a set of skills I wish I did not need. It will be good to get a break from some of it if possible.



suction machine - sam does not cough or manage secretions well
mobile O2 Tank
O2 compressor - produces O2 out of the room air


Enteral feeding pump - pumps food through Sam's MicKey extension tube to his G Tube in his tummy

Nebulizer for breathing treatments

Heart - Lung (apnea/ bradycardia) moniter


humidifier

medical cart of medicine, syringes and all the "junk"
They are talking about a nurse who would come 7 am to 7 pm and take over with everything Sam needs. I would be able to leave the house if I needed to or work with the girls on their projects.. wow.. the thought of even having clarity of mind enough to sort bills and make those necessary calls regarding all sam's medical junk.. priceless. I might even be able to work in some capacity to make a dent in all the medical financials.

Sam is doing pretty well this morning with some periods of respiratory struggling. He is very distended in his abdomen with all his usual constipation and slow gastric emptying. But he was smiling and happy and more playful than he has been for days..so maybe he is getting a little better. It always amazes me how this bright and beautiful baby boy is able to smile even with the tubes and all his struggles. We just can't let him get sick because one illness is like a total crash of everything for him.


Bella has a half day and so I am going to put the girls to work on something constructive before they go stir crazy. I got busy with Sam and peeked in on the girls after lunch and they were playing puppet show with each other. At least they have great imaginations to keep them busy!



Just as I was posting the last picture and talking on the phone with one of our contacts at Early Steps (who provide his early intervention therapy sessions) about the nursing care info, I heard Sam's brady alarm and went running to find Sam was bradying again, clamped down and not breathing well..it has been a several time a day occurance while he has been sick. Finding your baby greyed out or blue and in distress even once is like some kind of nightmare but having it happen over and over is indescribable. My heart must be beating off the charts during these episodes and I feel a little like a crazed animal trying to get the O2 on him. I have so much adrenaline racing through me that once I get him stable on the oxygen and stimulated and then calmed down I feel like I could pass out. That is why we need a nurse here. I hate being alone during these scarey times. I have to tell myself that God would not give us or Sam this life without a master plan even if I can't see it clearly right now. I read this scripture recently and it really fit with what I keep trying to remember.

John 9:1-3 NIV
As he went along, he saw a man blind from birth. His disciples asked him, "Rabbi, who sinned, this man or his parents, that he was born blind?"
"Neither, this man nor his parents sinned," said Jesus, "but this happened so that the work of God might be displayed in his life."

I really want to find the answers to why Sam has to struggle, but if he has been given more health challenges than most, I have to believe it was so that others would see the work of God displayed in his life. Through him we witness God's love every day.



Monday, November 22, 2010

Thanksgiving Week

Samuel is still fighting some kind of illness that is effecting him on many fronts. It started as congestion with cold-like symptoms and basically went into respiratory distress all at the same time. This was thought to be croup without the normal associated cough and would transition during the morning to easier breathing only to get far worse in afternoon to night. Then Sam became limp, weak and miserable. He stopped tolerating feeds and started exhibiting pain in his gut and a huge amount of gas in his tummy. He already has a slow moving digestion but I think it slowed down far more.

Today before we went in to see the pediatrician, I had tried the first milk feeding since doing all pedialyte for the past 24 hours... well we went to place him on the counter to be measured and weighed and the gas in his tummy caused so much pressure his g tube popped out all on its own. Ahh.. I can never get comfortable seeing that happen but at least I know what to do and I started moving rather fast at replacing it. I think the nurse was as suprised as I was. So his belly is overly full of gas.. not much room for food. Then a new problem appeared during the exam. She saw a double ear infection to top it all off.

So we are doing antibiotics and sticking with slow pedialyte and using the oxygen as needed. She suggested she could put Sam back in the hospital but I told her anything would be better than that which led to my breaking down and asking the Dr. if we could get the nursing help which in the past has been questionable for if we could get insurance to pay for it. I think she realized how exhausted and spread thin we are right now and she put me in touch with a service who is sending out their nurse and a director tomorrow to discuss 24 hour nursing or night nursing. Sacred Heart is doing the referrals as we were there not long ago. The woman freaked me a little when she suggested that insurance would pay for Sam to go live in a care facility in Plantation 6 hours away and that we could take the girls there to vacation and see him if I was interested in that option...WHAT?? - NO! That was all I could muster. I can not imagine a scenerio where our son would go live in a far away hospital. Home nursing is going to be enough.

1 Little 2 Little 3 Little Indians.. 1 Little Indian Girl..
Sofia had her Thanksgiving program today. Nana and Jason were able to be there to see her. Our cute little indian apparently sang her lungs out. The girls are very excited about the upcoming family feast. I am just excited about being home all together as a family for the holidays. I am setting aside all the worries this week to try to focus on them. I hope the nursing can help us to regain some kind of normalcy around here as it has basically been our lives shuffling around Sam and the cracks are starting to show. Everyone on Jason's side is coming to our house for the dinner. My mom is planning to see us over the weekend for some tree decorating, chili and football..Yay! I can't ask for more than that.. all in one weekend. I hope Sam will get better on the antibiotics and get back on some milk soon. I want him to get to enjoy his first Thanksgiving with the family. My prayers for tonight are for all those families that will be apart on Thanksgiving, and for the families that have just lost loved ones.

Bella's Soccer Gang.. We will miss those Saturdays!

Sunday, November 21, 2010

Prayers

I was so sad to find out this morning that my music pastor's wife has passed away after her difficult battle with cancer. She was far too young. She will be greatly missed and was loved by so many people. I am praying for her family today.

Sam went back to the ER with Jason at about 2 am this morning. Yesterday morning we were excited when he seemed to be doing pretty well. I had planned to skip the soccer game but got a call towards the end that Sofie had a potty accident and so I headed down to the field. Sam seemed downright happy as we strolled out there so we ended up staying a few minutes and then joining the group for the end of year party breakfast. I was relaly hopeful that we had turned a corner on the breasthing but now in hindsight all the past mornings have been far easier than in the afternoons and over night. By 3 he just could not breathe well and even putting oxygen on when his color would look bad did not seem to ease it. Then after an afternoon feeding, he stopped tolerating his feeds. This is where the food backs up inthe syringe if we try feeding it bolus style or if we are pumping it in with the feeding pump where it is moving very slowly he begins writhing in discomfort. So we backed off on the food a bit but I began to fear he was getting worse so after much back and forth over whether to go or not, we opted to play it safe and go ahead down there in case they needed to deep suction or check blood gas levels. We are getting very little when we use the suction machine at home but he sounds so congested.

They did a chest X ray and saw that he was clear but hyperinflated and had alot of gas in his tummy which we knew because every time we vent him it loudly releases air right away which is not typical for him. They did not do much more for him but he had calmed alot while he was there so he came back home and then we started trying pedialyte. Over the last hour or so he has dipped back into some periods of stress again. SO it will be a long day.. hoping it gets better over the course of the next few days..but mainly praying to get him the food and energy he needs asap.

Friday, November 19, 2010

Hard Days Night

SO.. Wednesday night we had a crash of sorts for Samuel. We had given Sam his 6 pm feeding and then he seemed to not tolerate it as well and was incredibly tired so we put him down. When an hour later he seemed to be breathing a bit more labored and raspy I was concerned we may have pushed the bolus feed too fast for him and hoped he had not aspirated anything. We kept him upright and kept trying to calm him. It was the beginning of a horribly frightening night. A hundred times we asked ourselves whether to put him through a trip to the ER or all the way to Sacred Heart but then he would seem to calm back down and somehow we made it through the night. We employed every piece of middle-of-the-night parents first aid for bad breathing..took him outside bundled to breathe cool air... ran a humidifier.. used the menthol rub.. used the suction machine.. patted his back for hours but he was in absolute distress and then he was bradying too and changing to his scarey grey color.. so we put him on the oxygen by cannula. I was so bleary eyed and tired I hardly knew my own name.

Then in the morning I called our Dr and they were not opening till 2 that day so she advised we go down to the ER. By the time I got over there.. wouldn't you know it he sounded so much better! SO I felt a little crazy to have him doing better and still going into the ER. Well my Dr met us down there and said this was croup but as Sam does not know how to cough so we don't have the usual barky cough.. the secretions just sit in the large upper airway. Somehow this coupled with his trachea issues is why he can't breathe so he had a nebulizer steroid treatment and she brought the machine to us at home to continue there. We have used it on the girls in the past but it has been a bit of an argued treatment for Sam. The Scared Heart pulmonologist said steroid treatments can make tracheomalacia worse as it can further weaken the cartilage so...we hope this is not going to harm him. My pediatrician was so kind in offering to deliver it to us so we avoided another trip out that day.

The next day through today have been waves of doing pretty good to almost normal and then absolute crashing waves of distress. It seems to have no real connection to the nebulizer treatments or anything in particular. He just starts acting sicker, more puny and breathing gets so bad then color goes and then heart rate. We took Sam and all his equipment tonight to my inlaws for a visit so the girls could decorate their tree. Right before he went into some distress so it was nice not to be alone with the worries for a few hours. It is times like these when I don't like being alone with him because I am so scared he will have a major event and am constantly thinking of CPR and what I would do in that scenerio. The time I did the rescue breaths is still fresh in my mind and I am absolutely unsure if I would have the composure to do compressions if necessary. I wish we had help at home with a nurse during these times. If any other parent saw their child like this they would be hitting 911 so fast it would make your head spin.. but as this is something that happens to him..we just have to find a way to handle it at home or live in the hospital every time he gets a cold. I am going to have to talk further about this with our pediatrician.

Right now he is on the oxygen due to a series of bradys since getting home and trying to fall asleep. He is pretty miserable but not an hour ago he layed on my chest and almost seemed okay other than some raspy breathing and a vibration feeling in his back. I hate this darn cold, croup, congestion or whatever it is.

Bella had a Thankgiving luncheon and program today, which thankfully Jason was able to attend for us. I was worried she might be the only child without a family for the family feast. Tomorrow is her last soccer game and end of year party. I hate missing these little experiences with her as it is becoming the theme this year. This is just not our year. There will be plenty of years to do those things together though. Sam is sounding a little better..better catch some shut-eye.    

Tuesday, November 16, 2010

Rainbows

After a couple of downer days, this morning left me searching for rainbows and trying to focus on the good stuff. Right before therapy I looked over to find Sam had picked up a wooden block on his own and was holding it above him examining all the sides with the different pictures and letters. It was a big step.. and I felt so happy in that moment. He has been making progress lately with his hands and body but most of the things he has been holding and picking up were more ring like or larger rattle type toys. This movement required a little more dexterity of his fingers and he held it in such a stable manner that I could see he was proud of himself.

He worked hard in therapy doing developmental play before he started to send out the messages with his body that we had hit the limit. It was around that time that he seemed to lock his gaze on something and the therapist could not gain his attention for a short moment. I could tell she wondered if it was a focal seizure and then later she mentioned that concern to me. It was just a short moment but I felt this lightening bolt to my rainbow of a mood.. I started worrying as I realized I was a little later in giving his P5P dose this morning..was that why or was it being so tired or was it nothing at all. I let it go and as I went on with my morning I found myself realizing that I was not going to let that moment "steal my joy" as a wise woman once told me. I watched Sofie have a better day at gymnastics than last week, tumbling and balancing away with a smile. I enjoyed a nice lunch around the coffee table with my sweet mom and caught up with a friend from Gainesville on the phone. Today is not going to be shot with worry.

Monday, November 15, 2010

Outings and Bradys..



I wear my up and down moods pretty visibly lately and especially in my blog entries but I think being honest is about all I can control lately. I have to be honest or find myself buried by the act of putting on a perfectly happy face all the time. Not that we don't have light and happier moments, but we bounce from extremely thankful at being together with Sam doing well right now to feeling sadness and worry over what might be around the corner or at the bottom of everything. We deal with heightened moments over toddler issues made worse by them feeling our stress. But overall the girls have managed to come through most of this year unscathed and generally quite content. They periodically communicate seperation issues about hating how much Sam and I have been away this year, but realize we did come home each time and all was fine.

The main thing I struggle with is how to answer questions about how Sam is doing from people I run into during the day..there just is not a simple answer. So I usually say.. fine and that we are just waiting for answers from biopsy tests. They always remark on how good he looks because that has actually always been the case.. until he doesn't in those horrible moments. It is pointless to hash out for people the good and bad of the current status but for my own journaling here is where I guess we fall today in what I call the Good, the Bad and the Ugly..

The Good: Sam is very engaging as usual and does not seem ill right now although he continues to become taxed rather easily. He seems to be making progress though over the last couple weeks with his hand skills and neck control and trying to stabilize his body more. I feel like we are about where the girls were at 3 or 4 months in some ways but he is 8 months this week. He is taking some food and drink orally although it is an incredibly slow progress and he struggles and is left exhausted after the session. We don't know if his progress could be partly an impact of starting the P-5-P supplements 2 weeks ago. We are doing ok getting back into the swing of a somewhat normal routine with the girls and this weekend I got to have a little one on one time with each for an "outing" as they called it.. I took Bella to roast smores with some friends who were camping nearby for a few hours. I took Sofie to trek along the toy aisle tonight just to look and make her Christmas wish list for her grandparents and Santa. I did get some organizational stuff done with setting up Sam's medicine/supply cart in his room which felt good. We are hoping to soon secure a piece of tumbleform positioning equipment Sam needs for feedings and sitting. We used it in Atlanta and it helped Sam to feel more stablized and not have to focus on his neck control while eating or working with hands on fine motor skills. It was also great to help him safely ride in the wagon or larger strollers. Biggest ups for the week was the aid of my wondeful mom who is always optimistic and my inspiration.


I organized a medical supply cart so my counters could be cleared off..

The Bad this week: Sam had 3 bradys last night that kept us up worrying and running to his room each time. I have to say it is in the wee hours of the morning when I am sick with fear or worry that I get bitter about mental baggage. I absolutely should not blog at that hour as I usually just need to pray at the time and let my frustration give way to my faith and hope and letting go. In last night's case, I was holding in alot of frustration over a letter I received last week from someone who basically did not know or understand the depths of what we have been experiencing of late and made some careless statements and tried to make me pretty much feel like I was failing to meet the "perfect" expectations of wife, daughter, mother etc.. I see that it is easier for some to not try to really know your situation. Heck, I could never have imagined this turn of life myself and am not sure I would have wanted to entertain this possibility. God has blessed us with the right circle of support in our family, church and friends. I don't have energy for anything negative right now.

The other downer of the weekend was a realization that I really need to be able to work but we will be hard pressed trying to find a way to do it amidst still needing to ensure proper care for Sam if I had to leave the home part of the day. So I am now on a mission to make inquiries to a situation that could work for us. I am applying for a few positions which has me both excited at the prospect of something different to assign some focus and bathed in uncertainty over how I can make it happen as far as a nurse or nanny comfortable with his complexities. I am praying about it and doing my homework. Cost as always is the issue. The costs can not exceed benefits. The only other bad of late has been the waiting.. waiting .. and more waiting for those test results. Finding out the first piece of the puzzle from the spinal tap findings just made it worse as now we have even more questions. 

As for the Ugly.. Even in the state we find ourselves in over Sam, our problems pale in comparison of those we are currently praying over for friends.When I think we are stressed about Sam but here he is breathing fine today and in his own crib tonight while others are on life support, I think I have so much to be thankful for... I hate that worries over finding a diagnosis, or financial worries due to medical expenses has made me blind to the fact that at least we are all together under one roof right now unlike other families tonight. I have several people on my heart constantly that are caring for critically ill loved ones or are seperated from their families. I fear I have not been able to do enough for them because I am a bit too deep in our own quick sand.  I am going to make it my mission to change that mode in whatever small ways I can. I have always tried to do as a friend of mine puts it..pay it forward but I have never felt so stretched as I do now. We have been the recipients of huge levels of prayer and kindness and although I might not always have enough moments in the day to personally write thank yous to each individually, I know they must know we feel incredible appreciation. I just lately feel emotional overload and then when you come into contact with more sad news from other families experiencing a painful situation or when you just sit and listen to the news about the state our country is in or the loss of deployed military members..it seems just too much..too much to process. So for now I am trying to process and help mainly with my prayers.    

Wednesday, November 10, 2010

Going Bananas..

It has been quite a full week for us. After learning from the people in Atlanta that his lumbar puncture results indicate a deficiency of pyridoxal 5- phosphate, a derivative of B6, we were asked to start supplementing him with a very large amount of the P-5-P. It is not the easiest vitamin supplement to procure locally but we got it and started it right away. I was rather nervous about the toxicity adverse side effects that can take place but so far no big problems. His urine has taken on a really particular smell and he began sleeping a whole bunch but I don't know if that is connected. There were days when  he slept 5 hour stretches only to wake for an hour and go back into another 5 hour stretch repeatedly. Could be a growth spurt but I am highly curious if it is a growth spurt brought on by some normalizing of his brain activity as this is what they have seen in others with this deficiency after starting the treatment. There have been babies with abnormal brain activity on EEG (encepholopathy) as Sam has, that after the supplements were in their system, they began having normal EEGs. Please Lord let that be true for our Sam.

 Normally your liver would pull this coenzyme derivative from B6 through a metabolic process but for whatever reason, Sam's may not be doing this properly. So what does that mean? Well we are still in wait of the results of all the testing they did with blood, urine, muscle biopsy and skin biopsy. They had also found his resting metabolic rate test to read as abnormal so something is going on but what is the whole picture..? We researched Pyridoxal 5-phosphate dificiency and a form of epilepsy dependent on this deficiency as this was the suspician of his specialist in Atlanta. This could explain many of his symptoms but not necessarily everything. There are genetic causes for this type of deficiency but there are many other causes as well. If his liver is having problems, that could be the source or if there are other metabolic issues, those could hold the answer. The mitochondrial disorders are the same way and it all could be down the pipe of one of those issues.. It could be genetic but  also could be from trauma at birth or in utero which has always been our worry as the Drs and the hospitals actions seemed concerning to us, from what I have read trauma could cause mutations to the genes for the first time in him... not coming down the line from us but actually as a traumatic series of events. There could be damage to his cells from free radicals that are created by an anoxic or hypoxic event that led to a systematic damaging of the systems. There is just soo much to question that my mind can't hold it all. I am at times..overflowing with questions.

My mom had a concerning day this past week when she all of a sudden experienced two seperate instances of disorientation and foggy to no vision in her one eye. My sister took her to the ER they ran CT scans and looked for signs of stroke. They have now set up a corotid arterial ultrasound for next week to be sure there is not a blockage of some kind but it seems rather strange to me that it take this long to get it checked by ultrasound.. but mom chooses consistently to stick with going on base, however limiting that is..she had surgery connecting vessels from her leg to her corrotid arterys for the facial reconstruction surgeries and so I would think there would be more immediacy in her case. I am concerned about scar tissue. Blood work showed her thyroid levels are way off. She has thyroid disease so I guess this is the other area that may have been causing the issue. The other thing that worried me was she said she thinks she experienced this episode once before back in April. I am just praying that everything is ok. She has been doing so well and has been very excited about some home improvements she has been making lately. I worry she is not always upfront about her health issues because she does not want to worry or burdan us with all we have going on.. of course she is my mom and never a burdan.

So the other thing that happened was Sam's biopsy site tests came back positive for strep infection but it did respond in the lab to the antibiotics and it seems to also have done so for him as well. He has been doing pretty well overall. He did have a brady episode on Friday night but came right back up in heart rate so just a small bleep on the radar. We were blessed by my friend Jen who watched the girls while Jason and I took Sam with us for some dinner out.. a rare event. So thankful for good friends! My sister and mom were also a big help to me this week picking up prescriptions and making it possible for me to go to Isabella's literacy day. She was Heidi and wore a dress I had from Germany when I was a young girl. It swallowed her up but she was pretty cute.

We celebrated my niece's 1st birthday and she recently began walking so it was alot of fun watching her walking all over at the birthday. Her dad is deployed and I know it was a hard one for my sister but they are returning to Germany next week and then getting reunited with him in the weeks ahead. Watching her daughter makes me remember so much of those same milestones with the girls. Sam's start has been different than theirs and on a different schedule, but he is making progress and will leave those lasting memories of the big moments too. He had a big day today in speech therapy, working with the eating and drinking skills. We went back to bananas which I am finding is his absolute favorite. He is swallowing and doing more with drinking especially. The eating is tricky but if I get the spoon far enough back and press on his tongue and hold it in there while the food is dumped in and long enough for him to trigger the swallow, then he does not push the food back out. It feels like such a small thing to us because it comes so easily but for him it is real effort and he gets so tired afterwards. He is moving forward though and I feel like we will get him there in time. One step at a time.

The big mind and time drains right now are coordinating the medical paperwork, backgound stuff and researching things for him when I need to be devoting more time to normal stuff. I am trying to find out about a deemed waivor for Sam to receive secondary benefits as they have deemed him fully disabled but because we own our own home and we acutally have an income, we don't fit social security disability parameters. We don't need an income for Sam but we need to be sure he has access to the care he needs. In this country you are punished for being able to afford health insurance.  With each child I forked over thousands for each delivery after insurance paid like $20k while the mom in the bed next to me paid nothing. Not that I am mad she is not paying anything but when you are paying $1600 per month in health insurance, you would think Sam would be assured the care he needs but no.. if he becomes seriously ill again, we won't necessarily have access to the nursing etc that medicaid would provide for other kids. If he needs a wheel chair, insurance will likely not pay for it as there is a list a mile long of what they don't cover..and these are the things I am just scared to death about. What if insurance costs go up even higher as they did last year for us by nearly double.. Just when you think you have done everything by the book..have everything financially under control, doing everything possible to make sound decisions, something happens and you just feel so broadsided.

I had planned to return to working after Sam's arrival since taking time off when my mom's surgeries and the girls had taken the lead. I just needed to help contribute as every month is different when you are self employed like Jason. It is such a catch 22 though now..because now we really getting hit hard by Sam's demanding medical needs but I can't leave him with anyone due to his health needs to even go back to work. I have to figure out a way to do all that a stay at home mom does naturally along with caring for Sam's unique needs, and find a way to help with financials.. seems like something has to give. I thought it was tough when I was working from home with 2 toddlers under foot but boy I am a bit between a rock and a hard place now.
 I am just praying for the right avenue. I wake in the night thinking about what ifs and find myself coming back to the same answer..there is never enough..never a perfect solution to an unplanned situation, God wants us to let him help us figure it out. I know we can't do this alone.. We are soooo blessed I know and have always known that we have gotten to live relatively comfortably but boy..I never planned for the turn of events of having one baby boy.. one sweet, defenseless, baby boy. Never knew how much more complicated life could become. But he is a gift and I am so thankful to have a complicated life if it means I have his sweet face spitting his bananas back at me. :)

Prayers this week mainly for my friend Robin who is away with her Dad in Houston at MD Anderson fighting for his life. He is on a ventilator and in critical condition. He has fought many health problems for so long and my prayers are that he not feel pain and that he feel all the love around him. I am praying hard for Robin who is trying to be so strong, a true daddy's girl. I pray for her own health through this ordeal and that she and her family have God by their side during this hard time. I know her and her mother are very tired and emotionally drained. It is so hard to not be able to help your loved one. We have so many friends in painful situations and with struggling loved ones. Our prayers are with those families in situations we know too well.