A Glimpse into Mendollyn's Crazy Life | |
To the ER and back againWe ended up back in the ER with Noah yesterday afternoon, but our home phone lines (and therefore our DSL) were down and I couldn't blog about it.The gist of the story is that somehow Noah got two holes in his broviac. I believe the holes had been there for at least a couple of days because we kept noticing that his pajama pants were getting mysteriously wet in front . . . but then again Noah soaks an overnight diaper every couple of hours so it was reasonable to assume that we were dealing with diaper leaks. It wasn't until I went to administer his noon antibiotics at church yesterday that I realized the the saline flush was coming right out of the line. The holes had apparently gotten larger shortly before I gave the antibiotics because they were obvious at that point. A couple of doctor friends took a look at it and gave us suggestions for stabilizing it until we could call Noah's surgeon after church. We called both surgery and infectious disease, then Hannah and the babies and I headed for the ER. The surgeon was able to remove about half of the outside length of the broviac and replace it with a new end. The repaired broviac is working like a charm, praise God! The bigger concern is one of possible infection. Noah is on antibiotics for gram positive bacteria (MSSA). The holes were placed right in the line of fire for all of the leaky messy diapers Noah has been having (thanks to the antibiotics), and the bacteria found in stool are gram negative. This means that he is not covered for any bacteria that may have entered the holes. The ID doctor ordered a blood culture in the ER, and he is going to be having lots more cultures over the next couple of months, so we are optimistic that if something DID get in the line, we will catch it quickly and be able to clear the line. Noah certainly isn't acting sick, so I'm hopeful that we are fine in that regard and will be praying to that effect. Other than that, we've just been enjoying being together and working toward restoring any order that was lost from a "mommy-less" week. :-) It seems unreal to all be together at home when a week ago we thought we could be losing our son. It was such a joy to sit down at the table together for dinner tonight. I even made a cute desert - mini caramel apples. I used a melon baller to cut round balls from apples, being sure to leave include skin on one side of the ball. After drying each ball well, I inserted a toothpick handle into the part with the skin, dipped the balls in melted caramel, and rolled them in chocolate sprinkles before placing each one in a tiny mini muffin cup liner. They looked just like real, but miniature, carmel apples complete with that bit of skin peeking out around the handle. They were a hit with the children, who seem universally charmed by tiny things. I can't take credit for the idea as I saw it in a magazine, btw. I'll definitely make this again. For those of you interested in all-natural cooking, Walmart stocks bags of caramel chips this time of year. They are the only all-natural caramel I've found outside of expensive websites. They can be melted for caramel apples or used in recipes, and I always stock up on them. India Tree makes all natural chocolate sprinkles. They are pricey but we buy them in a large container from a restaurant supply website and use them sparingly for special treats. :-) I need to give Jeff a hand getting Mr. Noah all hooked up for the night - it's a two man job! Blessings, Kate Heading home!It looks like we are going home tomorrow! Noah now has one officially clean culture (clear for three days) and another that has been clear for two days. They will be continuing to track his cultures and will call us back if something should grow out on the newer cultures.Noah will be going home on two IV antibiotics for about 2 more weeks. One is every 6 hours around the clock (midnight, 6 AM, noon, 6 PM)with IV benadryl a half-hour before each dose, and the other will either be once every 12 hours or once every 24 hours. The drugs aren't compatible with his TPN, so the TPN will have to be stopped and restarted before and after each dose. This isn't an easy schedule and I would covet your prayers that we can be faithful and diligent to wake up each night as needed to administer the drugs and TPN properly. Mind you, I'm not complaining!!! A bit of lost sleep is a teeny price to pay to be home with all of my family together again. Noah's hemoglobin is still dropping, but isn't quite low enough to need a transfusion yet. We tossed around the idea of doing one now to avoid another hospitalization in the near future but ultimately decided against it. Each transfusion brings a higher risk of complications, and since we know he'll be needing regular transfusions for the rest of his life, we want to spread them as far as we safely can. Noah will be getting weekly blood cultures for the next month to be sure nothing is lurking in his line. The hope is that if there is bacteria there, we can catch it and pull the line before he gets so gravely ill again. We'll be checking his hemoglobin with each culture and will be ready to admit him for a transfusion as soon as it drops a bit more. Noah's wonderful nurse had a beautiful baby boy last night. It was touch-and-go at one point with her almost needing a c-section, but things turned around and the surgery wasn't needed. She called the room today and I got to hear that beautiful newborn cry. She wanted me to thank you all for your prayers. Noah is still very tired, and his hemoglobin doesn't quite explain it. I know it seems obvious that he would be tired after having gone through a battle of this magnitude, but he has been getting more tired and more reluctant to walk with each passing day. He doesn't act sick, and he is walking some, but he prefers to lie back against pillows in bed or be carried around. This could be a mitochondrial issue (energy production) or a result of the stress of rebuilding all he lost. His gait is still labored and strange, so we'll be following up with neurology to see if he is showing the start of ataxia. Like I said last night, as long as we have his personality and heart back, we can deal with this other stuff! The only thing left before we can go home is one more set of labs to make sure his newest TPN recipe is going to be correct. His sodium keeps falling even though they've adjusted the TPN once already. We need to be sure his TPN is just right before the infusion company comes tomorrow with his new batch. After that, we should be ready to go home! Thank you again so much for your prayers and comments. It always amazes me to read about how God uses our simple little blog and our amazing little boy to touch the lives of others - and it might amaze some of you to know how deeply you encourage and uplift our family. I am going to run now. Jeff found a Chinese restaurant that delivers to the rooms here, so we are splurging on Chinese for two tonight. :-) Blessings, Kate looking better!Noah is really starting to look better in a lot of ways. His last two cultures still haven't grown anything! They won't be officially considered negative for 1-2 more days, but it looks like we are definitely on the right track and could think about going home very soon. He is getting his IVIG now and then he'll have some more labs drawn. My guess is that he's about due for another transfusion because he is getting paler and more tired even though he clearly feels better in general. It would be wonderful if a transfusion was the final hurdle we had to jump!He will need to be on some pretty high-powered IV antibiotics for a while longer, but we've got plenty of experience with administering IV antibiotics at home and his team is comfortable letting us do so once everyone clears Noah for discharge. Noah's speech seems back to 100% of what it was and he seems much more oriented. He hasn't really been willing to walk today so I can't report on his balance and gait. I'm not worried about working on those issues - I'm just so blessed and thankful to be seeing my treasure acting like his old self more and more. I missed him! I spent a long time cuddled up on his bed with him this afternoon playing with puzzles, talking, watching a video, and just snuggling. There isn't anything on earth I could have enjoyed more. I even got a couple dozen big loud Noah kisses! Thank you all for your prayers - please keep them coming. Thank you also for all of your comments - we read every single one. Hannah reads them also and they are a great source of encouragement to us all. Blessings, Kate quick updateI only have a second here - We are still waiting for a final answer on Noah's line. The most recent culture hasn't grown anything, but it won' be considered negative for a couple more days. If any of the cultures show up positive at any point, we go to the O.R. and get the line out. Noah's speech is SO much better today, praise God! He is more subdued, though, and wants to be carried rather than walk a lot of the time. When he has walked, he has fallen a few times and even walked into walls. His TPN line has snapped twice tonight, which means he immediately starts bleeding rapidly out of the line. We are very thankful that we saw it right away both times. It wouldn't take any time at all to lose a lot of blood that way. It loks like we'll be doing IVIG in the next day or two. Maybe that will help kick this bug into oblivion. We got our child care issues covered for tomorrow and Friday - thank you for praying. Please really pray for clarity on the line decisions. We are very deeply concerned about keeping the line in case a couple more of these bugs are luking somewhere in waiting, but losing the line is a lousy option too. I don't mean to whine - I just wish there were a choice we could feel good about! Please also pray for one of Noah's favorite nurses who is expecting her first baby and is being induced tonight due to a bit if pre-eclampsia. She's a little nervous and hadn't wanted to be induced - please pray that all goes well for her! I'll update when I can tomorrow. Blessings, Kate mixed newsWe've had lots of news today - it's probably a good thing that I couldn't get to the computer sooner or I would have had to update several times.Noah's infection is NOT MRSA. It is a plain, ordinary staph A. In the lab, his cultures are responding well to pretty much every antibiotic they try. In other words, in the lab it is very easy for them to kill the bugs growing out from Noah's blood. That's the good news. The difficult news is that even though it is easy to kill these bugs in the lab, Noah's blood cultures are still growing out bacteria. In other words, the bugs aren't being killed in Noah. It could be that he isn't metabolizing the IV antibiotics well enough. We know he doesn't metabolize them really well and he often needs higher than normal doses - in fact, he is currently on an increased dose of vancomycin. He is also getting riphampin and a third antibiotic is being added as we speak. Another possible cause of Noah's continued infection could be that some of the bugs are entrenched in his line and continuing to multiply and spread. Yesterday his line was treated with a substance that is supposed to strip it clean, but the cultures were still positive. His line was treated again today, and like I said, he's getting a third antibiotic added to the mix. We will draw another blood culture tomorrow and if it comes back positive he will have to lose the broviac in the hopes that this will allow the infection to clear. Tomorrow's cultures won't be considered clear until Saturday, so it will be a few more days before we know for sure if he can keep the line. He is still in a catabolic state where his body is breaking down. This is driven by the high fevers, so we are hoping that it will stop now that his temp is normal or just mildly elevated. The doctor made it clear that Noah is not out of the woods yet, but we are hoping more than ever that we are on the right track here. Noah is having some disturbing neurological symptoms. He is having increased trouble walking correctly and he is using the wrong words consistently (calling the fish lions, calling dogs monkeys, calling my sweater candy, etc.) These are words he knows and has used correctly for quite a while. He is also having episodes of extreme agitation. We were up with him until 2 AM and he would alternate between calm and restful versus suddenly screaming, hitting, throwing himself around, etc. It's like there are two Noahs and we can't tell when one will be exchanged for the other. The GI and nervous systems are the two biggest energy consumers in the body, and they are systems that are commonly affected by mitochondrial disease. Just as his GI system suffered terribly in January when energy was diverted to fight an upper respiratory system, it looks like his nervous system is suffering as a result of this infection. The doctors are saying that each infection has a cumulatively serious affect on mito children, and that this infection is a very significant insult to Noah's system. While these neurological symptoms may improve, they may also continue to worsen even after Noah gets better. It is almost beyond imagining to think of Noah changing this way forever. Having said all that, he doesn't look bad all of the time. Another little boy let Noah hold his stuffed monkey, and Noah had a hard time being willing to let go of the toy. He has some birthday money that he hasn't spent, so we took him to the gift shop a few minutes ago to pick out a stuffed toy. He came out with a frog purse LOL, a tiny stuffed dinosaur that roars, and a little stuffed kitten. He wanted to be carried the entire time but he was engaged and interested in his selections and carried them all of the way back upstairs to his bed. He also called them all by the correct names! He slept for almost 5 hours straight earlier this afternoon and the rest apparently did him some good. :-) We are so thankful for these happy times, the snuggles, and the ability to do anything at all that will bring a smile to his sweet face. I have a real sense of peace about Noah's current battle and I really do believe in my heart that we are past the worst of it. Please continue to pray for this infection to be cleared, the catabolic state to be reversed, the neurological symptoms to disappear, and for PEACE for Noah. We want so much for him to be freed from this agitation. There is a wonderful song called Peace Be Still by Rush of Fools and it has really ministered to me lately. I had a hard time finding a good video of the song to share. The video below is dedicated to victims of the So. Cal. fires, and it is well done, but it doesn't really fit our situation at all. Please just listen to the words and pray for peace for our little guy. It is a terrible thing to see him agitated, frightened, and inconsolable, but we know and believe that God can pour out His peace on Noah. We also need continued prayers for the other children. They are doing really well, but it looks like we are in a child-care gap for Thursday and Friday during the day. We don't like for Hannah to have to watch all of them all day and up until now we've managed to get care for the boys for at least a few hours a day. If any of you local friends could host the boys for a little while one of those days, please call us on Jeff's cell at 864-337-3725. They need some care tomorrow as well, and Jeff is going to go home for a little while to be with them when some friends come up here to help with Noah, but he can't go home like that every day. Blessings, Kate Difficult newsThis morning the head of the pediatric service came in and told us that Noah's newest blood cultures are continuing to grow out this still-unknown bacteria. This means that the antibiotics are not succeeding at killing the bug. He said that we should know what the bacteria is within 24 hours, and that he suspects that it is MRSA (antibiotic resistant superbug). The antibiotics Noah is taking are big guns and *should* be providing coverage for all gram positive bugs, but for some reason they don't appear to be doing the job.He also said that Noah is in what is called a catabolic state which means that his body is being broken down instead of built up. This is basically an acceleration of his mitochondrial disease caused by the severe stress of the infection. Whether this state can be reversed, and how long it takes to reverse, will be determined by the length and severity of the infection. The doctors are exploring the idea of removing Noah's central line, which is a catch-22. It is *possible* that removing the line will make it easier to clear the infection from his blood (if the line is harboring bacteria). It's also possible that removing the line won't make any difference. If they do remove it, they won't want to give him a new line until the infection clears, because putting a fresh line into an infected body will just cause the new line to get infected. Noah can't get his TPN (nutrition) through an IV - it must go through a central line. Most of us could get by with IV fluids for a few days, but children with mitochiondrial diseases must absolutely be kept from fasting . . . . Noah is still spiking high fevers, and when he is feverish he is either sleeping or very, very out of things. When the fever goes down lower, he perks up enough to take an interest in his surroundings, watch a DVD, get carried to see the fish tank, etc. He is even walking a little when he really perks up. It seems hard to believe that things are going this way. Noah has had sepsis and line infections and other horrible issues, but he has always responded beautifully. I guess we've always known that there could come a time when he didn't respond as well, but it still seems hard to believe. We should know more tomorrow. They drew another blood culture a few minutes ago and there is still hope that this one will show that Noah is winning and the bug is retreating. If any other big event or change happens, or if we receive any new news, I'll update - but I don't expect that until tomorrow. Again, please pass this on to anyone who might be praying for Noah. I want to get back to him (you could pray that we can get our in-room internet figured out!), but I want to close with a song that has meant so much lately. some test resultsI am sorry to have kept you all waiting so long. We couldn't get our laptop to connect to the internet in Noah's room, and he has been too sick for me to leave him and use the public computers. By the time we left yesterday morning, Noah's temp was up to 104.7. We called his infectious disease doctor and she told us to take him straight to admissions - by the time we got the the hospital, his room was all ready and we carried him straight up. He had a long and miserable night with a number of painful tests. By the time we went to sleep last night (this morning?) we were sure that he had a line infection based on his initial labs. For example, his CRP( an infection marker which should be under 3) was 47. This morning we got confirmation that he has a gram positive bacteria growing in his line and throughout his blood. This isn't a big shock - a septic infection is about the only thing that would make Noah so sick so frighteningly fast. We don't know which bacteria it is yet, so Noah is still on two very powerful broad spectrum IV antibiotics for the most possible coverage. Noah is very, very sick. All yesterday and almost all of today he has been basically unresponsive. He is sleeping a lot, and when his eyes are open it seems like he isn't really seeing anything. Except for very painful procedures, he hasn't even cared what anyone is doing to him. This has been almost unbearable to see and is very unusual for Noah. He has been septic before but has looked better than he has this time. About an hour and a half or two hours ago, he seemed to finally "wake up" for real and is feeling well enough to watch a DVD and even say a few quiet words. His other "awake" times have only lasted a few minutes so this is an improvement. The doctor came in a bit ago right after he got more alert and told me how bad and sick he looked - she was surprised to find out that she was actually seeing a dramatic improvement. We are still really battling his high fevers. He is getting tylenol and advil alternating every 3 hours, but it has stayed constantly very high. It is lower this afternoon than it has been which is almost certainly contributing to his newly alert state. As of yesterday's first labs Noah's hemoglobin was about 10 - another whole point drop from his last labs. He is having a lot of labs drawn and it wouldn't surprise me at all for him to need another transfusion before we can leave. We don't know the status of his line yet. While central lines are generally removed with this sort of infection, keeping Noah's line intact is of the utmost importance. There is a limit to the number of central lines a person can have (number varies from person to person), and since this is Noah's 4th line and since his line is essential to his survival, this infection will be aggressively treated. We are praying that the line can be kept. The other children are having a very hard time with this. His last hospitalization was obviously not an emergency, but even our youngest ones could see for themselves that Noah was frighteningly ill when we left yesterday. Please pray for them. We have had such a good long time at home, and these two hospitalizations so close together have been stressful for everyone. One of the young ones started crying before we left and said, "The whole world is changing all over again." It was extremely difficult to drive away from them. Through all of this, God has shown Himself in so many ways. Jeff and I had planned to spend the day in Greenville yesterday. We meant to leave early but Jeff was extra tired and decided to sleep in a bit. Had we left as planned, Noah would have gotten sick while we were gone and everything would have been much more difficult. Our minivan was on empty but we were able to find a station with gas after much looking and praying. Even calling the infectious disease doctor was a huge blessing. We tried to call Noah's home health nurse to ask her to inform the ER that we were coming (standard procedure for Noah), but she is out of town and her phone was't on. As a result, we called ID to ask them to inform the ER and were very happy to receive a direct admission order instead. Had we reached our nurse, we would have been in the ER for goodness knows how long instead of getting right to a room. There is more I would like to share, but being out of the room makes me antsy even though he has good friends watching him. :-) Thank you all for your prayers and your comments. Please continue. I'll update again as soon as I can. If needed I can call Hannah and ask her to update, but she doesn't know how to send out the email notifications, so you may want to check back if you don't hear anything. It's possible that there will be an update without an email notification. Blessings, Kate emergency prayers neededWe are rushing to the Greenville ER. Noah is vomiting, shaking uncontrollably, and running a fever of 103.5. He is extremely sick. Please ask everyone you know to pray. I'll update when I can.Kate Lots of photosI'm going to try something new with my photos. I want to put more of them on the blog, but Photobucket is driving me batty. I made a Picassa Web album of a few of the pictures I took Saturday at the mill, and I'm linking to it here: http://picasaweb.google.com/jkestes/HagoodMillPhotos# I could embed the photos right into the post instead like I usually do. Which way do you all prefer? Is it easier to read the post, then look at the album, or would you rather have photos right in the post? It isn't a big deal for me either way.We had a good time at the mill Saturday. There was a fiddling competition so there was lots of great music all day. We got to explore the working mill, take a long nature hike, watch a blacksmith, go through some restored old cabins, watch a cotton gin in action, eat some barbecue (good, but not nearly as good as Jeff's), and look at some newly uncovered petroglyphs. There was lots of local color, including a fellow who hand raises baby wild hogs for fun. He looked to be about 204 years old and could be seen (overalls and all) walking around with a wild (tame wild??) hog using a piece of rope tied around its neck. He also had a small puppy with a similar leash, and a chicken in a cage. Sarah is drawn to animals like bees to honey, and we just KNEW she was going to end up involved in some sort of hog/dog/chicken disaster. (Disaster is her specialty, as you all know.) Somehow she made it through the day unscathed! Noah didn't seem to feel well at all. He spent all but a few minutes with his head on friend "Uncle Zach's" shoulder. We persuaded him to get down by the river and walk around for a few minutes, but that was all. After our day at the mill, we went to our friend's sister's amazing log cabin (log mansion?) for a cookout. The children enjoyed kayaking on the pond and were thoroughly exhausted by the time we left! Noah perked up a bit at the cabin and actually got giggly and playful, which was great to see. Noah was also very tired at church yesterday. I sat in the nursery with Noah while I nursed Mary Faith and he spent a lot of the time just lying down on the floor. He would sit up and play a bit for a couple of minutes, then would just lie down again. His balance and coordination seemed off too. Having said all that, his color/energy seems to have improved a little as of yesterday. The doctor decided not to draw labs until the regular lab date next Monday. If Noah hadn't started looking perkier I would have really pushed for a CBC, but we all hate to draw extra labs if we can avoid it. I'm still reserving the right to get pushy this week though LOL. We got the full report from Dr. Shoffner in the mail. I can't decipher most of it. There are five mitochondrial complexes, and Noah has problems in three of them. That doesn't really mean anything in terms of prognosis or anything, it's just Noah trivia. We had hoped against hope that the report would contain SOME sort of suggestion, prognosis, anything specific to try or to monitor or to watch for. In this regard we were deeply disappointed. All he was able to offer was "Our options for this child are limited." It wasn't easy or pleasant to read the report, and I'm going to schedule an appointment to meet with our geneticist to go over it as soon as possible. Dr. Shoffner wants to do full DNA sequencing on Noah, but we no longer have health insurance. Noah has Medicaid-type coverage through a program for seriously sick children, but Dr. Shoffner doesn't take anything but private insurance. We are going to get on the waiting list for a follow-up appointment with Dr. Shoffner. The follow-ups are expensive but we feel that we have to have one. I can't imagine the cost of the DNA sequencing. He said that it is difficult to get insurance to pay for it due to the cost, so I'm sure it's something that we couldn't afford without insurance! I'm not worried about it right now. We know of two DNA mutations already. I know that they might not be the mutations causing Noah's disease, but finding out won't help or change anything. It won't give Noah treatment options. The only thing it would do is allow us to test the whole family (simple blood test) to see if anyone else is carrying this gene. None of the other children are sick or have any signs of mito, so this isn't a big priority. If Jeff ever ends up employeed and getting insurance through work, we might pursue it. (We are looking into getting insurance, but Noah is uninsurable unless the insurance were to come through an employer. We are VERY thankful for the coverage we do have for him.) A couple of you pointed out that I didn't list God as Healer in my last post. It's funny - I looked it up with the intention of listing it and THOUGHT I had! I had two babies on my lap part of the itme I was typing - is that an excuse LOL? Thank you for pointing it out. We are so blessed to know beyond a shadow of a doubt that Noah will be perfectly healed one day. We hope that we can see that miracle on earth, but know that Noah will be healed one way or another. God is also the healer of our hearts and we keep trusting Him for our own good as well as Noah's. I'll leave you with a "snapshot" of Noah right at this minute. He is sitting beside me, and while he is pale and does have dark circles under his eyes, he is smiling and enjoying singing (and dancing - best moves EVER) with big brother Matthew. He is very happy, unspeakably cute, and loved more than he can imagine!! Blessings, Kate P.S. Don't forget to let me know what you think about the web album vs. photos on the blog. (You can click the photos to make them bigger, or mouse over just to read the entire caption for each photo.) Noah newsLike I said in yesterday's post, we have some newer news to share about Noah. I usually tend to put the most positive possible spin on the news I share here while still being honest. If this post is difficult to read, it is because I feel the need to be totally open with no sugar-coating at all. Families whose children read the blog may want to read ahead and then "translate" as appropriate for their children.We have all really been blessed by how stable Noah has been since Mary Faith's birth (Jan 29th). He has had his small ups and downs, and of course he is still a very sick and fragile boy, but he has been home and stable and growing and learning - such a delight! Even when he was so sick in Atlanta at Scottish Rite, he bounced back pretty well and it didn't seem to be more than a bump in the road. When Noah got sick and was hospitalized last week, he didn't seem terribly sick at all. The only thing that really, really worried everyone was the fact that his hemoglobin dropped an entire point overnight with no obvious cause. We know that if he is very sick his hemoglobin can drop quickly (though never that quickly) so we assumed that maybe he had been a bit sicker than we thought. Thursday after his transfusion his hemoglobin was a respectable 13. We'd all love to see it higher, but that is the best number we ever see after transfusions. Since he had gone from the end of Jan - mid June, then mid-June to early Sept. between transfusion, we figured we were pretty well set until November or January. His hemoglobin falls at a fairly consistent 1/2 point every other week. Unfortunately it appears that something more is going on with our little guy than we had suspected. He had routine labs first thing Monday morning, and his hemoglobin had dropped to 11 - a two point drop from the previous Thursday. Since we transfuse at about 9, this means that he dropped halfway to a transfusion in just 4 days. All of his other labs look fine. There is no blood in his stool and he has no more unusual bleeding. His labs don't indicate any source of infection and he doesn't appear sick. There is no obvious, "fixable" reason that this is happening. We would love to hope that this is a fluke and that his hemoglobin will level out, but the fact that this also happened last week before the transfusion is very worrisome. We spoke with his geneticist yesterday and he felt that the only cause would be that Noah's partial bone marrow failure has worsened considerably. When we saw Dr. Shoffner (mito expert) in Atlanta in June, he told us that the 2 defects in Noah's mitochondrial DNA could very well be polymorphisms, or benign, random, symptomless mutations. He did seem to think that Noah had mito, but didn't think that those mutations were adequate proof of the diagnosis. If you know me, you know that that was enough for me to tuck away the tiniest morsel of unreasonable hope that maybe, just maybe, this wasn't mito and that he would just eventually outgrow all of this. As Dr. Shoffner talked with us, he indicated that there are a very few mito diseases that affect the gut and the bone marrow. His concern for Noah was that right now the TPN itself and the central line are the most lifethreatening issues that Noah faces, but in these marrow/gut mito diseases other symptoms can very quickly and very suddenly become extremely life-threatening. At this point, of course, the smidgen of hope was jumping up and down begging God that this could somehow not be mito. I was in Walmart with the girls doing some shopping yesterday and thinking about all of this, and hoping that by some miracle Noah didn't have mito after all and that what we are seeing is somehow not what Dr. Shoffner warned about. As I was walking and thinking and praying, my phone rang. It was Jeff calling me to tell me that our geneticist had called to let us know that he had received a preliminary report from Dr. Shoffner confirming a mitochondrial diagnosis for Noah. (For those of you who know mito, Noah has complex one). I raced home and called the doctor myself, but all he could/would tell me was that Noah definitely had complex one mito, and that he felt that Noah needed to see Dr. Shoffner again as soon as possible. He knows Dr. Shoffner. He knows that the waiting list is many, many months for a follow up, but he said that we shouldn't wait that long and that he was going to call Dr. Shoffner and request that Noah be bumped up to an earlier slot. That is all I know about that. We still haven't received the full report, and we can only pray that it will come quickly and that it will offer informationt that could shed more light on what we are seeing with Noah right now. Back when I first heard of mitochondrial disease, I wrote on this blog that it was the stuff of which nightmares are made. I didn't know what it was, so I did the "Kate thing" and googled it - much to my regret. Statistics are now showing that mitochondrial disease may be as common as childhood cancer. The difference is that there is no treatment, no cure, no surgical solution, no chance of remission. All you can do with mito is try your best to put out each fire that flares up. Some children are fairly mildly affected and live for quite a number of years. There are even adults with mild, mild mito. Some milder forms don't even manifest themselves until the teen or adult years. A year and a half ago when we were at MUSC getting a cardiac workup for Noah (thinking there could be a cardiac explanation for his sypmtoms), we were told by a leading genetist that the best possible outcome we could hope for would be for Noah to be diagnosed with a major heart defect - that even open-heart surgery offered the hope of recovery. He said that he felt that the only other explantion was mito (turns out he was right), and that if it were, Noah's prognosis was poor given how early the disease manifested itself. I've pretty much just refused to think about that very much - there was no point thinking about it much until we knew that we knew that Noah had mito beyond a shadow of a doubt. Now we know. I won't say that I'm not thinking about that at least a little now. It's surprising me a little - after all, we've all "known" that this was mito, but somehow my heart wasn't able to fully believe what my mind has known for so long. There is no more fooling my heart now. Whew. All that having been said, there isn't any real plan or any changes to share yet. (The more things change, the more they stay the same, right?) Noah isn't due for more labs until a week from Monday. Right now everyone is trying to figure out if we should do labs this Monday or wait for the regular day. I'm wanting labs now. I want them yesterday. You can't believe how much I want someone to draw labs and tell me that his hemoglobin hasn't dropped a bit - or better, yet, it's gone up. That has happened. Ok, it's only gone up on its own once, but that doesn't change this goofy hopeful heart of mine. By the same token, I understand that ANY blood loss is not a good thing for Noah right now. 5 mls of blood has to be drawn out and discarded before labs are drawn - it's just what has to happen when you draw from a central line - so his labs always require more blood than yours or mine. It isn't a ton of blood, but he doesn't do well with blood loss under the best of circumstances. I'll know Monday if that are going to do labs early or not. When they do labs, we will have a much more solid, definite picture of where Noah is going with this whole hemoglobin business. If he keeps falling, we will probably ask for the fastes possible hemotology consult. I do know that he is getting paler and more tired by the day. Each day this week has brought a very noticeable change - so much that if I had taken a photo a day of him this week, you could probably sort them in chronological order. Even so, he is relatively stable. Am I allowed a small laugh of disbelief that I could even describe this ill of a child as "stable?" Oh, how our standards have changed . . . . . He doesn't seem to feel bad, and that is important to us. He played with his therapist today, showed me his eyes, nose, mouth, ears, hair, hands, feet, AND tummy (and said each word as he pointed!), drank a little chocolate milk, and figured out how to defeat the spill-proof valve in his cup so he could paint himself with the rest of the milk. We've been planning an outing to an old mill with some friends, and tomorrow is the day we planned to go. We are still planning on going. Noah may look rough and will probably want to sleep even more than usual, but it's important to all of us to keep living and loving life and having adventures. I would like to think that the outing will do Noah good, but at any rate we don't believe it can do him any harm. With all that we don't know about Noah right now, let me remind you of what we do know. I know you've all heard this all before, but it bears repeating as many times I have breath to say it. We know that God is good. We know that He loves Noah beyond anything that we could ever imagine - and He loves us that much and He loves you that much. We also know Who God is. He is El-Elyon, The Lord Most High. He is El-Olam, The Everlasting God. He is Jehovah-Shalom, The Lord our Peace. He is Jehovah-Jireh, The Lord our Provider. He is Jehovah-Rohi, The Lord our Shepherd. He is Jehovah-Shammah, the Lord Who is Present. He is Elohe Chaseddi, the God of Mercy. He is El Emunah, The Faithful. He is Jehovah Mauzzi, The Lord my Fortress. He is Jehovah Mephalti, the Lord my Deliverer. Ending with a Scripture: "It is of the LORD's mercies that we are not consumed, because his compassions fail not. They are new every morning: great is thy faithfulness. The LORD is my portion, saith my soul; therefore will I hope in him. The LORD is good unto them that wait for him, to the soul that seeketh him. It is good that a man should both hope and quietly wait for the salvation of the LORD." Lamentations 3:22-26 Let us both hope and quietly wait. Blessings, Kate { Last Page } { Page 3 of 5 } { Next Page } |
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