Closer to Home

Alex is doing wonderful! We are trying to tie up some loose ends so we can get home. There is talk of discharge tomorrow or early next week. We are planning on spending a night or 2 with the Knights before we heaad home.

I have to say how very proud I am of the Knight (and Ladd) family. They have been so strong and full of peace. They have exuded love for Samuel and all around them. They have shared their faith and inspired us all.

The services have been beautiful. It has been so hard to say goodbye to "my baby", but so heartwarming to see how many people loved him. The final service is today. It is here at the hospital for staff. Missy's family will be here. Alex and I will attend and have been asked to speak. Alex has a few cute stories to tell. I am going to try and speak, but am not sure I can. If I can get it out, I plan on thanking the Knights for being our second family and for giving Alex the little brother he has always asked for. I watched Alex and Samuel grow so much in the last 2 years. I can't count the number of times they held each other hands for procedures. So many times when Samuel didn't want to do something we would say, "but Alex does it". I loved watching Samuel follow Alex and do whatever Alex was doing. I will forever be grateful that the boys had each other.

Please pray for Alex and I to have the strength to say what we need to today. PLease pray for the Knights as the week of services comes to an end and they try to find there new normal.
Ali

Trying to smile again

These last few days have been so full of tears, but also full of stories and memories. Memories that have made us laugh and cry. I have been so blessed to have so many people looking out for me and checking on me. I am so thankful for everyone here and and all my friends and family who are far, but wish they could be here. Your messages, calls, emails and texts have kept me going.

Alex continues to improve. His fever has been gone since Saturday. His headache is long gone. He has been up today playing and walking. He is off oxygen. We have started feeds at a very slow rate. We still have a few things to accomplish before we come home. We need to try a new seizure medication. We always have to be careful when trying a new medicine because of his history of anaphylaxis. It also looks like Alex may need to have a transfusion in the next couple of days.

Please keep the Knight family in your prayers as several days of services start tomorrow.
Ali

Sad, but okay

I know a lot of you have been worried about both Alex and I and I am sorry I haven't updated.

I'll start with Alex....Today he has been fever free all day. Praise God! He is still pretty puny, but he is talking a little more and his personality is back. I am so glad to see his little smile again. We will still be here a little while. We need to get him off oxygen, restart feeds, try a new seizure med and get his strength back before we can come home. I am thinking the end of next week.

Emotionally he is doing okay as well. Yesterday morning, Dr. Koenig, Stacy (child life), Glenda (chaplain) and me all came to talk to Alex together. I told him that Samuel had gone to heaven to be with Jesus. I asked him if he understood and he said that Samuel was playing and eating ice cream. Dr. Koenig talked to him about their differences adn why he doesn't have to worry. Later he told me that he didn't think Samuel needed oxygen anymore. I told him he also didn't need his port or tubes. Alex told me when he goes to heaven he still wants his tubes. Today he is asking more questions heaven and processing through his thoughts. Glenda and I have both talked to him and he seems pretty good. He also did some art for Lauren.

I am doing okay as well. I am surrounded by love and support. This is a great place for me to be. The staff here is like family to us. We have laughed and grieved together. We have shared hugs and tears. We have told stories about Samuel and comforted each other. I have spoken to Missy and her family and been a go between for many people. I appreciate all the messages, emails, texts, calls, etc. Please forgive me for not returning them all. I am emotionally exhausted.

Please continue to keep the Knights in your prayers. Lauren could especially use some extra thoughts. We all miss Samuel, but are so happy he is finally free of all his tubes and hurt, free to play and eat all day.

Ali

My precious Samuel

My second baby, Alex's little brother, lost his fight with mitochondrial disease last night. Samuel was a joyful, loving, sweet little boy who we all loved completely. I ask for prayers for some of my best friends, Missy and Ben, their daughter Lauren and their family as they try and make it through these next few days and weeks.
I also ask for prayers for Alex today as we talk to him and that his little heart understands and that he is not scared.
Ali

Up and Then Back Down

Last night was a much better night. We went for a ct scan around 11:00 pm. Alex's temp stayed in the 101 range all night. It really looked liked things were turning around. We saw ENT very briefly this morning and they said the CT report wasn't up yet, but that from the images it didn't appear that the sinuses were as bad as initially thought. Our team came by and we started brainstorming again. It was decided to do an echo of his heart and an abdominal US. But we all agreed that he was doing better and we just might not ever know. It was also decided that we would continue the antibiotics to finish a week or 10 day course since they seemed to be helping. Then all of a sudden around 1:00, Alex's temp spiked back up over 103. Dr. Koenig and Dr. Pacheco both came by to check on him. They also called in the infectious disease doctors to get their input. They are starting to think this could be a virus. They have run a bunch of labs looking at things like Mono.
Right now Alex's temperature is back to 104.6. We have the room temp at 55 degrees. Alex is once again wrapped in wet cloths and has a fan blowing on him. He is still on oxygen. We also have him on TPN and IV meds trying to lessen the stress on his body.

I also ask that you keep Alex's "little brother" Samuel in your prayers. Samuel got really sick last night. He has a nasty line infection and is in the ICU.

I will update if anything changes.
Ali

A Quick Update

A long stressful day and night! I never slept at all last night. Alex was really agitated and his fever climbed all night long. It reached 105.3 at about 3:30 in the morning. The doctors came and decided to run a blood culture and some other labs necessitating sticking him to get blood. We finally started to get him settled close to 5:00 this monring. He had not bee asleep long when he had a nother seizure. Once again the doctors had to come an dat that poin they stayed in our area until our regular team arrived this morning.
Alex's temperature stayed at 105.5 for most of the morning. Concern grew as we couldn't get it down and it was decided we needed to be aggressive so the temperature in our room was lowered to 55 degrees, his cooling vest was soaked in ice water and put on, we placed cool cloths on his head, neck and feet and ice packs under his arms and in his groin. Then we set a fan up to blow on him. With all of these measures we were able to get the fever down to 103.7.
In the meantime, Dr. Koenig came in with the news that they had found the source of infection. His brain MRI's showed some pretty nasty sinusitis. And so a plan was formed. We consulted ENT, immunology and hematology. At this point it is thought that it is likely they will have to go in surgically and clean out his sinuses. The ENT has ordered a CT scan to be done tonight or tomorrow morning and then a definite decision will be made.
Alex had another possible seizure this afternoon despite an extra loading dose of seizure meds. We are giving high doses of meds and watching closely. We have also left him on oxygen. I think that is all. I will keep you posted.
Thank you for all the thoughts and prayers.
Ali

A long day

Oh what a day it has been. I am tired and worn out and exhausted all rolled into one. Despite meds and cool cloths, Alex continued to run fevers over 102.5 all night long. The doctors were in early this morning and the decision was made to go ahead with the brain MRI, spine MRI, and lumbar puncture. Alex conintued to complain of his head hurting and we waited for our time to go down. As we were getting ready to be wheeled to the MRI suite, our team came by to tell us that they were cancelling the spine MRI. After the pediatrician, Dr. Koenig and the anesthesiologist spoke they decided it was too dangerous to put Alex under anesthesia for that length of time with him as sick as he is. We got downstairs and met a new anesthesiologist. He struggled to get Alex sedated. When we finally thought we had him asleep I got ready to leave and Alex started seizing. He had several seizures and I waited to be sure he was okay before I left. This was sure not the way I wanted to start the procedure. Apparently after I left Alex woke up again and it took them quite a while to get him under a second time. So the MRI portion took a lot longer then anticipated.

In the meantime, I got called to surgery waiting to meet the radiologist who would be performing Alex's Lumbar puncture. They needed me to sign consent. As i was speaking to the IR doc I asked her to check his clotting labs that had been ordered this morning. Sure enough they were high. So with about 30 minutes until they were going to get Buggy we had to come up with a plan. After calls to several doctors we decided that we would be okay to forgo plasma and just give vitamin k once we got up to our room. So all in all th eprocedure that should have been about an hour and a half took four hours.

Alex continues to run fevers over 103 tonight. His back is swollen at the incision site. We have added a pressure dressing. We are unsure if it is "just swollen" or blood that has accumulated. We will continue to watch and make sure that is okay. From the little bit of results I have seen, it does not look like meningitis which is great. But it leaves us struggling to figure out why Buggy is still struggling.

Thank you to everyone who has checked in by phone, email, or facebook. I appreciate all the thoughts and am sorry I can't get back to all of you.

Ali

Pretty Sick

Poor Buggy is pretty sick. He woke up this morning with a temperature of 98. By 9:00 it was up 10 101.4 and by 10:30 it was up to 104.3. We quickly packed up and headed to Houston. He has the doctors pretty concerned. He is barely talking and is just lying around. So unlike his usual self. His labs in the ER did not look good. There was some concern that he may need to go to the PICU, but we did wind up in our usual unit.
Other then the fever, Alex's main symptom is a horrible headache. He has been in tears 6 times today crying about his head. Because of the headache there is some concern that he could have meningitis. He is being squeezed onto the schedule tomorrow for a brain MRI (we will also do the spine MRI that needs to be done). There is also some discussion about whether he needs a lumbar puncture. We have stopped feeds and started IV fluids to prepare for these tests tomorrow. We are waiting on cultures from his central line. We have not gotten his temperature below 103 even with tylenol.
I am going to try and get some sleep. I will update as I know more tomorrow.
Ali

Mitochondrial Disease Awareness Week

If someone had asked me before Alex was born what mitochondria were? I probably would have looked at them like they were crazy. I have long ago forgot much of my early biology. (Although I better start learning so I can teach Alex!)And yet these tiny parts of our cells have impacted our lives in ways I never could have imagined.




The mitochondria are equivalent to a battery. They make the power for things to work. When the TV remote's battery dies, the remote can't do a thing until the battery is replaced. You may be able to coax a few more uses out of that battery. You take the cover off and spin the batteries. You shake and hit the remote. You stand in just the right spot and you may get the volume adjusted a bit. But, ultimately, you know the battery is done for. The same works for our mitochondria. When they are sick, they lose the ability to power our body. We can do little things to improve their function... take vitamins, avoid stressors, preserve the energy used, but ultimately, the mitochondria are failing. But, unlike the TV remote, there is no way to replace the power source. No fix. No treatment. No cure. (Thanks Missy for this analogy)

Mitochondria keep Alex from doing so much that every child should enjoy. He can't eat, he doesn't go to school, he spends 2/3 of his time sleeping, he tires out making it impossible to play sports or even go for a walk, he spends a 1/4 of each year in the hospital. Our biggest hope is that a cure will be discovered in Alex's lifetime. The way to find a cure is by spreading awareness and funding research.

This week as you get out of bed and go about your everyday activities, activities that most of us take for granted, please think about Alex and all his little buddies and spread the word about this devestating disease!

A Heart Full of Joy!

This post has been brewing in my head for the last few days. It is hard sometimes to take my feelings and adequately get them into words to express exactly what I want. But here's a crack at it!

As a little girl, my most fervent dream was always to be a mother. I was the girl who started babysitting at 10. The one who would gravitate towards the babies at events. The day Alex was born not only was the happiest day of my life it also was MY dream come true. Of course, God's plans for my life and the way my dream would play out are a little different then I envisioned! Our house is not like most houses. Our closets are filled with medical supplies. Alex has a refrigerator in his closet full of medications and IV fluids. I could practically run an ER in his room. Our days are full of giving meds and taking vital signs. And yet I strive every day to make life "normal" for Alex. We have a ton of limitations, but I am always looking for ways to push the boundaries. For activities for Buggy and friends and the childhood he deserves. Some days I feel like we do a good job and other days I feel like I have utterly failed. And so as I begged the doctors last spring to let Bug go to school this year which was met with a resounding no, 15 seconds into the conversation by 2 separate doctors, I pondered what we could do. Although Bruce and I are both sports lovers, sports were out. And so I asked about cub scouts and was told we could give it a try with restrictions. And so all summer I told Buggy how awesome cub scouts was going to be. How much fun we were going to have. How cool it was. It didn't hurt that one of his best buddies Walker was going to join him on this journey. And so last week our adventure began....

Kelly and I ventured to the cub scout store last Friday to buy the boys their uniforms. All I can say is thank goodness there are people there who know what they are doing because we were clueless! We quickly realized the pants were a no-go as the smallest size is a size 6 (about 3 sizes too big for Bug). But we gathered everything else. Saturday We got Buggy in his uniform for the first time. And oh my goodness (I know he is mine BUT) he is the cutest thing ever in a uniform!

We headed out to sell popcorn for the first time. As those of you who know him can atest too, he is a natural salesman. He had the other parents hysterical as he convinced person after person to donate or buy popcorn. I mean who could say no to the little pipsqueak with the sweet face. He came home exhausted, but so happy to have made new friends. Friends who were now his best friends forever.

Yesterday, was our first pack meeting. So once again we donned our uniform. He was so excited to see his friends again. His den all sat together. It warmed my heart to watch him make the cub scout sign, stand for the pledge of alliegance, and follow his friends outside to play while the parents met. I love this glimpse into what life is supposed to be. I love to see him enjoying being a kid. I love to watch the joy and excitement as he runs to catch up with friends. I soak in every minute. At times it is bittersweet because I realize how much he misses out on and yet he is so happy and appreciative of every opportunity he is afforded. And so we came home last night with new found friends and a community to belong to. We look forward to our weekly meetings and our upcoming camping trip. (I am still figuring out how I am going to swing 2 days of medical supplies/procedures while camping) And as a Mom I am so very proud of my little man who adapts to every situation and finds so much happiness in his crazy life!


Let's see what else....

School is going awesome! Alex likes his new math curriculum a hundred times better then last years. We have also changed where we are doing school. We used to sit in Alex's room, but he was having a hard time focusing. We have moved to the dining room table and he is doing much better. He enjoys history and already has favorite books. Creating our hideaways (forts) has been a hit. Overall, it has just gone really well.

Medically things are also pretty good. We are waiting on a date to head back to Houston to repeat the MRI and SSEP to determine whether we need to "fix" the syrinx now or just continue to watch it. We have also started the MCT oil to help him gain weight. He is at 1/5 of his dose and has had some minor issues, but we are continuing unless it becomes worse.

Last, Alex is starting to sell popcorn for cub scouts. This is hysterical to me. Alex's first anaphylactic reaction was to corn. Seriously...of all things popcorn sales! Anyway, I am going to attempt to post the link to his page here, but am not sure the shortcut will work. Do not feel obligated, but if you would like to support Alex on his cub scout journey you can click here:
http://www.trails-end.com/estore/home_alt.jsp?_requestid=327417 If you need his scout id #, it is 7676878.

Thanks for allowing me a place to share my feelings and more importantly my Bug!
Ali

PS- Guess who is the proud leader of Den 2....Me!

Exhaustion

Sorry I haven't updated yet, but I have been exhausted. We arrived home on Thursday afternoon. It is always hard trying to get caught up on household chores and get back into "normal life" routine.

I need to update you on his two appointments we had on Wednesday. We started with cardiology. It was a LONG appt. We were there from 9:00 until 1:15. After all was said and done, the cardiologist felt that his heart is doing well. She wanted to look at the images from the echo again and see his lab results, but she didn't expect to find anything. So that was awesome news! next we saw hematology. Dr. Brown is a doctor that I like more and more each time I see her. She increased Alex's Vitamin B12 a ton. We also discussed when we will use the Vitamin K shots that help with his clotting. The last thing we talked about is surgeries and what needs to be done before each surgery. She would like Alex to receive a plasma transfusion before any surgery that "could" cause bleeding. So all in all we had a productive appt. We were at the hospital from 8:30-5:00 so it was a long day.

After leaving the hospital, Missy and I left the kids with Ben and headed off to a meeting about an upcoming fundraiser for mitochondrial disease. Some of you may remember that 2 years ago I walked in the Christopher's Heart Fun Run. That event has teamed up with the UMDF and this year (February) it will be the Energy for Life Walkathon. I am excited to walk with Alex and to raise money for research to find treatments and hopefully a cure for mito.

I promise a "fun" update soon with some pictures.
Thank you for continuing to lift our family in prayer.
Ali

Last Day

Tomorrow is our last day in Houston before heading home. We will have been here almost 2 weeks. Today was a quiet day with only labs to draw. His labs looked pretty good. The only issues were hematology related which is funny because we see hematology tomorrow. We also see cardiology finally!
Many of you may remember that I walked in a fundraiser to benefit the UMDF a few years ago. I am planning to do that again this year so I will attend an informational meeting tomorrow before I head home.
Please say a prayer for PopPop (Mom's Husband). Mom had to take him to the ER tonight with a high fever and not feeling well. We love you Mimi and PopPop.
That is all I got! I'll update either tomorrow or Thursday from home.
Ali

Discharge

We are hopefully heading home (to Missy's) today!



Alex is doing very well. He is having some pain around the tube, but it is tolerable. Our feeds are up to 20 cc/hr. he is tolerating them like a champ and I don't see any reason why we won't be on full feeds by Tuesday. Yesterday was just a quiet day. Alex was happy to wean off his O2 in the morning and then spend the day "visiting" with all the nurses.



Alex's Vitamin B12 level came back okay. This is both good and bad. It is great that he is absorbing it better now. However, we were hoping low B12 levels were to blame for some of his other symptoms. Because it is normal, it is more likely that his syrinx is causing these issues. I will talk to Dr. Koenig this week and see when we will repeat the MRI of his spine.



We are still in Houston a few more days. Wednesday we finally see Cardiology and then we also see Hematology. We should be on the way home Thursday! Alex and I are ready to get home and back into our schedule and doing school.



Ali

Even Worse!

If I thought yesterday was bad I had no idea what was in store for me today! After updating last night I went to bed still unsure of what the final plan was. At midnight I was woken up by the surgery resident to sign consent. It was a consent to replace his port. I let him know that this was not the plan I had discussed with Dr. Tsao and was he certain we were not going to TPA the port. I was assured that this is what Dr. Tsao wanted. So I went back to sleep thinking Alex was going into surgery this morning to have his Jtube replaced, scar tissue removed and port replaced.
I was once again awoken at 6:30 this morning by the surgery team. I reiterated my concerns with the plan as it was not what I had discussed with Dr. Tsao. They agreed to call and double check. Less then 30 minutes later we were headed to pre-op. Alex cried the whole way down. He finally settled down when our favorite anesthesiologist came in and said she would be in the OR. We waited about 45 minutes and then someone came in and told us that we were going back upstairs. They had cancelled the surgery because Dr. tsao wanted to try TPAing the port. I was obviously frustrated.
We got up to the floor and immediately I asked how we were going to address his Jtube problems if we weren't going to the OR. We were told by the surgery resident that they were going to sedate him bedside and do it. That was the final straw for me. Alex is damn near impossible to sedate. The anesthesiologists frequently comment that "it takes enough to kill 3 grown men" to sedate him. That can not safely be done without the ability to intubate. On top of that I didn't want Buggy to be only half asleep and be in pain while they cut off tissue. The final thing is that Alex is VERY afraid of "going to sleep" (anesthesia). Right now he loves our normal unit of the hospital. I did not want to do anything to make him afraid of being here with as many days as we are here.
Since the nurses and I weren't getting anywhere, I decided to email Dr. K. She came to the rescue and got in touch with Dr. Tsao. She let me know not to let them do anything. That Dr. Tsao would be up to see us when he was out of surgery. In the meantine we TPAed the port and were able to get blood! Woohoo!
I waited and waited and Dr. Tsao finally made it up here around 1:00. He made the decision that it was best to head to the OR to remove the scar tissue and replace the Jtube. Less then 10 min later they were here to take us back to pre-op. So off we went with Alex crying again. Luckily, our child life specialist came down with us and helped me distract Alex. We had an anesthesiologist we know and his fave came by to check on us again and make sure they knew the ins and outs of taking care of buggy. Alex's was taken back around 3. Dr. Tsao was pretty quick, however, he came out of the OR telling me that the port had given them some issues. I am somewhat concerned at this point that we made the wrong choice by not replacing the port. So far it is working okay since we have been in the room. I am hoping that it will continue to work ok without any further problems.
Alex was as sweet as possible coming out of anesthesia. The first thing he did in recovery was pull me close and tell me that he loves me. He is doing really well. He is saying his belly doesn't hurt at all. He is also tolerating the oxygen (that he is always on after surgery) better then he ever has. He is tired and getting ready to crash for the night.
I continue to struggle with the care Alex is getting on the surgery team. They are not use to having to deal with the complexity of his issues. They don't know us and the way we do things. I feel like I am having to fight to get him cared for correctly. I am tired of putting out fires because they don't know us well. I am hoping they will listen to me and the nurses and that tomorrow things will improve.
Thank you for all the prayers today.
Ali

So Frustrated!

That has been the most exasperating day! We arrived at the hospital at 9 Am to be direct admitted. The plan was to TPA the port to try and get it functioning again and reassess his tube to determine whether he needed surgery. We never got a room until 3:00. Alex and I then sat and waited as the hours ticked away to see a surgeon. At 7:00 the surgeons finally came to write orders and left without ever speaking to me. Our nurse came in and told me that they would not write orders for the TPA. The orders were to stop feeds at midnight. We will head to the OR tomorrow and they will remove the tissue around his tube, replace the tube, and take care of the port. I have been trying to get clarification on if the port is being replaced or what will happen, but I cannot get answers tonight. From what the nurses have been told, the surgeon wants to assess the port when they are down there. I will not sign consent until I speak to Dr. Tsao and get a better feel for what is going to happen. In the meantime, Buggy had to be stuck to get labs because they have to check his clotting and blood counts before tomorrow.
Please keep Bug in your prayers tomorrow and I will update when I get a chance.
Ali