Follow-up on Maddy

We were all happy to be home together on Wednesday! Maddy was still not moving, but seemed more comfortable in her own space.

Thursday was a crazy day with multiple follow-up appointments. Maddy and I started with Dr. Benzick, our pediatrician. Dr. B was happy that her right lung sounded better, but concerned about her left lung as it still doesn't sound great. He wants us to watch her closely and continue our antibiotics. He will follow-up with us next week to reassess. If her lung still doesn't sound great, we will repeat a chest x-ray and decide on treatment from there. Dr. B also wanted to be sure that our immunologist was aware of the bilateral pneumonia.

We had to kill a little time in between appointments so we stopped to visit our beloved, physical therapist. Suzan was so happy to get a few snuggles and Maddy was all smiles to see "her Soozan".

We also followed up with Dr. Beam our hematologist. Overall, he is happy with how both kids are doing. Maddy's blood counts were the highest we have ever seen (not surprising with everything going on), but a good sign that her bone marrow responded which we haven't always seen in the past. He also wanted Dr. P (immunology) to be aware, especially since we are considering preschool in the fall. He is concerned about her overall immune system. Maddy attempted to take a few steps while we were at Dr. Beam's and she was so weak and unsteady, she had us all nervous.

As for Alex...from a hematology standpoint he has been great. It is hard to even remember the days when he required constant transfusions to keep his blood counts up. I did show Dr. Beam the picture of the mass that was found during his colonoscopy and we discussed the best way to image it further. he did agree wit all the other doctors that it looks benign, but will have to be surgically removed.

Friday was a special day. Zoey came home! Zoey was supposed to be with her trainer for several weeks in April. We were supposed to pick her up the week that Alex got sick. By the time we got home and could coordinate a pickup there was only 2 weeks before our Disney trip so we decided to grab her after the trip. We have had two other pickups scheduled, but both times Maddy wound up in the hospital. Friday we were finally able to coordinate to bring our girl home. When we arrived at Hilton's she was so excited to see Alex. She has stayed by his side for most of the time since we've had her. We are so grateful to Hilton and the butler Family Foundation for working with Zoey and taking such good care of her over the past few months.

Zoey trying to sit on Alex

She just couldn't get close enough to him

Happy together!

It's not easy when your dog is double your size!

It's Hard work being a Service Dog!

Maddy was a little more mobile Friday, but still extremely shaky. Saturday she seemed more stable as she is regaining strength. Unfortunately, we have run into another potential problem. Maddy has a lump next to her tube that is about the size of a marble and very tender to touch. She is not running a fever (although she is getting advil for pain). We will need to see Dr. Benzick in the next day or so to figure out what it is and if it needs treatment. 

I am going to do something I never do....I am not someone who is very into politics. I don't like the conflict and the deceit. However, we are at a very scary time. The uncertainty in healthcare should be frightening for everyone, but for a family like ours it is horrible. The concern is twofold. One, removing the protection for pre-existing conditions could leave both of my children with no insurance. Or premiums that are cost prohibitive to obtain. Reinstating lifetime caps on benefits could also be detrimental. Alex's care is astronomical in cost. His care has been well over the $1 million dollar mark. Secondly, Alex has medicaid as a secondary insurance. He has this based on a waiver aimed at keeping medically fragile kids out of nursing homes. Despite his having secondary insurance, our out of pocket costs are extremely high.  On our taxes last year our out of pocket medical costs accounted for almost 40% of our income. We struggle to make ends meet now. Without the blanket that Medicaid provides we would not be able to pay the bills necessary to have a home and food to eat. This SHOULD NOT happen. Please take a moment to call your representatives and ask them to oppose the current proposed bill especially removing the pre-existing condition protection and the cuts to medicaid.

We need some specific prayers and thoughts this week. 

1. Maddy to continue to recover and regain her strength with no further setbacks. 

2. Continue to pray for Alex. His feelings are much like mine. He is shaken emotionally with all we have struggled with. He told me after he almost died in 2010, things got a little better each year. Now he feels like we are right back where we were. I have tried to reassure him, but it is a scary place for all of us.

3. Prayers for Bruce regarding a presentation he is making Tuesday.

4. Prayers for me as I am emotionally and physically drained. 

Thank you for your continued support and love.

Ali

Poor Maddy

I wanted to quickly recap the last week with Maddy while it is still fresh in my mind!

Last Wednesday was the day of her surgery to place her feeding tube. She was not nervous. (One of the benefits of having a brother with a tube) She was happy that she was not going to have to take her yucky medicine anymore. They took her back for surgery around 8:00 and she was done pretty quickly. When I got to recovery they told me she was having quite a bit of pain and had already given her  2 doses of fentanyl. When we got up to her room she was grunting in pain and really struggling so we gave her a dose of morphine. While we were doing our intake with the charge nurse, we realized that Maddy's anesthesia report showed that she had gotten lactated ringers (which is extremely dangerous for a mito patient). Our nurse stopped intake and scrambled to get risk management involved as well as check labs to be sure Maddy was doing okay. I also asked her to check Alex's paperwork since he had anesthesia as well. After the investigation, it was determined that the lactated ringers was pulled , but they caught their mistake before she actually received it. However, Maddy's blood gas came back not great. All afternoon we struggled to try and get her comfortable. We increased her dose of morphine 2 times and also added in hydrocodone. She was so pitiful. She laid completely still in the same position all day and barely said anything to us. She only talked to ask for more meds. Her little HR stayed in the 150-170 range. That evening we started pedialyte through her tube and she seemed to tolerate it well.

In Pre-Op

Not feeling good

Thursday they really wanted her up and moving. We tried and tried and finally got her to take a few steps. She was crying and begging to go back to bed. WE put her back and she immediately fell asleep and slept 6 hours! While she was sleeping we tried her first feed of formula. When she woke up she  had a fever. We had been on the fence about whether to discharge or stay one more night, but a fever made the decision for us! It was decided to try one more small feed in the evening, but hold overnight feeds in case the fever was related. 

Big brothers make everything better

Friday she had had no more fevers and was a little more chatty, but still did not want to move at all. But the doctors and I decided that we felt like she was well enough to go home. We made it home around 3:00 on Friday. Maddy was still not sitting up or walking. She just wanted to lay flat or slightly inclined. We got home and I gave her another small feed. She fell asleep and I struggled to wake her up so I decided to check her temp. Sure enough her temp was back up to 101.8. I could not bring myself to drag her right back to the hospital so I decided to give her some meds, let her sleep and reassess in the morning.

Checking out her tube

Home

When she woke up Saturday morning she still had a fever and her pain was horrible. She laid totally flat on her bedroom floor for two hours because it was too painful for me even to pick her up and move her. I called the doctor on call and he told me I needed to bring her back in. Both the Dr and I questioned whether something was wrong with the tube. At the ER, they started with some labs and fluids. Most of her labs looked okay, but her Sed Rate and C-Reactive protein were both very high. We went to X-ray and I was shocked when the doctor came back and said it looked like she had pneumonia. We had had some low O2 sats in the hospital, but she was not coughing or wheezing. Next we went to ultrasound. That didn't really give us any answers so they decided to proceed with a CT Scan. The CT didn't show anything with the tube, but confirmed pneumonia in both lungs as well as a pleural effusion on the left. They admitted us under the diagnosis of double pneumonia. It was decided to hold feeds through the night and resume in the morning.

Back in the ER

Sunday we saw one of our favorite hospitalists. He assured me again that the new tube was fine, but I told him my mom gut was that we were missing something. Her significant pain just didn't seem to fit with pneumonia. He ordered labs for the morning, but didn't tell me exactly what he was ordering. We just had a quiet day on Sunday and let Maddy rest. It was decided that we would start slow feeds Sunday night at just 10 cc/hour (2 tsp) and if she tolerated them to start increasing in the morning.

Monday we woke up to the realization That her lipase was high and she has pancreatitis. I am so glad that Dr. W has enough knowledge of our family to think to check her lipase because I had tunnel vision on the tube! We once again stopped feeds. She continued to have pain, but as the day went on she was a little more chatty.

By Tuesday morning she was begging us to eat. Her lipase Tuesday morning was the same, so we decided to let her try and eat. She ate a little bit of breakfast, but never ate anything else all day. The only other development on Monday was that she was finally pain free enough to attempt to stand. When we tried to get her up she was too weak to hold herself up. That sparked a PT consult. We also restarted some feeds Tuesday afternoon and evening.

Wednesday morning her pain was a little worse because we were feeding, but her labs had stayed the same so after some discussion with the doctors we decided to come home. She continues on antibiotics for the pneumonia. The pancreatitis will either resolve or get worse.,. only time will tell. It didn't make sense to sit in the hospital just to wait and watch to see what direction she is going to go. We are happy to be home. Sweet girl is still having some pain, but otherwise tolerating her feeds. She is extremely weak. Alex's nurse helped me give her a bath yesterday, because she is too weak to hold herself up unsupported.

Happy to be home...hopefully to stay!

We follow-up with her pediatrician today as well as her hematologist.

Thanks for keeping us in your prayers.
Ali

Medical Update

The other post was much more fun to write, but I do need to also get out a medical update. I will keep this one pretty short.

Right before we left on the trip, we had a few major things happen. Alex had his CT Scan to try and look at the growth that was found during the colonoscopy. Unfortunately because Alex has such severe gastroparesis (dysmotility) we could not get any oral contrast in to him and they were not able to visualize it. He has another GI procedure on Wednesday with anesthesia to try and help his motility. Dr O was also going to talk to radiology and find out what we need to do to figure out what this growth is and what need to happen next.

As we were walking to the CT Scan, We ran into Dr. O and he stopped me in the hall. All of Maddy's testing came back. The good news is that she does not have Cystic Fibrosis or Celiac Disease and her motility is not as bad as Alex's. However, she is still having significant malabsorption. She eats over 2000 calories a day and has only gained one pound in a year. She is also chronically dehydrated. With all of that Dr. O has decided we cannot sit back and wait anymore. She will be having surgery on Wednesday to have a feeding tube placed. She will continue to eat (because anyone who has ever been with Maddy knows she LOVES to eat) and we will use the tube to supplement her with a specialty formula that she can absorb. I had hoped not to travel down this path with Maddy, but am hopeful that she will feel better and start to make more progress in therapy!

Please pray for this Mama's heart on Wednesday as I hand over both of my babies to the anesthesiologists and Dr. O and Dr I.

Ali

A Once in a Lifetime Adventure

I am not sure I can even put into words what this week has meant to us and how amazing it has truly been. I am mostly going to let the photos speak for me, but I have to touch on a few things. First, I have to say Thank you to Kidd's Kids, especially Caroline Kraddick, Lyndsay Davis, and Logan May who not only worked tirelessly to make this week happen, but spent the first three days with us and are such incredible woman. Their kindness, their passion, their love was apparent in every minute they spent with the kids. Second, Give Kids the World Village is a magical place. The amenities are incredible, but more then that the feeling, the atmosphere, the energy is full of hope. Third, sometimes you are lucky enough to meet people you connect with immediately and you know they will make a difference in your life. That is what meeting the Glenn's has been to us. Joy and I started communicating online a few days before the trip. The first two days in Orlando were fun, but our trip changed when we connected on Tuesday. From Tuesday on our families did everything together, We ate together, went to shows, rode rides. We were inseparable. The love they showered on Alex and Maddy was priceless. The 5 kids loved every moment together and I'm pretty sure us adults enjoyed it almost as much! Mitochondrial Disease has robbed so much from Alex, but the thing he misses the most is seeing friends on a regular basis. To wake up each day knowing that he would have unlimited time with friends brought more joy then I can express. Joy, John, Jaycie, Jayla, Jacob...Thank you for making this trip more then I could have imagined. The other families were equally remarkable, strong, friendly. This trip gave me something I haven't seen in a few months...my carefree, joy-filled, fun-loving son with a huge smile on his face. Thank you to everyone who made this week a week we will never forget.

Day 1- Travel and Group Dinner- This day was a hard one. We met the eleven other families at the airport and spent some time getting to know each other and talk to the media. The flight was uneventful. We had someone meet us at the airport and get us our rental car. By the time we arrived at GKTW it was POURING. Because of the rain, we were held up at the House of Hearts. We were all soaking wet and cold by the time we got to our villa. We had no time before we were meeting for a group dinner and surprise guest.  By the time we arrived at the dinner, Alex was feeling really bad. He could not enjoy that night at all. Maddy on the other hand had a blast. There was a stage in the room and we couldn't get Maddy off it. The Disney star, Laura Marano, from Austin and Ally came to spend time with the kids, but Alex felt bad enough that we left early and headed back to the Villa. We gave him multiple medications and he was up several times feeling terrible throughout the night. I was really worried about whether he would make it through the week.

 Getting ready to leave

 At the Airport

 Caroline and Alex

 On our Way

Alex and Laura Marano

Day 2- Animal Kingdom and Epcot- Alex woke up still feeling pretty puny. Luckily he started to feel better as the day went on. The highlight of the day was seeing the Lion King show as a group. Maddy sat on Caroline's lap the whole time and loved the animals and dancing. We all enjoyed the safari, but Bruce, Alex and I decided that the new Pandora- The World of Avatar was our favorite! After a quick break back at GKTW we took a bus over to Epcot for a dessert party and private viewing area for the fireworks. 

 Maddy and Caroline- These two had such a special connection

Day 3- Magic Kingdom and Hollywood Studios- Magic Kingdom is always magical. Maddy had been waiting weeks to ride on Dumbo. It was everything she hoped it would be. She also rode her first roller coaster, the barnstormer, and much to Alex's disappointment  didn't even cry! She did not like the Haunted Mansion. Alex loved Big Thunder Mountain Railroad and Seven Dwarfs Mine Train and we rode both multiple times. Alex's Genie Pass was able to gain us access to lunch at the Beast's castle. It was really awesome to see the inside of the castle and, of course, the rose!  After a full day at MK, we quickly went to Hollywood Studios to ride a few rides over there.

 Loved watching these two love on each other

 So fun to run into our Make-a-Wish friend, Pierson

Day 4- Universal Studios- This is the day we connected with the Glenn's. Alex loved the Harry Potter rides, the Simpson's ride and really just paling around. There wasn't much for maddy at Universal, but she was happy to stomp in muddy puddles with Jayla. Thanks to Mona and Melissa and Joy and Glenn, Bruce and I got to ride some big roller coasters together for the first time in 13 years!

 This bubble wand was the best purchase- kept Maddy happy for days!

Day 5- Magic Kingdom and Animal Kingdom- Alex's 13th Birthday! We let him choose what he wanted to do this day. He chose the Magic Kingdom and then back to Animal Kingdom to ride on a Banshee again! The Glenn's took Alex for a little while so Maddy could do some of the smaller rides and meet all the princesses. She LOVED this day and Fantasyland. Every time Joy texted me a picture, Alex was grinning ear to ear!

 Maddy was sound asleep for these next pics!

 Bruce and I even got to ride some rides together!

Day 7- GKTW and Heading Home- By Friday we were all exhausted. We decided to enjoy the morning at GKTW before heading home. It was so hard to leave the Village and even harder to say goodbye. 

 Alex's Star in the Star Tower

 Our Villa

 The lobby of our building

 The entrance

 Outside Amberville

 We couldn't get Maddy out of the spa!

 We rode this carousel countless times!

 Alex writing his name on his star

 Alex and Jayla goofing around

 Disney Springs

 We were lucky to have a flight at the same time as my sister (who was leaving on vacation). We decided to meet for dinner!

 Alex and Skye

 The two princesses

 Maddy with "her girls"

 The Entrance to GKTW

 House of Hearts

 Amberville

 Movie Theater

 Ice Cream Palace

 Castle of Miracles

Hopefully, you enjoyed that snapshot of our trip. I didn't take a ton of pictures because I really tried to be in the moment and enjoy this amazing week. Joy probably has more pictures of Alex then I do! So if you want to see more, check out facebook.

Thank you for to everyone who supported us in making this trip happen.

Ali