Pittsburgh, Round Five: Day Four

Today was our last day in Pittsburgh and it was a fairly easy one.

Tori had lab work to check her potassium levels first thing this morning. They have stabilized with the supplemental potassium we have been giving her, so that will continue.

 

We then went to do her Visual Evoked Potentials eye exam, which is an interesting experience.

They play a movie and then change the screen to a black and white checkerboard screen that moves. Tori had sensors all over her head to detect brain activity as she was being stimulated.

We checked out of our room at the Ronald McDonald House and were surprised to find out that someone had anonymously paid for our stay! (For those who aren’t aware, they suggest a $15 per night donation to help cover a fraction of the costs. They never turn anyone away for financial reasons.) Thank you to whoever generously paid for our lodging!

We had the privilege of meeting a young lady – Johanna – with metachromatic leukodystrophy (MLD) who has been at the hospital for months now receiving a stem-cell transplant. You can follow her story here.

Her mom has been following Tori’s story and reached out to us during the week. We discovered that we were actually next-door neighbors at RMH! We were so happy to get to meet them.

Please pray for Johanna (JoJo) and her family as they finish up their time in Pittsburgh and as the transplant continues to save her life.


After that, we had lunch with Anniston’s family and were then joined by two ladies who started a ministry to support families of leukodystrophy children. They call it Hope Alive. They brought gifts for the children and roses for Reesa and me. I love that they are advocating and raising awareness as well as being so focused on supporting the families who are going through so much.

We said our goodbyes to the Bazar family (who we love and are SO glad we got to see so much this week) and we drove home. Tori did extremely well and seemed to be so alert and comfortable the entire way.

We have many specialists with which to speak this week to address the things that were discovered in Pittsburgh but we feel encouraged about Tori’s overall health.

Thank you to each of you for praying for Tori – and for us – this week. Your support means so much to us! ❤

 

Pittsburgh, Round Five: Day Three

Today Tori had a Behavioral Audiology evaluation and a chest x-ray. It was a pretty easy day.

For the audiology test they played sounds and made noises to see how Tori would react. 

 
Tori did fairly well! There were some noises she didn’t care about but others either startled her or interested her and she responded. 

We then met up with Anniston’s parents while they waited for her MRI to be completed. We have had such a wonderful time visiting with them this week!

  

  
Then Tori went in for her chest x-ray. Dr. Escolar wanted to see how her scoliosis is affecting her lungs, if at all.

       

   

After the x-ray we went back up to our room in the Ronald McDonald House and rested for a few hours. We are exhausted and it felt so good! 

Dr. Escolar wanted to meet with us again so we went down to her office. 

  

She gave us some things to consider and we will be making appointments with some of Tori’s specialists in Hershey. Nothing is of great concern right now, but if we don’t address some of these potential issues they could be a problem later.

We will get a full report with her recommendations in a week or so.

She also showed us an image of Tori’s current MRI so we could compare it to the one from last year:

Last year:

  
Yesterday:

  

Unfortunately, her brain has continued to atrophy and Krabbe is still very present. But, God is sovereign and still in control!

We will return to Harrisburg tomorrow afternoon and are looking forward to getting back to “normal” life ❤️

Thank you all for continuing to pray for Tori and for us – your support and encouragement means more than we can express.

Pittsburgh, Round Five: Day Two

Today Tori saw Dr. Escolar and her team and had her MRI.

Dr. Escolar is pleased with how Tori is doing and said our next appointment will be in six months instead of the every three months we did this first year. That was encouraging to us because that means she expects Tori to still be with us by then. ❤️

She did say that it seems like Tori is in stage four now, but she would know more after studying the MRI. Many Krabbe kids have been in stage four for years, so it doesn’t necessarily mean a quick death.

They are overall pleased with her muscle tone and flexibility. She does have some scoliosis and we will be repositioning her more often to try to help with that.

Tori really showed them how much she drools this morning, which was great because they could see what we deal with each day. We will be adding back into her routine a low dose of a med to help her not drool as much.

We will also be adding a nebulizer treatment to our respiratory therapy routine for her to help her be more comfortable. Other than that, no major changes to her care.

Dr. Escolar is pleased with how well Tori is doing on breastmilk – we are SO thankful to all those friends who have so generously been feeding Tori! It has helped her be healthy, helped her digestive system work well, and Brennan and I believe it has attributed to her overall wellbeing. 

They said that we are doing a great job with her, and that was much-needed encouragement. There are so many things we are supposed to do for her each day and sometimes we forget things and feel like we have failed in a way. Hearing Dr. Escolar say that she is pleased with our care of Tori meant so much.

After that, we headed straight to radiology and they took Tori back for her MRI.

  

Can you tell how tired we are? 😉

  
  

Brennan napped (or tried to) while we waited 😄

   

 
It was about 3.5 hrs before they called us back to see her. She did well and is very alert and talkative.

 


One year ago post-MRI:

    
  

Back to today:  
    
  

We were so happy to see Nurse Ron again – he cared for Tori six months ago at her last MRI and he remembered us! He calls Tori his little buddy ❤️

 

Her potassium is low so we will be giving her a supplement to help her maintain a good level. 

We are back in our apartment resting and hoping that Tori sleeps tonight 😉❤️

Thank you all for your continued prayers!

Pittsburgh, Round Five: Day One

Tori had two appointments today: ophthalmology and audiology.

The ophthalmologist dilated her eyes to evaluate the anatomy and we will get the results later this week.   

Tori always makes the best facial expressions when her eyes are like this 😉

  

  
  

We brought her cool Minnie Mouse shades to protect her eyes after the appointment ❤️

  

  

After her eye appointment we were able to spend time with the Bazar family and precious Anniston! Anniston is almost two and lives in Louisiana. We are so glad they are here at the same time as us!

    

Tori then had her audiology test – Auditory Brainstem Response Evaluation. She was supposed to be asleep for the test so I guess it worked out that she didn’t sleep much last night 😉

   
 

Sweet Anniston and her family brought Tori some sweet gifts – things that Anniston enjoys ❤️

   
   
 

We are so thankful for those who have brought us meals and snacks! Your kindness is so appreciated. ❤️

Tomorrow we see Dr. Escolar and Tori will have her fourth MRI. 

Tori has been drooling like crazy because of teething and we have been suctioning constantly – please pray for relief for her ❤️

It’s been a good day 😄

Pittsburgh, Round Four

Today we had our quarterly visit with Dr. Escolar in Pittsburgh and things went mostly well.

Overall, Tori is still doing really well given her early-infantile form of Krabbe. She even improved in her visual/cognitive understanding from a 2-3 month level to a 5 month level! Vision therapy has been helping!

However, as we suspected, Tori’s blood oxygen levels aren’t consistently where they need to be. They put her on .25L of oxygen while we were here to see how her stats improved and they went back to normal.

Dr. E suspects that her breathing has been affected by her distended belly (she’s had a lot of gas in the past week) and by a need for deep suctioning (for which we didn’t have the correct supplies until today) to clear more secretions from her airways.

They did a belly x-ray and found that her colon was indeed enlarged, so we will begin doing a few different things to relieve the gas. They also did a chest x-ray noted that her lungs have some atelectasis, so we will begin treating that immediately with a nebulizer, oxygen, and some other therapies.

Tori will also begin having .25L of oxygen at night, just to help her out. Dr. E believes this will also help her sleep soundly FOR THE FIRST TIME because her body won’t be working so hard to breathe. Mommy is VERy excited about this prospect. This will also give her more energy during the day.

We knew some oxygen would likely be coming soon so we weren’t surprised, but of course we’d rather her be on room air for longer. At least it’s only at night and it’s a very small amount just to help her heart and lungs work less.

Dr. E will be tapering Tori off of baclofen (a medication for muscle tone) because her tone is so good and because she thinks that will also help with her breathing. It will be nice to remove a med from her routine! Tori had been on the smallest possible dose of baclofen anyway so it won’t be a drastic change for her body.

Because Tori was her only patient today we got a lot of face time with Dr. Escolar and had the opportunity to discuss many things with her that we hadn’t been able to before. We learned a lot and feel more prepared for future decisions.

This was a quick trip this time and we are back home already. Each visit brings added therapies and treatments and we’re more than ready to have nursing assistance in the home. But, for now, we’ll continue doing things on our own for as long as we can.

Please pray for Tori’s belly and for her lungs – we hope we can treat these successfully at home and not in the hospital.

Pittsburgh, Round Three: Day Two

Today was a very busy day and we are all so tired. 

We started with Tori’s vision and physical therapy evaluations at Dr. Escolar’s office.

   
 
We then ran to the other side of the hospital for her Nerve Conduction Study, which was thankfully MUCH more gentle than the adult version (I have had 4-5 of them so I was nervous for her). 

   
    
    
 
We checked in for her MRI and they gave her a colorful pinwheel that she enjoyed watching.

  

They took her back for anesthesia and the MRI around 1:30pm and they came to get us around 3:40pm. She did well and seems to be herself again!

I haven’t mentioned this before, but we were slightly nervous about her having anesthesia this time. Many Krabbe families have seen further regression/disease progression after anesthesia and, though she can’t regress much more than she did immediately in January (smiling, talking, laughing, moving, etc.), we were nervous that it would have the same effect on her as it has to others. 

Since she does still vocalize, that is what I was most concerned about this time. But she woke up and vocalized! Praise the Lord.

   
    
   
She then had an x-ray to check her hips to make sure they are in place before she starts using her stander.

We will receive all results via a phone call from Dr. Escolar next week. 

While we pray for miraculous improvement in her brain, we are realistic and are prepared for whatever news comes our way.

We are headed home, exhausted, but happy with how things went.

   
 

If you could please pray for me (Lesa), I am feeling a little abnormal and think I may be getting a cold. It is likely a sinus infection from allergies if anything, but since it is important for Tori to remain healthy, I must remain healthy! Please pray for protection for her against any illness.

Thank you all for praying us through this trip! 

Pittsburgh, Round Three: Day One

We traveled back to Pittsburgh last night for our third visit to Dr. Escolar and her clinic.

Such a beautiful sky!   

    

We arrived at our hotel around 9:45pm, and Tori slept the entire way in “her” van! That was such a blessing. 

Tori requires a lot of stuff 😄

    
    
  

Our morning started early and we got to the hospital around 8:30am. Tori was wide-awake and content. 

  
 

Dr. Escolar evaluated Tori and asked us questions about her current state. We were able to ask questions of her, as always, and we received the results of our genetic testing from months ago. 

To summarize, we both carry the same gene mutation (early infantile form) and Tori has the same mutation (sometimes it can be different).

After her appointment we headed to the zoo, as you saw in the last blog post. 

We were unable to stay at the Ronald McDonald House as we had planned because they were fully booked. 

Thankfully, thanks to our Team Tori account, we were able to book a last minute hotel room with a freezer for her breastmilk.  

What a blessing to have those funds and not worry about how to pay for things like this when we come out for her appointments!

  

Our view…

 
Huge thanks to the Howard family for having dinner delivered to us! It was delicious! We so appreciate the amazing support you are whenever we visit for Tori’s appointments ❤️

Tomorrow is the big day – several tests, more evaluation, and the MRI. 

Please pray that Tori will do well and tolerate everything that she will go through tomorrow, and, of course, continue to pray for a miracle.