July

July hit and summer was officially here! The first weeks of July were HOT... and we learned quickly that this didn't help Eli's worsening situation. The little boy that loves to be outside was overheating too quickly in this heat and vomiting easily. Typically bringing him outside can calm him down if fussy, but in the first few weeks of July it seemed like any time it was really hot and we brought him outside, he would vomit within minutes. Unfortunately he had to miss some of our normal 4th of July activities (like being at the lake house) because of the heat. Luckily July 6th was cooler and he could spend a day at the lake then! He enjoyed floating on the lake and almost fell asleep with the constant little waves. :)

ice cubes on at 3rd of July fest

On July 10th, Eli was invited to the first ever Neuro-Muscular Clinic at Lutheran General. Typically we have been going to our clinic days at Christ Hospital, which is about an hour away from our house and where only his neurologist practices... The rest of his specialists are through Lutheran General. We really, really value our neurologist - he has been wonderful to us! - so we've commuted to his clinic to check in with him... However, this new clinic at Lutheran General is bringing our neurologist, Dr. Coates, up to help run the clinic! HUGE win, win for us... We now get to be seen in clinic at the hospital that does all of Eli's care, with Eli's normal specialists, AND we get to continue working with the neurologist we love. This clinic on July 10th was great after a long month and a half... We left with some immediate changes and things to follow-up on.

1. Our pulmonologist reviewed Eli's sleep study... And long story short we needed to follow up with our ENT to make sure Eli's tonsils were not too large. There was possible talks of removing those and seeing if that help with his reflux/ vomiting and sleep apnea. We also began talking about the possibility of needing oxygen and monitoring at night... Not there yet, but wanting to see what our ENT said first, but will need to keep an eye on this.
2. We met with our neurologist, which is always great to check in with him! He really cares about Eli as a whole. He wants to know about his day-to-day life, his advances in therapy, issues in other areas of his life not relating to neurology, etc. We haven't done an EEG in a year, so we talked about doing one soon to just do a check-in. {fun side note... Dr. Coates' office loves that Eli wears bow ties, so anytime we take a picture of Eli in a bowtie... it has to get emailed to Dr. Coates so they can print it out and hang in their office!}
3. We added palliative care doctors to our team! Who knew these amazing people existed?! They're like social workers with a medical degree that can prescribe drugs :) They added 2 new medications to our routine that drastically helped Eli right away... 1 med was for bowel movements (BMs) - saw improvement immediately. Other med is for pain management... Something we learned is that children with Neuro-Muscular diseases can have extremely sensitive nerves in their gut... So when Eli has all this gas & issues with BMs - he basically turns in to a collicky baby, which is exactly what we had been seeing. This new medication for calming his nerves worked instantly and we started to get our happy boy back.
4. We had a quick check in with OT, PT, and Speech (because I had to get to work!)... And it was fun to talk to Speech a little about starting to give Eli more oral stimulation and tastes of food for fun!

Therapies for Eli in June were really difficult... Because of his discomfort and increased fussiness, it was hard for many of our therapists to get a good, full session in with him for anything. Also with his fussiness, his schedule was all over the place and he was at times napping during his therapy times! (Hard to do vision therapy with your eyes closed 😔) In July this began to turn for the better! As we started to get our happier guy back, he was able to participate more in his therapies again. We began to try some really cool new tools in his vision, speech, and developmental therapies. Eli began playing around with communication buttons that use language, vision, and play all together. How they work is I can pre-record a word or short phrase so that when Eli pushes the button it will say what I have recorded. We then also began to use these buttons with his adaptive switch toys! So a favorite for Eli became to push his button, which said "toy on," and it would make his toy go all at once. He definitely had a lot of fun "talking to us" through these buttons and playing! Looking forward to continue to use them.

working hard in PT

Post clinic, the 2 new meds really made a difference for Eli with his BMs and fussiness. We were getting our happy guy back, but the vomiting never decreased... We were still at least having 1 vomit a day. Then July 16th hit (the week we were going to Nashville), and Eli decided to increase his vomiting. He vomited his morning meds before we even started a feed, and then vomited a few more times during his first feed, vomited again in the afternoon & evening... And this new trend stuck around until we went to the hospital unfortunately. It seemed that Eli was worst in the morning and evening for these 2 weeks where we'd often get 3-4 vomits in the morning and 3-4 vomits in the evening. Thankfully on our trip to Nashville we had my parents with us helping out with the kids and watching them when we had Gabe's wedding festivities. Despite all the vomiting, we tried to make the best of our trip! We enjoyed the Grand Ole Opry & a backstage tour, lots of food, walking around downtown, and hanging in Franklin for a day.

When we got back from Nashville, Eli had a couple good days... Seemed to be happy to back in his home and back on his normal routine, but after 2 blissful days he went back to lots of vomiting in the morning and evening. We saw our ENT on 7/27 and he didn't have a lot of concern for Eli's tonsils. He wanted to look at the sleep study in more detail and get back to me, but he didn't believe we needed to take out his tonsils. So we're back to wondering why and where all this vomiting is coming from. We tried to enjoy the weekend with friends and family for Ava's 1st birthday party. We didn't give Eli near his normal feeds to try to prevent some vomiting, and he was just kind of out of it for the weekend and not his normal happy self. By Monday morning (7/30), I was on and off the phone with our GI doctor's nurse... All this vomiting has just not been right and we have to figure out something else to do. Eli has tried lots of diet changes, lots of things to help with BMs, and is maxed out on his reflux medication. The idea from GI was to do an upper GI x-ray for Wednesday and then see our GI doctor Thursday... Also emailing with our nutritionists all day on 7/30 (who are luckily so great at responding to me and care so much about Eli!), they began to get me worried for dehydration. So... we were in touch with the pediatrician this day too. Unfortunately by evening, Eli wasn't even keeping Pedialyte down on a slow rate... So we made the dreaded call to the on-call pediatrician, because we already knew we were going to have to take him in...

Super exhausted boy from all the vomiting... 

Praises:

• new clinic at Lutheran General we get to be apart of!
• new palliative care doctors on his team - trying to make sure he can have the best life he can
• fun celebration with little sister for her 1st birthday
• supportive grandparents!
• a career with summers off so I can be here for Eli with all of this

Prayers:

• source and reason for the vomiting so we can help stop it
• comfort for sweet Eli
• answers in our hospital stay

Not For A Moment - Meredith Andrews

You were reaching through the storm
Walking on the water
Even when I could not see
In the middle of it all
When I thought You were a thousand miles away
Not for a moment did You forsake me
Not for a moment did You forsake me

After all You are constant
After all You are only good
After all You are sovereign
Not for a moment will You forsake me

You were singing in the dark
Whispering Your promise
Even when I could not hear
I was held in Your arms
Carried for a thousand miles to show
Not for a moment did You forsake me

After all You are constant
After all You are only good
After all You are sovereign
Not for a moment will You forsake me
Not for a moment will You forsake me

And every step every breath you are there
Every tear every cry every prayer
In my hurt at my worst
When my world falls down
Not for a moment will You forsake me
Even in the dark
Even when it's hard
You will never leave me
After all

After all You are constant
After all You are only good
After all You are sovereign
Not for a moment will You forsake me
Not for a moment will You forsake me
Not for a moment will You forsake me

May & June

The beginning of May started with a continually worsening Eli in the evenings. We had gotten on a really good feed routine, breathing treatments, and relatively good sleeping routine for Eli. However, for some reason in the beginning of May... Eli only wanted to be held in the evening and lots of crying until he fell asleep. We soon learned after his nutrionist came for her visit on May 10th that he was struggling with growing pains! For the first time in a long time, Eli grew! He made it to the 20lb club and he also grew 0.75” in 2 weeks (1.5” total in the past 3 months before this). 

After the growing pains subsided... we got our normal happy guy back... but not for too long. By the middle/ end of May, Eli began struggling with bowel movements (BMs) again. It seems like every time we find something that works for him, it only works for a few weeks to a month. When he struggles with BMs, he becomes gassy and in pain. And when he is gassy... his reflux or vomiting is worse. It’s a bad cycle he’s been struggling with since last summer. Toward the end of May, his reflux began to change... he used to just spit up or vomit fairly easily and it would just be all over his bib or himself. At the end of May his reflux turned in to more forceful vomiting, which is actually quite scary for us. He whines, foams at the mouth, heaves for :30 or so, and then forces his feeds out his mouth/ nose... which can be scary for breathing issues & aspiration. This new reflux sometimes was just one quick vomit, but could more usually be 2, 3, or even 5 small vomits in a row... Which elongates the scare of breathing struggles. On bad evenings, which happened once every week or two, Eli would vomit on and off for an hour. Unfortunately once this new reflux started, it was here to stay. He would vomit at least once a day from the end of May until we were hospitalized at the end of July.

At the end of May Eli completed a sleep study, which was a recommendation after his Neuro-Muscular Clinic. Eli snores and gasps at times during his sleep, and he’s also had what seem like night terrors at times. All of these things on top of his diagnosis gave reason to make sure he’s getting enough oxygen while sleeping. His results were abnormal, but not extremely alarming... he didn’t require immediate oxygen during the night, but did have some slight apnea and a low dip in the 70s. We ended up talking about these results more at his next clinic in July!

June rolled around and mom was home for the summer! The first few weeks were honestly a little rough as we were getting used to our new routine... June was unfortunately the month that Eli’s fussiness continued to increase. As we kept trying new things for his BMs... we kept not finding the perfect balance to help him. Our normal happy guy was just uncomfortable. He wanted to be held most of the day, bounced, lots of burping, and needed lots of time with his heating pad stuffed animal (Georgia the giraffe). This was tough to juggle with 2 kids! While Ava is miss independent for the most part, by the end of long days with Eli- Ava would get pretty attention-deprived. 

With the gassiness and discomfort increase, so did Eli’s vomiting. In June, he typically had 1-2 heaving vomits a day. Because of all these GI issues, we saw our GI doctor in June. Unfortunately the only recommendation at this time was to increase his Erythromycin, which he had been taking for a year to help empty his stomach. He takes it 3x day, so we drastically increased this dosage hoping it would help empty stomach, produce more BMs, and slow down vomiting. At this point, Eli was still gaining weight... so vomiting was concerning but not being taken very seriously. The first few days after the medication increase, we actually had the opposite effect...  Eli had no BMs and we got an extra cranky boy. We then let our nutrionists be our experts and help us figure out some things we could do in his diet to help with BMs... the answer was to try Benefiber and the vegetarian Real Food Blends instead of the chicken one (because it’s naturally higher in fiber). These changes worked for a little bit... and then didn’t. Next new trial came in July!

We got GREAT news in June... we qualified for a DSCC program earlier this year that will provide home nursing hours, some home improvement funding for medical needs, and coordination of care... and in June we were finally at the end of the approval process and got to have nurses start. Through amazing friends and contacts we were referred a few awesome nurses to help us get started. I can never thank one of my co-workers, Suzy, enough for sharing her amazing bestie with us! She’s quickly become one of Eli’s besties and #1 fans. It has been so amazing having nursing support... it gave Eric and I some restful nights of sleep and gave me some freedom during the day to do some things with just Ava! We are still looking to add more nurses to our team so that we can fill and use our 30 hours of nursing a week... so send any Chicagoland nurses you know our way! :)

In June, the whole Peterman family got to attend the Chicagoland Team Momentum Chicago Marathon kickoff run & potluck. Eli was introduced to the staff & team of runners as an honorary hero! We had a great morning outside and meeting some of our other MDA team members. 

Praises:

• growing! 
• amazing nutrionists who are constantly brainstorming ideas to help Eli 
• nursing support starting!!!!
• Aunt Hanna Home for the summer

Prayers:

• comfort and answers for BMs & vomiting
• finding more nurses to care for Eli in our home

Romans 8:24-30 "For in this hope we were saved. Now hope that is seen is not hope. For who hopes for what he sees? But if we hope for what we do not see, we wait for it with patience. Likewise the Spirit helps us in our weakness. For we do not know what to pray for as we ought, but the Spirit himself intercedes for us with groanings too deep for words. And he who searches hearts knows what is the mind of the Spirit, because the Spirit intercedes for the saints according to the will of God. And we know that for those who love God all things work together for good, for those who are called according to his purpose. For those whom he foreknew he also predestined to be conformed to the image of his Son, in order that he might be the firstborn among many brothers. And those whom he predestined he also called, and those whom he called he also justified, and those whom he justified he also glorified.

Pool Nap!

Mother's Day!

Zoo Playdate with our besties!

Ready for marathon training to begin :)

Father's Day!

NIH Trip

Very delayed update about the NIH! It was a wonderful trip at the end of April. We didn't really know what to expect and who all we would be meeting, so we were pleasantly surprised!

Tuesday April 17th:

Flight to DC! Flying with Ava this time around was not any easier than the first time back in December... She needs to move and see and touch everything on a plane. Elijah of course was great! Once we landed, we headed to our rental car and went straight to the NIH campus, where the testing and housing was located. We checked in at the Children's Inn where families stay. The place was AMAZING! It had play rooms (plural), outdoor playground, game rooms, kitchens, exercise room, etc. Ava frequented the play rooms often to get out some energy. Once we put our stuff in our room, we headed right to the clinical center to get checked in so we wouldn't have to do that in the morning. Once we got our wrist bands, we headed back to the Children's Inn where dinner was being served. During the week, the Children's Inn has volunteers come in and cook dinner for the families. Tonight's theme: Greece! After dinner we all turned in for an early bed time after a long day of travel.

Wednesday April 18th:
We got to the clinic bright and early, and started with vitals. After that we began to meet some of the doctors who would be researching Eli. They did a physical exam and listened to me talk for about an hour to learn all about Eli. They knew his paperwork inside and out, but still wanted to just learn as much as possible about him. They were very sweet, informative, and knowledgable! After this he had a series of testing - most of which he has already had done before. He did a muscle ultrasound, GI ultrasound, drew blood, EKG, and then a skin biopsy. Skin biopsy was a new one for him and made me a little nervous, but ultimately sounds like it will be really good for learning about him. They will grow his skin cells and try to learn more about him at the cellular level. If our doctors in Chicago (or if we ever go to Mayo Clinic) need more information about his cells, the NIH can provide cell samples and information. The lead doctor on Eli's case specializes in neuromuscular diseases and neuro-genetics... Both great things for Eli! After the first day of testing, we enjoyed a family run and some time at the park. Another dinner was provided, and it was time for bed again!

Thursday April 19th:

Not as early of a morning and not as busy of a day... Eli only had some skeletal x-rays, an (interesting) eye exam, and an echocardiogram. All of his testing was done at the NIH Clinic Center, which was a 5 minute walk from the Children's Inn. It made it easy for Eric and I to tag team and bring Ava back for naps! Eli did amazing with all of his testing... Usually had a smile on his face, and the echocardiogram even had him cracking up! The eye exam was interesting because he was very tired and started to get cranky... the kid can't see much anyways, so add those elements together with a flustered doctor = interesting. He didn't really cooperate for anything she was trying to do (but most of it was too advanced for him anyways), so we went home for a nap :) Thursday night there was another family dinner - France themed.

Friday April 20th:
We were free to fly back on this day, but we figured if we were flying all the way here... We better take the kids to do some touristy things! So we headed in to DC for the day. We walked "the mall" and saw the White House, Washington Monument, WWII Memorial, and Lincoln Memorial. We ended the day with Georgetown Cupcakes!

Our results from all the testing showed us nothing new... however, it will be helpful to continue consulting with the team of doctors when needed in the future. From conversations with them, they are not diagnosing Eli with anything new. They believe his diagnosis of Limb Girdle Type 2S is accurate, and they are continuing to learn more about his specific gene mutation. Unfortunately they believe his type of MD behaves more like a mitochondrial disorder. Which explains all of the struggles we have been having in the past year that do not relate to a Limb Girdle MD. His brain atrophy, his breathing struggles, loss of vision, his digestive issues, the length of time it takes him to recover from illness, etc. - all of these are much more characteristic of a mitochondrial disorder.

Moving forward, this team of doctors is here for us whenever needed. They can fly us back to DC, do testing at Lutheran General, or just consult with us when we have new symptoms or aren't sure what to do. It was really encouraging to hear over and over again that they are very impressed with what our team of doctors has already done for him (especially with such a rare disease). They were very happy with all the breathing treatments he does every day, the amount of therapies he is getting, and the machines he already has. They said it is very typical that patients first come to the NIH with none of these things in place.


Praises from this trip:

• amazing new team of doctors to get to know Eli and provide us information to best care for him
• learning little pieces of information that explain his puzzle a little better
• hearing that we are doing everything we basically can be doing for him already!!!
• successful trip flying there and back with our 2 crazy kids
• a little time away as a family

Prayers from this trip:

• beginning to understand more of what his diagnosis will mean and coping with what he may continue to struggle with

Jeremiah 17:14 "Heal me, O Lord, and I shall be healed. Save me, and I shall be saved, for you are my praise." 

1 Chronicles 16: 23-31 "Sing to the Lord, all the earth! Tell of his salvation from day to day. Declare his glory among the nations his marvelous works among all the peoples! For great is the Lord, and greatly to be praised and he is to be feared above all gods. For all the gods of the peoples are worthless idols, but the Lord made the heavens. Splendor and majesty are before him; strength and joy are in his place. Ascribe to the Lord, O families of the peoples, ascribe to the Lord glory and strength! Ascribe to the Lord the glory due his name; bring an offering and come before him! Worship the Lord in the splendor of holiness; tremble before him, all the earth yes, the world is established; it shall never be moved. Let the heavens be glad, and let the earth rejoice, and let them say among the nations, “The Lord reigns!”