Trending Upward

I (Ron) am writing the update this time because things are getting busier for Joan and she is a little tied up. One of her constant jobs is to make sure Matthew is comfortable and isn’t grabbing at the drain coming from his head. When he is out in his chair he isn’t restrained, and Joan has to do that job.

One of the challenges is that Matthew has to recline at a 45 degree angle when in bed (because of the drain). It is hard for him to sleep that way. Just the same, Joan says, “We both had two, 2 hour stretches of undisturbed sleep. So I am feeling really refreshed this morning. Though he was ‘rammy’ at times, with lots of backrubs and Disney lullabies I got him back to sleep.” The drain will have to stay in at least another 24 hours.

She also says that everything is trending upward. He hasn’t had any fever. There is no sign yet that his shunt (from eight years ago) needs to be revised. His stamina is improving. His catnaps are shorter. His cooperation is “out of this world.” He is very positive! Dr. Kennedy (his surgeon) said this morning, “Matthew is my biggest encourager!”

As you can imagine, Matthew doing well has a big impact on how well all of us are doing. Here on the home front, Kari and Mom are doing so well! I really believe that Mom is energized by all the good reports. She is looking forward to seeing him with no seizures and to have a brighter future with this behind him.

Last night our daughter-in-love, Sarah, sent us this picture (taken at their wedding). She said, “Just thought you might like this picture.” Believe me that it was a great picture for me to go to sleep to. I know I’m biased, but don’t you think he is a most handsome guy?

Because some of you are asking, Matthew’s ICU room number at CHOP is 7 East Room 39 Bed 2, 3401 Civic Center Blvd, Philadelphia, PA 19104

Some of you are wondering about providing food vouchers. At this point I feel like Moses when he had to tell the Israelites to stop bringing offerings for the construction of the tabernacle (not because I don’t like offerings, but because we are at risk of having more than we can possibly use).

Thank you so much for checking in to see how Matthew is doing. CHOP is very strict with visits because of Covid and I know it is tough on Jeremy and Sarah not to be able to see him. But we are so encouraged by the number of on-line visits from each of you. Yesterday we had 141 visits! I can’t tell you how much that means to us!

I’ve gotta go, a wonderful group of women from Willow Street Mennonite Church are outside my house mulching the flower beds, cleaning windows, etc. WOW.

Just to let you know, if you want to receive updates by e-mail instead of checking in, if you haven’t done so already, you can subscribe.

Let me leave you with a Jackie Robinson quote. And I share this with you because it is encouraging to see how many people care about Matthew. It seems to us he is making a big impact and all of you are making an impact on us through your love.

“A life not important except in the impact it has on other lives.”

Up and Out of Bed

This is Matthew’s first time out of bed! Doesn’t he look great? He did fade fast, though. In five minutes of being up he fell asleep.

Joan’s comment: “This is where I have to believe the staff here, the the push must begin despite how mamma’s heart feels.”

Although a picture speaks a thousand words, and therefore this post is long enough, I have to say this: Curious George is a travelling buddy given to him by his teacher. And I can tell you that George is stuffed and overflowing with love from the teachers, therapists and administration of Smoketown Elementary. Earlier Joan wrote that Matthew only smiles on demand. Since she wrote that I was on a duo with him. I told him that Team Matthew is the best team in the whole world – he smiled! He’s getting the idea of how much he is loved by you all.

Medical Day 3

Matthew and I are still hanging out in the ICU. He continues to be on an external intracranial drain which comes out of the top of his head. It also measures the pressure within the cranium. This very much limits his positioning and movement in bed and is the reason he is in ICU.

I can still report that all is progressing, and we have had no setbacks in last 18 hrs. He has every- hour neuro checks which are impossible to sleep through, but the bigger deal is that he is passing each one and his paralysis on right side (which was totally expected) is improving each hour as well. Physical and Occupational therapy begin within the hour.

Emotionally: Matthew is a trooper and my superhero! He is extremely compliant and oh so sweet. Every nurse loves him. His voice is still a whisper and he still is wiped out by activities such as eating and neuro checks. He gets to face time his dad for about 3 min each day and today Ron gave him a tour of our kitchen and his toy shelf. Now Matthew wants to go home. I am not so much ready for that – seems like we still need a few things accomplished😊.

He will smile on command, but nothing makes him smile naturally yet. When he is awake his eyes follow me everywhere and he still wants me holding his hand. I wonder how long that will last. Once that independent nature returns, I am sure it won’t be cool to have mom holding his hand😥

Spiritually: God is sustaining us both in this spiritual desert. Our “window view” only allows us to be spectators of CHOP’s air-conditioning units, which are massive I may add, but not so inspirational! And we can see a very small sliver of sky – cloudy today. Feels a bit prison-like, but with God on His throne, you all hounding heaven with your prayers on our behalf, and Jesus’ presence in our room we are hanging in there and very hopeful that “lockdown status” won’t last forever! God is good!

* * * * * *

Jeremy sent this image to Joan, thinking that she might resonate with it. She did! She said, “I know enough about chess to realize the knight isn’t going anywhere – very appropriate.”

On the home front, just want to say that the ladies (Mom and Kari) are making life very easy on me. No “events” from mom and no seizures from Kari – just the way I love it. I am certain that many of you are upholding this side of the equation through your prayers and both Joan and I appreciate it.

End of Day Two

After a very rough start of the day, we are coming into an evening that is a bit more peaceful! He finally was able to eat something tonight and it exhausted him – he fell asleep while still chewing last bite.

I showed him a picture of himself and he thinks the surgeon did a really nice haircut on him😊. I will pass that on to Dr Kennedy in the morning. I will post a picture in another couple of days. The incisions are still yucky but no drainage this afternoon, a healthy sign. No seizures🙌

The biggest struggle is keeping his head elevated and his left hand from pulling out his drain. So, I keep on a vigilant watch – I think I am exactly where I need to be. He also likes me to hold his hand – likes me to stay close during our extended sleep over. Pray that I can catch a few wisps of sleep tonight.

Because there are no visitors allowed, the other mom in the room and I have become each other’s cheerleaders. I have a wonderful group that has ordered food vouchers for me so that I can have a tray delivered to my room. The first food tray I got tonight was immediately followed by a second one. The kitchen made a mistake and just told me to eat that too for free. So, since the other mom in the room had no food today- she got blessed by the “mistake” tray. Isn’t that cool of God to look out for us all?

Slow steady progress marks the end of day 2

News from the PICU

Day 2 at CHOP: Matthew woke up at 10 PM last night. He knew who I (Joan) was. He tried to talk, but his breathing tube was still in, so he finally got it pulled. Breathing great! By midnight he was talking sentences and moving his left side. By 2 AM I started to see movement on right side – way before doctor said I would! And by 2:30 he was trying to pull all his tubes out and was ready to go home (that is when restraints were brought out). The rest if the night he was talking about food and how great it was that we were having a sleepover. I started calling it an “over” because I was missing the sleep part. But so rejoicing for the incredible progress we are already seeing.

Yesterday in the post-op surgeon’s report he told us, “for every problem we may encounter, there is a solution!” Well at 5:30 this morning I encounter a big problem. Matthew’s head was lying in a big pool of blood. I called the nurse. The drain coming out of his head had snapped (it also measures his intracranial pressures). Although the nurses had not encountered this before, it was handled promptly and professionally. The surgeon on call instantly made a sterile field in our room and an hour later everything was secured! Matthew’s only concern through the whole thing: when can he have an egg sandwich? The whole lot of us were ready for egg sandwiches by the time we got that problem solved! Still no eating allowed yet. Shortly he will be sedated again for a 3-hour MRI.

Brothers and sisters, thank you so much for your prayers. Matthew and I are not alone!

News from home

Everyone doing well for me (Ron) here. Jeremy and Sarah did a great job covering this front yesterday so I could be with Joan and Matthew. No, unlike what Kari supposedly tried to say, she wasn’t in charge here. But as you can see, she is doing so well! For her part, she is making this whole thing extremely easy on us. Mom is also being very cooperative. Her vitals are very stable! No doubt many of you also had this wing covered through your prayers.

Note From the Editor

Hi everyone, Kari here, I thought I’d give you an update from the home front. I’ve been the one updating the posts for dad, always trying to do what I can to support this crazy family of mine!

I’m pretty proud of my littlest brother, I think he’s a cool little dude and can’t wait for him to be seizure free! As you can see I’m wearing my purple bandana today to support his fight to end epilepsy! I’m also trying to stay away from seizures and I’ve been alert and seizure free all day just praying for my bro! So thank you all for praying for Matthew, my mommy & daddy, and me as well!

Surgery Wrap Up

At 5:00pm we saw Matthew’s surgeon. Everything went well, there were no surprises. Matthew was stable the whole time and didn’t need any blood transfusion. He will probably have some right side muscle weakness from which he’ll recover during rehab. But first he has to recover from this surgery. It is possible he will need a revision of his shunt within the next week.

It will be around 6:00pm when he arrives in the ICU where we’ll get to see him.

We appreciate this surgeon so much. What a great guy. We also appreciate all of you and your prayers, your love, and encouragement. We give praise to God!

Will likely provide an update sometime tomorrow.

Surgery is Underway

We arrived at the hospital at 5:30 this morning. At 7:30 when we gave him our “see-you-laters” he was excited as anything to have his bed wheeled to the OR. He’s been excited ever since we told him about this hospital stay – so much so that he had trouble going to sleep last night. His only complaint is that they haven’t given him anything to eat yet.

CHOP is doing everything to assure us that they are going to take good care of him and have solutions for any possible problem that could come up. It will be late this afternoon before he is taken to the Pediatric Intensive Care Unit. While that is happening we will hear directly from Dr. Kennedy, his surgeon.

The nurse just reported to us that everything is going well, slow and steady. She also told us that Matthew was very entertaining to everyone as he entered the OR! We aren’t surprised.

We are very overwhelmed and very grateful for all the support we’re receiving through your love, encouragement, and your prayers!

Countdown

Our journey with Matthew and epilepsy…

Today (Wednesday): We are five days away from Matthew’s surgery at CHOP. For some details, see:  https://www.chop.edu/treatments/functional-hemispherotomy

Thursday: We continue the process of preparing Matthew. This morning we will let him know that he is having surgery on Monday, using a booklet and video (both prepared by CHOP).

Friday: I (Ron) take him to a CHOP campus for Covid testing. We also begin a bathing routine.

We continue a recent quarantine of Matthew to limit exposure to Covid.

Joan continues her training of me concerning the other members of our family who require care.  And I will train Joan on a few things – such as the use of iPad for communication and some educational things for Matthew when he is ready for that.

While this is a scary thing for us, we are convinced the timing is right. In more ways than we can count, we see ways that we have been prepared for this time.

Counting up

Our journey with Kari and epilepsy…

At this same time in which we are counting down toward the surgery for Matthew, we are counting the days in which Kari is seizure free! We are at day 10, which is quite remarkable given the countless number of seizures (often too many to count in a single day) she has experienced. This is day 13 on the ketogenic diet.

We are also counting calories. Ketogenic diets are popular these days. She is on it for seizure control. Kari’s diet is what is known as the Class Ketogenic Diet. It is 80% fat. Our challenge is to give her enough fat that she maintains and even gains some weight. She isn’t like most of us (at least I will speak for myself). She doesn’t need it to lose weight!

It was 15 days ago that we received a specific underlying diagnosis for Kari, thanks to the researchers at the Clinic for Special Children in Strasburg, PA. I’ve often said that if there is anyone in the entire world that can diagnose Kari, it is this group which is located just seven miles from our home!

Unfortunately, there is no cure or specific treatment for a RARS2 mutation. That said, within days of our learning of this, we were encouraged by a specialist in NYC to try the keto diet again for seizure control.

Kari’s disease was only discovered 5 years ago. Its potential was known since 2007, but it wasn’t linked to someone with it until 2016.

RARS2 is extremely rare. For those who wonder: 1) It is not a disease that shows up in our Anabaptist ethnicity. As far as is known, no one else in our ethnicity is even a carrier for this disease. 2) It appears that Kari (and her brother Ryan, died in 1994) are the only two known cases in the USA with a total of 23 in the world! 3) Yes, it is a genetic recessive condition. Joan and I are both carriers. 4) Our son Jeremy is not a carrier.

Counting on you

Counting on your prayers… And we know we won’t be disappointed. The Matthew Team is outstanding. What a group he and we have behind us.

And yes, you are at the right place to remain updated on our journey. Check back often or subscribe to receive updates by e-mail.

Hope, Healing, Holding Us Up

Thank you to those of you who read these blogs. We do not take your time in reading them for granted and try to keep them short. I (Joan) am going to work my title backwards.

HOLDING US UP: We are dealing with some immense issues, but this one thing I know: we have an incredible God. He and His body are holding us together. Lenten season has us focused on Christ’s suffering and knowing we walk a path of suffering now reminds us too of the Hope of heaven and glorified bodies that our coming because Christ conquered death. You, the body of Christ, are hugging us tightly through your prayers, care, food, and support. Bless You!

HEALING:  Seventeen days until Matthew’s surgery. We have begun the conversation with him, and he wants his seizures to go away. He knows that there are people on “Matthew’s team” and that they are praying for him. We have not yet disclosed how the doctor will get rid of the seizures, nor the long hospital stay. With the help of a CHOP video and book we will introduce him to an outline of those a week before. Ron and I are trusting God that this path is one of healing for Matthew. We must get past the “yuck” part before the path becomes a pleasant one.

My mom continuous to feel the affect of a body wasting away, and she is fighting to keep her inner self renewed. Of course, her ultimate healing will come and we both try to keep our eyes focused on that as we minister to her each day and night, trying our best to keep her at her best. The timing of her slow decrease in health, I leave in God’s hands.

HOPE: Much to juggle getting everything ready for Matthew’s surgery: training Ron and hospice personnel in mom’s care, thinking through all it means for mommy to be away from Kari, thinking through medical appointments, etc. But during all this a gift has been given us. After 34 years, we finally got the underlying diagnosis – the cause of our first two children’s severe disabilities. We now call Kari, ‘rare bird’ instead of ‘Kare-bear.” I will not go into details. Suffice it to say our children are the only ones our geneticist knows of here in America that carry the mutation on a particular gene in the nuclear DNA of the mitochondria.  All other 20 children live elsewhere in the world. Kari is the oldest. This is frontier medicine at the cutting edge with an amazing search that has brought us to this point. Researchers and geneticists that we have been in contact with regarding this are amazed that Kari still lives! Most have died in toddlerhood.  Any treatment of her condition if discovered would probably already be too late to help Kari, but if in five years we could see babies being treated with a gene therapy and if they could be prevented the kind of life our children have endured, we would be so blessed. One recommendation is to have Kari go back on the ketogenic diet and we do have some hope of seizures abating through this. A few days ago we were blown away to have a world-renowned mitochondria expert call us on the phone, expressing encouragement and praise for what we have done for our daughter. And he gave us some helpful insights, recommendations and leads. That very bright spot in our week is only outshined by our Heavenly Father reminding us that full healing is coming! In him is all the hope and to him belongs all the glory!