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!

Courage

by Joan

April 12, 2021 is the date of Matthew’s hemispherotomy. Followed by 8 weeks in hospital recovery and rehab. Matthew has not been informed yet of this information, we are still waiting to explain to him closer to the time as it will be hard for him to process. As it is for us his parents. This is the venue that we will use to keep you updated to his progress after surgery. As for the 7 weeks leading up to it, we also will use this method to process our feelings. We are both ready for this surgery and recovery to be behind us – looking in the rearview mirror instead of having it looming large before us. But I can say that after a week of processing, we both are feeling that this is so the right choice for him. Epilepsy is just interfering too much with Matthew’s life.

As I was praying for Dr Kennedy the other night, it struck me how much courage it takes for a surgeon to take the life of a little boy into his hands, slice half his brain away and return him back to his parents in a critical condition – reassuring them all the while that this is best for him. And he does this over and over. I am sorry, but I have a rough time just removing a splinter out of someone’s finger- not my own. There are people put on this earth, that are so dedicated to science and cutting edge of helping others that they are willing to risk their own reputation, skill, and fortitude. I am one who can’t be thankful enough. It takes courage for Ron and me to say, “yes” on behalf of our son, but then we sit and wait as Dr Kennedy does the actual work. Pray for this man!

This waiting period for us also happens to coincide with Lent – the season before Easter when the Christian world is thinking of “giving up” something in sacrifice for what our Lord did for each one of us on the cross. I am thinking that I need to give up worry for this Lent season – doesn’t really benefit anyone and certainly isn’t going to help Dr. Kennedy do his job or give my son courage to face his upcoming life. As scripture also says, “to obey is better than sacrifice” and that is probably what I will focus on more this Lenten season. Trusting Jesus, that He got this. He loves Matthew more than me. Obediently follow Him in my daily profession of caring for others. Watch my attitudes. Is my thinking lining up with Christ’s or am I going my own way? Am I allowing Him to do the finishing work of redemption in my life and am I clinging to Him for my every sustenance? Will I allow others to help and do the work I can’t do as I wait by the sickbed of my son?

Just as I marvel at the ability of this surgeon who wants to help our son, even more I stand in awe of the One who gave up his reputation, setting aside all that he deserved, and courageously endured the cross because we have all gone our own way and need a Rescuer. This is a good Lenten season, the best time to receive his salvation. And thank Him while we wait to be home with him.

Love you all, thanks for continued prayers! 

The Cutting Edge

by Joan

Matthew, our son, is eight years old. Since the age of four months, he has lived with the left hemisphere of his brain severely damaged. During the past four months he has been subjected to many tests of his brain to determine the source of the frequent seizures he lives with and that aren’t controlled by seizure medications (though several have been tried). A team of 40 (neurologists, neurosurgeons, etc.) from the Children’s Hospital of Philadelphia (CHOP) have been carefully studying his situation and are in 100% agreement that Matthew would be greatly benefited by a major brain surgery called a hemispherotomy. CHOP is on the “cutting edge” of this procedure and in fact has one of the best surgeons who does only this specific surgery. Ron and I and Matthew met with him yesterday morning for a long discussion regarding Matthew specifically. The first thing the doctor did was put the MRI of Matthew’s brain up on a large screen and before he said anything, Matthew piped up, “Well, that is my brain!” Dr. Kennedy responded, “And it is a smart one too, I see!”

Without going into a lot of detail (if you want more, call me sometime), Ron and I have made the decision to move forward with the surgery (it will happen sometime next month). After considering all that we’ve been learning, we believe, hope, and pray that the benefits of subjecting him to this incredibly involved surgery will far outweigh the risks and long-term effects if we do not take this step. It will require “cutting edge” precision to disconnect the dead (or mostly non-productive) side of brain that is firing seizure activity constantly and is negatively affecting the healthy, intact part of the brain. It is an all-day surgery; he will have some deficits he does not have now (temporarily); it is likely he will need as a follow up surgery within a week to address a side-effect and he will be in critical condition for a few days. Recovery and therapy will be in-hospital (at CHOP / Philly) for another 4 to 6 weeks. Those are the downsides / challenges, but the upsides are that following this grueling initial journey, he will be seizure free, medication free, and with intellect, cognition, and memory all very much enhanced long term.

Now is the time to do it because of the “plasticity” of a younger brain. Also, the sooner it is done, the sooner the damaged half of his brain will stop causing interference to his development and hopefully end any threat on his life from epilepsy. It goes without saying, we want your prayer support and as we navigate our way through this. We may tap a few on the shoulders for other support as well. It scares the joy out of my heart as I think about another thing that my son needs to go through. It seems to be my lot in life to cry out, mourn, grieve, sob over the medical conditions of my children, but it also is my privilege to place them in the hands of the Great Physician, walk along beside them, provide comfort, hugs, cheers, and a ton of “atta boys” to help them overcome.  Isaiah 42:16 states my hope in what God is doing. This will become my theme over the next two months. “I will lead the blind… in paths that they have not known, I will guide them. I will turn the darkness before them into light, and the rough places into level ground. These things I do, and I will not forsake them.”  That is my God speaking to our family as we traverse a dark, unknown, rough ground. He will not forsake us as we walk this cutting edge!

Don’t be shocked if I cry a lot. I am sort-of like Mary the mother of Jesus in that I ponder much in my heart and (for me) it comes out my eyes. I am ok – just need to process all my feelings. 

“Thank you for borrowing me!”

by Ron

So said Matthew to Joan after a full day at the Children’s Hospital of Philadelphia (CHOP).

First, Matthew loves that place because his five-day hospital stay back in November was that good. He especially loved the room service – how he could pick up the phone and order food, having Music Man Mike come by to sing and play for him, etc. It was amazing.

As to “borrowing Matthew,” this is a phrase he picked up from his mom. Now, people generally use the term because they have a use for someone. For example, “He is tall, can I ‘borrow him’ for a second to reach something on the top shelf?” In the case of Matthew, “borrowing him” is Joan’s code for: “I will take him for a while and give you an obviously needed break.”

And so, Matthew got “borrowed” (in his mind) to go to CHOP for neuro-psychological testing. And yes, it is all connected to the same process begun back in November. We are on a “path to end epilepsy” (if possible) for Matthew. This was the next step.

Good news: doctors are hopeful they can get Matthew to that goal. Not so happy news: Major brain surgery seems to be a necessary part of the pavement to reach the desired destination. We’ve not yet made a decision. We have lots of questions. There are people we need to talk to. Please pray.

Matthew is “borrowed” in another sense. Whether by procreation, foster care, or adoption, children do not belong to us. They are borrowed. We are stewards and we have a great responsibility to care for them during this “day” in which they are on loan. And so, this decision weighs heavily on us. Because he doesn’t really belong to us, and because we love him so much, we don’t want to say yes to something that could do more harm than good.

Something totally unexpected happened that same day at CHOP. Joan is 95% sure that she saw one of the other children who we once “borrowed.” Naeem (not his real name) was in our home for 40 hours. We will never forget that emergency respite experience. He was to be with us for a full weekend. His needs were beyond us – he needed an institutional placement with intense round-the-clock care. Naeem (if it was indeed him) clearly still has that need. How often we wonder about the others who’ve been part of our family (whether for hours, weeks or months). We pray for God to protect them.

Back to Matthew. CHOP was used by God to save his life (it will be eight years ago this month). Will he use this amazing place again – this time to dramatically improve the quality of his life? Pray for God’s leading and stay tuned.

The Christmas Star

by Ron

Tonight, I saw the conjunction of Jupiter and Saturn through my 6” Newtonian reflector telescope. This was quite a thrill for me. It has been over 20 years since I’ve looked through that telescope. On Saturday I took it from storage and began to clean it up for the event. Having had astronomy as a hobby in the past, but with too many other things pushing it aside, it took a once in an 800 year event – a Christmas Star – to have me look again to the sky for longer than what had become my typical quick glance.

On Sunday evening I pointed my “stethoscope” (as Matthew has been calling it) to a bright star above me, in a patch of sky without clouds. But I couldn’t seem to bring it into focus. Perhaps my instrument has seen too many years. I put it together when I was a young teen. And though I had rebuilt the shell decades later, the primary parts may not have it in them anymore. Was there something I was overlooking? Perhaps it was the eyepiece. I took it apart and reassembled it. I must have done something to improve it, because the stars were a bit closer to the pinpoint look that they should have, but not enough. My hope of renewing my stargazing hobby was fading fast.

And then I had a thought. Is it possible that temperature was giving the optics some trouble? My reasoning: The mirror is mostly glass. As a slow-moving liquid with the ability to expand and contract, it isn’t adapting quickly to being at 70 degrees and suddenly hit with below freezing temperature. Could that be it? I searched and found “Troubleshooting a Blurry Telescope: Top 6 Fixes.” Last on the list: “Trying to focus before temperature equilibrium.” Perhaps that is it.

How long would it take before I would know if this is the solution. “The actual time is directly related to the mass of the lens or mirror, and the difference in ambient temperature. The larger telescopes, like 6” or 8” and above, can take an hour or two to equalize.”

The “trauma” of being hit with such a sudden change results in a lack of clarity. That thought got my wheels turning. 2020. How many suggested have suggested that the name of this year would be the perfect time for greater vision or a year to bring things into focus. The year has been anything but that.

Unlike a circular piece of pyrex glass, human beings don’t find equilibrium in a few hours after being traumatized. For some of us it may be long after 2020 that we begin to see clearly again.

In our book “Joy in a Foreign Land,” we express how difficult it was to adapt to the unfamiliar and undesired land of disability in which we found ourselves. This year has been like that for us – for too many reasons for me to mention here.

But seeing a Christmas “Star” clearly was exciting to me, not only because of the joy that the old scope of nearly 50 years ago still works (and yes, I could still see the rings of Saturn and the four prominent moons of Jupiter), but also because of the reminder that things will become clearer. “We will understand it better by and by.” There is purpose. There is hope. And what better time for the reminder than the week of Christmas?

“Hang in there, Joan!”

Ah yes, my favorite line from the movie Frozen.  If your name is not Joan you may have missed it, but not me. Caught it the first time and I’ve repeated that line to myself a thousand times since! 🙂

Heard a psychologist say recently that a person who doesn’t share their prayer needs, out of fear that others are tired of hearing from them, is caught in a shame trap from being traumatized from the past. Ok, might be me, so in a step of faith I’ll proceed (knowing I need help in “hanging in there”).

I also need to update all of you who have been doing an incredible job at praying for our family – so appreciated, those prayers are being felt for sure. We are wrapped in medical needs at the moment. So many appointments, all the while trying our hardest to dodge the Covid ball with its ugly spikes!

This morning, very early, I went to get my own blood work completed. Yup that is right. I do try to keep a little up to date on my own health care. The lady checking me in asked what my occupation was. I said, “Oh just a caregiver”. She stopped her keyboarding, looked me directly in my eyes (between hair and mask, nothing else on my face on which to focus) and said, “That means you are everything! I am putting you down as an Essential Health Worker.” Woo-hoo, I finally reached that status!

Anyway, Matthew update!  We survived 5 days at CHOP! He did his first phase for the potential brain surgery to control seizures. It was quite the time but all in all he did well. Enough info for the doctors to say they want to move on to next stage. But Ron and I are asking if we could put it on pause for a moment. We feel we can control Matthew’s seizure’s enough with the medication he is on. If his little spasms cause him to space out a bit, we can still get his homeschool in – just takes a little longer. Because of upcoming holidays and Covid on the rise, we really don’t want to be in the hospital right now. And there is another issue: He is being started on a drug for attention deficit and we need to do just one new thing at a time. So, new med starting today. Pray that he tolerates it and that it would help.

Kari is top priority right now.  Between her seizures and infections, we need to get to the bottom of things. We’ve dragged the poor girl to 3 appointments in the last 2 days (she hates wearing a mask, but now seems resigned to it).  At her first visit, found out she needs surgery soon to change the battery on the VNS device she has implanted to minimize her seizures. The last few months the VNS has had to work overtime, which drained it more quickly. My prayer is that it only needs a new battery and that the wires around the vagus nerve are still good (keeping the surgery minor). Regarding her kidney/urinary tract, she has three tests scheduled for one day and at one place – so sweet that she doesn’t have to be dragged all over the place. In the meantime, she is on long-term antibiotics to control infection. She is quite the fighter.

My parents too have much to struggle with daily. With the pediatric wing taking so much time and attention, we feel like we are not giving them what we used to. Fortunately, they think otherwise. We keep praying for them as well, as I juggle mom’s up and down condition and watch dad taking backward steps in his health – but he is 88 so I guess that should be expected. Thanks for your prayers!