12/29/2023 ENT

 12/29/2023

Today is our first visit with Dr Lyndsey Stull. She is the only ENT doctor that we were able to get an appointment with in a timely manner. She is fairly new, so we were not able to find out much about her beforehand.

We were pleasantly surprised! She was very professional and seemed genuinely concerned for Jacob's well-being. She performed the first trach change since his surgery back in November. This should be done every 4 to 6 weeks, so this change was a bit difficult on him. He was terrified, and quickly saw how easy the process would be. It was a good visit! We are due back to see her in six weeks.

12/24/2023 Christmas Eve

 12/24/2023

Christmas Eve and Jacob continues to improve!

12/19/2023 Cardiologist

12/19/2023

Due to the respiratory trauma Jacob suffered on November 5, Dr. Ahmed Sakkal, the ICU cardiologist, had put Jacob on a drug called Amiodarone. Amiodarone is a very dangerous heart medication. It is generally referred to as a last resort medicine because of its long-term side effects, such as pulmonary fibrosis, thyroid dysfunction, hepatitis, neurotoxicity, and even death. Definitely something we did not want Jacob taking.

(12/07/2023) At our four week follow up appointment, I had asked Dr. Sakkal to take Jacob off of this medicine. It was only supposed to be a four week medicine to begin with. He would not stop the drug, but instead reduced it to an every other day dosing. After learning all of the dangerous side effects, I made the decision to call the doctor today with an ultimatum. He agreed to take Jacob off of Amiodarone and replaced it with a beta blocker, called Metoprolol Tartrate. 

Because of the doctors refusal to remove this dangerous drug from our 24-year-old child, in spite of my ultimatum, I still decided to seek a different cardiologists. We were able to get a referral to Dr. Stanton, arguably the best cardiologist in our state.

12/05/2023 Botox

12/05/2023

Once again, Dr. Hollinger made an awesome decision regarding Jacob's health. He elected to inject Jacob with Botox. In ALS patients with saliva issues, injections of Botox into the salivary glands, can reduce slobbers and improve quality of life for approximately three months before needing repeated. Dr. Hollinger injected 100 units instead of the 150 recommended units. This was to ensure Jacob tolerated the injection.

11/22/2023 Glycopyrrolate

 11/22/2023

Dr. Hollinger started Jacob on a drug called glycopyrrolate (Robinul) for slobbers. Things are going great, the stoma is healing nicely. The drainage is still present, but it is lessening. Not near as bad as the beginning.

11/15/2023 10 Days Later

 11/15/2023

Jacob's white blood count is down to 17, so we are being discharged! Due to the tracheostomy, he was transported home via ambulance transport. Jacob spent the day in his adjustable bed. We have loads of slobbers and drainage from the trach. They tell us, it will eventually dry up.

Jacob's plans for tomorrow are to spend the day in his easy chair, watching TV!

11/14/2023 The ICU Saga Continues...

 11/14/2023

Jacob's white blood count is down to 18! Mom and dad are getting much better at tracheostomy care. We will get stitches removed tomorrow and likely get to go home!

11/12/2023 STICU - Week One Down

 11/12/2023

We have been in the surgical trauma intensive care unit at the hospital for a week now. The hospital staff is amazing! Jacob is still doing great! His vitals are perfect, and his strength and his fingers seems to be increasing. He is getting really fast on his Tobii Dynavox iPad.

11/09/2023 Trach Day 2

 11/09/2023

Day two of the tracheostomy. Jacob's vitals continue to be perfect. He feels a little anxiety and occasionally thinks he is choking. This is normal. They are still pulling (suctioning) phlegm out of his chest, so he stresses when this happens. 

The Internet says it could take up to two weeks for him to get used to the tracheostomy. One thing is for certain, the hospital staff loves him!

11/08/2023 Tracheostomy Day

 11/08/2023

Wednesday… Today at noon, they took Jacob to surgery for the tracheostomy. The procedure took approximately one hour, and everything went perfect! Jacob had a very good day! All vitals are as good as could possibly be! Pulse – 92, oxygen – 97, blood pressure – 114/79! 

He is still communicating by shaking his head no and blinking his eyes once for yes. After the 5 o'clock visitation, we drove home to get his Tobii Dynavox communication device. This will definitely help with communication.

11/07/2023 Day 3 STICU

 11/07/2023

Jacob continues to recover! Cleaned and bathed by the nurses before we arrived. Tracheostomy is scheduled for tomorrow at noon! He is shaking his head  for "no" and blinking once for "yes". He cannot speak due to the tubes from the intubation. Our baby looks great! God is so good!

Blakelei took great care of Jacob. She played Chris Stapleton music and even styled his hair for him!

11/06/2023 Intubate

 11/06/2023

Over 24 hours a week now. Jacob is returning to normal, but in last night's frantic attempt to save his life, he became necessary to intubate him. This means moving forward, we will need a tracheostomy. Mom and I were able to visit during specific times, because he is now in this trauma area of the hospital. As the day progressed, everything became stable and perfect! Even the pneumonia was reduced to a minor community virus, easily treated with light antibiotics.

11/05/2023 The Worst Day EVER!

11/05/2023

Today's started just like any other Sunday. We got up around 7 AM and headed off to church. After the services, we headed back home and decided to enjoy a day of football. In mid afternoon, Jacob began complaining of some sort of restriction in his throat, but it was way down deep. Closer to his chest cavity. He thought it was a buildup of phlegm. I attempted to suction him, but could not get it out. We called a friend of ours, Nikki Michaels, an RN, and asked for her help. Immediately, she came to our home, but Nikki was also unable to remove the obstruction. Jacob was clearly choking! We made the decision to take him to the emergency room at CAMC in Teays Valley. I could not get him stabilized, and the tests and x-rays showed pneumonia in both lungs. He was finally admitted to the ICU.

Jacob's oxygen and pulse levels would not normalize. His pulse dropped several times, as did his oxygen, into dangerous levels. They put him in a full facemask to help, but his breathing remained extremely labored.

Around 1:30 in the morning, Jacob was in real trouble! All vitals dropped, and the nurses could not hear any activity in his left lung. They tried using an Ambu bag for breathing, but that was not working. Everything bottomed out, and he lost consciousness. 

The attending nurse had started feeding Jacob with a Joey pump. Due to either a faulty device, or wrong settings, Jacob was OVERFED and aspirated, collapsing, his left lung and subsequently stopping his heart... he was gone.

I ran out of the ICU to the waiting area to tell my wife what was happening, when we heard "CODE BLUE, CODE BLUE, CODE BLUE! ICU ROOM 6 CODE BLUE!" People swarmed from all over the hospital into the ICU. As the doors flew open, I heard someone yell. "GRAB THE CRASH CART!" (A crash cart is essential for cardiopulmonary resuscitation as it contains advanced cardiac life support (ACLS), first-line drugs, a defibrillator, a bag valve mask, and a suction device.)

The staff used epinephrine, shock paddles, and chest compressions, in an attempt to save Jacob's life. Our friend and youth pastor's wife (Emily), is an RN at the hospital and was there with us. Jacob was gone for 20 minutes! No heartbeat, no pulse, no life. Mom and I were on our knees, praying and crying harder than we've ever cried! Begging God, Jesus, all that is Holy, to save our son, and not take him from us.

Finally, after the 20 minutes of being gone, they were able to get a pulse, then quickly a full heartbeat, and Jacob almost instantly recovered! All vitals returned to normal! The collapsed left lung was filled with pneumonia, carbon dioxide, and fluids! The attending physician made the decision to transfer Jacob to the surgical trauma intensive care unit at CAMC, General Division in Charleston.

Jacob was transported via ambulance transport, due to his delicate and guarded condition.

10/11/2023 Legs Hurting

 10/11/2023

Almost every lumbar puncture has resulted in this leg pain within a couple days of the treatment. This one is no exception. Jacob's legs ached for three days following this lumbar puncture. Finally subsided on Friday night (exactly as before).

10/09/2023 # ION363 (#3 in Phase 2)

10/09/2023

We drove back to Ohio State University and received our 9th Jacifusen treatment today. Everything was perfect! Jacob's breathing is improving, and so are his muscles! The ruffling in his tongue is still there, and his speech is becoming more and more difficult to understand. This is largely due to the high pressure air being forced through his airways, via the Astral noninvasive ventilator. When the ventilator is off, his speech is easily understood. Unfortunately, you cannot be left off for more than just a few seconds, due to the weakness in his diaphragm.

10/08/2023 Church Dinner and Auction

 10/08/2023

Following church today, we had the TeamJacobWV "Room that Love Built" dinner and auction! We made over $7000 on knives (donated from various forged in fire contestants) for Jacob's new room addition.

Afterwards, we headed back to Ohio State for the next ION363 dosing. Marco was able to draw Jacob's blood on the first attempt! This is amazing and an indication that he is definitely improving!

10/07/2023 Speech

 10/07/2023

Jacobs speech is getting much more difficult to understand. The atrophy has moved into his tongue. Combine this with the forced air from his ventilator, and it is simply getting more difficult to understand his speech. Dr. Kolb made notice of a ruffling in Jacob's tongue back in August. We continue to pray, but as the ALS progresses, our prayers tend to change, based on the importance at the time.

10/02/2023 More New Strenth

 10/02/2023

Today, Jerry Jacobs, massage, Pam found new muscle mass in his upper thighs!

08/15/2023 NEW Leg Movement

 08/15/2023

Back home from Ohio State. Almost immediately, we notice new movement in Jacob's legs! The movement of his legs increase for the next several days. It is not a hugely significant amount of movement, but we are grateful for any signs of returning strength.

08/14/2023 #8 ION363 (Loading Dose)

 08/14/2023

Back to Ohio State University for the "Loading Dose". The loading dose is the second dosing after beginning phase 2. It's called a loading dose, because if Jacob was on a placebo for phase 1, it will be the ion363 drug. If he was on the ion363 drug for phase 1, the loading dose will be a placebo. 

This is to ensure he is not given too much of a good thing. Also, the loading does consists of the exact amount of CFS fluid that is removed. In other words, 20 cc out, 20 cc back in.

08/12/2023 Second Annual "Wheelin for ALS" Offroad Ride

 08/12/2023

Our second annual off-road ride was twice as productive as our first! We brought in over $20,000 cash, with another two to $3000 in various prices and services. We hosted this years event at the public land, known as Sanderson. This will likely be our venue for the next several events due to its popularity with the off-road community.

07/31/2023 NIGHTMARE FUEL

 07/31/2023

Monday - Around 2 PM today, Jacob had to go to the restroom. I disconnected his respirator and moved it with him to his shower chair. After he had finished, I cleaned him up and returned him to his recliner. As I moved his respirator back to his side table, I noticed a visitor, Deb Winters, pulling into our driveway. In my haste, I neglected to plug his respirator back in, leaving it on battery power. 

Later that evening, Jacob decided he wanted to take a drive. Mom grabbed the Astral respirator, and assumed I had just unplugged it. We made it approximately 20 miles from home before the battery warning started beeping. I turned around immediately, jumped on the interstate and drove 80 to 90 mph all the way down I64 to get home. A tractor trailer exited the St Albans exit in front of me, so this forced me to take the next exit, which ended up being much better for us anyway. As we exited the interstate, the battery finally quit, and the respirator shut off completely.

Mom was calmly rubbing Jacob's chest and trying to keep him relaxed and taking very slow, shallow breaths, but approximately 6 miles from home, he lost consciousness. I continued my 80 to 90 mph speed across Route 34 and into the town of Winfield! Mom what is performing CPR. I was blowing the horn and flashing the lights and weaving in and out of traffic as I dialed 911! Jacob was dying, and nothing we did could stop it! My 911 call put the Winfield volunteer fire Department first responders at our home just moments after we arrived! Mom had already ran in the house to grab his charger as both of us continued doing CPR! When the first responders arrived, they immediately swarmed Jacob and worked for 30 to 40 minutes, trying to save his life!

Finally, Jacob began to regain consciousness. They took him by ambulance to CAMC Teays, where Jacob receive numerous tests, scans, and IVs. We return home early Tuesday morning and, praise.Jesus, all is well!

Shortly after this event, we were showered with gifts of car power inverters, home battery backups, and additional charging cables. 

This event was a combination of multiple mistakes made throughout the day. It was a valuable lesson learned, one that... unfortunately poor Jacob got the worst of, but a valuable lesson just the same.

We now have multiple battery backups, even a whole home generator, to ensure this never happens again.

07/24/2023 New Leg Movement

 07/24/2023

Legs stopped hurting Friday night. Today… We have new leg movement! Swinging his legs back-and-forth in his shower chair! Video available on Facebook below!

https://fb.watch/pyLAJk3Gmj/

07/22/2023 Legs Hurt

07/22/2023

Every lumbar puncture, except one, has resulted in leg pain. Treatment number 6 resulted in a headache, but every other treatment has been leg pain. This one is no exception. Jacob's legs ached for three days following this lumbar puncture. Finally subsided on Friday night.

07/18/2023 Follow Up

 07/18/2023

Jacob's follow-up was perfect. Dr. Kolb took a voluntary skin sample from Jacob for an additional study. Biorepository and Brain Bank Study.

The idea is to grow the skin cells from an ALS patient and use that on laboratory specimens to help find a cure!

07/16/2023 #7 ION363 Dose.

 07/16/2023

Back to Ohio State University! 

In phase 1, there was a one in  three chance that Jacob was receiving a placebo. In phase 2, the placebo possibility is completely removed, leaving only the actual medication from here on out!

Dosing is scheduled for Monday 7/17... everything went perfect! Jacob's hydration is great. We are due back tomorrow, 7/18 for follow up.

06/17/2023 Parade of Steam

 06/17/2023

One of Jacob's favorite annual events is the Cass Scenic Railroad Parade of Steam. Jacob made sure, during his peg tube surgery, to ask his nurses and doctor if it would be OK for him to go to this. They assured him it was fine and also told mom and I that it was OK. 

So today, we had a very good day at the Cassie Parade of Steam!

If you are interested in the Parade of Steam, please take a look at our very brief video from this year's event.

https://youtu.be/h8EqVzJKHFo?si=vamv5m5lt0K4E14z

06/12/2023 Feeding Tube

 06/12/2023

Jacob's continued weight loss and fear of choking led to PEG tube placement. We were concerned about the surgery and anesthesia, but once again, prayer and God's mercy prevailed! The surgery was performed at Cabell Huntington Hospital and, other than a little bit of soreness, there were no issues whatsoever. Home health care (Tyler), visited us for the first few weeks, but determined that we did not need their assistance and discharged us. Jacob took three cartons of Jevity formula on the first day, and four cartons on the second day.

05/15/2023 More Blood Tests

 05./15/2023

New blood work at Dunbar medical shows the liver enzymes (ALT) down to 203.

04/30/2023 #6 ION363 Dose... FINALLY

 04/30/2023

Off to Ohio State for Jacobs 6th dosing of Ion363 (Jacifusen), over 4 months since his previous dose. His weight is now 137 pounds or 62.3 kg. The dosing was perfect, no issue!

Dosing side effects would include a headache for the next several days.

04/26/2023 More Bloodwork

 04/26/2023

Back to Dunbar Medical for more blood work, via LabCorp. ALT levels now down to 229!

04/14/2023 Dosing Postponed... AGAIN

 04/14/2023

Dr. Kolb called to push Jacob's next dosing out to May 1st! Huis liver ALT levels are still entirely too high and the sponsor is concerned. They fear ion363 may be, at least partially, to blame for these elevated levels. 

Since two of our previous lab results from Marshall Health had been compromised, I took Jacob to Dunbar Medical to allow LABCORP to perform the bloodwork. This strategy paid off, because the ALT levels were down to 245... a 123 point drop since his blood work two weeks ago! 

Another answered prayer!

04/05/2023 Dosing Postponed

 04/05/2023

Our upcoming dosing, scheduled for April 10th,  was postponed til April 17th, due to Jacob's elevated liver enzymes. We stopped the drug, Riluzole, back in March, but his numbers are still very high. On this day, April 5th, his readings were 368 (ALT)!

03/22/2023 Hemotology

 03/22/2023

Back to Ohio State University – Wexner Medical Center, in Columbus. We saw hematologist, Dr. Archarya. Jacobs ALT liver enzyme levels were now up to 244. Normal ALT levels should be around 30 to 40. Certainly not 244 which was borderline cirrhosis.

03/10/2023 Astral Vent

03/10/2023 

Jacob's breathing had dropped dangerously low and he required non-invasive ventilation. This is a ventilator that he was able to wear at night, much like a CPAP. The astral ventilator is amazing and Jacob loves it!

03/01/2023 Liver Problems

 03/01/2023

Jacob's bloodwork has been showing indications of a fatty liver, and very high liver enzyme numbers. Today we had an appointment in Columbus for a liver ultrasound and additional bloodwork. Ionis is concerned about the liver numbers. We are convinced it's from the drug, Riluzole, and not the clinical trial.

02/25/2023 Racing for ALS

02/25/2023

Today is Jacob's birthday! We drove to Wytheville, Virginia to meet Scott Lloyd, and his father, David Lloyd SR, to take delivery of our new 2021 Chrysler, Pacifica, ADA van! 

02/17/2023 ALS Clinic

 02/17/2023

ALS clinic today. Everything is still pretty much the same as 90 days ago. Breathing is now down to 15% but the oxygen levels are very good. Dr recommended considering a feeding tube. Jacob's weight is down to 146 pounds.

02/15/2023 Riluzole - BAD

 02/15/2023

Cortni, from Marshall Health, called and asked us to stop the Riluzole. Jacob's liver enzymes have risen and is likely due to this drug.

01/16/2023 More Movement

 01/16/2023

Movement on all five left toes. Jacob no longer has to concentrate to move them. The movement is very small, but still present. Core strength continues to improve.

01/09/2023 #5 ION363 Dose

 01/09/2023

5th ION363/Jacifusen dosing! Everything went great! Jacob may soon be moved to the open label (this means no more possibility of a placebo… Guaranteed drug). Shooting for June… We will find out in April, at our next appointment. 

Bloodwork shows liver issues. Possible Epstein-Barr virus or hepatitis A. I believe these issues are being caused by the ALS medicine (Riluzole). Liver damage is a known side effect.

01/05/2023 New Movement

 01/05/2023

Today at Teays Physical Therapy, Ben discovered new movement and Jacobs ankles and feet, which would allow them to slightly rotate! Still, unsure if this is placebo or actual drug, but positive God's got this!