Brinley & Porter

Brinley and Porter on Sunday afternoon, July 29.

Update on Porter

 “Porter Rockwell” (name some of the nurses called him sometimes) has been back home for a week now and is acclimating himself to family and home to the best of his ability. While in the hospital, he was observed every few hours by a clinical/research neurologist who determined that Porter needed to start a new medication to deal with spasticity in his muscles. When this boy gets crying, he stretches stiff like a surf-board and nothing is bending him. So we are hoping this medication (Baclofen) helps with that problem.

 Before leaving PC Hospital, Porter was re-evaluated by a lot of smart people who said, “I don’t know” to Thom and Abby quite a bit! In fact, several of the neurologists are not convinced what the problem really is! He doesn’t fit any mold that any of them are aware of. He is like a potpourri of little issues that don’t equal into one diagnosis…so the mystery continues.

Anyway we are finding some things out about Porter:

• He likes his mommy to hold him.
• He has no problem crying for 2-3 hours without stopping.
• He loves warm baths.
• He enjoys the battery operated massage spider on his back.
• He really likes Brinley’s high sing-song voice greeting him in the morning and reading him stories in the afternoon.
• He loves his daddy’s strong hands, especially when they rub the top of his head.
• He is now quite proficient at pulling his tube out, so he gets to wear fingerless gloves!
• He likes being rocked in the new chair from Great- Grand-pa/ma Bandley.
• He really likes car rides and feels safe and secure in his car seat.

Porter after his bath

In the meantime- and in the next few weeks-Porter will travel to various counties, visiting numerous specialists, who hopefully will assist us in our quest to give him the best possible future.

We also continue to be overwhelmed by the generosity, kind words and actions of many, many people.

We thank- and love all of you!

Grandma Cindy

A Five Day Homecoming

Porter arrived home to Lehi, Utah on the evening of Thursday, July 12. We were all so excited and Porter seemed to share that sentiment for a few hours before settling off to sleep for the night.

Brinley, Thom and Porter on Homecoming day.

Abby and Thom got all set up with the feeding pump and other necessities to live happily ever after. Problem is, when Porter woke up the next morning, he started crying- screaming at times- and wouldn’t stop until he wore himself out and went back to sleep. This pattern continued all day Friday, Saturday, Sunday and Monday. He would cry hard for 2-3 hours, sleep, cry, sleep. He didn’t have one moment of contentment except when Thom put him in a little bath of warm water on Sunday evening. Abby and Thom were exhausted trading him back and forth and the hope of comforting him was fading by the moment.   

Monday arrived with Thom heading to work and Abby about ready to lose it- mentally & physically. Sarah was there to help, but Porter seemed to get worse- not better. After a pleading prayer to the Lord for help, a doctor from Primary Childrens' called to say that blood/spinal fluid tests had come back which indicated more problems (and hopefully answers) for Porter. They had discharged Porter thinking the tests would take up to 3 weeks to get back, but in a miraculous way, they got back in 6 days! 

Primary requested that Porter come back to the NICU as soon as possible to be treated by a world-renowned neurologist specializing in neurotransmitter disorders. So, Porter has been admitted back to the hospital and a whole new team is taking over his care. He is back to where he started and we are anxious for answers to cause, effect and treatment for our little guy.

Thank you to everyone for your faith, prayers, visits, positive energy, sharing thoughts & talks, and yummy food. These kind acts of service mean the world to all the family and we love you for it!

Update: Tyrosine Hydroxylase

July 6, 2012

Porter anxiously awaiting his swallow test.

Porter spent his first Independence Day at Primary Children’s Hospital and today is 4 weeks old. Every day there seems to be something new to think about and test for, and the emotional roller coaster continues. We were so happy with the fact that Porter was sucking and swallowing from a bottle, but those hopes were dashed when we discovered he was aspirating milk into his lungs. Consequently, the oral feedings discontinued and he was back to the NG tube only while they allow the area to rest and medication is given to stop acid reflux.  He will have another test to check his swallowing next Monday.

In the meantime, Porter has started to exhibit neurological behaviors that make caring for him difficult. Any kind of stimuli seems to start a wave of body arching and posturing (changing diapers, moving, picking him up).  For several weeks we didn’t hear a peep from him except for periodic sighing, but now he is crying a lot and nothing we do seems to comfort him.  Once he is settled, he likes to be held with his knees tucked under him. He seems so peaceful while he is sleeping.

A few days ago, the goal of bringing him home was in sight (depending on the outcome of the next swallow test) and while we were waiting for that to take place, he would start evaluations with rehab and PT specialists.  As of today, that view has changed because the numerous tests -on blood and spinal fluid done in the first few days after birth- have started to come back and there are some interesting developments.

Doctors have found that Porter is deficient in Tyrosine Hydroxylase and they don’t know why. Is it because of the brain damage? Or is the brain damage responsible for this condition? Either way, they are doing more testing- including genetic testing on Abby and Thom. Doctors want to see if they both carry recessive genes for this very rare disorder. Presently there are only 30 known cases in the world. More probably have it, but have not been officially diagnosed.The fact that the ischemia in the brain impacted the basal ganglia  is the most likely answer- but who knows? The good news is that in most cases, this is treatable, and if successful, it would make Porter’s quality of life a whole lot better. Definition of TH:

Tyrosine Hydroxylase (TH) deficiency (also known as Recessive Dopa- Responsive Dystonia) is a rare metabolic disorder characterized by the lack of the enzyme involved in converting the amino acid tyrosine to Ldopa. TH is a critical enzyme in normal dopamine production. Lack of this enzyme means neurotransmitters are blocked from signaling one another appropriately. The neurotransmitters dopamine, norepinepherine epinepherine (collectively known as catecholamines) and serotinin are deficient in the central nervous system and periphery.

Patients with TH Deficiency can develop movement disorders, autonomic symptoms (blood pressure instability, temperature irregularities), extreme irrabnormal eye movements and neurological impairment.

So we will cross our fingers and hope that the pharmacists can compound a special medication to help restore normal dopamine levels. Dopamine itself cannot cross the blood-brain barrier directly and so it is necessary to treat with a compound called L-Dopa in combination with another medication called carbidopa. There is an adult version of this combination called Sinemet (which is used to treat Parkinson’s disease), but the doses on that are way too high for Porter.

So far, Porter is dealing with two major problems: HIE and TH. We are finding that patience is the name of the game- and if you are a Tullis, patience is not something that comes easily. (The Lord is always ever ready to teach us – isn’t he?) In the meantime, they will treat Porter’s muscle discomfort with Tylenol and we will continue to pray for peace in our hearts.

Posted by Grandma Cind

The latest on Porter

                                           Look at that cute face!

Friday, June 22, 2012:

Our little Porter is two weeks old today- and oh boy! - did he celebrate! More on that in a minute… Major accomplishments in the last week:

·         Completely off all medications (Phenobarbital, antibiotic, anti-viral).

·         Off Oxygen 90% of the time.

·         Weight is now 8.5 lbs with the help of an NG tube. This is good because the goal for all newborns at their two week visit is to be back up to their birth weight- and he has surpassed it.

·         Holding his own body temperature (often times injured babies have high temps)

·         Abby gave him a bath which he loved!

·         Development specialist indicates that his muscle tone and reflexes in arms and legs are good but there are a few concerns they still need to identify.

·         While resting on Abby’s shoulder the first time, Porter lifted his head and turned toward her face so he could see his mommy (this was a sweet tender moment). Abby cried and so did I.

·         No seizures since the 4^th day after birth.

These are all good signs- and we are so thankful because without priesthood blessings, faith and prayers of many friends and acquaintances, these miracles probably would not have taken place. Several amazing spiritual experiences/signs have manifested to us that our dear departed relatives absolutely KNOW what is going on here and that Porter was sent here to be the son of Thom & Abby.

So back to the way that Porter celebrated his two week birthday? He sucked AND SWALLOWED his mommy’s milk (16 cc’s, but hey, he’s just getting started)!  This accomplishment is a huge relief and tender mercy because the doctor’s assessment and prediction of Porter’s brain damage was very grim- especially when it came to eating. We were in fear that he would have to go home with a gastric tube surgically implanted in his abdomen.

Attached are several recent pictures along with a visit from Brinley. She has had a cold and hadn’t seen him for a few days- so she was excited to visit with him again earlier in the week. (Note her two octave higher-than-normal voice when she talks to him).

Other general facts:

• Thom is back to work (new job at Utah Valley Dental Lab started May 1).

• Abby is feeling better physically and was cleared by the doctor to drive, so her freedom was just expanded. Abby’s OB doctor continues to be totally baffled as to what happened to Porter and admitted to spending several sleepless night trying to figure it out. Usually babies who suffer hypoxia in-utero never survive, so the fact that the problem (that caused the injury) corrected itself IS a miracle.
•  Hospital social workers have given Abby a lot of information and advised her on a few issues, but the task is so overwhelming- including bills- that she is going to take things a day at a time.
• Developmental teams will continue to work with Porter after he leaves the hospital, so this will hopefully bridge fears of the unknown a little bit.
• Ward members, family, friends & co-workers of Abby, Thom and grandparents have all been so supportive. They say it takes a village to raise a child- but in order to meet the needs of special children- we need a village willing to work a little OT!!!

Saturday, June 23, 9:00 p.m.

When Abby called the nurse tonight for a report on Porter, she said that he drank 50 cc’s out of the bottle!  As my Baptist friends would say: “Hallelujah & Praise the Lord!” Oh heck, I guess Mormons’ can say that too!

Father's Day

For Father's Day, the whole Tullis clan drove to Primary Children's Hospital to visit Porter and celebrate the wonderful father's in our family (Rich, Ben, Thomas, Aric). After we all saw Porter, we went outside on the patio at PCH. Grandma Cindy made a delicious lunch that we all chowed down. Brinley, Emma, and Cooper couldn't get enough of all the birds that surrounded us. Hallie couldn't get enough of her baby bone that Grandpa Rich bought her. After eating and visiting, we all went back to see cute little Porter. Porter is off all his seizure meds and has not had another seizure. He is doing well and is taking it one day at a time!

Porter opened his eyes for his daddy!

Check back soon for another update today.

Thanks for all the prayers and support!

Video of Porter

As we try to come to terms with Porter's diagnosis, the only thing that comforts us is Porter himself! What a pure blessing he is- straight from our Father in Heaven!

(This video was accidental. Rich was asking me to stop rocking so he could get a picture, not knowing that the camera was in video mode).

Our Porter

Thom and Abby received the sad news yesterday that their little guy has sustained some brain damage in a critical part of the brain that governs speech, thinking, walking and several other critical components that operate a fully functioning body. Doctors were only able to give them best and worst case scenarios and admitted that “only time will tell.”

For clarification between the two MRI’s that Porter had: the first one at 3 days old was to determine if there were any congenital abnormalities (deformed, enlarged) that could easily explain the seizures. This was normal. The second MRI was more detailed and involved contrast so they could see the overall “health” of the brain. This is where they saw two small white/gray areas that indicated O2 deprivation possibly due to cord compression or some other unknown reason.

The doctors were adamant in their explanation to Thom and Abby that this was an “accident.” There was nothing she did- or didn’t do-to have something like this happen. Most likely this damage was sustained close to the birth time. The doctors called AF hospital hoping that they could retrieve the placenta and cord for examination, but were too late- it had already been disposed of.

Right now the goal for Porter is to wean him off the Phenobarbital- which they have already started-and to assess his reflexes and swallowing. Last evening when I was there, he was much more alert and was moving his arms and legs and arching his back as if saying, “This bed is really uncomfortable- can I get up now?”

Who knows what the future holds for Porter? All I know is that he was born to parents that are righteous and capable. He is also surrounded with a large family that will do everything possible to support, sustain and help him achieve his full potential-whatever that may be. Ben Tullis said it best as we all tried to process this new and surprising development,  “Parenting a child like this- with special needs- is really a calling. And who the Lord calls…he qualifies!"

Love,
Grandma Cind

Porter’s nurse reported to Abby this morning that he did very well in the night and he hasn’t had any more seizures with the second reduction of the medication. It is encouraging to know that with each reduction he continues to be stable. He is tolerating the feedings (through tube) and is awaiting his second MRI today. His nurse reports that with the reduced medicine, Porter is awake and moving around quite a bit! It will be good to see him with his eyes open. Grandma and Grandpa Schow were with him last night. We hope and pray that Porter can continue to improve.

Another Update from Grandma Cindy

We are cautiously optimistic of Porter’s progress. No seizures for the past 48 hours so the doctors’ have started to slowly taper down his medication. Hopefully the situation will hold and they can decrease it even more. Last evening as Rich and I visited, he was quite squirmy and at one point stretched his arms out, opened his eyes (first time I had seen that) and yawned as if saying: “…ah- what’s going on?”

Grandpa and Grandma Bandley came up and were able to see Porter for the first time. Brinley visited again and brought Porter a beautiful picture that she had created and sang him a little song.

Many, many tests are still pending: blood, urine, MRI, EEG etc. The pediatric neurologist told us that we may never know why these seizures started- “but we will give you our best guess after all the tests are back.”  He also said that progress is very slow- “baby-steps” were his exact words.

Several funny moments during this whole thing:

Abby and Thom hadn’t settled on the baby’s name that first day and both decided to sleep on it. They were startled in the middle of the night by Dr. Weipert’s loud knock on the door telling them in a business like fashion that their baby was in trouble and he needed to talk to them about it. Before he proceeded with the information he asked, “What is the baby’s name?” Abby responded that they were thinking about Porter or Liam but hadn’t made the decision yet. Dr. Weipert said: “Porter is a good name- I'll call him Porter.” So from that moment on, whenever he returned to their room to give them an update, he would say:

                “Porter is still not good.”

                “Porter needs to go to ICU.”

                “You need to sign this for Porter.”

So I guess we can attribute the final decision of Porter’s name to Dr. Weipert!

If you know Rich Tullis, you know that he is a go, go, go guy. If he is home for the day and every minute is not filled with some activity, task or something that mean PROGRESSION, the WHOLE day is wasted!  So here he is, day after day patiently waiting, watching, wondering, and pondering in Primary Children’s Medical Center. He is impressed with the people and the skills and is putting his trust in the medical people to take care of his little 5-day old grandson.

One evening after visiting little Porter he came out into the waiting area and said, “Is giving medicine and doing paperwork all the nurses do back there?” I answered no- then proceeded to tell them all the stuff they were doing in a 12 hour shift. After hearing this he said, “Okay, with what you just told me, now they have at least have 8 hours to go….what else do they do? I mean, c’mon, let’s get going with figuring out what is wrong with Porter.”

I explained that it takes time and that answers don’t come very fast. At this point, he is thinking and admits that yes- he is just wants things to move quicker and have answers so that Abby & Thom can take Porter home….but he still doesn’t understand what they are doing all day. Finally, I say, “Rich, what do you want them to do?” He replied, “Well- at a minimum, read him a story or something!”

Abby and Thomas are very grateful for the continued prayers and support and feel so much love from all of you.

Feeding Tube Placed

Not a whole lot of news today to report on. Porter now has a feeding tube, and a central line was placed so he can get all his meds without being poked so much. Porter will probably get another MRI on Thursday. Abby and Thomas are still waiting to hear back from other tests that are pending.

Abby and Thomas were finally able to hold their little baby boy today, which lifted their spirits.

Keep on Praying for Porter

Preliminary MRI Results

Prayers have been heard people. Porter seems to be doing really well today. His preliminary MRI was reviewed by a neurologist who said that so far the preliminary MRI looks normal. Porter will have another MRI on Thursday or Friday to check for brain damage.  Abby and Thomas are very optimistic. Abby said that Porter looked really well today. There are still many tests that Porter will need to have. Hopefully the doctors will figure out what is causing Porter's small seizures and why he is unable to swallow.

Check back soon for more updates and keep on saying Prayers for Porter.

Update From Grandma Cindy

Thank you all for your sweet notes and words of encouragement.

Baby's name is Porter Russell Schow and he is at Primary Children's Hospital where he was taken by life flight crew Saturday evening.  He has had consistent seizures since he was born weighing 7 lbs 11 oz. Abby is pretty sure the seizures started two days before he was born. They are running many tests but so far everything is normal except the EEG, which shows abnormal brain waves due to possible oxygen deprivation in the womb. Abby gave birth by c-section so any problem would have taken place before. Maybe cord problem? We don't know. Everything was perfectly normal. His body is perfect- his Apgar score at birth was 8 out of 10. We are just really surprised and shocked. We were all at Primary Children's Sunday afternoon to offer love through words and touch. His seizures continue, even on anti-seizure meds. He is hooked up to so much stuff you can barely see his body, but we can feel his sweet spirit. Prayers are appreciated-and we thank and love all of you!      

Love,

Cindy

                                                 

Brinley introducing herself to Porter.

Porter Russell Schow

Porter Russell Schow

June 8, 2012

Porter looks like a normal, healthy baby boy. He was 7lbs 11oz. Porter's mother had a C section. During the C section the doctors noticed that Porter had had a bowl movement that appeared to have happened weeks ago. Porter seemed fine and he was taken to the nursery where he was given a bath. While Porter was in the nursery, he seemed to "twitch" a lot. The docors said this was normal. Then when Porter would "twitch", his O2 stats would suddenly drop. After waiting for what seemed like a long time, Porter was able to be held by his mother, father and big sister for a bit. That night the doctors realized that the "twitches" Porter was having were seizures. He has had multiple seizures since being born. Several tests have been done on Porter to try and figure out what was causing the seizures. After all the tests came back normal at American Fork Hospital, it was decided that Porter would need to be transfered to Primary Children's Hospital where he could be better evaluated. While at Primary Children's Hospital, Porter has had several more tests. He is constantly with a nurse. They think that somehow Porter was cut off from oxygen the last couple weeks of being in the womb. It appears there is brain damage. Porter will be having an MRI tomorrow to detect if there is brain damage and how severe it is.

Mother and Father of Porter are hanging in there and are being as strong as they can. A week before Porter was born, Mother thought she was going into labor. She went to the hospital and was told that she was in the early stages of labor and was told to go home. Two days before Porter was born, Mother said that her stomach was "twitching involuntary and felt as if the baby was having a seizure". Well, most likey Porter really was having a seizure in the womb.

We have been praying a lot for Porter and have felt all the prayers from everyone. We had a family fast today. We believe in miracles but also know that it is up to the Lord. The Lord is aware of Porter and his family. He does not give trials to those who can't overcome them whether that be Porter or his family. We pray that the doctors will be able to figure out what is wrong and that his family can get some answers.

Thank you to all of you who have been concerned. On behalf of Mother and Father of Porter, they have felt your prayers and are grateful for your love and support.