Our short 2 month catch up...

First off, let me apologize for being so delayed in writing you an update. In truth, since my last post in October, we have gone through a very monotonous trend of altering Claire’s drugs, followed by some improvement, a steady decline, then an adjustment of drugs again after two weeks. That effectively sums the last two months.

Beyond that, we have tried to incorporate development therapy into the times Claire is conscience enough to interact with us. These times are infrequent and cyclical. On average we get 5 hours of awake time for Claire every other day. If this doesn’t sound like much, its not. But we cherish every moment we get. Claire will be spoon fed during those times and exercise. Most recently we’ve worked her on rolling over and strengthening her neck so she gains head control. We have actually seen some strides in these areas. Claire is slightly more mobile on her own now—we now have to watch her so she doesn’t scoot her way off the Ottoman.

She is growing fast—slightly ahead of clothing sizes. We also just upgraded her to a toddler car seat. We find that our “don’t fit” box is very full and her dresser is getting light quickly.

We have a consultation with Metobolics at OHSU on December 28. We are working on Claire’s Mitococktail—a milkshake of suppliments to aid her Mitochondrial deficiencies. We have steadily added suppliments over the past couple months. The Neurology Doctor will also be present at the consult. To be honest, I don’t know what to expect from the visit, but I do know that the Doctors at OHSU have discussed Claire frequently.

Update, Oct. 26

I know there hasn't been a whole lot of updating going on and I do apologize. In our day to day goings on I forget to stop and report what is happening because it all seems so status quo. Since coming home from Doernbecher this last trip I would say that Claire has had some slight improvement. Claire is able to wake up most every day for at least a few hours. We continue to work on feeding her new foods and playing with her as much as possible. Claire's seizure activity has changed yet again and it is difficult to say what has caused the change. Claire is having fewer seizures per day, but on the flip side when she does seize it seems to last between 1 and 2 hours. Thankfully these long seizures are not actually harmful to Claire. Our little girl is strong and can keep her airways clear and breaths well through these episodes.

In our previous post we stated Claire would be started on a "myto cocktail" but when all was said and done the metabolic specialist wanted to start Claire on one supplement at a time so we can more easily pin point what is actually working. Claire is currently taking Creatine twice a day to help with her severe lack of energy. Though we don't know if it has helped her seizures at all, Claire is much more alert and "with it" when she is awake and the creatine is helping her motor control a bit.

On Nov. 1, we will have a therapist out to the house to show us the exercises they would like us to do with Claire to help strengthen her muscles and help her gain head control. Though the therapist will only visit us 2 times a month...you can rest assured Jake and I will work with Claire every waking opportunity we get. Claire is currently trying to move her head more than ever, but lacks the strength to control the movements.

We are again consulting with the neurologist in Portland to see if there is any tweaking of Claire's meds that can be done to help reduce the length of her current seizures. We will as always try to post updates.

On a side note...baby number 2 is cooking along. :) I am now 15 weeks into this pregnancy and so far so good. I am healthy and we have seen the baby's heartbeat @ 11 weeks and heard the heartbeat last Friday. We are scheduled for the "big" ultrasound on Nov. 24...the day before Thanksgiving. Believe me when I say though times are more challenging than we would like...we have much to be thankful for this year.

Oh...one other quick praise report...we are receiving explanation of benefits from the insurance and the coverage they provided Claire while we were in Denver was outstanding. We thought for sure we would owe bunches of money....but so far we only owe $29.75...Praise God!!!

Claire tries Sweet Potatoes

A few days ago Claire got to try sweet potatoes. Much to my surprise, Claire never developed a liking for the potatoes and seemed to much prefer the squash. Our next adventure will be carrots...and of course there will be video of that too.

Test Results, October 13

Knock on wood, this actually looks like a short stay in Doernbecher's. Claire has had relatively few seizures over the past 24 hours. It is difficult to say whether this is due to a Phenobarbital loading dose or due her Phenytoin level dropping rapidly. At any rate, she has been able to eat by mouth and has had some rare awake time.

This morning, Dr. Roberts (the primary Epileptologist) came in and informed us of test results from the muscle and liver biopsies. Claire has what is called a Mitochondria Complex III Disorder. This will likely end up leading to a diagnosis in the Mitochondrial Myopathies family of diseases. You may find some helpful information on www.mitoaction.org. Rebecca and I see this as a positive development. Mitochondrial Disorders are treatable with what's called a Mito-Cocktail--a highly concentrated mix of vitamins, proteins, and antioxidants. The Metabolic team is currently working on formulating Claire's Mito-Cocktail. There is no certainty that this will impact Claire's Epilepsy. We have to operate under the assumption that we will still have to manage her with anti-convulsants.

We have weaned Claire off of Phenobarbital and have now dropped Phenytoin as well. This will allow us to use these drugs as back-up rescue meds when Claire's seizures get out of control as they did on Monday. We are adding a new drug called Vimpat. This is a newer drug that has fewer side-effects than some of the drugs that Claire has already been on. It is not approved for children, but that is not unusual in drugs used to treat rare diseases.

So long as the Metabolic Department is timely with their Mito-Cocktail and there are no complications from adjusting Claire's meds, we expect to be released on Friday. Please continue your prayers for Claire--that she responds well to the new treatments and begins the process of healing and developing as she should.

Jake

Video of Claire's first cereal

Claire is loving her cereal. When she is not awake enough to eat by spoon, we add rice to her formula and she eats it right down. Getting permission to feed Claire baby food was a big win for me (Rebecca). It is nice to see Claire learn an age appropriate skill and allows for a little normalcy in our lives. I have started thickening the cereal and Claire is tolerating the thicker cereal just as well. Up next in another week will be oatmeal. Once we've tried oatmeal for a couple of weeks we'll get to add veggies. Thank you for your continued prayers and for following all of Claire's milestones with us!!

Encouraging news...Sept. 23

Last Thursday we had a clinical visit with Dr. Roberts at Doernbecher’s. This was a short visit but we had some positive news. While in Denver we had a MRI, but the only information we really got back about it was that Claire’s Neuro connections were behind and that she was underdeveloped. Dr. Roberts went back over the report. Yes, she is behind, but not as far behind as the Doctor at Denver Children’s suggested. This was very promising to Claire’s future prognosis.
We are continuing to reduce Claire’s medications. If you’re keeping score, she’s currently on Phenobarbitol, Phenytoin, Topamax, Keppra, Parydoxine (Vitimin B6), and Ativian, her rescue drug. We are tapering Phenobarbitol and her last dose is October 3. This is very good. Phenobarbitol causes drowsiness and causes the liver to work in overdrive. Very likely dropping this medication will help her other meds be more effective. We will then likely taper Phenytoin which has similar side effects to Phenobarbitol. It would also allow us to go to Phenytoin as a secondary rescue drug.
We still have no concrete results from our visit in Denver. Dr. Roberts is most interested in the skin biopsy. They take the skin sample and actually grow the skin in the lab. The variances they see in the lab over time could expose metabolic disorders. Unfortunately this is time consuming. Other tests we did involved genome sequencing and are also very time consuming. I don’t expect to hear anything until our next visit with Dr. Roberts in the second week of October.
I wish I could say that Claire’s seizure control has improved. But the reality is that she still has good days and bad days. Her best day could only have 5 seizures. But a bad day can still easily clear 50 seizures. The only thing that has really changed is Rebecca and I’s ability to manage her. We are very strategic about her rescue drug. We know that Claire has her worst seizure in the stage between sleep and awake and vice versa. A long string of seizures during that time don’t allow her to rest therefore she gets exhausted and tries to sleep, causing more seizures. Those are time when we give her regular doses of Ativan to help her sleep. We have found that buys us more regular 5 hour stretches of awake time.
Those awake times are extremely valuable to us. We play with her and encourage her to exercise her muscles and interact with us and her toys. Last night, she picked up her rattle for the first time on her own and put in her mouth, quite the milestone for her. We also started her on cereal last week and spoon fed her for the first time. These little wins are very encouraging.
Next week Claire has her Early Intervention appointment with Willamette ESD. We hope to get therapy from both them and private therapy sessions to help improve Claire’s Cognative abilities and her strength.
In other news…. Well, let me preface this by reminding you all that Rebecca had 5 miscarriages prior to having Claire. For that reason, we were resistant to going on a Pharmacuetical Birth Control and risking damage to our delicate systems. In short, Rebecca is pregnant with number two. We had an ultrasound on Friday showing a healthy baby with a heartbeat. We are 11 weeks into this pregnancy. For my part, I am excited about this. I surprised myself by the joy I take in having a baby in the house, even with Claire’s special needs. Since our COBRA will not expire prior to the birth of number two, we will still be double insured at that time. Our newest addition is estimated to arrive on April 17, 2011.
I know the first question many of you will have is “Does another child risk the same disorder as Claire?” The truth is that nobody knows. Since Claire’s disorder falls into a 1 in 500 million category I would suggest we have a better shot of winning the lottery twice in a row than our second suffering the same disorder. That being said, if the geneticist are able to identify the issues, we will have our second tested for the same issues.
Since our family is expanding rapidly. I have decided to retire my company car and take the company stipend. On Saturday we purchased a Volkswagen Routan mini- van from a client in Medford. The van was delivered today.
Thank you for your continued faithfulness and prayer. We ask that you pray for Claire to improve and develop through her therapy. We ask that you pray for the perfect health of our second child.

Sincerely,
Jake

Praise Report!!!

When you have a sick child price is no object for their care. Jake and I decided soon after Claire was born that we would not worry about Claire's medical bills. "God is faithful" became our motto. I received 26 explanation of benefits from the insurance company today. In going through and reviewing each one I was in awe that each EOB stated "you may owe provider...$0." When I sat and added everything together...100% of the Dr's fees were covered from Claire's stay in the NICU @ the Salem Hospital. For those who are curious...that's just over $25,000.00 that we do NOT owe a single penny for. Give God the glory and remain faithful...if this is not a reminder I do not know what is. Thank you all for your continued support and prayers...we are TRULY blessed.

On another note, since being home Claire seems to be slightly improved. The travel home was rough on her, but she had awake time yesterday and again today. We are looking forward to getting her in therapy and enjoy every moment she is awake and playing. I am happy to report that Claire's appetite is improving and she is again eating a more appropriate amount of food for her age. Each and every day God gives us a miracle.

Going Home...Again

So it's September 5, 2010...and we are headed home from The Children's Hospital here in Denver, CO. Our 4 day trip quickly turned into 3 weeks but we leave knowing that no stone has been left unturned in the effort to find a reason for Claire's seizures. The good news from our trip is that overall Claire seems slightly improved and we'll chalk that up to the new medication that was started. We look forward to getting her in therapy so she can begin to strengthen some of her weaker muscles and begin to learn skills where she is lagging. Though we leave without a diagnosis or prognosis, we continue to trust and pray for a miraculous healing from our great Lord and Creator. We are also leaving knowing that the Dr.'s here feel confident in our ability to care for Claire. The neurologist that we were referred to said the only reason Claire should have to go back to a hospital is if she is requiring some iv medication...meaning her seizures are so bad we can not stop them at home. We hope and pray this is our last hospital stay! We thank you all for your continued prayers...they really do make a difference.

August 27, Spray & Pray Diagnostics

It is hard to believe Claire is 5 months old today. In many ways the time has flown by, yet in other ways time has felt like staring at a stove burner and waiting for it to turn red on low heat. We have learned a lot about a turbulent life over these months. We've learned that planning too far ahead is as stressful as it is futile. We learned to lean on faith as though taking each step on a high wire above the Grand Canyon. Your prayer and support help in more ways than you could know. The fact that you read and keep up with our story gives us strength.

Because it has been some time since I have written an update, yet most of you read our Facebook status, I will write in a sort of reverse chronology--starting with where we are going, and then moving into recent backstory.

Today, we had a "Care Conference" with the Doctors at Denver Children's Hospital to discuss the game plan moving forward. Tonight, Claire will start the third and final supplement trial. Pray it works, because we are running out of treatment options. It is a Vitamin B3 derivative called Nicotinamide. Dr. Van Hove had one case...Yes, ONE... case that broke infantile seizures. Chances are slim, but Vitamin treatments are low risk and we can treat without waiting for test results.

I am heading back to work this weekend. I am out of Paid Time Off and will head to Charlotte next week for a company meeting. Rebecca's mom is flying in Saturday to stay the week with her. With luck, we will be able to come home soon after next week, but as always, we'll cross that bridge when get there. I ask that you pray for Rebecca and Terry next week as they watch Claire and wait for test results. Hospital stays are not easy. (Not healthy, not restful, etc....)

Test results... Well, this is what we came to Denver for in the first place. Their world class Metabolic/Genetics department has blasted labs all over the country with Claire's blood. The blood-taking around here has really been Medieval. But the list of disorders they are looking for are pages long. Unfortunately most of the tests are 4 to 6 weeks out. This puts in a bit of a pickle. Claire's seizures over the past few days have been frequent and full of oxygen desaturations. Doctors here are not comfortable with releasing us at this time.

Claire's liver is swollen. Doctors aren't entirely sure why. But since she is on 6 medications, you can guess the most likely candidate. Unfortunately you can't quit anticonvulsants cold turkey. You have to wean them, else risk withdrawal seizures. The first drug she ever started, Phenobarbital will be weaned over the next six weeks, followed by Dilatin. Doctors want to make sure that the drugs are the cause of her liver swelling. There are metabolic disorders that cause seizures and mess with the liver too. So, likely this Sunday, Claire will undergo a liver biopsy.

As you can imagine, we are not thrilled about this. They will take a muscle and skin biopsy at the same time. This will be invasive surgery. But with so few answers, we are willing to look down every avenue. We have limited time too. Claire is 5 months old, but is really only developed to 1 month. We need find a treatment before we miss the small window for her brain to get pushed back on to the right track. In truth, finding a diagnosis with all these test will not likely impact treatment much--she still needs to be on anticonvulsants. But if we can name the disorder, we might be pointed to the right line of drugs.

There are a couple other reasons for the litany of tests to find a diagnosis, if not as satisfying. All of these tests, studies, and biologic samples will be put on ice for future use. In other words, they might not have a treatment for what they find right now, but we may get new answers in the near future, as fast as medicine moves. The second reason is to evaluate the risk to future children. Like I said, both are noble, but not satisfying for Claire's condition.

To be honest, we didn't know why we came to Denver other than OHSU really pushed us to go. What could Denver offer that OHSU could not? As it turns out, OHSU did not delve as deep into Metabolic/Genetics as we thought. So the consultation we've received from that department here in Denver was likely worth the trip in itself, regardless if whether they provide a diagnosis. Let me make a quick note on Dr. Van Hove--the room gets heavier from the sheer weight of his IQ when he enters.

Thank you for continued prayers and support.

Update, August 6

We finally convinced Doctors to put Claire on Fosphenytoin. They resisted doing that for such a long time due to a so-called allergic reaction she had a couple of months ago, though Rebecca and I never really believed that the reaction was specifically to the Fosphenytoin. But now that she has had a few days to get her level up, we have gone since 8:00 last night with only one seizure, by far her longest break in weeks. Unfortunately we have some hurdles to overcome before we get home.

Claire has been notoriously poor at receiving then keeping IVs. So, I guess that after 30 or so of these, we were statistically due for an infection. Yes, Claire has had a fever for 30 hours. Spiking as high 104, we had to continuously give her Tylenol and keep cool, wet, cloths on her to keep her cool. She also had a blood culture come back positive for infection. Claire is now on two Antibiotics.

What is most frustrating about this scenario is that the doctors refused to believe that the IV caused the infection, despite mounting evidence against their prominent theory. They believed that she had an infection in the lungs due to aspiration. We know Claire aspirates during seizures, but she has absolutely no track record of aspirating while eating prior to this incident. Moreover, the fluid in her lungs disappeared by the time the fever started and she's had no congestions that the doctors and nurses hear. The Doctors' lack of deductive reasoning and logic have confounded us and tested the limit of Rebecca and I's patience and civility with this hospital. It is frustrating to just get Claire’s seizures under control, then be delayed by some carelessness with her IV.

This evening the fever finally broke and the night time pediatrician recognized the infection in the IV spot and drained it of puss. Now that is being treated properly, we can move on to the next step of getting home. We need to transition the use of Fosphenytoin to Phenytoin, the oral form of the medication. This is tricky in the least. In infants, who so quickly adapt to drugs, Phenytoin is very unstable, when the drug level can strangely jump from below therapeutic range all the way to a toxic range—precisely why we haven’t considered this option till now. I suggested to the neuro team that the inject Fosphenytoin rectally instead, similar to what we are doing with the Ativan. As of yet however, the Pharmacy have not found any cases where this drug was used this way. There is a first time for everything, but we want some hypotheticals before we consider this option. The good news is that the seizure came into control before Claire got into the therapeutic range of Fosphenytoin. I believe her current regimen will help regulate her seizures in the event her Phenytoin levels get too low.

With your continued prayers we will get to test the home care drugs over the next couple of days then get home early next week.

Back in Doernbecher.

First, let me once again thank all of you that participated in the Pancake feed on Saturday. It was wonderful to see our friends, family, co-workers, and community members who were so generous with their time and donations.

As you most likely already know, we are back in Doernbecher Children's Hospital as of Sunday. Claire has, for all practical purposes, entered Status Epillepticus--the state of constant seizing. Late last week, Claire stopped responding to Ativan, the drug we used to break extended length seizures. After several adjustments to the medicine regimen with very little results, the Doctor recommended that we get admitted back into the hospital.

The Neuro team at Doernbecher's have not provided many new ideas. There are only four drugs left to try, but there is significant hesitation to start on any one of them. Two are fairly new, but don't show great potential for treating the type of seizures Claire has. One is a drug with really nasty side effects. And the last is a drug very nearly related to one Claire is already on.

So where does that leave us? There is one idea being passed around that I ask your prayers on. The Doctor's are considering doing what amounts to a brain reboot on Claire. They would place her into a medically induced coma for several days, then slowly reawaken her. As you can imagine, this is risky. But, no less risky than the large amount of drugs she is already on or the consistent seizing that could potential damage her organs.

We ask for your continued prayers and support. Pray for wisdom for the doctors and relief for Claire, Rebecca, and I.

-Jake

THANK YOU

Hello, this is Traci. I wanted to thank everyone who has donated funds through this blog. The funds are in a donation account set up in honor of the Hales family.
On their behalf I would like to say Thank you from the bottom of our hearts. If you are like me, you have been inspired and encouraged by their steadfast faith in the Lord, no matter what.
Please stand with me and continue to pray about ways to encourage, speak life and hope into their lives and bless them in whatever form you are able. The Hales are a bright light in a dark world as friends and family lets walk along side them in their time of need and not forget about all they are still going through.
Romans 12:12 says
Rejoice in hope, be patient in tribulation, be constant in prayer.
Thank you again for those who have already donated, you are a blessing to the Hales. The Hales clearly have a large circle of amazing family and friends... THANK YOU.
Sincerely,
Traci

Claire meets Great Grandpa & Grandma

The first time Claire got to meet her great Grandpa & Grandma.

Bath time...

Claire decided to sleep through her bath...making for a great photo op! Some day she will come to hate me for these pictures. :)

Metolius River...a little slice of Heaven on Earth

Claire wasn't having her best day ever on Sunday, July 11, but we took her to visit my parents who were camping at the Metolius River. I have been camping there since the age of 2, and though we will not be able to camp there this year...I was overjoyed to at least get Claire out of the house and to a most cherished family vacation spot.

July 8

We've officially been home for two weeks. Freedom from hospitals has been a blessing. We are significantly quicker at administering Claire's medications and other needs in a timely manner compared to waiting on nurses to get permission to do virtually anything. We've been able to take Claire on several small outings, which is tremendously rewarding for us.

Let me thank those of you from Morning Star Church and South Salem High School who have prepared meals for us every other night since we've been home. It really does relieve a burden. Thanks to Mary Lucas for coordinating that effort. And thank you again to South Salem High School Teachers for spending a day at our home and cleaning up our much ignored and overgrown yard prior to our arrival back home. Rebecca's workplace, Bank of the Cascades is conducting a Pancake feed on July 31 at the Applebee's in South Salem as a fundraiser for Claire's medical expenses. What wonderful blessing to live in a community so willing to reach out and help. We love you all and cannot thank you enough.

Claire has not really improved since being home. Last Tuesday Claire had her record seizure day at 65. It would likely have been easier to count the times she was not seizing. That was the end of a six day stretch where I couldn't say we really saw our daughter. When she was not seizing, we had her very sedated. That afternoon I spent speaking with the Epileptologist at Doernbecher's and we ended up nearly maxing out one of Claire medications. The move seems to have been effective, because Claire has had fewer seizures and fewer doses of her rescue meds. She has also had several long stretches of behaving like a normal baby. Hopefully this trend continues for awhile and we do not have to adjust her meds further.

Moving forward, we are still waiting to hear back from the List Serve and the Mayo Clinic in Rochester, MN. If I get any news from either, I will post immediately.

Prayer Request for June 23

Thank you to all of those following our story and praying for us! We have a major prayer request for today, June 23. We are scheduled to go home tomorrow!!! We will be going home with monitoring equipment and rescue medicine so we should be able to stay home this time. Please pray we actually get discharged from the hospital tomorrow, that we get home smoothly, and that Claire continues to show improvement (she's shown slight improvement this last week). We are excited and cautiously optimistic.

God Bless~
The Hales Family

Random photos

Random photos of Claire in the last month. The photos where there is a bandage around her head is an EEG...the bandage helps keep the wires in place. :) After her EEG she gets crazy hair from the glue they use. Claire loves bath time and her bouncy chair...even though I missed catching her smile on camera.

Father's Day

Father's Day~
Jake's first Father's Day was spent at Doernbecher's Children Hospital...as was my first Mother's Day. Though this is not where we anticipated we would be we were able to make the best of our situation and enjoy the fact that we have a beautiful baby girl who brings us joy on a daily basis. I (Rebecca) have been so impressed with Jake. I marvel at the love he shows Claire. I never doubted that Jake would be a great dad but I am nearly moved to tears every time he looks at Claire and tells her she is precious. I do not know if he will ever fully understand how I am impacted by his gentleness and patience and the tenderness he shows Claire and in so doing...shows me.

Claire gave Jake the best present he could have wished for...36 hours without a single seizure. It has been 16 days since she has had a seizure free day. The photos that will be attached to this post are from her seizure free time....the times we cherish the most.

Claire's seizures returned on Sunday and unfortunately we ended the evening with 2 failed doses of rescue medicine and had to resort to using her IV to bail her out. On the bright side, she has been seizure free since (12 hours) and woke up enough to feed by mouth. I also got to practice putting in Claire's feeding tube last night as she was too tired to eat after all of her medication. Though it is a skill I wish I never needed to learn...I placed the tube in the right place on the first try!

Today (June 21) we will be going up on her Zonegran which we feel is truly helping her but only time will tell. Please pray with us that the increased dose of Zonegran is beneficial and that we can still make it home this week.

God Bless and Thank you all for your support and prayers...we would not make it without you.

June 18

Something disrupted Claire's typical epileptic cycle. I can only hope its the Zonegran and its having a positive impact. Claire had one of her bad days yesterday. Although I can' say that today was great with 36 seizures, at least there was no Ativan administered since early this morning. Since I've been back at the hospital from my trip to Boise, Claire hasn't had any seizures. She just bottle fed extremely well, muscled her NG Tube out, and is cooing like she's trying to hold a conversation. Pray that all of that continues.

The hospital is working on getting us home next week. Unlike our first 3 day stint at home before returning to the hospital last time, they will be sending a fair amount of equipment home with us. We are learning how to administer the NG tube in the event Claire is on a bad day and can't eat without assistance. They will also send us home with oxygen and a Pulsoximeter Monitor for those rare occasions Claire's Oxygen desaturates. If I were to guess, I'd say we will go home on Wednesday or Thursday, when I am home for a full day from a trip to Spokane. You know by now, this can all turn on a dime. I ask that you share my cautious optimism.

The Neuro Resident has posted Claire's situation on the List Serve, asking if there have been any similar cases treated or diagnosed. We are also prepping for a 2nd opinion at the Mayo Clinic in Rochester, MN. As the plan stands now, we will do that as an outpatient. Claire's records will be sent over for them to review. In the event that we end up going there in person, it will be for a relatively short visit. As with all things, we will cross that bridge when we get there.

Medication Roulette...June 14

Once again we spin the medication roulette wheel. At this point, we feel like we have as much chance of winning the lottery as we do landing on a drug treatment that will cool Claire's seizures. We've already gone through so many.

Now we are going to try a diuretic called Zonegran. Claire will be peeing even more than she does already. Let me give you a sense for how this works. There are many, many anticonvulsants. And it’s not because pharmaceuticals are aggressively seeking a cure for Epilepsy. Actually, the pharmaceuticals are more likely looking for an antidepressant, diuretic, or some other treatment, but a side effect is as an anticonvulsant.

We do have some good news to report. The withdrawal from the Ketogenic diet has lead to Claire gaining weight again and having regular BMs. We have also ended the drug treatment Trileptal after a two week weaning. The vitamin treatments also appear to have failed, so we will be withdrawing those as well.

There is still no diagnosis for the cause of Claire's Seizures. So we have asked the doctors to be aggressive with genetic testing. I don't hold high hopes for getting a result. But having the tests done now may prove beneficial in the future.

As always, we ask for your prayer. We are desperately seeking a treatment that will allow us to get home. Claire has returned to this cycle of 36 good hours followed by 48 bad hours. We can anticipate Claire's bad days, but have yet to find a rescue medication other than Ativan. We hesitate using it for too long, because she is already growing a resistance to it.

Pray for a treatment that keeps us from having to use rescue meds. Pray for us to get home soon. The hospital is wearing thin as a place of residence.

Prayer Request

I Traci Kenitzer am praying and trusting the Lord do a miracle of huge proportions! As I was preparing to get baptized last week, the Lord put this on heart and here is what I prayed. Agree with myself and others who are trusting for this miracle for Claire and her family.

For Claire: We are praying that just as sudden and unexplainable as her seizures came on they would leave her body, for now and forever. That no medicine or Dr. could be to blame, that the only explanation would be or could be that she was healed by Gods hand and Gods hands alone.

For Rebecca and Jacob: That every single dollar of medical bills that have or will come up from the time Claire was born would be paid off in full. That the Hales would not have to fork over a dime, that the Lord would faithfully and miraculously provide every dollar for them. Then once all of their bills are covered, I ask that the Lord would bless them in excess of $7000. I know that no amount of money can make up for the memories and time lost. But instead, when they bring Claire home healed that money would be that last thing from their mind. That they could just enjoy their daughter and their time with her. They have come so far to have her in the first place!

Agree with us that Lord is going to provide this for their family. He has a greater plan than we will ever know while we are here on earth. I pray that the only outcome of all of this would bring God praise an glory and tremendously bless Jacob, Rebecca and Claire!!! Amen.

Making A Push..June 7

Since Claire has been at Doernbecher’s, she had a possible diagnosis of Tuberous Sclerosis with 50,000 cases in the US, Pyridoxine Deficiency with 100 cases in the US, and now possibly a disorder that has had only 36 diagnoses in the last 40 years.

Shelly came on Saturday to visit us (Thank you!). During her conversation, Rebecca mentioned that I had observed a correlation between Claire's BMs and her seizure activity. This was something I had not thought about in a few weeks. But the mention of it sparked an idea and did some reading.

There is a very rare disorder called Abdominal Epilepsy. It is well known that the Vagus nerve is tied to some types of Epilepsy. The Vagus nerve is also the primary neural pathway transmitting signals from the abdomen to the brain. It is my belief that pains generated in Claire's tummy may be causing at least some of Claire's seizures.

There is good and bad news with this. The bad news is that it would mean at least half the treatments Doctors have prescribed for Claire are not appropriate for this kind of Epilepsy. Doctors will be resistant to admitting that a good portion of her care may have lent to a decline in Claire's overall health. For example, the Ketogenic Diet, which has a high rate of success with most Epileptics, is utterly inappropriate for someone with Abdominal Epilepsy. Imagine having constant tummy cramps and the only food you could eat is sticks of butter. A high fiber diet is more appropriate.

The good news is that if we can confirm this as the diagnosis, her treatment may simplify considerably. Drugs with side effects including stomach pains will be removed. The Ketogenic diet will be supplanted with a high fiber diet. Relatively safe drugs such as Phenobarbital and Keppra will remain as her maintenance anticonvulsants.

Our challenge now is to convince doctors that they need to adapt their treatment. Although we can pull "Patient Rights" we want to be careful not to damage our relationships with her doctors. We were able to convince them to add a laxative in order to regulate Claire's BMs. After some interesting results, I want to take the next step and get her off the Ketogenic Diet. I may have to flex some muscle to get this done.

Added to the mix, the weekend attending physician added Folenic Acid as an adjunct to Claire's Pyridoxine treatment--some cases have shown this to improve conditions for Vitamin deficient patients. Claire had another EEG today. Fortunately it was nice and short.

I hope you get the sense that Rebecca and I are not sitting idly waiting for Doctor's to get this right. We want our baby home and soon. Pray for our strength. Pray for Claire.

June 3-Something Isn't Right

When I last wrote to you, I told you that we got a diagnosis of TSC (Tuberous Sclerosis Complex). Rebecca and I were of course frustrated with that, but who wouldn't be? Perhaps we were in denial, but it never sat well. They made the diagnosis based on the level of Claire's seizure activity and what they thought was a Tumor in her heart. For me, this was weak--far short of ALL the criteria needed make this diagnosis. In typical TSC cases, you would also see skin lesions and issues with the eyes--Claire came up negative for both those test. But Doctors were in such a rush to put together a treatment plan they insisted on sticking with TSC as a "working" diagnosis and suggested that more symptoms would develop over time.

Remember rule number 1? Prayer. I prayed a lot about this. Mainly I asked God for comfort. What I got was a great deal frustration, sleeplessness, and a nagging discomfort with the diagnosis.

I asked for the DNA test of TSC. What I didn't realize at the time was that there is only one lab in the country that tests the two chromosomes for this disease and that there was only a 70% chance of getting an accurate result. On top of that, it would cost $5,000, not covered by insurance.

When we received the paperwork to get the test, I stalled. I couldn't say why, other than I didn't feel right about it. To top this all off, my wife had to communicate this to the doctors on my behalf!!! I have been working during Doctor's rounds. My dear wife has some serious stones. She trusted me enough to arm herself with my discomforts and hold at bay the doctors. Way to go Rebecca! I also told Rebecca that I wanted the Doctors to give Claire Vitamin B6 and again, she conveyed that wish to doctors on my behalf...more on the Vitamin B6 to come...

This morning, after I left for work, the Panda team had come to take blood from Claire for the TSC test. When they were nearly finished taking the blood for the test, one of the Neurologists burst into the room and halted the procedure. Apparently, the Epileptologist was reviewing all of Claire's tests, and reviewed her echocardiogram with the cardiologist. There was a typo!! Yes, an F-ing typo!!! There is no tumor on Claire's heart. TSC is not the correct diagnosis.

But wait...there's more. My poor wife had to endure the lackluster communication skills and unfortunate data bases here at Doernbecher's. Not an hour later, the Pediatric Resident came in and told Rebecca that it still may actually be TSC! I can only imagine my wife's response, and it’s truthfully her story to tell, but that Doctor got a reaming I'm sure she'll not soon forget. Way to go Rebecca!

Remember rule number 4? Advocacy. No one can fight for your child better than you can. An uproar in OHSU's Neurology Department finally dealt us a confirmation that Claire does NOT have TSC. Thank you God for the strength to ask questions, stall, and follow our gut instincts.

Claire has gone 30 hours as of this note without a seizure. My hope is that she has a disease called Pyridoxine Deficiency Seizures (PDS)--seizures caused by a shortage of Vitamin B6. It is terribly rare and horrendously under diagnosed. There are only 100 diagnosed cases World Wide. Why am I HOPING for this? Well, it is easily treated with large doses of Vitamin B6. Pray about it. Pray hard. We're ready to be out of this place.

A Diagnosis, May 28

I wish I could say that there wasn’t much action while I was in Autotrader training in San Diego. My wife and mother-in-law found themselves in an all but forgettable adventure, starting with a trip to Salem’s ER where my wife was older than the ER Doc. Then they had another ride with the Panda Team to Doernbecher’s Pediatric ICU, where I am sure they went sleepless in an open area with monitors dinging constantly doing their best impression of a Vegas casino slot room.

I can’t say that I’m surprised. Claire averaged a nasty seizure every 12 hours and we had to refill her Diastat rescue medication twice during our short weekend away from the hospital. That is unacceptable, and a trip back to Doernbecher’s was inevitable.

I would have liked to have kept you all apprised of this, but I managed to catch a nasty bug from a hacking neighbor on my flight down to San Diego. I was a slug, only managing to get through the course material with help from my old friends Mountain Dew and Red Bull. The time not spent in class I spent attempting to sleep in my hotel room. Only after my last hour and half nap here in the hospital room in Doernbecher’s do I feel coherent enough to write an update.

We have a diagnosis thanks to a gut instinct by the Neurology Director here at OHSU. Claire’s hemorrhage and strong breakthrough seizures reminded him of a similar case which occurred 20 years ago in San Francisco. Yesterday, they did an ultrasound on Claire’s heart and found a tumor. Before you freak out, know that the tumor in the heart will likely go away without intervention. With her other symptoms, this is a lock for a disease known as Tuberous Sclerosis Complex. Rather than dive into a ridiculously long diatribe about what the hell TSC is, I will instead ask you to visit www.tsalliance.org for the gory details.

I will say what TSC is not. It is not cancer. It does not necessarily mean Claire will have severe disabilities. It doesn’t mean significant changes in treatment. If you take the time to research this disease, be prepared that many of the cited examples are worst cases and not what we expect for Claire.

Yesterday doctors began a steroid treatment called ATCH. If you Google this, like I did, you may dig and finally discover that this is a very complicated hormone, using a good bit of the alphabet just to spell it out. The word sounds like something in Swahili. Very complicated indeed. In fact, no one really knows why it can be effective as an epileptic treatment. However, it does seem to have a “Reboot” impact on the brain. It is a short term treatment that can lead to reduced medications and controlled seizures when combined with the Ketogenic Diet. Thank God the treatment is short term. The Co-Pay alone costs $14,500 for 1 month. If effective in the first month, Claire will have two more months of treatment.

Now let me introduce you to one last acronym—NORD. National Office of Rare Disease. This God send of a Federal Assistant Program has flipped the bill for the ATCH Co-Pays.

Please pray that this works. Pray for Rebecca’s sanity and wish her a happy birthday on Sunday. Pray I get over this cold quickly. Pray that Claire’s TSC is causing her seizures but does nothing else. Praise God for Terry, Wes, Calvin, and Erienne. My in-laws have been pressed into service and they are doing a remarkable job. Thanks to the church for providing some groceries and a gas card. The little things really help.

Finally Home...May, 23

Sorry about my delay in writing to you. But, finally getting home after 8 weeks in hospitals has proven hectic. We were discharged from Doernbecher's on Friday.

I wish I could say our discharge was a result of a treatment breakthrough. But really it was just that Doctor's felt that we could provide as good of medical care at home as they could in the hospital. Epilepsy treatment, if described as a single word, would be, "slow". Treatment in adults is modified by very subtle changes in a single medication and then, a noticeable change could take a week or two.

This gets infinitely more difficult with an infant because their mind and body changes daily. On top of that, pharmaceutical companies rarely pay for research in infants, for much the same reason. In research you need a control group and a steady state. Because of a lack of parents willing to allow their children into clinical trials, complete lacks of steady states, and a short range of payoff for the pharmaceutical, don’t count on a sudden surge of infant research any time soon. The only real data comes from case studies. But our country's unusually stringent medical privacy laws make it all but impossible for research universities to compare data and come up with any real solid conclusions.

That was just a long winded way of saying that there's very little doctor's can do for Claire right now. In the meantime, we have brought Claire home and started what looks like our own pharmacy in the kitchen.

On both Friday night and Saturday night we had to use Claire's rescue medication Diastat to abort extended seizures. All in all, Claire has only had 4 seizures since we've been home. I can't say at this point if that is a good or bad thing. These episodes don't match any pattern she's had in the past and there are too many variables to be able to ascribe them to any one thing. One of her medications, Klonipin, is being weaned and she has been in a state of ketosis for a few days.

All I can say is that we cannot afford to be giving her Diastat every day. Not only is it horribly expensive, but she could quickly build a resistance to the drug. In the beginning, it would put her out for 12 hours. More recently, it only puts her out for 2 hours. We can only wait, pray, and hope, she stops having seizures like that.


Some of you have offered and some have already brought us a dinner or two. Thank You! It is one chore that is nice to not have to worry about. And for those who sent gift cards, thank you again! You likely funded supplies for our home pharmacy. If you have something in mind or on your heart, just send us a note or call. With a child of special needs, we've learned already that it really does "Take a Village..."

That said, you're prayers are still most welcome.

A note on coping

Many of you have commented that Rebecca and I have coped well with Claire’s Epilepsy and our extended hospitals stays. I hope you’re right and we have handled it well. But there are no secrets to what gets us through. I want to take a moment to share with you.....................................................................

1. GOD. Okay, I might of lost some of you right there. But hang in. Ever wonder why EVERY civilization creates some kind of religion? Spirituality is imprinted into our DNA and you’d be hard pressed to find an Anthropologist in the world who’d disagree. When there are no answers in the world, God gives us peace, and it is difficult for me to put in words the value in that. There is a source that is pretty good and you may have left on the shelf thanks to poor media coverage: The Bible. Yeah, there’s some good stuff there. Prayer. You may not have a relationship with the Great Unknown, but you’d be surprised what some meditation and voicing your concerns aloud can accomplish.

2. SCIENCE. What? Aren’t I making some kind of fatal contradiction? A paradox? It would be like a Progressive and a Tea Partier compromising and working together—is that possible? GROW UP. Too many Christians forgo science and way too many scientists forgo religion. For me, the science is good…DAMN GOOD. I’ve read my fair share of Medical Journals and Neurology Text books of late. Some people have joked that I’ll have my MD by the time Claire is discharged. It’s true that science still knows very little about the Human Brain, but had my daughter been born a century ago, she wouldn’t have survived her first week.

3. ADVOCACY. Doctors and nurses are overworked, burned out, distracted, have huge egos, and lack any sort of communication skills. The technology is good, but still way behind what it could be thanks to a lack of government intervention in data systems. And, too much government intervention by way of the corrupt FDA. Don’t get me started on health insurance. Rebecca and I write down EVERYTHING. We educate ourselves, observe, and manage. More often than not, WE lead the doctors and nurses. But let me clear, we also LISTEN to what the doctors and nurses have to say. Most are dedicated and love the children. So we pay attention to the debates. We draw on their experience and wisdom. Most importantly, we ask a ton of questions and speak out when we think something is wrong.

4. ESCAPISM. We somehow need to step away and not feel guilty about it. I am able to grind into my work, exercise, read a book, or vegetate on my Nintendo DS. For Rebecca it’s a good cry, a good night’s sleep, some mother/daughter time, or me tearing her away from the hospital once in awhile. Just like our iPad or Phone, we have to shut down and recharge every couple of days. Leave your child with someone you trust. Rekindle your relationship with your spouse. If you two aren’t coherent, how can you expect to manage your stress and parenthood?

Claire & the Ketogenic Diet

Last Saturday night Claire had two really nasty seizures. The first, around 8:30pm, just before her evening meds, she began an unusual seizure. She did her usual eye twitching and when that stopped, we thought it might have been over. But I noticed Claire's arm doing an unusual motion with her right arm repeatedly--it looked like half the crawl stroke. Shortly thereafter she gave the signals for one of her prolonged seizures--she turns red, sweats on her upper lip, then gives a short scream. Then she desaturated and all hell broke loose. It was like a scene straight from ER. There were three doctors and 9 nurses in the room within two minutes. Atavin is the drug of choice for aborting a prolonged seizure and an IV is the fasted way to get it into the system. However, her IV had been removed earlier in the day. Nurses made four attempts to get an IV and failed. Finally, the resident doctor asked permission to administer an IO.

If you don't know what an IO is, count yourself among the lucky. This is an emergency process of administering drugs when a vein cannot be used. (The squeamish may quit here) A small, Dermal Tool like drill is used to drive a needle through the bone into the marrow. Diastat (essentially a rectal syringe of Atavin) would have been preferable, but they could not get the proper dosing back from the pharmacy quickly.

The pain with the IO was sharp enough to stop the seizure. Unfortunately, Claire began a second prolonged seizure at 10:30 that evening. By then, the Panda team had managed to get an IV in her and doctors administer two doses of Ativan to abort the seizure. She has been seizure free since--but we know Claire cycles in three days.

These episodes fulfilled the criteria we set to begin the Kenogenic diet. We started this today. It begins with a fasting period to get her blood sugar low and increase the ketones in the body. Stimulating the body to feeding itself on stored fat results in fewer seizures in most patients. In infants this is fairly easy to control, because you can use formula.

For Rebecca, this means the end of pumping and breast feeding, but only if it looks like Claire can handle the new diet. Rebecca is of course disappointed, but we have been prepping for this possibility for awhile now.

We have also begun learning the necessary procedures for home care. We are learning Infant CPR, how to make the formula, and how to administer Diastat. If you like, you can learn more about the Kenogenic diet at www.charliefoundation.org.

OHSU's Neurology Department is also agreed that the hemorrhage in her brain was not the source of the seizures. They have shifted gears and are prepping us for long term Epileptic care. I have heard from many of you and read many a case study about children who suddenly stop seizures sometime in their childhood. The fact is, most cases of what starts or stops Epilepsy is unknown by science.

Pray that this Kenogenic Diet works and we get out on Friday. As always, I'll give updates. Thank you for your faithful prayers.

Update from May, 14

Back to the drawing board. We tried eliminating one of her medications--Keppra, a couple of days ago. This resulted in over 60 seizures in a 48 hour period. The good news is that none of these seizures were bad. They were all about 3 minutes long and consisted of a little drooling and her characteristic eye twitching.

This afternoon they did a booster shot of Keppra and have added it back into her maintenance medications. The booster shot made an immediate impact and her seizures have slowed through the afternoon. Although we are disappointed, we now at least know that it is unsafe to withdraw medications at this time. So, if you are keeping score, she is now on 4 anti seizure medications--Phenobarbital, Keppra, Trileptal, and Klonopin (Clonazepam).

We are now seriously considering the Kenogenic Diet. This is a diet where 66% of patients see a reduction in their treatment medications and 33% can drop seizure medications all together. Scientists in the early 1900s recognized that religious groups that fasted for long periods had reduced incidents of seizures. Though not always called a Kenogenic Diet, this is an Epilepsy treatment that has been around for a while. It means that the body feeds off of fat as opposed to carbohydrates, which why this diet often gets compared to the Adkins Diet. Of course, in the case of an infant, the Klenogenic Diet will be regulated to encourage growth and has nothing to do with weight loss.

We don't intend on trying this diet unless one of the following takes play: 1. Claire continues to have seizures through the weekend on her four current medications. 2. We get more evidence that she will have epilepsy well into childhood. 3. She continues to quickly gain resistance to her medications over the next couple of weeks.

If Claire has a good weekend, we will go home Monday or Tuesday and continue outpatient care with OHSU. If we do end up going on the Klenogenic Diet, we will stay for at least another week. The diet requires constant blood sugar testing and urinalysis while Dietitians hone in her nutritional needs. The diet consists of 75% fat and as you can imagine, its tricky to get dialed in.

Claire had an EEG done today, showing reduced Electrical Seizure or "Background" seizure activity. This is great news and means that one or both of the new meds are working well. It gives us hope for a good weekend.

To add yet another wrinkle, Claire had some genetic testing done early in the week and some of the more complicated tests are coming back. We discovered that Claire has transposition of two chromosomes, 11 and 22. If you don't know what the hell that means, don't feel bad. The doctor's don't either at this point. They have yet to determine whether the chromosomes are balanced or imbalanced and have requested that Rebecca and I be tested for the same thing. Ultimately, the new information provided by this science will not change her treatment, but may determine how her epilepsy will behave long term or if there are risks for developmental issues. It may also provide more insight into our fertility issues.

Pray hard! We want outta here!

Update from May, 11

Doctors at Doernbecher's have been very aggressive with adjusting Claire's seizure medications. Over the weekend they stopped Phosphenytoin, which had been fairly effective but has some nasty long term side effects. They began a drug called Trileptal. This has a similar neurochemical impact on the brain as Phosphenytoin, without the negative effects. It does however take some time to get to a steady state, like keppra, Trileptal has to be stepped up over a few days--as of now she is taking a full dose.

The emergency drug they have been using is Ativan. Doctors have been using it to end very long seizures or seizure clusters. Its has a sedative effect on Claire, where she is too drowsy to even eat. They have put her on a fourth medication called Clonazepam. This will not be a long term medication, again due to long term side effects. This is from the same family of drugs as Ativan, but sometimes does not cause drowsiness. In Claire's case, it doesn't appear too and so far seems to be working. We hope to take her off it too and use it as our emergency drug.

Claire has not had a Seizure since 9:30 last Sunday night. Doctors are drawing down Keppra, which I don't believe has improved Claire's condition any and have pushed doctors to remove it if they can. Our hope is the combination of Phenobarbital and Trileptal are the winning maintenance medications. If she can go the rest of the weak without seizures, we'll be released by the weekend. But since we haven't made it through our three day cycle yet, we are very cautious with our optimism.

Doernbecher's has been very good with supplying us with a plan B. In addition to drug treatment they may try a dietary treatment called Ketogenics if the current plan fails.

Rebecca and I are doing fine. We are still staying at Doernbecher's in Claire's room. I have been working my accounts here in Portland and doing Webinars with remote accounts from one of the hospital's conference rooms. I have to travel next week, but hope we're out of here.

I think we are finally getting some progress. I will keep you posted and ask for your continued prayers.

Mother's Day...loving words from Jake

For those of you who have been faithfully following my updates on Claire, you no doubt have some idea about the nature of my dear wife, Rebecca. I am fortunate to have had nearly 11 years of marriage with her. We have gone through many trials, but this one is likely the most strenuous.

My wife has sacrificed much over the last six weeks. Though this is the expectation of a mother, she has also shown extraordinary, patience, strength, flexibility, and faith that places her amongst the noblest classes of human beings. I suppose I have some bias as a husband, but I doubt even a few of you would disagree.

Rebecca spent 14 hours a day at the Salem Hospital NICU, then showed amazing strength and faith to walk away and leave her baby in the capable hands of the nursing staff each night. She has kept a log of Claire's seizure activity and her own pumping. She has handled the stress with rationale blessed to very few women. Now we live in a small hospital room at Doernbecher's and she manages to smile and laugh.

My dear wife, I don't know if you can possibly imagine how proud I am of you. Your love for your baby is powerful and endearing. The management of your life and extended family has somehow been balanced, yet stridently dedicated. If anyone deserves a fantastic, escapist Mother's Day, its you. And yet, we will be here, at Doernbecher's logging our baby's seizures, interpreting doctor's speak, and tolerating cafeteria food. There are no flowers, jewelry, or spa treatments this year. But know this, there is no place I'd rather be than with you and our daughter. I love you. Claire will not remember these events and may not ever recognize the love you give her, but I do. And I will remember these days and their trials--and how brilliantly you manage to get through them.

Claire's move to OHSU, May 6

We hit a dead end at Salem Hospital. Our care there was very good. But ultimately we wanted the Neurologists to be up close and personal with Claire. Claire has had break through seizures with three and sometimes four medications in her. We need to find a treatment plan that reduces the risk of bad seizures when we finally take her home. OHSU will be more flexible and perhaps more "experimental" with her pharmaceutical treatments--more than Salem Hospital was willing to do.

The Doernbecker's Panda Transport team carried Claire by ambulance to Portland this morning at 10:30. The transport went well, though Claire did not appreciate being strapped into an adult gurney. We followed her up in our car about an hour later after we picked up some supplies from our house.

Claire is now being hooked up to what is her 8th EEG. This one will be for constant monitoring during our stay at Doerbecker's. Because we are no longer in a NICU, our presence is more valued. We are more responsible for her regular feedings and changings. We will have a button to push to indicate that Claire is having a clinical seizure, allowing the Doctors to review the EEG at the times it was pushed.

We are in a private hospital room. It is significantly quieter than the NICU in Salem. We have our own bathroom, TV/DVD, and a cot for sleeping. But it is not exactly comfortable. We have requested space in the Ronald McDonald House and may end up going there for some sleep opportunities. We may also consider an extended stay hotel, if there is no space available at Ronald McDonald. Fortunately, most of my scheduled work for next week is in Portland, so I can work from here.

We expect to to be here for at least one week. Many of you ask us all the time what you can do to help. Prayer, for the time being, is still the greatest need. Some of you have provided gift cards for dining, which has proven most helpful since we cannot store or cook food. Now that we are in a private room, guests are most welcome. You can find us on the 9th floor of Doernbecker's in room 42. Just pick up the black phone and let the nurse station know you are here to see us. This is a huge facility and the view of Portland is fabulous, with fine dining nearby--well worth the trip.

Thank you, for the umpteenth time, for your prayers, encouragement, and warm support.

May, 1

Claire has had her latest MRI. I wish I could say the results were astounding, but they revealed only that the hemorrhage area was a little smaller. This, of course, is very good news in the long term, but doesn't provide any short term answers.

Today Claire was relatively active and was seizure free. My hope is that she will go several days straight without a seizure and we can draw down the four medications that are in her system. Doctors are also looking at other drug options.

The game now seems to be Pharmaceutical Roulette. In any case, adult or infant, the goal of Epilepsy treatment is to prevent seizures with as few drugs as possible--ideally one. I think we are a long way away from that. So we are in a stage of trying different things. Pray for her to simply stop her trends of seizures after a couple of good days. The damage is healing so perhaps the seizures can become fewer and fewer rather than breaking through more and more drugs.

April, 29

Dear Friends, Family, and Prayer Warriors,

Rebecca and I have noticed a trend of Claire having a very alert day coming a day off a dose of Phosphenytoin. We've pointed this out to the doctors and hope they consider that in her plan to go home. Unfortunately it is a an IV drug, but perhaps there is a pharmaceutical solution that has not yet been considered. Today was a good day, and it was really nice to come home to Claire being alert and interactive.

The big news today is that OSHU had a 2nd doctor take a look at Claire's past MRIs. He thinks that the diagnosis of a stroke may have been incorrect and has ordered another MRI to confirm his suspicion. Instead he thinks Claire's symptoms are more consistent with that of AVM, a vessel strain disorder that causes a hemorrhage in the brain. This is actually has a better prognosis than the stroke, because the cells around the part of the brain in question are still alive, meaning Claire decreases her chances of developmental disorders.

If the New MRI confirms AVM and there is still a hemorrhage, they will consult with the Neurosurgeon for treatment options. I don't have any further detail than that at this point, but I will say that for us, these are the best answers we've had to this point.

Once again, we appreciate your prayers. They have brought us strength and patience. Please keep it up. And one day soon will have Claire home and healthy.

1 month in NICU

How do you do this? The warm, grandmotherly care we've had at Salem hospital has made a rough experience just a little easier. May God bless them in their stressful and sometimes thankless jobs.

We live day-to-day, trying not to contemplate the whys of being here and trying to pass off our stresses in any way we can. For myself, the ability to work in a job I love is a nice distraction. For Rebecca, she has buried herself in the love for her daughter. She makes it through, without a doubt with the power of your prayers and the knowledge that Claire is unconditionally loved by so many including many, who have not even had the opportunity to meet Claire in person.

We hope and pray that this chapter in Claire's life will soon come to a close. But as we have seen, there is a delicate balance to keeping Claire seizure free. She requires a large level of Phenobarbital in her system. Because she is an infant, and apparently metabolizes drugs quickly, it takes little to throw the balance off. We experienced this again yesterday. We introduced a new drug this week called Keppra. One of the side effects of Keppra is to increase liver metabolism. This led to regular seizures throughout the day yesterday. The good news is that the Keppra has definitely cut the effects of the seizures. Seizures were lighter, shorter, and less frightening than the monsters she endured last Sunday.

Today was a good day. There were very few seizures. She had great periods of alertness and activity. Though I dare not say when, I do hope we can be released soon. Please continue your faithful prayer. In addition, if you see a healthcare professional, please thank them for their service and care. No matter where you fall in the political spectrum, we must remember that these are people who sacrifice a great deal of time and exist in constant overwhelming circumstances. They need our encouragement and our sincere gratitude.

April, 18

It seems that every 5th day is rough. And sadly, today was the roughest yet. Seizures began lightly this morning and began increasing in intensity throughout the day. By 4:00pm, she had seizures lasting 15 minutes followed by sequential twitching lasting 45 minutes--you could call this one long clinical seizure. She would only have about a 15 minute break between seizures.

The Doctor isn't really sure what caused this series of episodes today. Though there are some clues. Last night she spit up a good amount of her Phenobarbital. This morning they sent some blood to the lab to analyze the level and it came back at 31. The Doctors want to keep the level in the low 40s. They gave her an additional does of Phenobarbital but it is slow to metabolize.

Later, they would try a oral dose of 2nd medication, Dilantin (Phenytoin). It too is slow, so eventually they put IV in and gave her that version of Dilantin (Phosphenytoin). At about 4:30pm, Claire was finally sound asleep without seizures. She has had to eat through a feeding tube for a majority of the day. While I am writing this, they are doing a Head Ultrasound to see if the hemorrhage in her brain has changed at all. We are awaiting the results of the Ultrasound. I don't expect an update until late this evening.

I want to thank you again for your prayers and the outpouring of offers of help. Truly believe, that we want to graciously accept your help. But while we are in the NICU, there is very little even we can do. Rebecca is here full time, managing her nursing and pumping schedule. I am here when I have office work to do or when I am back from visiting clients. We do get home during the night to get some sleep.

It is rather therapeutic for me to do the house work about an hours per day, so there is little to be done at the house. We aren't allowed to eat in the NICU and we don't care to spend too much time away from Claire. So many of our meals are spent in the Cafeteria of this building. It is difficult for us to accept and use meals at this time. Yesterday Rebecca and I took some time out to have a nice dinner at Alessandro's. A nice treat from Autotrader thanks to winning Sales Person of the Month (given to our entire team in the month of March--quite the story in itself).

This week I travel. But, if our prior experience is any indication, there should be very few seizures until Friday, when I return. I purchased a webcam for my wife so I could see my daughter via Skype while I am away. Please keep us in your prayers, it truly is the best gift we can receive right now.

April, 5

Little Claire had a busy day. We started with a large blood draw for many lab tests. Then went into an EEG followed immediately by an Ultrasound. The Ultrasound showed that the stroke area is continuing to shrink, which is tremendously good news.

The EEG is still showing abnormal activity and what is known as Electronic Seizures--or seizures that don't necessarily manifest in physical behavior. The Pediatric Neurologists has upped the Phenobarbital treatments and has begun consulting with a Pediatric Epilepsy Specialist at OHSU.

The Doctors have ordered an MRI for tomorrow. They believe that the reduction of the stroke area is inconsistent with the electronic seizures she has and they are going to be looking at the blood vessels around the damaged area.

For us, this means more waiting.

Easter Sunday

Easter, the day of Christ's resurrection is a day of new beginning and new life. I have thought much this year about how Westerners adopted symbols of fertility like the egg and the rabbit for this day. The life of our daughter is an incalculable blessing on this day.

Let me thank you for your continued prayer. Yesterday was a rough day. We started off with about 1-2 seizures per hour for the first 8 hours of the day. But, in no small part due to your faith and prayers we turned a corner at 4:00pm. Claire received step-up levels of Phenobarbital until her seizures stopped. She had a feeding late in the day where she was alert and interactive, giving some much needed peace of mind to her mother and I. Overnight she fed well, consistently, and had no more seizures.

Claire's activity level on Friday was likely a contributing factor to her troubles yesterday (in our opinion). Rebecca and I were starkly reminded of the frailties of her brain. We have limited how often she is held and are treating her with the delicacy of a Faberge Egg.

Claire has a very big day tomorrow. A full lab panel, an EEG, and an Ultrasound will likely be the determining factors as whether or not she can come home by the middle of the week as we hope. We ask that you pray through tomorrow during these vigorous activities.

We hope you took time this Easter to visit church and take some time with your families. Each moment is precious.

Thursday, April 1

Rebecca and I began noticing some inconsistencies with Claire’s behavior and asked the nurses about them. She was having trouble focusing on eating and had sporadic reflexes and motions---behaviors not necessarily uncommon among newborns, which is why we thought she was fine for two days. On Monday March 29, she tested high for jaundice and was placed under UV lights for treatment. She is strong and able to remove her eye protection during some of her sporadic motions. I had to adjust her position and eye wear about every five minutes. That evening, we asked that Claire be taken to the nursery so Rebecca and I could get some sleep. The nursing staff observed her having seizures and transferred her to the NICU. She was placed on a drug called Phenobarbital to stop the seizures. Tuesday morning she had a CT scan that identified she had some hemorrhaging in the left frontal lobe of her brain. They then gave her an EEG to confirm her seizures. Fortunately, this is a treatable seizure disorder. Infants are biological adaptation machines. Claire’s brain will be able to compensate for the minor damage. In most cases like this, babies end up living normal healthy lives. The trick for now is balancing the level of the medication against normal behavior. Phenobarbital acts as a sedative and is somewhat restrictive to allowing Claire to feed normally. Over Wednesday, Claire began showing signs of building a healthy resistance to the medication and began bottle feeding. This Morning, Thursday April 1, she had her feeding tube removed and we expect her to be removed from her IV sometime today. We hope to be release from NICU by the middle of next week. Please continue your prayers. They really do work! She is improving with every day. EEGs and anti-seizure medication will be a regular part of our lives over the next year. So I will try to offer consistent updates on her progress. Thank you for love and support. It warms our hearts that we have such dedicated and loving family, friends, co-workers, and clients.

As you continue to read through the updates, you will see that our journey is still in progress…

Welcome Claire Verna

Welcome Claire Verna…
(by Rebecca)

March 27, 2010…our precious baby Claire arrives! I went into labor (for real) about 10 pm on Friday night March 26. At about 4 AM on March 27, Jake decided I was in enough pain to go to the hospital…I was holding out making sure we did not get sent home!

Active labor is intense…and no amount of practiced breathing will help! I did well getting through my contractions but was ready for the epidural by the time we arrived at Salem Hospital. My labor was not difficult. At 8 cm the Dr. finally broke my water and an hour or so later it was time to push. It was discovered at the time my water broke that there was meconium in my water…meaning the baby had a bowel movement in the womb. Meconium is very dangerous if inhaled so we had a respiratory therapist and a nurse from the NICU in the room when Claire was born to suction her out and make sure she didn’t inhale the meconium.

After about an hour of pushing Claire Verna Hales was born at 2:30 PM on March 27, weighing 7 lbs. 5 oz. and measuring 20 inches long. Claire was worked on for about 30 minutes, being suctioned out and getting her respirations under control. After that time…Claire was placed on my chest and our lives were forever changed. The moment your baby is born is truly indescribable. I will cherish that memory forever and am thankful for the pictures that captured our first moments as a family.

We love you Claire Verna…more than you will ever know!


Matthew 7:7
Ask and it will be given to you; seek and you will find; knock and the door will be opened to you.