Finally Out!

They finished the surgery up at 7:15 PM and Isabel is now in recovery. (Adam will be with her shortly; poor Kari has a cold and cannot be in the PICU.) No complications: she lost a bit more blood than usual, but that is likely due to her slightly larger, more robust body. The doctor released the suture and removed the faulty bone from the left side of her head as planned, and then decided to harvest healthy bone from the right side of her skull to patch up her fontanel. She is being moved to PICU soon and will hopefully be released in just three days.

We are currently hoping and praying that she doesn't catch any respiratory bugs in the PICU, which would greatly prolong her stay and complicate her condition.

Thank you for all the love, prayers, and good thoughts. We'll keep things updated as she recovers.

Surgery Update

She is having surgery today and is currently in pre-op. They expect to begin around 2:00 PM. Will update as soon as there is news...

December 15th Surgery

Late this morning, Isabel is being evaluated by an anesthesiologist to see if she can undergo another cranial surgery today. Over the past few months, one suture near the front of her skull has re-fused and needs to be released. Isabel's surgeons scheduled the operation for today, but Isabel has caught a cold and might need to reschedule to avoid complications. We'll update around noon with details: please keep her in your prayers.

Isabel At Six Months

It has been about two months since the last update on Isabel. In the meantime, she has been doing all of her cute baby business, charming her family, and causing a few sleepless nights here and there. Kari and Adam (as well as Karen and Ron) have been working with Isabel to take a bottle. Following her G-tube surgery, Isabel has been receiving sustenance solely from the tube. Everyone has been hard at work trying to help her develop a preference for oral feeding instead. Ideally, the G-tube would then act as a back-up to her normal feeding. This process has been very difficult, as Isabel has not consistently fed through a bottle yet. This effort is very important in her overall development. Oral feeding is a big milestone for babies, so the household has been hard at work on this. The family would certainly appreciate prayer about this ongoing effort.

Also, Isabel is preparing for yet another surgery. Since her last surgery, Isabel has developed hydrocephalus (excess fluid in the cranium). Her doctors have been monitoring this regularly, and have determined that it is not going away on its own. Her surgery is scheduled for next Wednesday, September 3rd. The procedure will consist of the placement of a device called a shunt that will allow the fluid to drain out of her cranial area. The shunt is very small, and the surgeons have stated that this procedure is fairly routine. The maintenance and monitoring of the shunt is critical to minimizing the risk of harm. Of course, Isabel and her family would greatly appreciate prayer about this procedure, but also her ongoing care, which just seems to get more and more complicated. Adam and Kari are about to become shunt specialists as they have already become experts in the fields of G-tubes and hydrocephalus. I’m sure that they would also appreciate prayer as their day to day life gets ever more complex.



Isabel’s family was also informed by her doctors that it is absolutely imperative that Isabel avoid the possibility of getting sick. As we enter into the cold and flu season, the household is going to have to limit their contact with the outside world for the time being. This is certainly not an ideal situation, as the family has such a large network of friends and supporters. Unfortunately, the family cannot receive visitors (even the pizza guy!) until further notice. Of course they can still be reached via phone and email, and would love to hear from you. Until further notice this little family must hunker down for the cold season.

Again, thank you for all of your love and support. Your care means so much to the family. God Bless.

The Folwells & Emrys

Second Cranial Surgery

Isabel will behaving surgery this Friday (June 27th) in order to once again release her fused coronal sutures. The good news? Her plastic surgeon believes the surgery will be relatively brief (about two hours) and the recovery time short (about one week in the PICU). Here's hoping!

Her surgery is scheduled for first thing Friday morning at about 7:00 AM. We will keep everyone posted the moment there is news.

An Update From The Plastic Surgeon

Well, much has transpired since our last update. Isabel was again released from St. Luke's on Mother's Day . In the days that followed, Kari and Adam were able to finally settle into caring for Isabel at Ron and Karen's home. Kari has been receiving regular visits from home-care nurses, occupational therapists, etc. She is getting along wonderfully with her home-care nurse, who provides them both with assistance, medical information, and moral support. Ron and Karen have also provided an enormous amount of support, especially since Adam returned to his job full-time. (Speaking of which, Adam recently received a promotion to warehouse manager! Congratulations Adam!)

Today, during a check-up with her plastic surgeon, it was determined that Isabel would need to undergo another cranio-facial surgery. Dr. Griffiths discovered that the front sutures located at her temples (coronal sutures) have partially fused back together. You will recall that these same sutures were released as part of her first surgery. It is obviously important for these sutures to remain free as Isabel's skull continues to grow and form with the aid of her helmet. The surgery will likely be scheduled in the next few weeks.

The family is trying not to view this recent news as a setback, but it is discouraging to hear that Isabel will have to undergo another procedure so soon after returning home. It is a blessing, however, for Isabel to have received such optimum care and personal attention from her medical team. Dr. Griffiths has been monitoring Isabel's condition meticulously, and this surgery is part of her ongoing care. Isabel and her parents (as well as the rest of the family) are so thankful for YOUR care and support. We have been overwhelmed by your gestures of love and compassion. Please continue to pray for Isabel and her parents as they approach this next milestone. We will keep you updated on the surgery when we know more.

Surgical Update

Isabel is in recovery and the surgery went just as planned--no complications. Barring anything unexpected, she is set to go back home in about a week.

Adam and Kari will be in charge of changing and managing her G-tube could certainly use some prayer and support for that daunting endeavor.

Will report back soon with any news...

Small Surgery Today

Isabel is still in the hospital recovering from her virus. Her breathing has gradually improved but her doctors have decided that a gastric feeding tube (or G-tube) is the best way to ensure she will consistently get enough nutrition after she goes home again.

The surgery to insert the tube is both simple, short, and routine, and Adam and Kari are expecting the procedure to be at noon today.

We'll keep everyone posted as soon as we have news.

A Little Set-Back

On Tuesday, Adam and Kari drove Isabel back to St. Luke's on a recommendation from their home health nurse. Isabel showed signs of having a cold and, consequently, was having difficulty breathing and keeping her blood oxygen high enough. On Tuesday evening, Isabel was admitted into the PICU (pediatric intensive care unit) and was given an IV and oxygen. She'd lost approximately 7 ounces since returning home, despite the best efforts of her parents.

Isabel will be in St. Luke's PICU for at least five days or until the symptoms of her cold have dissipated. Since her airways are very small, the common cold virus can make breathing extremely difficult, even more so than normal, and her oxygen levels must be monitored until she's recovered. The pediatrician is meeting with Isabel's ENT specialist to discuss any options that might make her life easier.

Please continue to pray for Isabel and her parents. It is likely that Isabel will have to return to the hospital every time she gets sick, until her airways have developed sufficiently. Thank you very much for your prayers and your support.

Byron

Breathing and Bottles

The only thing currently standing between Isabel and a discharge for home has been her issues with nasal breathing and bottle feeding. The ear, nose, and throat specialist met with Adam and Kari on Easter morning and confirmed that, rather than performing surgery to open her passages or insert stents, they would be opting for a "wait and see" approach. Isabel has been receiving steroid nose drops for about a week and the medication seems to be working.

On Tuesday, she was taking about 32 ml at a bottle feeding. By Friday morning, Adam reported that she's been taking about 60 ml at a feeding. A huge improvement!

In the next few days, Isabel's caregivers are planning on removing her feeding tube and letting her go 24 hours with only bottle feeds. If Isabel can get enough down and greatly increase her nutrition intake from the bottle, she could be off the tube for good and possibly going home in a week or two.



Adam and Kari have been putting in extra-long hours at the hospital in an effort to personally give Isabel as many of her feeds as possible. Adam, particularly, is a bottle task-master and seems quite determined to bring his baby girl home.



Isabel is, as always, beautiful and alert and strong. She is thriving and growing, pushing the capacity of her newborn bassinet.



She particularly likes a good diaper change.



And she's in excellent hands.

Meeting With The Geneticist

On Friday, Kari and Adam met with Isabel's pediatric geneticist and received news about Isabel's symptoms and condition. Based on the doctor's evaluation and the results from the genetic testing, Isabel has been formally diagnosed with Crouzon Syndrome.

Crouzon (pronounced KROOZ-on) Syndrome is a rare genetic condition that, in Isabel's case, occurred as a random, spontaneous mutation of one gene. The main feature of Crouzon Syndrome is craniosynotosis, or the premature fusing of the cranial plates. This fusing does not allow the skull and other cranial bones to grow and expand normally, potentially affecting the shape of the head and the structure of the face. Many children with Crouzon Syndrome also experience problems with their soft palate, breathing, hearing, and vision.

That said, it is important to know that Crouzon Syndrome and the severity of its symptoms vary greatly from patient to patient and evolve differently over time. There are some characteristic facial features of a child with Crouzon Syndrome, including a tall and narrow head shape, prominent eyes (due to shallow and wide-set eye sockets), a small nose and mid-face area, and a more prominent lower jaw.

For children with Crouzon Syndrome and craniosynotosis, surgical intervention is usually necessary to give the brain adequate room to grow and expand. As you know, Isabel has already undergone one extensive (and successful) surgery to release and rearrange the bone in her skull and face. As she develops and changes, Isabel may need additional surgeries but, hopefully, her surgeons have bought her some extra time with their excellent care.

A few more facts about Crouzon Syndrome:

• With rare exceptions, Crouzon Syndrome is not life-threatening and does not shorten a patient's life span or interfere with basic life functions.
  
  
• 97% of children of Crouzon are of normal intelligence.
  
  
• Since neither Kari nor Adam have Crouzon Syndrome or a familial history of the condition, their risk of having another child with Crouzon is virtually the same as the general population (roughly 1 in 60,000 to 100,000 births).
  
  
• Crouzon Syndrome is hereditary, and Crouzon patients have a roughly 50%-60% chance of passing on the condition to their children.

If you would like to learn more about the condition, here are some helpful links: The Crouzon Support Network FAQ, The Seattle Craniofacial Center, and The National Craniofacial Center.

Thank you all for your continued interest, prayers, and support. We'll update soon about the progress with Isabel's feeding/breathing and plan for going home.

Isabel's New Helmet

Last week, Isabel was fitted for a shiny, new, pink-and-purple-butterflied helmet.

In person, it looks impossibly tiny. It swivels open like a clam shell so that it can be placed carefully on her head, secured with a little chin strap. The helmet is made of a lightweight, plastic outer shell and a contoured foam interior which will be gradually adjusted to accommodate Isabel's head as it grows and changes. With the help of this helmet, Isabel's brain will be encouraged to expand horizontally (side to side) rather than continuing to grow upwards and, with time, the large gaps between her skull plates will fill in with new bone.

Though she is currently wearing the helmet for just a few hours a day, she will eventually wear it for 23 hours a day through the first year of her life.

Adam and Kari report that Isabel seems to be calm and comfortable when wearing the helmet, and they are relieved to have this extra protection for her sweet little head. (Isabel confidentially told me that, yes, she likes the helmet, but mostly because it keeps the NICU nurses from pasting that pink bow to her head. I hear you, girlfriend.)

We'll try to report back later with updated news on Isabel's breathing situation.

Warmest regards,
Melanie

Post-Op Recovery

During the post-op meeting, Isabel's neurologist indicated that Adam and Kari wouldn't be able to hold Isabel for at least four or five days, possibly a week. In an exciting turn of events, Kari was able to hold Isabel today. It's very careful kind of holding, of course: the nurse places a pillow across Kari's lap and delicately lays Isabel down on one side. (The drain from the back of her head has already been removed, but it still very sensitive to pressure.)

Isabel is doing great: she was off the ventilator by Saturday, her CT scan looks clear of bruising, and her post-surgery swelling is already going down. She is still on pain medications and resting peacefully.

The Full Surgical Report

After an amazing nine hours of surgery, Isabel's surgeons met with Kari, Adam, and their families to deliver the following pieces of information:

1) The surgery went well, so well that Dr. Cherney was actually able to release and reposition more of the coronal suture than previously expected, hence her additional hours in the OR.

2) Isabel was very stable throughout, and experienced only two ounces of blood loss.

3) There were no complications, including no accidental damage to her brain or the very thin membrane encasing her brain.

4) Isabel, who is resting comfortably, will have a CAT scan tomorrow to check for bruising or blood on the brain, but everything looks great right now.


In a frightening and difficult situation, this was the best possible news to receive. Isabel's family extends the most heartfelt of thanks to their amazing community of friends and family. Your kind words, thoughts, gestures, and prayers have meant more than could ever be expressed.

Thank you, thank you, thank you, and God bless you all.

Isabel's First Surgery

First of all, I want to thank each of you for your prayers and thoughtfulness in supporting Kari and Adam as well as the rest of our families.

The families met yesterday with Dr. Cherney (Isabel's neurosurgeon) and Dr. Griffiths (Isabel's plastic surgeon) to review their plan for Isabel's treatment. The doctors informed us that Isabel will require at least two surgeries in her first two years of life, possibly more, depending on complications that may arise in that time.

Her first surgery is scheduled for tomorrow, Friday, March 7th, at 9:30am at St. Luke's in Boise. It will consist of two procedures, together lasting approximately 4 hours. Her recovery time is expected to be around one week. The procedures are part of an effort to remove the obstacles that are preventing Isabel's head from growing the way it needs to. In simplified terms, a few of the growth plates in her skull have become fused together. This is causing her head to grow in only one direction (up) instead of all directions at once. The surgeons will be working exclusively on Isabel's head, and although blood loss is not expected to be substantial, it is always a critical issue in infant surgeries since any loss of blood in someone so small is significant. This and other risks of surgery were explained to us all in the meeting.

As you can imagine, this information is difficult to hear and to process. However, this surgery is absolutely necessary, and will be the product of much planning and careful consideration on the part of the surgeons. They conferred with a number of specialists from around the country at the conference this weekend. The plan for this surgery has been reviewed by the most skilled and knowledgeable cranio-facial specialists in the country. Isabel is in in good hands. With prayer support, we are hopeful for a full recovery.

We will give an update on Isabel immediately following her surgery.

Some Test Results

Based on the results of her EEG and MRI last week, Isabel's doctors indicate that 1) her brain isn't exhibiting any seizure activity and 2) the swelling in her brain hasn't noticeably increased. These are both encouraging signs. Additionally, a test of her vision and hearing shows both senses to be in great working order.

Today, we are waiting on additional news from her neurologist and feedback from the weekend conference. We'll post an update as soon as possible.

Adam, Kari, and their families offer big thanks to everyone for the continued outpouring of support, meals, and prayers over the past week. Your kindness is overwhelming. Isabel can't wait to meet you all.

First Update

First of all, I want to thank you all for your prayers and your concern over our Isabel. Kari and Adam and the rest of us have had a pretty tough and scary week, and it looks like it may continue into the foreseeable future. Since she was born, we have had to practice some monumental patience as we wait for little bits of information to come our way.

To date, we have been seeing the various symptoms that Isabel has been suffering from, but the cause (or the source of the problems) wasn't discovered until yesterday. Dr.Cherney, a St. Luke's children's neurosurgeon studied Isabel's CAT scan (and various other imaging and test results) and met with Adam, Kari, Matt (Adam's brother), and myself yesterday afternoon to give us his assessment of Isabel's condition. Here is a simplified summary of what we know to date:

The blood being sent to Isabel's brain is not leaving the area as fast as it is coming in. The arteries bring blood in, and the veins take the blood back to the heart. The veins leaving her brain are not large and effective enough to allow an adequate amount of blood to leave her brain. This is causing a certain degree of pressure in Isabel's head, which seems to be the source of her other symptoms. This is not part of any condition or syndrome that the doctor has ever seen before, so he will be conferring with other specialists in the country to examine her case. One blessing that we noted is that Dr. Cherney is attending a cranial/facial specialists conference this weekend which will be attended by the most experienced and knowledgeable doctors in the country in this field. They noted that Isabel would probably be the star of the conference, and more importantly, she is likely to have the attention of the particular doctors that can help her.

The doctor gave us a lot of information rather quickly yesterday, so everyone is still processing what they heard. The four of us in the conference room talked for a little while after the doctor left, so that we could all recount what was said, and be able to articulate it to other people. There was much more information discussed than what I have summarized above, but in the interest of keeping things concise and easy to pray for, I decided to paraphrase it.

There is one clearly defined action that we can all be praying for. The pressure in Isabel's head is not critical right now, but prolonged, chronic pressure is a big problem. Until the pressure is relieved, her condition is not likely to improve. We were disheartened to hear that this cannot be solved via surgery. In fact, Dr. Cherney stated that there is nothing that he or anyone else can do to solve the pressure problem. This is something that Isabel's body has to do by itself. When there is inadequate means of circulating blood out of the brain, the body will try to create other channels for this happen, in the form of new blood vessels. So, in effect, we have to give Isabel some time to generate these. This couldn't be more in God's hands at this point. If you would like something specific to pray for, this would be it. Isabel's body has to create these new channels in a timely fashion, and in locations in her head that don't cause other problems later...all on her own.

One thing to note: babies are metabolically superhuman. They produce and develop cells as part of their normal growth at an alarming rate. They are extremely resilient and can do things ( like growing new veins ) very well. As fragile as they are, babies were designed to survive. Isabel's problems seem to exist entirely in the cranial (head) area. The rest of her body has been imaged and evaluated, and appears to be in perfect working order.

She is a beautiful little girl who is very strong and doesnt like to have I.V.'s in her hands (she has pulled out at least three). She grabbed the doctors arms when she was born and didn't let go. When she sleeps, she folds her arms to her chest, and was even caught "praying" with her hands folded a couple days ago. She crosses her legs like she's "taking it easy", and absolutely does not like to be disturbed from this lounging for diaper changes and check-ups. She has beautiful blue eyes that are very alert and attentive to what is going on around her.

Her breathing has been improving day by day. Her oxygen levels are perfect as long as she is calm and resting, or eating (or lounging). When she gets upset, the oxygen levels go down, so this is another thing we can pray for. She has to be consistent with her oxygen levels before she has any hope of going home.

Adam and Kari are amazing parents. They are so committed to Isabel and her care. They are both at the NICU with Isabel every day, all day, with short breaks for fresh air and food. They are staying with Ron and Karen for the time being, to make travel to and from the hospital easier. They appreciate your prayers and concerns very much. As you can imagine, they really need the space to be good parents to Isabel at this time. Isabel's grandparents can also use some space to process all this new information. We ask that you keep us all in your prayers, and if you have any questions, please contact me via email and I'll try my best to answer them. If you would like to contact Isabel's parents or grandparents, send me your contact information, and I'll make sure that it gets to them. I try to see Isabel everyday and keep up to date with her treatment, so I will update you all as we know more.

Thanks again for your prayers and your concerns. We very much look forward to the day that when Isabel can meet you all.

Byron