THANKFUL

It has been a roller coaster year in the Pheif house and we have so many things to be thankful for.  We are so thankful for Hayden's health. He is still on continuous oxygen during the day and ventilator at night. Each month we go to see the pulmonologist to test his lung capacity and each month his lungs are slightly better than the last. We look forward to the day that he can go without supplemental oxygen and return back to his old life....but we take one day at a time.  We are thankful for all of our family and friends who continue their support of us and Hayden. You all are so amazing and we really don't know what we would do without you. We are thankful for Mt Tam School and families who have embraced Hayden and have been amazing at making him feel just like a normal kid. We are thankful for Old Mill School and families who continue to support Elsa and help us guide her along life's unpredictable path...at an 8 year old's pace. I am thankful to everyone at Carnegie Fabrics who have been there for me this entire time and made it possible to take five months off of work to be there for Hayden. We are thankful to all of Hayden's friends, new and old, who embrace him for who he is and ignore all of the nurses and machinery that surrounds him. You are the ones that keep him going every day. You are the ones that make him a very happy kid. And at the end of the day, that is the most important thing of all...to be happy. Happy Thanksgiving.

More good news on FOP Research

NIH TRND program announces next round of drug development projects

An inhibitor compound for treatment of fibrodysplasia ossificans progressiva. 
Kenneth D. Bloch, M.D., William T. G. Morton Professor of Anesthesia
Harvard Medical School, Massachusetts General Hospital, Boston.

Fibrodysplasia ossificans progressiva is a rare inherited disorder where muscle and connective tissue such as tendons and ligaments are gradually replaced by bone. The compound under development has shown efficacy in a mouse disease model.

9th Annual Hayden's Hope Fundraiser for FOP Research

November 2011

Dear Family and Friends:

As you know, this has been a challenging year for our family. This letter is written to provide an update on Hayden, to share how your contributions are moving FOP research forward, and seek your continued magnificent support of this cause.

Hayden's last night at UCSF PICU after a 4-1/2 month stay.

On July 11, Hayden came home from the UCSF Benioff Children’s Hospital after a four and a half month stay in the critical care intensive care unit for a collapsed lung that stemmed from a bout with pneumonia. Due to the critical state that he was in, a tracheotomy was performed and his breathing is now supported by a ventilator at night and supplemental oxygen during the day. Although challenging, home has been a wonderful place for more recovery. Our home’s many stairs have turned into a blessing in disguise as physical therapy, as Hayden can go up and down the stairs better and better each day. He was also able to enjoy six weeks of “summer vacation” by having lots of play dates and catching up with friends, going to the movies, visiting Swirl (his favorite frozen yogurt store in Mill Valley) and hanging out with cousins Anika, Zachary and Sammy Kaplan.

Hayden & his sister Elsa enjoying frozen yogurt from Swirl.

Earlier this fall, Hayden started sixth grade at Mt. Tam School in Mill Valley. Nervous and excited at the same time, we made it through the first day knowing this will be a great place for him.

In August, a team of 27 scientists representing 11 universities gathered at the University of Pennsylvania for a two-day workshop designed to establish priorities for developing effective treatments and eventual cure for FOP. This meeting marked the first time that such an extensive team of scientists all working on related aspects of the FOP condition were assembling in one room. The number of targets for therapy and compounds that offer potential for treatment are growing, and there are many other talented research partners assisting the great work of our core FOP laboratory. More information about this progress is found in the October 2011 issue of Milstones, the IFOPA newsletter.

Since 2003, Hayden’s Hope has been a pacesetting fundraising event for FOP research. Over one million dollars in revenue has been generated through the eight special events that have been held in the Mill Valley area. The progress of researchers is made possible by your ongoing financial support, and those of other FOP families dedicated to the cause.

This year we are asking our family and friends again for their support by making a donation to the International FOP Association (IFOPA) in honor of Hayden’s Hope. Please make your donation either on the IFOPA website or via check to the IFOPA.. All contributions are tax deductible. They will be credited to Hayden’s Hope and directed toward FOP research.

We are excited to get back to normal life and take each day one at a time. As always we are thankful for your ongoing support and encouragement.

Love,

John & Megan Pheif

Hayden enjoying a sunny day.

My Friend Adrienne

When Hayden was in the hospital, during the darkest of days, I started reading my friend Adrienne's blog.  I've known Adrienne since high school and she has always had a quick and humerous whit.  Adrienne's blog is called The Momalog - Good Enough Parenting (http://www.themomalog.com/).  I would read her posts late at night when I couldn't sleep and her words used to make me laugh so hard I would have tears in my eyes.  It was such a great escape from what was really going on around me.

Adrienne has honored me and helped to spread the word about FOP by posting a piece I wrote back in April, when Hayden was so very sick and we weren't sure if he would be able to come home or not.  Here is the link to the post.  http://themomalog.com/2011/11/08/my-friend-megan/

Thank you so much Adrienne.  I couldn't have gotten through the 4-1/2 months without your humor.

Genetic Technology Breaththrough

Proof-of-Principle for FOP Treatment

Editorial explanation of landmark paper published in the Thursday October 20, 2011 online edition of Gene Therapy a Nature Journal is available. Read how Dr. Fred Kaplan, Dr. Josef Kaplan and Dr. Eileen Shore have developed a new genetic approach to specifically block the damaged copy of the FOP gene in cells while leaving the normal copy untouched.

Click on the link below to read Dr. Fred Kaplan's and Dr. Eileen Shore's editorial explanation


Genetic Technology Breakthrough



Click on the link below to read the paper published in Gene Therapy a Nature Journal

Restoration of Normal BMP Signaling Levels

Hayden's Input to the Future of UCSF Mission Bay

Well when you have spent as much time in the hospital as Hayden has, you have a little bit to say on what you think could be improved.  Dr. Seth Bokser, head of UCSF IT, took time to listen to Hayden's input as it relates to IT and presented at this year's Salesforce.com Dreamforce Convention. 
Start at minute 16:40 to hear.
Information Technology and the Bleeding Edge of Healthcare

Tonight I said good bye to my mom

Tonight I said good bye to my mom. She is the best mom in the world and it was one of the hardest things I have ever done. I don’t know if she will still be with us when I wake up tomorrow so I am going to tell you about her tonight while I can still write in the present tense.


Here is what I can tell you about my mom.

My mom is an “Okie” and a “Sooner”. You’d never know it because her Tulsa accent is long gone. She moved out to San Francisco to follow my dad. Every time I pass the Swenson’s on Hyde and Union, I look to where her first apartment was just across the street in the cute 2nd story corner building.

My mom can sew a prom dress just by looking at the picture from VOGUE that I tore out and gave to her. It is a beautiful dress. I still have it.

My mom can make a mean Halloween costume out of a paper grocery bag. I walked many laps around the hills of Sausalito with a paper bag over my head and a huge bag full of candy in my hand.

My mom taught me how to be a good mother. To be there for her kids on hot dog day at school, as a troop Girl Scout leader, teaching us to ride our bikes and reading us every single Nancy Drew book.

My mom believes in me. She still tells me that I can do anything or be anything I want to be if I put my mind to it.

My mom is a great cook. Her recipe box is a treasure trove. I love all of her meals, except for the one time she served us sole with bake bananas. ..still won’t do that one again.

My mom is an amazing singer. She loves to sing in the car and embarrasses me like any mother does when she is singing in the car with her daughter. She sang the loudest and the best at church.

My mom is very creative with flower arrangements. She did all of the flowers for almost all of the fundraisers that I held. And she made sure that Star of the Sea was embellished with beautiful bouquets.

My mom has an infectious smile and laugh. She loves being social and I swear she seems to know everyone.

My mom is a great person, mother, wife and friend. Her DG friends are to this day some of her dearest friends.

My mom has instilled in me the importance of family and spending time together. We would spend time as a family skiing at Squaw, laying on the beach in Glenbrook, driving across the country to see family in Oklahoma or just hanging at home in Sausalito.

My mom’s spirit is so genuine and loving. Lately she has taken it to an extreme by giving hugs to people she barely knows…such is the disease that is consuming her mind and body. I suppose it could be worse. To die blissfully unaware that you are dying is a good way to go.

I love you mommy. I hope that you will find peace when you leave us. I know you will be so happy to be reunited with your sister, parents and friends that have left before you. And when you do go, I know that you will always be my guardian angel.

A Good Way to Live Life

May today there be peace within. May you trust that you are exactly where you are meant to be. May you not forget the infinite possibilities that are born of faith in yourself and others. May you use the gifts that you have received, and pass on the love that has been given to you. May you be content with yourself just the way you are. Let this knowledge settle into your bones. (Author unknown)

"New Normal"

It's hard to believe that Hayden has been home from the hospital for a month.  Summer is flying by and Hayden is getting better and better everyday.  In the mean time we are all adjusting to our new normal.  I hate that word "new normal", but I guess it's kind of true. Its all new and its as normal as its going to get for the time being.

Top 10 Ten Reasons you know your life is sort of getting back to normal.
1. You’ve been home for one month and haven’t had to call 911.
2. The sweet sound of Sponge Bob permeates the house at all times.
3. You go out to dinner with the entire family.
4. You take the Hayden to the friend’s houses, movies, to the pool club, Best Buy and Swirl.
5. The quiet nights are free from alarms and Code Blue PA announcements.
6. Hayden misses the cute nurses from UCSF…but he would never admit it!
7. Hayden used his “bribe” money to buy an X Box and now his biggest worry is what games to buy.
8. The kids are starting to annoy each other again.
9. I have taken control again of the social schedule for the entire family.
10. John and I don’t have to sleep in a foldout hospital chair anymore.

Really Mom, my sister is annoying me!

Dinner at Marin Brew Co. while watching the Giants game.

Yummy frozen yogurt at Mill Valley's Swirl.

Hanging at the pool with friends.

Top 10 Reasons you know you’re not quite back to normal:
1. Your son is still on a ventilator at night and oxygen during the day.
2. You have to pack up O2 and life saving equipment whenever you go out of the house with Hayden.
3. You have registered your house with the police and fire department as being potentially "explosive" because of all the oxygen in house.
4. You're constantly worried he's going to get sick again.
5. The Master Bedroom is now Hayden’s room and Hayden’s Star Wars room is now our room.
6. When I go to sleep at night it is in a king size bed situated in Luke Skywalker's Sand Cruiser and there are glow in the dark stars on the ceiling.
7. All of the grown up art in the master bedroom is covered with Simpsons posters and Sports posters.
8. Life size images of Buster Posey and Lionel Messi are permanent fixtures in the master…I mean Hayden’s new room.
9. The first night nurse fell asleep on the job….John and I are doing "night watch” until a replacement is found.
10. Instead of the fold out hospital chair, which ever of us is on “night nurse” shift sleeps on the blow up bed next Posey and Messi.


The blowup bed in Hayden's "new" room where we keep watch
over him at night until a new night nurse is found.




O2 Tanks waiting to go out on an adventure with Hayden. 
The machine next to them is a condenser that provides oxygen
for Hayden and refills the tanks for us. We are high tech here!



Scientists Discover Why Bones Grow on Muscle

Researchers think they've found out why some people's muscles mistakenly grow bones.

The condition, called heterotopic ossification, occurs when an area of the body is signaled to grow bone rather than other tissues. In short, the condition gives rise to bones growing in places they're not usually found -- in muscles. People may experience the phenomenon after recovering from injury or may have it from birth.

NEWS: New Artificial Bone Made of Wood

Though there's no way to prevent or know when a person will develop heterotopic ossification, scientists now have a starting point to develop drugs that could help treat the problem.

Approximately 1 person out of every 2 million people worldwide is born with a permanent type of the condition called fibrodysplasia ossificans progressiva (FOP), which causes muscles to consistently produce bone, especially when a person is injured or sick. In more serious cases, the disease can hinder a person's mobility and even shorten his or her life.

In a study in the Journal of Cellular Biochemistry, researchers at Northwestern University's Feinberg School of Medicine found high levels of a neuropeptide called "Substance P," or SP, in both FOP patients and individuals who developed heterotopic ossification. From previous studies, it's known that SP causes inflammation and is used by the brain's neurons to help send white blood cells to injured areas of the body.

NEWS: Gel Heals Injured Brain and Bone

By studying 16 tissue samples from patients living with heterotopic ossification and FOP, scientists noticed a spike in the compound. They also compared the samples with four male controls. In other experiments, they discovered that "knocking" out the gene that produces SP in transgenic mice lowered their chances of acquiring the condition.

Despite potential treatment options in the future, researchers will need to find more about how SP works in a larger number of patients and whether it can be realistically controlled in people with life-long forms of heterotopic ossification.

Photo by Minervaaa/Wikimedia Commons

Research Breakthrough - Neurological Connection to FOP

The Nervous System and FOP

New insight into the neurological connection in FOP bone formation, and potential new treatment targets. Pieces of the puzzle are coming together!

New research findings recently published in two complementary articles have found a distinct connection between the sensory branch of the nervous system and the formation of Heterotopic Bone. A very exciting new discovery! An editorial by Dr. Fred Kaplan, Dr. Eileen Shore and Dr. Bob Pignolo of The Center for Research in FOP and Related Disorders at the University of Pennsylvania describes the significance of these findings as it relates to FOP.  Click here to read: Sensory Nerves Regulate the Innate Immune System and Amplify Heterotopic Ossification (PDF format)


Article published by Baylor University describes the connection between the sensory nerves and the regulation of the immune system. Two researchers/authors of this article, Francis Gannon and Elizabeth Olmstead-Davis have previously worked in the FOP laboratory at the University of Pennsylvania. Click here for article: Sensory Nerve Induced Inflammation Contributes to Heterotopic Ossification

Article published from Northwestern University in a collaborative research effort with the University of Pennsylvania describes the connection between a neuro-inflammatory factor (Substance P) and FOP/Heterotopic Ossification. Click here for article: Substance P Signaling Mediates BMP Dependent Heterotopic Ossification

Home Sweet Home

I can't believe Hayden has been home for over a week now. It seems a lifetime ago that our home was at UCSF. The doctors and nurses were so amazing. I was so giddy with excitement that I couldnt sleep the night before.

Last night at UCSF...too excited to sleep

Getting Ready to Go Home

 When we got the discharge orders all set and the nurse rolled in the wheel chair, one of Hayden's doctor's said "Oh no Hayden can't use the wheel chair. I want to see Hayden walk out these doors!" And with a typical smiling smirk on his face, he got up and started walking. Hayden walked out of the ICU at 10:30am Monday July 11th to the standing ovation of applause from all the doctors and nurses. It was such an emotional moment. Tears streaming down my face. I felt like we had won a race, accomplished an incredible feat, had a major victory and I guess you could say we did all of those.

Home is like Miracle Grow on Steriods for Hayden.
He is thriving and getting stronger everyday.

Home for Hayden has been like "Miracle Grow" on steriods for him. He is blossoming and thriving like you just can't do in the hospital. Hayden has been walking up and down our stairs and getting stronger everyday. He is busy entertaining friends, interviewing nurses and catching up with just being a regular boy.

Here is a short video of Hayden walking out of the ICU after four months.http://www.youtube.com/watch?v=8Unsal87R4w



Thank you Desanne, Tracey and Alison for
the welcome home decorations!

Packing Up & Moving Out

Four Months ago today Hayden was intibated and put on a ventilator. It was one of the worst days of John's and my life. We cried because we knew the worst was a distinct possibility and you never want to see your child that sick. We have endured because of all of you. All of you nourished us with love, food, visits and support. We really do not know what we would have done to make it through these four months without all of you by our sides cheering Hayden on to get better.

So now we are ready to go home. Monday July 11th at 9am we are walking out of the ICU and never looking back. We've made some good friends here who we will always stay in touch with, but hopefully we will never see them in the "working" environment again. Hayden will go home with a ventilator and we are still working on getting him being comfortable breathing room air during the day so that then he would just have the ventilator at night.

Yesturday we started packing up. Check out those empty walls! We gently "de-decorated" the room yesturday and now Hayden's room feels so baren. All of the cards, drawings, posters have been taken home to be put in a memory book. All that is left hanging is the Giants Calendar and Hayden's target for Nerf Gun Target Practice.

Hayden excited about packing up. Check out the empty walls!
Yikes!

Lonely Giants Calendar

Target for Nerf Gun Target Practice

Going Home!!!!!!!!!!

Hayden sent me and everyone he knows an email yesturday. Here it is verbatum.

"Yaaaaaaaaaaaaaaaaay!!!! I'm coming home from the Hospital on Monday July 11th, in 3 days after 4 MONTHS!!!!!!!!!!!!!!!!. WoooooooooooooHoooooooooooooo!!!!!!!!!!I'm so EXCITED!!!!!!!!!!!Send this to any of your friends!!!!!!!!!!!!!!!:):):):):):):):)"

I think he might be just a little excited to come home. What do you think?

Lazy Days Waiting to Get out of UCSF

Discovery Science / How Stuff Works on FOP

A great article by Discovery Science/How Stuff Works about FOP.
Click here to read "How Fibrodysplasia Ossificans Progressiva (FOP) Works"


The skeleton of Harry Eastlack, a man with FOP,
from the collections of the Mütter Museum,
 College of Physicians of Philadelphia



Hayden's Rehab Days

We are so ready to go home!! Of course we could actually go home today if the nursing was lined up for him, but apparently we've been told the it can take up to a month or so. UGH!!

So we are keeping our time busy by doing some fine tuning on his night time ventilator, slowly weaning off of the ventilator during the day, working on eating more and more food such at pizza, chicken nuggets and smoothies. Best of all we are able to do lots of physical therapy to get Hayden moving again. Hopefully by the time we have our nursing worked out, Hayden will be able to walk, literally, out the front door! For a kid who has been in bed for three months that would be a great victory for him. He has been working hard every day with a physical therapist. We work on weights, balance, walking, walking backwards and of course if you live in Mill Valley, especially our house, we are working on stairs!

Hayden does some fun things during Physical Therapy. Some games he playes to practice balance include target practice with a Nerf gun, Basket Ball and Tic Tac Toe Toss.


Hayden in the Pediatric Rehab Room



Hayden Plays Basketball to help with balance.




Practicing Stairs...we have a lot of those at our house.








Hayden plays with the Nerf Gun to practice balance.

 

Hayden Takes Aim

Have You Ever Bribed Your Child?

Have you ever bribed your child? I mean seriously, what parent hasnt and dont' lie to me! OK I know it is not at the top of the list for "Better Parenting" and teachers would definately not approve. So if you are a teacher, just close your browser now because you are NOT going to like the rest of this post :)

I currently owe Hayden $300 dollars. You may ask yourself what in the world did he do to get that kind of cash? I personally am wondering if I've lost my marbles too and am thinking I might try to see if he'll take an IOU or a credit card to cover "charges incurred".

It all started with this little purple valve called a Passy-Muir valve which enables Hayden to talk with his trach. So you may remember that over these 3-1/2 months, Hayden has not been able to talk because he was nasally intibated. As a result I am now proficcient in reading lips, a skill that required a high amount of concentration when your child is talking 100 words a minute!!!

But now that Hayden has a trach, he can talk if we put on the Passy Muir valve. Hayden has decided that it makes it harder to breath with the valve on and is stubbornly refusing to ues it. Strangely the Docs did an evaluation on him while he had the Passy Miur valve on to look at O2 intake and it did make his O2 intake a tinsy, tin,y minutely, bit, slighly lower. So I said to Hayden, "Really? Dude you have got to wear this thing Mommy's eyes are really worn out from trying to read your lips." and what do you think he said...."Nope!"

So being the desperate mother that I was, I told Hayden that I would give him $1 for every minute that he wore the Passy-Muir valve. And do you know that kid wore the valve for 5 hours. No problem. Playing Wii, computer, doing physical therapy, the whole shabang. Yes 5 hours is 300 minutes.

When Hayden woke up the next day and wanted to put on the valve, I warned him that my deal with him was a one time only gig. He just smiled his cute impish smile and said I know....and then asked when I was going to pay him the $300. Ha! Kids!

New Beginnings

With milestones come new beginnings and new chapters in our children's lives. The pang of nostalgia is coupled with the pride of a parent as Hayden participated in Graduation from 5 th grade last week. Its an amazing milestone, especially for Hayden considering what he has been through these last three and a half months. The milestones also mean new beginnings, a new school, new friends, new adventures. Its hard to believe how quickly they grow up. I can remember when he was just born and mothers would come over to see the new born baby and remark "Enjoy this time. They grow up so quickly!". At the time I thought "What are you talking about?", but now I know exactly what they mean.

What is it about each of these new beginnngs in my children's lives that brings a tear to my eye. Their first breath, their first spoonful of food, their first step, their first word and each one of these milestones is such a precious moment for us as parents. Case in point, I told myself when Hayden's first day of kindergarten arrived I wouldn't cry. Of course, as the day was upon me and I watched him being led by his teacher into the classroom,sure enough I started crying, "My little baby is growing up!" Its the same feeling I get with each new beginning of my children's lives. It's not that I'm sad. It's that I can't believe how quickly these milestones come and how proud I am of Hayden and Elsa as I watch them embrace each new beginning.

I feel like I am reliving these precious moments as I watch Hayden once again say his first words off of the ventilator, take first bits of food and especially learn to walk again. Seriously, the pride of being a parent couldn't be more as I watch his determined face walking down the hallway.

I find myself giddy with excitement at the prospect of Hayden coming home and getting off the ventilator. I could be even more excited, but I can tell I am holding my emotions at bay a bit because I just dont want to be devastated if Hayden has a big set back. The "what if?" is always lingering in the back of my mind. But most of the time I push those thoughts aside, look to the future and savor each day watching the "old Hayden" return. A new beginning happens here every day for Hayden. Eating strawberries, going off the ventilator for 12 hours, walking across the room, talking again. Its wonderful.

To see one of his lastest accoplishments, click here.

Holy Cow!

Just the words "family meeting" create a pit in my stomach and my eyes automatically start to shoot darts directly at the doctor that is mentioning one of these horrid things. However this time was different and by far my favorite Family Meeting. We talked about setting goals for getting Hayden out of here. Yeah!! We decided that a great goal would be for Hayden to come home so that he is mobile, off the ventilator during the day and on the the ventilator during the night. I can't wait. And the million dollar question that I know you are all wondering is - When will this happen? We hope earliest end of June or possibly mid July. We have lots of "exercise" to do on his lungs and his legs but we strangely very motivated!


Today we celebrated. Hayden, his sister and cousins will be treated to a special private viewing on Kung Fu Panda 2. Conan from DreamWorks brought the movie and showed the kids his very cool job of being an animator. He drew some fabulous Kung Fu Panda sketches for the kids.  Very fun.
 

Conan from DreamWorks, Hayden, his cousins Zach & Anika,
and sister Elsa Lilli

Tomorrow, Hayden is looking forward to graduating from 5th grade. I can't even believe he is graduating from 5th grade let alone realizing he will probably be able to go to 6th grade, possibly without a ventilator. Official ceremonies will be held at Old Mill School where Hayden will participate via Face Time ( Thank you Steve Jobs and Bryan Van Vliet! ). Graduation party to be schedule upon Hayden's homecoming!

Hayden Practicing "Facetime" for Graduation.
















And the Holy Cow part is that Hayden was off the ventilator for a total of 6 hours today!!!  Just awsome.  That boy totally inspires me and makes me so very proud!!

Moving n' Shaking

Exciting things are happening in Room 8 at the UCSF PICU ward! Not sure which I find the most exciting but will start off...

First of all the CO2 monitor was uncerimoniously thrown out. (Picture me throwing it out the window...) Yeah!!! Not that I didn't like the "Iron Man" look, but the new one also didn't read accurately and the doctors decided that Hayden has been doing so well that we really don't need the CO2 monitor any more.

Hayden has been doing great at his drinking. We've had Coke, Diet Coke, Odwala Mango Tango, Jamba Juice. He has been allowed to drink 2 oz five times a day. Plan on moving up to some sort of pureed food i.e. applesauce.

On a personal note, I am getting closer to earning my degree as Nurse Ratched. I have officially learned how to suction Hayden, although I told Hayden he is NOT under any circumstances to wake me up from my beauty sleep for suctioning. In the middle of the night he can press the call button for the nurse. I also am now experienced in lowering and reinflating Hayden's cuff when he drinks. I feel so important :)

Tonight he lost a tooth. I got to pull it out. Those little buggers are harder to pull out that they look. This tooth will be donated to the FOP lab becuase baby teeth have stem cells in them. The FOP lab will use the stem cells in Hayden's tooth to do FOP research. I hear the tooth fairy pays triple when you're in the hospital.

The two best things that have happened this past week is
-One: the ventilator is now permenantly on a CPAP setting which means that it is basically just blowing oxygen in at a set volume. The volume has moved from 15 to 8 in the last week. So Hayden's lungs are really showing us what they are capable of doing.
AND
-Two: the very, very best for last. Today Hayden was disconnected from the ventilator for 45 minutes AND he is still alive to talk about it! So utterly amazing. I started crying when John called to tell me about it. What they do is attach whats called a Trach Cuff to his Trach and it just provides 100% O2 with out any type of support, just like breathing outside. They called it breathing sprints. We are getting Hayden's lungs in shape to get the heck out of here!

Look Mom I'm off the Ventilator!!

Thank you for all of your prayers and well wishes for Hayden.

Well the Trach went fabulous. It’s so wonderful to see his beautiful face again. Hayden’s vitals were strong during the entire operation. He returned to his room and woke up almost right away. Of course we are in pain management mode, but clearly the morphine is working because he is playing on his computer right now. Today Hayden’s biggest concern is making a get well card for Buster Posey.

The other exciting news is that they did a change on his ventilator. Hayden has been on a ventilator setting called PRVC (Patient Rate Ventilator control) support – where the ventilator can totally support all of Hayden's breaths. In this mode Hayden can also breath over the ventilator. This morning we tried out a new setting called CPAP and he did so well that now when Hayden is awake he will be on this setting. CPAP (Continuous Positive Airway Pressure) support is where Hayden initiates all of his breaths but the ventilator provides the volume of air to go into his lungs. When Hayden goes to sleep, they will change the setting back ot PRVC. This is a HUGE step forward for Hayden and he is very excited about "breathing on his own" so to speak. Its so exciting in fact, that all of the nurses and RT are coming into Hayden's room to congratulate him.

Also the nurses disconnect him from the ventilator for certain procedures and during this time he is sort of breathing on his own a bit where in the past when had to be hand ventilated. I am so proud of that boy!!

T minus 14-1/2 hours

Some of you may have already correctly guessed that I am actually really going to nursing school here at UCSF. I am moonlighting as a mother of a really sick child to gleen as much information as I can before taking my boards. I figure why take out a student loan when the insurance company will cover my costs going this route.

There are a lot of pop questions in my studies here. Strangely most have to do with Hayden's FOP, but I do feel that I have suffuciently burned to memory all of the genetics, details, statistics, medical terms etcetra to be able to asnswer any question in that realm.

Today's pop medical question was a good one. I failed. The nurse asked me (as clearly I am all knowing), "Is Hayden's Pick line central, perifferal or in line?" Adopting my most knowing voice I replied, "Well according to the Pick Line Nurse, Hayden's pick line was central but then it got slightly pulled out so i'm not really sure. Does that mean its still central, centered or just left of center? I dont know.". Just for future refence, it is no longer "central", but now a mere "in line". The difference a few fractions of a centimeter make.

I also provide translating services for the nurses. I am now fluent (well almost) in reading Hayden's lips. The most important things to relay are "bed up, bed down, sit in chair, wii off, wii on, movie, tv, suction, pain medicine, more pain medicine and sleepytime medicine."....all very highly medical terms.

I am currently cramming for tomorrow's test which will involve pre-op, hand-holding down to the O.R, post-op and general hovering to confirm vital statistcs.

I hope to finish my studies here in less than three months.

Family Meeting #3

So you may be wondering "What happened to "Family Meeting #2? I never read a post on that one." Well I didn't write about that one because it was too depressing, but I'll tell you about it now. Family meeting #2 was about 3 weeks ago. It was the meeting where the doctors told us that basically Hayden wasn't going to make it and there was nothing more they could do for him. John and I were so very upset....lots of tears were shed. But we both told the attending doctor that how could he tell us this news when Hayden at that very moment was playing Wii in his room. We told him that we just can't believe that what he was telling us was true. It was just so devastating.

I tell you the gods were shining down and the power of prayer was working because from that point on....I just know that (and I'm not that religious) God whispered in Hayden's ear "Show them that they are wrong." ...Hayden's respiratory stats started to improve. And when I told him his birthday was coming up, he got another spark and announced that he wanted to walk! And you all know how the birthday went....FABULOUS!! Hayden showed those doctors that they were wrong and it was so beautiful to watch.

Family Meeting #3 was to talk about what the options are for Hayden now. We were told that Hayden would need to be on a respirator for the rest of his life....another statement John and I sort of knew deep down and it was still hard to hear. But we also still believe that there is a long shot that Hayden could get off the respirator and the doctors confirmed that miracles do happen. The doctors highly recommended Hayden have a tracheostomy. This would enable him to move around more safely. He also could possibly go home. When you say? They say maybe three months, maybe more, maybe less, but that is also a huge guess on tne doctors part. All depends on how well Hayden's lungs continue to heal.

So after the meeting, we told Hayden about the trach. He was sad and scared. We cried together because we are and he is even more so, sick of being on the ventilator, being attached to tubes, frustrated about not being able to speak, eat or drink. Its more than any 11 year old or any child should have to go through,

So many tears, so much frustration. How do I make my boy smile again? The solution walked right into the room. A nurse named Linda was with us that day. She had told us that she had run the Bay to Breakers with her boyfriend and during the course of the run her boyfriend had been running next to a naked man...Linda of course had taken some pictures. I asked her "do you have any pictures of your boyfriend running next to the naked man in the Bay to Breakers?" and she smiled wickedly as she pulled out her iPhone. "Hayden you want to see something that should never happen?" she asked. A small glimmer of a smile started to spread over Hayden's face. Linda showed Hayden the pictures. So funny!! Hayden started laughing. So hard in fact that he started getting tears in his eyes. HA!!! We laughed for a good five minutes at the funny pictures and talked about how people really shouldn't be running naked!.

The trach is scheduled from next Wednesday at 2pm. Remember to say a prayer for Hayden during that time. We hope it will be the next chapter for Hayden towards getting better and getting out of here.

CO2 Monitor and Early Birthday Presents

I have a love hate relationship with the CO2 monitor in Hayden's room. This is the machine I look at right before I go to sleep, in the middle of the night when I wake up and in the morning when I get up - all to see what number is on it. It can change my mood from happy to sad in a heart beat. I've learned that sometimes the number is correct and sometime as the nurses tell me the machine is just used for "trending" either up or down. The way I know when it is correct is when the nurses draw Hayden's blood at 4am to check his CO2 level and we get the most accurate reading for Hayden. I hate the monitor when it is showing anything above 80. I hate it when it randomly flashes any number over 100. I hate the monitor when it is trending upwards and I really feel like throwing the monitor out the window when all night it shows a CO2 of 75 and the blood draw shows a CO2 of 105!

The other thing to note is that doctors and nurses like to call the CO2 reading the "Entitle". They say "Oh, his Entitles look great". Sometimes it sounds like they are saying "His End Titles look great" or "His entrails look great." which I could see as an appropriate comment if we were in surgery. I've asked several nurses and doctors what this word "entitle" has to do with CO2 and no one seems to know....interesting. Entitles, Entrails, End Titles, Titleist...hmm maybe Tiger Wood knows?

Anyway, I digress. There are times when I love the CO2 monitor. I love it when it is trending downward, when it shows any number below 80 and especially tonight when it flashed for the first time a 49! Wow! Can't wait to see what his "blood gas", as they call it, shows for the real CO2. As of this writing it is flashing in the mid 50's - 60.

Hayden's birthday is Sunday and he has started getting some special birthday gifts. You all know what a Giants fan he is and on Wednesday night Bobby from the Marketing Dept at the Giants stopped by with a gift for Hayden. First was a special birthday video of Buster Posey and Pablo Sandoval giving Hayden a pep talk, telling him to get better soon, wishing him happy birthday and they both told him that they would be playing Sunday's game in honor of Hayden. How cool is that? Then Bobby pulled out of a bag a real Giants Jersey signed by Buster Posey. Well that kid was so excited!! He has been using the Jersey as a blanket. Thank you so much Giants!

He also got another birthday gift from Amy Trapp and her daughter. Many of you may not know, but Amy's company Jane Hammond Events caters all of the fabulous food for Hayden's Hope Fundriasers. Anyway, they came by the other night. Amy brought her daughter because she and Hayden went to preschool together. They gave Hayden the cutest stuffy. A replica of Loni the dog, complete with dog collar that says LONI on one side and I Belong To Hayden on the other. So sweet!! So now Loni will always be with Hayden.

A Happy Mother's Day

I had such a wonderful mother's day! I woke up to my beautiful Elsa Lilli looking at me asking when I was going to get up - love that girl! Nice long run up on Mt. Tam. Wonderful brunch with my sisters and parents. Elsa and I got to the hospital at around 3. When I walked into Hayden's room, he was sitting in a chair holding his iPad which was faced towards me showing the above beautiful Mother's day card he had made. Big smile on his face. So great to see him out of bed again. A little later, we got him up out of the chair and he took the four steps to him bed with a some assistance. It made my heart soar. Lucky mom!!

Happiest Mom Today

I am the happiest mother alive today. Hayden’s prognosis has always been tentative. A child with restrictive lung disease coupled with pneumonia is not a promising mix. But today he gave me the best surprises; early mother’s day gifts if you will.


First when I got here, I learned that his CO2 was the lowest it has been since he arrived on March 6th. It was 79. I also learned that his chloride was back to normal levels. Dr. Kitterman had found chloride in Norway that we could bring in if we need it, but now we don’t and I am so happy. Hayden has lost all of his edema, fluid swelling and is back to a normal weight. The only thing left to fix are his lungs. The darn respirator is still on really high settings. On the respirator the lines show yellow when he initiates a breath and red when the respirator breaths for him. Currently it almost always shows red lines, which is the hardest part to see because he now has so much energy and is almost back to his old self.

The second best thing was when Hayden and I were talking about his birthday and doing some planning for it. I told him that the hospital is going to let us use their special kids technology room to have small family birthday and Hayden announced that he wanted to walk there instead of going in a wheel chair. Wow! How cool is that. I told him that we should start practicing because he has not walked for 2 months. So he decided right then that we wanted to try to stand up and we got him up out of bed with a lot of help from the nurses and he stood up. A little wobbly on his legs and I held him to make sure he didn’t fall. But oh boy, that was really cool. The best was yet to come…..

Hayden decided right after that he wanted to go out to the patio so we got him into the wheel chair and rolled him out there with all of his IV’s and meds and a mobile ventilator in tow. We sat out there for a little while and he got a great visit from his special friend in Kindergarten, Mac Swergold and his mom Dawn. We decided to show Mac where we were going to do Hayden’s birthday. So we rolled Hayden in to the Technology Room with Mac and then we went on to the Playroom to check it out as well. The Playroom was closed, but our friend Loni the dog was hanging out there. It was then that Hayden decided he wanted to try walking so holding on to me, we got him out of the wheel chair and Hayden took two small steps on his own. It was so great to see the determination in his face. I was so proud of him.

The best was the last. Brought him back to the room and after a quick rest on the bed, Hayden announced that he wanted to try to walk again. This time we got him out of bed and he held onto my arms while he walked to the window and looked out. Its about 10ft from the window to the bed. It’s the first time he has been able to look out the window. And after about 5 minutes of checking out the view, he turned around and we walked back to bed. I swear it was just like watching your baby take their first steps. I was so happy I was crying. And when I told Hayden that I was crying because I was so happy and I got the biggest grin from him which made me cry even more. I’m surprised he didn’t roll his eyes at me!

That kid amazes me! Miracles can happen.

Wonderful Family

20th Annual Research Report from the Collaborative for FOP Research

John and I are very excited to share with you the 20th Annual Report for FOP Research.  We have made such strides and a potentail therapy is already being studied.  This is all because of your support for Hayden's Hope and our fundraisers over the last 8 years. Becuase of you we have raised almost $1million in research funds for FOP. So exciting! Below is the link to click on which will redirect you to the IFOPA website where you can down load the report.
http://www.ifopa.org/en/research/annual-reports.html

Top Ten Reasons You Know You've Been in the Hospital Too Long

Top Ten Reasons You Know You've Been in the Hospital Too Long:

10.) you are on a first name basis with all doctors and nurses in your unit....as well as the janitors and admin staff
9.) you feel totally comfortable silencing alarms on the various machines your child is hooked up too....he looks fine and is sleeping.
8.) you no longer hear the alarms on the machines your child is hooked up too because you've finally gotten 10 minutes of peace and are fully engrossed in a book that you haven't had a chance to read in a week.
7.) you feel completely comfortable barking at the resident during rounds (i.e. in front of 15 other doctors) because he does not communicate well.
6.) you feel completely comfortable complaining to the cheif of the department of said resident about his horrible communication skills
5.) you recieve apology from said resident and preach to him like his is a kindergartener about how good communication is the key to success in any profession. (Julie Althoff - Hayden's kindergarten teacher- would not approve of his commmunication skills either.)
4.) you start telling the nurses and doctors what to do and feel completely comfortable and commanding in your medical knowledge even though your degree is in Political Economies of Industrial Societies (but your sell textiles to the healthcare industry! )
3.) you forget what you say to who and when you said it
2.) you forget what day it is
and the top reason your know you've been in the hospital for too long is....

1.) you have run out of space on the walls to decorate and start decorating the ceiling!!! Hayden's Room Remodel Complete!

Potential Cure to FOP - Article in Nature Medicine

I am so excited to announce an article of great significance to the FOP community to be published today in the online addition of Nature Medicine. Below is a press release describing this exciting news. Look forward to more information regarding this release in the April 7th release of the 20th Annual Report of the FOP Collaborative Research Project, and also an editorial in Nature Medicine by Drs. Kaplan and Shore, which will be available on the IFOPA website.
Click here to read the press release
http://www.prnewswire.com/news-releases/potential-treatment-found-for-debilitating-bone-disease-in-wounded-soldiers-and-children-119146519.html

Exciting News!!!!!!!!!!!!!!!! Congratulations to our collaborative research team on this amazing work.a
 
As a supporter of Hayden's Hope, know that you are responsible for helping to make this happen!! 
 
Thank you!
 

Lots Of Helping Hands

The wonderful crew at Old Mill School (thanks Andrea, Nicole, Tracey & Danielle) has set up a website for Hayden on LotsaHelpingHands.com. If you are interested in joining the "Pheif Family Support" community. Click on the below link. If it asks you for a password, just click that you dont have one yet and a site administrator will approve you as a member. http://www.lotsahelpinghands.com/c/639072

Field trip to the hospital courtyard

Our plan today is to get Hayden out into this fabulous sunshine. The respiratory therapist is going to get a portable ventilator and Hayden will go in a wheelchair as he is too weak to walk. There is a courtyard that is about 50 yards away that gets great sun. I can't think of better medicine to get Hayden feeling better.

Three Steps Forward, Two Steps Back

Three steps forward, two steps back, one step forward, two steps back....I just wish I could put on my Ruby Slippers and click my heals together three times saying the magic words..."There's no place like home, I want Hayden to come home, I just want Hayden to get better and come home"...and the Good Witch of the West will waive her wand and magically transport us out of this dream and back home safe and well.

Get Well Cards from Hayden's 5th Grade Class and their special Kingergarten Buddies

Hayden got a great care package from Old Mill School today.  A bunch of get well cards made by his 5th grade class mates and their special Kindergarten buddies. We posted all of the awesome cards up in Hayden's room.

Hayden Fights Influenza and Pneumonia

Today is day 20 in the hospital.  Our odyssey started at Marin General Hospital on March 1st.  Sunday March 6th we went transferred to UCSF and on March 9th we made the difficult decision to put Hayden on a ventilator.  Although being on a ventilator is not fun in any way, Hayden keeps himself occupied by watching movies, occasionally playing Wii and computer,  but mostly lots and lots of sleep.  Hayden does lots of hard and often painful work on the ventilator to help the doctors clear his lungs.  He is very brave and a true champ. He is an inspiration to me.
A big thank you to the families at Old Mill School for providing wonderful dinners for John and I while we are at the hospital.  Also a big thank you to all of the warm wishes and get well cards sent to Hayden.  We have started decorating the room with them.

FOP, a very rare genetic disease, has only 700 known cases in the world.

Get involved at http://www.ifopa.org/

2011 Rare Disease Day

A disease or disorder is defined as rare in Europe when it affects less than 1 in 2000.


A disease or disorder is defined as rare in the USA if it affects fewer than 200,000 Americans at any given time.

One rare disease may affect only a handful of patients in the EU, and another touch as many as 245,000. In the European Union, as many as 30 million people alone may be affected by one of the 6000 to 8000 rare diseases existing. 80% of rare diseases have identified genetic origins whilst others are the result of infections (bacterial or viral), allergies and environmental causes, or are degenerative and proliferative. 50% of rare diseases touch children.
Characteristics of Rare Diseases
The 6000 to 8000 rare diseases are characterised by a broad diversity of disorders and symptoms that vary not only from disease to disease but also from patient to patient suffering from the same disease. Relatively common symptoms can hide underlying rare diseases leading to misdiagnosis and delaying treatment. Quintessentially disabling, the patients quality of life is affected by the lack or loss of autonomy due to the chronic, progressive, degenerative, and frequently life-threatening aspects of the disease. The fact that there are often no existing effective cures adds to the high level of pain and suffering endured by patients and their families.
Common Problems Faced
As aforementioned, due to the broad diversity of disorders and relatively common symptoms which can hide underlying rare diseases, initial misdiagnosis is common. In addition symptoms differ not only from disease to disease, but also from patient to patient suffering from the same disease. The lack of scientific knowledge and quality information on the disease often results in a delay in diagnosis. Also the need for appropriate quality healthcare engenders inequities and difficulties in access to treatment and care concluding in heavy social consequences for patients.
How can things change?
Although rare disease patients and their families face many challenges enormous progress is being made every day. The ongoing implementation of a better comprehensive approach to rare diseases has led to the development of appropriate public health policies. Important gains continue to be made with the increase of international cooperation in the field of clinical and scientific research as well as the sharing of scientific knowledge about all rare diseases, not only the most “recurrent” ones. Both of these advances have led to the development of new diagnostic and therapeutic procedures. However, the road ahead is long with much progress to be made.

Whant to get involved? click here

Happy New Year

Looking back on 2010 we are thankful for all of your ongoing support for FOP research.  Hayden's Hope Picture a Cure event brings a smile to me when I think about how many people we had attening (over 250) and the incredible amount of money that was donated just over $108,000. 

We are hopeful for a New Year filled with health, happiness and scientific breakthroughs that will propell our quest for a cure to FOP forward

Please browse our photos of the Picture A Cure fundriaser event by clicking here.

All the best for a wonderful 2011.

Sincerely,
The Pheif Family