All is Well

All is well.

My "Christian's" ankle bleed has finally resolved, a month later.  We have enjoyed our Christmas and are looking forward to the New Year as we begin with an inhibitor titer of 13!  The possibility of immune tolerance is actually within reach!

I hope that you are well, enjoying the holiday season and are looking forward to what 2012 may bring.

Blessings to you,

The Best Thing We Ever Did

I took "Lance" and "Christian" to the HTC in Denver this past week.

It was the best use of time and money I have ever put out.  My husband and I decided that the boys, especially Christian, needed another consult/opinion since our HTC in Albuquerque in undergoing many transitions right now.  With this inhibitor, Christian needs to be followed closely, so we finally were able to get up to Denver.

I have been involved with three previous HTC's since Lance was diagnosed and this I can't begin to tell you how amazing this visit was.  Complete, thorough, not rushed for time, beyond professional...and warm and welcoming.

Christian was fitted for a new brace for his ankle, and the Physical Therapist, Sharon Funk, was incredible!  I learned so much during the PT portion that it was almost overwhelming.

The amazing part of this trip is that Christian's inhibitor was tested.  The last time it was in the mid 40's...now it is…13!  I could hardly believe it!  Lucky 13!

I know that the "number" is not the most important part of dealing with an inhibitor, because it can consume you when the level goes up.  But you know what, we are celebrating!

This inhibitor battle began in 2006.  It's been a long haul, and it may continue to be quite some time before we get to "0", but it's the little successes that keep you going.

Big Improvements

It's so nice to see my "Christian" walking again.  He is turning in his ankle quite a bit and is moving pretty slow, but he is managing to get where he needs to go (but resting on his own as well).  He actually was able to put his tennis shoe on today!

It's the little things that mean a lot when healing from a bleed.

We go this week to Denver for a consult.  I am excited and nervous at the same time.  This has been a few months overdue.  I just hope we can get there without problems with the weather:)

Lucky Eleven!

Day eleven and my "Christian" is on his way home!

He took a few steps today, is on NovoSeven every 4 hours for a few more days and oral morphine every 6 hours.

Much progress has been made.

Tonight will be the first time my family has been all together at home in eleven, very long days.

Day Eight

"Christian" had a really good yesterday.  He was able to take a good bath (he was getting really ripe) played with his trucks (surrounded by them in his bed) and ate fairly well.  The big news is that he pooped!  Whoo Hoo!   Now, if we can only get him to put a little pressure on his foot…

Today he will be off the continuous infusion of morphine and move to oral morphine.  The only problem last night was that his oxygen dipped down into the 70's so they put his nasal cannula back on.  The hope is that he is discharged Monday or Tuesday.

I am so glad we received our Cryo-Cuff!  Our homecare sent one to us, here at the hospital, and I am putting it on him regularly to help speed along this last part of his recovery.  It's a great device to have on hand.  Talk to your homecare about obtaining one.  Insurance may pay for it, but if not, it is well worth the investment.

The best part of today is that his dad and brother will be here later!  We have missed them terribly.

The waiting, and healing, continues...

Day Six

We are in day six of our hospital stay and there is finally a glimmer of hope.  "Christian" extended his leg almost completely!  His ankle is still swollen but it finally seems to be improving.  He is starting to eat but the continuous morphine is still running.  The doctor backed off on it a little bit last night and I am hoping tomorrow they will consider backing off some more.  This morphine causes crazy itching!  Christian is scratching himself and leaving marks on his little body.  He has been on a regular schedule of Vistaril to help alleviate the itching but I went out to Walgreens today and bought some Aveeno anti-itch cream.  It has worked beautifully!  Great stuff :)

This has been the worst bleed we have encountered.  Seeing your child in pain is heart breaking.  It was to the point where I did not think he would ever get any relief.  Feeling helpless is awful.  It's enough to take away your self esteem and begin to doubt what you know about caring for your child.

That's when you have to dig down deep and have faith.

Still Here

We are still in the hospital.  "Christian's" ankle bleed is not resolved and he is still in a great deal of pain.  He is on a continuous infusion of morphine and a 3 mg dose of NovoSeven every three hours.  He's only had three bites of cereal (very early this morning) in a few days.

I am really worried about him.  He even has oxygen running by his face because his oxygen levels tend to go down on the morphine. 

I am taking "Lance" to the airport this morning so that he can be with our family for his grandmother's viewing and funeral.  I have to make sure Christian is healing.  That is what my mother-in-law would have insisted I do.

A Doozie

This ankle bleed has been one of the worst yet.  "Christian" has been on morphine every two hours today and with the half life of the morphine being so short, his pain goes away for a very brief time then he is moaning in pain until his next dose.

I hate that I can't take his pain away.

The swelling is not going down as much as I would like to see.  This blasted inhibitor does not make these things any easier.  I think tomorrow we need to talk about trying a dose of FEIBA. 

I just can't miss the funeral on Wednesday...but things are not looking like my little guy will be able to travel.

A lot can happen overnight.  I am trying to stay positive.

Never a Good Time

Bleeds never happen at very good times.

When you are walking out the door….

On test day….

Right after you tell the HTC there have been no bleeds for months…

2 am…

Last night, my "Christian" started complaining that his ankle was hurting, sure enough the entire night was filled with pain and constant infusing.

Then we got one of those phone calls that haunt you forever...word that my mother-in-law passed away.

Life goes on.  Bleed or no bleed.  It just what we have to deal with.

There's no sense getting upset about a bleed...we just have to treat the bleed and deal with the other stuff around us as it comes (not always as easy as that just sounded).

My mother-in-law adored my children.  She wasn't scared of hemophilia.  She embraced it.  She saw past the bleeding disorder and looked directly into the souls of my children and loved them like only a grandparent can love a grandchild.  They are going to miss her hugs and visits...and suitcase surprises. 

I miss her terribly already.

Appointment is Set!

I made a few calls, talked to someone I knew at the Denver HTC and we have an appointment for "Christian" on December 14th. I am so relieved that he will be seen sooner rather than later. He is having a bleed in his hand that is taking some time to heal, but his ankle is doing much better.

His wheelchair sits in the office, ready when needed, and with an appointment on the books, I feel some relief. I am looking forward to hearing what Dr. Manco-Johnson will have to say. Will we do another round of Rituxin? Start the Cellcept again? Or simply wait and treat with Novo daily until the inhibitor comes down?

I am starting to make my list of questions for the appointment. I want to be as prepared as I possibly can. Is that really possible? So many things to think about ahead of time….

A Huge Realization

It was great to be back at the NHF Annual Meeting this year. We were not able to attend last year, so we have really been looking forward to Chicago. We had a great time, did lots of walking and enjoyed catching up with old friends and making new ones.

My husband and I had a huge realization. We were knocked out of complacency with our little "Christian." His target ankle is just in bad shape. He struggled with endurance and getting through the airports on the way home was really difficult for him.

When you are in your familiar surroundings, home, school, church, etc. things look a certain way. Then when you get out into the great, big world, sometimes things are more apparent. It is critical that we get "Christian" to the Denver HTC for an appointment. We were hoping to wait until January, but it is just not in the best interest of our son. I am waiting on a call this afternoon to confirm our appointment for December 14th.

I feel so alone with our HTC in Albuquerque being in such a big mess. Lack of staff and passion for the program is affecting our state in a huge way. Thankfully we have a nurse with tons of passion holding down the fort, but you never know what you are going to get in the way of physicians and it is scary.

I am writing my questions down and preparing for our visit in Denver.

In one of the sessions this past weekend, a mom who has a son with an inhibitor said, "Having an inhibitor is like hemophilia on steroids." That made a lot of sense to me. I just realized how specialized our situation with inhibitors truly is.

All I can do is keep in mind that my little guy struggles more than most. I need to not get frustrated and at the same time not coddle him.

That is what is truly difficult.

It Never Fails

Our family is preparing to travel to Chicago for the National Hemophilia Foundation's Annual Meeting. And what happens? "Christian" is having a bleed in his left foot and is starting a nasty cough. It never fails that something happens as we are about to leave town.

But we do the best we can and move through it. It may take some extra infusing and cough medicines, but we forge ahead.

This is a huge opportunity for my children to be involved with others who have the same struggles and make friends in the community.

If you ever have the opportunity to attend the NHF Conference, go! It is a wonderful experience for the entre family.

I'm Just Amazed

I have a Facebook account. I normally check in during the evenings, but sometimes I get messages during the day when someone posts to a couple of the groups I belong to. One of those groups is "Living With Hemophilia and Inhibitors." I was reading through a few posts the other day and was just blown away at how amazingly smart these caregivers are! When you look at the comments, often you would think that "these must be healthcare professionals."

No, most of us who parent children with a bleeding disorder are not clinicians, but we do know what is best for our children. Sometimes it's that good old "intuition" that kicks in and leads us to making the best decisions about the care of our children. I tend to believe that God has a big hand in the "intuition" department, but call it what you like, it's an important part of caring for our children.

I know, I know....

I should never say this aloud or even write it, but hemophilia has really been behaving in our house lately.

"Lance" had a bleed around his knee (soft tissue) several weeks ago and it was a wake up call for him to get back to his regular infusing schedule without his Dad and I hounding him constantly. Sometimes that's what it takes.

"Christian" has the "normal" bruising, but all in all is fairly "clean", with the exception of the big bruise on his chin (who knows what he did to get that one:)

I even caught myself wondering when the last ankle bleed was for Christian. For some time it had been a weekly thing for him as he struggled with his target right ankle, but all is calm for now.

I am so grateful.

Grateful that we have plenty of factor.

Grateful that we have a fantastic homecare.

Grateful that Christian's port is "flushing beautifully."

Gratfeful that hemophilia is manageable unlike so many other disease states.

I

If this is what we have to deal with, so be it. I am just grateful beyond words that I have been blessed with two beautiful boys.

The School Nurse

Yes, I think my "Christian" has become a regular at the school nurse. Part of the problem is that his teachers are still pretty nervous about hemophilia. When in doubt, off to the clinic! Don't get me wrong, I would much rather he go to the clinic than have an injury overlooked. But, yes, I am getting a few calls weekly and go up to the school at least once during the week to check on him.

I am a little worried that he might think that going to the clinic is a "treat." I've already been telling him to "stay in class and not go to the clinic all of the time." I think he gets it, he's just excited about everything at school.

Have you had issues with your child at the school clinic?

Change

I don't like change. It is inevitable. But I still don't like it. We are having some pretty major issues with the deterioration of our current Hemophilia Treatment Center. It is very scary, especially when the next closest one is at least six hours away. But sometimes the decisions that are most difficult are the most important and extremely necessary.

I need a hematologist that is experienced with inhibitors monitoring my "Christian." Any hematologist will not do. That was an important piece of advice I picked up at the Inhibitor Summit this past summer. I can't keep my head in the sand and think that overnight everything will be in place at the treatment center. It will take time.

No matter what it takes...traveling out of state, making phone call after phone call to follow up, driving a long distance for routine blood work...I have to make sure my son is being monitored as best as possible.

Beads

My "Christian" brought me a surprise from school...a pipe cleaner bracelet with five beads. I wear it proudly. Not just because my sweet boy made it for me, but because a year ago he could have beaded this pipecleaner. The nerve damage done by the first PICC line attempt was severe. It took many, many months before he could touch his index finger to his thumb. I am stepping back and taking joy in the little things...assisting with kindergarten homework, cleaning the kitchen after a satisfying meal, and little beaded bracelets.

Why in the world....

Why in the world am I saying this, but since we bumped up "Christian's" daily dose of NovoSeven from 2 mgs to 3 mgs, things have been really good. Even when his tooth fell out recently, there was not any blood. Maybe I am over reacting a bit by thinking that the boost of his factor has been that big of a deal, but maybe it truly has helped to even him out. Christian has been through so much and a few good weeks of being a typical boy has been really nice.

We will take it when we can :)

The Tooth

As the mother of a child with hemophilia, you always dread the call from the nurse at school. What in the world happened today?! Friday afternoon, with an hour of school left, I received that call. Actually, it was the school secretary (she steps in when the nurse is away from the clinic). My "Christian" was enjoying his afternoon snack and when he bit into his orange, his tooth fell out! There was not any blood and all seemed well, but I told the secretary to let the teacher know I was on my way to make sure everything was okay (mainly to ease their minds….they are still getting familiar with hemophilia). I walk in and here comes the teacher at the end of the hallway with Christian and his buddy "Jason". They were coming from outside. They were looking for his tooth that he dropped on the playground! Like a needle in a haystack :)

That sweet teacher went out of her way, and into the afternoon heat, to try and help my boy find his little tooth (so completely not in her job description). My heart was overwhelmed with that act of kindness. I reassured Christian that we would leave a note for the tooth fairy and all would be well (the tooth fairy did stop by the next evening).

I give thanks to the teachers who teach my children. From learning to sit and focus on an activity for a few minutes to conjugating the newest Spanish verb, I don't know what I would do without these men and women.

Teachers are not paid enough and not respected enough. We are surrounded by people who are giving information and pushing us to believe a certain way. But some of our biggest heroes are the men and women in classrooms day in and day out. Working after the last bell rings for the day and even on the weekends. Paying for supplies out of their own pockets and making sure every child understands a new concept. Going the extra mile to make sure each child feels special.

A great day...until we came home

We have had a fantastic weekend! Getting things done around the house, spending time together as a family, eating some great, home-cooked meals and we went on a picnic in the mountains today (high of 62 degrees).

Just to come home with a bleed. I thought his ankle had swollen up due to walking around in the creekbed (it sure felt nice) but it also looks like a bug bite may have been the culprit. Who knows …… the reality is that "Christian" is having an ankle bleed. Here we go every three hours :(

A Big Difference

Before our trip to Florida, "Christian" was having a few issues and we ended up bumping up his dose of Novo from 2 mgs to 3 mgs. It seems to have really made a difference. Our fantastic Walgreens pharmacist is the one who brought up the dosing change. Apparently the European dosing is different than here in the United States, and it is being adapted more and more here. We talked to the hematologist (who is trying to hold things together through some pretty big growing pains at our HTC) and he was very agreeable.

I hate to even think about the fact that Christian has been doing so well, but he really has been! I am looking forward to his next inhibitor draw. It would sure be nice to see his numbers continue to go down.

It's not about the numbers, it's about living life to the fullest each day (but starting immune tolerance would sure be nice) :) :)

When You Wish...part 2

We are some tired puppies! Day two of theme parks completed! I was worried about "Christian" keeping up with his ankle problems, but so far so good. Roller coasters, simulated rides, Hogwarts...we are having a great time:) We sat down to dinner tonight (at the Give the Kids the World Village) and a huge revelation came over me. My family's biggest worry is how to get in all of the rides and attractions we want to see in the next few days (while avoiding the possible hurricane) and sitting next to us at dinner is a family with a child who is a parapalegic. Another child in the dining room is wearing a leg brace with a strap around her waist for someone to assist her as she walks and other children are wearing caps over their hairless little heads. They have and are continuing to struggle.

Yes, my Christian and Lance have their struggles too, but I will take our struggles any day and make the best of what each day brings.

Each member of my family can walk, sweat and keep up with each other while enjoying ourselves. And when we need to slow down, we do. We love each other like no one else possibly could. This "wish" trip has given me the opportunity to see true, pure joy on the faces of my children. That is a huge gift. It is something I haven't slowed down enough to see lately...and I am grateful.

Today I Am Thankful For:

1. Rain
2. Soft, chocolate chip cookies
3. A racing heart (after a thrill ride)
4. Bright colors
5. Swimming pools

When You Wish...Day 1

Did you ever wish upon a star? Maybe you looked up in the night sky, saw that special twinkle in the night, and wished for something...to be famous, for a new television, to have a talent like none other.

Sometimes our wishes are big (maybe they even qualify for big dreams) and other times they are simple and unassuming.

Children with chronic illnesses struggle in so many ways and there is an organization called "Make a Wish" that helps to grant these children's wishes.

My "Christian" was referred by a member of our hemophilia treatment center and we are now in our "villa" at the "Give Kids the World" resort in Kissimmee, Florida. It is absolutely unbelievable. Not just the fact that our family is here, but the love that has been shown to us by complete strangers. At this resort, you are treated like kings and queens and for a moment in time, your child is "given the world" and in this case, "the world of Disney."

Some of these families have never been on this kind of trip and would possibly never have the opportunity to take this kind of trip. You have absolutely no worries about getting from one place to the other, food, entertainment, the whole bit.

Another amazing thing is that is takes over 1,500 volunteer time slots for one week to make the "GKTW" Village run. These people are giving their time to make our family have an experience we will never forget.

Sometimes it's just too much to accept and let sink in.
>

Maybe a Little Normalcy?

When you live with a bleeding disorder, is there ever "normal"? I mean, the minute you think about life going along without issue, a bleed happens, the port stops working, you run out of syringes…..etc.

"Christian" is recovering from a bleed in his foot and leg, we accessed his new port yesterday and it is working beautifully and he went back to the sitter's house today and played hard. My "Lance" is doing well without bleeding issues (if I can only get him to infuse regularly without my nagging….). We even have a new dog in the family and she fits in as if she has been with us forever.


We are looking forward to our Disneyworld Make-a-Wish trip in 12 days and are hoping to just spend some quality, family time together without work worries and cell phones. I can't wait.

The Saga Continues

On Tuesday evening, the second IV blew.

It took six sticks/pokes to start a new IV. My "Christian" broke out because of nerves. He was so upset. I can't believe I had left. My husband was there to take over, and I hate to say it, but part of me was relieved I didn't see all of that. I had been with Christian on the previous Thursday when we access his old port several times and it was horrible.

Sometimes you have to rely on others to help. I don't know what I would do without my husband. How people survive as single parents is completely beyond me and single parents with children that have a chronic condition? I don't know if I could do it. I admire them for what they do.

Today Christian developed a bleed on the top of his foot from one of the IV tries. My husband tried to keep him calm, as calm as you can keep a five year old, so that the bleed would start to resolve.

The other good thing is that the nurse accessed the new port tonight! It's working great! And his inhibitor is down to 34!

I just hope that the bleed is under control so that he can come home tomorrow as scheduled.

It's been a rough week, but my Christian is one tough cookie.

Precautions

My "Christian" is doing very well. We have even move to every 12 hour factor infusions. His incision is healing and all looks great. The only problem is that he must keep an IV in until Friday. His veins are pretty torn up and traveling home, two hours away, poses some concern. In our little town we do not have a lot of back up with nursing and are afraid to depend on one person (the nurse that helps us out is not always available).

The team decided it was in our best interest to stay in the hospital until we can access the new port on Friday. I know it is the best decision, but it sure stinks to live in the hospital. I knew the minute we would leave and get home that the IV would blow and have to turn right around and come back.

All in the name of what is best for my son.

Port #4

Surgery went very well. The hardest part this time was "Christian" coming out of the anesthesia. He was extremely agitated and would not stop crying. Finally, after some more pain meds and Benadryl, things calmed down and we made it out of recovery.

I think the hardest thing about the hospital is entertaining your child:) We are so grateful that the University of New Mexico has such a wonderful Child Life program. They are just fantastic!

A second IV did need to be started on Saturday because the first one became painful when infusing. My Christian is horribly afraid of needles and starting IV's are never an easy task. He did do a great job.

Now we wait to see if we get to go home on Monday or not. The HTC does not want a new port to be accessed until a week following surgery, so we would have to go home with an IV. His veins are pretty torn up from previous sticks, so depending on a peripheral stick in the case of a blown IV is slim. We may have to stay the week...we'll just have to see. He must have his factor on a regular post-op schedule, so we may not have a choice of going home just yet.

I'll admit, I've been pretty upset and wondering "why" my sweet son has to deal with hemophilia and his crazy inhibitor. But then, reality hit me upside the head. The same day we had to endure a second IV, a child on our floor passed away from a long illness.


Whatever I have to help my son deal with is just fine. I'm just so incredibly fortunate to have him in my life, hemophilia and all.

The Hardest Day Ever

My day with "Christian" looked like this…..

Left at 6:30 am for the Hemophilia Treatment Center (2 hour drive to Albuquerque)

9:00 am appointment

The hematologist accessed Christian's port and we thought all was well (I was not able to access it the night before and my husband is out of town…..I had no back up)

When the hematologist left the room, I mixed factor to infuse before we headed home….the port would not work.

Between me and the hematologist, we attempted to access the port at least 8 times. No luck.

The screaming was from deep down….Christian has an intense fear of needles. A boy with hemophilia that has a fear of needles is not an easy situation. I had to help hold him while the doctor was manipulating the port and every time I held time down, a piece of my heart broke. The shuddering took over his entire body...the tears flowing like a river. His cheeks were bright red, and there was nothing I could do to take away the pain.

The x-ray showed the port at a severe angle and we thought we had it figured out.

No luck.

An IV was started, because we had to get factor in Christian. He has a high titer inhibitor and needed to be infused.

3:15 pm the decision was made to replace the port. (This is Christian's third port).

We were allowed to leave the hospital, drive back home (2 hours), packed our bag, took the dog to the dog sitter, went by the church to get a couple of things and fold bulletins for Sunday morning (because hubby is out of town) and then me, Christian and Lance hit the road back to Albuquerque (another 2 hours).

9:20 pm arrived at hospital and waited for room to get ready. While waiting, I mixed factor to infuse, and the IV blew.

Could the day get any better?

9:50 pm our room was ready, my husband walked in, we were all together and a specialty nurse came up to our floor to start a new IV (two sticks).

Now I am listening to the beep of monitors, looking out an all too familiar window, while smelling "hospital." I am comforted by the familiar faces of the nursing staff, and even the workers in the café downstairs that have become friendly to me in the wee hours of the morning.


I am thankful for the familiarity, the comfort in knowing my child is in great hands, but I have yet to truly "feel" the events of the day. I am little scared to let myself completely give in to what happened today.

A Very Stressful Trip

We traveled to the Inhibitor Summit last week in San Francisco. It was probably one of the most stressful trips we have ever been on. "Christian" was having a slight soft tissue bleed in his hand as we were leaving town, our treatment center was not being very helpful, and when we called Friday evening, the doctor on call told my husband, "looks like you all made some bad choices." You don't tell that to a family living with a chronic condition, especially during an active bleed while out of town! I took Christian to the ER in San Francisco and 7 hours later was told we were doing the right things. Sunday morning, at breakfast on the bay, we received a call from our dog sitter that my sweet Sheltie, Rosie, died.

Could this trip get any better?

Monday was filled with phone calls to the treatment center for detailed information so that we would file our grievance with the doctor who was out of line and we also made calls to the Denver treatment center for a second opinion.

A very stressful Monday, but what I have learned out of this summit was to not be complacent in the treatment of my Christian's hemophilia (with an inhibitor). I need a hematologist who knows what they are doing in the area of inhibitors. Being "monitored" by a group of doctors who do not necessarily specialize in inhibitors is not good enough.

I will be the biggest advocate possible for my son. I don't care what it takes, I will speak up for him and my family and refuse to be treated badly. This doctor will not forget us, because I will make sure he knows that unless he lives with a severe, chronic bleeding disorder, he can never understand how we feel. He can never understand the decisions we make, but he will know that we make the decisions for our child and family that best benefit us as a unified group. We know what is best as parents.

If you have experienced a joint bleed, read on....

I received this email and wanted to pass it along. If you have personally experienced a joint bleed, this group is looking for you.

It sounds like a worthwhile project.

EMAIL
Hi!
I am interested in understanding more about the experiences of hemophilia. My company is currently doing research on the sensations of joint bleeds and the experiences that patients go through. We are currently developing a simulator for one of the major pharmaceutical companies in the United States. We want to help doctors better understand what their patients experience, not just what they read in textbooks. I'd be interested to know more from the source. If you are interested or anyone you might know, please contact me via email abond@gwfassociates.com; we are located in NJ. Thanks for blogging as well, it brings the disease closer to home and helps people like myself who are not closely familiar have a better understanding.

Thanks,
Ann Marie

Best Regards,
Ann Marie Bond

GWF Associates, LLC
1 Sheila Drive
Tinton Falls, NJ 07724
732.933-8780 (t)
732.933.8783 (f)
abond@gwfassociates.com

ATTACHED LETTER

July 5, 2011

Dear Patient,

Permit me to introduce you to GWF Associates LLC, a multimedia education company that has been in engaged in teaching physicians and other healthcare providers since 1974. We have developed programs for diverse Medical Society Congresses that have been shown in 23 countries and translated into 13 languages.

Our current project is to help physicians better understand the challenges to their patients with Hemophilia and we are developing a “virtual experience” in which physicians briefly “become” a patient with Hemophilia. This educational experience includes simulation of the sensation experienced during a joint bleed. We are inviting you to participate in a brief discussion about challenges faced by hemophilia patients, with emphasis on the sensations that occur during a joint bleed. It will take less than an hour and you will receive a stipend of $150.00 plus any travel expenses to our Tinton Falls office.

Importantly, you will be helping doctors to better understand the challenges to daily life that individuals with hemophilia encounter. Experiential learning takes physicians beyond what they read in a text book to actually “feel” the restriction imposed by their condition.

The following is our address and contact information:
GWF Associates LLC
1 Sheila Drive
Tinton Falls, NJ 07724
732-933-8780
morsetti@gwfassociates.com

We look forward to hearing from you.

Best regards,


Marian Orsetti

Senior Medical Writer

The Fun Begins

We started our journey with Rituximab (an immunosuppresant) in the hopes of eradicating Christian's inhibitor, back in the fall of 2009.

I just received a letter from the insurance company stating that our treatment (Rituximab) in January of 2011 has been denied.

The fun begins.

Happy 4th! ? !

The last two "July 4th's", my "Christian has been in the hospital getting a new port. He is on his third, so far so good. While there on the 4th, we have watched the fireworks from his hospital window...a fantastic show in Albuquerque.

We were so glad that this year we would actually be home to watch fireworks!

Saturday morning my husband, out loud, made the comment about not being in the hospital on the 4th. Less than an hour later, our hematologist called and said that Christian's port culture from Monday (he went in for an MRI) grew positive!

Thankfully we did not have to go in, because he is not showing symptoms of fever or any kind of side effects from an infection. She thinks it may just be a topical contaminant kind of thing. We went on Saturday to the local hospital to do another culture, so we wait.

You just never know when something is going to come up. You have to be prepared for anything when you have a child with a bleeding disorder. Automatically the plans you had start to change and your mind begins to race. Sometimes, you have to change and go along with what needs to be done. Other times you may luck out and not have to race to the hospital or clinic.

You have to have a little humor throughout this process. Not that a bleeding disorder is something to joke about, but as a parent we have to learn to handle situations with grace, calmness, a positive attitude, and know that our children are always watching how we react.

I Knew it Was Worse

"Christian's" ankle has not been right. As he has been playing tee ball, it has been extremely obvious that he can't run like the other kids. It has just broken my heart. He doesn't seem bothered by it, but the truth is that he can't keep up.

I insisted on a new MRI and I am so glad I did.

He has joint damage from repeated bleeds into his right ankle, but the MRI showed significant change from his last MRI a year ago to the "talus" bone in his foot. Now we know why he has changed his gait and protects his foot.

The good news is that it can heal, the bad news is that tee ball and soccer are out of the question, at least for now. We have signed him up for swim lessons and hope that he loves it.

I almost ignored the problem and hoped it would just go away on its own. But deep down I knew it was something more. The doctor even admitted that he wasn't expecting anything to show up except a possible bleed, and was very surprised.

You have to go with your instinct. Sometimes it makes absolutely no sense, especially if a doctor is telling you otherwise. The "professionals" do not always have it right. When you are the parent of a child with a chronic condition, you are the ultimate expert! Don't forget that. Speak up! Be heard! Make a little noise if you have to!

The bottom line is that you are advocating for your child.

That is reason enough to be the squeaky wheel.

Different

My "Christian" is in his second week of tee ball. He doesn't really know what he's doing yet, but is having a great time :) Tonight at practice, my husband said it was really evident that Christian was having trouble with his ankle (target joint).

When he runs he has a little "hop" to compensate due to all of the pain he has been through with that ankle. He's the last one to come in when they run the bases and it's just obvious that something isn't right.

He is scheduled for an MRI on Monday to check and see what kind of damage has been done. I'm a little afraid of what we may find. I know something is wrong, and it may be in the very early stages, but my gut just tells me to start getting prepared.

Our first stop tomorrow is the city pool.

The First Practice

"Christian" had his first tee ball practice last night. I was totally fine with the whole idea until I started to see the wild throws those little kids make! I was pretty nervous. He was a little clueless, but started to get the hang of it.

His coach is a young woman who has an amazing amount of patience. God bless her! She was so caring and loving with the kids, that it makes my nervousness all the worth while.

Truth of the matter is that my boy is a "social butterfly" and needs that environment...even if wild balls are whizzing around. I have factor with me at all times :)

Trusting through Transition

My "Lance" is fourteen. He will be fifteen on the 23rd! I don't know where the time has gone, but before I know it, he will be on his way to college.

Over the past few years my husband and I have been working on him taking responsibility for his bleeding disorder. He keeps track of his supplies and orders his factor every month. Here lately he's been slipping on infusing regularly. It often takes more than a "gentle" reminder.

Lance is part of a group of boys that have not experienced joint bleeds. I am so incredibly grateful for that, but it also leads to complacency on his part. You would think that since he has seen the extremely painful bleeds his little brother has gone through that it would encourage him to keep to his infusion schedule….well, it's a nice thought.

He has been in California this week on choir tour and I have to trust that he infused according to schedule.

Letting go is not easy. We just have to make sure we give our children the tools they need when they leave us.

Full Circle

I love seeing things come "full circle." You know, when something generous or kind comes back to you.

I have a sweet friend in Texas, "M". When her son was diagnosed with hemophilia, she reached out to me and my husband and we became regulars in their home. Those bleeds early on for her son would bring us to her home for support. I did the best I could to reassure her that hemophilia could be dealt with and that her son could live a very full life. He is now a very active baseball player with no joint bleeds to date! M and her husband are avid workers in the local hemophilia community and have helped numerous people in their same circumstances.

Knowing they are big baseball folks, I called her and asked her advice on tee-ball. I am clueless when it comes to sports and my "Christian" is going to be a big sports kid. She talked me through the basics and I feel much better.

She came back and shared her experience with me, reassured me, and left me feeling as if I was not alone.

I think that is what it is all about in this community. Not feeling alone.

We must reach out to others. We need to stay "in the loop" with our local chapters and meet others who may need our help, but also meet those who may help us when we least expect it.

Occupational Therapy

Last summer my Christian's body rejected his 2nd port. It was pretty awful. The port pushed its way out of his chest. It was removed and we had to pack the open wound and let it heal before the 3rd port was placed. This was the second time I ever felt as if I was going to faint. I didn't think I would be able to do it, but when your child has a hole in his chest and the only way to care for it is to clean and dress it, you dig down deep inside and just do it.



Before his 3rd port was placed, the doctor recommended a PICC line be inserted while the wound healed. It was an awful experience. Christian was having a bleed in his left arm, so the doctor attempted to place the PICC in the right arm. Nerves were hit and major damage was done to his arm. The doctor placed the PICC in the left arm and all was well, we thought.


Christian endured pain for the next few months. He could not use his index finger and thumb and was in constant pain. It was just awful. His saving grace was occupational therapy and Mr. Bob. It was very tedious work, but Mr. Bob made his therapy fun (actually I'm not sure who enjoyed it more, Christian or Mr. Bob.). By the end of the school year, Christian was finally able to use his fingers without pain. Honestly, I thought therapy would be a waste of time, but is was a huge blessing.

The Brace

My "Christian's" ankle is not in great shape. It's a target joint and has continued to give him problems. I am noticing that when he runs he adds a skip into his gait. Without his brace, he turns his ankle to 90 degrees. He's protecting it. Without knowing he is doing it, he protects his ankle so that he doesn't endure the horrible pain he has had in the past.

Am I doing the right thing letting him playing tee ball? I am really starting to question myself.

Christian was fitted for a brace to support his ankle several months ago. It has really helped him out. His hematologist was worried that Christian might even develop a bleed in his hip because he was over compensating for his ankle. I have to let him take a break from his brace occasionally because it rubs against his leg and it gets hot. I also hate that it makes him "look" different.


It just breaks my heart.

I am waiting on a call to have an MRI taken as well as an initial consultation so that he can start physical therapy. With his inhibitor, we just have to keep on going with the hopes of getting on Factor VIII soon. At a fellow hemophilia mom's persuasion, I am going to insist that we have regular MRI's to keep tabs on any damage that is being done.

As a former colleague of mine used to say, "we'll just keep on keeping on."

How Many Medic Alert's Have You Purchased?

When you have a medical condition, it is imperative that you wear a Medical Alert type of emblem. You can find them at the drugstore or by visiting www.medicalert.org.

When my oldest was little, I would put his medical bracelet on his backpack, then his shoelaces. Now you can even get medic alerts made to go on the shoelaces. He has tried the sportsbands and the necklaces, but the best bet, for him, has been the dog tag style.

Have I purchased more than one...let's just say that I have lost count.

I just left the Medic Alert website and ordered yet, another dog tag. My son is going on tour with the Albuquerque Boy Choir in a couple of weeks and he must wear his identification. He is going to be away from home for a week and I want to make sure he is doing everything possible to keep himself safe.

The crazy thing is that we think horrible things like a car accident might happen when our children may be away from us, but I have heard more than once that some of the most devastating accidents happen within a few miles from home.

You never know when something might happen. Maybe not to your child, but to you, and you will not be able to be your child's voice. Do everything you can to protect your children. Insist that they wear their medical alert identification.

You just never know.

To Play Ball or Not to Play Ball?

My "Christian" is the kind of boy who is going to love being on a team. He is "made" for sports. I am afraid the day will come when I am having to direct him to another sport other than football. He's just that way.

I've not had experience with the "sports" thing because my oldest is a creative type. He did play some soccer, but it was not his thing. We sure did have fun while it lasted. I really enjoyed going and sitting under the trees with the mountains in the background watching the kids play.

Christian recently went to a short soccer workshop. He absolutely loved it! He didn't really know what he was doing, but he had a great time. We signed him up for a week long soccer camp in July.

Tomorrow is tee ball sign up. My husband is nervous about it.

How dangerous can five year old tee ball be? Do we take the chance and let him run his little heart out and build his social skills, or do we hold him back? Do we let the fear of his target joint keep him from participating, or do we infuse before he plays and watch over him?

I just can't see holding him back.

Yes, we are going to have to "steer" him to certain activities to make sure he is safe.

Boys will be boys, despite hemophilia. My job is to encourage my children and let them experience life fully.

The Inhibitor Journey

My "Christian", who is five, has a high titer inhibitor. When he was first diagnosed, during his circumcision at 11 months of age, the inhibitor registered over 2,200 Bethesda units! It's now down to 41.

It's been a long journey, and the journey is not close to over.

I remember attending an NHF rap session for parents of children with inhibitors a few years ago. There was a parent talking about a drug, Rituximab, that is a chemotherapy drug being used to help eradicate inhibitors. I thought she was completely crazy! How could you give that kind of drug to your child without knowing for sure the track record it had?

Boy did I eat my words.

It ended up being an avenue we decided to take. Christian started a protocol of Ritximab and Cytoxan in September of 2009. It's not been easy, but it has produced some really great results.

But one thing I learned from a new friend of mine (that same mom I heard back at NHF) is that I need to let go of the numbers. If I continue to measure everything based on the inhibitor level, I'll not only make myself a little crazy, but we will forget to live life and take each day as it comes and let my son be the five year old that he is.

It's all about living with the inhibitor one day at a time.

The inhibitor may go away in a few months, a few more years or maybe never. We just don't know. What I do know, is that we have product that helps with his bleeds and a great team of clinicians that help us cross each bridge as we get there.

Ankles

I just did a four day stint in the hospital with my "Christian." He had an extremely bad ankle bleed. It's his target joint. He stopped walking on Monday, May 9th and finally walked on his own on Sunday, May 15th. It was very painful. When you watch your child screaming in pain, despite pain medication, it just breaks your heart.

Something happened over the past few days.

My eyes opened.

Yes, I have accepted that my boys have hemophilia but when you have great treatment you just kind of go with the flow and become complacent. Over the past couple of days I have watched my Christian struggle to walk and it hit me like a ton of bricks.

I have a five year old with a bad ankle.

It kind of puts things into perspective.

It makes bickering with family, wanting to "fix" other people and feeling sorry for yourself kind of pale in comparison.

Is Hemophilia Ever "Easy?"

My initial thought is "NEVER!" But when you have more than one child with the same bleeding disorder (and severity), you see the differences. Sometimes one brother's hemophilia is easier to manage than the other's.

It doesn't make either one more or less important because the bottom line is, they both have a severe bleeding disorder. A condition they will live with the rest of their lives.

I don't want to make light of anyone's journey with a bleeding disorder. It is real for each person, difficult and demanding in its own way.

If I had to use one word to describe hemophilia for my oldest son, "Lance," it would be easy. For my little "Christian," heartbreaking.

Lance had his first bleed (soft tissue in his right hand) at 6 months of age, several bruises, a low titer inhibitor that we immediately began immune tolerance the day his port was placed. His port lasted for five years with not one infection and he sat completely still during the access of his port, every day for 2 1/2 years of immune tolerance. He had a very difficult time transitioning from port to peripheral, but has never had a joint bleed. Just one pretty major thigh bleed 3 years ago and an appendectomy.

In the grand scheme of it all, his hemophilia has been easy to manage (if only he would infuse M-W-F without me nagging him to do it). The only reason I can say that is because of the experiences I have had over the past 5 years with my little son.

Does it take away the reality of hemophilia for my Lance? Absolutely not. Sometimes I worry about him more than Christian simply because I think he is pretty complacent with his disorder. My husband and I are as well. When treatment has gone so well, bleeds avoided and prophylaxis a reality in his life, we almost forget that he does have hemophilia.

It's one extreme to the other.

Day and Night

My boys are like day and night.

"Lance" is 14, creative, artistic, musical and cares nothing about sports (unless it's on the Wii).


"Christian" is 5, loves Thomas the Train, anything with wheels, being in the dirt and it looks like he will have a passion for sports.

Despite their differences and the many years between them, they do have one thing they share that will never go away...they both have Severe Hemophilia A.

On June 23, 1996 my life changed when I gave birth to Lance. Little did I know that on June 26, 1996 I would be devastated. Absolutely heartbroken. After his circumcision I held him and his little diaper became soaked in bright, red blood.

He was diagnosed with Severe Hemophilia A and I did not know if he would live or die.

After lots of education and getting hooked up with the Lone Star Chapter, my husband and I realized that Lance could have a normal, full life.