Wednesday, October 3, 2012

Dear Aven,
       It has been awhile since I have made a public post.  I have since then written you and your sisters a few letters and have made videos documenting our journey. (One of the videos I have been working on can be viewed here )
        So much has happened over the last month that I know I will forget to touch base on many things, so I will just focus on a few.  Probably the most important thing is that you are happy, doing extremely well, and are surrounded by so many people that care and love you. I wish I could blog about how you no longer need to take your enzymes nor that you have to do your treatments, but you know by now, that these are the things we have to do to keep you thriving.
         It has been very difficult to teach full time and to give you and your sisters as much attention as I would like and you need. I am very positive about the things to come and try to only give out information that I feel will bring awareness about cystic fibrosis.  I think your father and I are managing, but I will not lie, it is tough. I find myself crying every day on the drive home.   I do feel that you have the best possible set of parents to help you through this and I know that we will be there every step of the way.
          I finally did something the other night.....on September 28, 2012, I held your little hand and I looked into your innocent blue eyes and I told you that you have cystic fibrosis. It dawned on me that yes, you have been going to the doctors appointments and you have been the one that has been 'poked and proded', but everyone has just been talking around you.  I wanted to be the one to tell you. I explained to you in great detail all that I knew about the disease; the science behind the mutations, the discoveries that have been made over the past two decades, the treatments and medicines that you will have to endure on a daily basis, and even about a cousin that passed away due to CF.  I told you then and I will continue to remind you, that I will never know what it is that you are going through, but I do know that CF will never define who you are, it will only be a little piece of who you are; hence the name of this blog:  A Little Piece of Aven.
       I have had several people tell me that they love the name of the blog and liked how I played on the saying "A little piece of heaven."  When I created the title, that saying did cross my mind, but I knew from day one, that the real reason that I would entitle it this is just like I mentioned before,  CF will just be a little piece of who you are.
        Over the next couple of weeks we will be increasing your enzyme dosage, the amount of salt you take in on a daily basis and the length of time with your percussive therapy.  You had a good check-up on September 21, which resulted in no new bacteria growth, which means no anti-biotics.  We will head back to see your CF team on November 1.   You will also be in calendar to promote CF awareness and to raise funds for the Cystic Fibrosis Foundation. Hope to post pictures real soon.
          I will try to post a little more next time, until then......

                                                                      Much Love, Today, Tomorrow, and Always,
                                                                                       Your Mother Autumn


This is a picture that I took of you a couple of weeks ago.  I just love this!
This is Aven! This is who you are!

Saturday, September 1, 2012

Dear Aven,
    It has been challenging for me to sit down and find the time to update the blog.  Once school starts, I am pretty much at a stand still as far as leisurely activities go.  I do not know at the time that you read this if I will still be in the classroom, but currently I am in my 15th  year of teaching general and AP (advanced placement) chemistry courses at Carthage Senior High School.  Carthage is so very dear to me because I have lived her my entire life and I feel very fortunate to teach in a district that I feel provided me with a wonderful education.
      When you first received your diagnosis, there were so many things running through my mind, and yes, a big concern was whether or not I would be able to continue teaching.  As of now,  I am still in the classroom, but I do feel that my days at Carthage may be numbered, simply because I want to spend more time with you and your sisters.  And if the going gets tough for you, I want to be there beside you every step of the way. I have also looked into the best places to live if you have a CF diagnosis....the feelings amongst the CF community is that lower elevations work best as well as places near the ocean due to the salty air.
       You just finished a 14 day anti-biotic because of a throat culture that tested positive for staph. You were a little trooper as always, but you have this past week suffered from blockage of the bowel.  You saw your pediatrician this past Wednesday and you received your four month shots.  I have several friends that know about the dangers of vaccines and I respect their advocacy.  I have read, and read myself, educating myself on preservatives used and which companies manufacture which vaccines.  No matter what the consensus is, we as parents have done what we feel is the best for you.  You are not like your sisters.  The chances of you contracting a bacteria or a virus is incredibly high and once you do the chances of it developing into something worse could be very serious.  We ask  that others respect our decisions to do what we truly feel is the best for your well being.
        The night you received your shots you ran a little bit of a fever, which we were allowed to give you Tylenol to help, but this past Thursday I was up with you from 1:00 am to about 4:45 am holding you as you had severe tummy issues.  You threw up several times, but I was able to get you back in your crib to rest before I had to get ready for school and go teach for a full day. Needless to say...I don't think my students realized I was functioning on only 2 hours of sleep, but I sure could feel it by the end of the day. One day isn't too shabby, but a week averaging between 2 - 4 hours of sleep and then being in a classroom from 7:00 am - 5:00 pm really takes its toll.
          I called your team at Children's Mercy because you have been doing so good with your weight gain, that I knew throwing up was not an option.  I was worried about you getting dehydrated and of course the loss of calories that you endured through out the night.  I actually thought you were having side effects from the vaccines.
         Once I called the physicians and answered a variety of their questions, they told me that it probably wasn't the vaccines.  That you probably had been suffering for a blockage in your bowel and the fact that the milk going in was not moving out, then you just had to expel it the best way you knew how. Not just a common constipation like your sisters might have had, but with you we have to be concerned about the thick mucous in the pancreas and intestinal walls.  So many newborns diagnosed with CF actually have surgery to help remove the blockage caused by the thick mucous, so we have to stay on top of things.  They told me to give you your Albuterol so that the added salt would help thin the mucous and not force feed you.  Give it time and hopefully in a day or two you will be back on track. (there is a lot more that I could probably type about the discussion I had with your team over the phone, but really not into talking about all the different poos you do in a blog :o)  )
           One more thing.... I really wanted to add this to the blog a couple of weeks ago, but am just now finding the time.  It was the evening of August 19, 2012.  You had just been given all of your meds and treatments and were laying in your crib. Tara was already asleep and Arya ( your 7 year old sister) was cuddling with me on the bed.  Out of the blue Arya said, "Mom, I wish I had CF too, so that way when Aven gets older she will not feel like she is all alone.  I could be the one she looks up too."   I know Arya doesn't understand everything that is involved with cystic fibrosis, but she has seen the needles from the blood work up, she has heard you cry from taking all the syringes full of meds., she has watched you squirm and fight as you have chest X-rays and put on a mask for your treatments and knowing all of that still made the comment that she wished she had CF too.

 Your older sister loves you very much and I know she will always be there for you.
                                                                          Much Love Today, Tomorrow, and Always,
                                                                                        Your Mommy - Autumn

I know I posted this picture last time. Both of your sisters are amazing and I think you can see in this picture how proud Arya is of you and how much you love being with her.

You taking your Albuterol though a mask.  As you get older you will have several different medicines that will be dispensed through nebulizers and inhalers.


Saturday, August 18, 2012

Dear Aven -
     There are so many things that I want to sit down and post about you, but I find that when you are happy and doing great, I would rather spend the time with you and your sisters instead of on the computer.  Just the other day, I realized what makes you the happiest at three months of age.  Your favorite thing has to be taking your diaper off!  Yes you grin when your family is around and are happy when you see toys and a bottle, but you smile with your eyes and are truly happy when the pampers cometh off!  So, it seems that when we hit a rough patch is when I tend to be a little more motivated to sit down and post.  Like I had mentioned before, this has been very therapeutic for me. 
      I had mentioned in the last post that you had your throat swabbed so that we could determine whether or not there might be bacteria growing.  Your team is very proactive and even though they were so pleased with your weight gain and the fact that you were not exhibiting any symptoms at the time, I did receive a phone call on Tuesday of this week stating that your culture had grown staphylococcus aureus.  A big fancy word for a very common bacteria.  Of course the mother side of me teared up a little, but the scientist in me along with your father's knowledge on staph infections, quickly put things into perspective.  S.aureus is a very common bacteria in which about 20% of the population are long term carriers. It likes to live  on the skin and in the mucous membranes of nasal cavities. This is the bacteria that is responsible for common skin infections like pimples and impetigo, but if not caught and treated, someone with a compromised immune system could develop more severe symptoms leading to pneumonia and meningitis.
      I found myself asking the one question that I have asked so many times thus far...."What do we do now?"  We are treating it aggressively with an antibiotic for the next 14 days 3xs /day  - 3 mL per dosage ( 9ml a day for the next 14 days).  If for some reason a cough develops, then we head to KC.
I have had some great struggles with our pharmacy and was dreading going to pick up the anit-biotic.  You see, according to the pharmacist, you are their only CF patient.  According to their supplier they are not wanting to provide the full script of enzymes at once because of the expense. Instead of getting all the enzymes for one month and being able to run the Live 2 Thrive card ( which can only be used every 30 days) they have told me when I need the enzymes filled, then I need to go in every two days and pick up a bottle at a time until the script is filled. I have gotten a lot of advice as to how to handle the situation and will be making several phone calls over the next few days.
      As far as your anti-biotic goes,  my first thought of course, was when comparing the medicine and dosage with antibiotics your sisters have taken, WOW there is just no comparison.  I remember your sisters taking dosages of  1 ml, twice a day for 7 - 10 days.  My next thought was wondering if we were exposing you to antibiotics too early.  I know we have to treat the bacteria, but I was a little worried that we were starting the path of building up drug resistance bacteria.  MRSA is common among CF patients from what I have read.
        I just found myself smiling when typing. Not because of the words that were just put down, but because of a thought in my head.  I just imagined you as a young lady reading these posts and thinking to yourself, that your mother was so cute trying to explain what was happening.  You see, I know that as you get older, you will have more of an understanding of your disease and you will be the one explaining and teaching all of us so very much.
                                                                      Love Today, Tomorrow, and Always,
                                                                                 Your Mother, Autumn

Above:  A great picture I took of you with your oldest sister Arya.  You just light up every time she is near.
Below: Spent some time today trying to figure out the best way to organize and store your meds.  At 3 months of age this is what you are prescribed.....will only increase as you get older.

Monday, August 13, 2012

Dear Aven-
      This has been a very hectic and difficult week for me.  I have been back to work now for two weeks and even though we do not officially start until this Thursday with students, I have been very busy attending/presenting at teacher workshops.  It has been very difficult to return to work and to leave you and your sisters, but the last two weeks have allowed me to gradually get acclimated before things really start moving at full speed.  I have caught myself from time to time; however, breaking out into tears, just like I did today.
       The first time was when I had walked into my classroom after a full day of meetings and found plastic spoons on my desk.  I know that seems silly; your mommy crying over plastic spoons, but I have been on the hunt for them for about a month.   Since we have to give you your enzymes in your applesauce we are constantly trying to sanitize the spoons that we use.  Your daddy and I thought that the little ice-cream sampling spoons would be great especially when we travel and when I finally made it to my classroom, there was a bag full of sampling spoons with a note that said  "For Aven and her Mommy."  I was trying to remember what triggered my second cry, and honestly I think it was a picture of you and your sisters that I had sitting on my desk combined with physical exhaustion from lack of sleep.  After I was able to wipe those tears away, I then had a special delivery which made me start crying again.  Former students of mine, two beautiful, young women, made me a teacher toolbox and as I read the meaning behind the items in the box, I realized how much I will need others during this process.  And yes, I even cried for a fourth time today.  It was when I left the pharmacy.  I had told them a week ago that I was going to need to refill your enzyme script in five days and they reassured me that they would have your enzymes in stock when I arrived today, alas they did not.
       I would like to thank RB, SL, and NR ( like I had mentioned in a previous blog, I really do not know proper blog etiquette so I hope these individuals recognize who they are) for being a wonderful support system and by providing me and my girls with wonderful "pick me ups" over the past two weeks.  It really means a lot to know that you are there.
              As a side note, I do have to say that I am thankful for the insurance that we have as well as a program called Live 2 Thrive which assists in the purchase of your enzymes for the first two years of your life. You see we can not purchase generic brands when it comes to your meds. According to the doctors, generic just doesn't work with CF patients. If we did not have this assistance we would be paying close to $1000.00 a month just for the enzymes
       We did make a trip to Children's Mercy this past Friday and we met with your CF team...the CF doctors, dietitians, and therapists.  Everyone agrees that you are doing WONDERFUL! You are gaining weight accordingly which means we have the right dosage of enzymes and your lungs sound great with a pulse ox (oxygen) of 100%.  We received your very first percussive cups today to aid when "clapping you" to break up the mucous in your lungs.  You were such a trooper.  As the respiratory therapist demonstrated, you just sat there with a content look on your face - not seeming to mind the process one bit!  You had your throat swabbed to see if there is any bacteria 'hiding' out, which you will have done at every visit, and once again we must now wait a few days to hear the results.  Like I had mentioned before, your team is very pro-active.  I hope we receive a call saying that the culture showed no signs of bacteria, but if for some reason the culture grew, then we will drop what we are doing, head back to KC and provide you with the right anti-biotics before anything can develop.  It's this whole waiting thing that I am not used to yet.
       Every time we go meet with your team, your daddy and I feel more and more confident that we can do this.  They are wonderful.  By simply being honest with us and answering all of our questions, we feel better equipped to handle what may come our way.....and believe you mean if you don't know by now.....your daddy and I like to ask lots of questions.
         Your doctor really stressed to us that even though we know you have two mutations of deltaF508, that each individual is truly different.  I try not to dwell on the emotional videos and information that is out there.  I would like to think that I am trying to get all of the emotions out now, so that if you have struggles in the future, I can be strong for you and focus on your needs instead of my own.  When we left the hospital, I just felt like you are going to be that one little girl where we all learn to expect the unexpected.  So many little ones have suffered greatly, but your gaining weight, your lungs sound great and you are just the happiest, best natured little girl in the world! I truly felt happy  leaving this visit.
                                                                        Love Today, Tomorrow and Always,
                                                                                   Your Mother - Autumn

This is the wonderful picture that I have sitting on my desk of you and your sisters.

Here you are having your chest and side "clapped" with the percussive cups as your sisters look on.
                                         You just kept smiling at the doctors the entire time.

Wednesday, August 8, 2012

Dear Aven -
     I feel that these early posts are not just for you, but also to allow those following this journey to become informed about cystic fibrosis.  I am all the time running into individuals around town that ask about you and your disease and so many want to know what exactly is involved.  They usually admit that they do not know much about CF and many have asked if it is something that you will simply outgrow.  I do not want to come across that I have all the answers especially this early in the journey, but very few people know about this life altering/chronic disease.
      I have joined so many groups and have 'liked' so many pages dealing with CF.  I try to use good judgement when selecting what it is I view on-line.  Perhaps the best organization to follow is the Cystic Fibrosis Foundation.  This is a nationally recognized non- for profit organization established in 1955.  They are a major  driving force behind finding a cure. Currently they are making enormous strides in finding the best possible treatments for CF patients.  There is currently (2012) a drug on the market called Kalydeco which has extended the life expectancies of some CF patients. This drug has been successful for approximately 4% of the CF community.  Unfortunately it does not target your mutations DDF508.  However, on May 7, 2012 the CF community received wonderful news that Kalydeco in conjunction with VX809 had completed phase 2 trials and were showing great success for patients with the most common form of CF...your form.  I just know that each year you grow and you get stronger, there will be so many things that will be there to assist you on your journey.  Perhaps that is why I have a very special picture frame in my classroom this year.
       Having a chemistry background, I have also been very drawn to studying pathophysiology and genetics.  This has been great since your daddy took so many of these classes in college; he has been able to retrieve his old textbooks and allow me to view them.  As I read them it gives me chills in a way to know that so many years ago when your daddy was reading the chapters on CF and highlighting the words to prepare for a test, he was highlighting the words that would be used to describe your condition. It makes me sad because the emotions are still very raw, but I am learning how to handle things. I am getting stronger each day so that I can be there for you every step of the way.

                                                                               Much Love Today, Tomorrow and Always,
                                                                                              Your Mother -Autumn

I have this in a picture frame in my classroom!

Saturday, August 4, 2012

A Little Piece of Aven

Dear Aven-
    I have been so very proud of you.  We just finished taking your "vitamin bottle"  which includes your milk, Vitamins A, D, E, and K, salt, and your Zantac.  Your oldest sister was on Zantac as well, as so many babies are, but she quickly got off of it in about three months.  You on other hand will probably make the transition from Zantac to Nexium ( or whatever is the most appropriate for you at the time) as you get older.  From what the doctors have told us, since CF patients have to use so many nebulizers and inhalers on a daily basis, they tend to be very prone to acid reflux and so this type of medicine will just become one of those things that you will take every day for the rest of your life.
   You are also doing very well taking your enzymes.  We had tried for a short while doing a "dry run" where we just pour the capsules in your mouth, but you struggled with choking on the 'beads' so we decided to go back to the applesauce since you seemed to do so much better.  I remember the first time I gave them to you at three weeks of age.  It probably took a full 10 mins to get you to consume all of the ZenPep, but now at three months of age you are like an old pro; even taking them with a smile.
     We have been very blessed to have so many people keep us in their thoughts and prayers.  I would love to list individual names, but to be honest, since I will be making this blog public, I do not know the proper etiquette and I always felt bad about including someone in a conversation if they were not informed of it.  So maybe if I just say thank you to HG, JS, and WJ, they will know one day that I am grateful to them for continually providing me support by answering questions, sending me emails and messages and even a little back to school gift basket.
     When I started this blog, the only intention was to provide documentation for you so one day you could look back on our journey together.  I can foresee you being in the hospital for an extended time and maybe these words will give you comfort when needed.  I do not claim in any way shape of form to have all the answers or to even begin to understand this disease in its entirety.  I was very surprised; however, when I went to our local bookstores and looked in the health/disease section to try to find books on cystic fibrosis that there was nothing on the shelf.  To be honest, I was looking for "CF for Dummies". I did find a few books online, but even at that they were very limited.  I do have to tell you a funny story about Arya.  While we were out searching for books and searching the diseases alphabetically, Arya picked up a book and asked "What's this one mom?"  I told her that it was for people who suffer from celiac disease.  She grabbed it and said, "well we definitely need this one for daddy.  Sometimes he just does not know how to act."  It kind of reminds me of the time when we were trying to explain your heritage to your sisters and the fact the you are all part Indian and Arya spoke up because she was getting frustrated at Tara and said, " It's because daddy is 1/4 idiot!" (not Indian)
    I have been in the process of trying to figure out how to make this blog public so that friends may keep up with what we are facing.  I do not know if I will always make every post public ( I know that there will be quite of few things that I will keep for your eyes only) or how long I will keep it opened.  It seems to be therapeutic for me; to put my thoughts down. Hopefully from comments, I may learn and be motivated to keep the blog going.  So much I would like to type, but I hear you crying so I must go.........
                                                                  Much Love Today, Tomorrow and Always,
                                                                                   Your Mother - Autumn

You taking your very first enzymes at 3 weeks of age! (took about 10 mins)
Now look at you! At 3 months you are able to take them with no problems! Enzymes will be something you will have to take everyday for the rest of your life.  You will take them before every meal, snack and/or drink that contain fats.  This is what allows your body to absorb the nutrients you needs from food inorder to thrive.


Wednesday, August 1, 2012

A Little Piece of Aven

Dear Aven,
        I am so in love with you.  I look at how excited you get when you see me come into your view and I know that you know who I am.  That is such a wonderful feeling for a parent and so I thank you for making me feel amazing everyday. We've had some good and some bad days as of late.  
        The biggest thing I have noticed is trying to get the right dosage of enzymes with every feeding.  Most of the time we do just great, but there are those moments when you take more than usual from your bottle and the dosage of enzymes seem to be 'off'.  You struggle with the pain in your tummy and all I can do is hold you and cradle you until the pain goes away.  You tense up and you turn your head away from mine, almost as if you do not want me to see you struggle.  The pain goes away and once it does you look at me once again with those beautiful blue eyes and a big smile as to say thank you.
         These past few weeks have been extremely hard.  Your father had a wonderful opportunity to go to Chula Vista in California and work at the Olympic Training facility for two weeks; so exciting, especially as the athletes were getting ready to leave for London. But wouldn't you know it...the first full day he is gone, I break my finger in the car door!  I never realized how much I relied on my little finger especially on my dominant hand.  Even though daddy was gone for awhile, we managed even with a broken finger.
          I am a worrier.  That is what I do; just ask your father.  I worry about your future and that of your sisters.  I want to make sure that I have done everything that I can to make sure that all of my girls live the best quality of life possible.  I have been ashamed that I have not done all that I can for you.  It is hard; but I am going to try harder.  Maybe that is why I did it! I have always wanted to donate my hair so I finally went and had 11 inches cut and I donated to Locks of Love. 
            At his point in my life, I understand that my hair does not make who I am and that it will grow back, but to a young girl who is struggling to find a sense of identity in the crazy, materialistic society maybe my donation will help provide a sense of security as they face struggles that no young person should have to deal with.
          We will begin your chest physiotherapy on August 10.  I am trying to get a schedule established now so that I may do as much as I can for you.  I am dreading the start of school.  As of now it looks like we will be getting up around 4:30 am every morning so that you and I can get your morning meds and treatments taken care of and hopefully that will leave enough time for Arya and I to get ready and get to school.  I teach some rigorous courses and I truly believe in grading everything that I ask my students to complete; so grading along with your evening treatments should put bedtime around midnight or so.  Your daddy mainly works nights, so I know I have to be the one that sees this through.  He will be there for you for sure, but his schedule is always changing., and I have read so much about the consistency of treatments.

           I just don't wont to be weak.  I know that I have to stay strong and I will.  Every mother has that feeling that everything  they do is for their children.  I have always felt that way, but now I will truly live it!
                                                                          Much Love Today, Tomorrow, and Always-
                                                                                        Your Mother,  Autumn
Mommy's hair before getting it cut!
And Mommy's hair now!  (This is the shortest I have ever had my hair)

Friday, July 20, 2012

Dear Aven,
        I awoke this morning excited about typing you a short message and was stricken by sadness as I learned of a horrific incident which occurred in Colorado today.  If you take the time to look up Colorado movie shooting July 20, 2012, I am sure you will be able to find the details. 
         I gave your sisters and you the biggest, most sincerest hug, as I know many parents across the country will do tonight.  Those parents who have taken for granted their children were reminded today that life is so very precious and we do not know when or where we may take our last breath. 
         As I looked at you, I could only think about how you did not choose to come into this world, nor did you choose your diagnosis. I thought about the battles that you will face in your future as you will fight for just one more breath on a daily basis and how someone by an act of violence could so easily take it from you.
         They do not have a motive at this time as to why this young man in Colorado did what he did, but I feel as a high school teacher, that there is so much that we are not doing in preparing our young adults to handle frustration, depression, stress and anger.
          I am reminded of the meaning of your name.  Aven, means courageous and brave.  Yes you are. Yes you will be.  My thoughts are with all the families and friends who suffered today.

                                                                                   Much Love Today, Tomorrow, and Always,
                                                                                             Your Mother Autumn

Thursday, July 19, 2012

A Little Piece of Aven

Dear Aven-
           I have found myself as of late wanting to sit down and write to you through the blog; however, the moment I start to sit, I think to myself that it would be time better spent holding you and watching you smile.  So I go to you and I grab you and look into your blue eyes. You  look so innocent and sweet.  You just came off from having a cold and you did quite well.  I had called the doctor to ask about what we needed to do. I knew from here on out your colds would be anything but common.  We would have to handle them a little bit different than the ones that your sisters have. Your CMH physicians (Children's Mercy Hospital) had told us that if you developed a cough then that is when we needed to get you to Kansas City and your pediatrician stated that we should not give you any cold meds because the medicine has a tendency to thicken up mucous even more so for CF patients. So we just held you, used the Vick's rub like crazy and suctioned your little nose constantly to see you through.  It was hard for me to watch you sleep at night.  You would lay there with a smile on your face while your little chest looked as if it were racing to provide you with every little breath it could.  Your father and I even noticed a discoloration in your skin as you slept through your cold and the slight blueness in your cheeks worried us about the lack of oxygen you were receiving.
          Several people were glad to hear that your cold had passed.  I have been very thankful for all the thoughts and prayers that are being giving to you, but what so many do not understand is that just because the sniffles are gone, the damage has been done.  Every cold is a catalyst and it disturbs me that I can not see the damage other than through an X-ray machine.  Even if I could see it with the naked eye, there would be nothing I could do.
         Your sisters, Arya and Tara, love you so much.  I get tickled at Tara who will turn 4 in October, because she truly believes in her mind that when you cry, you are crying out for her and that she is the only one that can take care of you.  Both your sisters had their genetic tests and we did receive the results on July 10, 2012.  Arya and Tara are both carriers of CF.  They DO NOT have CF so they will never experience the symptoms and treatments that you will have to endure.  Their genetic profile shows that they each have one mutation of deltaF508, which means they are a carriers.  Two mutations, such in your case, would have indicated that they had cystic fibrosis.  This is very important information for your sisters because as they get older and if they find out that their spouses also are carriers of CF, then there will be a chance of them having children with the diagnosis.  Their chances were as follows:  25 %  of not being a carrier and having no mutations,  25% of having CF, and 50% of being carriers.  1 in 25 Caucasians are carriers of CF and many do not even know it.  Your father and I did not know that each one of us carried the mutation that would lead you down this path.
                                                                    Much Love Today, Tomorrow and Always,
Sleeping during your first cold!  We had a rough night prior!
                              Mommy Autumn
Big Sister Tara taking care of you!

Sunday, July 8, 2012

A Little Piece of Aven

Dear Aven-
       After your official diagnosis, it was hard.  I think the Internet was actually more of a hindrance than a resource, or at least in that first week.  I wanted to look up the the worse case scenarios.  I wanted to prepare myself for what our journey may look like.  Your daddy seemed more at ease.  Not that he didn't care, but had more faith in the advancements that were always being made in the medical field.  I think he also knew that we, together, were the best parents for you. 
        When we traveled to Children's Mercy after your diagnosis they were very surprised that you had gained weight.  Most babies with your mutations, struggle with weight gain and have to start the enzymes immediately.  Many infants even have surgery within the first 72 hours of birth simply due to the pancreas and bowel already being blocked with thick mucous.  They had done a little bit of testing to determine that even though you were gaining weight, you were severely pancreatic insufficient and that since our plan from the 'get go' was to stay pro-active then we needed to start you on the enzymes.
        We were instructed to take a small amount of applesauce and open a capsule and mix the enzymes with the sauce.  I remember thinking that this was going against so much that I knew as a mother.  I never even introduced solids to your sisters until about 6 months of age, and here you were still a tiny little thing, not even 4 weeks old, eating from a spoon.
         I could see a big change within 24 hours.  The enzymes were actually helping.  Prior to the prescription we were changing your diaper as many as 20 times a day and we knew the enzymes were doing their job; they were allowing you to absorb the nutrients you needed from your food.  You see, that is usually the first stages of CF as an infant.  You have difficulty absorbing the nutrients that you need form the fats in your diet.  Many individuals with CF have faulty or malfunctioning protein channels.  Your protein channels were just fine; however, the number of channels you had were insufficient.  Your channels were not allowing the chloride ions to escape from their cells.  This chloride ( which easily comes from table salt - NaCl and is naturally in our bodies) is needed to attract the polar water molecule to allow for hydration to occur.  The process of hydration is what is needed to thin the mucous in our bodies.  Most individuals can easily sneeze and cough to break up the loose mucous when an infection sets in, but CF patients develop a thickening of their mucous which first tends to block the pancreas and interrupt digestion and then slowly works its way into to the lungs where it settles. While others can easily remove the mucous by blowing their noses, we will have to go through a series of "treatments" to help thin and loosen the mucous so it does not serve as a petri-dish for any foreign bacteria that finds its way into your system.
         When others had heard of your diagnosis, I had several individuals ask me if you tasted salty.  It took me awhile to test and see, but I would lick my lips, give you a big kiss on your forehead and then I could taste for tasted like a little salt lick.  You see, since your cells were not releasing the chloride ions inside of your body, they were leaving with the cells through your pores.  That is why everyday for the rest of your life you will have to have salt added to your diet.  Right now as a 3 week old, we are adding table salt to your bottles.
           I feel that I am starting to get this blog caught up to date.  There is some information that I have not included, but as I reflect in future blogs, I believe the details will make themselves known. There is one more thing I need to add.  We had traveled to Children's Mercy again on June 25, 2012 for two reasons.  1) was so you could have the sweat chloride test and 2) both your sisters Arya and Tara were to be tested to see if they possibly had CF or if they were carriers like your father and I.  Even though we knew you had CF through genetic testing and had already started treatment, the sweat chloride test would inform us as to the rate at which your body was losing your chloride ions.  I received a phone call on July 2 stating that your numbers were 90 and 120.  As I did further investigation I found out that for your age these numbers were astounding.  Under 30 millimoles/L = not likely CF,  30- 60 millimoles/L = borderline CF, and 60-80 millimoles/L = CF. When I asked the doctor what it meant, she had stated that as you got older and participated in sports we would have to really increase the amount electrolytes you consume.  Dr. Bear also mentioned that you would be most likely adding salt to the standard glass of water that you would be drinking. As for the results of your sisters' blood tests, we are still waiting.  It has been 13 days and I expect we will here something tomorrow.
                                                                Much Love Today, Tomorrow and Always,

Gettig the elctrodes placed on your arms for the sweat chloride test.
Your Mother Autumn
Arya and Tara after they had their blood taken
for their genetic tests.

Saturday, June 30, 2012

Dear Aven-
         We just returned from the 66 Drive-In.  I was a little reluctant to go because this week we have been faced with extremely hot temps.  It actually got up to 105 degrees today and I was worried that you would not handle the temperatures well, even in the evening;  however, we went ahead and took the car so we could keep the AC on so you would not get too over-heated.  It was important for us to take your older sisters.  I have felt that the last two months have been centered around you, as is the case with all newborns, and we wanted to get out and do something that your sisters would enjoy.  They were showing Madagascar 3 and Pirates: Band of Misfits.
         As I type this, eventhough it is late, I am looking at your father who is feeding you.  I had mentioned that I had to cut the last post short because I had to go prepare your enzymes.  Well, let me try to catch you up as to why we are giving you enzymes.
          The first five days out from your genetic test, which was taken on May 9, 2012, was a little stressful. We had gone to Children's Mercy due to your newborn screening and eventhough two screens came back positive for CF, there was still a part of me which believed that they were simply false positives. What made it stressful was when we went to the clinic we met so many people....nurse practioners, doctors, social workers, dieticians, and nurses.  They kept referring to themselves as "Aven's team" and explained how they would be there for our family as we started the CF journey.  Your father and I had lots of questions which they did a great job answering; however, as I sat there conversing with the physcians, there was a little voice inside my head that kept saying "why do they keep treating us like our daughter has cystic fibrosis when all we have done is newborn screening which is known for having more false positives than not?"  I remember crying once in front of the doctor.  They were presenting CF as a life-threatening disease and so when I asked about life expectancy, they were very positive when stating that they are always making strides in the CF community.  Years ago a child with CF was not expected to make it past their teen years and now the average life expectancy was 37.  That's when I cried.  Your father put his arm around me. You see I was currently 37, and I could not imagine you not making it to this same time in your life. Great things happened to me at the age of 37....I had you.
              Maybe I was in denial or maybe it was just hope, but after that visit I was able to walk with both feet on the ground and just kept thinking about all the false positive tests I had read about. It wasn't until May 18, 2012 that I really felt like my world had fallen apart.
            When I think back about the phone call, I really wish I had not cried in front of your sisters.  Dr. Baer from Children's Mercy had called and stated the the results from the genetic tests were in and that "your genes had cystic fibrosis written all over them."  I remember trying to hold back tears, but she could tell that I was choking up. My first and only question was "What do we do now?" She went on to explain that there were so many mutations out there, but yours was the most common.  You had two depletions of Delta F508 ( which will be referred to as either homozygous DF508 or DDF508). We went ahead and scheduled your next appointment for May 22 and at that time we would sit down with your team and begin our plans for your treatments. 
            As soon as I hung up the phone I cried out in pain.  I remember it feeling like when I had cried over the death of a loved one.  An overwheling sense of loss.  I just held you in my arms and kept repeating "No, No, No."  These were the tears that I wish I hadn't shed in front of your sisters.  Your daddy was not home and Arya and Tara came running into the room to see if I was hurt.  Once I composed myself, I called your daddy to tell him the 'official' life altering news.

                                                                                       Much love today, tomorrow, and always,
                                                                                                   Your Mother - Autumn

Wednesday, June 27, 2012

A Little Piece of Aven

Dear Aven-
          I have wanted to start this blog for quite sometime now, but to be honest, everytime I sat down at the computer I became overwhelmed with emotions and had to walk away. I know this first blog will be rather lengthy, because I will try to get caught up on what has happened over the last two months.
          It has been two months and two days since you were born.  The pregnancy was like no other.  I was never sick with your sisters and they arrived  within 72 hours of their due date. You on other hand, made your presence known every morning before I left for work and was ready to get out and meet the world three weeks before your due date.
           Your delivery has quite a story as well.  I actually had gone into labor on Monday, April 23rd as I was teaching my Advanced Placement Chemistry students.  Your father and I actually went to the hospital around 2:00 am on Tuesday, April 24th just to be sent home.  They stated that I was definitely in the early stages of labor, but because it was three weeks until your due date, they would not induce me and recommended that I go on home.  I remember telling your father around 9:00 pm that we needed to head back to the hospital because the pain was getting worse.  Your father felt that once we got to the hospital they would probably just send us on home.  I remember him finishing a load of laundry and mopping the kitchen floor before we got your sisters loaded up in the car to take them to Mimi's and Papa's. 
            We dropped your sisters off around 1:48 a.m. on Wendesday, April 25th and headed to the hosptial.  Your sisters were born in the old St. John's Hospital in Joplin, Missouri, but due to the May 22 tornado, that hospital just stood as an empty shell.  I was very excited about delivering you in the new, temporary delivery ward that Mercy had created, which was called the Walden Building. 
            As we drove along the interstate there was time when I really thought we would be naming you Prius or giving you a nickname of I-44.  I knew that when we arrived, if we arrived, that they were not going to send us home this time.  As soon as we entered the parking lot, you arrived within ten minutes.   Neither my OBGYN nor the doctor that was working that evening made it into the delivery room. No drugs, no doctors and in one push, a nurse by the name of Cindy and your father  welcomed you into this world.
             There you were.  Born at 2:38 a.m. on April 25, 2012 and weighing in at 6 lbs. 6 oz. Our perfect little girl.  Beautiful in every way.  We had several names in mind, including yours, but we were not 100% certain until daddy looked at me and said let's call her Aven.  Your middle name on the other hand took about 48 hours to come by.  We had thought of Greer, Mckinnley, and Mae, but decided upon Renee because of the mathematician Rene Descartes. ( but don't tell your Aunt April who also has the middle name Renee.)
            You were a tiny thing. Wearing preemie outfits and sleeping so innocently.  Once given the all clear we left the hospital and headed home.  You  fit right in to our home and your new surroundings.  Your big sisters could not get enough of you and wanted to continuosly hold and kiss you, which I have to admit made me a nervous wreck.  You were very easy to soothe and seemed to be doing great.  That is why I was so suprised when I received a phone call from the State Health Department as well as your pediatrician's office seven days out informing us that your newborn screening had tested positive for cystic fibrosis and that we needed to have a another blood test immediately.  That exact same day of receiving the phone call we were back at the hospital having another blood test ran to check for CF again. 
              I was definitely surprised, but I also knew that mistakes happen and being a newborn there were bound to be certain levels elevated.  I had read a lot of information on the internet about the high number of "false positives" with the CF test and I took comfort in thinking that your screening was indeed a false positive, but when I received the second phone call in regards to the second test also being positive, I knew that this was serious.  Most newborns have IRT counts ( protein counts)around 70 - low numbers are good and should continue to decline as you grow.  Your first test came back as 246 ng/mL  and the second test had jumped to 294 ng/mL. Within 48 hours we had an appointment to go to the CF center located at Children's Mercy Hospital in Kansas City, Mo.  I knew that they would be able to do more testing to determine if CF was really an issue.  We had scheduled a sweat chloride test as well as DNA genetic testing.  Unfortunately you were still too little to have the sweat chloride test performed.  They were worried that the electrodes would actually 'burn' the skin and so we decided to wait until you were a little bigger; however, we were able to draw blood and have it sent to the genetics lab.  The bad thing is now we had to wait for 7 - 10 days for the results.
       * I will try to catch you up in the next blog, but for now I must go.  You are stirring in your crib so I must go get your enzymes ready.
                                                                                            Much love today, tomorrow and always,
                                                                                                                Your Mother - Autumn