Alayna 11 Month's A Little Late

This post is a little late as we were all on a family vacation during Alayna’s 11 month birthday.  In less than 1 month our miracle will be turning 1 year old!!!!!!!!!!!! What???? I still cannot believe how fast 1 year has gone.

So what has been going on in your life Alayna? You, finally can say dadadadad. In fact, that is how you say it.  I honestly thought you would be walking before you said dada.  I guess I was wrong.  That also means you still are not walking yet.  But WE know in a few days you will take your first steps.  You have become so brave these last few days holding on with one hand and then letting go when you pull yourself up on the corner of the wall, window ledge, baby gate, or your play pen.  I think you are just being cautious.  

One of your new favorite things is to stand up with your walker or even a box and you start walking behind it.  But I think you still feel safer crawling everywhere. Not just that its safer, but you can get to where you want to go faster.

You are learning to say a few new words minus mumumum and dadadad. When you see a picture of a dog or even see a dog you try to say dog.  On vacation I would tell you to wave to the birdie or the dog and you would wave and try to say bird and dog. It looks like you are going to be one smart little girl here real soon.

This past month we had our first family vacation to Topsail, NC. During the stay your grandma and grandpa were there, uncle tom and you also got to meet daddy's cousin. On the way back home you got to meet your great aunt and uncle from DC.

We were originally suppose to go to Niagara Falls  back in May but you were not feeling to great.  But that was fine because if it wasn’t for you getting sick we wouldn’t have had the chance to put a bid on the house we know live in.  Mommy and daddy were both anxious on how you would do in the car.  We decided it made sense to break the drive up and stop halfway.  I think for mostly mommy and daddy’s sanity.  But you were really good in fact I would say you did FANTASTIC in the car.  You really didn’t even cry once. I guess it must have been watching a few Barney videos that put you to sleep in the car.

We stayed two weeks at the beach and it was a great time.  The first week was really a big adjustment for all of us.  I think mainly for mommy and daddy who are use to just relaxing at the beach.  We focused all of our attention on you as we made sure to not expose you to the blistering sun.  When we would go to the beach it was well past 4 pm everyday to minimize the UV rays.  I think you really dislike sunscreen!  It was like torture when we tried to rub the sunscreen on you.  But when we got you out to the beach you really enjoyed it.  I wasn’t sure how you would react to the ocean but you LOVED it.  Never once were you afraid of the sound or the water. You would smile every time I put you in the water or held you in the sand.

The place we stayed at had its own pool so we took advantage of that.  We put you in your little baby float and you would just splash away.  There was on day you just fell asleep in your raft in the pool.  I guess from this trip we could really conclude that you LOVE water.

During this trip I got to see firsthand how hard your mommy works everyday as a stay at home mom.  I really was able to see a first hand appreciation for how hard she does work everyday.  I know one day when you read this you will tell us how easy a child you are.  It’s not that you are destructive you are just one active little toddler already.  I guess we would prefer it that way.

I still cannot believe in 20 days you will be 1 year old!!! It really is hard to imagine how fast time really goes.  You have given us more joy than you would ever know Alayna.  We love you more than words could ever express!!!

 Time for a bunch of photos at the beach.

Family!!! 

Someone is happy

She loved everything about the ocean!

She made her own little footprints

First time viewing the ocean when we arrived. 

Two beauties. 

Mommy is happy 

I still cannot believe Maria had a baby less than a year ago! 

Mommy and Alayna in the pool 

Rockin the ball cap!!! 

First time touching the sand!!! 

First time ever in a pool! 

No she actually did not eat a chip! 

This is an awesome view daddy! 

I am the princess of the beach. 

WE just love her smile. 

Only official 11 month photo! 

CF Visits & Colonoscopies, Oh my???

I didn't realize this until recently but my CF doctor is kind of like a blogger.  No he doesn't have his own blog site but he hands out what he calls his "Communicator" every so often.  He types a double sided paper about what's going in the CF world, in his career, or any oddities that he may find.  Well in this issue I wanted to summarize some of his update because I think we could all benefit from it. 


*****Disclaimer- this is a summary from my doctor’s research and writing.  I am not taking any credit for any of these opinions or suggestions.  Again, its appropriate to consult YOUR CF care team.  I just thought it would be useful to share with all who read my blog, because I care!!!*****


His first section is titled "Routine" clinic visits.  I think most of us attend clinic on a quarterly basis.  But I on the other hand tend to go more frequent.  I was never really sure what the method for my doctor's madness was until reading this.  In fact it kind of makes some sense.   My doctor started practicing medicine way back in the early 60s. Yes, he could technically be my grandfather which is awesome.  He is so full of wisdom and knowledge.  But back in the 60s at my CF clinic patients were seen on intervals of every 6 weeks!!! At these visits the focus was on continued home treatment and antibiotics were adjusted based on patients symptoms and culture reports.  Then during the 70s and 80s my clinic reported dramatic additional progress in survival, now extending into the late teens and early 20s. 


Almost all patients, regardless of how far they lived from the clinic would come every 6-8 weeks to be seen.  Patients would even travel from various other states to my clinic.  But then there was a gasoline shortage in the early 80s and that made accessing clinic every 6-8 weeks virtually impossible. 


Even after the gasoline shortage was over, many of my doctor’s patients didn’t return to the normal 6-8 week interval.  He claims that it was due to family stresses and the cost of travel.  Routine visits started to become an exception rather than the rule. My doctor states that we now believe that the decrease in aggressive out patient evaluations is responsible for some the decrease in health as reflected in pulmonary function test results, and more persistent symptoms, in a substantial minority of patients.  Patients, who should be doing better, aren’t doing as well. 


My doctor is suggesting that we return to not only quarterly visits but to actually increase the frequency of visits from 6-8 weeks.  Basically, for all of you go to clinic quarterly we should be adding at least two more visits a year.  So make that 6 visits a year.  In fact, my doctor believes that its even more important for many patients, especially for those who are doing very very well to be seen at this frequency.  No wonder why he wants me to come back so often! For younger patients my doctor recommends visits every 6-8 weeks “to catch new infections early” when the pseudomonas may still be eradicable.  This must be one of the main reasons I have not cultured pseudomonas in a well over a year and a half.  When we discovered I had pseudomonas we treated it aggressively with aerosolized meropenum and oral antibiotics.  It is now long gone and I hope to keep it that way. 


My doctor and others at my clinic are now recommending being seen every 9 weeks.  The main reason is to achieve better health results.  Not only do you get to see your CF doctor you are also able to see the Respiratory Therapist, the Nutritionist, and the Social Worker more often.  The more you have the opportunity to be exposed to these individuals you have a better opportunity to ask your questions and build a relationship with all your CF care team. 


Also, lets not forget the more often we attend clinic for our visits, the more often we will have access to the increasing number of research studies.  These studies can actually improve a patient’s health (like the Kalydeco studies).  In some of the clinical trials you also need to have had regular out-patient visits to your CF team. 


So going to your CF clinic is more beneficial then we may actually know.  Yes, we get our throat culture, weight checked, PFT, and all the other stuff but the more we go the more positives I can see from all of this.  If you are not attending your CF clinic at least quarterly you MUST start going every 3 months.  For those of us who are going every 3 months maybe we should increase that to every 8-9 weeks.  This is just a suggestion from me and my CF care team.  But lets think about it……….I have never said to myself this “I attend my CF clinic way to much”  I think the more we go the better we all COULD be. 


Lastly, here is the last part of my doctor’s communicator.  Colon Cancer. You may be thinking what????? Honestly, when I started reading this I thought the same thing.  But here are the details. 


The incidence of cancer, mainly cancers of the gastrointestinal tract has increased in CF patients.  Almost 20 times the incidence in non-CF.  The youngest colon cancer CF patient at my clinic was close to 30 years old at diagnosis.  The youngest in the country was only 19 years old.  As we as CF population continue to survive and thrive into our 40s, 50s, and 60s, cancer threatens to become the cause of death in small percentages of the CF population. 


So what are the reasons for increased risk?  Well that is still not yet known but we need to find out why.  Possibly, the increase risk could be due to the abnormal CFTR gene in the GI tract.  But with the development of new drugs like Kalydeco that correct the actual function of the gene this may reduce or eliminate the increased risk. 


But there maybe other reasons or possibilities.  Maybe all the pancreatic enzymes CF patients take reach the colon while they are still “active”.  If they arrive active maybe they could irritate the colon cells and result in cancer.  Or perhaps digestion in CF is still not totally normal even with adequate enzyme treatment.  With this maybe some toxin in the food which should be destroyed by digestion reaches the colon and increases the risk for cancer.  Also, maybe enzymes are the problem or insufficient enzymes could be the problem.  I as a CF patient have wondered this for years if taking these enzymes will result in another problem down the road.  But I would rather gain weight than be sick due to being to thin if I could have a say in it The jury is out on this. 


How can we prevent colon cancer in CF?  Well we all know about the lovely Colonoscopy.  If we have a colonoscopy it has been shown to reduce the risk of colon cancer by 70%.  If there are benign polyps found increased frequency of colonoscopies are recommended (every 3 years).  When no polyps are found decreased frequency is recommended (every 10 years).  However, what to do in CF patients is unclear.  My clinic has reduced the aged of beginning colonoscopies to 35 years of age.  Then they recommend repeat colonoscopies every 5 years until 50 years old.  Once we hit 50 they recommend every 3 years to have a test.  My clinic has found typical pre cancerous but still benign polyps in patients at relatively young ages.  There are some gastro dr.s who have suggested that the progression from benign to malignant polyps may be quicker in CF patients.    


As for colon cancer treatment, at present surgical treatment is the same as for non-CF patients, assuming that the CF patient is otherwise a reasonable candidate for surgery.  Chemotherapy, at present, is also the same, but the effect of chemo on CF lung disease and or CF liver disease is not clear.  There are not enough patients who have undergone cancer with CF. 


So with all this said, these are recommendations by my clinic for colonoscopies.  It looks like I am less than 10 years away from that lovely test. I guess for my 35^th birthday that will be my awful gift to myself.    For those, of us who are not close to 35 but may have a family history of polyps or colon cancer it may be wise to consult your CF team and see what they suggest for colonoscopy timing.  Anyone who is over the age of 35 and has yet to have a colonoscopy maybe you should consult with your CF care team at your next appointment.  Again, its better to be safe and catch a polyp before it turns into cancer.  Nip it in the bud people.


*****Disclaimer- this is a summary from my doctor’s research and writing.  I am not taking any credit for any of these opinions or suggestions.  Again, its appropriate to consult YOUR CF care team.  I just thought it would be useful to share with all who read my blog, because I care!!!*****

Liver Results Are In!

Today, Maria received her liver biopsy results and was also able to talk to her liver doctor.  The results are actually mixed.  I will start with what we know.  The biopsy revealed no cirrhosis of the liver which is GREAT.  During her appointment this afternoon she was able to talk to her liver doctor about the biopsy results and the recent blood test.  The blood test did reveal that something is going on with Maria's liver but the question is what................

The doctor claims her liver enzymes should really be a lot lower than they currently are.  So he is thinking that the elevated liver enzymes are related to an autoimmune disorder based on a few blood tests.  Still the test is not 100% conclusive of her having an autoimmune disorder but Maria shows many signs and symptoms of it. The doctor really believes that Maria could have what is called autoimmune hepatitis.  No, it's not the viral hepatitis and no you cannot spread it to anyone.

 Autoimmune hepatitis basically is when the body is attacking the liver. To read more about autoimmune hepatitis click here. Your immune system decides to take action and attack your liver for whatever reason.  The reason behind this is still unknown.  Researchers believe that autoimmune hepatitis in some people maybe pre genetically disposed.  

So where do we start with a treatment.........Well it looks like Maria's liver is still really really healthy which is excellent.  The doctor does believe we have caught all this very early as there is no scaring in her liver.  The current plan will be to give Maria the lovely Prednisone (steroid) to reduce liver inflammation.  She will be on this for a month and they will then perform another blood test.  They will see if her liver enzymes are lowering from the steroid.  If her liver enzymes are lowering that will confirm that she does have an autoimmune disorder.  Then she will be taking steroids and another pill most likely for the rest of her life to prevent cirrhosis of the liver.  Also, she will have to have blood tests frequently and liver biopsies performed every few years.  They will be monitoring to make sure the liver is still remaining  healthy and there is no cirrhosis.  But we shall see how Maria responds to the steroids as that will confirm if there is an autoimmune disorder attacking her liver.  I really do not want to jump to conclusions so just maybe there still is that hope she doesn't have to worry about this.  

I can tell you that Maria has shown me what it really means to be determined.  She has been going through this elevated liver enzyme period for almost a year now.  If it wasn't for her determination we may not even know why she is feeling the way she is.  Sometimes when it comes to your health you have to take charge and demand answers.  That is exactly what she is doing demanding an answer to all this.  I really admire this quality in her more than she knows.  I know she wants to be here more than anything for Alayna and for me and she is determined!  I just hope soon we will have some final answer to all this.

It is really hard seeing the one you love having to deal with not feeling like her normal self.  I almost feel helpless in all this.  All I can do is show my support and let her know it will be OK.  I know this family will get through this no matter what and come out even stronger than before.  Thank you again for keeping this family in your thoughts! It really means a lot to US!

I will update when we have more information........

 

Month of Thankfulness - August

It looks like I fell off the monthly thankfulness blogs I had been doing.  But I wanted to get back on the band wagon again.  As we all know we have so much to be thankful for every day we get out of bed.  So take a moment and think of a few things that you are thankful for today.

As many of you know, Maria has been having a rough battle with her liver enzymes ever since Alayna has been born.  I am so thankful for a wife who is determined and motivated to do whatever she can to find what is causing all this.  We are still waiting on the results of the liver biopsy but we should know very soon. I hope by the end of the week!!  I will post what we find out from the appointment. 

I have to say I have an awesome wife.  There truly is no one like her.  The other night she was cleaning my nebulizers with a tooth brush! Yes, a toothbrush. They have never been so clean before.  I just have to say my wife is an amazing lady.  I LOVE my CF WIFE!

I am so thankful for a community (CF community) who cares!!! Many of you have asked not only about how I am feeling these days but how Maria is feeling and if we have any results from the biopsy.  You are all truly FANTASTIC people.  Even though we have never met we all have a bond that really no one outside the CF community would ever understand.  I am so proud to be a part of it and to share my life with all of you.  Thank you again for keeping this family in your thoughts and prayers.

I know not only am I thankful for this person but I know Maria is.  Maria is seeing a physical therapist and it is really helping her.  Every week Maria gets to spend some time with whom she calls her sister.  Yes, Maria’s physical therapist and her are basically identical.  Honestly, it is really great to hear how much Maria and her have in common.  Not only do CFers come to this blog but now many others do.  I find it great to be sharing our life with many people around  the world including my wife’s physical therapist.  You just never know where you will meet friends in your life.  Also, thank you for reading my blog : )

I am so thankful for a happy baby. Alayna is really the BEST baby we could ever ask for.  She is always full of life and energy every day.  I hope she continues that for years to come.  I honestly don’t know where the time has gone though.  Soon she will be 11 months and then she will be 12 months!!!!!  I still remember that day that I held you Alayna for the first time.  Something I will NEVER EVER forget!

I have been compliant, committed, dedicated for the past 211 days or 7 MONTHS without missing a single treatment, pill, or neb.  I have to say it’s not easy at all with a baby now when it comes to my CF life.  (That will be another blog topic for another day). I have done vesting at 2 and even 3 am. I really have to thank my wife for telling me to get out of bed.  She knows I am nuts about being compliant.  I am just so thankful that I have been able to keep up with all this CF stuff while being a dad as well.   I couldn’t do it without the motivation I get every day from Maria and Alayna.  These 2 girls make me want to be here for 1,000 years. 

I am so thankful that people in this CF community ask me QUESTIONS! Yes, I know sounds crazy but I love to have people ask me questions about what I am doing for my treatments.  I also have people in the CF community come to me to find inspiration and motivation.  To me that makes what I am doing on blogger, facebook, forums all worth it.  In my eyes, I am just being me trying to give 100% every day I get out of bed.  If I can encourage, motivate, inspire just one person that is what counts when it comes to putting my life and CF life out in the public eye. 

Lastly, I am BEYOND thankful that I was able to shake my recent cold WITHOUT taking any antibiotics.  In the world of CF that is pretty rare.  For us fibros and cysters when we get a cold it really takes a toll on our lungs as we all ready have bacteria in there and then our body has to decide what is more important to fight off the cold or our CF bacteria.  But anyway I am back to 100% John.  I honestly think that has to do with being compliant and nebbing my HTS!!!!

Vesting with Daddy!!