Friday was Maggie-Faye’s latest trip to the Cystic Fibrosis clinic.  She goes every two months vs. the recommended every three months.  This is part of our aggressive approach to her treatment.  The visit went very well.  Her lungs sounded clear, her weight/height is now “off the charts” in a positive way, not in a negative way.  We did her annual labs which came out normal.  Although, keeping an almost-3-year-old still and calm while having her blood drawn is something I would not want to do more than once a year.  That was by far the hardest part of the visit.

During the appointment, Bob and I continued our talk with Dr. Terry Robinson, Maggie-Faye’s pediatric pulmonologist – or as I call him, her CF doctor, regarding her treatment.  I made reference in an earlier post to a telephone call I made to Dr. Robinson regarding Maggie-Faye’s current treatment and the shear amount of drugs we have her on and the validity of each.  He immediately returned my phone call from his vacation.  Amazing.  We continued the discussion on Friday.  He feels that since we are having such success, we should keep doing what we are doing.  He understood my concerns and even said he was glad that I raised them.  Since we will be doing a controlled ventilated CT scan and infant pulmonary function test next year, we agreed to keep Maggie-Faye on her current treatment protocol so when those tests are run, we have a consistent pattern of treatment since the last round of these tests.  (Next year will be Maggie-Faye’s third CT scan (2nd controlled ventilated one) and third infant pulmonary function test.)  Bob and I felt comfortable with that.  Well, as comfortable as parents can be about putting 9 medications into a 3 year old child.

What I wasn’t expecting was the phone call I just received on a Sunday afternoon.  It was Dr. Robinson.  He had been thinking this weekend about the concerns Bob and I raised.  He really wants to make sure we are comfortable with this path we are taking.  He said he has put together all of Maggie-Faye’s information and history and, with our permission, will send it to 4 highly respected CF researchers/doctors around the world.  Two in Europe, one in Australia and one in the US.  I was stunned.  The fact that he cares and is so invested in Maggie-Faye’s care that he took the time to put this together and send it to get other’s opinions blows me away.  He then went over Maggie-Faye’s lab results with me.  Once again, it’s Sunday afternoon.  We are so blessed to have him for Maggie-Faye’s doctor.  

We are having tremendous success.  We don’t doubt what we are doing with Maggie-Faye’s treatment is correct.  We trust Dr. Robinson.  What we are questioning is if what we are doing may cause her problems further in her life.  We must question and make sure that every drug we are putting in her body is necessary and part of the success we are achieving.  As parents, we feel that is what we must do.  We look forward to hearing what the 4 experts have to say about Maggie-Faye’s case.

Well, better go.  Maggie-Faye is pushing me off the computer so she can do her treatments and watch Toy Story 2.

When I first started this blog, I fully planned on posting at least weekly.  Well, as they say…best laid plans…

My husband Bob and I knew that we wanted to have 2 children.  After we found out by having Maggie-Faye that we carried the CF gene, the decision to have a 2nd child became a weighty one.  After much discussion and soul searching, we decided we needed to do what was within our control to keep our second child from having this disease.  After all, if God wanted us to have another child with CF, no matter what we did we would have another child with CF.  

We went to Pacific Fertility Center in San Francisco and met with Dr. Carolyn Givens to discuss In Vitro Fertilization and Preimplantation Genetic Diagnostic testing.  I thought that since I didn’t have any fertility issues this process would be a piece of cake.  Wrong.  It did not take long for Bob and I to realize that having a child via IVF was as much of a miracle as if we had lit a candle, had a glass of wine and let nature take its course.  The procedure itself has a low success rate…then you throw in the fact that I am 41 years old (without fertility issues) and it makes the odds decrease.  We started with a 38% chance that the IVF would work at all.

I followed the same protocol as others going through IVF.  Daily shots, fluctuating moods, emotional stress, etc.  Then it came time to harvest my eggs.  I had more than 18 eggs to harvest, but they were only able to reach 13.  They fertilized them all and 3 days later removed one cell from each embryo to test for CF.  Because of my age, we also tested for Downs Syndrome.  2 days later, we had the results and were ready to transfer the embryos.  Of the 13 embryos, 3 had Cystic Fibrosis and all the rest were carriers.  Statistically very interesting considering that 1 in 4 should have been unaffected.  One of the embryos had Downs Syndrome.  It was decided that 4 embryos (2 boys and 2 girls) were ideal for transfer.  Well, needless to say the thought of quadruplets put Bob and me in a cold sweat.  However, the Dr. gave us some statistics on having multiples and felt strongly that to have a true shot at this working at all, we should transfer all 4.  So, we did.  

This process is not the most romantic.  However, Bob was determined to make it as romantic as possible.  I was lying on the table with my feet in the stirrups when they dimmed the lights to make the transfer.  Bob took my hand in his, looked me in the eyes and proceeded to sing “Let’s Get It On” by Barry White.  God love him.

After the transfer, it was a long wait for the first pregnancy test.  I must admit I cheated and did a home pregnancy test.  I don’t recommend doing that as it told me I wasn’t pregnant.  Turned out…I was!  However, my HCG level was VERY low with my first test.  They told me I had to take another one 2 days later to see if my HCG level doubled.  If it did, that was a good sign.  Turns out, I ended up taking 3 such pregnancy tests.  Each time the HCG level more than doubled.  Now the concern was that I had an ectopic pregnancy since my HCG level was so low to start.  I would have to wait 2 more weeks for an ultrasound.  This was not easy.

The ultrasound showed that we were pregnant with one baby (thank goodness!) and the baby was located in my uterus!  Now, we had to wait another week for another ultrasound to see the heartbeat…and we did!

I now sit here almost 7 months pregnant.  We did an amnio to confirm the process worked and it did.  Our daughter Eleanor Dorothy “Ellie” carries the CF gene, but does not have the disease.  This pregnancy has me tired, plus I have to keep up with a toddler that has CF.  Bob is a true partner with Maggie-Faye’s care and has done more than his share as I have been dragging for the past 7 months.  I don’t know what I would do without him!  I am literally counting the days until I can hold Ellie in my arms…and have my body be mine again.  Can anyone say coffee, coffee, coffee?

So, going through this adventure and all the joys and challenges that pregnancy brings has lead me to not post as often as I planned.  I have laid in bed and written many postings in my head only to be too lazy to get out of bed and post them.  Perhaps the many sleepless nights I have ahead of me will change that.  But for now, I shall go sleep.

I can’t believe it has been over a month since I posted!  Ugh!  Pregnancy is getting the best of me.

Maggie-Faye had her CF clinic appointment on June 26th.  She received an excellent review from Dr. Robinson.  Lungs clear, height and weight spot on.  It was a bit surreal as he kept being pulled away from us.  Turns out, the child in the exam room next door was being sent to hospice.  I don’t know the age of the child, nor do I want to know.  The point is, a child is dying from this disease.  It is 2009 and a child is dying from Cystic Fibrosis.  Not acceptable.  I took a moment and thanked God for the success that we are having with Maggie-Faye.

During our visit at the clinic, we discussed Maggie-Faye’s medications.  We have had no success at getting Flovent in her.  She refuses to do an inhaler.  She will nebulize anything.  But don’t even think about asking her to use an inhaler.  Dr. Robinson asked if we would be open to Pulmicort.  I said, “yes”.

Confession time…I haven’t given it to her yet.

This is the third time in almost 3 years that I have hit what I call “the wall”.  These are times when I stop and think about what we are doing and wonder if it is right.  I will shout from the roof tops every day how I KNOW taking this aggressive approach is the right approach.  However, it doesn’t mean I don’t doubt myself.  I’m having one of those moments.  Pulmicort is a steroid.  Steroids worry me.  However, Dr. Robinson feels that by having Maggie-Faye on a steroid, we could possibly reverse what little damage there is to her lungs.  Where I am having a hard time is that Maggie-Faye is already on so much.  

When I’ve “hit the wall” in the past, I’ve reached out to Dr. Robinson.  He has always been there to talk and provide me information so I can get over these “walls” and continue our aggressive approach.  I’ve reached out to him again and am waiting for his call.  We are fortunate to have a true partner for Maggie-Faye’s CF care.

I want this blog to be an encouragement to others to follow an aggressive approach to fighting this disease.  However, I would be amiss if I didn’t let you know that while it is the right road to be on, it isn’t always an easy one.

Last night Bob put in front of Maggie-Faye her dinner and beverage.  Her response?  Daddy, don’t forget my enzymes!

Another benefit of early diagnosis…she knows no different.  As far as Maggie-Faye is concerned, everyone takes enzymes before they eat.  I just hope she has this commitment to her medicines every day for the rest of her life.  If she does, that’s half the battle.

(Insert heavy sigh here).  She’s growing up…

The day after we received Maggie-Faye’s diagnosis, I went to work.  I am very blessed to work for a company that makes a drug used to treat Cystic Fibrosis.  I spent that day making connections.  I reached out to co-workers for information and scheduled a meeting with the one of the medical directors that was (and still is) a pulmonologist at a CF clinic.  He turned out to be a major resource for information and support throughout my pregnancy.  I Googled and discovered the Cystic Fibrosis Foundation and Cystic Fibrosis Research, Inc..  It was Cystic Fibrosis Research, Inc. (CFRI) that connected me and Bob to parents of children with CF and adults with CF.  One person in particular reached out to us and invited us to her home for dinner.  Her son was in his late 20’s at the time.  She had spent a lifetime traveling the world educating herself on the disease and fighting for the best care for her son.  We had dinner with her, her husband and her son.  That evening was life changing.  We had heard many stories and had received advice on how to approach this disease.  However, that evening hit it home for us.  We were going to fight and face this disease head on as aggressively as possible.  We scheduled an appointment with the Cystic Fibrosis Clinic at Lucille Packard Chilren’s Hospital on the campus of Stanford.  A month before Maggie-Faye was born, we met with Dr. Terry Robinson and discussed how we would approach Maggie-Faye’s treatment.  A team was born that day as our want to be aggressive matched Dr. Robinson’s approach.  We were as ready as we could be for Maggie-Faye to arrive.

Emotional doesn’t even begin to describe my pregnancy.  We went to our first Cystic Fibrosis conference when I was 8 months pregnant.  I wasn’t able to attend all of the events as I just couldn’t handle it emotionally.  There was a little girl there not quite a year old.  She was so little and thin.  Her parents were having to deal with getting her a feeding tube soon.  There was hope at that conference, too.  However, once again I was hit with the unknown.  We wouldn’t know until Maggie-Faye was born how this disease would personally effect us.

I know that we were given a gift when we received an early diagnosis.  I know this every day when I look at Maggie-Faye.  I know we are doing the right thing by approaching this disease aggressively.  I know this every day when I look at Maggie-Faye.  I’m taking time to visit the past ( and will continue to periodically do so) in hopes it may help someone in the future.  Someone who may have just received their child’s diagnosis and stumbled across this blog.  If that is you, know this.  There is hope.

I still remember getting the phone call.  I was at work and wasn’t expecting to hear the results of the CVS (Chorionic villus sampling) for a couple more weeks due to an issue with the growing of the tissue.  We had already been waiting 4 agonizing weeks to find out if our unborn child had Cystic Fibrosis.  When the phone rang and the genetics counselor was on the other end I was already in shock.  Then she told me my unborn child had Cystic Fibrosis.  I can’t think of a word to describe how I felt at that point.  It was the pinnacle of a string of phone calls that had taken me to an unexpected place.  First, I find out I’m a carrier of CF.  Then I get the phone call telling me that Bob is a carrier of the CF gene.  At that point we knew we had a 1-in-4 chance of our unborn child having the disease.  Each phone call was like a knock-out punch.  The best way I can explain it is by saying each time I received a phone call and dealt with the news, it was as if I was building a new muscle I didn’t even know I had.  It would take me a couple of days, but that muscle grew stronger and I was able to deal and recover quicker when the next blow came.  Recently, I found the email I sent to my boss the day after Bob and I found out about Maggie-Faye having CF.  It had not even been 24 hours and I was telling her what Bob and I were going to do to deal with this news and be the best parents we could be for Maggie-Faye.  I don’t know where that strength came from other than from God himself.

When I received the phone call from the genetic counselor, I called Bob and we agreed to both leave work and meet at home.  I probably shouldn’t have driven considering my emotional state.  I just needed to get to Bob.  I was curled up on the bed sobbing when he got there.  News like this can either bring a couple closer together or tear them apart.  At that point, Bob and I had only known each other a little over 2 years and had only been married 8 months.  For us it could have gone either way.  Early in our relationship  when Bob was “wooing” me, he gave me lavender roses.  He had researched and found that lavender roses mean love at first sight.  When he walked in that day and found me mourning the health of our unborn child, he had in his hand lavender roses.  He gave them to me telling me that no matter what, it would be love at first sight for him when he met our daughter.  I knew we would be okay.

We cried a lot that day.  But before the sun set, we agreed we had to figure out a way to get through this.  The future was unknown and for a person that needs control, I found myself having none.  I was scared, sad and overwhelmed.  I didn’t know how I was going to deal, but I knew I had to figure it out for the sake of Maggie-Faye.

I was just on YouTube where I saw the CysticFibrosis USA subscription.  I subscribed and then took a moment to watch the “most watched video”.  It was well done and a great explanation of life with Cystic Fibrosis.  However, like so many videos, it focuses on the tragedy of CF.  Don’t get me wrong, I know Cystic Fibrosis is tragic.  I just don’t need to be reminded of it.  Perhaps it is my way of coping.  

I choose not to think about what will happen to Maggie-Faye one day.  Recently, I participated in a research study regarding being a care-giver of someone with CF.  The questions that were asked revolved around how I cope and what type of support I receive.  Do I attend therapy and if so is it individual or group?  Let me tell you, by the end of the discussion, I needed therapy.  For a good day after that I was depressed.  I guess that is when I first realized that I don’t take time to think about how I’m coping with my child having CF.  

As my mother once said, “crying is a luxury I can’t afford”.  I choose to focus on the positive and the hope.  I choose to focus on Maggie-Faye and her life as a 2 1/2 year old that has to do a lot of extra things as part of her having CF.  I choose not to have CF be the focus of our life and I choose not to think about coping with this.  I guess that is how I cope.  There is a fine line between hope and sadness.  I find by watching just a simple video like I just did can put me over that line.  I can’t do that.  So, I have decided that I don’t do sad.  Maggie-Faye needs happy and positive, especially from me.  There will be times for tears.  But, not now.  Some of you reading this may think all of this is just one big “therapy point” for me.  Maybe you’re right.  But, having this approach gets me through the days and allows me to be the Mother that Maggie-Faye needs me to be at this point in her life.

I was getting an ultrasound Wednesday.  Bob and Maggie-Faye were with me.  The room for the ultrasound is right next to the lab.  As we are waiting our turn, a woman comes in to get some lab work done.  We overheard the exchange between the lab tech and this woman.  The doctor had recommended the woman be tested to find out if she is a carrier of Cystic Fibrosis.  The woman declined the test.

Bob and I sat there in stunned silence.  We both wanted to say something so badly.  However, it is such a personal choice we didn’t want to be intrusive.  But, look what having that test did for us.  Maggie-Faye is doing as well as she is partly because we found out BEFORE she was born.  We just sat there and let the woman leave without saying a word.  Later that evening Bob looked at me and said, “we heard that for a reason”.  He’s right.  There are no coincidences.  We were there and we heard that comment for a reason.  But why?  Should we not have sat there biting our tongues?  Should we have said something then and there that could have made a difference in her life and the life of this or a future child?  I don’t know.  Nor will I ever know.  But I do know this.  Next time, I will say something.

I learned in high school never to say never.  I said I would NEVER go to the University of South Carolina for college.  Ended up that’s all I could afford.  A few years ago I said I would NEVER move for a man and I would NEVER live with a man before I got married.  I did both.  Fortunately, it worked out.  I am happily married to that man.  Oh, and I did have a good time in college.  Now, I don’t ever say never.  Wait…I’ll never win the lottery.  Now, I won’t ever say never.  

Lately, I find myself doing things that I thought I would never do.  Tonight, I found myself talking Maggie-Faye into taking a new medicine.  She didn’t buy it.  I found myself prepared and ready to hide it inside one of her other medicines so that we can get it into her.  I find myself happily giving Maggie-Faye lollipops in the morning and at night as that is what keeps her doing her vest.  At 41 I find myself pregnant with our 2nd and final child.  I thought I would give birth to my 4th and final child in the year 2000.  Hah!  I didn’t even meet Bob until 2003.  So much for girlish plans.  I find myself hearing all about the Swine Flu and not being scared.  If I can face my child having Cystic Fibrosis, I can face anything.  I’m not saying I’m headed to Mexico any time soon.  However, I tend to now handle news without a “knee-jerk” reaction.

Finally, I never thought I would have my own blog.  However, I feel the need to share with others the success that we are having with Maggie-Faye’s Cystic Fibrosis.  Our story can be found in the “About Me” section.  We had our most recent clinic visit on Friday.  At 2 1/2, Maggie-Faye has had no hospitalizations.  Her lungs are clear.  She is in the 75th percentile for her BMI.  Her growth is doing so well, we were told to have her on a “normal” diet with no added calories.  Using this blog, I want to share with others what it is like battling this disease aggressively.  I am hoping others can learn from our journey.

Well, as I mentioned, I’m pregnant and it is way past my bedtime.  

Holly