Archive for November, 2009

I am a mother to three wonderful children.  I submerged myself into motherhood very carefully–one foot at a time.  We made sure we had an income sufficient to support a family and medical insurance to go with that. Austin came first, big, healthy…everything perfect.  As I think is the case with all firsts, Austin was a challenge for me.  Olivia was second.  Olivia was a bit harder than Austin, always fussy and constipated.  But, she was healthy, strong, and always growing.

With Julia, things were very different.  There were red flags from the ultrasound.  I had two ultrasounds, both indicating that she had an echogenic bowel.  We were told that there was a one in several thousand chance that she could have Down’s Syndrome, or a one in several thousand chance that she could have cystic fibrosis.  Naive as I was, I immediately ruled out cystic fibrosis, as we had no family history.  I believed if she had anything that she would have Downs and if she had anything at all there was nothing we could do about it at that point anyway.

I did go home and do a little bit of research on cystic fibrosis because I had no idea what it was.  I learned that it affected the lungs and digestive system.  In the back of my mind I thought that if this new baby developed a cough that wouldn’t go away, then I would get concerned.

Julia came weighing in at 9 pounds 4 ounces.  There was meconium in the amniotic fluid during delivery, so as soon as Julia was delivered, she was whisked out the door to have her airways cleared so none would be aspirated.  Other than the meconium, which isn’t all that uncommon, things seemed very normal.  Julia slept four hours straight that first night in the hospital.  I thought finally–I got an easy baby!  She breast fed perfectly and pooped a lot, which I thought was so great because my Olivia never pooped.

Julia seemed to be just an average new born.  She did get a little cough after a few weeks, but quickly recovered.  She was slow on her weight gain, but not dropping off the charts by any means.  When I visited my parents when Julia was almost four months, my grandmother made a comment that Julia was fussy, but all my kids were that way, so it didn’t faze me.

It wasn’t until Julia’s four month check up that it finally hit me that something was very wrong.  Julia weighed in on that check up at 10 pounds 10 ounces.  She was now in the second percentile.  To compare…Austin was well in the 18 pound range at four months and Olivia was well into the 16 pound range at that age, so to have Julia still lingering in the 10 pound range was very wrong.  After Julia was weighed in by the nurse, we waited for the doctor to come in.  As I sat there, I thought of everything I could tell him that seemed strange to me–salty lips when we kissed her, pooping all the time, still nursing every two hours like a newborn would, spitting up excessively, crying all the time, etc.  I filled him in on my ultrasound and gave him all I could think of in terms of Julia’s behaviors.  I mentioned cystic fibrosis in that meeting.  I could just see the look on his face and I knew that he already suspected it.

He sent us up to Primary Children’s Hospital immediately.  We left the doctor’s office and headed straight up there.  I called my husband and told him to meet us up there and take Austin and Olivia to lunch while Julia was sweat tested.  They sweated her and took blood for labs.  I knew she had CF and was just waiting for the call that followed that evening with the final confirmation.  It was so hard to wait.  I got on line and read all I could about cystic fibrosis.  After all my reading, I truly felt that cystic fibrosis was an awful thing to have…something you wouldn’t wish on your worst enemy.

I got the call around five that evening and the sweat test was indeed positive for cystic fibrosis.  No surprise, but it didn’t make hearing it any easier.  We were to check into Primary Children’s the next morning.  I cried a lot that night.  It should have been just like any other night, but I thought for some reason that Julia might not make it through the night having been starved for so long.  I felt her feedings that night were completely pointless as now I was aware that it was all going in one end and just passing out the other side.

We stayed for two days at the hospital with Julia.  It was like a two day training seminar on cystic fibrosis and how to care for Julia.  The most important thing was to get her on enzymes and to get her gaining weight.  The enzymes were very challenging for Julia.  She was only four months and hadn’t eating anything solid her entire life, so she was gagging down applesauce with enzyme granules on top.  I was afraid she would inhale it into her lungs and we’d end up with a bigger problem.  It took a lot of practice.

Another issue was that I was breast feeding Julia and she needed more calories.  Big dilemma!  I wanted to breast feed, but I knew she needed more.  Being the person I am, I wanted her to have the best of both worlds, so I was determined to get her on a bottle, pump, and add formula to breast milk using the recipe they gave us at the hospital.  That would have to wait until we got home.

My husband and I were very overwhelmed at the hospital.  When we thought there couldn’t be any more to this disease, people would come in and give us more, and more, and more.  For example, when we were getting trained on chest physical therapy, in my mind I would think, “OK, I can do this,” thinking it would only be when she was actually sick and coughing.  Then my bubble would burst when I was told, “No, it would be every day…for the rest of her life…oh and not only once a day, but twice a day, or more than that if she’s sick.”  What!  Are you kidding me!  My bubble was burst so many times like that and I felt like I was drowning.  That’s looking at it from my selfish side…not to mention how a four month old was going to handle all of this.

They didn’t start us on the lung treatments right away.  I was so glad for that.  I needed some time just to get Julia’s diet under control.  We got home and felt completely depressed and helpless.  I cried a lot as I was going through some kind of mourning process.  I went to work on pumping and getting her to take a bottle.  We experimented with every nipple we could get our hands on.  There was a lot of screaming and resistance, but we finally got her to take the Dr. Brown’s bottle.  The nipple was much softer and more pliable than many of the other brands.  What I didn’t like about that bottle is all of the extra parts I had to wash with the whole air vent system.

So, we got Julia taking her Prevacid and Creon 5 enzymes as well as pumping by day and power packing the breast milk.  I didn’t pump at night.  At night I just fed her from the breast.  I figured I needed the rest and felt that what I was doing during the day was sufficient.  Julia was having her cake and eating it too.  I was exhausted, but felt good that she was getting the best I could give her.  I wouldn’t recommend this for all new mothers.  It was very difficult and it would have been much easier just to do straight formula in the high calorie recipe.

A year after Julia was born, the state of Utah passed a law that included cystic fibrosis in the newborn screening tests.  This a wonderful thing, allowing babies to be diagnosed at a very early age.  For me, as hard as it was, I am somewhat glad that I got to see, first hand, the effects of this disease.  I’m not a person that relies on faith to a great extent…I’m more of a ”
show me the money” type of person.  I think it would be hard as a new parent to have a health care professional tell you that your newborn has CF and here is all that goes with that.  It would be hard to make a newborn gag down enzymes if you never got to see the harmful effects of enzyme free living in a person with CF.  For this I am grateful.  CF is very devastating if left untreated.  If your newborn is positive for CF, trust that the medications and treatments are essential for optimal health.  I know because I’ve seen what it does and it’s awful.

This is how our family began our journey with our little CF angel.  Having a child with cystic fibrosis is a very bumpy road.  As if being a mother isn’t hard enough…you throw CF into the mix and you literally have to become superwoman over night.  This journey has transformed me in ways I could have never imagined.  I am a completely different person because of Julia.  She is such a strong spirit and seems to have come programmed to be tough, durable, meek, submissive, patient, and so much more.  She teaches me every day that it’s not about me…as much as I would like everything to be about me…it just can’t be.  I have to force myself to be completely selfless when it comes to this disease and that’s a very hard thing to do.

Having said that, I found it essential that I take a little me time.  I found myself on a downward spiral and I knew that if I didn’t step away from Julia for a bit I would find myself on some medication very quickly.  I began working a few hours a week with my husband’s business to step away and get my mind in a different place.  I also began hitting the gym, taking spinning classes and again, getting my mind in a different place.  I truly felt that if I didn’t do this, I was going to go crazy, literally.  These outlets saved my sanity for sure.

This is just the beginning and I hope the end never comes in my lifetime.  I love my life, my kids, my husband, and all of the crazy twists and turns that come with it.  This is what makes me who I am.