A brief break for awareness

I promise that this blog will not be a vehicle for CF education.  I know everyone who eagerly sent me their e-mails to be added to the party wanted to follow the fun and the magic of our Disney Wish trip.  But I have to take a short break to insert some education for a few reasons.  First of all, today is the first day of May, which also happens to be the start of Cystic Fibrosis Awareness month.  More importantly, however, is that my head is echoing with some comments I have received.   I have had a very small subset of people who have learned about our wish trip that have had a pretty odd and negative reaction.  The reaction was pretty selfish and dealt with a comment that basically indicated that my daughter doesn't even look sick....maybe THEY should get a trip or someone to buy them a house.   I can understand that to look at Alyssa and others with CF it may not be obvious all they go through and deal with, nor is it obvious how serious this disease truly is and will be.  If you know the rest of the story, feel free to skip the rest of today's post, but if not here is a little background.

Cystic Fibrosis is a genetic disease that causes the mucus in the body to get particularly thick and sticky.  Since mucus lines most of the body's organs, it can cause a multitude of problems throughout.   The two most common systems to be impacted by CF include the digestive and respiratory systems.  Sticky mucus fills the airways and sinuses making an ideal environment for dangerous bacteria to grow.  This bacteria multiplies and colonizes, causing respiratory infections and permanently damaging the lungs.  The lung infections are life threatening, needing weeks of IV antibiotics each time.  The lungs can only take so many infections of this magnitude and the body can only handle so much of these high powered antibiotics from which the bacteria are learning how to evolve and survive.    For most CF patients a lung transplant may eventually be the only way to push off death for a few extra years.    The mucus also lines the digestive track making weight gain and nutrient absorption difficult, leaving the potential for bowel obstructions, and destroying the pancreas.  For many CF patients, CF related diabetes is highly likely.   In addition, as they age CF patients develop liver issues, joint problems, and a variety of other life limiting symptoms.

Pulmonary Function Tests are one way to assess the disease

CF is a progressive disease.  There is no cure.   Right now there are very good treatments available to help with the symptoms and to try and keep them healthy longer.   But these treatments are far from perfect, come with their own side effects, are very expensive, and take HOURS out of their every day life.   And even though the median predicted age of survival is somewhere around 36, only half of kids with CF today are actually living past age 18.  We hope that the medicines and treatments will improve this prognosis, and we look to those that are living with CF into their 40s and 50s as a source of hope for the possibilities.

Alyssa was born with something called meconium illeus (MI).  Basically MI is a bowel obstruction from the meconium or first stool.   Almost always MI  is the first symptom of CF, and was what led us on our path toward diagnosis.  Since that diagnosis seven years ago, Alyssa has been hospitalized seven times.  Just before age three we had to have a feeding tube placed to help her gain weight and grow on a normal curve. We had hoped the tube would be temporary, but every time we try to wean from it, she drops weight dramatically.

Alyssa's vest and CF machinery

Overall, Alyssa looks and acts like a very typical seven year old.  You can't SEE her CF.  Part of the invisibility is luck.  Right now the disease is in the background, not bubbling to the surface all that often.  But her cultures reveal that she is colonized with Staphylococcus Aureus in her lungs and we have no idea the damage it can be doing.    The doctors don't do much unless she is having symptoms because they fear other more aggressive bacteria may use the lowered numbers of staph to swoop in and take residence in their place.    There is no way to predict the progression of this disease, so we are lucky right now and hope to stay that way as long as we can.    The other part of the invisibility of this disease is that we work really hard to keep it away.   Every day, twice a day when she is healthy, more when she is ill, Alyssa does two different inhalers and two different aerosol medications (taking over half an hour).  After the inhaled medicines, she hooks up to an inflatable vest that fills with air and tries to shake the mucus loose, adding another half hour to each treatment.   She works hard to fight this disease, even when she feels well.

A sleeping breathing treatment means less

 fighting but is also less effective.

Her disease may be invisible, but its impact on her and our family is not.   Every day we have to find a way to fit in the time for each treatment.   I can assure you that she does not enjoy treatment time and has found very creative ways to try to avoid it daily.  Some days are more of a struggle than others.   We all have to bend to make treatment time fun, even when  Rich and I have our own things to get done or the boys have something else to do, we must stop it all to help her get through the treatment.

CF can be overwhelming and exhausting; and just when you have a routine down and a way to get through the days, CF hands you something new.   From trying to get her to intake 3,000 calories each day to worrying about the germs she is exposed to at school, CF is consuming.  I can't remember a single day in the last 7 years that CF did not play a part in our lives.   It may be invisible for the external observer, but from my vantage point it is never too well hidden.

So when I hear that someone doesn't feel Alyssa deserves this trip (or equates a wish trip for only those that are immediately terminal) my heart just aches.   Wish trips are for kids with chronic and terminal illnesses.  The charities that support wish trips want to make a difference in the life of a child who struggles with a disease that is potentially life threatening.   When a child has a cancer, no one seems to question whether they deserve a wish trip or not.   And if the prognosis improves before or after the trip, there would be nothing but happiness for the child and their family.   These wish organizations WANT it this way, they want the trip to be a source of inspiration to a child, an opportunity to bring hope into their lives and for them to beat their terminal illness.   Regardless of the nature of a child's qualifications, even the naysayers should be rest assured that these kids have been  properly vetted and their doctors had to get involved in this process. . . wish organizations do not arbitrarily pass out these wishes, in fact I have many friends with very deserving children who have not been able to get their child a wish yet.   So we are even more grateful for this opportunity to take a break from the daily grind of this disease and inspire us all to fight it even more vigorously.  In the end its all about hope!!

And with that we return to our regularly scheduled programming.....three more sleeps until DISNEY!!!!

Three EXCITED kids for Disney!!