Causes: National Marrow Awareness Month.

November is National Marrow Awareness Month.  I have personally seen the impact marrow donation can make for individuals and families alike.  This story belongs to Mary, the wonderful and strong mother of my good friend, Adam.  It seems that blood donation is common knowledge (which is great, it's important!), but marrow donation is not discussed or considered nearly as often.  I would say the impact one can make by donating bone marrow is difficult to put into words, but Mary proved me wrong.  After following Mary's story and now reading this post, she sums it up well when she says, "Cancer took things from me, but it also gave me things that I never could have expected." Thank you so much to Mary for sharing this journey.  I'm resisting the urge to go on and on in this intro and I will let Mary do the talking.

When Ali asked me to write something for her blog, my first thought was I can’t do this… but how could I say no? So, please bear with me and ignore any grammar issues :)

2011 was fast approaching; I was in the midst of changing jobs, busy with the holidays, and doing the juggle act between being a wife and mother. Life was crazy busy. January came and I was feeling run down. I found myself having to take breaks during my Zumba classes because I was so short of breath. I caught a cold, with a bad cough, head ache, and low grade fevers. After much nagging from my dear husband, Ross, I made an appointment at our family clinic. 

It was Friday, January 21^st, 4 pm appointment. I was seen by a resident, who after examining me thought it was most likely a sinus infection and gave me an antibiotic. He also asked me to have a blood sample drawn since I had been feeling run down. At 6 pm that evening I received a call from the resident. He explained that my blood sample showed some things that were a little worrisome, and they would like me to come in to the clinic the next morning to see a staff physician. The warning bells in my head went off. I have worked in the medical field since 1983, mostly in GI, but I knew enough to know that this was not good. I asked the physician, “what labs were they concerned about?” He told me my white count and platelets were very low. I remember saying "it’s not leukemia right, my white count would be high?” He reassured me it could be lots of things; they would need to do a little more testing to find out for sure what was going on. I remember hanging up the phone, and thinking I have CANCER. I found my darling husband, and told him about the call; he was in total shock as I told him I think I have cancer. I felt numb, I called my sister and told her, as I talked it was like I was talking about someone else. This really couldn't be happening to me. I am only 47, I have too much to do. I can’t have cancer. Saturday morning my husband and I sat and listened as another doctor told us that this looked like it could be a blood disorder, possibly cancer. He wanted us to have another blood draw on Monday morning and scheduled an appointment with oncology. Oncology…the cancer department. We were in total shock. I don’t even remember the ride home.

I come from a large family with 6 sisters, who are my go-to people, my rock. One phone call was all it took and within minutes the rest of family had been notified and the prayer chain and Google searches had begun. It was the longest weekend, waiting for Monday so we could get some answers. I didn’t cry, or break down even once. I had calmness about me, call it shock, or faith, but it got me though that awful waiting period. Ross was a wreck, hovering over me.  My symptoms were increasing fast. By Sunday evening Ross was calling the emergency nurse line because my fever had increased, I had uncontrollable shakes, and my heart rate was extremely high. They told him to continue to monitor me and to bring me to the ER if things progressed. We survived the night. Monday he dropped me off at work, I had my blood drawn. I went to work and started to clean up my work area, it was like I was in nesting mode, like when you are pregnant, and you start to organize everything to prepare for what is coming. I asked my new supervisor to meet with me. We sat in the cafeteria, and I told her I think I might have cancer. I might need to take some time off. I was in robot mode, checking things off the list, still not crying. By 10 am I was feeling awful, so Ross took me home and put me to bed. At 11 am my physician called and told me that I needed to be admitted to the hospital as soon as possible. The blood tests were back and it appeared to be Leukemia. More testing was needed. I was crashing fast, feeling extremely week, and nauseated, I could feel my pulse beating like a drum in my head. I vomited in the hospital lobby waiting room. The first person who saw me was a 1^st year medical resident; he was so calming, and caring. As he was getting ready to leave the room he stopped and smiled at me, and said “do not be afraid, God takes care of those who take care of his people”. I felt this peace wash over me.

The days that followed became a blur of blood test, IV’s, medication, and a bone marrow biopsy. I developed sores in my mouth and tongue, making it hard to eat or talk. The biopsy results confirmed what we feared, Acute Myeloid Leukemia, a very fast spreading blood cancer. They explained my options: chemo alone, giving me a 20-40 percent life expectancy of 5 years; Or chemotherapy, radiation, and a stem cell transplant upping my odds to 50 percent. I remember looking over at Ross and saying well I have always been average so 50 percent sounded pretty good to me.

My chemotherapy started the next day, which consisted of 24 hours a day for 7 days. My fevers soared to 105 degrees, I was hallucinating, and my body shook uncontrollably. They moved me to the intensive care unit, putting me on an ice bed to try to bring down the fevers. My family hovered in the waiting room, praying, feeling helpless. Ross was exhausted, staying with me 24/7.

The transplant doctors were so excited to hear I had 6 sisters who could possibly be a stem cell match. Siblings offer the best chance of survival, and less chance of side effects post-transplant. All of my lovely sisters stepped up to the plate and got tested. It took about 2 weeks to get all the results, we were devastated to find out not one of them was a match. There are 10 things they look for and we needed a 10 out of 10 match. Our transplant doctor, who I think of as my guardian angel, told us not to worry. He would find a match. They contacted the Be the Match Foundation and had them run their registry to see if anyone was a match. I started to lose my hair, so I asked the nurse to shave it off. This gave me a sense of control, when everything else in my life was turning upside down. I just needed to have a say in something.

Cancer is like a snake curling though your body, eating away at you. Chemotherapy is like the Marines coming in to destroy the cancer but killing all that is in its path: The good, the bad and the ugly. It leaves you weak, bald, nauseated, vomiting, anxious, and worst of all you never know what horror to expect next. Cancer took things from me, but it also gave me things that I never could have expected. It strengthened my faith in God. It pushed me to really evaluate my life. It encouraged me to talk to people in my life with whom I needed to make amends, and also with ones that I needed to ask forgiveness from. It reminded me to tell my family and friends how much I loved them. I started to read the bible, and began to build a relationship with my creator. It deepened my relationship with my husband, and children.

Throughout this nightmare I witnessed so many good things. People from all areas of my life began donating blood, platelets, and signing up to be bone marrow donors. It introduced me to so many others fighting the same disease as me; children, teens, men, and moms just like me. Each of them taught me so much about life, courage, and love. Most of all, this experience and the people that I encountered during it taught me about faith.

Fast forward a little while, and we were given the news we were waiting for. They found the PERFECT MATCH! A 24 year old girl from Germany was my match.  We were overcome with joy.

The cancer journey is a lot like the Wizard of Oz.  You’re traveling along the yellow brick road, admiring the beautiful scenery and BAM, out of nowhere you are hit with flying monkeys, a wicked witch, and a whole lot of questions. The good old doctor informed us that he had been worried about my high antibody count and had some additional tests done on my potential donor’s blood, and mine. We were eventually told that the transplant was canceled, because the donor’s and my antibodies would not do well together. The transplant would have most likely killed me.  We felt broken. What now? The search began again for another donor. Another round of chemotherapy began.

Imagine being on a crazy roller coaster ride. That best describes the ups and downs my journey. Each time we were given a little ray of hope, we celebrated. We were excited to think we were at the end of the ride, only to have it take a crazy turn that had us free falling into another nightmare. Cancer teaches you that you really don’t control of much in this life. Those things we treasure are just those…things. Like my pastor often says you don’t ever see a hearse pulling a U-hall behind them.

Late April we were once again blessed. Another donor had been found! This time it was a 20 year old female. A truly PERFECT match. Within a month the process had begun.  The transplant was scheduled for July 15^th; I would have more chemotherapy and 3 days of full body radiation prior to the transplant. This would once again kill off all my cells, and prepare me for the new stem cells. My donor would be given a growth hormone for a few days to increase her stem cell count. Once her counts were high enough they would draw the stem cells by running her blood through a machine which pulled the stem cells out, and then returned the remaining blood to her. The stem cells would be placed in an IV bag, then into a little red cooler and flown immediately to our hospital. I was feeling overwhelmed for the first time since this journey began. I worried about my donor. I kept thinking about what she had to go through.  Would I want my child to go through something like this? I worried for myself also. I was petrified of radiation. I am not really sure why, after going through chemotherapy, how bad could it be? But I was shaken; I asked everyone I knew to pray for me. Our doctor, the guardian angel, sat us down and gave us all the dirty details of the transplant. He explained that there was 25 percent rate of dying in the first 100 days. We would need to stay in a transplant house for this time period to be close to the hospital in case of an emergency. I would need 24 hour a day supervision. Ross had already been away from work since January, and his boss was putting pressure on him to come back to work.

The roller coaster of emotions was running high. My sisters rallied, and put together a list of caregivers which would allow Ross to work during the days and watch over me during the night. I felt so blessed to have such a loving group of family and friends.  At the same time I was fighting emotions of helplessness. I was always the caretaker, the server, but I had been demoted to being cared for. It was humbling.

The hospital stays, tests, medications, and not working was causing a great financial strain on my husband. Once again our friends and family rallied by putting together fundraisers to take some of the pressure off. This too was blessing, and very humbling. Over and over people stepped up to help us. Cancer has shown us there is so much good still in the world. People DO care.

We witnessed the true power of prayer. We didn't get every prayer answered with a yes, and many times we didn't understand why, but after time passed we saw that God had a better plan. This journey has taught me to trust in God’s promise that I don’t need to fear, because I will never be left alone. God will always go with me. He didn't promise life would be easy, but He did promise if I placed my faith in him He would provide me with all the strength and courage I would need to get through anything.

The evening before my first radiation treatment a peace washed over me, and I slept like a baby. Morning came; the escort wheeled me to the treatment area with my husband, son and youngest daughter in tow. As we waited, I felt so much peace.  It was as if my wheel chair was floating off the ground. I can do all things through Christ who strengthens me.

July 15^th, Transplant Day, had arrived. The skies opened, and rain poured down. The clouds were black as night, and streets began to flood. We waited, and worried the little red cooler would not be able to make the flight. My family and friends came, decorated my room with streamers and delivered a birthday cake. We waited. Around 5 pm the little red cooler arrived and the transplant began. With my husband, and 3 beautiful children present, the liquid gold was placed into me. My sisters, parents, and best friend took turns celebrating with us blowing birthday horns and singing Happy Birthday.

In June of 2012, I was blessed with the opportunity to meet my donor and her friends and family. We got a chance to thank her, and to show her what her selfless gift she had given to me and my loved ones. She had given us sweet, precious time.

I do not fear death. I believe this is earth is a stepping stone to something far greater than I can ever imagine. I am so grateful for this precious time. I have learned on this journey that in life there will always be troubles. Some are big, some are small. It is how we respond that will affect the outcomes. We each have a choice with how we react; I choose to be faithful, to live life to the fullest, and enjoy all that is given to me. I choose to make a difference on this earth with every second of time God blesses me with. I want to die with no regrets.

Here is the link to the Be The Match Foundation registry.  It only takes about 15 minutes to register. It consists of signing a consent, swabbing your mouth with a Q-tip (a kit can be sent to your home) and then having your data stored in a database. This database is used by hospitals all over to find matches for patients in need. If you are one of them, the Be the Match Foundation will contact you to see If you are willing to be a donor.  If you still are, they will schedule a set of tests to see if you qualify. If you do, you will be asked to donate stem cells or bone marrow. 75% of donations in the US are done using stem cells.  A donor is given a medication to bump up the stem cells in their blood, which can take 2-5 days. Once the magic number is reached, a clinic will harvest the stem cells. They start an IV and draw the blood which goes into a machine that pulls only the stem cells, and returns the remaining blood to the donor. This takes about 4 hours. For a bone marrow harvest, they collect the cells under anesthesia; it takes about 2-3 hours. You do not have to take the medication prior to this procedure. They insert a needle to collect the cells from your hip area. You wear a bandage for 24 hours, and may experience some aching. Overall, a small price to pay to give someone life!

Be The Match: http://www.bethematch.org/Support-the-Cause/Donate-bone-marrow/Join-the-marrow-registry/


Click here to read the other Causes series posts

Causes: Breast Cancer Awareness Month.

Breast Cancer affects many of us, whether directly or indirectly, myself included.  I have known quite a few people who have/have had it themselves or their loved ones have.  I will admit, I focus more on other causes in October, not because breast cancer isn't worthy or important, but because it's not the only one that is.  My friend, Taryn, was gracious enough to share her story and that of her family with me and now all of you.  How and when breast cancer affects people can vary a lot, just like with many other things, and this is her story.

We all know that October is Breast Cancer Awareness month.  We all see a lot of pink on a daily basis during the month but what does it mean for someone who has been personally affected by this.  I have been affected by this cancer in many ways, my maternal grandma, my paternal grandma, along with great aunts (my paternal grandma’s sisters), and countless friends.  These life touches are on top of the fact that I am a previvor, I carry the breast cancer gene and will one day most likely develop cancer.  I’m sure most people will be shocked that I say that so bluntly but it is my way to deal with it and it is also a real fact for me.  I originally wrote this blog post after Angelina Jolie came forward with the fact that she had a double radical mastectomy.  By no means am I belittling the decisions that she has made for her body but am talking about my choices and my decisions.

Before I get into the facts about BRCA I am going to go back quite a ways on why I decided to get tested and how we found out that our family had the gene (and the shock of the side that had it).

Going back to way before I was even born, my maternal grandma was diagnosed with breast cancer in about 1952.  My mom was born in 1954 my grandma passed away 12 years after my mom was born.  In Nature Outlook Journal there is a statistic listed that from 1944-54 there was just a 40% chance of survival rate to 5 years.  By 2004 that same statistic was up to 85.8%.  

 

 

My mom and her sisters always prayed that they would get passed the age of 52, the age at which their mom passed away.  Her cancer went on to spread to her bones.  They all got passed this age and had mini little parties.  We were always worried about this side of the family.

 

 

In January of 2003 my paternal grandma was diagnosed with breast cancer.  She has been cancer free from that time forward.  My grandma had 2 sisters, 1 was diagnosed with ovarian cancer and died of this at the age of 83.  The other sister was diagnosed with breast cancer at 52 and died at 72.  We do not know if these sisters had a genetic mutation as they passed away before we started the testing.

 

 

My paternal aunt decided that she would like to find out if we do carry the gene.  They started the genetic process after seeing these strong indicator of genetic possibility.  My grandma was tested and she was found to have a deleterious mutation on the BRCA2.  My aunt proceeded to get tested at this point, there was a 50-50 chance that my aunt would have it.  My aunt found out that she had this mutation also.  

 

 

My father decided that he would not be getting tested for this mutation, as what he had to change due to age didn't warrant knowing in his mind.  I decided that I wanted to have my test done.  At this point we didn't know if I would be able to be tested as the link wasn't there for me.  The genetic counselor decided that it would be a good thing for me to be tested.  I came back positive also (this was July of 2011).  At this point we were batting 100%, grandma, my aunt, my father and myself. Grandma's gene's are strong!!!

A maternal aunt of mine went and talked to a genetic counselor and they said that there was a very very small chance that that side of the family having a genetic mutation.

There are many facts and myths out there.  I am going to start with some facts and then go into the myths.

 

Facts:

With BRCA1 or BRCA2 the chance of getting breast cancer is 50%-80% (87%) depending on what statistic that one looks at, general population 7%.

With BRCA1 or BRCA2 the chance of getting ovarian cancer is 27-44%, general population <2 o:p="">

If you are male and have the BRCA1 or BRCA2 gene you have a 6% chance of getting breast cancer compared to .05% of general population.

If you are male and have the BRCA1 or BRCA2 gene you have a 20% chance of getting prostate cancer compared to 15% of general population.

If you have BRCA1 or BRCA2 you have a 2-4% chance of getting pancreatic cancer compared to <1 general="" o:p="" of="" population.="" the="">

You can not be denied insurance coverage because of the genetic testing. You can not be denied a job for having the genetic testing (thou the United States Armed Forces can deny you).

Myths:

I won't have insurance just because of this test, can't happen Congress has passed this as a law.  Fact see above.

Breast tissue is only in your breasts (what is in front).  Fact: Breast tissues goes all around to the back of the body, these are hard places to find tumors if they start there.

What can be done?

In this section I will talk about what I am doing about my positive diagnosis. I will also talk about what can be done.

I have decided to NOT get a mastectomy at this time due to using other surveillance methods.  The mastectomy would reduce my chance by about 90% but as with the fact that breast tissues goes a lot farther than can be removed with a mastectomy.

I have decided to NOT have an oophorectomy (a hysterectomy along with my ovaries) at this time due to wanting to have children. I will most likely have this completed in the next 10 years if not 15 years.

I have also decided to NOT go on any drugs to reduce my risk, drugs such as tamoxifen, my grandma took something like this for a total of 7 years, 2 year of it was tamoxifen and 5 years of another drug after her breast cancer to help keep it in remission.

I have decided that I will get semiannual testing, for myself in February I get a breast MRI, this is due to the density of young breasts.  In August I get my annual mammogram.  I have twice had to go back in for more testing, I am thankful that I have never had to have biopsy but I have had to have ultrasounds.  At my yearly female exam they do an ultra sound to check my ovaries, if anything would come back abnormal there we would get another ultrasound in 6 weeks.  At the age of 30 I will also start getting a blood test to check for ovarian cancer.

If you have made it this far you might be wondering what my hopes for October are. My hopes for October are that we no longer have to have an awareness month for breast cancer that we can stop seeing potato chip bags, lip gloss, batteries and everything else under the sun in pink for the month of October.  I hope that we will have a cure so that we can focus on other awareness’s by no means am I saying don’t buy the pink items, they do help but to be aware of why you are buying pink and to think about it all year and not just for one month. 

Please ask any questions that you have! Also please feel free to share this!

 

Note from Ali: I do not want to turn this into an entire different post, but this is one statistic I did not know about until recently.  While I clearly have no issues with formula feeding babies and toddlers (my own has been thriving on formula since she was 10 months old), this is important for women to know. From the American Cancer Society, "For every 12 months of breastfeeding (either with only 1 child, or as the total period of time for several children), the risk of breast cancer decreased by 4.3%, compared to women who did not breastfeed. Risk decreased by 3.4% for each child breastfed, compared to women who did not breastfeed.  This lower risk did not differ by women's age, race, numbers of births, age at birth of first child, family history, or country of residence."  Read more on this correlation here.

Click here to read the other posts in this series.

If you have a story you'd like to share regarding a specific cause, please feel free to contact me using the contact tab above.

Causes: Pride Month.

I met Taryn early in college.  Our paths crossed in a couple different ways and we've grown much closer in recent years.  As soon as I decided to implement this series on Causes, I thought of her hoping she would write for me.  As luck would have it, she contacted me about it as soon as she read the Causes intro post.  I'm so glad Taryn approached me about doing this post because I have always thought of her as 100% her own person.  She has always been so genuine and kind, and I believe those are two of the most important traits one can have.  I love her advice, "Open your heart and mind and you might meet some truly amazing people."  (Side note: this is an especially personal topic, so I have been hesitant to approach people about writing on it.  If you have a perspective to share here and are interested in doing so, please contact me as I would love to clutter June with Pride posts.  Or, if you have anything to say about any Cause, I would love to hear more.)

What does June mean to me?  There are so many things that June brings to mind, summer time, beers at The Terrace (Go Badgers!!!), spending time outside new beginnings and sad good byes (graduations). It is also a month that brings PRIDE to life.  Pride in so many things but mostly pride for me!  Who is “me”, I am a daughter, a loving partner, a military girlfriend, and I am gay.  June is National Gay Pride Month.  There are so many terms that go into homosexual, gay, lesbian, bisexual, transgender, queer and the list goes on and on.  For this blog post I am going to use the word gay to encompass them all, this is a word that I am comfortable using but not all people are.  These are my own beliefs and feelings and I don’t write for all people.

As I have been thinking about what to write these past few days, I’ve been thrown back to the fact that I have had it pretty darn easy.  As I was scrolling through Facebook I was drawn to the fact that Storme DeLaverie died (May 30, 2014) at the age of 93.  She fought for so much change, she sat for the Stonewall riots and was considered by some to be the Rosa Parks of LGBT rights.  To think of what she had seen in her 93 years of life, not only for LGBT rights but for mankind as a whole.  I feel so very blessed to benefit from her fight and struggles, I don’t have to fight as hard as she did for me to be the person that I am today. 

                I don’t have to hide behind a front with the person I love because we are gay.  My partner doesn’t have to worry about her career because of who she loves any longer.  Not too long ago we wouldn’t have been able to be together at Military functions because of Don’t Ask Don’t Tell (DADT).  I no longer have to worry what will happen to her Military Career because of who she loves.  I still worry about it, I had many friends that we had to be very careful when we were out and about in college because of DADT, and we had to pretend to be straight because it was guilt by association at times also. This wasn’t something that only they had to worry about we all worried about it together.  There were more than a few tears of joy shed that day as we learned it was repealed.  My friends could be themselves and not have to worry about being discharged for it.  While it didn’t happen overnight I am still amazed at how quickly the military has moved with implementation of the changes.

                I live in a state where marriage equality isn’t a reality.  While there is a lawsuit to change that we also live in a state where if we went to a state that allowed gay marriage to get married that we could be thrown in jail and receive a fine for being married.  While this hasn’t been put on the books for us it is also not off the books either.  What would having the right to marry mean to me? It would be security, acceptance and openness.  I would most likely not have to list my partner on health insurance as my domestic partner and have to pay her whole premium for health insurance while those in an opposite sex marriage can just pay the family rate (yes higher than a single rate but not the whole premium).  Acceptance that we are equal in the eyes of the government, our taxes come out the same way and we spend money the same way, yet in many ways this puts us as a second class citizen.  This would also give me the openness and protection for being me and loving who I love.

                I was one of the very lucky ones, I went to a high school that accepted me when I came out.  I moved onto a college that had an amazing LGBT (Lesbian, Gay, Bisexual, and Transgender) Center where I fit in.  It was by no means perfect, we still had our cases of discrimination and gay bashing but we were lucky in many ways.  We had each other and it was a group that worked together to make it inclusive, accepting and in a lot of ways pretty darn fun.  The University of Minnesota Mankato has come a long way, from the LGBT Center being in a closet in the counseling center (yes you can laugh we all did), to having a wonderful full time staff member who fights for us every day.  I watched and listened to so many stories of people who didn’t have it as easy as me.  Many people who were kicked out of their homes or told to not come back after they did come out.  I watched a lot of people come into themselves in that LGBT Center, I came into myself there also.  We worked as a pretty good team and tried our best to watch out for each other as much as possible.

                I look back and think how lucky I have been, I have friends that accept me and I have a family that accepts me and my partner as one of their own.  Not only does my family accept her but her family accepts me also.  While it wasn’t easy for either of our families to come to terms with it, they did and they love and support us.  I have an amazing extended family but my extended family isn’t just blood, I have an amazing support network of friends who are there for us no matter what.  Family isn’t just blood, it is whomever you pick to be your family. 

                Looking back on things that have happened in the past few years makes me realize how far we have come.  I remember saying to Ali when the Supreme Court of the United States overturned DOMA (Defense of Marriage Act), “Save papers and other items to show Myra, she will never believe the fight we have had!” We have come so far, yet we have so far to go. 

                You may be wondering "how does gay marriage affect me, I’m not gay!"  This affects everyone by being a right of equality to no longer have second class citizens.  To show that we are loving and accepting nation.  You also never know who around you is gay, the stereotypes come from somewhere but we don’t all fit them to a T.  Open your heart and mind and you might meet some truly amazing people! 

Click here to read the other posts in this series.

Causes: Cystic Fibrosis Awareness Month.

A friend of mine, Taryn, came to me saying a relative may be interested in sharing their family's Cystic Fibrosis story as part of my Causes series.  My friend connected me with Tami and she graciously shared Emily's story with me and now with all of you.  I love Emily's awareness and responsibility in managing her Cystic Fibrosis, but I wish it weren't necessary.  I couldn't agree more with Tami's question, "Wouldn’t be wonderful if her children got to live in a world where people are only aware of CF as a disease that used to be?"

May is Cystic Fibrosis awareness month. 

My daughter, Emily, has Cystic Fibrosis. 

Diagnosed just a precious few days after birth, she has never, not been aware of CF.  She does not know life without taking multiple medications every day and spending up to two hours every day wearing a vibrating vest to help clear mucus from her lungs.  She is as aware of CF as she is of her own breathing, her own thoughts, her own being.

Today, as we participated in Great Strides, the Cystic Fibrosis Foundation’s biggest fundraiser at Milwaukee’s lakefront, I caught a glimmer of how deep her connection of self to CF is.  The Emcee of the event said something like “Let’s find a cure for CF” and Emily, in a quiet moment between just the two of us looked up and said, “No.”  I wasn’t surprised. She has a tendency to be Little Miss Contrary.  But I asked, “Wouldn’t you like a world without enzymes and your vest every day?”  “Yes,” she replied, “but without CF, I’m not me!”

At age 8, Emily’s awareness of her disease is changing.  She has what some people might find a surprising amount of knowledge about CF.  She can give a pretty good, age appropriate explanation of why she has CF and her sister doesn’t.  She understands that in order for a person to get CF, they have to have received the “Bad” CF gene from their mom and their dad.  (“Bad” is her word in this case.  I have always been very careful to use “Changed” when talking about the gene mutation to my girls, to try to avoid a negative association.  But, go figure, her brain translated it to “Bad” anyway.) She knows that she got two CF genes and her sister may have gotten just one, or none at all.

Emily can tell you that CF causes her body to make too much mucus.  She doesn’t understand, yet, that it is a defect in the movement of sodium and chloride (salt) through the cells that causes the mucus production.  She does know, however, that mucus is where infections like to grow, she must not only wear her vest to break up the mucus, she also needs to inhale two medications through a nebulizer, and use an inhaler, just like Dad does when his asthma and allergies make it hard for him to breathe.  She also knows that even though she never misses a treatment, infections can still grow.  Catching a common cold can bring on a “pulmonary exacerbation” (words she does not know) and land her in the hospital for a “tune up” (words she does know).  Tune ups are 2-3 week hospital stays during which Emily gets IV antibiotics through a PICC line placed in her arm, and respiratory treatments four times daily.  During her first grade year at school, she did this twice.  Her doctors gave her a special 4-hour pass so that she could go to school to sing in her Christmas program, but, she missed a lot of fun End Of The Year activities during  her next stay in May/June. 

Emily knows that she MUST take her digestive enzymes whenever she eats.  At school this year, her teacher simply provides them each day in a small pill case for Emily to keep in her desk.  Emily is in charge of knowing how many she needs (it varies with fat content of the food, but we stick to a general “2 with snack, 4 with meal” rule, with a few notable exceptions.) The enzymes are a necessary part of meal time because the same salt imbalance that cause too much mucus in the lungs causes too little enzyme production in the pancreas.  Emily can explain that the red and white pills she pops down four at a time do the job that the rest of our bodies do, while we aren’t even aware.  In addition to her enzymes, there is a small buffet of pills that she takes at dinner time.  There’s prevacid to combat reflux (a common malady in people with CF, due to incomplete digestion); a special fat soluable vitamin ABDEK tablet because her body cannot absorb these nutrients from food; an extra vitamin D tablet because her annual blood work always shows a deficiency; a probiotic to keep her intestinal tract healthy because God-forbid we have to live the C.diff nightmare again, and azithromycin on Mondays, Wednesdays and Fridays which she takes as an anti- inflammatory agent.  Emily no longer needs to be asked to take her pills.  She just gets the container out of the pantry and does what she needs to do.  She just told me that she’d like a “pill schedule box” for her birthday so that she can load it up on Sunday with everything she needs for the week instead of opening each individual bottle every day.  Some birthday present!

About 4-6 times a year, this daily regimen is joined by oral antibiotics, which she takes in the morning and evening.  They cannot be taken with some of her other medications.   We start a three week course of antibiotics as soon as we start to hear an increased cough or see other signs of a looming exacerbation.  It is a necessary tool in the fight to stay out of the hospital.  Unfortunately, spending so much time on antibiotics can wreak havoc with the gut.  C. diff, mentioned in the last paragraph is an overgrowth of bad bacteria in the gut, made possible by the killing off (from antibiotic use) of the good bacteria that normally keeps it in check.  C. diff is, well, for us it was hell.  It made her a very, very sick baby for a pretty long time.  We had a hospital stay when she was 15 months to try and get rid of it, but it wasn’t an easy fix.   

There are other things Emily knows about her CF.  Like how her feeding tube, which she got at 18 months and still has in her tummy, helps her get extra calories while she sleeps.   Even with the help of digestive enzymes, a CF patient’s body has a hard time getting the necessary calories from a normal diet, and need to supplement.  Emily is encouraged by her doctors to eat a high fat diet.  She does, but the supplement through the tube has been a God-send.  She is absolutely a normal height and weight, and her body has the energy to run, play, do karate and fight most infections.  She can also explain why, even though she hates them, nightly sinus rinses are a necessity.  She has had several sinus surgeries to clean out mucus and remove nasal polyps, commonly found in CF patients.   

It’s a given that Emily is aware of and knowledgeable about her disease.  She hasn’t been given another choice.  None of us has.  CF is a family thing.  We all take turns sitting with Emily while she shakes in her vest; her big sister sometimes carries enzymes in her purse when we go out; we all take part in fundraising events like Great Strides and Climb for a Cure at the US Bank Building.  I have found that connecting with other CF parents (just the parents, though as patients are forbidden by infection control policies from having close contact with each other) helps me to cope with the stress inherent in any chronic disease, so I am active in a Parent Advisory Board for the CF Clinic at Children’s Hospital, and I volunteer at several events each year.

But, we try and make sure that our lives are not ONLY about CF.  Emily does all the stuff that any other 8 year old girl does.  We’ve even begun to navigate the world of sleepovers…not an easy task when you have to take along a couple large pieces of medical equipment and a host of pills.  She loves to play outside with the neighbors and she absolutely LOVES her karate class.  Karate has captured her heart to the point that she re-named our Great Strides team this year.  We became Emi’s Ninjas, and she designed our team t-shirt!  Most of the time, I feel like we do a good job helping her be a balanced, well rounded kid, not just a kid with CF.  When she expresses worry about someday not having CF, though, I have to wonder.  Have we let her become too aware?  Have we pushed her into seeing herself only as a patient?  Does CF comprise too much of her life?

I hope that in time she will be able to understand that SHE will not cease to exist if we should be so lucky as to someday live in a world where CF does not.  I want her to be able to look back on these years and see how CF has made her who she is becoming.  She is responsible, passionate, determined and empathetic because of CF.  But these qualities are only part of what make her the person that we love so dearly.   

There is no cure for Cystic Fibrosis.  Eventually, all CF patients succumb to the mucus that drowns their lungs and other organs, weakened by the stress of not receiving enough oxygen or nutrients begin to fail.  Currently the median life expectancy is 42 years.  It is our dearest hope that a cure will be found long before Emily reaches that point.  Wouldn’t be wonderful if her children got to live in a world where people are only aware of CF as a disease that used to be?

To donate to the Cystic Fibrosis Foundation visit: www.fightcf.cff.com

To read the other posts in this series, click here.

Causes: Food Allergy Awareness Week.

Today marks the start of Food Allergy Awareness Week.  Myra's FPIES has brought us frustration, tears, sleepless nights, many trips to multiple doctors, and a variety of other things.  Fortunately, it has also taught us a lot and introduced us to some very wonderful people, people like Fallon Schultz.  Fallon is the Founder and Chair of the International Association for Food Protein Enterocolitis (FPIES) and has been such a great resource to our family and many others.  Thank you, Fallon, for this contribution to my Causes series and to the FPIES community as a whole.

Working to Make the Unknown Known: Fallon Schultz and IAFFPE

As the mother of a child with Food Protein-Induced Enterocolitis Syndrome (FPIES), I truly understand the joys and immense challenges of raising a child with this rare and poorly understood condition. My baby is now 5 years old and attending kindergarten; he lives a full and happy life despite the hurdles that FPIES still presents.

 

 

FPIES can be a very frightening and confusing condition to deal with, especially prior to diagnosis, as a child can experience severe and unexplained reactions to common first foods and/or formula. FPIES is a serious non-IgE reaction in the gastrointestinal system. Unlike most food allergies that produce immediate reactions such as swelling and hives, FPIES reactions are characterized by delayed, profuse vomiting, lethargy, diarrhea, and even shock. The most common triggers are milk and soy, but any food can cause an FPIES reaction. At this time, little is known about FPIES, and few doctors are able to recognize and diagnose the syndrome. Unlike common food allergies, standard skin and blood testing are routinely negative in FPIES patients. There is currently no cure or standardized treatment. 

As Founder and Chair of the International Association for Food Protein Enterocolitis (www.fpies.org), a nonprofit, volunteer-run organization for families raising children with FPIES, I want parents to know that they are not alone on this journey. I want them to know that there is a world of support and resources waiting for them. I also want them to know that we are working diligently on their behalf to educate physicians worldwide, to develop tools that will ease the day-to-day burdens parents face, and to fund research that will lead to better diagnosis and treatment options.

IAFFPE started in a hospital room in the hours and days following my son’s FPIES diagnosis. Like many other FPIES families, we had been through a long, worrisome struggle just to get a diagnosis. I was scared, concerned for my son and didn’t know where to turn. I was shocked to learn that there were no formal channels of support or information available to FPIES families. IAFFPE was launched in September 2011, and in a few short years, we have taken the lead on a number of initiatives that are bringing real, impactful change to the FPIES community. Here are a few recent highlights: 

·         Last June, we received news that FPIES finally has an official diagnosis code: K52.21. This was a major initiative for IAFFPE, and when this code is enacted it will impact our knowledge, awareness, research, and funding for this condition.

·         IAFFPE is the only organization actively funding FPIES research. In 2013, we funded a new CHOP study that has helped shape our understanding of this condition.

·         We have worked to put FPIES in the spotlight with media coverage from ABCNews and Good Morning America.

·         Last fall, we presented our first national FPIES Education Conference in Philadelphia. IAFFPE is planning more opportunities to bring our community together and offer access to leading FPIES experts.

·         Our Medical Advisory Board has teamed up with other leading FPIES researchers to develop the first consensus guidelines for FPIES. The guidelines will provide a course of action for diagnostic and treatment protocols, research needs, and daily management of the condition.

Every day, we’re in touch with families who live with FPIES. They are resilient and resourceful; their courage and determination is a constant source of inspiration. But there is no denying that managing this condition can be difficult, demanding and isolating. FPIES adds an extra level of stress to our daily lives, from the financial burdens to meal preparation to the impact on social activities and relationships. It’s important to remind ourselves of the impact that this stress can have. We’ve shared on our blog some tips to help manage the fear and anxiety we often feel as parents of a child with FPIES.

 

I encourage families to reach out to us when they are getting started on their FPIES journey. IAFFPE exists to help families feel less alone and improve the quality of life for both parent and child. For those parents who have been in the trenches managing FPIES for a while, we welcome you to get involved and share what you know with others who are new to FPIES. For those who have time and talents to share, we encourage you to get involved in IAFFPE with volunteer or leadership opportunities.

Thanks to Ali @ bettertogether for this opportunity to share IAFFPE’s efforts and help raise FPIES awareness. I invite to you follow us on Facebook and Twitter and visit our website to learn more about FPIES and find out how you can help. 

Click here to see a list of the other posts in this series.