Husband, Daddy, Pappaw

Today I write with a heavy heart. I lost my daddy, my daughter’s pappaw, last month. He was always the rock, the one that could beat anything. This time would prove to be different.

My parents live in Kentucky. Life's events led my sister and I both away once we were grown and out of high school. My dad had been a heart patient for over 30 years and was most certainly not in good health at the age of 78. He was always the strong stubborn man that always said nothing would take him easily. In 1984, he was one of the first in the country to have open heart surgery involving six bypasses. The first successful open heart surgery was done only 23 years earlier in 1961, so heart surgery was not as common then as it is today. He was scheduled for a triple bypass, which at that time, was very serious, but more commonplace these days. Once surgery started, they found other blockages but since he was doing so well during surgery, they went ahead and did the other 3 bypasses while they were there. He recovered well. He was only 47 years old.

There were many health scares throughout the years for him, including another open heart surgery in 1998 that involved doing another 3 bypasses. Yes, that is a total of 9 bypasses. We all had grown accustomed to the many hospital stays that he would go through. Somehow I knew, though, that this time, this hospital stay, would be different. I am not sure how or why I knew, I just did.

I received the call on a Saturday morning around 11 a.m. I can’t be completely sure of the timing of what followed, as after hearing my mother’s voice, my world just seemed to stop. She had told me that Daddy was in the hospital and that the doctors had told her to call the kids home, that he was in very critical condition. I could feel the confusion, the fear, and the pain in her voice. This had never happened before. Before the doctors had always said that he would be just fine. They weren't saying that this time.

After hanging up the phone with my mom, I took a few minutes to process what she had just told me and then panic set in. This is where things become more of a blur. I know I called my boyfriend, Greg, who also lives in Kentucky. As it turns out, Mom had just called him too and he was already on his way to the hospital and was only a few minutes away. I had always been one that was strong and could handle anything. Remember, I am the mother of a PID (primary immunodeficiency disease) kid, I am used to sudden illness and health crises, but this time I couldn’t seem to function. My whole body was trembling. My hands were shaking so hard that I kept dropping things as I was trying to pack. I then called my best friends, Fay and Tammie. They immediately came to my rescue. They would stop by and pick up Emily, who was visiting with her dad, so I know that somewhere during those first few moments I must have had the sense to call Emily’s dad too and let him know what was happening, although I don't remember talking with him. He had her ready to go when they arrived to pick her up.

I had managed to get a few things packed and once they arrived at my home with Emily, Fay and Tammie helped me finish getting things together and into the car. By a little after noon, we were pulling out of the driveway. The time span was only a little more than an hour but it seemed like a lifetime.  I will forever be grateful for what those two ladies did for me that day and the days that were to come. I could not ask for better friends than those two ladies are to me.

Normally, it takes about 4 hours to make the drive to Kentucky from my home in Missouri. That day, I made the drive in 3 hours and 15 minutes. During those 3+ hours of driving, so much was going through my mind. At some point during the drive, a light switch went off in my head: we are headed to a hospital in the height of flu season and my daughter has PID. What do I do?!!  What was I thinking? Taking her into that kind of environment is crazy! At this point, all we know about Daddy is that he is critical and they are still working on trying to figure out what exactly is wrong. Greg had been calling me periodically to give me updates, during one of those phone calls I asked him what he thinks I should do about Emily. We both decided that we would leave it up to her as to whether or not she wanted to go into the hospital. She was very adamant about seeing her pappaw. Greg checked with the nurses to see if he could get some surgical masks so that Emily could see her pappaw. The nurses were more than willing to supply them. We decided that when I pulled into the hospital parking lot, I would call him and he would meet us at the door with the masks.

Before that day, I had always erred on the side of caution when it came to Emily visiting someone in the hospital. The risk to her for infection is very high. When she gets sick, it is much more severe than in people with normal immune systems and lasts much longer and can be damaging to her body's organs, but I just couldn’t deprive her of seeing her pappaw. Emily was absolutely determined to see him. She didn’t care if she had to wear a mask, she wanted to see him and that was that.

Emily and Daddy had a very special bond. My daddy, loved all of his grandkids with all his heart. He was so proud of all of them. His face would shine when he would be talking with someone about any one of them. He has five grandkids, but out of the 5, Emily is the youngest and the only girl. She was his “Little Emmy” and always will be. She had him wrapped around her little finger and he had her wrapped around his. Their favorite thing to do, when we were visiting, was to go into town and get Slushies, which somehow always involved getting more than just a Slushy. There always seemed to be sunflower seeds (Emily’s favorite) or gum or candy that came along with the Slushies.

We arrive at the hospital and are met at the door with masks. Emily promptly puts one on and we are headed to Daddy’s room.  We walk in the door to his room and Emily goes right to his side. His eyes brightened and a big smile came to his face and he said: “Hey, there’s my Little Emmy”.  He was so happy to see her as he always was.

The next 3 days were long and hard and really seemed more like a bad dream than reality. No matter what the doctors tried to do, it wouldn’t help. Emily would go and sit with him for several hours each day, always wearing her mask. On the 3rd day in the hospital, Daddy seemed better. He was sitting up slightly and actually ate a little bit. Emily was right there by his side watching the evening news with him, holding his hand. She would hold his cup of water so he could sip through the straw without spilling it.  After Emily went home with Greg for the night, things seem to change with Daddy. I can’t really explain what it was but a very uneasy feeling came over me. I reluctantly left that night to get some rest. We all were functioning on very little sleep. The plan was that I would get some rest and then when I got back, my sister would go get some rest. Mom absolutely refused to leave his side. They had been together for 55 years and she was not about to leave him at all.

Early the next morning, I am up, showered and getting dressed. I receive a text from my sister saying “Come Now!”. Obviously alarmed, I immediately leave and tell Greg that I will call him when I find out what is happening. When I get to the hospital, Daddy was failing. The time was drawing near and I believe Daddy knew it just as much as we did.  Emily and I had already had this discussion and even though she knew it would be hard, she wanted to be there with him when it happened. I call Greg to get her there quickly. While waiting on Emily, we, my mother, my sister and I all have a special moment with Daddy. I thank God that we all had the chance to say our I love you’s and our good bye’s.

I am not sure if anyone else noticed what I am about to tell you other than myself. Daddy was fighting, he was struggling to hold on. After Emily arrived, she went right to his side and held his hand. He turned and looked at her and whispered “I love you Emmy” and she told him she loved him too. They had a moment together and then he kind of drifted off to sleep and within 30 minutes his heart stopped. I truly believe that my Daddy fought and held on until he had a chance to talk to all of his girls, my mother, my sister, myself, and his “Little Emmy”. I truly believe he held on to tell each of us, especially Emily, that he loved us, and then he gave in and went to Heaven.

Emily wanted to be included in all of the arrangements.

She participated in all of the planning, even picking out old pictures to display on a video and posters at the funeral home. Fay, who I mentioned earlier, and her daughter, Addison, who is Emily’s best friend, made the trip to Kentucky to attend the funeral. Addison was another favorite of his. Daddy loved Addison as one of his own and I was so grateful she was there with Emily. During the funeral, Daddy’s brave “Little Emmy”, with Addison by her side, stepped up in front of everyone and read the poem “Do Not Stand At My Grave And Weep”. She stood there so bravely, so strongly, just like her pappaw. I was so proud of my little girl and her best friend.

I will never regret the decision I made of allowing Emily to spend those last days with her pappaw, even though it was at a high risk to her own health. Those last days together were more precious than anyone could imagine and I am so happy to have had those moments. There are so many that don't get those moments and I thank God for allowing that time for us. I thank God for allowing the time for my Daddy to be able to tell his Little Emmy that he loved her. Those were the last words she heard from her pappaw and I can't think of anything better.

Family

If you look in the dictionary, you will find the definition of family is referred to most as a group that is related by blood or marriage. As you read further down the list of definitions, you will find a definition that states a family is a group of things related by common characteristics. In the world of chronic illness, the word family can take on many definitions, leaving me to ask the question: How do we determine who becomes a part of our family? Through our journey with PI (Primary Immunodeficiency Disease), I have found that my daughter and I have several families, some of which might not fall under the traditional definitions.

Family of Non Believers

Although we have been on this journey for 9 years, we still have those, friends and relatives alike,  that do not believe that this disease is real. I try to look at things from their perspective and I do realize how it is that they come to believe the way they do. PI is a rare group of immune system disorders with very common symptoms. The commonality of those symptoms is what makes it difficult for the mainstream public to understand. I get this! In the beginning, it was difficult for me to understand as well. My daughter kept getting sick with common illnesses such as ear infections, strep, pneumonia etc. She was in daycare thus exposure to the many germs that young children can come in contact with was large. It was easy to dismiss the frequent bouts of sickness as "a child in daycare is going to get sick". When we were finally referred to an ENT,  we were made aware of a possibility, that blood work later confirmed, that this could be more than just "a child in daycare is going to get sick" thing . People will believe only what they know and understand. It took many hours of research for me to understand and come to terms with what this disease meant and that this disease could become a life long battle for my daughter. I don't expect everyone that we know to do this same research just to understand what my daughter's diagnosis means. If PI garnered as much research and publicity in the mainstream media as other diseases such as the many different types of cancer, Alzheimer's, Parkinson's and many others, I believe the thought that such a disease as PI is not a real diagnosis would go away and the family of non believers would begin to get smaller. While I do not mean to imply that the diseases I have listed above are not worthy of publicity, it is the fact that the research and publicity they have received have made them recognizable terms within the general public and people understand now how they affect the patient and the caregiver. Ask some stranger walking down the street what Primary Immunodeficiency Disease is and the answer will be: Huh? Never heard of it. This is what needs to change and also what makes this group of family members important because they keep me motivated to teach and preach the world of PI.

Family of Medical Professionals

The immune systems affects several of the body's organs thus making it necessary to build a trusted family of medical professionals. This disease makes a patient more susceptible to common infections as well as other diseases such as Leukemia and Lymphoma and autoimmune diseases like Lupus, Rheumatoid Arthritis and many others. I mention these specifically only because these are the diseases that my daughter has already been tested for and is on the "watch list" to be monitored against. The list of doctors that she sees is ever growing. This list, so far, consists of a pediatrician, ENT, gastroenterologist, orthopedist, rheumatologist, psychologist, and immunologist. Some PI patients have more specialists while others have fewer. The fact remains, however, that these people become a big and important part of our family. Without this family of  "ologists", my daughter would not be healthy. I don't even want to think of where she would be without these wonderful family memebers.

Family of Social Networking

When a diagnosis is made of a rare unknown disease, a patient or caregiver is left devastated by the news and the search for information begins. With the Internet, there are many articles to be found with information on just about everything but just how reliable is all that information? The information gained from trusted resources is invaluable to a patient or caregiver. The information provided by such organizations as the NIH, NORD and IDF, to name just a few, is priceless. But who is a more trusted resource of information than a fellow patient or caregiver struggling with the same disease. Social networking has allowed these otherwise strangers to get "connected" and become a part of our family. I have gained as much knowledge from our social networking family as I have our medical professional family. This family can help guide me when something arises that I am unsure about. I can post a question and instantly get advice as to how they have dealt with similar issues. I can read other posts to learn what may lie ahead for my daughter thus allowing me time to prepare for the "what ifs". This family has taught me how to maneuver through the medical world and stay on top of this disease and has helped me to gain the knowledge and strength to help my daughter get through each illness that comes her way. This family, like the medical professionals, is an important part of our lives.

Family of Compassionate Friends

Everyone has friends. There are friends and then there are friends who truly understand our circumstances. This group of  family members consists of those people who are not related by blood or marriage but have a place in your heart as if they were. This family is made up of the people that have taken the time to read, research and understand the disease they are now connected to through friendship. This family is made up of the people that have taken the time to lend a helping hand or have offered a shoulder to cry on or just the time to allow a patient or caregiver to talk, yell or scream and have a moment of breakdown without judgement. These members of our family find ways to uplift and keep us strong. This family gets involved and helps keep the focus on fighting this disease and providing an emotional outlet when needed. I could go on and on about what this family means to the patient or caregiver but there is not enough words to describe their importance.

True Family

This family is the one that is closest to us. Our husbands, wives, parents, grandparents, brothers, sisters, aunts, uncles. This family is our step parents, step brothers, step sisters, our partners and our significant others. These our the closest members of our overall family. This group of people, like the family of compassionate friends, sees us at our worst and at our best and everything in between. They are right there beside us every step of the way. They celebrate the victories with us and they console us with our defeats. They are the first ones we reach for when times are good and when times are bad.  This group is our first line of defense against this disease. Without this core group of people, life with a rare chronic disease would be a much more difficult road to travel. Their love and support is unconditional which makes them the most important of all the families.

As you can see, family can mean different things to different people. This is our family and I love and treasure each and everyone of them. From the Non Believers who keep me motivated to educate and promote awareness, to the medical professionals who use their skills to care for my daughter, to the compassionate friends who provides the focus and emotional outlet, to the True Family who keeps me grounded and my spirit alive, our lives would not be complete without any of them.

How Do You Do It?

The comment "Girl, I don't know how you do it." has been said to me many times. So I decided to respond to that statement in this blog.

As all parents know, parenting any child has it challenges. Parenting is filled with ups and downs, right ways and wrong ways. None of us get it right all the time. We all make decisions every day to help our children be happy, healthy and successful. Parenting a child with Primary Immunodeficiency Disease isn't really that much different than parenting a "normal child" when you look at the whole picture.

When my child was a baby, I changed countless diapers and got up in the middle of the night for the middle of the night feedings and took my naps when she napped, just like any other parent does. I doted over her and shared her pictures to all that would see, just like any other parent does. I have been the dance class mom, the soccer mom, the softball mom and the myriad of other activities that a child can enlist in, just like any other parent. I have sat with her and helped with homework and have discovered that I, in fact, am not smarter than a 5th grader. We play with them, we do projects with them. We are their chauffeur to their many activities and birthday parties. We become their doctor and nurse in times of illness. We take care of their needs and most of their wants. We laugh, we cry, we worry, we love them with all that we have. We do all this because of our love for our children and our deep desire to teach them the things they will need in order to face the world on their own. So you see, when you look at the whole big picture, my job as a parent is no different than anyone else.

One of our projects while at home recovering from strep.

It's only when you break that big picture down to smaller snapshots that it changes a little. I have snapshots of helping to hold her down to have IV's put in her arm because she is sick with pneumonia, but I hold her down because that's what is needed to help her get better. I have a snapshot of her being carried off for surgery to put tubes in her ears because she has had 7 ear infections in 4 months, but I do that because it will make her better. I have a snapshot of her being wheeled away to have her tonsils removed because she has had countless bouts with strep, but I do that because it will make her better. I have snapshots of the countless blood draws over the last 9 years but I do that because I know its how her health is monitored to make sure I can do what's best for her. I have snapshots of more hand sanitizer and disinfecting sprays than any one human should own. If smell could be incorporated in a snapshot it would smell of bleach, the ultimate house cleaning disinfectant. There would be many, many snapshots of doctor visits and ER visits. There would be snapshots of daily medicines. Snapshots of meetings with school administrators and teachers about 504 Plans. I do all these things because I know it's what will help her get better.

The statement, "Girl, I don't know how you do it", should actually be directed at my daughter, for it is she that gets sick all the time. It is she that gets stuck with the needles all the time. It is she that has to go to the doctor and ER all the time. It is she that has to take six medicines daily, every day. It is she that has difficulty waking up in the morning because her head and body aches all the time. It is she that, even though some days are harder than others, she gets up and she gets through the day, every day. You see, she is the amazing one to endure the life of chronic illness. All I do is what any other parent would do: I support her in any way I can. I remind her to take her meds. I drive her to the doctor or ER. I am there for her when she cries because some days it gets a little overwhelming. I do what ever is needed at any given time.

When placed in a world that decisions have to be made daily to protect your child, a parent will do what is necessary to protect their child without a second though about it. Even though, my snapshots may include much more medical mumbo jumbo than the normal family, I do no more for my child than any parent would do for their child if faced with the same circumstances. Only our daily routines are different but the ultimate goal is the same: you do what you need to do to raise a happy, healthy, ready to face the world child.

Our Friends, Our Village

Recently I wrote of true friendship and how it takes a village to help raise a child with chronic illness. This became so joyfully apparent a few weeks ago in my daughter's fifth grade class.

Almost immediately after the start of the school year, my daughter starting getting sick. It was the normal stuff; colds, sinus infections and I am pretty sure, every viral respiratory infection known to man that Primary Immunodeficiency Disease brings to bare. Every two to three weeks we were visiting the doctor's office or the urgent care facility and even some emergency room visits. By October, I had lost count and started trying to think of ways to protect her yet keep her in school. I struggled with the idea of possibly sending her to school in a surgical mask.

I struggled in my own mind of how wearing a mask could affect her. We had educated her teacher, and through him, her classmates, of some of the things she needed to do to help keep her from getting sick; disinfecting her area after changing of classes, frequent use of hand washing and hand sanitizers. Her classmates had grown accustomed to those procedures and had accepted them; but would they accept a mask?

I sat down and talked with her about my concerns and the idea of wearing a mask. She is only ten years old but I felt as though this was a decision that she needed to be a part of since it would certainly be her to endure any teasing that might occur. So we had a long talk and ultimately agreed it was time to try something new.

The school had been wonderful so far in accommodating her needs and helping with ideas of how to keep her infections at a minimum. I placed a call to her principle and also texted her teacher that the following Monday I would be sending her to school wearing a mask and hoped they could help with any teasing that might occur. Her teacher texted me back and asked where he could get masks. I told him the pharmacy and wondered what he was up too. I had a suspicion that he was going to ask the class to wear the masks as well, but would the kids really want to do this for my daughter?

Monday comes and before we leave the house for school, I ask her if she is ready. She assures me she is. She is a little nervous but is ready to tackle the day. Not long after I dropped her off at school, I received a text from her teacher asking a favor of me. He had left a bag from Walgreens in his car that had been left at the repair shop that morning and wondered if I would go and pick it up for him and bring it to class. My suspicions grew. I did as he asked and when I delivered the bag to school, he told me he had purchased masks. So as not to influence the class, I delivered the bag and promptly left but requested he send me a picture of whatever resulted from his plan.

Nothing could have prepared me for the text I would receive! Almost every child in my daughter's fifth grade class had chosen to wear the masks and support Emily in her battle to stay infection free. The picture he sent was absolutely amazing! All I could do was sit there and stare at my phone and cry. The kids wore the masks all day with her. My daughter was so excited. She greeted me that afternoon with a smile on her face and telling me all about how the kids wore the masks with her. There were even some kids that finished out the week with her. There were kids from other 5th grade classes that were asking to wear the masks. I was completely amazed.

I posted the picture on my Facebook page and it went viral, now having been seen all over the world. It was chosen as Facebook Picture Of The Day on one of our local television stations and a story has been printed in our local newspaper. I am so proud to know these students. I am so proud to be a part of our community.

There have been many reports in the news of random acts of kindness. I can not express what this act of kindness has meant to us. This random act of kindness was so much more than a financial gift or service, it was a gift of inclusion. A gift of acceptance and the willingness to assist in a simple yet grand way to keep a child healthy. I think there is much to be learned from this 5th grade teacher and his students.

These are our friends and this is our village.

Friendship: The Best Thing In Life

This is a story of friendship. True friendship. One that has never wavered no matter the circumstances. One that has endured the many illnesses, cancellation or rescheduling of activities. A friendship that when one is hurting, be it physical or emotional, the other is hurting. This is about my daughter and her best friend and what they have taught me through the years.

They were only about 3 years old without a care in the world when they met. They met in preschool and become the best of friends. They couldn't wait to get to "school" so they could play together. They were inseparable.

As luck would have it, they would be in the same kindergarten class. Their friendship blossomed so much so that it was determined that going forward it might not be a good idea to have them in the same class together. It was heartbreaking to them at the time but we laugh about it now. It seems they were a bit too chatty.

Now they are 10 years old, 5th graders, and beautiful young ladies. Believe it or not, still as close as they were then. They are more alike now than they were when they were little. They enjoy many of the same things, mostly computers and Nintendo DS. They admire each others individual abilities. They watch out for one another and take care of one another. When one is having an issue, the other is always there to help. When shopping, "Do you think she will like it too?" is often the question asked. Another common question is "Can she go with us?" or "Can we have a sleepover?". So as you can probably already tell, I don't just have one daughter, I have two and I love every minute of it.

Their friendship can teach people many things. Through our journey with Primary Immune Deficiency Disease, their friendship has endured. Although disappointment does happen when plans need to be changed, there is understanding.  When sickness is at hand, there is compassion. When there are delays due to doctor appointments, there is patience. Most of all there is empathy for the disease that is a part of not just one, but both of their lives.

Primary immune deficiency doesn't just affect the individual that has been diagnosed, it affects their friends and family as well. Through it all, these two girls have learned that it is better to accept one another for who they are and to love each other in spite of the disease that makes them different. If all people could be as understanding as these two are with one another, the whole world would be a better place.

We should all learn what these girls have been living since they were 3 years old

:
Be accepting to one another for who they are.
Be caring to one another not only in actions but in words as well.
Be considerate of one's situation.
Be understanding and not judgmental.
Be most of all love one another for God made us all, he just made some of us a little different than others.

What Do I Do Now? It Takes A Village.

As parents, we watch our children growing up and we think of how we would like their lives to be. We often think of their first day of school. We think of who their first boyfriend or girlfriend may be. We think of high school and prom. We think of their first car and where they will go to college. Who will they marry, how many children will they have? While they are still babies, we have already thought of all the wonderful possibilities that life will bring to them.

One of the worst things a parent can ever experience is a doctor telling them that their child has a chronic, incurable disease caused by a genetic defect in the immune system, primary immune deficiency. A disease that renders their body at greater risk for infections of all kinds, big and small. A disease that even the simplest of colds could potentially turn into something more serious such as pneumonia. A disease that causes repeated infections that increases the risk of permanent organ damage, cancer and even death. There truly is nothing worse than being told your child is "sick" and may continue to be "sick" the remainder of their life.

So what does a parent do when given such news? The first thing you do is cry. Then you cry some more. That's right, you take a short time to have a pity party just for you and only you, it's ok. Life will be changing and you need to harness all the energy you have to do whatever needs to be done.

Next, the questions start popping up in your mind. Why did this happen to "my" baby? Was it something that I did wrong?  What does this diagnosis mean? How will I keep them from getting sick? There will be many questions that you will ask yourself but the most important one will be WHAT DO I DO NOW?

The most important thing you can do is educate yourself and everyone around you. You have already experienced the long road to diagnosis. For some, diagnosis can come fairly quickly; for others, it can take several years. I'm sure you have already experienced some of the stigmas associated with chronic illness. Although I truly believe most people do not realize that their response, or non-response to what you and your child are experiencing can be hurtful, it really can leave you feeling isolated and alone.

You are about to become a doctor, a nurse, a counselor, an insurance expert and advocate by default and it will be so important for you to know and understand your child's specific diagnosis. You will need to learn what steps you need to take to help keep your child healthy. You will need to learn what your child's needs are going to be in the school setting and meet with school administrators to educate them on those needs. Know and understand your child's right to an education. Work with school personnel to implement a healthcare plan for your child while at school.

Family and friends will also need to know and understand the needs of your child. Grandma needs to know that if she is sporting a simple cold; she may need to postpone a visit until it has been resolved. Your child's friends need to understand the importance of hand washing before and after play. Your own child needs to understand what they need to do to help themselves as well.

Caring for a child with primary immune deficiency can be very challenging. You want to keep them healthy but also want them to be a kid. Surround yourself with supportive people. People that will listen when you feel like screaming. People that will offer thoughtful advice in times of doubt. People that will rejoice with you when things are going well. People that will lend a shoulder to cry on when you feel you have lost control. Most of all, surround yourself with people that will help you help your child stay healthy and be a child all at the same time. It was said once that it takes a village to raise a child; no statement could ever be more true when that child has been diagnosed with a primary immune deficiency.

Ready For Back To School? I Hope So.

I can’t help but feel mixed emotions with the start of the new school year inching ever closer. I am excited to watch Emily get ready for her first day of middle school, yet saddened at the same time that my baby is old enough to be in middle school. I am excited to see her rejoin her friends, some of which she has been unable to see during the summer break, yet worried at the same time of what that rejoining will bring.

We had a wonderful summer break with sleepovers and a vacation to Kentucky Lake. I’m not ready for it to end. She has stayed relatively well all summer. No major illnesses to deal with other than the occasional asthma attack and one sinus infection. We even got a definitive diagnosis from her immunologist and started a treatment plan. I’m not ready to send her back to the jungle of germs, but I must.

So with school supply list in hand, off for a day of shopping we go. One by one the items are checked off the list. Now we switch to another list, mom’s back to school list: medications refilled, hand sanitizer and sanitizing wipes on hand. She is ready for fifth grade, but mom isn’t ready.

The last couple of weeks of summer break will be filled with meetings with the teachers and school administrators. We now have documentation from a doctor explaining her disease and a mound of educational supplies provided by IDF (Immune Deficiency Foundation) to supply each of the teachers that will have her in their class. I just hope that they actually read it. Armed with my tote bag filled with information, I will meet with her teachers, her principle, her guidance counselor and the school nurse. The nurse and I have come to know each other quite well over the last couple of years. We will develop a 504 Health Care Plan and have everything in place to start the new school year. I have even prepared and stocked her school bus with hand sanitizer and sanitizing wipes, it helps that I am the bus driver. I have done all I can do to prepare Emily and the school, but I’m not prepared. I’m not ready.

Last year, within the first week of school, Emily came down with strep. By the end of September, she had already missed eight days of school. Some of which due to the fact she ended up having to have surgery to have her tonsils removed. Will this year be a repeat of last? I hope not. She is taking the prophylactic antibiotic to help ward off infections. Will it work? I hope so. Will the school actually do everything they said they would do to help keep her healthy? I hope so. Will the year go by with little illness and fewer days missed? I hope so. Have we thought of everything possible that we can think of? I hope so.

Only time will tell if we have prepared properly. All I can do is hope so.

Dona Darr

Our Primary Immunodeficiency Disease Journey 

Our journey starts in January 2003. God blessed me with a beautiful little girl. I had no idea the joy and love I could have in my heart until she arrived in my life. She was so tiny, at least to me. I always worried if I could ever be the mom she deserved.

Emily grew so fast. It was obvious from the beginning that she was going to be a tall and skinny girl. At every check up she would be in the 95% in height and 25% in weight. Emily was also a happy baby. I don’t believe I have ever seen a baby smile as much as she did. Rarely cried; and if she did, there was a diaper to change or a bottle to give.

At 18 months of age, I realized that it was time to allow her to explore the world. I had been fortunate enough to be able to stay home with her from the beginning but knew it was time to let her start learning how to play and make new friends. To this point I had been her only playmate and I knew it was time to “expand her horizons”.

I enrolled her in daycare. I worried she would not adjust well. I had been her only playmate for so long. Boy was I wrong! It only took a couple of days of crying at departure. After that, she would run to the daycare door as soon as I lifted her from her car seat saying: “Cmon Mommy, I wanna play!” She loved going to play with her “fwends”. To this point, she had always been a happy healthy baby girl, but that was about to change.

I had enrolled her in daycare in August, shortly thereafter, she began getting sick. She had been diagnosed with an ear infection, and then another and another. Her pediatrician indicated that it was normal for a child in daycare to be sick a lot. I knew something just wasn’t right. By December, she had been diagnosed with seven ear infections. I challenged the doctor as to why so many infections. The answer: children in daycare get sick. I understand children get sick in daycare but seven infections in just four months; I just knew something wasn’t right.

After talking with a few friends and family, I decided to take her to see an ear, nose and throat doctor. I wanted to just make sure everything was as it is suppose to be. Our first visit was right around Christmas time. I went into the appointment, expecting to be told she was fine and that children in daycare get sick. But after I had given then doctor the history of infection and antibiotics she had been on, he stops and turns and looks me in the eye and asks: “Has she ever been tested for immune deficiency?”

I was stunned. What? Seriously? What the hell is immune deficiency? He briefly explains that children with immune deficiencies usually present early on with recurrent ear infections and that I should get her tested. So we finish the appointment with a surgery appointment scheduled in January to have tubes put into her ears. The ENT states that he will send an email to her pediatrician concerning his thought of immune deficiency.

January comes and eight days after her second birthday we are at the hospital. They come to take her back to surgery. I am terrified! I know its just tubes for the ears but this is my baby girl and they are taking her away from me and I am not allowed to go with her. I think I cried the entire time she was gone. Silly, I know, but she is my baby girl!

Finally, after what seemed like forever, they bring her back to me. It really had only been about half an hour but seemed like a lifetime. She was crying from the anesthesia and the sound of it was absolutely glorious.

Later that January, I follow up with her pediatrician concerning the immune deficiency thing that the ENT had mentioned. She confirmed that she had in fact received an email from him and placed the order for blood work testing. I had no idea what to expect. How do you draw blood from a squirmy little two year old? Well I was about to find out and it was the worst experience of my life! It took four of us to hold Emily still enough to draw the blood. I felt like I was the most horrible mother in the world that day. The fear in her eyes as she looked at me screaming while I helped hold her body still enough for them to do what they needed to do. Little did I know that day that this procedure would become a common event in her life.

Two weeks later, the results were in. I received the phone call from her pediatrician that would change our lives forever. It had been confirmed that Emily’s IgG levels of her immune system were in fact low. IgG makes up the majority of the body’s ability to fight off infection. Her levels weren’t horribly low but she is going to be more susceptible to infection than other children. I was floored! I did not understand and did not know what questions to ask all I could think was: “What do I do for her?”  The doctor told me that I should always call if Emily gets sick. The most important thing is to catch any bacterial infection as early as possible so it can be treated immediately. Then she told me that it is very important for her NOT to get the flu or pneumonia, as those could be very dangerous for Emily. Oh and by the way, Emily’s body did not respond to the pneumococcal vaccines she received as an infant. This could explain why she is becoming sick so often so we need to vaccinate again. I hung up the phone in disbelief.

For the next two years there were countless trips to the doctor’s office and emergency room. Many of the illnesses were viral, of which there is nothing one can do but let it run its course. However there were several bouts with strep throat and sinus infections. All along, the doctor treated each illness individually. After so many trips to the doctor's office, I started to feel as though they were getting frustrated with me, but the doctor had told me that she wanted to know every time Emily had a fever. I guess she didn’t realize that it was going to be every two to three weeks. Complacency was setting in.

Late one Friday night, Emily fell sick suddenly. Fever was around 100 and throwing up. I called the doctor’s exchange, they already new me well there. They told me to give her Tylenol and Motrin alternated every three hours for the fever and to offer small sips of water over night and to bring her into the office the next morning. The next morning I am waiting at the office doors as they open, and the welcoming committee was not particularly happy to see me.

The doctor looks at Emily and decides that its just viral, lungs sound good, nothing to worry about. Take her home, let her rest and drink plenty of fluids. If her fever spikes to over 101 then take her to the emergency room. Ok, I can do that; problem is that within one hour after returning home, her fever jumped to 102; so off to the ER we go!

By the time we arrive at the ER, Emily’s body had become limp. She was conscious but did not have the strength to move or even cry. She is three years old and just laying there; I am scared out of my mind. The diagnosis: dehydration and pneumonia. WHAT? PNEUMONIA? THIS IS THE ONE THAT WE WERE TOLD TO TRY AND AVOID!

We spend the night in the emergency room pumping her poor little body with IV fluids and antibiotics. It is winter: peak of flu season and the hospital is full of flu cases. They want to keep her but are afraid to expose her to the flu on top of the pneumonia she already has, so they send us home with medication and instructions to return if not feeling at least a little better by the end of the next day.

We return home and I become angry. Her pediatrician says it’s viral; even mentions that her lung sounds are normal, nothing to worry about. How can it change in a matter of two hours? I chalk it up to complacency and a missed diagnosis. This same story would repeat itself again six months later. It was time to change doctors.

After much searching, I find another pediatrician and set up an appointment just to talk with him. You know, interview him. We meet and I give him the brief version of Emily’s medical history. The first thing he asks: “Who is her immunologist?” I must have had a blank look on my face; he repeats the question. I tell him I don’t know what an immunologist is and he immediately shows disgust, and explains what it is. And here we go with the blood work again. And again, her IgG levels are low, but not low enough to be referred to an immunologist. We need to watch closely and address any illnesses as soon as possible. At least this time, I am told that there is a possibility that she may grow out of this.

Three years go by and Emily being sick frequently has just become a way of life. A change in our insurance required us to find a new pediatrician under our new plan. After reviewing the medical records, another order for blood work, to see where her levels are now. Still low but not low enough to refer to an immunologist.

The next coupe of years, Emily is sick more than healthy. Countless visits to the doctor’s office and emergency room. There are countless strep infections and viral infections. Now we are told that since she is in school she is going to get sick. I think to myself, this can’t be right. Yes, kids in school are going to get sick, I understand that, but this can not be right!

Finally, I start to research what little I knew about immune deficiency on the internet.  I was surprised at what I was learning. Why did I wait so long to do this? The patient stories I was reading are carbon copies to our own experiences. We had experienced complacency in the doctor’s office as well as the emergency room. No one ever thought that maybe it was time to dig further into why Emily was getting sick so often.

I also found that many patients experience isolation within their community and families. Statements such as, but you don’t look sick, or she must be faking, or you just don’t want to go to school kept popping up everywhere I read. We had experienced the same thing. Truth be told; I had been convinced of the possibility that Emily could be faking to get what she wants. She is an only child so she has to be spoiled and this is her way of getting what she wants. But in the end, I knew deep in my heart that something just wasn’t right so I start my search for an immunologist. If no doctor will refer us then I will refer us myself.

Eight years from the start of our journey, we are finally sitting in the office waiting to be seen by an immunologist. No surprise, first thing on the agenda: you guessed it, blood work. For some reason this time I don’t mind. Emily has even become an old pro at it as well. She will even show them the best arm to draw from these days. I look at her with such admiration; she has gone from a kicking screaming two year old, to a very strong and poised ten year old.

Two weeks later lab results are in and the same old story repeats itself. IgG levels are low and her body still is not responding to the pneumococcal vaccine, but this time there is more. They also find that her IgE (allergens) is low and parts of the complement system are low as well. What? What is all this?

Through my research, I had seen many references to the fact that some medical students are taught to look for the likeliest of possibilities of what an illness might be. “If you hear hoof beats, look for horses instead of a zebra”.  Is it really possible that this doctor is actually looking for that zebra? I had often thought that Emily was a “zebra” in the medical world, but I am not a doctor, so there is no medical training only research on the internet. The immunologist ordered another pneucoccal vaccine and another round of blood work to dig even further. Really? Seriously? Finally, he is certainly looking for that zebra.

Just a few days ago, we had our follow up visit. Yes, he was looking for that zebra and he found it too. The diagnosis is IgG Subclass Deficiency with Specific Antibody Deficiency. There is a suspicion of Complement Deficiency as well and testing is still on going for that one. Asthma and allergies are also included in the diagnosis. FINALLY!

Through my research, I had suspected but for obvious reasons, could not confirm that she was in fact immune deficient. We have endured the accusations of just over reacting and faking the illnesses. We have had people roll their eyes at us when we try to talk about what Emily’s needs have been. Finally, a doctor has confirmed what I knew down deep in my heart was there.  For eight years, I knew something just wasn’t right. I just didn’t know how to fix it; even some of her doctors didn’t know how to fix it. This doctor DOES know what to do!  I finally had found a doctor willing to look for zebras instead of horses and the feeling of relief was amazing.

There is no cure for immune deficiencies only treatments to help the body fight off infection so organ damage can be avoided. There is still a chance that Emily could outgrow her disease, however it is becoming more and more unlikely the older she gets. We now have a treatment plan in place and Emily will be monitored and tested every three months initially to ensure that treatment is working and that her disease is not progressing to something more serious.

Her life is forever changed and may include a life filled with doctors and hospitals and labs and medications but she is a resilient and strong young lady. I am so proud to have her as my daughter. I am so glad that I never gave up. I am so glad that even though I strayed away from that gut feeling that something just wasn’t right, that I came back to it and fought with all I had to find a way to help her.

The majority of my research was found on the website of the Immune Deficiency Foundation. I have found a second home there. Without the wealth of information they have provided, I am not sure Emily would be where she is today. The biggest thing I have learned is never give up. Never quit looking and learning. There are times that your child has only you to depend on for help. Our journey is only beginning, but we are finally heading down the right path thanks to the information provided by IDF that led me to the decision of breaking ranks and finding our own immunologist.

Because of what the IDF did for us, I have joined with them in an effort to educate and promote awareness to immune deficiencies. If you know someone with recurrent infections, infections that are difficult to recover from or would just like to learn more about immune deficiencies, go to www.primaryimmune.org. Find out how it may not be just another infection but another zebra waiting to be found.

Dona Darr