I have 2 daughters, Sarah and Laura. Sarah is the oldest. Ever since Sarah was a about a year old, she had repeated ear infections and lung infections. Doctors treated her with antibiotics and things would seem to get better for a while.
When Sarah was in 6th grade, she was diagnosed with asthma and we noticed that the tips of her fingers would never extend to a straight position. They are constantly bent but never interfered with her writing, playing or playing the clarinet. During this time up to the time she was a junior in high school, she was getting more frequent infections. Each year they were becoming more numerous and harder to clear up. Sometimes her doctors would treat her with antibiotics for up to a month. I can remember being so upset in the doctor’s office and telling them that this was NOT normal and something was wrong. I told that story to every specialist she was referred to and no one listened.
Sarah was finally referred to a pulmonologist from a hospital in Birmingham, AL that was practicing here in Huntsville. He tested her for histoplasmosis, TB, cancer, you name it, they checked it. Still nothing and the illnesses persisted.
One afternoon, while she had been home sick, it became very hard for her to breathe. My husband took her to the emergency department while I went to band practice and got my youngest daughter. On my arrival to the ER, the pulmonologist was there and was comparing x-rays…one that was just taken to one taken a week earlier. She had lower left lobe pneumonia, atelectasis (collapse of small airways), and a pericardial effusion (fluid around her heart). No one knew what was going on so they flew her to the hospital in Birmingham. They would not let either her father or I ride with her so I watched my daughter fly away not knowing if she was going to live or die. My husband left immediately for the 100 mile trip. I stayed until the next morning to take care of things here so we could go.
When I arrived, I was immediately surrounded by doctors, pulled into a room and the history and physical questions began. They would not let me go see my daughter until I had finished with them. She was in ICU isolation. She had enlarged lymph nodes and a very low white count in addition to everything else. Her pericardial effusion was not clearing so the doctors decided to do a pericardial window (surgery near her heart) and take some lymph nodes for biopsy to rule out lymphoma. They did it by scope and entered from her right chest. By the end of the week, they said they had to get some deeper lymph nodes and those had been inconclusive.
The doctors were also going to insert a scope into her left side. The moment they entered her chest, they punctured the lung and had to do an emergency surgery to stop that bleeding. We had gotten pizza for her after her surgery but of course when we saw her, we were alarmed, scared and did not know what had happened. She was to be on a ventilator for several days. I was demanding to see the surgeon but he never showed up. A resident did and was not willing to tell me what happened. I had to remind him that I was an OR nurse, and assisted with chest surgeries and it was not normal for post op patients to be on a ventilator unless there was some type of breathing issue. After I finally threatened him, he told me that the lab tech had messed up the previous biopsies, that when they entered the chest cavity the scope punctured the lung because it had adhered to the chest wall and she had some pulmonary compromise.
After several days, they took her off the ventilator but still did not know what was causing the problems but that she had numerous bacterial, viral and fungal infections. Finally, after almost 2 weeks, a doctor diagnosed her with CVID: Common Variable Immune Deficiency. They decided to give her intravenous immunoglobulin. When it came time for it to be given, the nurse started it and walked away. (Robert and I were not in the room at this time and we were not told they were going to start it). The nurse did not take baseline vital signs or start it slow- NOTHING.
When we walked back in, Sarah was having a very bad allergic reaction and no one was around. I saw the nurse eating at the desk when we walked back in. Of course, I hit the call bell and stopped the infusion. I told the nurse what was going on but she did not seem to understand or care. Luckily there was a doctor in the unit when it happened and heard me barking at the nurse telling her what she needed to do to. I was PISSED to say the least. They all knew I was a nurse and still provided overall crappy care. Sarah did have a couple of nurses that were wonderful and knowledgeable.
That night, another doctor wanted to try the same brand of IV immunoglobulin again. I do not remember the name of it now but it is the one that has the highest allergic reaction rate (I had done my homework). I told the doctor “no” and of course she said that Sarah would not have the same reaction again. I knew better and insisted on another brand. That one worked well and she has been on it ever since.
The scary thing about it all was that I WAS a nurse and still had people telling me wrong things. All of this was so new and I did not understand her disease process so I asked for all the technical information, did research, etc so I WOULD know. I became scared for all the patients and families that don’t know and either do not or are scared to ask the questions to find out. I have always believed having knowledge is a good thing and is needed to make the best informed decisions and encourage parents and patients to learn and ask questions.
Lisa Johnstone RN
Huntsville, AL
