It wasn’t long before it became clear to us that Jake wasn’t ok. The Remicade wasn’t working like we’d hoped. The Sarcoidosis /autoimmune theory wasn’t completely wrong- something was causing his body to respond, but they couldn’t find it. We were back and forth several times having excess cerebral spinal fluid drained and IV bags of prednisone. This bought us time… we’d show up at the emergency room, and the doctor would look at his file, and inevitably say, “There is nothing we can do for you…” I would respond, “We just need pain control until Dr. S. gets in and can admit him.” During one extended stay, they inserted a tube/drain into the spinal meninges to relieve the pressure. We found out later that the amount of pressure was unprecedented. They were able to keep it under control so Jake remained coherent and alert. A young doctor was on rotation again and got to talk to Jake. “Man, I am so glad to see you again and talk to you. I was so afraid that before [when you were so incoherent ] you’d never be back [to normal].” No one had ever suggested the possibility it could cause permanent damage. When word spread around the floor that he was the valedictorian and planned to go to an engineering school, people treated him differently. I know people assumed, especially in the ER, he was out of his mind from drugs. His hair had grown longer and his beard was pretty scraggly. All I knew was he was my baby, and I wasn’t ready to let him go.
Jake ended up having about 11 gram bags of prednisone his senior year. It is a brutal drug, and it took months to wean him off of it. He started an IV drug called Remicade before he was discharged. We cautiously took him home, and the plan was a monthly infusion of Remicade. He planned to return to school for 2nd semester. My mom was living in the hospital by that time, and we intended to have Thanksgiving in the cafeteria. At first we tried to keep Jake’s illness from her, but she was used to me visiting once or twice a day. I finally had to tell her. I could see the spark go out of her that day, and her health continued to decline. Dave and Jake decided to grill steaks on Thanksgiving because the hospital seemed too risky for his compromised immune system. Things were looking hopeful. Jake’s second infusion left him feeling “Remicade good”. He went to see my mom in the hospital. The look on her face when she realized it was “her Jake” was priceless. She forgot by the next day that she had seen him, and she was back to fretting. Within a few days, “Remicade good” was wearing off. His headache returned, and he was speaking jibberish again.
We were thrilled when the pulmonologist said she had identified the bacteria in Jake’s lung. They started him on the proper antibiotics, and we were hopeful- it was very treatable. A few days passed with no change, and eventually a negative result came back after the lavage cultures had time to grow…nothing. Dr. S. decided to start him on gram bags of IV prednisone. Jake started to come around after weeks in the ICU. He had been hallucinating, delirious and couldn’t sleep for a few days straight. I don’t know how I drove back and forth those two hours. I basically cried and said the Rosary the whole time. It became winter, but we kept taking turns and driving back and forth. We had to make a bed out of a chair and a bench in his room. One nurse made it clear they didn’t want family members in there. We had a different nurse every shift. I realize now they didn’t want anyone getting too attached or familiar with the ICU patients because it might not be a happy ending. If it hadn’t been for Dr. S. and him taking a little longer to talk and listen, we would have lost it. We looked forward to his daily rounds, and hated the long, lonely weekends when he wasn’t here. We still didn’t have a conclusive answer, but the gunk in his lungs appeared to be an autoimmune response, and the prednisone seemed to be working. Jake was weak and thin, but it looked like he’d be home for Thanksgiving. Dr. S. made a plan to start Jake on Remicade for an autoimmune disorder. This made sense as it went back to the local doctor who thought Jake had a connective tissue disorder. Yay! Home with a plan…
I have never felt so alone or scared in my life. I finally found my way to the ICU of the new hospital- no one was there to greet me. The doctors were going to do a spinal tap.
I was surprised they did it bedside so I was there for the whole thing. They have to puncture into the lining of the spinal cord and draw out cerebrospinal fluid. There is a glass tube used to measure the pressure. Jake didn’t flinch. He probably had 10 -12 of these procedures before it was all said and done. What I didn’t realize until much later was that Jake’s pressure was always exceeding the tube’s ability to measure. 13 to 18 was normal; most cases of meningitis would go to around 30, but Jake’s was beyond 50- the highest reading. Sometimes they would drain off what they thought was enough, and it would still read too high. They started culturing his blood to try to isolate the culprit. There were several teams of doctors who would come visit each day, but most wouldn’t say much. The waiting and lack of knowledge was really frustrating. Eventually the blood tests started coming back. ..negative. They reluctantly decided to start him on an antifungal. I started to panic more because the negative tests were discouraging, and with the “help” of Google, I knew a fungal infection was more likely to be lethal. The antifungal medication is pretty harsh so it was a difficult decision. As more time passed, they decided to do a lavage and bronchial scope of his lungs. I kept repeating his history and symptoms and it kept going back to his lungs. The neurologist suggested sarcoidosis as an explanation to the gunk in his lungs and negative cultures for infection. After the lavage, the doctor came out and told us, “I think I have identified the bacteria,” Is it treatable? “Very…”
Initially I thought Jake had meningitis, but then he seemed kind of stable in the ER. No spinal tap- just other tests. Pretty soon they admitted him. It’s a nice hospital 25 minutes from home, so by the end of the day my dad and husband came over. Things started to go down hill from there. Jake started talking jibberish. He was irritated or upset about something, and he kept repeating the same incomprehensible “words”. I thought maybe he was worried that he was missing work. Pretty soon he was trying to get out of bed. It took a lot to hold him down. Still no spinal tap, but he was moved to the ICU. Initially that scared me to death, but then I came to realize it meant more attention from his nurse. They started the broad spectrum antibiotics just in case it was bacterial. I started telling the doctor about Jake’s history. “Who told you that? I’ve never heard of anything like that. What hospital? ” I told him the name of Dr. N. and the hospital. Pretty soon they started him on anti viral medication and ordered other tests like an MRI of his brain. An allergy/immunologist stopped by eventually and reassured me that Dr. N. was the best in the Midwest. He has an immune deficiency. The doctor replied, “Well I think he has a pretty good immune system,” Puzzled. ..I let her continue…”He’s still with us…” That was the first time I thought my son might not survive this. Jake went in and out of consciousness and coherence the rest of that week. His girlfriend came to visit one evening. Jake turned to his nurse, Greg and asked, “Who is that girl staring at me? She’s kind of stalkerish.” We giggled about it later, but he had no idea who she was. He lost control of his bladder, and over and over they had to change his sheets. This greatly agitated Jake, and it became unbearable for me to watch. I started to realize he would fidget when he needed to go, and I would grab the urinal and try to catch it. A team of doctors and I were standing around his bed when he started to fidget. I nonchalantly grabbed the urinal and tried to assist Jake when he came back into awareness. He looked around, and my son who was offended by cursing yelled, “What the f*$% are you doing? Why are YOU touching my junk?” By the end of that week, a couple of nurses were hinting that we needed to get Jake to a larger hospital when the doctor came in. “Jake is not responding to our treatments. His MRI shows nodules in the brain that I’ve never seen. His immune deficiency complicates this further, so we believe it is time to transfer him to a larger hospital.” Our daughter and dogs were at home, so my husband, Dave, and I took turns. It was my night, and I couldn’t ride in the ambulance. I followed them in my car to an unfamiliar place that would be our home until late February.
At 5:30 AM on October 23, 2014, Jake woke me up, “Mom, I have the worst headache ever, and I’ve been vomiting all night.” Meningitis popped in my head. I wasn’t going to mess around. I immediately drove him to the emergency room. They gave him morphine for the pain, and he was hungry and texting on his phone. Maybe I overreacted… A year ago I had a patient in the pharmacy who was irritated that the Tamiflu prescribed to him was over $100.00. We discussed it- he didn’t have to buy it. It would shorten the duration of the flu, if he had the flu. Had he been tested? No. I told him the medicine would be here if he decided he needed it. The next Tuesday I heard he was fighting for his life in the same hospital that I took Jake to initially. The gentleman had bacterial meningitis. The Tamiflu would not have made a difference. Bob survived. I later talked to his wife and him about his ordeal. There are many types of meningitis. There is a vaccine for meningococcal meningitis that helps protect people living in close quarters like a dorm or in the military. Meningitis literally means swelling or inflammation of the lining of the brain and spinal cord. In 20 years, Bob’s doctor had only seen 2 cases of this type of meningitis, and his first patient died. It’s crucial to identify the cause so the proper treatment is given. They guess initially and administer broad spectrum antibiotics. It takes weeks for the cultures to come back. Many patients don’t have days, let alone weeks. Generally viral meningitis isn’t as serious. People don’t really understand that they are often infected by a virus, and viruses do not respond to antibiotics. Everyone wants an antibiotic. They flew out of the pharmacy like candy. I poured them down my kids’ throats. I found too that people don’t understand there are many types of meningitis, and they aren’t all contaigious. Bob’s was. No one else contracted it, and after a difficult battle, he regained his health. I met an 18 month old girl when my daughter was about the same age. The little girl was missing both feet and one hand and her other thumb. The young mom volunteered her story. She and her husband worked opposite shifts so their baby didn’t have to go to day care. She became critically ill with a bacterial meningitis, and they have no idea where she contracted it. Her body, in an effort to survive, focused all of its energy on the core organs and brain. Her extremities began to die. She survived with the loss of body parts. I swear the Holy Spirit spoke to me that morning, I just had no idea that meningitis could last for 4 months…
In 2005, my mom took my kids and me to Walt Disney World. I could not think of a place I wanted to go LESS than there. Expensive, hot and crowded… it was my mom’s dream to take all the grandkids to Disney World, so I had to suck it up and accept our free trip. Don’t start hating on me just yet; it was a “blessing in disguise”… First of all we had the ability to take the kids out of school a few days. We went in early December on a Wednesday through Sunday. The kids had to be at least 7, tall enough to ride all the rides, and walk the park according to my mom’s “rules”. We stayed on site and had the dining plan. My mom had a terminal lung disease. She was starting to slow down considerably. I’m a take charge kind of gal and not very patient with her “turtle” approach to walking. The first thing I did was aquire a wheelchair for her at the resort. The magic of Disney enveloped us quickly. The weather was perfect, the lines were short, the food was delicious, and I found myself hugging Disney characters like no other. Pushing my mom in the wheelchair was the blessing in disguise. In 2005, the parks weren’t overwhelmed with obese people in motorized chairs unable to walk the park. My family was treated to VIP service everywhere we went with my mom in that chair! I was able to control the pace, and my kids were old enough to keep up. We rode everything- we even stayed on some rides and rode them again because there were no lines! They had decorated for Christmas by then which added to the magical beauty of it all. I do not like parades typically, but I didn’t miss a parade or show in those few days. “Why hadn’t we been here before? We could have taken our honeymoon here! I am going to work here when I retire….” I would look at my mom and say over and over, “This IS the happiest place on earth!” My mom took my sister and her boys the next year, and we tagged along (I had to push the wheelchair, right?) for another magical trip. After that my mom was too ill to accompany my brother’s family. My pictures our our trips hang where I see them every morning. I think of it often. I wasn’t able to take my mom back. Her dream was to stay at the Grand Floridian….maybe someday I will take my own grandkids, and I have a feeling she’ll be there with us…
I took a lot of biology in high school, undergraduate and graduate school, but I had never heard of Natural Killer cells or their deficiency. Actually there have been several doctors who aren’t familiar with it either. When Jake finally got a diagnosis in 2012, it was a let down. Dr. N. explained it the best he could. “Natural killer cells are like the moat around the castle. They are your first line of defense. They ‘shoot first’ and ask questions later…” “Jake has a deficiency or a glitch. He has NK cells, but they don’t turn on like they should. That is why he gets sicker than normal. The rest of the immune system has to come in and do their work plus more. It shouldn’t cause problems; it will be a nuisance but not life threatening.” “There are no treatments; there is nothing we can do…” This did not bode well with me. I found a little when I Google searched, but nothing concerning treatment. (There is more now, but I don’t advise looking it up.) We didn’t see Dr. N. again until 2014. What I’ve realized in the meantime is that it is very rare, or maybe not identified in patients. There are different degrees. It is said to be inherited, but it could also be a gene mutation in utero. I think Dr. N. thought Jake would be ok because he had lived this long (2012) without a major complication. The cases he had seen were younger kids who presented with lethal cases of herpatic meningitis or Epstein Barr. Jake’s deficiency must have not been as serious or he probably would have already died. It is viruses that cause lethal infections in these patients. Healthy people with healthy NK cells fight the viruses. Looking back, Jake should have started Acyclovir, a viral medication, to help protect him. Warts are caused by the HPV virus. I had unwittingly had my kids vaccinated with the HPV vaccine. Genital warts are rampant, and I wasn’t going to pretend that teenagers not only have sex, but they may have multiple partners who have had multiple partners. Turns out, that was the right call. The vaccination is recommended for NK cell deficiency. The warts that had developed on Jake’s feet were also caused by HPV (not the sexually transmitted type). This was a strong indicator of his NK cell deficiency, but no one ever seemed to connect it. The other day I was surprised that Dr. N. wanted to see Jake’s feet. His warts have disappeared. Dr. N. was surprised and at a loss of explanation. I assumed he went back over Jake’s notes from our first meeting. Hindsight is 20/20. The 3 symptoms of Jake’s deficiency are chronic ear infections, warts and chronic respiratory infections. Serious complications can occur from viruses like HPV and herpes. Most people only think of sexually transmitted diseases, but chicken pox, shingles, and Epstein Barr virus are 3 of the 8 types of herpes virus that can infect humans. Most of us can fight and live with these viruses, but they can be deadly for a NK cell deficient patient. Knowing what I know now, I definitely count my blessings.
I have never experienced chemotherapy. It sounds horrible and scary. Cancer is a word that has always terrified me. I have worried that I would get skin cancer from all the summers at the pool unprotected from the sun. I have waited a little longer than recommended for my mammogram or Pap smear. I’ve lain there thinking, “Ok, you blew it off, and now you’re going to have cancer…” Jake was home for Thanksgiving break. He had just completed his first trimester at college. He was tired; I thought it seemed normal. On Wednesday, December 2, 2015, Jake called us and said he had a fever and was short of breath. I met him at the student health services office and assumed he had pneumonia. He had gone to classes for 2 days, but was too weak to walk to the cafeteria to eat. It took several weeks of testing to get the diagnosis. It was AML leukemia. The chemo would start that night. I was scared of the toxic chemicals going into my 19 year old baby’s body. To my surprise the chemo was only for 7 days. It actually made Jake feel better. Even though he was dependent on blood transfusions for awhile, and it wiped out his immune system, he did quite well. He perked up and his cough got better. He looked good and asked us to hook up his Xbox. His only side effect was his thinning hair. His dad and he let a CNA shave his head. It was not what I expected. The leukemia was gone… This chemo for the preparation of the bone marrow/stem cell transplant was supposed to be more difficult. I have to say, again I am surprised. I pictured him vomiting, horrible diarrhea, begging for help as I watched unable to do anything to ease his pain. Nope…he looks good. His biggest complaints are a sore throat and his bladder is irritated. They have to give him a lot of fluids to protect his bladder from the chemo. The chemo is over- hopefully forever. It is not the chemo of the 1970’s. I am surprised and amazed at how far medicine has come. I am thankful my son has not suffered though something I imagined to be much worse.
My sassy, sweet, funny daughter told me I owed her three hundred and some dollars the other day at lunch. “Well I just paid the Visa bill, so we’re even…” I was out to lunch with Molly and one of my best friends, Stephanie, when a group of my mom’s classmates came through. I stood up for hugs and they wanted to hear how Jake was doing. For the past few months, it has been all about Jake. He was in the hospital preparing for the stem cell transplant. Molly had told me after one of our outings, “Mom, you seriously cannot go 5 minutes without mentioning Jake.” She isn’t jealous, just annoyed that I can’t keep a conversation short. I started conciously thinking about it, and she is right. I have said to people, even strangers, while exchanging small talk, “I know you really don’t care, but I’m gonna tell you anyway…” I can talk every minute of a two hour car ride. I start one topic, get distracted, move on to a different topic and end with, “well now I don’t remember what I originally started to tell you…” My dad just walks off and waits in the car when I start talking. He was telling my brother, “She has a relationship with the cart pusher at Walmart. Not just “Hi, how are you?”, but a relationship… and yes, he knows about Jake and has met my husband. So back to why I owe my daughter the money. She and Stephanie played a game while I was talking to my mom’s friends. “Every time I said ‘Jake’, it was 5 dollars” (unbeknownst to me). The actual point of this blog is that there are many times when I have referred to something as a “blessing in disguise”. At this moment it is about Jake. We are literally in the hospital. He has just finished 2 types of chemotherapy. His immune system, which was born flawed by some undiscovered mutation, is being wiped out. He will receive his only sister’s stem cells. She is an unsung hero, but she doesn’t appreciate what she is actually doing. The gift she is able to give her brother is worth a million times more than that $345.00. Yes, right now it is hard not to talk about Jake and to share his remarkable story.