We had our second visit to the CF clinic yesterday.
It went much more smoothly than the first visit last month. Except for the part beforehand. I had taken a shower. Was wearing real clothes! Makeup! My editor Kathryn arrived at my house to watch Laila. Laila was so excited someone new was coming over she declared, “I like Kathy!” before meeting her. I had packed my stylish new TJ Maxx bag, made tea to avoid an impulsive Starbucks run and given Kathryn a tour. Ready to go. So ready to go I practically popped my collar and kicked up the heel of my fashionable boot on the way out the door. Then my keys were no where.
I tried in vain to find them, but Mark had to come home early from school to give me his set. He relieved my boss, and I went ahead to the appointment rather sheepishly. Thanks any way, Kathryn! Luckily she is empathetic and supportive and kind, obviously, or she wouldn’t have been at my house in the first place. I was running about 15 minutes behind at that point. Then I got stuck behind a train. A train that went on forever. Of course! It stopped its slow, endless journey forward on the tracks in front of me. Naturally! I called the clinic – they cancel after you are 15 minutes late. However, on account of the train, I shouldn’t worry about it, the receptionist told me. I arrived, cursing the fact I had worn boots with a heel as I ran from my car awkwardly lugging Eli’s car seat. Is there any good way to hold on to one of those things? Buddy was screaming. We went from the lobby to the 9th floor of the wrong side of the hospital, then back down to the lobby, and back up the 9th floor on the right side of the hospital.
I was sweating and out of breath at the receptionist’s desk.
The receptionists were very nice about me being so late. We even kibitzed about the phenomenon of getting stuck behind trains when you are already behind schedule.
I took a deep breath because I wanted to be focused rather than flustered so I could learn a few things this time. They gave me my own pen to take around – something CF clinics do to avoid the spread of germs. Last time I got all freaked out and sad that I needed my own pen. I thought nothing of it this time, other than to note the difference a month had made in my reaction to little things like this. Ah, progress.
We went to our exam room. There, I signed lil buddy up for a study and to be on a national registry of CF patients. The study is observational, meaning the research group will be monitoring about 200 CF babies’ nutrition and growth over a yearlong period. Eli gave the researchers his DNA via a cheek swab.
The patient registry is run by the Cystic Fibrosis Foundation, the highly-organized nonprofit that is a driving force behind seeking a cure for cystic fibrosis. The registry has, for 40 years, tracked how patients have done over time. Eli will be anonymous and assigned a number.
The woman working the study sign-up is Deanna. She is kind and smiles a lot at buddy and fusses over him. That’s how she convinced me to give up my DNA for the study, too, even though scientists aren’t yet sure how the parents’ DNA will be used. You want my genetic code? Yeah no problem, now tell me more about how adorable my child is. Anything else? My soul? That shouldn’t be a problem…now, what were you saying about his beautiful eyes…
The other thing about the study — Eli will be put in this sci fi pod that measures his body fat composition. Deanna says this is really cute and I should definitely plan to take pictures and record. She speaks my language. I told her I’m all for that as long as she doesn’t force me into the pod.
And lastly, for your participation, you get $50 cash a visit. This offsets the cost of seeing a specialist, which is $40. So yay science…and cold, hard cash.
I next talked to the lovely Debbie, CF clinic coordinator, about a host of things. We’ve been doing Eli’s chest physical therapy every day for an hour, I relayed. The point of doing this – tapping on his chest in a series of different positions – is to get him to cough up thick sticky mucus that gathers in the lungs of CF patients. Eli doesn’t appear to have much of this mucus because he rarely coughs. However, he has been coughing about one to three times per day for the last three days.
“It’s freaking me out. Is this normal? Is it CF? Is it an infection? My husband has a cold. Now I have a cold. We’ve been doing everything we can to avoid colds but have them any way. I feel guilty. Should I…?”
Debbie halted my increasingly insane rant with some reassurance.
Debbie assured me Dr. Royall would listen to his lungs and check for post-nasal drainage to see if Eli was sick, but it might be Eli is coughing because we’re doing the therapy. Or he could just have something in his throat. And people get sick. Really, all you can do is wash your hands a lot, get a flu vaccine, avoid sick people, keep your house as sterile as you can and hope for the best.
The reason colds scare me – in those with normal lung function, viruses and bacteria that cause colds, flu and other infection mostly cycle in and cycle out. Infections clear with the help of an antibiotic or go away on their own. Eli’s chronic disease means the mucus sits in the lungs and encourages infection. Colds and viruses linger. Over time, lung function is reduced because common strains of bacteria will cause him to have chronic bronchitis. I hate to put it like this because I refuse to think this far ahead, but the reality is, lung infections suffocate a person with CF over time – hopefully decades. The medical treatments – chest tapping, eventual antibiotic breathing treatments, etc., and even lung transplants if it comes to that, stave off the reality of the disease. Well look how I was able to write that without passing out, wanting to spew, sobbing or getting sad. Because my buddy is a healthy little boy today. He is very much a gift, not defined by his genetic disorder, and CF, shmee-F.
Another thing — Lung function is typically good through infancy.
I next wanted to know more about the vest Eli got to wear last time he was at the hospital. It’s an alternative to tapping on him but does the same thing. It’s also 1000 times more convenient. Instead of crying during the therapy like he does when I tap on him, Eli kicks back with his vest on, throws his hands above his head and goes to sleep. I find this stunning because the air coursing through the thing is noisy and the vest really shakes buddy up. The vest is connected via black tubing to a pump on a stand. I want a pump on a stand. And a vest.
Debbie was all over it. She filled out the paperwork, I signed, and we both crossed our fingers that this would be covered by insurance. I hope so too, because the pump alone costs $16K.
While I was talking to Debbie Eli pooed all over himself through his diaper, which leaked onto me.
Unlike flipping out like last time – he had been wearing a poo bag, which busted, and essentially did the same thing – I was able to keep talking and listening and change bud’s diaper and outfit. He does not have a central line any more, threatening to fall into poo explosions and infect his bloodstream, which helped.
Dr. Royall saw Eli, too. He upped his dosage of enzymes to help buddy gain weight. Eli was a big boy before surgery but lost almost a pound, poor boy. He weighed 9 pounds, 10 ounces this week. Before his latest surgery, he was over 10 pounds.
His medical team wants to make sure bud gains weight and doesn’t just poo it all out. We do not know he is doing this. But having him eat more enzymes will help assure he is not. His body will be (or is already) defective in producing digestive enzymes that help those without CF gain and keep weight on. CF people poo out fat. The enzyme pills are the way science prevents that. I pop open a pill and spill tiny white beads onto a spoonful of applesauce before little man’s meals. Eli sees the applesauce coming and has a Pavlov’s dog reaction, making a little sour face in preparation for his wee meal.
Eli does not have crummy sounding lungs or post-nasal drip, Dr. Royall found. He doesn’t appear to be symptomatic of anything, unlike his mommy. If he does start sneezing or coughing a lot, I should call the clinic, the doc told me.
Eli has another appointment next month – hopefully the 3rd time’s a charm.
I am sucking down my third cup of coffee this a.m. Now it’s time to do a Valentine’s Day crafts with wee Lai Lai.
Happy Valentine’s Day.
We still haven’t found our keys.
XoXo, Gossip Girl…I mean, me.
PS: Here are some faces of Laila J I achieved by bribing her with M&Ms.