Saturday, May 25, 2013

brighter outlook/ Memorial Day

Jillian still has three days in the hospital, but she's receiving a treatment developed in France (I think they have a higher incidence of cystic fibrosis in the northern European region than in the US, hence a higher level of research) that appears to be doing good things for her. it's not fighting the pneumonia all that faster than are the other drugs she's being given, but it appears to slow or prevent the damage to the lungs and airways that typically occurs with each case of pneumonia that a cystic fibrosis patient gets. Cystic fibrosis is sort of a cumulative thing - a patient typically loses a little ground and a little bit of their lung space to the invading bacteria each time they contract pneumonia. i believe it's known as colonization. The bacteria (for most CF patients it's more often than not a particular bacteria that invades; for Jillian, it seems to be pseudomonas, although she's undoubtedly had other forms somewhere along the lone) tend to gradually take over the lungs. that's the reason CF patients eventually need lung transplants.

As bas as the idea of a transplant sounds, this wouldn't be such a terrible thing except that lung transplants are probably the transplants that are the least likely to "take, or to be successful. There's a high incidence of rejection. The drugs a patient has to take to or even rejection of the new organ -- and lung transplant recipients need more than do the recipients of most donor organs) also lowr a patients resistance to everything else, so it's quite the vicious cycle.

So if Jillian can hold off the major damage each time she contracts pneumonia, and she's probably going to get it once or twice a year at least no matter what she does to prevent it, she's greatly postponing the day that she one day needs a lung transplant. this means that she might live to be a grandmother. Additionally, this new treatment is safe during pregnancy, so she can have less aggressive antibiotics and ones that are safer to a fetus during pregnancy, which is a huge deal to her.

Tomorrow Jillian will try to walk a few steps totally unassisted.

Classes have been a bit boring, mostly because I've had trouble paying attention. I've managed to focus during "The Physics of Fractures." I found out that at my three top choices of med schools, that's a 1st -year med school class, and with an A, they'll all accept it from my university. So far I'm still easily in the A range, so unless a disaster occurs, I should be good.

             where most of the people in my prospective band live, which may be indicative of just how sophisticated the band is; at least they're not frat rats

This really grungy guy in "The Physics of Fractures" with me wants me to join his band as a keyboard player and fiddler. I don't know if I'm up to hanging out with the band and with the crowd that follows his band, and I don't think it would be a particularly impressive addition to my med school resume 9to the point that I won't list it, obviously) but I told him that once finals are past, I'll consider it, since all I'm doing is a daytime internship and a private violin to prep for the senior recital next year.  Some things you just need to experience for the experience of it. as long as I don't drink anything with PCP in it, and I'll be very careful to carry my own sealed drinks everywhere, I'll probably be OK.  I personally think I'll look a little stupid on stage with them, as I don't exactly look like a rocker chick, but that's really their problem, isn't it. I'm NOT dying my hair purple to fit in.

I hope everyone is prospering and is planning a lovely Memorial Day weekend.  I'm kicking in my share of the money for flowers for the various dead relatives, but visiting cemeteries gives me the creeps, so I'm not doing the grave-cleaning and flower distribution work myself.  If i get the opportunity, I will travel north to visit my older baby twin brothers' graves, though. my brother and i try to do it on or near their birthday and on Memorial Day weekend even though Memorial Day is reall about the military.I make the exception to my usual wimpiness and squeamishness in my brothers' case. for a few reasons, one of which is out of respect for my parents and out of empathy for the loss they suffered.  Another reason is that I don't for sure know that my parents would have had Matthew and me had Nicholas and Christopher survived. My parents have never said, and we've never asked. If my parents would have stopped at two children, in a way Nicholas and Christopher involuntarily made the ultimate sacrifice for Matthew and for me.

To those of you who are less easily bothered, I offer my admiration. It's an unpleasant task, but cemeteries would turn into areas of disrespect for the departed if someone didn't take care of the graves.


  1. You are such a thoughtful and empathic young woman, Alexis. I'm sorry for your parents' loss of your brothers, but I am glad she had you.

    I don't have any big Memorial Day plans. My husband has the whole weekend off. I imagine I'll finish writing up our vacation and maybe get some housework done. Maybe I'll also do some house hunting.

    1. Online house-hunting, as opposed to traveling to Texas?

    2. I look for houses online using a database operated by the military. Then in early July, we will go to Texas and see the houses on our short list and make a selection. Sometime at the end of July, we'll pack up to arrive in Texas during the first days of August.

    3. Sounds like a very efficient way of doing things.

  2. It's good that prolonged treatment options are being explored in Jillian's case. At this point, are they saying that a lung transplant is inevitable? I guess they are for the majority of CF patients, though for Jillian individually, could her later diagnosis have compromised her treatment? I remember when she was first diagnosed with CF (something about Scott kissing her and realizing that her skin was salty...) and that in the beginning it explained a lot of symptoms though, everyone was hopeful because she made it to adulthood without a diagnosis. Are they thinking that the disease is progressing more rapidly? I've read a little about CF since you posted to try and understand it more on my own without having to ask you one thousand questions... but, here I am doing it anyway.

    Anyway, it's good that she's doing better now, and that they are exploring more options to (hopefully) keep her stable, if that's what these options are supposed to do.

  3. I can recommend a couple of good books about CF. One is "Alex, The Life of A Child" by Frank DeFord. The other is "The Power of Two: A Twin Triumph Over Cystic Fibrosis" by Isabel Stenzel Byrnes and Anabel Stenzel. The second book is a lot more recent and pretty fascinating, since it's about twins who are half Japanese and half German. CF is pretty much unheard of in Asians, but the twins explain that their mother must have had some Caucasian blood in her ancestry. I'm pretty sure the twins had lung transplants.

  4. i've read the Deford book. It was even a mde-for-TV movie a long time ago, with Craig T. Smith playing the Deford part, but I missed the movie by a couple of decades. I haven't read the twins' book. They're right that chances are their mother carried some cauasian from somewhere along the line, but it's not impossible for any race to carry the one of the many gene mutations. I also liked "Beyond Breathing" by [Ithink] Margaret Cassalina. "The Drive at 35" by Andy Lipman, was too motivational-speechy for me. I've read a few othrs as well. Though it's not promising, my favorite was the Defor book. He's always been one of my favorite sprts writeers, and he carried the writing style to his book, sad as it was.

    The weird thing is that many genetic illnesses cn be tested for, by the /cf ene muttion occurs in so many places that they only know where to staart looking if a relative has it, and even the, they'd have a tough time finding it even if it existed on the other side of the family. On Jillian's side, of the four kids, Jillian has the illness and her brother Vincent is the only carrier. He and his wife already have two kids who do not have CF. They resumehis wife is not a carrier, but thy're quitting while they're ahead.

    Northern Europeans abd those of northern European descent are, obviously, the vast majority of carriers, and Jillian's parents are both Cuban, but their family was only in cuba for about two generations prior to her parents. Everyone came directly from Spain, but who knows where they were before they were in Spain.

    I read that those twins had double lung transplants. Lung transplants are practically the least likely transplant to take, but some obviously work extremely well.

  5. I remember reading "Alex" when I was about 13 years old. Alex was not even a year older than me, so her story hit close to home. The twins are also my age, but their CF wasn't as severe as Alex's. I was interested in reading about how their parents came together-- basically, both were affected by World War II and came together for a somewhat brief marriage which produced the twins. They said their mother asked her relatives about their ancestry, but no one wanted to admit they might have white blood from her side of the family.

    If you can get your hands on a copy, you should read it. It's a very good book.

  6. Becca, Jillian's case was atypical. They can't necessarily say it was a mild case, because she had pneumonia at least once basically every year of her life. On the other hand, if it was CF in its severest form, she would have died in infancy. Her parents and pediatrician knew something was wrong, and took her all over the place trying to find out what. The best explanation is that someone screwed up theneonatal test - either another child got the CF diagnosis worngly because he or she got Jillian's results, or the test was performed incorrectly in the first place. When her parents took her medical records to wherever they took her for diagnosis, they always had all her complete medical records, including the negative CF result from when she was a newborn. No one thought too question it becuase people don't screw those things up often. When it was finally done again after scott noticed her salty skin, the test screamed positive.

    Good things that her parents did were that they were extremely proactive each time she got pneumonia. She'd cough two times, and fifteen minutes later she'd have a 105-degree fever. Her dad would run a culture, then while waiting, make a wild guess as to the causative bacteria and put her on the antibiotic that would combat the particular strain while waiting for the results, which were always rushed. When the results came in, he'd change the antibiotic to the appropriate one if he'd guessed wrong. The proactive treatment kept the colonization of bacteria way down.

    Also, the kids were all tennis players. If Jillian wasn't sick, she was playing tennis and running daily since she was little. CF patients have to have regular aerobic exercise unless they are feverish or just barely getting over something and are too weak. The exercise gave her very strong lungs.

    What they didn't do was the thoracic physical therapy, which allowed too much buildup of mucus in her lungs and led to too much pneumonia, and also the mucus made its way into her digestive system and forced things through way too fast, so she didn't metabolize enough calories. Her mom's 5'7", her dad's 6'2", her three brothrs are all 6'3" and she's 5'2". it's unlikely she would have ben so small without the inability to absorb nutrients. She should have had the mucus shaken out of her and had some pancreatic drug therapy, and also probably should have had supplemental sleep nutrition gastro-nasally. Thye just didn't know.

    So it's been a mixed bag for her. Someone on Facebook told her that she couldn't have had it her whole life -- that she just contracted it as an adult, but in her case that makes no sense. What healthy child or young adult gets pneumonia at least once every year of her life, in addition to all the other things that went wrong?

  7. The thought is that it's moved along at a normal pace, but she had a couple of factors going in her favor. The goal with almost any CF patient is to postpone a lung transplant as long as possible and to treat it as a last resort. There are some that take wonderfully, but many do not. Before it gets to the point that a person is practically an invalid, that person needs to be on a transplant list, but a lung transplant is generally considered a last resort.

    At the rate Jillian's lungs are going because they're strong and the colonization of bacteria has been kept to a minimum, the treatmont of choice will be to blast the lungs with the molecularly treated oxygen as soon as she contrcts pneumonia, in addition to an iV antibiotic and a regular inhalation antibiotic, and suctioning the lungs right before the oxygen blast. Also, she has to try to stay out of situations where germs are prevalent. If she flies, she needs to be on oxygen with a mask. She has limited exposure to her nieces and nephews during flu and cold season. If she has a kid of her own, he'll play with kids from the family but won't go to preschool just to give her that one extra year before she has to deal with the germs of every family in the class.

    My dad was explaining some of it to me. It's a complicated lifestyle.

    Knotty, I should probably try to obtain a copy of the twins' book. Actually, chances are that Jillian's mom owns a copy. I should ask her.

  8. It sounds extremely complicated, and I sympathize. I was under the impression that she had always had difficulties, though up until the point that Scott realized that she had CF, it wasn't the severity of, say a person who received the diagnosis at birth, and through the years things just became increasingly worse up until this point.

    With transplants it seems like a catch-22. People who are candidates to receive a transplant are usually very ill, last hope status. Sometimes they're too ill to survive post transplant. I know a woman who has pseudo-obstruction syndrome and gastroparesis, who received a visceral transplant in Pitt a number of years ago. It didn't really help her problems, as she waxed and waned between feeding tube dependency and TPN dependency, and now several years later she's looking at having a second liver transplant because her new liver is failing. And she's TPN dependent now, which is making things more complicated.

  9. 'Jillian had all the symptomsform the very beginning the clogged airways, too frequent pneumonia, major digestive upsets. They had a hard time finding anything she could keep down that would also stay in her system long enough for her to get any nutrients from it. She had poor growth from the beginning, and the classic skinny CF neck. She always had it, but was lucky that the things they did not knowing it was cF were enough to keep her alive and slow down colonization.

    The time she came really close to dying was when she had practically simultaneous colon and ileum perforations and bled heavily. Those were both related to the gastric issues she's ad all her life with cF. They just monitor her digetive traact more crefully now.