Transplanted: My Cystic Fibrosis Double-Lung Transplant Story

My transplant story turned book is ready for pre-order!! The publishing date from Nimbus Publishing is April 30 if you want to wait and buy the book from a store. 

It's so official and it's hard to believe that me sending a manuscript to Nimbus with a "let's see what happens" plan is at this point where I'm going to have a book in stores. It started out as a compilation of blog posts from my transplant but through the magic of editing and being told by my early readers "this is much too long," it's now a concise narrative that explains the transplant process and about growing up with CF. I hope everyone enjoys it.

"When Allison Watson awoke that day, she knew she was in a hospital bed. That's all. She had no idea how much time had passed since she had seen her family. When she tried to focus, her vision was blurry, and when she tried to wave someone down, she became so exhausted she thought she was dying. Hours later, when Watson was able to communicate, she asked a nurse if the news was good or bad. "It's good news," the nurse replied. "You had your lung transplant four days ago."
About 4,100 people in Canada have cystic fibrosis, and many are living longer today, thanks, in part, to transplants. CF mainly affects the digestive system and lungs, and there is no cure. In this candid memoir, Watson describes living with the disease and her life-altering surgery in 2014. Watson and her sister, Amy, both grew up with CF, and Allison had always believed that Amy would be the one to get a transplant first. The decision to undergo surgery was not easy. Nor was the road to full recovery. In this book, Watson, who cycled across Canada with her brother in 2008 to raise awareness of CF, describes her journey."

The brain and the body

It's amazing how fast it took after finding out that my lung function has dropped to me feeling super tired and unwell in general. It's as though as soon as my body realized my lungs were crapping out, it just stopped trying.

I realize it is mostly psychological as one rarely goes from feeling ambitious and energetic in the morning, to having a killer headache and exhausted for the next two days. I mean, sure it happens, but seeing as it started to happen immediately after my clinic appointment, it's fairly clear that the appointment zapped all the energy out of me. I didn't even make it to pottery class last night.

However, just because my lack of energy yesterday and today is mostly psychological, doesn't make it any easier. There is now added anxiety of going to the hospital and if I'll have to go through the emergency department. I hope once I actually get into a room at the hospital, I'll feel a bit better. And perhaps, I truly am tired from my lack of sleeping-in this week and would have crashed out yesterday either way. I will never know.

It would be interesting to study the effects of being told your health is either improving or declining and see how you feel the next day. I would bet there is a good correlation between people believing that they're sick and immediately feeling ill. I would imagine it's been done at some point (a brief search turned up nothing for me) even though it would be really hard to get past a ethics committee. I can't imagine they would look kindly on researchers telling a bunch of healthy participants they had cancer just to study how ill the participants made themselves. Universities try to stay away from research that will get them sued.

The rollarcoast ride continues

Arghhhh!! The moment when I was starting to feel more energetic and my body was finally getting rid of the side effects from the antibiotics (nausea, bowel issues, etc...), I went to clinic today to discover that my lung function has dropped down to 19%, or FEV1 of .67. Surprise! Not exactly the news I was hoping for.

I knew that the bump from two weeks ago wouldn't last but I didn't think it would take two weeks to wear off. I was hoping to have at least a good 6-8 weeks as I'm still on one oral antibiotic (that is clearly doing shit-all). I was so excited to have finally responded to the medication but that was obviously a bit premature. I did not expect it to drop to the lowest I've ever been without me even noticing. How do I not feel that my lung function is down to 19%? This perplexes me so much.

I knew my lung function had declined some and wasn't awesome as I've been needing a bit more oxygen while walking around and feel short of breath more often, but I really didn't expect it to be as bad as when I had pneumonia. That time, I couldn't make it through the night without coughing, I could barely stand up straight for any period of time and couldn't make it through a shower without sitting down. I definitely feel better than that.

It's so weird to have your body crap out on you when you don't feel symptomatic. I wonder if this is how people with cancer feel when being told they have stage 4 even though they thought they were perfectly healthy in every way. Although for my hypothetical person with cancer, it would probably be way worse as they wouldn't be expecting it at all, at least it's not a complete shock to hear that my lungs are not doing well.  The delcine always makes sense in hindsight (so that's why I've been feeling more short of breath when walking to the bus), but it's shocking at the time. I think that just speaks to how well the body can adjust and compensate loses.

After the doctor grimaced for awhile and said a lot of uplifting things such as "your lung function numbers are freaking me out", "I don't know what meds to give you anymore", and "we're stuck between a rock and a hard place," she came up with a plan of action, kind of. I was sent home for two days on some new oral antibiotics and then once my sputum culture report is completed, I'll be admitted to the hospital to start IV antibiotics based on whatever I'm currently growing.

The solution that sounds easy "you were on the one antibiotic and responded well, went off it and crashed, clearly you should go back on it," unfortunately doesn't work if I want to keep my kidney for any length of time (and I do). The side-effects of that medication are pretty strong and can destroy the body. The nausea, vomiting, and diarrhea I experienced are considered 'mild side effects.' They don't like to keep people on it for longer than a month at a time and my month is up.

They also want me to try a new antibiotic in my aerosol mask. However, since it can cause chest constriction, I have to wait to try it while I'm in the hospital as (quoting the doctor) I "don't have much lung function to work with if constricted too much." Again, comforting words from the doctor. 

I could really use that transplant about now....every time I hit another bump, I think 'well, this must be it,' and so far it has never been 'it'. Maybe this time? I don't want to get my hopes up to high only to have the crushed once again. 

Off to St. Mikes I go.

I'm being admitted to St. Michael's Hospital. Booo!!!

Clearly my CF clinic visit today did not go very well. My lung function was down and the doctor said since I just finished a course of oral antibiotics two weeks ago, it was time to bring out the big guns (or meds, in this case).

They wanted to admit me immediately but since there were no beds available anywhere in the hospital, after waiting for 5 hours, they gave me the option to either go to the emergency room and be admitted through there (but the lack of beds means I would be in the general emerg section all night), or go home for the night and hope there is a bed in the AM. So obviously, I choose the second option.

If there is still no bed available in the morning, I'm off to emerg to wait it out there. The Dr said there is little chance of getting a room in the respirology wing as no one is leaving there soon but they would try to shuffle people around to put me in a good second option.

Fingers crossed on someone being discharged tonight because spending all day/night in emerg tomorrow sounds like a nightmare. Plus imagine the germs in that place *shudder*.

The doctor seemed quite panicked about letting me leave for the night but since I promised to rush to emerg if anything changed, she let me go. Her intensity made me feel like I should be more worried about my health but I'm not. I honestly still feel better than I did over the weekend and freaking out will do nothing to help my body. I'm just happy that I get one last home cooked meal and sleep in my bed before being admitted.

Surprisingly enough, I'm not feeling too bad emotionally about having to be admitted. Clearly, it's not my first choice and I would rather be at home but it makes sense treatment-wise. My lungs have been on a steady decline since June and strong oral antibiotics haven't helped on a long term basis. So, hopefully, the IV meds will help give my lungs a boost until I get my transplant.

The two major bummers are that David and Cindy are visiting this weekend so their Toronto vacation is going to be very hospital-oriented and that we have tickets to the Book of Mormon musical in two weeks and I doubt I'll be out by then. I can't do anything about David and Cindy's trip but hopefully my IV schedule can work around a 3 hour outing so I can see the show. However, there is nothing I can do about it right now as I'm not even at the hospital yet.

The one tiny silver lining in all of this is that once I'm admitted, I move from priority 1 to the priority 2 (weirdly, the higher) grouping. Better chance at getting lungs! Maybe the angry oxygen man that I met when I first arrived in Toronto was right about people only getting lungs after having a serious infection. Let's hope he is right about this one thing!

I guess I better start packing my bags for tomorrow. Urgh.

An explanation of how it sometimes feels to have CF.

I spent the evening tonight talking with Amy on the phone about the shittyness of CF. Well, I more listened while she ranted. She had a check up appointment today that didn't go as well as hoped. And there is nothing more discouraging than going into an appointment feeling fine only to be told that your lungs are actually worse despite all your healthy eating, exercising, and never skipping an aerosol mask. It's beyond frustrating. The worst part is when no one can tell you why you’re lungs are crapping out. You wish you had pneumonia because at least then you could point and say ‘that is the reason my lungs are worse.’ Then you feel weird about wishing you had pneumonia.  

The trying so hard and experiencing failure makes everything seem pointless. What’s the point in exercising when it doesn’t seem to be helping your lungs anyway? Why should you spend two hours each day on four aerosol masks when it doesn’t help? That is the thing with having CF, there are never any victories. Never a moment to say "yes, I’ve worked hard and put all my energy into treatment and self-care and now the disease will not bother me again."

It doesn’t happen. There is never a break. It can wear anyone down. You sometimes get angry at people who say ‘hope you get better soon’ (then immediately feel bad because they were just being nice) because you know while you may recover from a certain infection, you will never be able to recover to where you were 5 years or a year ago.

To put it intp the battle terms that everyone loves so much: You never get to beat or fight off the disease. You only experience losses until you have nothing left to lose. You never get a win. You might sometimes gain back some lost ground but it will never last forever. There is never a chance that it will one day it will be defeated and you’ll come out the victor on the other side*.

I'm not sure if people without CF or a debilitating disease would ever have a similar experience. Sure, everyone has disappointment and goals that they fail to accomplish. But have that happen over and over again and it's a bit like having CF. I wish I had a brilliant metaphor but the only thing I can think of is that it’s like standing at the top of a descending escalator, not wanting to go down. So you keep climbing the steps but it's moving too fast for you to make any progress. You try as hard as you can but you never make any gain. And then you try to sprint up it but you can’t keep that speed for very long and then you end up losing ground because you have no more energy. Or you stop for one second and suddenly you're lung function has dropped 10%....okay, maybe I'm bad with metaphors. I think you get the idea.

It just. never. stops.

The feeling of hopelessness can be unbelievably overwhelming. So overwhelming that it's sometimes hard to shake off or crawl out from underneath. It takes time to recover from a decline and there are only so many times you can put on a positive smile in the face of horrible news. You crawl out of it and shake it off, but it’s damn hard sometimes. Eventually you reach a point of acceptance that it’s out of your control. That it will happen despite your best efforts. And while you may not be able to ‘win’, you can sure as hell delay the loss for as long as possible.  

….I think that's the end of my rant. I've probably already posted one like this before but after talking to Amy, I needed to rant again. Because it's hard. If you didn't already realize it, having CF sucks.

 

*I just wanted to add as an aside, that while having a lung transplant may rid my lungs of CF and may seem like I have 'defeated CF', I still have the disease in the rest of my body. Also, transplants are hard and avoiding rejection is it’s own never ending process with little or no reward. Getting a lung transplant is just replacing the inevitable doom of CF to the-just-as-cheery-inevitable doom of organ rejection. It’s not a victory so much as trading one thing that will almost-definitely kill you right now with something that will almost-definitely kill you in the future.

St. Mikes Apt

Well, the pulmicort bump has officially worn off. I returned to clinic today and my lung function was back down to 22% with a .77 or .79 outflow (I don't really remember). Booo! My coughing has increased a bit over the past few days, especially on any kind of exertion so I wonder if it's the start of an infection. Or maybe it was because I was tired, or maybe it was the weather. Surely there must be a reason other than it being that my lungs are on the decline. I can rationalize this away, right? Right.

I don't have any other real 'getting sick' symptoms other than the fact that a few times this past week, my sputum has been streaked with blood. Even though everyone tells me that it's common for people with lung disease, coughing up blood always sends me into a panic. Not as much since it's been happening with more frequency over the past year but it's still enough to bring on the 'I'm dying' tears.

I was told if I'm feeling worse to take the oral antibiotics that were prescribed to me in May. They are a potent cocktail of drugs so I hope I can just sleep away whatever is happening as the drugs are as bad as the infection. I had a two hour nap this afternoon as part of my new 'sleep my way to better health' initiative.

Infection Control and CF

Cystic Fibrosis Canada along with the the Cystic Fibrosis Foundation (USA) recently brought out a new infection prevention and control policy. While I don't know what the old policy entailed, here are a few excerpts from the new policy:

• People with CF may attend Cystic Fibrosis Canada hosted or sponsored outdoor events but are advised to maintain a distance of at least six feet from other individuals with CF at all times. 

• The organizer(s) of Cystic Fibrosis Canada hosted or sponsored outdoor events may assist with voluntary self-disclosure through the provision of items such as clothing apparel, stickers or wristbands.

• Avoid activities associated with events that encourage crowding.

• Always be aware that there may be other people with CF present at the event, including those with B. cepacia, epidemic Pseudomonas, MRSA and other potentially harmful infections.

• Be aware that young children with cystic fibrosis are usually less vigilant about observing proper infection prevention control practices.

• Only one person with CF may be invited to attend a Cystic Fibrosis Canada hosted or sponsored indoor event or meeting. In the case where a person with CF has been selected or invited to attend a Cystic Fibrosis Canada hosted or sponsored indoor event or meeting, the organizer(s) shall make every reasonable effort to ensure no other persons with CF attend that indoor event or meeting.

Basically: Avoid each other at all cost and beware of the children!

While I understand that the infection control people are just doing their job, is it really necessary that only one person with CF can attend an indoor event? Even with outdoor events like the Great Strides Walk which seems like the perfect place to meet other people with CF, the 6ft rule means unless there is screaming involved, conversations between people with CF would be at a minimum.

I know that it is the job of the infection control people to reduce cross infection and the best way to do that is to isolate the cause of the infection, ie people. They are the ones who get yelled at if I go to a CF sponsered event and catch a weird germ. However, the policy and the constant need to live in fear seems incredibly isolating for people with CF.

How are you suppose to meet other people or families affected by CF if you aren't allowed to approach anyone or attend indoor events? I'm sure a lot of people break those guidelines with friends they already have but if you want to meet anyone new, the only real option available seems to be the Internet with its facebook, blogs, or CF websites.

However, while I'm sure those help some people find connections but personally, it's hard to find any that are actively used and those that are seem to be quickly taken over by people who want to promote their latest 'cure' or annoying people who post that their lung function is so high because of their positive attitude and everyone needs to be more positive (I wish I was kiddening...apparently I've been following the wrong treatment plan this whole time with my puffers and medication). 

Although to be honest, I've probably never given it a fair shot as growing up I've didn't have to look very far to find someone else with CF as Amy's bedroom was across the hall from mine. Having Amy around helped normalize the experience for me growing up as I wasn't the only one doing aerosol masks every day or taking pills every meal.

It helped growing up to be able to rant about a situation and have the other person know exactly what you mean. Sure, other people can sympathize but unless you have CF, it's hard to fully understand the frustrations of endless coughing fits, being exhausted from those coughing fits, struggling to breath deeply, having an obsure type of diabetes that no one understands, or having a million digestive problems.



Reading the new policy made me realize how without knowing it, having Amy to talk to has been invaluable in my life. I've never realized before how comforting it is to have a sister with CF until I thought what it would be like to not have anyone to call about a weird lung pain that is bothersome but enough to have to go to the hospital, or to rant about the gross-ness of Boost, or to ask how many pills she takes with a smoothie.

I think I would feel isolated if I didn't have Amy to text at all hours about all my problems as it would be hard to have these experiences and not be able to talk to anyone who 100% understands. The new guidelines, while trying to ensure that everyone leaves an event with only the same germs with which they arrived, will make it even harder for those with CF who want to make connections with other people with CF.

St. Mikes appointment

I had a doctor appointment today and while it wasn't all gold stars and high fives, it could have been worse.

My lung function awesomeness of 28% from 2 months ago has not maintained and has returned to my apparent baseline of 24 or 26% (in my disappointment, I didn't remember what the doctor said but it was either 24 or 26%). St. Mikes CF team seems to be more focused on the actual FEV1 number rather than the percentage so if you prefer that, I've dropped from an output in the first second from .9something liters to .8something liters which is still higher than pre-magical-puffer of .77 liters.

The drop wasn't enough for the doctor to want to do anything about it as I'm feeling fine energy-wise and my cough is about baseline (although it's so hard to tell). While it is disappointing, I'm not shocked by the news. I felt my 28% was too good to be true or to be maintained more than a month. While a part of me is hoping that my results were lower today because I was more tired or something, my realistic part thinks it's probably just my lungs getting off the initial steroid high and becoming more accustomed to the medication. It won't be long before I start asking for the better steroids.

In other news, I've lost a few pounds which I don't feel is a huge deal seeing as overall I've gained about 10lb since being here but dietitians always seems to panic whenever there is any decline. I promised to eat more chips and less veggies, even though it's super hard with all the fresh fruit now in season. I just want to eat bowls of fresh strawberries and raspberries. Probably why I lost the pounds in the first place. More whipped cream for me!

I asked about the results from my echo to see if I really do have a heart murmur but of course they had no idea and couldn't get ahold of anyone from the Toronto General to find out. The doctor reassured me that someone would be looking over the results but seeing as how every appointment the doctor reads my chart and looks up the tests for the first time while sitting in front of me, I have my doubts. Apparently someone will call if there was a problem or more likely, I'll find out during my next transplant clinic.

My last exciting piece of news (the excitement bar is set pretty low for dr apts) is that I met my new transplant coordinator. The old one who I saw on listing day to sign papers, handed me a binder, and have never seen again, turns out she has retired and I now have a new person to never see. Although maybe since this person is new and young, she'll be more excited about her job and check in once in awhile.

Transplant Clinic

I had a doctor appointment with the Transplant Clinic team today and much to my surprise, I was out of there in 30 minutes. It was a very pleasant surprise.

The doctor reviewed the results from the Echo and CT scan I had done in early June. The CT scan showed an increase of mucous in my lungs, particularly my left lung, since last year. The doctor recommended that I increase the frequency of my chest compressions to try to dislodge some of the buildup. It's not surprising that there is more buildup since last year considering everything that has happened in the past year.

The surprising part of the visit was that my Echo showed that I have a small murmur in my left pulmonary vein. At least I think it was the pulmonary vein, the doctor rambled off the information so fast that I didn't really catch it all. He called it something that had a really long name, I think it was cardio-something-scary-sounding-osis. However, I was told not to worry as my echos have always been normal so he thought it may be that the technician was being over cautious. He is going to check with the cardiologist to get a second opinion. I foresee a repeat Echo test in my future.


Other than the Echo and CT scan, there was nothing to talk about as once again, the doctor couldn't find any of the information from my appointments with the CF team.

For those who have asked me recently about the record wait time for a lung transplant, I tried to get the answer from the doctor but he refused to answer. Instead rambled on about how the average wait is 6 months (it's a different average from every person), and that since I'm level one, the wait can take longer (obviously). I repeated my question three times but he just kept repeating that the average wait is 6 months. He could have just said he didn't know.

Great Strides Walk!

Today is the Great Strides Walk where people all over the country will walk to raise money for CF research. Thank you people across the country! And thank you to everyone who donated to those people! If you wanted to sponsor my family and forgot, there is still time. You can do it here.
Even if you just want to leave a message wishing them well, they would appreciate that too. Thank you to everyone who supported them and CF research!

Speaking of CF research, really randomly, one of the ladies in my pottery class works with sputum samples from people with CF from the St. Mikes and the Sick Kids Hospitals. When I told her that I was waiting for a transplant due to CF, she was excited to tell me all about her research. She is an evolutionay microbiologist who studies the evolution of one of the common viruses in people as they age.

They hope that by understanding the adaptations of the virus, they will be able to come up with better treatments over a persons life. We tried to figure out if she would have any of my sputum samples but all of hers are over 8 months old so she wouldn't have any of my stuff. However, someone else in her lab looks at the antibiotic resistance of the new samples so my sputum has definitely been there. The research is happening and it's pretty exciting. Her research won't help me now but hopefully it can help young people with CF so they can keep their lungs as healthy for as long as possible.

Hope everyone has a great walk today! 

The CN Tower was blue and turquoise last night in honor of CF Awareness Month and the Great Strides Walk. I'm not sure how much awareness is raised by turning the tower blue but judging by the Tower's list of lightening schedule , it seems to be a thing that organizations do.

Lung decline in the family

People often ask why it's me and not my older sister, Amy (who also has cf), who requires the lung transplant. Logically, it feels like the older person should require a transplant first. Unfortunately, cf doesn't behave logically and there is no way of knowing why I am the one who requires a lung transplant first.

Well, that's not true, the reason I need the lung transplant is because her lung function is significantly better than mine and she would not be eligible for one. But as to why my lungs crapped out before hers, who knows! Cf presents differently in everyone so although we have the same genetic mutation, there may be a variable that makes me more susceptible to infection.

Or maybe it's not genetics at all, maybe I was exposed to more viruses over my life or maybe I didn't exercise as much or maybe she didn't skip her aerosol mask that one time, or maybe...or maybe... the reasons could be endless. I've given up wondering 'what if' or 'what could I have done differently.'

The thing with cf is that there is such a slow decline in the lungs that it is hard to pinpoint overall health to a specific infection or behavior (unless you smoke or refuse treatment or something obvious). 

So basically, I have no answer for you. I realize that isn't very satisfying but such is life.

Caregivers.

Ok, enough complaining from me about being sick. Let's move on to more interesting things. Like this article in the Globe and Mail about how illness in women is related to an increase in divorce rate. 

"The newest study, presented Thursday at the annual meeting of the Population Association of America in Boston, found an increased risk of divorce in older couples when the wife – but not the husband – became seriously ill."

Awesome.

The article typically blames the fact that men are not 'traditionally caregivers' and that they flee at the idea of having to care for someone (I may be paraphrasing). However, a women from the Canadian Cancer society said that women shouldn't be afraid of being abandoned when they get cancer because the study just looked at people with brain cancer so it shouldn't be extended to all types of illness (not so hopeful for those with brain cancer). Also, she says that the study didn't look at who initiated the divorce so maybe the women are deciding they don't want to spend their now limited years stuck with a super annoying partner (again, paraphrasing).

The whole point of the study was to look into caregiver supports and ways that that social workers and doctors can better support a couple in the stressful time. This is important because being a caregiver is a tough thankless job. The man that they profiled (he photographed his wife's struggle with breast cancer for three years) said that he 'has never been as happy as I was during that time.' That does not always happen.

Being someone's caregiver is hard. It's exhausting and challenging and while it may bring some couples together, it can just easily drive them apart. There are countless reports of caregiver burnout; stories like how the caregiver becomes sick from the stress and refuses to seek medical treatment because they refuse to take any time for themselves.

Burn out can happen so easily if the illness is prolonged or if things don't improve as expected. Caregivers can have guilt over taking a break because 'why should they be having a good time if the other person can't.' Or have fear that no one else would be able to meet the ill person's needs as well. Or feel like it is a failure if they are unable to provide all the support. Then there is the extra guilt about venting frustrations because no one wants to be the jerk complaining about how hard it is to take care of a person with cancer. No wonder so many couples end up separated.
 

I think that Isaiah and I have been pretty good in avoiding caregiver burnout. It helps that while he has been picking up more than his share of the housework, I can still help out some and have my own activities. He still has his weekly board game nights and often goes out cycling or to the market during the day. Also, my family consistently remind him that they can step in, if he wants to go back to NS for a bit. The fact that he is the most laid back person ever, helps when dealing with stressful news or when I freak out with emotions.

One of the things that isn't often discussed and that I have found the most challenging is finding the balance between being partners as well as being in a caregiver/care-y (care-ie? care-e? being the sick person?) relationship. It really changes the dynamic of the relationship and can be hard to figure out.

I've spent the last hour writing and re-writing how to best explain the struggle and I honestly can't find the right words to convey the frustration and helplessness and vulnerability and all other emotions of having your partner become your caregiver. It adds such as weird element into the relationship that was never discussed in any relationship advice guide I've ever read (not that I'm super proficient in relationship advice books). Honestly, it's still something that we are trying to figure out but at least he doesn't seem to be having caregiver burn out at the same time. 

Feeling feelings.

Being sick has brought back all my 'ahhh I'm dying' feelings. I started having them after my last doctor appointment when my lung function was low but Amy's visit helped distract me from it all.  I was hoping that enough time had past that I would have subconsciously processed all the information but apparently two weeks is not long enough! It doesn't help that I got sick and don't have the energy to leave the apartment.

Stupid feelings. I thought I dealt with all of them awhile ago. They shouldn't be allowed to return. Arghhh!!! The thing is that when I'm 'healthy' (ie. better than right now), the whole 'dying' part isn't as prevalent. It's easy to ignore that I was given 2-3 years to live (without a transplant) because I can still participate in activities I enjoy. I still get to go to museums, shows, pottery class, do art, and read books but when I get sick like this weekend, than all I seem to do is curl up on the couch and think about death (good thing Netflix exists otherwise I would feel even worse).

I know I should expect this more. I'm sure everyone has feelings of 'arghh I'm dying!' when they are on the transplant list. And I know this is just a passing phase. I know that most likely in a few days, I'll start to feel better and my coughing will reduce from a thousand to a hundred times a day. I just need to process this latest bump in the road and move on. I know that I'll get through this but right now, it just seems hard to shake.

Arghhhh

I hope everyone had a great Easter weekend! I had a great time with family eating yummy food. So much so that my weight was still up today at my doctor appointment with the CF team at St. Micheals. Yay! I actually believe their scale this time because my scale at home also tells me that I've gained weight.

I've been feeling good health-wise since my last visit so I was confident that my lung function was going to be the same or up. And I was wrong. It was down by 2%. Aaaaarrrrgggghhhhhhhhhh. I really wanted to scream that their machine must be broken but since I did the test three times with the same results so that probably isn't the case. Stupid lung function.

I was super nervous they were going to hospitalize me or start me on a host of crazy antibiotics that would zap all of my energy but thankfully no. The doctor said because my lungs didn't sound worse and that I don't feel sick, it wouldn't make sense to pump me full of more meds. Obviously if I wasn't on the transplant list they would be treating it but they seem to be hoping that I can remain stable-ish until I get a transplant. I go back in a month and hopefully it will have gone up again and I was just having an off day today or something.

I thought this would change my transplant status so I would now be in the 'high priority' grouping as  the nurse practitioner went on and on last week about to the importance of telling them when anything changes. But the doctor today said it wouldn't. They really need to make their information a bit more consistent as now I really have no idea what classifies a person as 'high priority.'


I'm pretty bummed about my lung function dropping but I'm trying to be zen about it and am repeating to myself that it is pretty much out of my control at this point. I will focus on the fact that if I wasn't working hard at physio than it would probably be even worse.

Amy arrived today and I imagine I'll be busy too busy with her to obsess over this latest setback. Or blog. If I don't update as much this week, it's because we are off dining on copious amounts of sushi and apple fritters.

Coping with physical decline.

Today at physio, there was a person (I'm keeping it vague for confidentiality reasons) who was struggling with the 20 minute treadmill walk. They were checking their oxygen levels every 5 minutes or so resulting in a lot of loud beeping as the machine beeps whenever levels are below 88%. Afterward, the physiotherapist had a long talk with them about how it was time to decrease the intensity of their workout as their oxygen levels have been on a slow decline (I realize that it is super creepy that I know their entire conversation but it's really hard not to overhear what is happening 2 feet away). The person looked so upset that I wanted to give them a big hug and tell them it is not their fault their lungs are failing.  

The person looked how I felt back in Nov after my 6 min walk test when my oxygen levels dropped earlier than they had in the test a month prior. Despite the fact that I had started physio during that month and was doing all I could in that time. It is so hard to be physically declining when you follow the treatment plan and exercise three times a week. It can feel like there is no point in trying because trying doesn't stop the lungs from failing. It does not guarantee success.  Hell, not only does it not guarantee success, it doesn't even guarantee being able to maintain a level of health.

Everyone with CF or any other degenerative disease has had to come to terms with this lack of success at some point in their lives. The idea that if you work hard you can succeed and 'beat it' doesn't work for us.  There is no 'beating' some diseases. We don't get to see it slowly reduced or eliminated from our bodies. It is something that is always there and is always getting worse.

You can do everything right; aerosol masks twice a day, exercise regularly, stay away from smoke, eat healthy, take all medication regularly, and still end up needing a lung transplant. The best you can hope for is to delay the decline as long as possible. Following a good treatment plan can help but the disease will progress despite anyone's best efforts. The hard part is that the body adjusts so easily to small declines that the change can be almost imperceptible on a daily basis. Until you wake up one day and wonder when it became so hard to climb a flight of stairs.

I don't think the frustration of degeneration gets any easier. Nothing is harder than making the best possible effort and still feel as though you are failing. It is hard to adjust from blaming yourself to accept that it's out of your control. Or to accept that all you can do it control what you can and realize that while your efforts will not stop the decline, it may help delay it somewhat.  And sometimes delay is all a person needs. Delay can mean the difference between transplant or no transplant.

Yay Times and Transcript!

The Times and Transcript did an article about me! And it was in today's paper. Eeek! Unfortunately the online copy of the paper is behind a paywall so I can't link to the article but the gist of it is that I have CF and I'm waiting for a lung transplant and that I wrote a book about my cycling trip during my free time. All stuff that you most likely already know if you're reading my blog.

The journalist who called me asked if I had any 'words of encouragement for younger people who have CF or anyone who has a disability.' I kind of froze and couldn't think of much on the spot but of course thought of a better answer a few hours later. If I had to answer that now it would be something like: "Don't let yourself or your life be defined by your disability. It is amazing what you're capable of when you ignore the perceived limits that come with your label. Take opportunities when they arise because you never know when you're going to get a second chance. Focus on the positive and when you're struggling, don't worry about the future and take everything just one day at a time." (that blurb just about covers all the wisdom I've accumulated over the last 27 years)

I love that a staged-book-reading selfie made it into the paper.

Alternative therapies

Writing about cupping got me thinking about all the alternative therapies for CF that I've looked into over the years. I have phases where I look up what the alternative medicine world has to offer and to see if they have discovered a cure and forgot to tell people about it. I have yet to find one.

Yes, there are blogs out in the blogosphere about people treating their CF through oils and herbal supplements. There will always be one person on the Internet who says the latest thing has worked on them (not surprisingly, a lot of them are then willing to sell you the product). Reading their stories wants me to abandon all of my pharmaceutical medication just like them and start drinking salt or silver (yes people do this) or whatever it may be. That is, until I read that the person has a lung function of 80% (mine is 24%). So perhaps it does work for that person but I can't even remember when I had 80% lung function. Are the persons lungs at 80% because they drink the stuff or do they just happen to have a different type of CF that does not require as much medication?

CF can be so varied from person to person as the disease presents so differently in each individual. Some people get more lung issues, others more digestive issues, the lucky ones get hit with both. So if someones lungs are healthier to start off, often the less invasive treatments will be all that is required to maintain that level of health. To say that a treatment that helped someone with a lung function of 80% is going to help my poor 24% lungs would be absurd. And that is part of the problem with looking at these alternate therapies on the Internet. One case study is not going to sway me when the scientific studies have shown results that are not statistically significant. 

The problem with Internet research is that people are often very passionate when they find alternative method that works for them. I have yet to find a blog where the person said how much they loved their Symbicort puffer and how much it changed their life. However, there are quite a few blogs when people talk about how much Reiki treatments, Himalayan salt, colloidal silver, or an oil concoction increased their lung function by an almost unbelievable amount. I can't see anyone passionately blogging about their puffers (perhaps that will be a topic of mine next week) even though the majority of people with CF use them as a treatment. 

The Internet can be a great equalizer when trying to find information which is not always a good thing. People with an opinion about a topic can make a website that seems as credible as a medical researcher who is writing about the latest studies. There is so much information available that it is hard to decipher what is true in the depths of Google. I tend to skew on the side of being skeptical until I have talked to a health professional who can give me their opinion about the topic. They have the expertise and have talked to way more people than me to know about the latest therapies.
 
Before you start telling me all about how the evil pharma companies have control over everything, let me just say that I am not anti-alternative therapies. But I am also not anti-pharma companies either. Pharmaceuticals are the only reason I'm alive. I sit somewhere in the middle where I think that well researched medicine and treatments are absolutely necessary but that, of course, exploring other options can be beneficial. In the same way that drinking a lot of water, sleeping well, and eating non or low-processed food is beneficial. Helpful for overall health but not enough for me to abandon my puffers or powerful aerosol masks.

Doctor appointments.

It seems that the weather is not cooperating with my declaration of it finally being spring as it is still freezing cold out. Maybe next week will be a bit better.

I had a doctor appointment at the CF clinic today. It was not very exciting. They took some blood and I agreed to be part of a study looking at diabetes, CF, and exercise. They claim that I gained 2 kg in the past month but I think they are wrong.  There is no way I gained that much weight in one month. I think it was different because they used a different scale than they have for my past visits. I also think it is wrong because I according to our bathroom scale, I have not gained 2 kg. I told them not to be too disappointed when next month I lose about 2kg but they didn't listen to me!

I also had a resident doctor who knew nothing about CF or the transplant process. I understand that people need to learn but can they do it with the other patients? His top three ridiculous questions of the day were: "Did I know when to expect the transplant?" "Was I working?" and "Is there a CF clinic in the Maritimes?" I understand he is just learning but surely the other patients in the morning would have given him some idea of how the transplant process works? Or maybe he should have read about it before attending a CF clinic? Or maybe he should have looked at my chart for 10 seconds before asking me all the silly questions. Most of my frustration was because it was 2pm and I had been there since 11am and I really just wanted to talk to the doctor and go get some lunch.

It was all good news though! Well, 'good' might be a stretch, it was all the same expected news. It seems that my head cold was just a head cold as I had thought as my lung function remains steady with fev's around 0.79 or 24% (still not very awesome). So I basically have to just keep doing what I'm doing and I see them again next month.

I did learn that a random side effect of the transplant medication is that Vit A and E absorption tends to increase so peoples' levels super increase for an unknown reason. One less pill I will have to take! Ignoring all the anti-rejection meds I'll be on, of course.

I am not my disability.

On Friday, one of the physio assistants called me 'a cf'. Not a 'cf-er' (which I've heard a plethora of times), a cf. She looked at me standing in the doorway, turned to her colleague and said "we have a cf at the door."

Argh! How many times does it have to be said? "I AM NOT MY DISEASE!!"

(No, I didn't say anything to her because I have to still see this person three times a week and rely on her for stuff like 'getting my weights' and 'cleaning the room.' Let's save the 'people being uncomfortable speaking up to professionals on whom they rely' conversation for later.)

I know that she probably didn't mean to dehumanize me with the comment. She was probably just commenting on the fact that I have CF which meant that she needed to figure out which side of the room I should be on and whether or not she will have to clean that side before I enter the room. However, at that moment, being called 'a cf' reduced my identity and person-hood to my disease. Nothing else about me mattered.  

I know there are people out there who use the word 'cystic' or 'cf-er' to describe themselves in certain situations (note the 'er' attached to the 'cf', if nothing else, at least there is a 'er' to attach the disease too). That is a personal decision and one that each person get to decide. Not you. You do not have the right to define me by my disease. I am a person first, a disability last. I do not like being referred to as my disease any more than you would enjoy being called a 'acner' or 'dandruffer'.

When we call each other by a medical label, we reduce that person to their disease. We are saying that we see that person as first having a disease or illness and second as being a person. We reduce each other to a condition on which we then use to base all of our future interactions. Calling someone 'a cf' or 'epileptic' or 'autistic', reduces that person to one characteristic about their lives over which they had no control. Why would anyone have the nerve to assume that the other person wants to be defined by a medical diagnosis?

Look, I understand that we, as humans, are lazy when we talk and it is faster to say 'cf-er' or 'disabled person' over 'person with cf' or 'person with a disability' therefore the former seems to be the go-to option. But when doing so makes a person feel dehumanized and as though they are being reduced to their disease, we have to get our butts in gear and start treating and calling each other people first and foremost.

Language is important. Let's be more aware of how we use it. 

I'm sick, Booooooo.

Since Sunday, I've been feeling more tired than usual. On Monday at physio my oxygen levels were a bit low and I found the work out to be harder but I was hoping that tea and sleep could get me out of the lull. Then on Tuesday when I coughed up some blood, I figured I was actually getting sick. I have only coughed up blood (or had a 'hemostasis episode' as the doctors say) a few time in my life and it has freaked me out every single time. Especially when the blood is fresh looking. In Hollywood, people always cough up blood and then immediately die so surely that must be true? Apparently no. Hollywood has once again let us down.

Previously, when I've panic-y called my doctor about coughing up blood, she has always said that it was probably just irritation from coughing so much. The doctors never seemed to understand why I would be panicking.Well doctor, it's because it is scary to cough and then have blood come up!

Thankfully, I already had a transplant clinic scheduled for today. This was my second transplant clinic and so far they have both involved sitting around for hours for a 10 minute doctor visit (despite being told both apts I will be seeing the transplant coordinator, dietitian, and maybe the social worker).  I told the doctor about the week and asked if I could get an x-ray to see if I had pneumonia. She agreed and send me for an x-ray, blood work, and nose swab.

She sent me home and called me around 6:30 to say that I don't have pneumonia! However, I do have elevated white blood count levels so I am to start an antibiotic first thing tomorrow with strict instructions that if I cough up any more blood to go to emerg. It has been about a year since I've needed an antibiotic for a inflammation so I guess yay for that!