Cross-posted: When a Doctor Said 'Hope Won't Help' My Chronic Illness'

An article I wrote is up on The Mighty​ today. Below is a clip of the article. Please feel free to read more at the source.

"The day that I was diagnosed with pulmonary hypertension, a doctor waltzed into the room and announced that I had five, maybe 10 years left to live. He had spoken to my parents first about the news. I looked over at my parents to gauge their reaction. Was this really happening? My mama said very strongly, “It will be OK, because we have hope, and we will get through this.” The doctor seemed almost angry by what my mama said. He nearly cut her off to say, “This isn’t cancer. Hope won’t help you.” That was pretty much the end of the conversation. Well… actually, it wasn’t.

I was going to leave this part out, but it didn’t feel authentic. After what the doctor said I threatened to end my life. If hope couldn’t help me… if there was no hope for me, how could I possibly face each day? I was instructed to go have more blood work and go home after my parents convinced the doctor I would be safer at home with them than at their ward. It was a few days before Christmas. None of this felt like the movies."

Please follow the link below to read more:

http://themighty.com/2016/03/pulmonary-hypertension-how-having-hope-helped-me-get-better/

The Side Effect That I Wasn't Warned About

"oh brother"

When you are first diagnosed with something, you are usually warned about side effects. Upon the diagnosis of Idiopathic Arterial Pulmonary Hypertension I was warned about side effects of the disease and side effects of the medication. PH can cause a variety of symptoms, or side effects, such has shortness of breath, chest pain, swelling, and heart failure. Some of side effects of some of the medications I take? Liver failure, cancer, anemia, low blood pressure (in all the wrong places,) head aches, stuffy nose. General malaise is probably a side effect of both the medications and the PH- and maybe life. I was prepared for all of that, but then something else set in.

Guilt.

I have suffered from both external and internal guilt as a side effect of PH. I feel such incredible guilt, although the feeling has faded as the years have gone by. I sometimes still find myself plagued by it. It is difficult to see how such a catastrophic that I had such little control over runs down hill. It effects so many other parts of my world.

I feel guilty because my life isn't the only life effected by all of this. Sometimes I find myself saying "sorry, I am sick." Apologizing for something that I have very little control of. Saying sorry, even though I know these situations will happen again. Unable to work, I still live at home and rely on my parents. I am sure they planned to retire soon, and I feel like I messed up their golden years because my golden years currently overlap with theirs.

Me living at home is also expensive for my parents. There are obvious medical costs, plus my crazy expensive food (because I eat mostly fresh and organic foods.) I see how my side effects so deeply impact them in various ways from socially to their well being. I hate that something that happened randomly hurts us all so deeply. I try to handle this human experience as graciously as possible, but it isn't always possible to sugar coat things. A positive attitude will help get you through the day, but it won't change the fact that I struggle going up a flight of stairs. I also think that you have to admit when things suck. It wouldn't be healthy to ignore your challenges, or to try and dress up a wolf as unicorn and hope that it doesn't eat you.

I feel guilty because the life I planned with my boyfriend is now very different. Sometimes I feel like I have very little to offer to the relationship. On paper, I feel like I kind of sound like a lemon. "Free to a good home: 28 year old girl-woman who can't drive, work or breathe on her own (for certain basic activities.) Very expensive special diet. Comes with Boston Terrier that you must also take care of." Hopefully from that line you can tell that I have a sense of humor, which is one of my selling points, beside my really cute dog.

Spencer has stuck by my side through my diagnosis, which I am so grateful for. (But it also has made me feel guilty.) He has so much life a head of him, and I don't want to stop him from following his dreams. This experience has really shown me what is valuable. Through all of this proverbial crap I have become a more supportive and grounded person. However, that doesn't change the fact that I probably won't have biological children. Or the fact that I am not sure if I will be comfortable adopting because a few doctors gave me the life expectancy of a hamster at the start of all this. I worry that my situation will effect what jobs he will want. I don't want him to take the job with benefits over a dream job because of me. I don't want him to have to take a high paying job that he hates because I cannot work. We were supposed to be partners. Equal partners with different strengths and weaknesses to balance each other out. I am strong as hell- but are these new found strengths valuable for "real life?" I feel like I am not apart of real life, but a mere by stander watching from the outside. I know he doesn't view me as an invaluable counterpart, but sometimes I wonder what I truly can contribute to our relationship.

I also feel a lot of internal guilt. At the very beginning of all of this I blamed myself for getting sick.

"I must have done something wrong."
"Healthy people don't get sick."
"Was my diet really that bad?"
"Did I really stress that much more than my peers?"
"What did I do so differently than other people my age that this happened?"
"Am I being punished? Did I get sick because I am a bad person?"
Those are some of the thoughts that I really struggled with. I felt as if that I must have done something wrong to get sick, especially because I was diagnosed with a rare disease. Overtime I have tried to accept that the perfect storm must have been going on in my body that allowed the disease to develop. I didn't do anything to "deserve" this, it just happened.

I believe that holding on to and hoarding negative emotions are not healthy. I have made progress releasing a lot of the guilt that I feel over being sick. Obviously life happens, and the feelings bubble up from time to time. However, it is important to handle those feelings appropriately. I accept that I didn't do anything wrong to get sick. There was probably nothing that I could have done to prevent it from happening.

I also accept that my parents love me so deeply and unconditionally. My parents helping me, taking care of me and going well above and beyond the call of parents of a 28 year old child are all an act of love. I know that them taking care of me, and supporting me isn't even a question, hesitation or thought for them. They want to take care of me. I know that they would want me to feel loved through their actions, and not like a burden.

I also know that Spencer loves me, and rarely sees the disease. He understands that although being sick dictates very important aspects of my life, the disease isn't who I am. He supports me through all of this, and sees who I am truly am- which is why he sees me as an equal and valuable counterpart to the relationship. I know that his companionship is something that he chooses to offer me, and he that this is another act of love.

It has taken me a few years to slowly release all of the guilt, and to slowly accept all of the love that others want to give me. I've learned that being sick isn't the time to start denying yourself love and simple pleasures. For me, it has become a time to indulge in all the good things that I can. For me, I try and find the beauty in every day.

(Please note: I refuse to refer to it as "my disease" or "my illness." I have no ownership over it, and it is not a characteristic or trait.)

Happy International Nap Day!

Sammy enjoying one of his many daily naps

Happy International Nap Day!

As someone with Pulmonary Hypertension I know how valuable a good nights rest and cat nap can be. I couldn't resist the opportunity to write about the benefits of napping for this special occasion.

I come from a family of troubled sleepers. Getting a good night's rest is something that I have struggled with since my youth. Throwing a sleepless night on top of dealing a chronic condition can feel like the ultimate recipe for disaster. A lot of people with conditions like Pulmonary Hypertension can tire easily because of all the extra work their body is doing to function. Having lower than normal oxygen levels can also be very tiring on the body. I know that when I don't have a good nights sleep I usually have a harder time functioning the next day. Sometimes it feels like I have a flare up of symptoms as well. Other times I can get a great night's sleep and still feel tired.

Along with struggling to get a good night's sleep, I have always tried to avoid naps. When I was younger my mom would try to encourage me to take a nap. I would always say that I didn't need one and try to wiggle my way out of it. My mom, being the smart lady that she is, said that if I didn't fall asleep after resting for 10 minutes that I was allowed to get up. This trick always worked, and I always ended up napping despite believing I didn't need one. This has been a habit that I have recently adopted again as an adult. I will often lay myself down for 10 to 15 minutes. If i don't fall asleep, at least I rested and relaxed. Making time to rest and relax and something precious that has been lost in our society. We often think that if we make time to surf the web or watch TV that we are making time to relax and rest, when that simply isn't the case.

Sometimes I feel like a need a good nap after having a big night the day before. I am always sure to set a timer because napping for too long can actually make you feel worse. The sweet spot for napping is 45 minutes or less. If you nap for more than 45 minutes you might end up feeling more tired. I enjoy 20 minute cat naps because they leave me feeling recharged. Power naps are great if you just need a little rest. It will leave you feeling refreshed (and in a better mood if you are like me and get reeeallllyyy cranky if you are tired!) If you decide that you need a 45 minute nap, you will benefit from going into REM sleep which can aid with creativity.
Casper has created 'Napping 101' to tell you more about the benefits of napping, and how to achieve the perfect nap. I tried to avoid napping after diagnosis. I didn't want to admit that I was tired or that I needed extra sleep, mostly because I didn't want to seem "sick." I felt ashamed of needing naps in my 20's. I have learned though that napping is for people of all ages and abilities. My boyfriend (who is younger than me) naps after playing hockey or having a late Saturday night. Napping is for everyone, regardless of their health situation. In fact, our bodies are designed to want to nap because human survival depended on alertness.

The benefits from napping range from an increase in productivity, to reducing stress and anxiety. The best times for napping are between 1 and 3 p.m. Avoiding napping after 4 p.m as napping too late in the day or evening can interfere with your regular sleep time. Make sure your nap area is a comfy cave with a comfy mattress. I always change into comfy clothes, hop into bed with my teddy bear, eye mask and of course...my supplementary oxygen! If you don't have an eye mask, I would recommend one for napping as it can help block out the sunlight. I also use my eye mask at night. I find it works as a "horse blinder" and encourages me to sleep. As I mentioned in a previous post, a bit of Badger Balm Sleep Balm hits the spot as well! The Sleep Balm has lavender and bergamot oil in it which is super soothing when trying to wind down to sleep.

Please see the Caper 'Napping 101' image below to learn more about the benefits of napping, along some helpful tips on how to become a napping expert!

Happy International Nap Day! Do you have any favorite napping habits? Share in the comments below!

Accessibility of PAH Therapies in Canada: Part III - Patient Testimonials

Part III: Testimonials From PH Patients Who Are On A Treatment Not Available or Accessible in Canada

This is the third and final part of the series. The first part of the series, Access to Opsumit in Canada can be found here. The second part of the series about Treatments That Are Not Available in Canada Can Be found here.

Below are testimonials from PH patients in the US who are on inhaled PH medications that are not available in Canada. These testimonials are unpaid, and are shared by fellow PH patients in an effort to show the positive effect these medications can have for some PH patients.

"I started Tyvaso on the trial in November of 2004 at UCSD medical center. I was asked to go on the trial due to my high numbers.  I was diagnosed in April of 2004.  My mean pressure in my heart during right heart catheter was 96(normal is between 15 and 25).  I had gone years with a misdiagnosis of severe asthma.  I went in for another right heart cath to start the trial.  My numbers had come down, good news to 56 with the use of a medication called Tracleer.  While on the operating table, I took my first puffs of Tyvaso, I took nine.  I was the first patient ever to take nine puffs of the medication.  My mean pressure dropped from 56 to 46.  It was really a miracle for me.  At the end of the study my pressure was 35.  Darn right near normal.  Sadly though, I had to quit the Tracleer and start on Revatio due to liver problems.  But I have been on Tyvaso since starting.

I started going downhill a little November of 2013, I was needing evening O2, and usually I need only at night time.  At this time I was on Revatio and Tyvaso.  I had another dreaded right heart cath in March of 2014.  My doctor was happy with my pressure of 50.  But I was not.  My husband and I pressured him to add another medicine.  He decided to add opsumit, since I did so well on the tracleer and it is very closely related to tracleer without the liver complications we decided to give it a shot.  Fast forward to June of 2015.  I had an ECHOcardiogram.  I asked my doctor my "estimated" pressure as it is not as thorough as a right heart cath.  He said 29.  Wow...almost normal.  I have been feeling pretty good these days.  I am very reactionary.  I have good days and bad.  Without these medications I would for sure be in pretty bad shape of worse dead.  I have 3 adult children and a husband.  A grandson.  Also, raising my niece and nephew, ages 15 and 12."
- Julie, 48 years old
San Diego, California, USA

"I started the inhaled therapy, Treprostinil (Tyvaso), in 2011 after going back and forth with my doctors about IV therapy since my pressures were high and my physical activity was low.  They decided to try me on Tyvaso just to see how I would respond on it.  At first it was a bit difficult to fall into a routine of breathing on a nebulizer every 4 hours, but I wasn't too concerned after I started to feel better.  Before Tyvaso, I'd have a very hard time doing the simplest daily tasks like making my bed, or even showering, without getting short of breath.  Now after almost 5 years on Tyvaso, I'm able to exercise, walk long distances, go on bike rides, almost anything and everything that I wasn't able to do b efore the medication.  It has also improved my 6-minute walk test results, as well as my echocardiogram results.  Tyvaso, in conjunction with my oral medication, has improved my quality of life tremendously and I'm so grateful to have the therapy available to me."

-Reinee
Bay Area, California, USA

"In 2009 I was diagnosed with Familial Pulmonary Hypertension. At that time I was put on two different treatments. One was a long established drug Tracleer, and the other was an inhaler that had been FDA approved only months prior, Tyvaso. The doctor has switched my pill form of medication, but Tyvaso remains my main treatment. I always credit Tyvaso as saving my life. Before diagnosis I was not able to walk up a flight of stairs without getting out of breath, now I can go hiking and jogging. I can breathe easier with Tyvaso, if I am without it for a day I can feel the difference in my chest and my exercise capacity is diminished. "

- Raeana Rader, 28 years old
USA

"I was diagnosed with pulmonary hypertension after I passed out while walking into work. That
was simply too much exertion for my body. I was placed on I.V. Veletri and two oral medications. They got my symptoms under control but I had terrible side effects from the Velerti. The nausea, vomiting, diarrhea and headaches were the worst. I eventually improved so much that my doctor let me do a conversion from Veletri to Tyvaso. Tyvaso gave me my quality of life back. The only side effect I have with Tyvaso is flushing. I feel like a normal human being again. Because I am no longer on Veletri, I can take my kids swimming and no longer have to sit on the sidelines. I am able to exercise on an elliptical for an hour at a time and I have recently started taking a spin class. Tyvaso is also much cheaper than Veletri and because it is noninvasive, I do not have to worry about line infections which could be costly and detrimental to my health. I feel very blessed and grateful to have Tyvaso available to me."
- Ashley, USA