just not looking the part

That's one of the hardest things I find about being ill with IPAH. At least for now, that's the story: I do not look ill, no matter how I might be feeling. I look perfectly fine. In fact, I look better than I have for quite a while, because I have lost a ton of weight - the woman exaggerates, of course - and am mighty pleased with myself. I'm standing right outside the door of 'normal weight for height and age', right now.

 *jumping for joy*

But, because I don't look like I'm ill, I worry that people won't believe that I am ill. And who are these people, you ask. I don't know. Just people. People I don't know, or care about, or whatever!!! Hahahah. I know it's absurd, but that's how I feel, and sometimes, I feel uneasy about it.

Like when I have to turn down an invitation to visit.
Or, an invitation to dinner.

Or, someone who says they haven't seen me for a long time, and can they drop by tomorrow, next week, whenever?

I am feeling all right now. That's all I know. Tomorrow, I may be feeling horrid. Next week, I may be having difficulty breathing - and trying to stay calm, and not bring on a panic attack by worrying that my heart is going to stop any minute now - because it sure feels like it's planning to!

Whenever, I might be suffering from horrid bloating, and be sitting up so straight, and rubbing my rib-area, and trying to not say out loud that I wish I were dead, because the pain is so bad.

But, how do I explain that to whomever's asking?

How do I say what's-what to people who are acquaintances, and don't really understand what's up with me.

I mean, I have explained this disease to people, and they still do not understand what it's all about. 

'Is it your heart, or your lungs?' 

'You can't breathe? Then, why are you going to IJN*??'

'You/she looks fine!!'

So, my always reading-between-the-lines mind wonders if they don't really believe me.

And because I am an idiot, this upsets me. It upsets me because I wonder if people think me a fraud. I wonder if they think I'm like Emma's valetudinarian** father, Mr Woodhouse!  

I think I am fast becoming a Mr Woodhouse, as it is! I worry about getting the flu, or a cough. Because if I get it, it will take me a long time to get over it. And, I know this is true: Because I have been ill before I was diagnosed, and I did take months to shake off something like a cold, or a cough.

All this valetudinarian-ism (nope, no such word!) is helped along by my dear husband, of course. Stay away from that side, there's someone with a hacking cough over there! Hahahah. Such fun, I tell you.

But, it all feels so selfish. And I've always said that I do not want to become my disease. It is just what I have, but I want my life to go on normally.

Easier said than done, though.

I tire easily, and often, I need to rest. Remember, it's my superpower, k!

And, even when we have guests, after a bit, I need to excuse myself and go off to my bed - yup, you guessed it: To rest.

Resting is a big part of my day. Without my multiple rest-times, sprinkled generously throughout my day, I'd be out like a light by about 7 in the evening!!! The medications help me get the oxygen I need, and ease the flow of blood from my heart to my lungs. But, any kind of activity is a strain, and when the strain gets too much, my muscles actually hurt. I realise now that this hurt is my body telling me that I've not got enough oxygen in my muscles, and that I need to rest. So, I feel the hurt in my shoulders, and often, in either my thighs or calves. All cries from my body, saying: Rest time!

So, these are little things I've slowly learned about me and IPAH, and I'm slowly learning to live my life around it all. 

But, what I find hardest to do, is to explain this to others. Because, frankly, it seems so very lame!!! Hahahaha. "I'm tired. I need to lie down." Another one of Jane Austen's characters getting the vapours, eh?

Talk about life turning around and biting me in the bum!

So there, I've written about it, and I hope this will get it out of my system.

Note to self: It is all right to be tired, and to say you need to be excused so that you can go lie down. It's all right if people think you're a slug. You don't care what those people think anyway!

Note to self

* IJN: Institute Jantung Negara, our National Heart Institute

** valetudinarian   noun (I had to look this up! 😉)

1. a person who is unduly anxious about their health.

   • 
     

     a person of a weak or sickly constitution; 

     especially: one whose chief concern is his or her ill health.

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13. .

singing away the blues

The one constant in my life has been singing. From a little tot (I'm told), I have loved to sing and thought I was the bees knees at it, too! Hahaha.

One of the first songs I learnt to sing was 'The little Dutch girl', and it was my dad who taught me the words and the melody. I think I'd have been about 3 or 4 at the time. He'd sing along with me, I remember, and I'd say to him: Please don't! I don't know if that's a real memory, or if it's been planted by my parents, and aunts and uncles, and cousins!!! ;)

One of the first ways IPAH affected my life (besides not being able to breathe!), was the fact that my voice changed. From almost croaking, in a hoarse whisper, to not being able to hold a note when I tried to sing. I'd initially think it was a sore throat; then, I thought it was the effect of my asthma inhalers; and finally, I'd learn that it was a direct result of the pressure of my pulmonary artery, under high pressure, pressing on my vocal chords.

But, as with all things with this disease, I have good days, and bad days. And this past week, even though I've had bad days emotionally, my voice has returned. Inside my own head, it almost sounds like the voice I used to know. And, because the medications are doing their work well, this week, I can hold a note, and not wobble. Not too much, anyway.

You be the judge.

This is me, singing Stevie Wonder's 'Lately'.

And in this one, I am channeling the Bee Gees, from 1971, with 'How can you mend a broken heart'. HOW appropriate, aiteilyiew!

Yesterday was a good day.

And, I have learnt to not expect perfection. Crazy things happen during recordings, but I just keep on singing. If I do a better version on another day, I'll replace these ones I've done. But, I do know that years from now, I'll look back at this and be grateful for the good days that I've had.

I have other songs on my You Tube page.

How lucky to be alive in an age where everyone can be a 'recording artiste', eh?! Hahahaha. Where you can record yourself, and keep it online, and listen to it whenever you choose. And all for free. Yay! Good times.

and the living is easy

I've been having a good week. Some bumps along the way, but overall, I'd say I'm pretty happy with how the week has gone.

It's been raining all week, and it looks like there's more rain to come in the week ahead.

All that rain makes it cool. And all that rain cleans the air, and that's good for my lungs. Hot-and-dry ain't so hot for these babies. I become raspy, and it feels scratchy in my chest. But not now.

And I can sing again. My voice has made a return, and I've enjoyed singing, on many days this week  - either singing in harmony to John Denver, or the Bee Gees, or taking my turn on some karaoke! Always, when I can sing - read: no hoarse whisper - I am a happy girl!

the scales don't lie

I hope they don't, anyway! Hahahaha.

Weighed myself, and I now weigh 58.9 kg. That's 129.6 lbs. That's 14.5 kg/32.6 lbs down from the 73.7 kg/162.1 lbs I weighed on 28 July 2016.

Finally, something to be happy about!

I've got a little more to lose, but I know it will happen if I stay focused on eating healthy; eating only when I'm hungry; and eating only the things I enjoy, that do not not lead to weight gain. 

I cannot say that this has been a difficult year for me, where eating is concerned. I have eaten well, enjoyed my food, and never once felt like I was being deprived and give me that cake already!!! There is sometimes that temptation to eat delicious rubbish, but the thought of fighting bloating, or putting on weight, has kept me relatively good. When I have strayed, it's been with yummy chocolate, some cake or biscuits, or something really scrumptious.

I think that it worked this time - this needing to lose weight, and actually doing it - because my brain believed that I really needed to do it. That I didn't have any other option. Less weight means less work for my poor heart. It's already working overtime, so it made sense to bring down my weight, and give it an easier time. Having wrapped my mind around this concept has helped me stay on track.

And here I am! Happinessss!

Sometimes 😋

management, not cure

Yesterday, I learnt what this means: management, not cure.

It is something that I heard on day 1, after being diagnosed with IPAH. I understood that there was no magic pill to take, no operation that could be done, no nothing that could make it go away.

There is medication - more now, than say, even ten years ago - and it helps keep all the bits-and-bobs  working; helps keep them from deteriorating further, at least. But, they don't repair them; don't make them as good-as-new. But yes, they have surely improved my quality of life: I no longer feel faint after walking up a slope, or a few steps. I have not had a day of breathlessness since being on them.

But yesterday, I realised that I am here, and comfortable, because my medicines work.

If I stop taking those meds, however, I will stop being well.

The penny has finally dropped.

And, it has taken me close to six months to realise this.

Yesterday, I finally understood what it means. And, yes, it is upsetting. I've not been feeling very well for the past few days, and that always makes me down. Tears so ready to fall, behind my eyes.

Yes, yesterday was the day that I understood what it really means to be ill with IPAH. Yesterday, I reaslised that I am now probably the best that I can be, and that eventually, I will not be so good. Bummer.

Yesterday.

Talk about someone being a bloody slow learner, eh?! Hahahaha.

Yes, yesterday, the coin dropped. I have to make the most of my life, however long I have. I won't say I am sad. Maybe a little. But, I am scared - about what may lie ahead, and that maybe it will be less-than-wonderful! Hahahaha.

Yes, yesterday. I still can't decide if it was a good day, or not.

lazy days

Most of my days are lazy days. I sit on my bum and go online and read the news, check on my mail, and then visit Facebook. Sometimes, I'll also visit Instagram, or Pinterest. Not rivetingly exciting, but that's usually how I spend my mornings.

Sometimes, I read. Usually, on my Kindle - because I can set the font-size a little larger for days when I feel my eyes need help. If I read a book, I need to sit by a window, in goooooooood light - or my fixed-in lenses don't work so well.

Once in a while, I'll get off my arse and actually do some art. I have done a couple of mandalas, using a bit of my acrylic paint. But, I've promised to not work with acrylic paints anymore because they have formaldehyde as a preservative, and my lungs don't need that kinda excitement, thankyouverymuch!

(I've packed up all my acrylic paints and stuff to give to a friend!)

I also doodle, sometimes. Or work on mandalas.

For some reason, my watercolour muse seems to be away on holiday. Probably on distant Mars or Neptune.

After an hour or two of doing anything, I usually have to have a bit of a lie-down.

I do a lot of resting!

When I was young, resting was for lazy arses. My mum would get so mad with us for just lying around doing nothing (reading!). We needed to be up and about: cleaning stuff, mopping floors, shimming up the walls dusting corners. Not that we did much of that - Mum kept our home spotless all by herself! Our main 'work' was keeping it that way, or else . . . .

But, my brain has been conditioned, and so sitting on one's arse is a no-no. So, I've yet to be one-hundred percent comfortable to just be sitting around, or lying around, doing nothing.

Most of what needs to get done at home is gotten done by Chuan. And no one is more UNLIKE my mother than Chuan is. He mops the house smiling. When he's cooking, he does a little Ringo Starr with his knife on the board - like when he's chopping garlic. And Chinese food uses a lot of garlic!

He is happy!

But, because my mother was never happy slogging to the bone, I am often in doubt about Chuan being really happy. Then, I get very uncomfortable with my resting all the time.

I feel like a fraud.

I feel like I am pretending.

Maybe I should just get off my arse and do something. Sweep. Mop. Make like Michelangelo: Paint the ceiling. Something!

I do do some stuff: I do the laundry, every few days. And fold it all, and put it all away. I dust the bookshelves, once in a while. I sweep the house, sometimes. I loaded the dishwasher - when it used to work.

Take the dogs out for their toilette, every few hours or so. When I forget, Jolie will give me a loud-dachshund shout, and I'll be reminded.

But, I am tired many times during the day. After a meal, is one of the times I need a rest. Especially, if I've eaten a bit too much. Then, I am even more tired!

After a shower, is another time. It only takes about 10 minutes or so. 15, if it's my wash-my-hair day. But even so, after that, I need to rest! Showers are quite exhausting. They are meant to be lukewarm, because hot water makes one's blood pressure drop, and that can cause one to pengsan. Faint. We don't want that.

When I told my daughter that I had to rest after my shower, she said she didn't know if I was being serious, or joking! Hahahah. I know, right?! What on earth do I get up to in a shower to get so tired after it?! If I knew, I'd tell you!

By 8 at night, I am quite, quite tired. So, I have decided that I will no longer accept dinner invitations. Because by 8, my body has had enough of walking around, and sitting, and resting, and other exhausting stuff! It is time to wind down, and get ready for a nice, long rest. Usually, by about 10, I'm horizontal and ready for the eyelids to drop into place.

Little by little, I am learning about this disease and I'm also learning about me. Things are different, and I am wrapping my mind around how I need to change and adapt to them. I have never been a very get-up-and-go kinda person, but this is taking me to a whole new low! Hahahahah.

Note to self: Get used to being tired. But, more importantly, get used to not feeling guilty about needing to rest!

Make this my new credo:

That's me: Horizontal, a lot of the time!

payback time

Yesterday, we went out for a little walkabout at a nearby mall. Not a whole-day thingy, but only for about four hours or so. There was some walking involved - but slow stuff. No rushing or anything.

I needed to buy some curtains for our living room, and some other stuff. Then, we had a bit of lunch, walked around the supermarket for a bit, and returned home by about 2.30 in the afternoon.

Very tame.

I felt a bit tired as we were walking back to the car, but that's about normal.

But, last night, it happened again:

My bones ached, and I felt like I was going to be ill. Maybe get a fever, or a cold. Or, both. I just felt awful!

But, I'm wiser now. Because this has happened before. When I've been out and about - I'll return home, feeling fine. But, by night, this feeling of being all wrong will set in.

So, when it happened again last night, I was prepared for it. I knew I would be fine in the morning, and I was. I feel just fine today! But, it was a totally different story yesterday.

And even though I was so tired, I couldn't fall asleep. I tried.

After a while, I gave up and watched a movie on You Tube until about 3.30 in the morning. I only stopped because my tablet ran out of juice!!



I guess I must've eventually dozed off, but despite this little-to-no-sleep, I woke up feeling fine.

So, this has happened a few times, and because it has, I no longer feel worried that I am going to fall ill. Falling ill with a cold, or the flu, is not good. Especially if it involves antibiotics - which can adversely affect me, because of the medications I am already on. I need to be mindful about what I'm prescribed, and don't take anything that doesn't jive with my need-to-take meds.

This post is to help me remember that this is what happens after I go out and enjoy myself. Payback time! Hahahah.

feeling a little off

For the past few days, my 'chest' - I don't know how else to describe this - has felt a bit scratchy. It's like there's a little sandpaper in there, and the air I breathe has a little job flowing over it, and getting into my lungs.

I have something to take for that: Spiriva: It is an inhaler that I take once daily, and it works by relaxing the airways and keeping them open. It works for 24 hours, and it eases breathing.

As always, there are side effects. And they are a bitch! Hahahaha. These are the most common:

• sore throat
• cough
• dry mouth
• sinus infection

I now have a nice sore throat going, and my already sad voice is a little hoarser, and it is a little harder to get the sound out.

And, I am tired. Even more tired than I usually feel. And when I sleep, I am out like a light, and don't wake till the next morning. And I could gladly sleep on!

I must be patient and let the new medicine work. It will take time, but this will pass. And in the meantime, let's get out that tablet, and watch another episode of Inspector Alleyn Mysteries on You Tube.

this and that

We had a good visit with the eye doctor, on Wednesday. My lenses are working well (after my cataract operation, last year, I had new lenses put in, in both eyes.) and my vision is good. I don't need glasses for anything! Chuan's eyes are good too, so all is well.

Mentioned my headaches to my doctor, and told him that I had been worried that reading sans reading glasses was giving me a headache. So, I had a pair made.

But, my visit to the optometrist - part of my eye check-up - just told me I don't need them - unless the print is very, very small! For normal reading, I am good-to-go.

So, tell me about these headaches, he says. And I tell him. And I also tell him that they're probably from the Sildenafil I take. Actually, I know it is: three times a day, one half-tablet goes into me. And three times a day, I get a headache afterwards. Sometimes, it's barely there; sometimes, especially after the night dose - around 8.30pm - it can be quite bad. If I sit tight, and try to think of something else, it recedes after about an hour or so. It's not debilitating, so it's something I've gotten used to.

Ahhh, sildenafil, he says. In some people it constricts the blood vessels to the optic nerve. Some people have gone blind.

Nice to know, doctor-man.

Well, I haven't gone blind. What a relief.

20/20 vision: Reading that line at 20 feet

But, I've thought it through, and this is what I thunked:

If I'd been told that that was a probable side effect of the drug, would I have still gone ahead and taken it?

Hmmm . . . .

I would have.

For PH, the drugs out there that work are limited. And sildenafil is one of them that does. Not everyone can it, I know. I've read of people who've had adverse reactions to it, and had to be taken off it.

So, my doctor would've tested it on me, during the cardiac catherisation, and he'd have seen that it actually did relax my blood vessels to my lungs, and that's why he's prescribed it for me. And it's doing its work helping my blood vessels and my lungs.

And if it meant that I may have gone blind - or that I may - I'd have still have agreed to take it.

The choice is quite simple really: It's either I take the drug and breathe. Or, I don't take the drug and my heart enlarges even more, and my lungs wither and die.

While it was a carpet-pulled-from-under-me moment, I've had time to think about it, and I'm good!

Doctors seldom tell you anything. Even when you ask about side-effects, they often tell you the wrong thing. I'll grant you that it is the pharmacist's role to tell me about the medicines I take. And they do, sort of. But, they can't tell you everything. And, most people do not want to know anyway, I feel.

At the start, Chuan read up on all the medicines I have been put on. I didn't want to read because I was afraid I'd actually imagine all the side effects and make myself nuts!!! I did eventually go look at some of the drugs, and discovered how they work, and their side effects, but not very much actually stuck in my brain. A touch of information overload, for sure.

However, when I started this blog, I had to talk about the meds, and that's when I really did look at them all: why they are prescribed, how they work, and what their side effects are. And, it has been a good experience for me.

I try.

FAQs about PH

Here are some insights into PH, that I found on a PH site, from India:

Idiopathic Pulmonary Arterial Hypertension is considered a very rare disease, and its symptoms are not unique. They can have a lot in common with other respiratory diseases. 

Symptoms can include:

• breathlessness with everyday activities (especially climbing stairs or going uphill)
• tiredness
• discomfort in the chest
• palpitating/ throbbing heart
• swelling of the abdomen, in the legs and around ankles
• dizzyness
• fainting spells
• bluish lips or skin

(I've experienced all, except for the last one.)

The FAQs


Outwardly, I look healthy. People don’t believe I am ill.

One of the first and gravest issues that a patient might face, it has no easy answers.

While one may outwardly look healthy, there is no overlooking the fact that PH is a serious ailment and it helps to have alert and helpful people around who can call out for medical aid, if need be. If privacy is not an issue, it is suggested that one does let trusted friends/colleagues/aides know. In addition, helping hands like the housekeeper, the lift and the car park attendants could be instructed to push open heavy doors or help with weighty bags on account of a ‘heart condition’.

What lifestyle changes should one effect?

Abstain/ keep off:

• polluted air
• smoke (including cigarette), Dust & Talc
• carrying weight
• speaking loudly (seriously?! But why?)
• climbing (including staircases)
• high altitudes beyond 2500m
• hot tub/sauna/long hot baths/showers (can lower blood pressure suddenly leading to fainting/ sudden collapse)

Limit:

• processed foods
• water intake (less than 1.5 Litres/day)

My PH has been given a “Class”. What is it?

Once diagnosed with pulmonary hypertension, the doctor may classify the disease using World Health Organisation guidelines:

• Class I: When symptoms (fatigue, shortness of breath or chest pain) do not exist at all / No limitation of physical activity
• Class II: When symptoms do not exist at rest, but come about with normal activity / Slight limitation of physical activity
• Class III: When comfortable at rest, but have symptoms with less than normal activity / Marked limitation of physical activity
• Class IV: When symptoms exist even at rest / Inability to carry out any physical activity without symptoms / Impairment of the heart

(I was told by my doctor that I am in Class II)

Does one need to take precautions as a PH/PAH patient?

Precautions that are desirable:

• having supplemental oxygen available during air travel
• careful and medically advised use of antibiotics for respiratory tract infections
• getting vaccinated for pneumococcal pneumonia and flu (pneumonia can be a  serious complication with PH/PAH patients)
• prevention of infections (like endocarditis) before undertaking dental procedures

(I need to find out more about those last two bullets.)

a timeline: the diagnosis

It started with a cough that wouldn't go away. And, a voice that was so hoarse, it sounded strange in my own ears. And then, there was the high blood pressure - though, at the time, I didn't know it was part of the mix.

I went to my local GP, and asked for help. My lungs seemed clear, he said, but he decided to put me on a 'strong' antibiotic, and asked me to up my asthma medication (the inhaler), to twice a day. I wasn't happy, but I agreed. I'm never happy when I'm put on antibiotics, but I felt I had no choice, here.

So: Antibiotics, inhaler, hbp medication. Draw an unhappy face for me.

But the antibiotics didn't work, like it usually does. And with the echo of my daughter's see-a-specialist-ma in my head, that's just what we decided to do.

Dr Usha came to mind. She is the only doctor I know who treats asthma patients. And I only know that because her clinic is next to her husband's - who is my orthopedic doctor. Random, much!! But, Dr Usha listened to my lungs and realised that they weren't quite right. 'Crackling', that's what she said she heard. I also had reduced effort tolerance: When I walked, or climbed a slope or stairs, I had trouble breathing. And, I remember describing it this way: "When I struggle to breathe,  I feel that I am getting no oxygen into my lungs". (My description is accurate. Later, when they do a test of the oxygen level in my arterial blood, it will be shown to be lower than normal. Much lower.) So because of these sypmtoms, along with the edema in my ankles, she suggested I come in for some tests. Reason for admission: heart failure.

A heart specialist - Dr Teoh - was brought in because it was also about my heart.

I was admitted to hospital, and over two days, the tests happened. I didn't know it at the time, but these were 'elimination' tests - because that is how pulmonary hypertension is diagnosed. It started with a guess - so clever of Dr Usha - and they needed to see if that guess was right. And if it was, to determine what type of pulmonary hypertension I had.

There are many kinds of PH, and mine is the one with no obvious cause. It remains a diagnosis of exclusion,

Google says: IPAH - idiopathic pulmonary arterial hypertension is a rare and progressive disease that affects the heart and lungs. From what I've read, diagnosis is a bitch. Many of the cases I've read about took years to be diagnosed. They were treated for asthma, or told to go home and start exercising, and to lose their excess weight. And how many of us don't carry around excess weight? And how many of us don't know we're lazy arses and need to exercise more?

I was admitted to hospital in early November 2016. By Christmas, I'd been told that I probably had IPAH. That's two months. That's quite brilliant, actually.

To confirm diagnosis, I needed to see a specialist in IPAH. And there is only one in Malaysia: Dato' Dr David Chew, in IJN (the National Heart Institute). It was time to sit and wait - another month or so - to wangle an appointment to see him. January.

In the meantime, I was put on a host of pills and puffs: HBP and asthma medication. The result, my blood pressure dropped drastically, but my breathing improved.

Low blood pressure is another bitch. You need to be always cognizant that you have it, and when you feel woozy (yes, that's a technical term!), you sit or lie down. And that is just what I feel I did for most of those two months, while I waited for my IJN appointment.

So, more tests awaited at IJN, and I see the specialist, and he says that it's probably IPAH - but he needs to perform a heart catherisation to conform it. That's more-or-less like an angiogram, but it would measure the pressure in my heart, lungs, and my pulmonary artery.

Why they need to do this - from an article I found:

Pulmonary hypertension is a lung disorder in which blood pressure in the pulmonary artery rises above normal levels. The pulmonary artery is the blood vessel carrying oxygen-poor blood from the heart to the lungs. Pulmonary hypertension is present when the mean pulmonary artery pressure is greater than 25mmHg at rest or 30mmHg with exercise. This abnormally high pressure (pulmonary hypertension) is associated with changes in the small blood vessels in the lungs, resulting in an increased resistance to blood flowing through the vessels. This increased resistance, in turn, places a strain on the heart, as it has to work against a higher resistance to pump blood to the lungs.

(from here)

And, while they're fiddling around in there, in my heart and lungs, they'll 'test' for the right medication that works to bring down the pressure.

Another hospital stay - five days, this time - and my diagnosis is confirmed. IPAH.

I am put on the correct medication, and here I am today. I've seen him again, since, and I am doing well. Except for my cholesterol, so I need to see him again - in September (blood test) - to see if that reading has gone down. He's worried about blockages, which I guess I do not need to add to the list of things that are already wrong with me.

From start to finish, it took four months. Bloody brilliant! And this is in rural Malaysia!! Hahahah. I am so blessed, and I know it 💖