National Poetry Month!

April is National Poetry Month! Poetry is a great way to express every emotion a person is feeling or has dealt with for any type of situation. Whether it be a poem about falling in love, describing nature's beauty, or how hard it is to deal with the death of a loved one, poetry can be a therapeutic and creative way of expressing oneself.

In this blog are a few poems by some phriends who were willing to share their words about living with pulmonary hypertension. I can certainly relate to their creative expression. Can you?

The Dastardly Disease ~ by Merle R. November 2010

I used to be healthy; I used to be strong,
I used to work most all the day long.
And then it hit me without even a clue
I wasn't quite sure just what I could do.
I saw many doctors and then all those tests
I was diagnosed with IPAH and you know the rest.

Pulmonary Hypertension the dastardly disease
It’s rare with no cure and progression will be
It affects the right side of heart – the lungs big time too
Those arteries get constricted and give out on you.
It’s so complicated; makes it so hard to breathe
With having this dastardly pulmonary hypertension disease.

Shortness of breath are symptoms at first
And sometimes it feels like your heart just may burst.
That weight in your chest, fast heart beats oh my
And walking too fast may just make you cry.

Some may get dizzy, or maybe pass out
Some are so tired - what’s that all about?
Some cannot work and don’t understand why
With little exertion they falter not fly.

Some have edema, that swelling so bad
Retaining that fluid just makes one so sad
Some may have chest pain and may have blue lips
This disease is too serious and needs to be nipped.

Some may have Scleroderma, Sleep Apnea or HIV
Lupus, Raynaud’s Phenomenon or maybe COPD.
There can be some causes or no cause at all
That’s when our ph doctors need to make the right call.

We cut back on salt, smaller portions for meals,
Nutrition’s important and helps us to deal,
We deal with life changes a whole new life style
Remember it’s doable – we'll be here awhile.

Many will ask when not feeling too well
How do I cope, how do I dwell?
Do not despair and let me just say
There are treatments out now – with more on the way
With hope and with faith we have a good chance,
To fight this disease and maybe then dance.

Too many doctors; some specialist too
Do not understand what this ph disease can do
Luckily though and I praise God on high
There are ph doctors and nurses who do know the why
They treat us with wisdom and caring that shows
I thank them so much and I just hope they know.

They schedule those testings they start off real slow
An echo and blood work and others you know
A six minute walk, many PFT’s, oh gee
We scurry, we’re dazzled, we come then they see.

You may have a CAT scan, a bron-chos-co-py too
And there could be several others they may ask of you.
And then the right heart cath the gold standard of all
This proves the diagnosis is proper – they made the right call.

Some take an oral an inhaled or such
Some with IV’s and more, oh, oh so much.
Revatio, Adcirca, Letaris, Tracleer,
Ventavis, Tyvaso are a few that are here.
Then Veletri, Remodulin or Flolan may do
And some of these meds are almost brand new.

A hose in the nose; a tube in the chest
We struggle; we strive and hope for the best.
It is very doable this dastardly disease
With research abounding, there’s hope – so let’s breathe

Those researchers out there, those researching now
How can we help you, with what and the how
We’re counting on you to brighten our life
You give us more hope to end all this strife.
We’ll give you some blood or whatever you need
And hope for a cure of this dastardly disease.

The cost of these meds is abhorrently high,
We suffer, we struggle, oh my how we sigh.
Some insurance companies won’t give us a dime
The government too in their wisdom declines
Then say they will help – but they have special rules
Most are careless and thoughtless and actually cruel.

There are specialty pharmacies and pharma reps too
With guided persistence they know what to do,
They tell all those doctors those specialists out there
About the ph meds; how they need treated with care.
Some have special nurses and advocates now
Who teach the new patients the why, what and how.
They treat us so special it’s learning one on one
They do have support for us, their work’s never done.

There’s a ph community it spreads far and wide
We meet in some chat rooms and support groups with pride.
I've made many phriends and I value them well
Unfortunately though, and I do have to tell
I've lost ooh too many, that hurts thru and thru
This dastardly disease can do that to you.

With our phamily support system we’re able to cope
We share with each other, there is always hope.
Hope for a future, hope for a life
Hope we’ll endure without too much strife

Although it’s not cancer the symptoms may be
As bad, sometimes worse than that horrid disease.
PH is progressive and can cause us much pain
We must not let fear grip us, there’s still much to gain
As mentioned before and remember this now
Pulmonary Hypertension is doable – we’ll be here a while.

Let’s take a deep breath; so slow if you please
It can strengthen those lungs with this dastardly disease
In through your nose and out through your lips
Slow is the key – please remember that tip.

And last but not least, remember to smile
As smiles are contagious you see
And when you feel down; and bring on a frown
Turn that frown upside down just for me
Smiles make us happy and will help us cope
With having this dastardly pulmonary hypertension disease. :o)

PH Poem ~ by Catalina L. 2006

~To all of us who are dealing with this terrible disease, may there soon be a cure~

You came into  our lives when we least expected it.

Because of you we are weak.

Sometimes unable to speak.

You are such a threat. 

Our hearts throb and we even sigh.

But it doesn't mean we love you!

We are just trying to catch up with our breath.

We think of  you night and day.

We wish you would go away.

We're stuck with you no matter what.

If you only knew the pain you have brought

You're not innocent or pure.

And right now there is no cure.

But we will not give up hope you see.

One day a cure there will be.

Just as easy as you came into our lives.

You will always come

But will not stay.

And we all hope and pray for that day!

"Breathe" ~ by Laura G., September 2013

Pumped full of life and this is true

The only breaths I have are few

The medicine runs through my viens

The best days I have are when it rains

If it's too cold outside my lungs are tight

If it's hot they just dont feel right 

It's so scary when you lose your breath

I just wonder how many these lungs have left

I'm few of the lucky ones who can walk around

Without an airtank dragging them down

I'm still really lucky if I haven't already mentioned

But it really sucks to have pulmonary hypertension

My Life with PH + Lupus

by Stacey Gausling

I have two children named Lupus and PAH. Lupus is 14 years old and PAH is 7. Lupus tends to be on the quiet side but when she gets upset, she confuses everyone. PAH came into my world with forceful energy. She demanded so much attention that I was wiped out like never before. Her tantrums were so bad in the beginning. She requires a lot of daily care and has her special “buddy” that has to be with her at all times, ever since she turned 2. I have to carry her “buddy” in my purse or a fanny pack 24/7. She also requires a special diet and I can’t run too far without her tugging at me wanting attention.  On top of that, I am trying to be a super mom and working full time. 

Last year, Lupus got tired of all the attention that PAH was getting and decided it was her turn. She threw a major tantrum and accidentally kicked me in the kidneys. Then she got involved with a bad influence named Anemia which got so bad that it did a number on my blood pressure.  I had to take a one week vacation at La Resort Hospital to get over that one! Well, PAH was having none of that. She demanded to have a makeover for her “buddy” which required another one week stay at La Resort.

As a parent of a teen and an adolescent, I am challenged daily with these 2. The level of care is more that I would have ever imagined, but it gets easier with each year. I am looking forward to seeing them in their 20’s and hopefully they will find their own lives and leave the nest. 

May is Lupus Awareness Month!

May is Lupus Awareness Month, and today is World Lupus Day!

So what is lupus?

It is an autoimmune disease which can affect any organ system in the body. There are 4 types of lupus. SLE, or Systemic Lupus Erythematosis is the most serious form of lupus and it commonly attacks the heart, lungs, and kidneys. CLE, Cutaneous Lupus Erythematosis, is limited to the skin, causing rashes and lesions (sores). Unfortunately CLE can lead to SLE over time, and children (making up 5% of patients diagnosed) who develop lupus early in life are more likely to have serious complications. NLE, or Neonatal Lupus Erythematosis, affects the infant of a mother with lupus.  The mother’s antibodies can cause lupus symptoms in newborns which disappear after a few months. There is also DLE. Drug-induced lupus Erythematosis is a reaction to a few specific drugs by a small minority of people. Generally the symptoms of that lupus disappear after stopping the medication causing the issues.

How is Lupus related to Pulmonary Hypertension?

Well, lupus is significant cause of Secondary Pulmonary Hypertension.  Studies have shown that up to 60% of Lupus patients develop some kind of pulmonary involvement, including PAH. Related to scleroderma, lupus can also cause damage to the blood vessels of the lungs, leading to increased pressure.

Who can get lupus?

Anyone is capable of having lupus. It is not a contagious disease, so you cannot “get it” from someone. While 90% of the patients diagnosed with lupus are women, men are capable of developing the disease as well. It is most common for women to develop the disease from the ages of 15 to 45; however children may also develop lupus.  For men, diagnosis later in life is more common. African Americans, Hispanics, Asians, and Native Americans are most likely to develop the disease.

So what are the symptoms?

Since SLE can affect any organ system there are many different symptoms of lupus. The most identifying of these is the “butterfly” rash that gave lupus its name, because it looks like the “mask” of a wolf and lupus is the Latin name for wolf! Other common symptoms include extreme fatigue (we all know the effects of that!), headaches, painful and sometimes swollen joints (90% of patients experience joint or muscle pain), oral lesions or ulcers (the most common symptom with 95% of patients developing these), fever, hair loss, and sun sensitivity. As the disease progresses, more organs are likely to be involved. While up to 40% of all patients will develop renal complications, over 50% of children diagnosed with develop renal disease, and this is thought to be caused by the long duration of the disease.

How is lupus diagnosed?

Because there are so many different combinations of symptoms, and those symptoms are common in many diseases, it is often very difficult to diagnose a patient as having lupus. Like patients with PH it can take 5 or more years to diagnose each case! There are several laboratory tests that are given and analyzed along with the patient’s entire medical history in order to rule out other possibilities. As with PH, doctors are taught to look for the horse first, seeing the zebra after much testing!

Will the children of lupus patient develop lupus as well?

You might think that since lupus is genetic in nature that it would be hereditary. That is not the case. Only about 5% of children with one or more parent living with lupus will develop lupus as well. Unfortunately for those children there are no genetic tests available for early diagnosis.

  How is lupus treated?

Like PH patients, those diagnosed with lupus must see a specialist, a Rheumatologist. The involvement of other organs may require the addition of other specialists, and the coordination of all doctors on the overall care of the patient. Many patients will take many medications often for the rest of their lives. There is no cure, and no treatment is guaranteed to work. It has been found in many cases that, while PH cannot be cured or treated with other medications, controlling the lupus will also keep the PH manageable. Drugs include steroid, anti-malarial, and immune-suppression drugs. Care and management of this disease can be very expensive, especially when a patient is no longer able to work or provide for themselves.

What kind of life can those living with lupus expect?

80-90% of patients diagnosed with SLE can expect to live a relatively normal life. There are many organizations out there to help all patients cope with the challenges presented in their life, including utensils to assist opening jars! To assist the patient with medical costs, many drug companies offer financial assistance. Many communities also offer help with housing and mobility.

Where can I get more information on lupus, or offer help to patients?

The Lupus Foundation (www.lupus.org) is the lupus equivalent to PHA. My favorite patient support group online is But You Don’t Look Sick, the origin of “The Spoon Theory”. (www.butyoudontlooksick.com)

Resources:

http://health.nytimes.com/health/guides/disease/systemic-lupus-erythematosus/complications.html

http://www.lupus.org/newsite/pages/lupus-facts.html

http://www.lupus.org/newsite/index.html

http://www.lupus.org/newsite/pages/lupus-awareness-facts.html

http://www.phaonlineuniv.org/Journal/Article.cfm?ItemNumber=718

Jenny's Story: PH and Lupus, Fibromyalgia and Sjogren's syndrome

In August 2010, Jenny Klick of Winter Springs, Florida found herself out of breath – she couldn’t even walk to the bathroom. She attributed her symptoms to smoking and her limited airway disease. However, she wasn’t quite sure and went to the doctor anyways. Her doctor ruled out upper GI problems and sent her to a pulmonologist who concluded she just wasn’t taking her Advair correctly. Still out of breath, Jenny goes to a cardiologist. He performed an echocardiogram where he determined Jenny may have pulmonary hypertension. Like too many patients, she was already in respiratory failure and immediately went to the emergency room. 

In order to confirm pulmonary hypertension, the cardiologist performed a right heart catheterization. However, Jenny’s doctor could not complete the procedure because her pressure was too high. Nonetheless, Jenny finally had a diagnosis – Class IV PAH due to the lupus that never really caused her too many problems. 

Though the diagnosing cardiologist noted she would eventually pass away from the disease, he didn’t faze her. Having several other chronic illness including lupus, fibromyalgia and Sjogren’s syndrome, Jenny understood she could not be cured but knew she could continue life with the positive outlook she’s always had. Once Jenny was placed on Revatio and Tracleer, she was released from the hospital and began to feel a lot better and continued life as usual.  

A few weeks later, Jenny made an appointment with Dr. Tarver, a well-known PH specialist in Orlando. Like many patients, Tracleer was beginning to affect her liver and began taking Tyvaso instead. Jenny began feeling better and stopped thinking about her new diagnosis. As Jenny describes, living with PH became her “new normal.”

It wasn’t until Jenny came across an issue of Pathlight did she realize how devastating the disease could be. Soon after, she attended her first support group where she met other patients and caregivers. Months later, she became a support group co-leader and is now in charge of scheduling guest speakers for the group. Jenny loves her role citing “everyone in the PH world is eager to lend a hand and tell their story.” Jenny’s involvement in the PH community extends to her participation in PHA’s 10^th International PH Conference and Scientific Session last summer. There, she met more supportive individuals who directed her to PH Family, a community-operated Facebook page. She remains active with the community on Facebook, PH Central, and goes to the PHA website frequently. Jenny is also a Tyvaso Peer Mentor for United Therapeutics’ Peer Network.

Though Jenny has been fortunate throughout her journey with PH, in January she was hospitalized for a week with a serious respiratory infection. Since then, she reports feeling like “a typical PH patient” as simple task like going up stairs is now very difficult for her. Her doctor concluded her limited airway disease decreased the effectiveness of Tyvaso so her dosage was increased from 12 puffs to 15 puffs though the average dose is 9 puffs. However, her heart rate is still out of control and hopes to find a solution soon. 

It is important to mention Jenny has a strong faith in God and has kept a positive outlook throughout the last couple of years. Though she lives with other serious chronic illnesses, she admits PH has been another beast and is thankful for the support she has received from her family and friends. Her husband especially has been a great caregiver to Jenny and has worked to learn as much as he can about PH. He even called family and friends to talk to them about her new diagnosis so she wouldn’t have to. Jenny knows she is “very blessed to have support and understanding” from those who care about her. She is also thankful for PHA and wishes for a day when she can receive the same great support for her other illnesses. 

By Imani Marks, Patient & Caregiver Services Intern

Based on an interview with Jenny Klick