That Dastardly Disease
Pulmonary Hypertension
A poem by Merle Reeseman ©
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 a while.
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 make 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. )
A little bit about Merle: As a PHA Helpline/Support Line volunteer, a PH Mentor, an advocate, a chat room moderator and leader of three PHA support groups (Mercer Area, PA; Cleveland Area, OH; and Pittsburgh, PA) Merle has exhibited unyielding dedication to the PH community. In support of the 2010 Path to a Cure Mt. Kilimanjaro climb, Merle helped organize a Unity Walk with her Pittsburgh, PA Support Group earlier this year. In February 2010, she helped raise awareness of pulmonary hypertension and heart transplantation at and in a ballet at the Byham Theater in Pittsburgh. This past September, the Cleveland Area Group had their 4th Annual Walk and Roll to raise funds for research and awareness for Pulmonary Hypertension. Merle is a tireless advocate and lobbyist for the PH community, and has reached out to government leaders from a local to a national level and she doesn’t care which state it involves. If a pher needs help, she is there for them. This past May Merle was asked to speak at a luncheon group the day before the Ohio primary. With that presentation to the UAW, she was able to mail over 200 signatures to each Ohio Senators about S 2803 and also letters to 5 Congresspeople about HR 1030. She is passionate about being a face for the PH community, and has appeared on television and radio in the Pittsburgh and Youngstown-area on many occasions, as well as featured in numerous Pittsburgh, Sharon, Cleveland and Akron-area newspapers. Her boundless energy, enthusiasm and caring spirit make her a true asset to the PH community.
With this passion and enthusiasm Merle was awarded PHA’s 2010 Outstanding PH Citizen which was presented at the 9th International Pulmonary Hypertension Conference and Scientific Sessions: “Riding the Wave” June 25th – 27th 2010 in Garden Grove, CA. Although she was unable to accept the award in person, arrangements were made and it was re-presented by a member of the Board of Directors and now sits proudly on her living room mantle.
How to choose a pulmonary HTN doctor
Now more doctors and patients are aware the disease exists and many more patients are found who are suspected of having PAH and major medical centers no longer have the capacity to screen and treat all of these newly suspected PAH patients.
One common problem is that over 66% of patients suspected of having PAH really have other conditions such as left heart disease (particularly a condition called Left Ventricular Diastolic Dysfunction) that can mimic PAH but that are actually worsened if treated with PAH medications.
Doctors who finished their training before about 2000 will have had little or no useful training in diagnosing or treating PAH and to keep up must have obtained most if not all of their training and experience in PAH diagnosis and treatment after completing their formal residency and fellowship programs.
PAH is a hot area of research and in the last 10 years or so we’ve gone from a single FDA approved therapy to now having 10 approved therapies with many more in various stages of development. Unfortunately, most pulmonary and cardiology doctors have been far too busy to develop the knowledge and skills required to properly screen and treat patients with this still relatively rare disease.
What is a patient to do? There are some sources that can help. One is the Pulmonary Hypertension Association
http://phaassociation.org
They have lots of information for patients, caregivers, and physicians about PAH and they also have a searchable doctor directory where you can find a doctor who has an interest in and experience in diagnosing and treating PAH. Many doctors also post information about their practices so you can get a feel for the volume and complexity of patients they treat.
Find a Doctor who treats PAH
If you find a doctor in your area you’d like to consider seeing you will want to ask a few questions to help evaluate their capabilities:
1. How long have you been involved in treating PAH patents?
experience is good
2. How many patients do you have on PAH specific therapy?
doctors treating fewer than 5 or 10 patients are probably dabblers and not really committed to this specialty area
3. How many patients have you treated with PAH specific oral agents, inhaled agents, and parenteral agents?
you want a doctor who can use any or at least most of the available agents when needed
4. Do all of your patients on therapy have to have a Right heart catheterization before starting treatment?
if they answer no then you need to keep looking as a Right heart cath is standard of care for PAH diagnosis
5. Do you participate in clinical trials?
if not then you won’t have the option of using new agents for PAH before they are FDA approved
6. How many doctors will I be seeing?
If the group is too large you may have trouble getting to know or getting known by anyone and spreading the patients around too far within a group may limit how much experience any one doctor in the group can get in treating the disease
These are a good start toward evaluating who should evaluate you for PAH or other conditions that can be easily confused with or accompany PAH. After all of the above you have to meet and establish a comfortable working relationship with your treating physician.