In honor of spreading the word about Pulmonary Hypertension, on the PHA's Blogging day, this post will be all about Pulmonary Hypertension. Some of you may have heard this information before. Some may have not. Feel free to link anyone to this post if they have been diagnosed, or have had a loved one diagnosed with Pulmonary Hypertension....
Lily - not even 2 yeares old - has Primary Pulmonary Hypertension
First off - I'm not a doctor. I'm not going to use a lot of medical words. I'd probably get half of them wrong. So I'm going to break it down as nice and simple as I can.
Q: What is pulmonary hypertension?
A: It is high pressure in your heart and lungs. Specifically, how hard/much pressure the heart has to use to squeeze blood through your pulmonary artery.
Q: So, its like high blood pressure then?
A: Uhm..in the way that a 1 dollar bill is like a 100 dollar bill. Both are printed on the same paper. One is vastly different in terms of effect it has than the other.
Hypertension can be deadly. However, it is manageable, and in some cases if you can negate the circumstances that lead to the hypertension, you can negate the hypertension itself. PULMONARY HYPERTENSION HAS NO CURE. No amount of 'eating right' or anything like that is going to make it go away. A lung transplant might...a heart lung transplant might...but do you really consider something that drastic to be a cure?
Side note: I solidly vow to shove my boot up the next person's rear who makes a comment about how "oh, my (so-in-so) has hypertension" or "Maybe she just needs to exercise more" etc. I have boots with five-inch heels. Don't be stupid. THINK before you speak.
Q: What causes Pulmonary Hypertension?
A: Depends. If Pulmonary Hypertension (Herein called PH) is related to a heart defect, a medication/pill you had once taken such as Phen ..something. I can't remember the name of it. That diet drug... It is called "secondary pulmonary hypertension". The prognosis for Secondary is generally a bit better. GENERALLY. Not always. Now, if the doctors can't figure out why you have PH, its either called Idiopathic (unknown cause) PH, or Primary PH.
Q: Why haven't I heard of PH before?
A: Because its a rare disease. If you don't believe me, look up NORD and Pulmonary Hypertension.
Q: How rare is it?
A: The last estimates I read said 2 in every MILLION people. However, it could be more prevalent than that. There is now some research that indicates a good portion of SIDS deaths are caused by PH.
Q: Babies can have PH?
A: ALL babies have PH in the womb. Then, when they are born, those pressures drop dramatically. However, in some cases, they do not. Unfortunately there is NO standardized newborn testing which would indicate PH. Symptoms generally present as labored breathing, blueish skin, etc. However, the baby could present perfectly fine in the hospital, only to have it develop rapidly a few days later. Rapid on-set. Now, I'm going to say something. I'm going to say it in Bold, Italic, and Underlined. So, just be aware. Because, duh, this is freagin' important.
IF YOU HAVE HAD A BABY, AND YOUR GUT IS TELLING YOU SOMETHING IS WRONG....BUT THE PEDIATRICIAN IS SAYING EVERYTHING IS FINE...DON'T LISTEN TO THE PEDIATRICIAN. PUSH TO HAVE AN ECHOCARDIOGRAM DONE. THIS IS THE ONLY SURE-FIRE WAY OF GETTING A GOOD ESTIMATE OF THE PRESSURES IN THE HEART.
Get it? Got it? Good! Most run-of-the-mill pediatricians/adult doctors have never even heard of Pulmonary Hypertension. So don't fool yourself by thinking 'oh, my doctor would tell me if something was wrong with rugrat/me.' Don't be stupid. Be empowered.
Q: What's the life expectancy for someone with PH?
A: This depends. It depends on how early the person was diagnosed. It depends on how well they respond to medications. It also depends on how well they obey basic tenenaments of self-care like "Take. It. Easy!" In the 90s, someone diagnosed with PH had an expected life span of three years if left untreated. For children? 10 months. Now, with treatments, doctors have managed to extend the expected life-span of someone with PH to 10-12 years. Obviously, there are people out there who have lived longer, and people who have died sooner. It depends on how severe your PH is, and the other things I just mentioned.
Side note: So, yes, I am having to prepare myself for the fact that if Lily does not respond well to medications, my daughter is going to die, and die soon. Even if she does respond well, the prognosis is poor. Will she even live to see 21? After about 10 years old, when a child starts going through puberty, their reactions to vasodilators drops dramatically. So does the survival rate. So, even if Lily does well now, who knows if she'll make it past puberty??
Q: You say in your title "It still feels like a death sentence"??
A: When speaking with an adult who has been diagnosed with PH, I asked if her if she thought that the diagnosis feels like a death sentence, and if she still does. Her answer? "Yes". I, too, feel like its a death sentence. Unless there's some cure that miraculously arrives in a few years, this situation is NOT going to resolve in rainbows and kisses for everyone. My daughter has, effectively, been given an expiration date that will come a lot quicker than it should for anyone.
Q: What treatments are there?
A: Flolan, Remodulin, Sildenafil, Tracleer, Lasix, Letairis (sp?), Oxygen. Those are the ones I know about.
Let me break that down a bit more: Sildenafil (also known as Viagra) and Lasix are two common, low-risk treatments. Lily has been on those for longer than I care to remember. The Lasix gets the extra fluid out of her body, making it easier on her heart and lungs. The Sildenafil is a 'vaso-dilator' (vessel-expander). You know how a man takes that little blue pill, and gets a happy? Its because it opens up the blood-vessels more, and allows his 'little head' to get more blood-flow/oxygen, etc. Well, same thing with Lily, except she doesn't get a visible salute going when it starts working because 1. she doesn't have boy parts and 2. its working on her heart.
Tracleer - the only medication for PH that (as far as I know) comes in Pill form. Tracleer has some SERIOUS side-effect possibilities. Like, you know, if you get pregnant, your baby might have no head...or two heads. That type of thing. There is also a serious risk of liver damage. To the point you have to have a blood-test for liver functioning every month before you can continue to take the medication. This is what Lily is going to be on. Tracleer is 5,000 dollars a month.
Oxygen - I shouldn't HAVE to explain this...but just in case. Oxygen is a medicine. Yes. A medicine. Putting Lily on oxygen at night has ensured she gets a healthier sleep, has increased her energy levels, and helped her heart and lungs a little bit. Lily is lucky to be on it only at night. Some patients with PH have to be on it constantly.
Flolan, Remodulin, and Letairis are out of my realm right now. I just know that one of them is an inhaler. One is administered via a catheter leading straight into the heart. One is administered subcutaneously. I think Flolan is the one straight to the heart. I do know with that medication that you are required to be hooked up to a continuous infusion to help keeps things level. If for some reason your pump would stop working? You have 5-7 MINUTES to fix it, and get the medicine going again. If not, well...lets just say you should always keep an updated will around. The consequences can be that serious. All of the medications I just talked about in this paragraph also have serious side-effect possibilities.
Ok, so, now that I've written everything I can think to write without your eyes glazing over...
This is what I'm dealing with. So, pardon me if, over the course of the next several months, I get a little grouchy and depressed.
Also, let me list a site for you.... http://www.phassociation.org/
Wonderful website.
If you have any questions, you can ask me and I'll try to answer them.
Regular bloggy-update on Lily coming later.
