Tuesday, May 14, 2013

Pulmonary Hypertension?!?!

Today, I'm angry. I want to scream. I want to shout and hit out at whoever dealt us this hand. This isn't FAIR. This isn't right. This isn't happening!

Why??? I just can't wrap my head around it. Every time Monkeyman complains about his heart beating too hard, or too fast, I just want to cry and run away. I can't face this. I honestly can't. I don't have the strength for this again. It hurts, and I can't take that anymore.

When my firstborn was 5.5 years old, we played outside in the garden. He was chasing me, but I heard his footsteps slowed. So I turned around to see why. He had this really funny look on his face. Not funny haha. He was smiling, a surprised smile. But there was so much confusion on his face. As if he saw/felt something he just couldn't understand. I asked him twice "Mr N, what's wrong" and he would answer me "I'm tired." So I walked over to him while he tried to walk to me. But he was disorientated and swerved to the right. I caught up with him and grabbed his arms, by which stage his legs buckled. He lied in my arms with the strange smile on his face. His eyes looked fine. He didn't loose consciousness. Next moment, he sat up, his face normal, this time with a real smile on it. He told me "come mommy, let's play!". You could've ticked me over with a feather. I'm sure I had almost the same look of confusion on my face!! So I stood up, frowned and told him that I think he should rather go and sit down for a moment. He couldn't understand why, but obliged me. I made him comfortable and kept my eye on him for a while. Then the shock and confusion hit me and I teared up. I excused myself before he (or Boeboe) could notice, and at that moment my friend phoned. She heard in my voice that something was wrong, and after explaining, she urged me to phone the pead, even though he seemed fine at that moment.

By the next morning, I was sure nothing was wrong. That I was just being melodramatic and got a fright for nothing. He must've been tired, maybe a bit dizzy. That's all.

Pead felt different. She scared me. She assured me that what I'm describing, wasn't normal and that there's probably one of 3 causes. Epilepsy, a bloodclotting problem or a vein-problem. My eyes stretched in alarm on hearing Epilepsy, and she told me that I must hope it's that, out of the 3. That caused me for the first time, to realise we're not dealing with low bloodpressure or something innocent here.

What followed, was days and weeks of tests, waiting for results, waiting for dr appointments, etc. The absolute worst was the moment the technician's shoulders slumped during the EEG and I just knew it was epilepsy. And the 2nd worse was the 3 days after the EEG, before we could do the MRI to rule out a brain tumor. To just even imagine for a moment that your 5-year old might have braincancer. It was too much. My husband was unable to work half the time, we didn't sleep, we barely ate. We didn't talk.

His MRI was clear! Then we had to wait to see the (extremely busy) pediatric neurologist to get confirmation on what type of epilepsy. Then settled him on medication, dealing with the inevitable side effects. Noticing how your son changed from who he was to his new normal. The heartache to say goodbye to the boy he was forever, and to accept this new version of him. It was a hard, hard few months. With time, we accepted the diagnosis, the worry got a bit less, the heartache was soothed.

Then, 2 years later, my 5-year old daughter bumped into me on Christmas Eve and her grandpa noticed it was because of a foot deformity. Well, the rest as you know, is history. Two YEARS of going from dr to dr to dr, doing horrifying tests, trying medications and procedures and worry worry worry. The stress on our family was immense.

We already had baby monkeyman. Else, I don't think we would've had another child. But he was such a joy, and soon became the spill around which our family functioned. Everyone wanted to please him. He was spoilt absolutely rotten.

Then, at long last, Boeboe was diagnosed and operated on age 7. Things turned to a new normality in our house. Feelings calmed down. New routines were created. Mr N was doing extremely well with his medication. There was even talk of him maybe getting off of it at some point. Boeboe was getting better, the operation was a huge roaring success. So, we decided on another, last baby. Monkeyman was fine, we had 2 close calls with our eldest 2, but surely those days were behind us now?

Not.

Monkeyman is 5. Yes, exactly the age the other 2 were when it all started with them. WHY? This canNOT be chance?! What lessons aren't we learning, that we have to be taught over and over and over. What are we missing? Where are we going wrong? Why is this happening for a THIRD time? I can't. I simply cannot go through this again. I don't have the strength anymore. I can't face it. I honestly cannot put another child of mine through so many tests, so many procedures, so many painful experiences, so many side effects from medication that is sometimes worse than the initial symptoms they're trying to alleviate. I can't. I really, honestly can't.

I'm hanging onto hope. Maybe, just maybe, the dr was wrong. Maybe the diagnosis was just a prelimanory. Maybe it needs confirmation, where it would just be ruled out as not true? Surely that must be it. There's just no way that Monkeyman might be really, really ill. He's my baby. He's our everything. This can't be. They can't tell me he's facing a life threatening disease. This just ain't happening. So the dr must be wrong. She has to be wrong.

She said he had Pulmonary Hypertension. I've never heard of it before, and had to ask her to repeat the term. She did. And she didn't sound concerned much. Is it because she works with the worst of the worst every day? Because she sees newborn babies die due to heart disease? Because she has to operate on tiny little bodies every day? In the bigger scheme of things, pulmonary hypertension is a much better diagnosis than some of the others she has to make every week or month? Or maybe she's just a very relaxed type of dr.

Or, maybe, the diagnosis isn't so bad? Maybe we're lucky, and whatever the underlying cause, it will be easily rectified and it may even reverse the damage to his arteries and heart? I tried to find stories on the internet of children this happened to. I couldn't find anyone. Yet. In theory, it seems possible. In practice, it doesn't seem likely. Except in Pulmonary Hypertension of the Newborn. But that seems to be like a totally separate disease on its own. There's a number of types of this out there. But the 2 most important distinctions to be made, seems to be if there's an underlying cause they can find, or not. If they can, prognosis is a bit better. If they can't, prognosis, especially in a child so young, seems to be pretty awful. So I'm not even going there.

At this stage, you're probably asking but what is Pulmonary Hypertension. Well, for 1 thing, it's NOT hypertension. Hypertention is when the left heart that pumps oxygenated blood around the body, experience resistance (due to a number of possible causes) in arteries throughout the body. This resistance is measured by the blood pressure cuff. Pulmonary Hypertension, is when the right heart experience resistance when trying to pump de-oxygenated blood from the heart to the lungs. High bloodpressure in just those arteries leading to the lungs. There's a major big artery leading from the heart that splits into two big arteries going to the lungs. When resistance is experienced, the heart has to work faster and harder to overcome this. Since the heart is a muscle, working harder leads to enlargement. So yes, Monkeyman's right ventricle (lower right heart chamber) is enlarged, as well as the pulmonary arteries. Unfortunately, this can cause heart failure eventually, or other damage to the heart, arteries and/or lungs. The disease is progressive, incurable and life-threatening. Walking out of the cardiologist's office, we didn't know that. She didn't mention any of this. We had to google over the weekend, to realise what this disease precisely means.

So what could the underlying causes be? Well, for now we're going to the ENT. The cardiologist seems to want to exclude structural abnormalities as a cause. My guess is, that she wants him to check if his throat is as narrow as his dad's. In his dad's case, this causes sleep apnea. Apparently, sleep apnea can lead to a lack of oxygen at night, causing the heart to work harder and damage to the arteries of the lungs - leading to pulmonary hypertension. Adenoids and tonsils could also cause sleep apnea. After a visit to the ENT, a sleep study will be booked to check for sleep apnea.

The other possibility, is connective tissue disorders. I looked into those they list (scleroderma, Lupus, Crest, Ehlers Danlos), and I just can't fit Monkeyman in one of those boxes. I just don't see it. Maybe I'm wrong, maybe time will tell. But for now, I honestly don't think that's a cause for him.

He doesn't, as far as we know, of course, have HIV. He also doesn't have bloodclots (will probably be ruled out at some point?). He doesn't have a chronic liver disease apart from Gilberts (which is too benign to be a cause). He doesn't have sickle cell anaemia. He doesn't have any heart defects. He doesn't use illegal drugs. He hasn't used some dangerous appetite surpressants. He doesn't live 2 500m or higher above sea-level (high altitudes). All of these are possible causes. None which seems likely for Monkeyman, except maybe bloodclots or sickle cell. Both which I'm sure will be ruled out.

Asthma and other lung diseases can also cause this, but the pead and cardiologist both seems to think that this is also very unlikely in Monkeyman, because of how good and normal his lungs sound/react/look.

So ya, that leaves us with sleep apnea. After that, Idiopathic Pulmonary Hypertension. No known cause except maybe in the genes. I don't even want to go there. The fear is too great. My mom died from heart failure without they ever being able to pinpoint the cause. It's a sore, sore, sore point. I can't go there. Not yet.

So I see 1 of 4 possible roads for Monkeyman (and us).

  • 1 : Dr is wrong, Monkeyman is/will be fine.

  • 2 : Monkeyman getting diagnosed with something like a structural abnormality which could cause the pulmonary hypertension. It gets fixed via an operation or sleeping with a CPAP machine rest of his life (not ready to face that either yet). It reverses the pulmonary hypertension. No permanent damage done yet to any artery or the heart and he turns out to be just fine.

  • 3 : Monkeyman getting diagnosed with sleep apnea, but the hypertension doesn't reverse and damage was done. Leaving him on medication(s) rest of his life.

  • 4 : No cause find for the hypertension. Things goes quickly from bad to worse (since the disease is progressive in all cases). He's on medication(s) rest of his life, and would be lucky if it doesn't include a lung transplant, or worse, a lung/heart transplant.

So I'm praying so so so hard for nr 1. And if not, then at the least for nr 2. At this point, I just can't face nr 3 or 4 yet. We'll deal with that if we ever get there.

So why my whole melodramatic post? Well, think about nr 2. Wouldn't that alone, be awful for any mom&dad to face? An enlarged heart, possible damage to some of the big arteries from the heart, tests and procedures that lies ahead. The possibility of awful medications with awful side effects in yet another child. And sleeping with a CPAP machine. Sounds easy hey? So google it a bit. Look at the images. And tell me how you're going to convince a stubborn 5-year old that it's for his own good to sleep with that. Yes, maybe the fact that daddy does, will make it easier in our case? Who knows. But it must break daddy's heart to think that his child will have to go through what he does, every night.

So yeah, anything other than my nr 1, freaks me out. But, what's the chance of a pediatric cardiologist to be wrong?!??

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