Ralph - Cardio Update

Ralph is three. He is fun. He has a mind of his own. He is a diaper escape artist. He totally cracks me up!

He gets medicine at 11pm. Here is what I found last night when it was medicine time:

He had shorts on when I put him to bed, but they were obviously not snug enough!

This is what I found this morning when I gave him his 7am meds:

Do you see him? Under the sheet? I'm laughing just thinking about it now. The sheet was still secure at all four corners. I can't imagine how he got under there without ripping a corner of the sheet off. That boy!

Ralph had his cardiologist appointment yesterday. He's a little rock star at the cardio office. All the ladies fawn over him whenever he shows up. Yesterday was no different.

EKG time first. It is always mildly humiliating...the things we have to do to get him to sit still and not pull wires when he gets his EKG. Bubbles, silly dances, anything to get his mind off the wires stuck all over his chest. This time wasn't so bad.

Then came the echo. We took a walk down the hall to the echo room and they already have a Sesame Street video playing. Ralph wanted to watch the video, but he didn't want to lay down to do it! I convinced him but he kept scooting around.

I used to keep my eyes glued to the echo screen, trying to see if I could guess what the doctor would say about the size of his heart and the pulmonary pressures. But, I came into this appointment very confident that these things were improving, so I only snuck a quick glance.

We don't have to wait long for echo results upon returning to our room. Ralph's heart is less large than it was five months ago. Good news! Especially since he has been off his diuretics for about that long.

Ralph's pulmonary pressures have not improved. They are about the same as last time. This is NOT what I was expecting. It's not terrible news, but it is enough to take the wind out of my sails.

Here is the reality: Ralph has pulmonary hypertension. It is a chronic, often progressive, disease. He can grow out of it, sure. He might not. At this point in time, we cannot afford to let his pulmonary pressures increase at all.

I've been sloppy with his medication schedule, thinking that he was doing just great. Well, no more of that. He is back on a strict 8 hour schedule. And his dosage has gone up, since he is growing.

This is not what I wanted my family to have to deal with when I'm out of the country adopting Alec and Zhenya. I need to put together a little check sheet to make sure the important things like medication get done on time. I'll put that on my list. To make a list.

Good news!

Dawn. Not always my favorite time of day. But, a view like this makes getting out of bed a little easier.
Ralph had a great visit to the cardiologist on Wednesday. For the first time ever, he walked in under his own power. He stood on the big scale and not the one with the baby seat. He stood by the wall for the nurse to measure his height instead of her using the measuring tape.

He let her take his blood pressure and pulse ox. He only ripped of an EKG probe twice...he's NOT a perfect angel! The echo was like a rodeo. I have to laugh when I think about how much wiggling and bucking he did. It is a wonder that any decent measurements were taken.

Apparently, the measurements were good enough for the doc. He would have taken us back to redo the echo himself if they weren't! His pulmonary pressures measured normal.

Normal.

That's a word we don't hear around here all that often. It has a nice ring to it! We are cutting back on Ralph's heart meds over the next three months and then stopping them. He goes back for a recheck in six months.

You can take your pulmonary hypertension and shove it!

One year ago...

A year ago, approximately.
It's hard to remember adjusting the cannula a thousand times each day. It is easy to forget all about changing tanks and tripping over tubes. It seems like a far off dream.

There is the one keyboard that Ralph has never destroyed!

I never knew about Pulmonary Hypertension before Ralph came along. Now I know more than I care to. When Ralph sees his cardiologist tomorrow I hope to hear that his PH is history. I hope to hear that he has grown out of it. He's been off of oxygen for nearly one year and has done well. The next step is weaning heart medications, IF his pressures look good tomorrow.

I've been disappointed before. But I never lose hope.

Day 8 - PH

It's 10:30 pm. I'm mentally done for the day. Actually I've been done for awhile! But I can't go to bed yet.

Ralph gets medicine in one hour. He takes Sildenafil every 8 hours - 7:30 am, 3:30 pm, 11:30 pm - for his pulmonary hypertension. He'll be soaking wet because of the Lasix that he takes at 7:30 pm, so I'll have to change his diaper and possibly his PJ's and bedsheets, too. 

It's getting old.

I know of a few other kids with DS who have pulmonary hypertension. It's a terrible disease. Ralph seems to be outgrowing it! (I'm almost afraid to type that, but I don't believe in jinxing.) Some other kids...not so much.

When I start feeling weary, or sorry for myself, I like to think about people like Parker and his family. When you send your kid into a heart cath procedure expecting improved numbers because he's looking SO healthy lately...and you get a slap in the face...it just sucks.

Parker is touring the medical specialists again so some good friends have put together a Scentsy giveaway. Could you spare a 5 spot for a darling boy with Down Syndrome and a case of pulmonary hypertension that won't let up? You could win some yummy holiday scents!

6 months

Ralph saw the cardiologist today! He was soooooo good at first. A real big boy!

First we have the EKG done by the most wonderful nurses. They just love on Ralphie like he's the best baby ever, which he is by the way! He let her check his O2 levels and sat real still. He let her take his blood pressure nicely, too. Then the EKG...he didn't try to rip off all the stickers or the wires at all! The past few visits were very exhausting, trying to keep him still for the EKG and pulse ox and blood pressure. The nurses and I end up dancing around the room, blowing bubbles and singing like fools to keep his mind occupied elsewhere.

Then the echo.

Well, it wasn't all that bad. The echo tech knows Ralph and always has a video going when we come to the room. Ralph had a hard time keeping still. He kept kicking his legs and trying to grab the echo probe. There were actually two techs in the room. One was working on Ralph and the other was on FACEBOOK!! I could hardly believe it. I thought about playfully scolding her about FBing at work, but I decided to keep my mouth shut. I usually don't keep my mouth shut, so I was pretty proud of myself for being so mature.

Ralph's ASD closure device looks like a little yo-yo on the echo. Pretty cute, huh? He was done with the echo in no time today. I took that to mean things were looking pretty good.

We went back to the exam room to wait for the doc. Oh man, did we wait a while! I'm glad I brought Richard and a box of toys with me today. Richard played ball with Ralph and played a "name the toy" game, too. Ralph wore him out and started trying to escape from the room. He figured out how to open the door and got out a few times!

When the doc came in he talked with Ralph for a minute, but Ralph grabbed his stethoscope and made the doc listen to his chest. I guess he likes to cut to the chase! The echo looked good, he said. The pulmonary pressures were running about 25! YAY! This is lower than I can ever remember. (Normal is around 15. Ralph has been as high as 70) AND he is getting bigger so he is outgrowing his medication.

We are going to continue to let Ralph outgrow his medication over the next six months. Do you realize that puts us into January before we see the cardiologist again? 2010? Wow. Barring any severe illness, Ralph should continue to outgrow his pulmonary hypertension. Praise GOD!

Ramblin' catching-up

Life is so stinkin' busy around here right now! I feel like I'm just hanging on tight so I don't fall off the ride. Ruby is snoozing for the moment so I'll have to make this fast!

Thomas did well on his state assessments last week. It was three days, two tests per day. Thankfully dad was in town last week and was able to help me get him there and picked up several times. He was signed up for morning testing, but he had to leave early and come back in the afternoon one day so that we could attend a memorial service. Phew.

Yes, a memorial service. James aunt passed away on April 1. She was young, too young. Cancer sucks and then it sucks some more.

Wesley passed his first CLEP test last week. Thomas, Leroy, Ralph, Ruby and I dropped him off on Thursday afternoon and then went to Eastborough Park to kill a couple of hours. Leroy likes to harass the ducks there!

Chipper had his first track meet last week and it got rained out. :( He had another one today and then an orchestra concert tonight! Richy is also running track this Spring.

Oh joy! Summer rec baseball practice starts this week. I've gotten a few emails from coaches about practice times. It's always funny because the emails usually say "I'm your child's coach this year" and so I have to do a little research to figure out which of the children they are coaching! Five children, five different teams. Next year I'm going to offer them $20 ($50 even!) NOT to play baseball. I think my sanity is worth a couple hundred dollars, don't you?

Wesley's graduation is coming up! WOW! He is so incredible...really a treasure to me. We ordered some announcements today because tomorrow it the deadline to turn in announcements for the graduation ceremony scrapbook. That's me...last minute scrambling. Oh, also turned in a graduate info sheet to church today...2 days late. I know people understand my being lame, because I've been preoccupied with Ruby, but it still feels bad to be so lame!

Ralph had his appointment with the cardiologist today. I brought along a Signing Time video for him to watch during the echo. I thought he would be so mesmerized by it (like he is at home) that he would be still and good. Well...no. Of course today he wanted to show off all his signs! I had another bright idea to slip his socks on his hands during the EKG! He still managed to pull some wires off with his toes!

Ralph's test results were pretty good. He has been off of oxygen for a little more than a month. And, he is staying off of oxygen!!! His pulmonary pressures were only slightly higher than last time when he was still on oxygen. He is running at 35-40 mmHg. Not terrible. Now we are working on taking him off of some of his medications. After some blood tests this week, I'll know more.

One thing the doc said that bothered me...he mentioned Bosentan in passing. I'm pretty comfortable with the medications that Ralph is taking right now. I've been under the impression that his PH would be improving over time, but maybe I'm wrong. Bosentan scares me. It would be something to consider is Ralph gets worse...a lot worse. He is such a yummy little piece of sunshine! So, I can't think about that right now. If I do, I'll go crazy.

I'll leave you with a little sample of my sunshine:

This is Peter

Last week was an amazing week for commitments at Reece's Rainbow. Several of the most at-risk children that I had been praying for had families commit to them. While I was looking at their sweet faces this morning I noticed a new face. This is Peter.

I noticed that he is only a couple of months older than Ralphie. He was born in February 2007. Then I noticed this in his description:

"congenital heart disease (ventricular septal defect with high degree of
pulmonary hypertension"

and my heart skipped a beat or two. High degree of pulmonary hypertension? This little guy needs a family to love him and access to excellent medical care right away!! Ralph has pulmonary hypertension, and let me tell you this - untreated it is a killer. Forget about the institution he will be sent to when he turns 4 or 5 years old. This sweet little guy may not live that long!

I just donated $10 that I don't have to his adoption grant fund. I challenge to you do the same thing. And please pray that the family God has chosen for him will step up to the plate soon and rescue him. God bless you.

Donate to Peter's adoption grant fund on this page.

Pulmonary Hypertension

Thanks to my friend Pam at Rhett's Journey I found out that November is Pulmonary Hypertension Awareness Month. There are not many words, outside of cancer perhaps, that strike terror into the heart of this mother like pulmonary hypertension.



I describe PH to people as high blood pressure in the lungs. It's really a big deal because the high pressures in the lungs cause the heart to work harder to push the blood through, resulting in right heart failure. This can be deadly as the heart simply wears out.



Symptoms of PH are chest pain, coughing, shortness of breath, edema, fatigue and lightheadedness. OK, now imagine asking a baby if they have chest pain or feel fatigued! Ha! Ralph has a regular EKG, and also an echocardiogram which can approximate the pressures in his lungs. He has had a heart cath done twice. With a heart cath the doctors can directly measure the pressures in the right heart.



Ralph is on PH medications as well as oxygen around the clock. He doesn't mind wearing his nasal cannula most of the time. If I leave him in his jumper seat too long, though, he rips it off and starts chewing on it! Also, if his 50ft. of oxygen tubing gets hung up on something he will take it off and keep right on crawling. I suspect that he has learned that I cannot find him if he crawls away without the cannula on!



At night I monitor Ralph's blood oxygen levels with a pulse oximeter. This is a handy dandy piece of equipment, even though it doesn't work when he is awake. When Ralph was sick with rotavirus last Spring it was the oximeter that let me know he needed immediate medical attention. I could not get a signal on either of his feet that one morning. The meter worked on my finger, so I knew that it was working properly. I figured out quickly that his pulse was too weak for the meter to pick up. We raced to the doctor's office and then on to the hospital immediately. Just in time, too.



Ralph has been nearly completely healthy since that scare. And since his heart repair in June and an adjustment in his medication in July, his heart size is improving and his pressures are normalizing. We are on a long and winding road. Will he ever outgrow his PH? Children sometimes do, although it is considered a chronic and incurable condition for adults. Will he EVER get off oxygen? I sure do hope so. Probably not for a while.




When you are dealing with PH, Down Syndrome doesn't seem so scary. Sometimes it seems that the docs, at the hospital especially, are so hung up on Down Syndrome that they don't see him as a baby with PH. I get so worried about the PH that I sometimes forget about the DS!



I'm convinced that there are certain aspects of gene over-expression that come along with Down Syndrome that may aggravate his pulmonary hypertension and it drives me nuts. I don't have the knowledge to read and understand the research in these areas and I don't really have anyone who is all that interested in helping me.



Perhaps Ralph will live to see amazing advances in treatment! We briefly discussed a heart-lung transplant with the doctors in Kansas City last year, but the survival rate is poor and can be a case where the cure is worse than the disease. New drugs are being developed, but many take a terrible toll on the liver. I'm quite thankful for Sildenafil, AKA Viagra! It has done wonders for him. This drug was initially developed for high blood pressure but the researchers found that it had an interesting side effect - and the rest is history.

Changes?

A large percentage of babies with Down Syndrome are born completely healthy. Many are born with heart defects and other problems that are easily repaired. In a few cases the problems are serious. In Ralph's case, pulmonary hypertension makes DS look like a walk in the park.

Ralph has been pulling off his oxygen at night and when his blood oxygen levels fall his O2 alarm goes off. Nothing really new about this! I've been getting up countless times at night to fix the cannula for many, many months. Some days I wonder if I will ever get a full night sleep again!

Lately things seem to be changing. The alarm is going off less and less often. Even with the cannula off his O2 levels are not falling enough to set off the alarm! During the day, Ralph likes to take the cannula off and chew on it. Of course, I can't monitor his O2 levels during the day, because the meter just doesn't work when he is up and moving around.

So...do these changes mean that his pulmonary hypertension is resolving?

I talked to Ralph's cardiologist's P.A. today and we have a plan. Since he has an echo scheduled for later this month I will cut his liter flow in half, from 1/2 liter to 1/4, for the next couple of weeks. We will then see how the pulmonary pressures look on the echo. As I know from experience, blood oxygen levels don't always tell the whole story where PH is concerned. But, I'm still optimistic that Ralph's lungs are growing and remodeling and his PH may eventually be nothing but a bad memory.