August 17, 2009

“Profitability Gap” Means Adult Treatment for Children

Posted in Uncategorized at 10:22 pm by bk2nocal

Patti, a member of the Children’s Cardiomyopathy Foundation email listserv I am a member of, posted the WSJ article, “Little Hearts, Big Problems,” that speaks to the reason why, despite congenital heart defects being the number one birth defect in America, there are few treatments designed specifically for children.  The “profitability gap” was something that our social worker at LPCH spoke to us about the Berlin Heart (manufactured in Germany and only available in the US under a “compassionate” clause from the FDA).  I posted on here a little while back about a new alternative to the Berlin Heart that just received NIH funding for research, but these are few and far between.  We also experienced the problems with medications discussed in the WSJ article.  When M first went home, she was on a liquid form of Enalipril that the hospital pharmacy had provided to us on discharge.  When we ran out a couple of weeks later, there was not one pharmacy in our home town that would/could provide it in liquid form.  So, we had to go to our apothecary (yes, there still are apothecaries around) in order to have it made!

I am becoming more and more aware of the importance of raising the profile of pediatric heart problems.  This article just goes to prove that more needs to be done.


New Resource for Parents of Children with Heart Defects

Posted in Uncategorized at 3:06 pm by bk2nocal

I came across this report on and thought some of you readers out there may be interested in reading the original article and getting the book it discusses, It’s My Heart from The Children’s Heart Foundation.  Although M’s heart problems do not seem to be congenital (they are not absolutely sure as her first X-ray in the NICU was “inconclusive” as to whether her heart was already large and my ultrasounds did not seem to show any abnormalities) and the structure of her original heart was absolutely normal, a large number of people I have met here at the Ronald McDonald house and Lucille Packard have children with structural heart defects.  Prior to our experience here at LPCH, I had no idea at all how high the numbers of heart defects were in babies.  During my prenatal visits to the doctors and my ultrasound consultations, we discussed physical and developmental problems that may arise in our baby – Down’s Syndrome since I was older, Spina Bifida, etc., but I do not recollect ever reading or hearing about the possibility of heart defects.  But, the ABC article says that congenital heart defects are the number one birth defect in America.

The Its My Heart booklet comes in both English and Spanish and Cardiomyopathy, which M suffers – or do I say suffered now since she has a new heart? – is included in Chapter Three, “Acquired Heart Conditions”.  After reading the description of signs of heart failure in infants, I realize that it may be difficult to detect some of these in preemies, as they are considered somewhat “normal”.  For example, what I read about preemies indicated that since they had small noses they would tend to “grunt” a bit, especially when feeding.  In addition, preemies tend to have higher heart rates than full term babies.  And, in our case, the X-ray that was taken in the NICU showed a “slightly enlarged heart” but that can be caused by the baby taking a big breath right before they shoot the X-ray, which causes a distortion from the lungs pressing the heart up toward the X-ray machine.  So, she may have been born with it, but she may not have been born with it.

I think the booklet is a great resource for those who have just found out their child has a congenital heart defects.  It will help them communicate effectively with their doctors and nurses, feel more comfortable with the treatments their child receives and just generally understand what is happening with their child’s health.  I hope that some of you might find it helpful!

August 7, 2009

Alternative to the Berlin Heart Being Researched

Posted in Berlin Heart, Research and Development at 1:41 pm by bk2nocal

I found out sad news this week.  The little girl who was on the Berlin Heart in the CVICU when M got transferred there passed away a little over a week ago.  She had been placed on hold for a transplant because her lungs were in such bad shape prior to going on the Berlin Heart and they had hoped that she would be able to recover some lung function with the help, but her lungs were just too damaged to recover.  My heart goes out to her family.  She was such a cute little girl…

Although the Berlin Heart is a great and life-saving device, it can not work in all cases.  And in many cases, it is very invasive.  I know that with M, when she was at her worst, they definitely considered it, but said the risks were very high in smaller babies.  We were lucky in that each time M seemed to be reaching the point where it would be necessary, she was able to recover and become stable again in a relatively short amount of time.  Unfortunately, other babies and children are not so lucky.  But, there are few alternatives available.

Enter Circulite and the Synergy Micro-blood Pump for Children and Infants.  The NIH has just awarded a grant to develop this a version of their adult device for children.  The major differences between this and current choices are:

(1)  Size – the Synergy Micro-blood Pump is the size of a AA battery.  It can be implanted in a pacemaker-like pocket, so it would not require a large device to be positioned outside the body.

(2) Invasiveness – the Synergy Micro-blood Pump is minimally invasive.

(3) Term of use – the Synergy Micro-blood Pump can be used for long-term intervention.  Most of the current systems are only usable temporarily while a child/infant waits for a heart.  This pump can be placed and in adults has been used as a long-term mechanism for taking the “load” off of the heart and allowing it to “rest” and potentially even recover.

The timeframe for development of this device is pretty long.  They will first redesign the adult device to be used in Children.  They will then have to do clinical trials of the device in Children.  They will then develop the device for infants and do the same study and testing.  But, at least there are options being developed.  Because heart problems like M’s – cardiomyopathy in particular – are so rare (M is one of twelve out of 100,000 kids to have it), there is really not much financial incentive to develop devices just for their use.  But, with the help of these government grants, it becomes a bit more financially feasible.

I hate rejection…

Posted in Gift Ideas, Health, Itty Bitty Baby News, Transplantation at 12:07 pm by bk2nocal

For a whole new reason now that I have a daughter with a transplant.  The biopsy came back with moderate to high rejection (Level 3, I believe), which put M back in the hospital for three days for infusions of a steroid.  If it were not for the biopsy, we would never know that she is in such high stages of rejection.  She is doing great outwardly.  Good vitals, good tolerance of feeds, and she has even started to take more from the bottle.  But, inside, her body is fighting off her new heart as a foreign invader.  This means that she will have to have another biopsy sooner than planned.  It will be scheduled for a week from this coming Tuesday, so about two weeks earlier than originally planned.

This level of rejection doesn’t seem all that uncommon and most of the people I have talked to have said that after these infusions of steroids, the numbers start to go down immediately.  According to statistics in our heart transplant manual and The Experience Journal from Children’s Hospital Boston, nearly every transplant patient has at least one period of rejection usually in the first year.   Hopefully this will be what she needs and the next biopsy will show better numbers.  I’m just thankful all her function and vital signs, etc.  look good.  So, she at least doesn’t feel bad even though she is going through rejection…

It is a somewhat stressful process…this not knowing.  The doctor said they are currently testing out ways of checking for rejection that will not require biopsies, but none are far enough along to be expected for a few more years.  But, he said that by the time she is a teenager, we hopefully won’t have to go through this.  Medicine is advancing all the time, and we are already so lucky to be doing this now instead of many years ago when they didn’t know as much and weren’t able to solve the problems as quickly and as easily as they can now.

We should get out of the hospital today and get to return to the Ronald McDonald house.  We are currently just waiting for her to have her echocardiogram done once again and the orders written for release.  Thank goodness!

The other area of stress right now is worrying about infection.  I am ordering signs for her car seat, stroller and crib that say “Please wash your hands before touching mine”.  They are available from My Tiny Hands.  (As a sidenote, these also make great gifts for parents of preemies as they are so prone to illnesses in their first few months of life.)  I also carry around Purell everywhere I go.  I may write in on the signs that she has had a transplant – just to make sure that people know its not just some paranoid germaphobe thing (I think a lot of people think that those signs are just suggestions, not requirements) but an actual health threat.  I’m sure people are put off by it at times too.  I know prior to this experience I may have been prone to some eyerolling based on these signs, but now I really understand the importance of them!  Never assume that its just overprotective parents…

Well, still waiting for the echocardiogram…and then to the RMH if all looks okay!

August 4, 2009

Biopsy #1 Done…Results Tomorrow!

Posted in Itty Bitty Baby News, Transplantation at 11:18 pm by bk2nocal

Today was M’s first biopsy post-transplant.  Older kids would have had three by now, but because M’s veins are smaller and more difficult to navigate, they only do them once a month instead of once a week.  All signs are positive at this point.  The biopsy showed good heart function and while they were there, they checked the arterial walls to get a baseline.  I guess that hardening of the arteries is a problem for many transplant recipients.  Having a baseline allows them to track Mackenzie’s arteries and start her on a drug regimen if her arteries start to show signs of hardening.  This approach has been successful, so I’m glad they have the baseline now.

They also made a few changes to her drug regimen.  They went up on her cyclosporin a slight bit because her readings are still slightly below where they want them to be.  They went down on her prednisone slightly – she is on a long wean from it.  She is still on her methadone wean as well, but will be done with that in another week.

She is eating a lot – 100 ml at a feed, but only taking about 20-40 ml from the bottle.  The rest has to go through her NG tube through a pump.  We are going to call Occupational Therapy tomorrow to make an appointment with them to work on her feeding issues.  I feel like she will be good once we move to solids because she really chews on the nipple more than she sucks on it, but who knows?  I am hoping OT can give us some ideas on how to improve it as putting the formula through the pump is time consuming, but for now, we’ll take it.

She has not gained any weight since she got out of the hospital, which is a bit disappointing as well.  But, I am hoping that upping her feeds to 100 ml from 70-80 ml and the increase in the calorie load in the formula from 24 to 26 calorie will make this week a big week for her growth-wise.  She is so skinny and little still, its a bit disconcerting when we think about how old she is.  I don’t really think about it much until I see another baby that is younger than her, but seems so much bigger than she is!  I figure she will probably always be a bit on the small size, but being almost six months old and still in newborn clothes is a little ridiculous!

We will get the biopsy results for rejection tomorrow.  We are optimistic since all of her heart function has been very good on both the echoes and during the cath today.  But, it will be good to know for sure.

So, more on that tomorrow!

July 30, 2009

Thankful for the Daily Grind

Posted in Itty Bitty Baby News, Transplantation at 11:12 pm by bk2nocal

Well, things have been moving right along in the past few weeks!  M got a heart on July 7.  She came off the ventilator about a week later, was moved up to the Intermediate ICU for about a week and came home to the Ronald McDonald house with us this past Saturday.  It has been a whirlwind.  We are just starting to somewhat figure out the meds schedule, etc. almost a week later!  I’ll give you an idea of our typical day:

At 7:30 a.m. we are supposed to take her vital signs – blood pressure, heart rate and temperature.  Right now, we can only do the temperature part because our custom made blood pressure cuff and machine has not been delivered.  This means that we have to go to the heart clinic at the hospital to have it taken.  We did this three days in a row at the start of the week, but the cardiologist said we could take today off and go in tomorrow…take the weekend off and go in on Monday.  It was nice not to have to get up and immediately leave the house this morning!

At 8:00 a.m., she gets the bulk of her medicines.  Right now, she gets cyclosporin, cellcept, magnesium glutonate, a shot of lovanox (my husband typically does that), predinisone, nystatin (a mouth wash that keeps her from getting thrush) and pepcid.  All of these have to be loaded into syringes and then placed in her feeding tube.

At 9:00 a.m., she gets methadone.  This is temporary as she is on a wean from it right now and will be done with the wean in the middle of August.  I also have her getting her 1/2 tab of baby aspirin, which needs to be crushed and then dissolved in water and given through syringe.

At 10:00 a.m., she gets lasix (a diuretic), enalipril (a blood pressure med), and iron.  I could give these all at 8 a.m. too, but it just seemed like too much medicine all at once.  This way, she at least gets a couple of hours to get the first stuff out of her system.

We are letting her eat on her own schedule right now, just to give her a bit of a break from the constant “messing with” she was getting at the hospital.  So, we let her sleep until she wakes up crying and then we feed her.  Sometimes it coordinates with her feedings, sometimes it doesn’t…so, we aren’t on a really tight schedule at this point.  She will also sometimes go 4-5 hours between feedings, while other times she won’t even make it 3 hours.  But, I feel like she needs some good rest, so the longer sleeps are okay with me.

As the day goes on, she gets more medications.  She gets the methadone every six hours right now.  She also gets the magnesium three times a day, so she gets more of that in the middle of the day.  She gets a nystatin treatment (we put it on a little dental swab and swish it around her cheeks, tongue and gums) three times a day as well.  At night, we do it all again starting at 8 p.m., but she gets septra at night as well as the meds from the morning, but no aspirin.

I have read different people’s ideas about keeping track of all the meds – color coding, etc.   But, it seems easier to me to just have a daily schedule sheet where I check them off as I give them.  I have it done hour-by-hour and we also record her diapers and her feeds and any other notes on the sheet. That way we have one sheet and we know what the other person has done.  I think it will be helpful to anyone who comes in to take care of her in our absence as well.

We had our first clinic appointment on Wednesday.  She had to go to the Short Stay unit to get a blood draw first.  This is also where we will go on Tuesday when she gets her infusion of some other medicine that has to be infused.  After that, we went to the heart clinic and she got a LONG echocardiogram (they were training a fellow on the machine and it took a really long time – longer than I remember taking in the past) and then we met with the docs for a short time.  They said that everything looked good.  They made a slight adjustment to her meds – up slightly on the cyclosporin, down a little on the predinisone (I guess she is being weaned off this as well…but, it will be a slow wean), everything else stayed the same.

We are constantly a bit nervous.  I mean, its hard to know what is going on in a five month old.  You can’t ask them how they are feeling or get any feedback from them.  So, when she is fussy, we get worried.  When she sleeps for a while, we are worried.  Whenever anyone gets close to her, because of her immune compromise from the meds, we are worried.  Its really tiring…but, its great to have her home.

She has her first biopsy on Tuesday and I THINK I will feel better after that is done as we will REALLY know the level of rejection she is in.  The echos are somewhat indicative, but the biopsy will be more specific.  And it will just make me feel better to know for sure.

Other than that, she is still having to take half her feeds through her NG tube, which is a little disappointing, but she’ll get better at feeding with time I’m sure.

Well, enough time spent typing this…back to the daily grind!  A daily grind I am sooooo thankful for!

July 18, 2009

M Got a Heart!

Posted in Cardiomyopathy, Itty Bitty Baby News, Transplantation at 11:47 am by bk2nocal

I have not posted in a while because things were pretty hectic in my life as of late.  As those who read this blog know, my daughter was suffering from dilated cardiomyopathy and she was admitted back into the hospital with cardiogenic shock on June 1.  She was listed for a heart transplant on June 11, after the pre-transplant team decided her heart would not recover and was too big to allow for her lungs to function on their own.  She was doing okay until about the fourth week into the hospital stay and then she took a turn for the worse.  We spent three miserable days in the PICU watching her get put on drug after drug to try to deal with her blood pressure, tachycardia, lack of oxygen saturation, etc.  Finally, the cardiology team decided to move her into the CVICU, where they gradually weaned her off many of the drugs and got her back into pretty good shape by the time her fifth week in the hospital began.

On July 6, we were informed that there was a heart for M.  At first, they were going to do the surgery that night, but then it was postponed until the next afternoon.  M was in surgery for about six hours total, was on the bypass machine for less than two hours and the new heart kicked right in.  She came back from surgery looking good, but with a pacemaker because the new heart was not beating fast enough.  She did good post-op and came off the ventilator on July 14, chest tubes and pacemaker wires out July 15, moved to the Intermediate ICU July 16 and we are scheduled to be released to the Ronald McDonald House on Thursday, July 23!

It has been a whirlwind of activity!  M is doing well.  She is on 11 medications, which is actually much less than I thought she would be.  Some she takes twice a day and others only once a day and a few three times a day.  The hospital provided us with a daily schedule that will really help out with keeping track of things and we will have to stay at the Ronald McDonald house for three months to make sure that the heart and M is doing well.  She is still struggling a little to get all her feeds by bottle, so she will probably go home with an NG tube in to help with that and insure that she gains some much needed weight (she is five months tomorrow and only weighs eight pounds!) and grows a bit.   She was a preemie, but even for three months, eight pounds is not nearly big enough!

I plan to keep this blog going and posting new information on cardiomyopathy, trasnplant, etc.  I know there have been some stories in the news as of late, so I will have to catch up with those over this coming week!  Our miracle has happened – M has a new heart and will now be able to get out of the hospital – and all very quickly for such a small baby!  We are very, very, very thankful!  And now we’re looking forward to learning a whole new set of skills as we take her home!

June 22, 2009


Posted in Uncategorized at 3:03 pm by bk2nocal

I was reading the blog of another family who had a child in the PICU with Mackenzie and came across a post on courage that I feel can not be restated with any improvement.  So, I’m providing a link to it below.  Just a bit of background on this family and their connection to us:

When Mackenzie first went into the hospital back in April, she was next door to a little girl whose room was covered in pictures and art projects and happy objects.  I used to walk by that door and hear music coming from it and see all the visual happiness and it would make me feel a little better about being there.  When Mackenzie came back to the hospital early this month, she was placed in a room with two other children and one of them was the girl from the visually happy room!  She was now not in a private room, so there was not the volume of objects, but her bed still had pictures and art projects on it.  On the second day, her sisters and brothers were standing bedside singing Twinkle, Twinkle Little Star to her and it made me smile.  Over the coming days, her brothers and sisters would come to visit – always happy and smiling and taking pictures and singing – it was truly a happy distraction to have…when I saw their mom in the lobby one day I told her that I appreciated her kids being so well-behaved and always so happy and that it was nice to see healthy, happy kids in the PICU every once in while.  She thanked me and we began greeting each other in passing.  Soon after, Mackenzie was moved into a private room and her second day there, we got a gift from “Charity’s Family”…the mom and her kids had dropped off a hand-wrapped cute little outfit for Mackenzie…I was truly touched.

I went to thank them and met the grandmother – an incredibly nice woman.  Charity has since moved on to home and I hope she is doing well and will continue to thrive.  The following is a blog post that Charity’s mom wrote while Charity was still in the hospital and it truly captures the understanding that you begin to gain when you have a child in the PICU…things sometimes seem bad, but we are LUCKY that Mackenzie has something that can be treated and that she will most likely recover from and go on to live a happy and healthy life.  So many others are not so “lucky” as us…and yet they go on each day hoping for the best and giving their children what they can.  I can’t say it better, so I encourage you to read “Courage…”

June 20, 2009

From Germany with Love…The Berlin Heart

Posted in Berlin Heart, Cardiomyopathy, Itty Bitty Baby News, Transplantation at 1:40 pm by bk2nocal

Although M has now stabilized and we think she will be able to go without any “bridging” devices, when there were doubts talk began of devices that will allow her to stay alive even with a non-functioning heart.  It used to be that the only available “bridging” device for heart patients was the ECMO.  Although this machine is effective, the length of time a baby can be on it is limited – usually a matter of weeks.  Because infant-sized hearts are often not available that quickly, another “bridging” device – the Berlin Heart has begun to be used more frequently.  This device allows children to be on the bridging device for longer periods of time AND allows them to live semi-normal lives, moving around, playing, doing all the normal kidstuff they usually would be doing, while waiting for a transplant.  It is truly amazing what medicine technology can do today – and we are thankful for each new device to which we are introduced – as it means that M has a better chance of both surviving and thriving despite her big heart!

Right now, it is looking like M will not need to be placed on the Berlin Heart, as her heart is functioning well enough to not need external interventions.  But, this recent story on a baby in Canada, not only can the Berlin Heart help in cases of bridging, but it can also be used to heal a heart!  If you’re craving more information and an example of the heart being put into use in an infant, you can read this press release from 2007 on an infant in Florida…

Hearts in the News

Posted in Health, Transplantation at 1:14 pm by bk2nocal

I wanted to share a few articles that I came across on CBS News this past week that have to do with heart problems and transplants.  Some are informative, some are inspirational…enjoy!

Pump Gives New Hope to Heart Patients This article discusses the Heart Mate II which is an external pump designed in a size that fits women, that can provide assistance to hearts that are failing in women who are too small for other devices currently on the market.  The article discusses the fact that currently these devices are only available to those awaiting transplant – much like the Berlin Heart was for Mackenzie, but could, in the future, be made available to those who are perhaps older or are not transplant candidates for one reason or another.  This would allow them to live longer, more active lives with their failing hearts.  The other thing the article gives a short amount of discussion on is the fact that in this 27 year old woman, the pump actually HEALED the heart.  So, she was able to come off the machine and not have to have a transplant after all.  So, there is a chance that transplants could become a much less necessary intervention!

Doc’s Prom Pomise Fulfilled This article is a tearjerker!  Stefani Pentiuk had a heart transplant at age 8 and one of her doctors then (sounds like he was probably a cardiology fellow at the time) made a promise that she would not only survive the transplant but that he would take her to her high school prom.  Well, he didn’t take her to the prom, but he did show up to dance with her!  Although Stefani had to have a kidney transplant recently due to rejection problems, she is not only surviving, but seems to be thriving!

Study: Heart has ability to Heal Itself This article discusses new findings that heart cells are regenerating (something that was unknown before) and gives hope to improving heart functioning after heart attacks, etc.  It has some greaat interactive extras included as well!

“Polypill” for Heart Passes Big Test As someone who had to give her three month old mulitple medicines multiple times a day for a heart problem, something like this sounds incredibly promising (although I’m sure it would not be calibrated for infants very soon).  This one pill combines the necessary ingredients to ward off heart failure – “low doses of three blood pressure medicines (atenolol, ramipril and the “water pill” thiazide), plus the generic version of the cholesterol-lowering statin drug Zocor, and a baby aspirin (100 milligrams).”  M was on Carvedilol, Analipril, Lasix (water pill) and baby aspirin.  I don’t think she had to take the cholesterol-lowering drug…but everything else…

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