August 21, 2009

The Bittersweet Reality of Transplants

Posted in Uncategorized at 6:50 pm by bk2nocal

Receiving a transplant is bittersweet in many ways.  First, the fact that one needs a transplant is bad, but the fact that they could actually find an organ compatible is good.  Second, the fact that one now has a functioning organ is good, but the fact that one will have to be on immunosuppressants, with all of their side effects, for the rest of one’s life is bad.  Third, the relief as a parent of a child receiving a transplant is huge, but the constant worry that follows it is also huge.  I am lucky in that I am typically a pretty optimistic person who does not want to worry about things that may not happen until they happen.  But, in this situation, I find myself constantly in a state of underlying worry and stress.  I am happy that M’s rejection level went from a 3 to a 2 in the last two weeks, but I am also worried that the number was not a 1 or 0 as the transplant coordinators seemed to want.  I am also worried that M is now having to have twice as many biopsies as they original had her scheduled to have (she has another in two weeks).  I am also worried everytime I think about all of the side effects of the medicines she is required to take.  I find my stress level going through the roof everytime she has spits up more than a very slight amount (the digestive system is usually one of the first signs of heart failure in infants).  It all weighs heavily on me, even if I don’t regularly “think” about it directly.   I just want her to be well again, and really she is right now.  She is doing great.  But, I don’t want this rejection to get in the way of that.  I can only make sure she gets her medicines that she is supposed to get and hope that they do the job they are designed to do and do not cause her any more health problems. I am so thankful that she has received a new heart that, by all medical tests and appearances, seems to be functioning well.  But, that thankfulness is always tempered by these other worries – hence, the bittersweet reality of transplants.

On a positive note, research is moving forward constantly in the area of transplants.  I am hopeful that some of this research can have a positive impact on M’s life – give her (and me) some relief from her lifelong pursuit of balancing rejection against maintaining her basic health.  On that front, research being done right here at Lucille Packard Children’s Hospital is attempting to identify gene markers for those likely to be able to tolerate transplants without immunosuppressants and those who have a necessity for the immunosuppressants.  For me, the most exciting prospect is this:

“The real value of this technology is the ability to easily and repeatedly monitor patients over long periods of time,” said Sarwal. “We can keep an eye on this genetic signature and watch for changes that might indicate the beginning of rejection before any clinical signs are apparent. This could be a very exciting advance for both patients and physicians as it can lead to the ability to, for the first time, safely customize immunosuppression for an individual patient.”

Right now, the immunosuppressant levels seem like such a guessing game for the doctors that this idea of being able to customize for the individual patient seems huge.  You can read more about this research here.


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!