November 17, 2009

Preemies Deserve a Voice…

Posted in Cardiomyopathy, Health, Hospital Life, Itty Bitty Baby News, Preemie Development, Research and Development at 7:11 pm by bk2nocal

Today is Prematurity Awareness Day and I am participating in the Bloggers Unite “Fight for Preemies” campaign. I figure I will share my story and encourage those of you reading to explore the March of Dimes website for more information on how you can get involved and help the cause.  It may surprise you to find out that the US “scores a “D” and fails a half million babies each year” according to the March of Dimes.

My pregnancy was nothing special.  I was pretty normal in every way except for a creeping blood pressure that seemed to get higher and higher as the pregnancy went on.  My doctor was concerned, but not overly fearful until I went in for my 32 week appointment.  My blood pressure was elevated even more than before and the baby’s heart beat was showing some irregularities.  At first, he was going to send me home and have me report to the hospital the next morning for further fetal monitoring, but just as I was getting ready to leave, he decided that I should go to the hospital immediately to “play it safe”.  So, my husband drove me over and I was hooked up to a fetal monitor.  The heart beat continued to show irregularities and after a couple of hours, my doctor came by and told me that he was going to have me airlifted to Sacramento where they had a NICU available “just in case”.  Both my husband and I were struck with fear.  This was our first, and probably only, pregnancy (I am “advanced age” at 41) and we could not imagine losing our baby – and my husband was worried for my health.

That night, I was flown via helicopter to Sacramento, where I was once again hooked up to a fetal monitor.  My sister met us there and the doctors said that I may have to remain in the hospital for up to two or three weeks while waiting to give birth.  The hope was to “keep the baby inside as long as possible”.  The next day there were doctors in and out and in and out and finally in the afternoon, they said that the heart beat and my blood pressure were not recovering, so they were going to go ahead and take the baby C-section that night.  It was February 19, almost a full two months prior to the due date.  We were visited by a nurse who gave us the low-down on what to expect from a baby only 32 weeks along – and none of it was terribly comforting.  We were past the most crucial point in the pregnancy, but there were still possibilities for difficulty breathing, problems with eyesight and/or hearing, etc.  We were prepared for the worse when I rolled into the operating room that night.

Mackenzie beat the odds – she came out breathing on her own and was able to maintain that state.  She was on oxygen for a little while, but in every other way, she did just fine.  She was only 3 lbs. 2 oz., which by preemie standards is pretty big!  🙂  She had to have an IV and receive her food through a tube.   But, she checked out as having all her internal organs developed normally and she progressed along quickly.  My husband and I stayed with my sister and spent hours and hours at the hospital, scrubbing in to the NICU, helping with diaper changes, temperature taking, bathing and feeding.  We practiced breast-feeding and bottle-nippling all under the bright lights of the NICU, with Mackenzie in an incubator for most of the three weeks she was in the hospital.  At three weeks, and weighing just under five pounds, Mackenzie was released.  We were so excited to bring her home, even though we were woefully unprepared.   We had no bassinet (thankfully, our friend bought us one as a gift and had it ready to go), we didn’t have preemie clothes (again, my sister and friends bought us enough to get us going – my first baby shower actually came while she was still in the hospital!), but we were excited to be bringing her home.  We had medications to give her, strict rules on washing up before touching her (preemies have underdeveloped immune systems) and routines for taking her temperature each day to check for infection.

We did well for three weeks – we had weekly pediatrician appointments and we didn’t leave the house much for fear of exposure to infections, but she seemed to be doing well.  Then, in the third week, she started to have some problems breathing.  At her regular check-up we pointed it out and the pediatrician told us to just keep an eye on her.  When it seemed to get worse overnight, we made an appointment for the next day.  She was tested for RSV – a viral infection that she received a shot for before leaving the NICU – and the result was negative, but the pediatrician sent us to the emergency room immediately “just in case”.  She was tested again there and the result came back positive this time.  She was admitted and put on breathing treatments and I moved in to the hospital room with her.  We spent a week there – uncomfortable, exhausted and just plain depressed – she didn’t seem to be getting any better.  Finally, on day five, they decided she may need to be intubated and she was put on a helicopter to Sacramento (same nurse flew her down that flew me down for the pregnancy) and my husband and I got on the road to drive the two hours.

We received a phone call about an hour into the trip from the admitting doctor in Sacramento and he verified he was going to have intubate her and asked “has anyone talked to you about her heart?”  My own heart dropped.  He went on to say it was five times the normal size and it was sitting on top of her left lung making her breathing very labored.  We were left to wonder what all this meant on the rest of the drive down.

We soon discovered that she had this thing called Cardiomyopathy and that she was, as the doctor in Sacramento put it, a “very sick little girl with a very sick big heart”.  They went back and examined her X-ray that was taken in the NICU and because X-rays are easily distorted in little ones if they take a big breath, it was difficult for them to tell if her heart was enlarged at birth or not.  It had obviously gotten bigger since then either way.  But, now she would have to be on a ventilator for weeks.  Long story short, she was treated with oral medication, but failed to recover and ended up having a heart transplant on July 7, 2009.

I would like to know that every baby born, especially preemies, are tested for congenital heart defects!  These little ones are struggling enough when they come into the world, they don’t need a missed heart defect to make things more difficult.  This NY Times article talks about the “pulse oximetry” or “pulse ox” as they call it in the hospital and the findings of a study that shows it can be especially effective in catching and solving a heart issue in a newborn baby.  Although this is not just a preemie issue, I feel that it is something that can help a lot of preemies who are fighting a fight they can’t win without timely intervention.

So, let’s give all babies a voice and get these tests done – request it for your baby or tell a friend to request it for their baby.  And if it shows any abnormalities – get a closer look!


October 18, 2009

Moving…and an update on Ittybittybaby

Posted in Cardiomyopathy, Habits, Health, Itty Bitty Baby News at 11:19 am by bk2nocal

I hate it….but, I’m excited to be back “home”.  We are in the midst of unpacking and setting things up and M has officially moved from Pack n Play to her first crib!  Its been very exciting and she seems to like it.  I’m sure it has to be more comfortable to be on a solid surface.  She has her ladybug bedding and her ladybug lamp and a LOT of tubs and boxes in her room right now.  But, at least she has a solid place to sleep!

We had some disappointing news at her clinic appointment last week.  She just isn’t gaining much weight.  I guess I appropriately named this blog – she truly is ittybitty.  She is almost eight months old now – and even adjusted, she is almost six months old and last week she only weighed 10 lbs. 11 oz.  She is still swimming in the 0-3 month clothes.  They are a bit concerned and they upped her calorie mix again – to 30 cals per oz, but then she just wouldn’t eat.  I think it was too thick or too strong-tasting, but she just wasn’t eating much of it at all.  So, we went back to the 28 cals per oz and she seems to be eating much better now.  She has been terribly fussy and wouldn’t nap the past week or so, but she’s been carted from my sister’s to appointments to my parents and then moved up here and had my husband’s parents here the last couple of days and I think she’s just been really overstimulated.  She ate great this morning and has now been napping (at her normal time – hallelujah) for an hour this morning.  Let’s hope it continues!

I thought I would post a positive article to end this today and I hope to be posting more regularly now that we are settled!

September 10, 2009

Desperation or Choice?

Posted in Cardiomyopathy, Health, Research and Development at 8:43 pm by bk2nocal

When your child gets sick, you will do just about anything to help them get better.  Sometimes this means watching them suffer through painful treatments and difficult procedures.  But, you do it in the hopes that after its all over, they will be better and no longer have to suffer whatever condition brought them to the hospital in the first place.  Sometimes, the treatments have side effects that cause equally bad health problems.  For example, I have met two children in the last three months who have contracted cardiomyopathy due to treatments for leukemia.  The medicine did away with their cancer, but in the process, it caused their hearts to enlarge and stop working well.  One of them contracted it when she was 2 and did fine on medication until earlier this year, when, at the age of 15, she had to have a heart transplant.  One of them is only two right now and just found out about the cardiomyopathy.  She is in remission and the cardiomyopathy seems to be controlled with the medication she is on for the time being.  But, it is yet another blow to a family that already had too much pain and illness in their young lives.

So, what do you do?  If you know that the medication can cause side effects (many of the medications M is on have some possibly serious side effects, including, ironically enough, injury to the heart itself), do you choose to not give it to them.  Of course not.  First of all, in most of these cases, they would not survive without the medication in the first place.  Second of all, with the medical advances being made, we can all hope that by the time serious problems arise, they will have solutions for those problems that are less invasive and less injurious then at present.  And hope is hope…even if sometimes that hope is tinged with fear, we choose hope.

So, it did not shock me to read in a Times article that families in Britain have begun to seek unregulated and untested stem cell treatments in China.  It is easy to get caught up in the hope, and lose sight of the possible damage.  And, at times, you wonder how much treatment restrictions are based on marketing decisions versus safety (see my prior post where I discussed the profitability gap) and how much is based on actual safety.  I know that Europe is a little less arduous in its regulatory control and cost of bringing items to market (hence the Berlin Heart being available for children there but not yet cleared in the US), but I’m sure there are still some issues with cost-benefit analysis driving medical advances.  Its just hard to know what to do, who to believe and how to proceed.

I think having a doctor you trust in these situations is of the utmost importance.  I know when I was going through the worst days with M in the hospital, I always felt like her transplant team had HER best interest in mind when making decisions.  I never felt like they gave me false hope, but I also felt like they provided me with fair assessments and all possible alternatives.  Because they are asking you to make life and death decisions based on their information, it is key to have people you totally trust giving you that information.  I think it would be worth changing doctors and even hospitals if you didn’t feel that level of trust.  Too many people stick with medical professionals because they feel “stuck,” not because they trust them.  This must be a horrible place to be.

We were lucky to end up where we did.  I had only a couple of incidents when I felt like treatment could have been better for M, and they were really driven by individuals and were remedied soon after.  I am so thankful we had and have the team of doctors and nurses working with us that we do.  I can honestly say that I can’t imagine having had a better experience in this process – its a horrible experience to have, but it is made so much better when you are surrounded by people who you can see really care and are willing to be there for you and your child.  It may be difficult, but if you are someone who has a sick child, don’t just settle for a doctor or staff you are not comfortable with – look for one that you feel is working and caring for your child and your family.  Its hard enough without additional stress.

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 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…

June 12, 2009

Coping Mechanisms

Posted in Cardiomyopathy, Hospital Life, Itty Bitty Baby News at 7:42 pm by bk2nocal

Its strange to be spending so much time in a place where emotions run so high and to see different people coping with it in such different ways.  Obviously, not all of us are in the exact same situation, but they are at least similar – children in the hospital for long periods of time and with serious conditions.  Many are far from home and some do not have any support system other than those they meet at the hospital.  My husband and I are lucky.  Although we are away from home, we have a strong support system, with many friends and family in the area.  Rare is the day we do not have someone to visit with outside of those we meet at the hospital and ourselves.  It is good to have distractions and people who are just visiting and not here continuously.  I can see where this would become all-encompassing if you did not have an escape.

I have always been one who has thought that I should let the experts do their jobs.  I know that doctors are human and they can make mistakes, but in this environment, where there are so many people working on the same case and so many checks and balances, I think that mistakes are probably relatively rare.  And I know that cardiomyopathy is pretty rare and there are not answers to every question.  I imagine that must be frustrating for the doctors as much or more than it is frustrating to me.  Today, the doctors spent four hours replacing a Central Line in M after her last one became dislodged.  Because she is so small, this is a difficult process and she went through a number of doctors attempting to do it before they finally called someone in from the cath lab to get one placed in her groin.  She has sutures in her other groin and in her neck from previous attempts and she looks like a little pin cushion or voodoo doll with all the holes.  I guess I could get upset and impatient and complain that they did a poor job, but it seems to me that they all were doing the best they could.  They felt bad and were extremely apologetic.  They looked tired after the four hours.  Their jobs are much more difficult than I could probably ever imagine and I can’t think of complaining to them about the way they do the job.  Others are not so understanding.  They may complain and demand explanations and that may make them feel better.  It makes them feel more in control of the situation.

I guess for me, I’m not supposed to be the one in control of this situation.  My daughter is sick, with a very serious illness and I don’t have the training or experience or knowledge to be in control of this situation.  Its hard to not be in control, especially when it comes to your own children…but, it has to be done.  I don’t have any choice but to relinquish control if I want what is best from a medical standpoint.  I wish I wasn’t in this situation, but I am, and I have to do what is best for her, not what makes me feel better about myself or my situation.  I consider myself to be a pretty intelligent person, but I didn’t go to med school and I didn’t get hired by Stanford to practice medicine.  All I can do is be there for her, make sure she has what she needs from a personal standpoint – blankets, toys, etc. and then let the doctors do their job and hope for the best.

So, another day in the hospital, where human behavior is at its best (a family that had a baby next to ours brought Mackenzie a gift this week – a cute little pink outfit and just dropped it off for her while we were out) and its worst (people complaining about and to doctors and nurses about things beyond their control).

I’m thankful for all of the people who choose to go into the medical profession and choose to work with children in seriously difficult situations.  It can’t be easy and I certainly could not do it myself.  I can’t imagine working with sick kids all day and being able to keep a smile on my face – but we have many nurses and doctors who do just that.  They make you feel confident and comfortable and well-cared-for.  And, that, right now, is really all I can ask for.

June 6, 2009


Posted in Cardiomyopathy, Transplantation, Uncategorized at 2:54 pm by bk2nocal

brokenheartThe last post here was April 7.  Closely following that date, M was put in the hospital for RSV, which is a respiratory virus.  She was in our local hospital for four and a half days with respiratory distress, getting breathing treatments and on oxygen, before being flown to a bigger Children’s hospital to be put on a ventilator to give her some breathing relief.  When she arrived there and they looked at her x-rays, they noticed a very enlarged heart – five times the normal size!  Being new to this whole mom thing, I had no idea what this meant.  It was diagnosed as cardiomyopathy.  After EKGs and an Echocardiogram, she was found to have an extremely low-functioning left ventricle.  She also still had a respiratory problem, so she remained on a ventilator and was put on milirinone and heparin for her heart.  After one week there, she had a heart catherization to check the structure of her heart, which was found to be fine.  After that, they decided with the low functioning of her heart, she should be in a place where they would be better able to handle her heart failing, so she was shipped to Lucille Packard Children’s Hospital (LPCH) at Stanford.  We were informed that a heart transplant could be in her future, but that they would do everything they could to treat her by other means.  She was still being treated for her respiratory problems as well.  She spent three weeks in LPCH, two weeks with a ventilator and one week without.  She was weaned off the milirinone, which is an IV-only drug, and started on Enalapril and Carvedilol, which do similar things to milirinone but can be given orally.  It isn’t easy to give a three month old anything orally, but its a lot easier than having her on an IV!  In addition to those two drugs, she was also on Lasix to keep fluids from building up in her system and causing pulmonary edema, among other things.  In addition, she was on procrit or epogen to build up her red blood cells.   After almost a week in the hospital, getting her back on the bottle and learning how to give her shots of procrit and put in an NG tube if necessary (all of which my husband did because I was sick with some kind of bronchial infection), we were able to take her to the Ronald McDonald House with us.  After three days there, we were able to take her home with us. 

Life was definitely different.  We had a tight schedule for feedings and medicines…she was on the three heart medicines – enalapril, carvedilol and lasix twice a day, the procrit shots once every other day, a multivitamin once a day, iron three times a day and aspirin once a day, which we had to grind up and put in water to give to her.  It was all a little overwhelming to be sure.  We were home with her for two weeks and came back to Stanford for a check-up and she seemed to be doing well.  They took her off the procrit and iron and sent us on our way.  We decided to go to my in-laws for a visit before a return appointment to Stanford for a genetics appointment the following Tuesday, since their house was about the same distance as our house from Stanford.  She seemed to be doing okay, although she didn’t like the long car ride very much.  She had gained weight at the genetics appointment and although she had a touch of diahrrea, all seemed pretty well.  We headed home with her.

We were home for another week, during which the diahrrea continued and she became extremely fussy each time she was going to have a bowel movement.  She would scream, fight us, scratch, turn very red, etc.  Sometimes for more than an hour.  It was extremely difficult to deal with, but everyone – our pediatrician at home, the cardiologist on-call at Stanford, friends and family, just kept saying it was some kind of GI condition and we would just have to wait it out and deal with it.  I posted on the Children’s Cardiomyopathy Foundation’s  (CCF) listserv and asked about it and a number of people wrote back saying they had experienced the same thing with their babies.  Finally, she seemed to improve for one day, although she was now having bowel movements every feeding, sometimes twice, whereas before she was having one or two a day at most.  That last night she was really “pulling”  or retracting at the chest and around her lungs and she was looking really pale, so we called the cardiologist on-call at Stanford again and she advised us to take her into the ER. 

So, last Sunday night at 11 p.m., we took her to our local ER where they attempted to stabilize her and were advised to ship her back to LPCH.  She was flown out at 5 a.m. on Monday morning and intubated as soon as she arrived.  She has been on the ventilator since.  She now has tested positive for an infection of some sort.  The pre-transplant team came and spoke to us yesterday, and they have advised us to list her on the transplant list ASAP once her infection is over.  So, we are looking at the middle or end of next week.

In the meantime, we MAY be able to take her home, but we are going to have to move in order to be within the requisite two to three hours of Stanford in case a heart comes in.  At this point, there is so much to think about – moving, coordinating work with hospital stays, being in Stanford for months afterwards if she were to get a transplant that I can’t even really sort it all out.  We are currently staying at the Ronald McDonald House again.  It is a lifesaver to be sure.  Although we have friends and family in the area that would be happy to house us, but its just easier to be close to the hospital and not have to worry about being an inconvenience to others…there is enough to worry about without having to worry about that.

So, our experience with transplant starts now.  I am amazed when I see the kids who have had transplants and how well they are doing.  The statistics for success are pretty high nowadays.  Its a difficult thing to deal with, but at least it can be dealt with.  So, this blog is taking a slightly different focus and I am hoping that it can help others who may be in similar situations in the future and help our friends understand what we’re going through.  I have another blog at Carepages under Macpeterson that logs our experience so far in more detail. 

I am including links to as many things as I can think of that other people may want to read about and understand.  Cardiomyopathy is a pretty rare thing – according to the CCF, 12 children out of every MILLION are diagnosed with cardiomyopathy.  So, M has a pretty rare disease.  But, she is in very good hands.  The LPCH does a number of heart transplants on children every year.  So, if we have to have something done to her, she is in the best possible place for it. 

This has been a pretty lengthy post.  I could probably go on forever, but I won’t.  I am sure I will have plenty of future posts to share information and more of our experience!