A Disclaimer

For those of you who know me personally, you know why I began this blog. For those who do not, I'd like to tell you "why."

First off, it's funny to me to say that I blog - no offense to my fellow bloggers out there - I subscribe to many beautifully written blogs and you don't write fluff!  But I have a science degree, I've worked in the healthcare industry since I was 18 years old, and I've been in clinical research for several years, therefore when I write, I do so based on what I have read in medical journals.  I present what's established by the medical community.

I was an inquisitive child and I am an inquisitive adult, therefore I am always asking the question "why."  When my son was diagnosed in utero with Hypoplastic Left Heart Syndrome, I had A Lot of questions.  Four repairs later (Arch repair, Norwood, Glenn, Fontan) I still have questions.  Why the Arch repair? Our doctors thought Carter had a chance at being two ventricle. Why it didn't work and he had to become single ventricle? It just didn't work for him. Some questions are easier to answer.

Over the past seven years I have met many heart families. An amazing bunch you all are I've learned. Some of their children are doing great, some struggle, and some we have lost. That makes me ask WHY??  Some questions are harder to answer.

My son has HLHS. The future is bright, but there will always be questions.

So that's why I write this blog.

And here's my disclaimer:

I am not a doctor; I do not give medical advice. I'm just a mom of a heart kid who will always have questions. I hope that through my asking questions I can answer some of yours too.

Julie

New Research Survey for Post-Fontan Patients!

The University of Michigan C.S. Mott Children’s Hospital has an important Research Study for children who have completed the Fontan (third stage) surgery. This research study is to evaluate Protein Losing Enteropathy (PLE), and Plastic Bronchitis (PB) in post-Fontan patients. You are invited to complete this anonymous survey whether your child has or has not been diagnosed with PLE or PB. This information will help the doctors learn more about PLE and Plastic Bronchitis and find a way to successfully treat these complications. 

Complete the Survey!

 

Meet Chelsea!

About a month ago, I had the pleasure of meeting a very sweet young lady through the HLHS-Hope Facebook page by the name of Chelsea Schnell.  Chelsea is an HLHS survivor, age 19. She is a college student, athlete, fashionista, and blogger.  Chelsea was kind enough to answer what I think are common questions asked by parents of children with HLHS.  Chelsea began her own blog to help others with HLHS. Please visit Smile, life is beautiful.   

Q&A:

How old were you when your parents first explained to you that you had a heart defect? Do you remember what they said?

I am sure that they had told me before but the first time I really understood that I was a little bit different, but wasn’t till elementary school that I got the “heart talk”. I remember my mother saying “Now remember you aren’t like everyone else, you cannot push yourself. When you are tired, you are tired so stop and take a break.” My parents never said you have a heart problem so you cannot do this or that. We never talked about it; there was no reason to when I was young because I was never really affected by it. I get told more about my heart now than I did when I was a child.

Do you remember if when you were in elementary school you had a hard time keeping up with your peers? How did you handle this?

No I never had a hard time. I ran and played at recess just like everyone else. I never felt different during physical activity. Elementary school was easy, Middle school is when it got harder because that’s when people realized I was the only one with a scar.

Do you feel different from other kids because you have HLHS?

No I don’t feel any different; I just know that I must be healthier than most teens.

Do you ride on roller coasters?

Yes! Of course I do, I love heights and scary rides. There is not a ride I won’t do!

Do you play sports?

I love sports! The first sport I played was T- ball when I was 5, then I started figure skating when I was 6 and still continue to skate. Throughout my sports career I have played basketball for 6 years, I rode motor cross for several, I threw shot put and ran track in middle school and high school I was captain of the Varsity Tennis team.

Do you take any heart medications? Do you feel any side effects because of your medications?

For my heart I am on Linsinopril which I take every day. Along with that I take Actigall, folic acid, baby aspirin (3 days a week) and Centrum vitamins and Caltratep. I do not feel the side effects from the medicine but if I miss a day then I feel side effects which are tiredness and lack of energy.

What has your doctor said to you about your heart in the future? Will you need any other surgeries?

No, no surgeries are in my future and thank the Lord! I am different than most cases of HLHS when I was born all of my stats were in the 95-98% it was like I never even had heart surgeries or a half a heart. I am so blessed to say that my future looks just as bright as any other persons. My future is up to me and how I live. I must take care of myself and know what’s good for me and what is not!

What has your doctor said to you about drugs, smoking and alcohol?

NO, NO & NO! Drugs absolutely not, I even try to stay away from Tylenol and Advil. Smoking is not even something to consider. I do go to college and of course I go to parties and out with my friends to events, but if someone is smoking I walk away or leave plus it makes me feel so sick if I do inhale it. Alcohol; never had a sip or even a finger dip. I do not want to risk it. Alcohol is just as bad as caffeine for HLHS babies. I was told to stay away from all soda drinks, coffee, teas and absolutely no energy drinks. I drink tons of water since the only way my blood travels throughout my body is by pure gravity, the water helps pump my blood around faster and better since my heart cannot!

What have you been told about birth control and having children in the future?

This is probably the toughest part about having HLHS, I could go through numerous surgeries, doctors’ visits, stress test, IV’s, anything but being told I could not have children was the hardest and most painful. I always wanted to be a mother since I was young, I wanted triplet girls and then to adopt, my mother thinks I am crazy but that is what I want. I cannot have children of my own the doctor said but there are ways around it. Surrogacy is most likely what I am going to do and is the best and really only option other than adoption. But see also I use to pray to God when I was little that he would let me adopt a little girl from Asia, well God granted my wishes. So me not being able to have children of my own leaves me to adopt a daughter I have always wanted.

Birth control has also been a sticky situation. I cannot go on the pill or shot or patch because I cannot have estrogen in my blood stream. I can get an IUD which is what most HLHS women have. But my mother is always worried that is it not 100% guaranteed protection. My Gyno is working on finding me something that is easy just like the pill; she is still working on that. My Gyno said the safest and most practical thing to do is get my tubes tied. I am contemplating that and am pretty sure I will go and get the surgery for it.

It is just a risky situation altogether because if you cannot use birth control, then if you do get pregnant your heart is not strong enough to support both you and the baby but you also cannot even think about abortion because your heart cannot handle the medicine from an abortion.

Protein Losing Enteropathy: A Post-Fontan Complication

As defined by PubMed Health, Protein Losing Enteropathy (PLE) is an abnormal loss of serum protein from the digestive tract or the inability of the digestive tract to absorb proteins. PLE is a rare, but well-known complication of HLHS, which may develop in some children weeks or years after the Fontan procedure. Symptoms include swelling in the abdomen and feet, diarrhea, fever, and abdominal pain. Complications include malabsorption due to diarrhea, an impaired immune system which increases the chances of infection, and low cardiac output.

Sadly, PLE is associated with an alarming mortality rate. The 2007 study, Protein Losing Enteropathy after Fontan Surgery - Clinical and Diagnostical Aspects by Tarnok, Bocsi, Lenz, and Janousek, from the Department of Pediatric Cardiology, Cardiac Center Leipzig GmbH, University of Leipzig, Germany, states that between 2,000 and 5,000 children have been diagnosed with PLE. Of the children that have major cardiac surgery, about 7-10% becomes afflicted with PLE. Of these, there is only a 50% survival rate.

 

The cause of PLE in post-Fontan children is unknown. There are many studies on this condition suggesting the use of various medications such as steroids and diuretics for treatment, but further research is necessary to better understand this condition. 

Currently, the Children’s Hospital of Philadelphia, through its Single Ventricle Survivorship Program, follows a treatment protocol that has proven effective in managing PLE. The use of Sildenafil (Viagra®, Revatio®) lowers pulmonary flow to improve cardiac output after the Fontan operation, in addition to the use of the steroid Entocort, to reduce bowel inflammation. 

Talk to your child’s doctor to learn more about PLE and the symptoms of this condition.  

If your child has been diagnosed with PLE and you are interested in connecting with a support group, please contact the Congenital Heart Information Network for more information. 

Current Research Studies of the Top Ten Best Children’s Heart Hospitals

As a parent of a child with HLHS, I often think about what concerns other heart parents have about their children. Although our children have a common diagnosis, they are very different developmentally, physically, socially, and emotionally. A general internet search of Hypoplastic Left Heart Syndrome leads us to basic information on the diagnosis, heart anatomy, surgeries and transplant and very little else, but leaves us with many questions. How does a child develop HLHS? Can HLHS be prevented? What does this mean to a child with HLHS? What does this mean to a family of HLHS?  How best to share our information with one another? How can we help the doctors put the pieces together of this puzzling heart defect? One goal of mine is to find ways to expand on the current published knowledge parents can access regarding HLHS. I also strongly encourage parents to participate in research studies involving our special kids. This is the key to answering the many questions we have about HLHS.  In the meanwhile, I would like to share with you some of the current research studies the Top Ten Children’s Heart Hospitals are currently working on to better care for our children.

Children’s Hospital Boston

Principal Investigator: Jane Newburger, MD
Protocol Title: Brain Structure and Function in Adolescents after the Fontan Operation
http://www.childrenshospital.org/research/clinical/TrialDescr.cfm?id=236

Principal Investigator: Pediatric Heart Network Multi-Center Study
Protocol Title: Single Ventricle Reconstruction Study Extension

http://www.pediatricheartnetwork.com/svrextensionforparents.asp

Principal Investigator: Pediatric Heart Network Multi-Center Study   
Protocol Title:  Fontan Follow-Up Study Basics

http://www.pediatricheartnetwork.com/fontan2forparents.asp

Principal Investigator:  Mark C Wesley, MD   
Protocol Title:  Pharmacokinetics of Tranexamic Acid (TXA) in Children Undergoing Repair of Congenital Heart Defects Utilizing Cardiopulmonary Bypass

http://www.clinicaltrials.gov/ct2/show/NCT01045356?term=Congenital+heart+defect&recr=Open&rank=6

Principal Investigator: Michael Agus, MD  
Protocol Title: SPECS: Safe Pediatric Euglycemia in Cardiac Surgery 

http://www.clinicaltrials.gov/ct2/show/NCT00443599?term=Congenital+heart+defect&recr=Open&rank=22

Principal Investigator: John M Costello, MD MPH
Protocol Title: Effects of Perioperative Nesiritide or Milrinone Infusion on Recovery From Fontan Surgery 

http://www.clinicaltrials.gov/ct2/show/NCT00543309?term=Congenital+heart+defect&recr=Open&rank=34

The Children’s Hospital of Philadelphia

Principal Investigator: Jack Rychik, MD
Protocol Title: Sildenafil After the Fontan Operation (SAFO) 

http://clinicaltrials.gov/ct2/show/NCT00507819

Principal Investigator: Robert E Shaddy, MD   
Protocol Title: Evaluation of Panel Reactive Antibody in Children Following Stage I Palliation for Hypoplastic Left Heart Syndrome

http://www.clinicaltrials.gov/ct2/show/NCT01135485?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3APA&age=0&rank=4

Principal Investigator: Felice Su, MD
Protocol Title:  Understanding Dexmedetomidine in Neonates After Open Heart Surgery

http://www.clinicaltrials.gov/ct2/show/NCT00576381?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3APA&age=0&rank=8

U-M C.S. Mott Children’s Hospital

Principal Investigator: John Charpie, MD, PhD    
Protocol Title: SPECS: Safe Pediatric Euglycemia in Cardiac Surgery

 http://www.clinicaltrials.gov/ct2/show/NCT00443599?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3AMI&age=0&rank=1

Texas Children’s Hospital

Principal Investigator: Tia Tortoriello Raymond, M.D.   
Protocol Title: Trial of Vasopressin and Epinephrine to Epinephrine Only for In-Hospital Pediatric Cardiopulmonary Resuscitation

http://www.clinicaltrials.gov/ct2/show/NCT00628550?term=heart&recr=Open&state1=NA%3AUS%3ATX&age=0&rank=12

Lucile Packard Children’s Hospital

Principal Investigator: Stephen J Roth, MD, MPH  
Protocol Title: Aminophylline to Prevent Acute Kidney Injury in Children After Cardiac Surgery (KID PRO AM).

http://www.clinicaltrials.gov/ct2/show/NCT01245595?term=Congenital+heart+defect&recr=Open&rank=27  

Children’s Healthcare of Atlanta Pediatric Hospital

Principal Investigator:  Brian Kogon, MD
Protocol Title: SPY Imaging System: It’s Role in Pediatric Cardiac Surgery 

http://www.clinicaltrials.gov/ct2/show/NCT00446927?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3AGA&age=0&rank=1

Principal Investigator: Shannon E Hamrick, MD      
Protocol Title: Magnetic Resonance Imaging (MRI) to Evaluate Brain Injury in Congenital Heart Disease (CHD Brain) 

http://www.clinicaltrials.gov/ct2/show/NCT00932633?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3AGA&age=0&rank=4

Principal Investigator: Denver Sallee, MD  
Protocol Title: Abnormal 3-D MRI Flow Patterns in Adolescents Patients with Bicuspid Aortic Valve

http://www.clinicaltrials.gov/ct2/show/NCT00412386?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3AGA&age=0&rank=6

Children’s Hospital of Wisconsin

Principal Investigator: Steve Zangwill, MD      
Protocol Title: Erythropoietin and Pediatric Cardiac Surgery (EPO) 

http://www.clinicaltrials.gov/ct2/show/NCT00451698?cond=congenital+heart+defect&state1=NA%3AUS%3AWI&age=0&rank=1

Principal Investigator:  Rohit Rao, MD  
Protocol Title: Multi-Site Near Infrared Spectroscopy (NIRS) Monitoring of Children During Exercise 

http://www.clinicaltrials.gov/ct2/show/NCT00556231?cond=congenital+heart+defect&state1=NA%3AUS%3AWI&age=0&rank=2

Principal Investigator: Nancy Ghanayem, MD  
Protocol Title:  Near-Infrared Spectroscopy (NIRS) Neurodevelopmental Outcomes (NIRS ND)

http://www.clinicaltrials.gov/ct2/show/NCT00464100?cond=congenital+heart+defect&state1=NA%3AUS%3AWI&age=0&rank=5

Cincinnati Children’s Hospital Medical Center

Principal Investigator: Katherine Krawczeski, MD     
Protocol Title: Intravenous L-Citrulline to Treat Children Undergoing Heart Bypass Surgery 

http://www.clinicaltrials.gov/ct2/show/NCT01120964?term=citrulline&recr=Open&state1=NA%3AUS%3AOH&age=0&rank=1

Nationwide Children’s Hospital

Principal Investigator: Vidu Garg, MD
Protocol Title:  Genetics of Congenital Heart Disease

http://www.clinicaltrials.gov/ct2/show/NCT01192048?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3AOH&age=0&rank=1

Principal Investigator: Aymen N Naguib, MD           
Protocol Title:  Measures to Lower the Stress Response in Pediatric Cardiac Surgery

http://www.clinicaltrials.gov/ct2/show/NCT00848393?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3AOH&age=0&rank=2

Back to the Basics

Every spring, I perform the customary ritual. Spring cleaning. I clean all of the windows, dust the winter’s cobwebs away and scrub my house from top to bottom.  And most definitely, I clean out the closets.  Why is that every year I have to do this? Well, winter is a long season and this is how I welcome in the clean fresh spring air. And as far as the closets, well, quite simply, kids grow and so out go the pants and sweaters from fall.  Of course it doesn’t stop there.  The kids have grown since last summer so the summer clothes have to be sorted too!  Now in our house, only Carter as the youngest can get hand-me-downs from Augie – and it stops there because Augie’s next older sibling is a girl, and the next oldest is a boy, and the very oldest is a girl, so they all have to get new clothes, which means I have a lot of sorting and boxing to do and then a trip to the donation center. 

Which brings me back to the basics.  Spring cleaning is a ritual we perform every year in our homes. So this year, I’ve decided to do this not only to our home but to our mind and bodies as well. I feel this is very important for a heart family.  This venture of mine stems from two campaigns; first, the We Can! Program sponsored by the National Heart Lung and Blood Institute, National Institutes of Health to enhance children’s activity and nutrition, and second, the book I have been reading titled, “Simplicity Parenting,” by Kim John Payne, which offers simple strategies to simplify your home to reduce stress and over stimulation.  

How does all of this relate to a child with a congenital heart defect?  Physical fitness, good nutrition, fun with the family, and a safe and clean home, are basics. In my home, and in my readings, I have found that learning to live with a chronic condition is not only challenging for the child who is affected, but for his/her family and friends as well. Our school-aged heart kids may be fearful, feel they are different, and may struggle emotionally and socially. My theory is that happy and relaxed, healthy parents will raise calmer, well-adjusted, healthier children. 

Spring cleaning to me – it’s a holistic approach to life.  Consider spring cleaning your family!

http://www.nhlbi.nih.gov/health/public/heart/obesity/wecan/index.htm

http://www.simplicityparenting.com/

http://www.med.umich.edu/yourchild/topics/chronic.htm

Can We Talk?

In the roller-coaster life of a child with a heart defect, sometimes parents need someone to turn to when they want to have a conversation with somebody else who understands what their daily life is like.  We’ve all been there – the sympathetic nodding of the head, the jumbled response, the pause in conversation – When all a parent wants to say is I’m frustrated because my child …… and I don’t know what this means.  It’s the unknown, the unpredictability, and the everyday challenge of living a normal life.   I’ve spent some time researching support groups and I’ve listed the ones I think have something to offer to everyone.  

Mended Little Hearts
Mended Little Hearts partners with hospitals to offer resources to families diagnosed with a congenital heart defect and provides support to families by trained volunteers through Hospital Visiting, Internet and Telephone Visiting, Group Meetings and Social Events.
http://www.mendedlittlehearts.org/

The Congenital Heart Information Network

CHIN has a very nice Community section where you can communicate with others in the Chat Room during regularly scheduled discussions, join an Online Support group, visit the Family Room to read other families’ stories and share yours, see the Portrait Gallery with photos and stories of members, and visit the Memorial Garden which was created to honor the "brave warriors" who are no longer with us.  Visit their Resources page for a list of Book Reviews and links to local, regional and national support groups.

http://www.tchin.org/

Saving Little Hearts

Saving Little Hearts is an organization that is dedicated to helping children with congenital heart defects and their families by providing emotional assistance and educational information primarily through the distribution of Care Packages to children undergoing heart surgery.

http://www.savinglittlehearts.com/

Little Hearts, the Children’s Heart Society, and Heart Defects Families Association

These non-profit, volunteer based organizations provide support groups to parents and parent-matching programs.

http://www.littlehearts.org/

http://www.childrensheart.org/

http://www.chdfamilies.org/index.html

Whether you decide to join a group and become an active member or just drop in from time to time to visit the resource pages, I hope you find comfort in the knowledge that you are not alone in your struggle.  There is a community of families, volunteers, and dedicated medical professionals who are there to support, educate, inform, and connect with you in a very positive way.

Can My Kid Really Do That?

Being a parent of a child with a heart defect is a frightening thing when you consider physical activity and the stress on the heart.  But summer is almost here; it's a time for swimming and baseball and lots of outdoor physical activity, which brings a discussion about exercise to mind.  Single ventricle kids have to work pretty hard sometimes to match what they want to do, with what their bodies allow them to do, but that is no reason to keep them out of the pool or off the baseball field. The heart is a muscle after all, and it needs exercise to stay conditioned. Research studies have shown that exercise can improve cardiac function in a child’s heart that has been damaged by a heart defect. Please talk with your child’s cardiologist about what is recommended for your child.  A general guideline for single ventricle kids is no competitive or contact sports due to the excessive strain on the heart and the risk of bleeding. There are however many other activities to enjoy; swimming, golf, tennis and bike-riding are great low impact aerobic activities.  Hydration is important in maintaining body temperature for single ventricle kids, so drinking a lot of water is a must when outdoors.

I recently read a wonderful story by an undergraduate student who wrote about her experience as a volunteer at a camp for heart kids.  Please follow this link to read her story:

http://www.pelinks4u.org/articles/negron0809.htm

The Hospital for Sick Children in Ontario Canada has an ongoing research study titled, “Optimizing Health among Children with Congenital Heart Defects,” which can be found at:

http://clinicaltrials.gov/ct2/show/NCT00974688

The warm summer months are when our children thrive, so get out there and enjoy it!  

What is Hypoplastic Left Heart Syndrome?

If you're visiting here, you are most likely not new to this diagnosis, but I thought it would be helpful to share what I learned when my son was first diagnosed with HLHS.

Hypoplastic left heart syndrome (HLHS) is a combination of several abnormalities of the heart and great blood vessels. It is a congenital (present at birth) syndrome, meaning that the heart defects occur due to abnormal underdevelopment of sections of the fetal heart during the first 8 weeks of pregnancy.

In the normal heart, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, and then is pumped through the pulmonary artery into the lungs where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, and then is pumped out to the body through the aorta.

           

In hypoplastic left heart syndrome, also known as single ventricle condition, most of the structures on the left side of the heart are small and underdeveloped. The degree of underdevelopment differs from child to child. The structures affected usually include the following:

           

·         Mitral valve - the valve that controls blood flow between the left atrium and left ventricle in the heart.

·         Left ventricle - the lower left-hand chamber of the heart. It receives oxygen-rich (red) blood from the left atrium and pumps it into the aorta, which takes the blood to the body. The left ventricle must be strong and muscular in order to pump enough blood to the body to meet its requirements.

·         Aortic valve - the valve that regulates blood flow from the heart into the aorta.

·         Aorta - the largest artery in the body and the primary blood vessel leading from the heart to the body.

           

Perhaps the most critical defect in HLHS is the small, underdeveloped left ventricle. This chamber is normally very strong and muscular so it can pump blood to the body. When the chamber is small and poorly developed, it will not function effectively and cannot provide enough blood flow to meet the body's needs. For this reason, an infant with hypoplastic left heart syndrome will not live long without surgical intervention.

           

Hypoplastic left heart syndrome occurs in up to four out of every 10,000 live births. The syndrome comprises 8 percent of all cases of congenital heart disease. It is one of the top three heart abnormalities to cause problems in the newborn. HLHS occurs slightly more often in boys than in girls.

           

What causes hypoplastic left heart syndrome?

            Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. In hypoplastic left heart syndrome, there may be abnormalities of other organs, such as diaphragmatic hernia, omphalocele, and hypospadias.

In many children, HLHS occurs sporadically (by chance), with no clear reason evident for their development.

           

What are the symptoms of hypoplastic left heart syndrome?

            Infants with HLHS usually develop symptoms shortly after birth. The following are the most common symptoms of hypoplastic left heart syndrome. However, each child may experience symptoms differently. Symptoms may include:

           

·         Cyanosis (blue color of the skin, lips, and nail beds)

·         Pale skin

·         Sweaty or clammy skin

·         Cool skin

·         Heavy and/or rapid breathing

·         Fast heart rate

           

The symptoms of hypoplastic left heart syndrome may resemble other medical conditions and heart problems. Always consult your child's physician for a diagnosis.

           

How is hypoplastic left heart syndrome diagnosed?

            Your child's physician may have heard a heart murmur during a physical examination, and referred your child to a pediatric cardiologist for a diagnosis. A heart murmur is simply a noise caused by the turbulence of blood flowing through the obstruction from the right ventricle to the pulmonary artery. Symptoms your child exhibits will also help with the diagnosis.

A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. However, other tests are needed to help with the diagnosis.      

Chest x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.

Electrocardiogram (ECG or EKG) - a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle damage.

Echocardiogram (echo) - a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves.

           

Treatment for hypoplastic left heart syndrome:

            Specific treatment for hypoplastic left heart syndrome will be determined by your child's physician based on:

           

·         Your child's age, overall health, and medical history.

·         Extent of the disease.

·         Your child's tolerance for specific medications, procedures, or therapies.

·         Expectations for the course of the disease.

·         Your opinion or preference.

           

Your child will most likely be admitted to the intensive care unit (ICU) or special care nursery once symptoms are noted. Initially, your child may be placed on oxygen, and possibly even on a ventilator, to assist his/her breathing. Intravenous (IV) medications may be given to help the heart and lungs function more efficiently.

There are two approaches offered to treat HLHS. Your child's cardiologist and cardiac surgeon will explain the risks and benefits to you. The options include the following:

           

·         A series of three operations that are done in stages: one shortly after birth, the second at about 6 months of age, and the final at about 18 months of age (these stages may vary). In this series of operations, the right ventricle is used as the main pumping chamber to the body, and blood flow is redirected to the lungs and the body with various surgical connections.

·         Heart transplantation.

Although we chose the route of surgical repairs over heart transplantation, I see the outcomes in the same way - our children are breaking the mold.  If you are interested in reading more about the treatment of HLHS at Children’s Hospital of Wisconsin, please follow the link:

http://www.chw.org/display/PPF/DocID/23065/router.asp