Today we’re talking to Jill, an HLHS mom and the blogger behind The Real Life of a Redhead about her journey and story.
I’m honored to share Joshua’s story and help others through their journey with HLHS. It gives meaning to Joshua’s life and purpose to mine. I will forewarn you that some of the details have become fuzzy as time has gone by. I have all the details of Joshua’s life documented in my blog, but I haven’t been able to go back and reread those posts. They are too difficult for me. I don’t ever mind talking about it and sharing our experiences, but I may have to estimate time periods or details because time has a cruel but helpful way of helping to soften the blow by helping your mind forget.
Tell us a little bit about HLHS
HLHS is a congenital heart defect. 1 in 100 children are born with CHD. There are many different types of CHD’s all varying in degree of severity and treatment. HLHS is short for Hypoplastic Left Heart Syndrome. HLHS is the most severe heart defect that an infant can be born with and is 100% fatal without intervention. An HLHS heart is underdeveloped on the left side. This causes a lack of blood flow and oxygen circulation to the body. It is an extremely difficult CHD for doctors to manage, but significant strides in treatment for HLHS patents have been made in the past 20-25 years.
How did you discover your child had HLHS?
During my pregnancy, I had a subchorionic hemorrhage. For two months, I bled profusely which caused the doctors to monitor the baby weekly. The hemorrhage was completely unrelated to the heart defect, but was likely what helped doctors catch the defect early due to weekly sonograms. At 18 weeks, the ultrasound tech had a difficult time seeing the left side of Joshua’s heart. She attributed it to the position that he was laying in, but they sent me to a specialist just to be sure. At 22 weeks, we saw a Maternal Fetal Medicine doctor who confirmed through 3D ultrasounds that the left side of Joshua’s heart was underdeveloped. We were given the diagnosis of HLHS and were promptly sent for an amniocentesis to test for further defects or anomalies.
How did your friends and family respond to the diagnosis?
Our friends and family tried to be supportive of the diagnosis. Their first response was shock, which then turned to searching for answers. As we learned more about the defect, we shared what information we could with as many of our family and friends in an effort to help them understand the severity of it. Some of our loved ones began to shy away from us, unsure of how to help or how to process what was happening while others began to step up and support and encourage us however we needed it. We truly learned who were in our lives for the long haul, and who were more “surfacey” friends and family.
What were your main fears after the initial diagnosis?
After the shock of the diagnosis wore off, I was confident that we would give Joshua the best chance at life that we could possibly give him. I began researching treatements, life spans, quality of life, and the financial aspect of it all. The scariest part of the diagnosis was figuring out how to make it work financially. At the time, my husband and I were both working full time jobs, and raising 2 young children. We were barely making ends meet financially, debt was piling on top of debt, and we could not survive without me working. However, we knew that I had to quit working to take care of Joshua, so we took the leap and I quit working completely. We trusted that God would provide for our every need and that we would be surrounded by family and friends who would do anything they could to help us. We began applying for government assistance and put our house on the market as a short sale to get out from under the debt that it created. Finances were truly the most horrifying part of the diagnosis.
Tell us about your son’s journey with HLHS.
Joshua was born on August 16, 2010. Shortly after birth, he was taken to the NICU and started on a drug called prostaglandin to keep a valve called the patent ductus arteriousus, an opening between two of the major blood vessels in the heart, open until he could have the Norwood Procedure. I was warned that I would not really be able to do anything for Joshua until after his surgery. I was encouraged to begin pumping breastmilk for him and to be consistent in visiting and loving him as best as I could. Because of the many tubes and wires that he was connected to administering various drugs and oxygen to his body, I did not get to hold him for the first 2 days of his life. On day 3, Joshua was scheduled for the Norwood procedure. After finally being allowed to hold him, Joshua underwent a 6-8 hour surgery to reroute his heart to begin to allow his heart to function as a 2 chambered heart instead of a normal and healthy 4 chambered heart. Joshua’s Norwood procedure was fairly uneventful and we had high hopes that he would do well from that point forward. We were warned that after Joshua came out of surgery, he would be swollen and his chest would be left open to allow room for swelling without putting too much pressure on his heart. No words could have ever prepared us for what we saw the first time we went into his room after the surgery.
He was almost double in size and bruised. His chest was open and covered in what looked like plastic wrap to keep it sterilized. It’s not very often that a parent literally gets to see their child’s heart beating inside their chest. Joshua’s chest was left open for 3-5 days to allow his body time to rest. During that time, he was on powerful pain medications as well as a paralytic to keep him comfortable. We were told to keep his stimulation to a minimum, meaning we couldn’t really talk to him, touch him, or have a lot of noise in the room. After approximately 4 days (approximately 1 week after his birth), the doctors decided to close Joshua’s chest in an effort to get him to start breathing on his own and to begin the process of weaning him off the drugs and let rehabilitation begin. The doctors were able to successfully close his chest, but it didn’t take long before I began noticing that Joshua was turning blue. I kept telling the nurse that something was wrong and that he looked horrible, but all of the observations and tests they performed on him were within normal ranges. After watching and insisting that something wasn’t right, his alarms began going off, and sure enough, he began to code. His O2 sats (oxygen saturation levels) were quickly dropping. I screamed for help, and doctors and nurses came running to help revive him. After a harrowing 5-10 minutes, that seemed like an eternity, Joshua was stable and still with us.
Things began to become a lot less exciting in the days that followed. This was a good thing! A boring day in the life of Joshua meant things were progressing as they should without any hiccups or setbacks. Joshua’s swelling slowly decreased and the doctors began weaning him off some of the drugs. Because of the nature of the drugs he was on, Joshua began displaying symptoms of drug withdrawal. They began treating him with Methadone to help wean him off the drugs and keep his symptoms at bay.
After approximately 3-4 weeks of recovery, we began noticing that Joshua’s oxygen saturation levels were at 100%. For a normal baby, this would be expected and anything lower would be cause for concern. However, in HLHS babies who have just undergone the Norwood procedure, they like to see the O2 sats at 60%-80%. 100% meant that his lungs were receiving too much oxygen and putting an extra stain on his heart. One day, I was caring for Joshua in his hospital room, and noticed that he was acting different than he normally did. I began telling the doctors that there was something wrong, but again, all tests were within normal ranges and they couldn’t find anything wrong. I kept on top of his care and watched him like a hawk, all the while, the doctors were telling me to go back to my hotel room and get some sleep. I had, after all, been spending 15-16 hours a day in his hospital room by myself, providing most of his care except for when a doctor or nurse came to administer drugs or monitor his progress.
Finally, after a few hours of trying to convince the doctors that something wasn’t right, Joshua’s O2 levels began dropping. He turned blue and unresponsive. I screamed, again, for help and doctors and nurses came flooding into his room. They whisked me away while they continued working on him, and promised to do everything they could to save my baby. After what seemed like an eternity, the doctors were finally able to stabilize him. Although he was back on the ventilator, he was still with us.
When the doctor came to tell us what happened, he told us he couldn’t believe that Joshua was still with us. He explained that he had never seen a baby come back from the kind of cardiac arrest that Joshua had just experienced. I was never more thankful in my life to hear those words.
After Joshua coded for a second time, the doctors began looking for the cause of it. It was determined that his oxygen saturation levels had been too high for too long and his body was deprived of oxygen. They quickly began deciding if Joshua needed another surgery to revise the shunt that was currently in his heart. They thought that, perhaps, his shunt was too big, allowing too much oxygen to his lungs. He was scheduled for a shunt revision just a few days after he coded.
However, in the days between, he began having episodes of convulsions. After many tests, including a lumbar puncture, it was determined that he was without oxygen for too long during his last episode, and was having seizures as a result from minor brain damage. The neurologists weren’t horribly concerned about it, but they decided to monitor him over the next few days and weeks. In the following days, Joshua had his shunt revised through yet another surgery.
The surgery was successful and his body began responding positively to his new heart flow and shunt. We began working on feeding with a bottle as well as breastfeeding instead exclusively using the feeding tube. We began physical therapy to address some of the physical issues that accompany spending your first 8 weeks of life paralyzed and addicted to pain medication. We began working towards a common goal of being discharged from the hospital and bringing Joshua home to continue growing and healing until his next surgery, the Glenn, at 6-12 months old.
After 7 long weeks in the hospital, the doctors began feeling comfortable that Joshua would be discharged soon and we would be able to go home. However, on October 6, 2010, I arrived in Joshua’s room around 8am like any other day. I noticed he was crying and I picked him up and tried to comfort him. It was then that I began noticing that his alarms were going off. His oxygen levels were dropping and his heart rate began decelerating. I, again, began screaming for doctors and nurses to come. They flooded his room and I began trying, helplessly. to get his toys and blankets out of the way so the doctors could treat him.
I was whisked away and taken to a quiet room where a nurse went between me and Joshua with information about what was going on. I knew this time, that things were not great and that this was the end. Every time Joshua coded, only his oxygen levels were affected, never his heart. This time, his heart stopped while he was in my arms. The doctors tried their hardest to revive him, but when the nurse told me to come and in hold my baby, I knew it was time to let him go. My husband was at work, 2 hours away, that morning and I called him to tell him he needed to get down to the hospital as soon as possible. He left almost immediately, but it was too late. Joshua was gone. At 8:58am, Joshua took his last earthly breath in my arms and entered into Heaven.
Did you find it was hard or difficult to get information on HLHS? What sources of information were most helpful?
I did not find it difficult to find information. I am a realist by nature and began searching both the internet and the library for any information I could find. I wanted to know the good and the bad of HLHS and began searching for blogs with both positive and negative outcomes. We were also referred to the Fetal Care Coordinator at our hospital who provided us with information about the heart defect as well as what lay ahead for us in the hospital.
The most valuable information I found was through other families who were already living with HLHS children. The provided valuable information about the defect itself, as well as information about how to survive. They provided invaluable tips about surviving the hospital stay, what to expect, good questions to ask both before Joshua was born and while he was receiving treatment. They provided information about the real life struggles of juggling medications, government assistance to help cover the costs of mounting medical bills, juggling other children in the family, and information about FMLA. We truly would not have survived if it weren’t for the wisdom of other families who were already walking the HLHS journey.
What made you decide to blog about your journey?
Writing has always been a passion of mine. From the time I was married, in 2004, until 2010, I had been blogging on another blogging platform. I wanted to take the next step and my “blogging career” and begin earning a small income from my writing, so I opened my blog on Blogspot. It was shortly after I opened that blog in December of 2009, that I began simply to chronicle my life as a young wife and mother. When I found out I was pregnant 2 months later, it was only natural to chronicle my pregnancy along with everything else. My blog was my way of recording our life events, but quickly turned into a way to keep others updated on what was going on with the pregnancy, our kitchen fire, and eventually Joshua’s fight against CHD. Since all of our treatment was 2 hours south of where we lived, my blog became a way for family and friends to keep up with ways to pray and Joshua’s progress without having to call or text myself or my husband.
What was your time in the NICU like?
Our time in the NICU was like an emotional and physical vortex. We were surrounded by sick and dying babies. Families would come and go, babies would be born, released, or pass away. All the while, we were in our own private little room simply trying to give our son the best chance at life that we could. My mornings would start around 7am. I would grab a quick breakfast at the hospital cafeteria or the hotel that I was staying in while Joshua received his care. I would quickly head to his room at 8am and spend the next 15-16 hours there, leaving only to pump breastmilk, use the restroom, or grab a quick bite to eat. I always tried to arrive at his room in time for doctor’s rounds so I could have a better understanding of the care Joshua was receiving as well as the game plan for his future. After rounds, I would simply stay in Joshua’s room and “mother” him as best as I could. I would give him baths (sponge baths and a quick dunk of his hair under the faucet, which he LOVED!), I would change his diapers, I would administer any medication that I was allowed to administer. We worked on feedings and I participated in physical therapy. I sang to him, rocked him, and watched him while he slept. I blogged while he slept, keeping up as best as I could with what was going on at home. I Skyped with my other children and husband nightly. While I was in the NICU, I learned the comings and goings of the doctors. I learned the medical terminology that was used to describe Joshua’s progress and heart defect. I became an advocate, a nurse, and a cardiologist all at once. I was completely submersed in the medical culture of the NICU.
Was there anything that would’ve made your time in the NICU easier?
I would have really loved to have been able to connect with other parents who were staying in the NICU. Because of the privacy laws protecting patient privacy, I was not able to connect with other parents who were in the same shoes that I was. I would have loved to have someone to eat lunch and dinner with. Someone that I could talk to and find camaraderie with. I also would have loved to have been able to sleep in Joshua’s room instead of being forced to leave every night to go to the hotel. I would have gladly stayed and helped provide for his care at night, but NICU policy did not allow me to stay.
How important was it for you to connect with other parents? Do you think it’s been important and helpful?
It was extremely important and still continues to be important for me to connect with other parents. Even though Joshua is no longer with us, I love connecting with and offering support to families who are facing the chaos of life that we faced. When we were first exploring the world of CHD, it was important for me to hear both the good and the bad of raising a child with CHD. I drew strength from others struggles and triumphs. I felt supported and loved in knowing that I wasn’t the only one facing the trials that we were facing.
What are the things that they monitor in the NICU in an HLHSer?
They monitor everything. First and foremost, they monitor the oxygen saturation levels and heart rate. These are very good indicators of how effective their surgery was. They also monitor the I’s and O’s- the in’s and out’s. This monitors the amount of fluid or “food” the babies takes in and how much they put out. This tells the doctors if the babies are receiving the proper amount of fluid and if their kidneys are functioning properly. They also monitor blood gasses to ensure that the babies bodies are producing the proper amount of gasses. They monitor drug dosage, general disposition of the babies, physical growth, and any other issues the babies may be facing.
What are important vital signs and things to pay attention to?
All of the things that the doctors and nurses monitor are important as a parent to pay close attention to. It not only helps you monitor your child and figure out what is normal for your child, but it also helps the doctors monitor your baby more closely. Parents provide valuable data for the doctors to use in your child’s care. It’s almost important to pay attention to your intuition. My intuition was right every time Joshua coded. The tests weren’t showing anything negative, but I knew something wasn’t right. Because I continued to “pester” the doctors and nurses about it, they watched Joshua closely and when he finally did start showing signs of distress, they were on top of it quickly. A mama’s intuition is a powerful tool. It’s also important to pay close attention to what the doctors and nurses say and do. While most hospitals strive to provide continuous and consistent care, sometimes there are mix ups. Doctors and nurses are human and can make mistakes, it’s important for parents to ask questions about every procedure, drug, treatment, therapy, and test that their child is put through.
What advice can you give to other parents during their time in the NICU?
Be involved in your child’s care. Ask questions about everything, and if you don’t understand, keep asking. You are your child’s best advocate. If you think there is something wrong, don’t take no for an answer. You have the right to ask your doctors and nurses “why” and if you aren’t satisfied with their answers, you can ask to see a supervisor. Be involved in every aspect of your child’s care. Also, get to know your nurses. Bring them treats, write them thank you notes. Be kind to them. Sometimes they are the neck the turns the doctor’s heads. Their job is to advocate for your baby, and they are more likely to be on your side if you treat them with respect. It’s amazing the strings they can pull for you or your child if they care just as much about you as they do your baby. Finally, take some time for yourself. Chances are, your child will be in the NICU for a while. It’s long and exhausting to sit in the hospital each and every day. Take a few minutes for yourself each day and read a book, take a walk, take a nap, or even take a long hot shower. Just be sure to care for yourself. If you aren’t at the top of your game, you are no good to your baby. Getting good rest allows you to think more clearly and make better decisions regarding your child’s care.
Did you keep bag of emergency items for when you had to go to the hospital at a moments notice? What were they?
Since our hospital was 2 hours away, I kept a couple days worth of clothes in a bag along with extra toiletries and some laundry detergent. I also packed some snacks, some reading material, a notebook, pens/pencils, emergency contact information, and a list of other items that would need to be packed that I couldn’t include in the bag right away (that way I could look at the list and quickly gather the things I needed without having to try and remember all of it while panicked or in a hurry). I also carried a small card in my purse at all times that explained that I was pregnant with an HLHS baby with the contact information for my OB as well as the hospital’s phone number just in case I was in some sort of an accident or incapacitated somehow and couldn’t explain that I was carrying a critically ill child.
Can you explain the difference between the NICU and PICU and when your child goes to one or the other?
The NICU is the neonatal intensive care unit. At the hospital Joshua was at, that is where he spent a majority of his time. It houses babies who are newly born and usually not older than 6-8 months old. Often times they are babies who were born premature or have difficulty breathing when they are first born. The PICU is the Pediatric Intensive Care Unit. Joshua was transferred to the PICU for his Norwood and shunt revision. This is a unit that specializes in pediatrics. The nurses and doctors in this unit care for children from birth up to 21 years of age who suffer anything from severe dehydration to severe illnesses to surgery recovery. The NICU offered all of the specialized care such as feeding therapy and body temperature control.
What should parents know about feeding tubes?
My only experience is with an NG feeding tube. This is a tube that is inserted through the nose and ends directly in the stomach. The baby is either continuously fed or bolus fed. A continuous feed provides constant nutrition for an extended period of time. A pump usually pushes nutritious fluids through the tube slowly. A bolus feed generally takes between 20 to 30 minutes to complete. A bolus feed typically depends on gravity to push the liquid through the tube into the stomach. Tube feeding is typically done to help HLHS babies put on weight in an effort to help the child grow for their next surgery. The feeding tube is also usually needed because HLHS children are typically dependent on breathing tubes for long periods of time which causes oral aversions which can hinder eating in an HLHS child. Feeding tubes can seem scary, but once you become used to using it and inserting it, it becomes manageable.
Any tips for nursing an HLHS baby?
Joshua had an extremely difficult time breastfeeding. It was difficult to monitor how much breastmilk he was receiving which caused concern for weight gain. However, it was extremely important for me to continue to try to breastfeed throughout Joshua’s life. Even if he didn’t receive any of my milk through nursing, he was receiving skin on skin time and was given attention and the closeness that a heart healthy baby receives. Continuing to attempt nursing was an important part of bonding for both Joshua and myself. On top of regular “nursing” sessions, I pumped breastmilk every 2-3 hours around the clock. The NICU kept an ample supply of my milk frozen for Joshua’s regular feedings. My milk continued to provide the many needed antibodies he required to help keep him as healthy as possible.
What about an HLHSer who tires quickly and isn’t able to feed enough?
A feeding tube or g-tube is the answer to feeding issues. It doesn’t typically seem like an ideal solution, but it truly does help alleviate the stress of feeding. It allows the babies to conserve their energy to use for growing and healing instead of using it in an effort to feed.
How do you remember your HLHSer Joshua and deal with grief?
Currently, I make handmade baby dolls called Joshie Dolls. These dolls are made, in memory of my son, for children with major medical scarring on their bodies. The scars on the dolls bodies are matched specifically to the child that is receiving the doll. It is an incredible way for Joshua’s name to live on while helping other children across the world to have a doll that looks like them.
Other than the Joshie Dolls, I continue to write about and verbally share Joshua’s story. By talking about my grief and the journey with Joshua, I am able to help others who may be facing difficulties in their own lives. The greatest joy and blessing I have had through Joshua’s life is being able to minister to others.
