April 15th 2008 was the day we found out that our baby had a very complicated Congenital Heart Defect. I was 24 weeks pregnant and was at a Targeted Ultrasound without my husband Mike. I remember praying and crying a lot. Dr. Snyder did a Fetal Echo that same day and told us the hard truth about Hypoplastic Right Heart Babies. He explained the three surgeries and advised us to do an Amniocentesis to check for chromosomal disorders. Two days later we flew to Ireland to visit my family. Upon our return all our care was transferred to Columbia Presbyterian, NY following a negative amniocentesis.
In naming our baby, we wanted to make sure that we gave him a strong name so we named him Matthew Joseph, Matthew means “gift from God” and Joseph was close to Mary, mother to Jesus who we prayed to a lot through the rosary for Matthew! He was born on August 6th, 2008 via scheduled C-Section. His PICC line became infected during the first week in NICU and he ultimately ended up with his first open-heart surgery on August 21st, 2008. Six days later he had a Ladds Procedure, an abdominal surgery. He finally came home on September 2nd to two very happy older brothers Michael and Ryan not to mention two ecstatic parents. Weight gain was an issue and we added Polycose, Human milk fortifier and Rice Cereal to Breast milk to help with this. He gained weight slowly. Matthew is asplenic and has heterotaxy. He had a Grade IV Reflux in his right kidney which resolved untreated! His liver is midline rather than right lower quadrant. His Glenn Procedure was done on February 25th, 2009. He returned home six days later. He ended up with a right sided Chylothorax and was on low fat diet and formula, Portagen for four months.
On June 16th 2010 we learned that Matthew’s Ventricular Diastolic Pressure were high, meaning his heart beats OK but does not rest adequately. After a Case Conference his Fontan surgery was postponed and he was placed on a medication regimen including Sildenafil, Aldactone, and Digoxin. After seeking second and third opinions from Boston Children’s and CHoP, Coreg was added to the med regimen thanks to Dr. Rychik at CHoP.
In May, 2011, a cardiac cath revealed decreased Ventricular Diastolic Pressures and Matthew was eligible for the third surgery, The Fontan.
Matthew had his Fontan surgery on August 17th, 2011. All went well in the Operating Room, the surgery was done off the lung heart machine and he was extubated before coming up to ICU post operatively. He had many ups and downs in the post-op period, including pain control, large right pleural effusion, fast heart rate, constipation and low Potassium levels. He came home on August 26th and did OK for about two days. He was readmitted on September 1st with Bilateral Pleural Effusions and finally came home on September 6th. He started Preschool on September 27th, just shy of six weeks post operatively
A Cardiac Cath in the summer of 2014 revealed a serious problem, the blood return from the lower part of Matthew’s body was mostly returning to the heart via a collateral vein due to a blockage at the anastomosis (joining) of the Inferior Vena Cava and the Pulmonary Artery. After much discussion and a second opinion from Children’s Hospital of Philadelphia, it was decided that Dr. Bacha would need to redo Matthew’s third surgery The Fontan and replace the blocked conduit. This was done on November 10th 2014 and Matthew had an uneventful recovery and was home with his brothers in one week. He did start on Coumadin which requires weekly INR checks which I do at home with a machine and a simple finger prick.
He continued to do well until March, 2015 when he was admitted to Cardiac ICU in Respiratory Distress, he was initially supported on BIPAP, weaned to CPAP and then nasal cannula. He was treated with antibiotics since it was assumed he had Pneumonia. He was readmitted six days later in severe Respiratory Distress. An emergency Cardiac Cath and Bronchoscopy was scheduled for Friday, April 10th, coincidentally the ten year anniversary of Matthew’s Uncle Chucky’s passing. We found some comfort in knowing he was watching over him that day. They found that his left lung was completely blocked and full of what we now know were casts. Casts are formed when lymph leaks into the lungs and combines with proteins and form a thick substance that looks like chewing gum. Matthew now had another diagnosis to add to his already long list called Plastic Bronchitis. A Cardiac Cath a few days later revealed good pressures in his heart with a closed fenestration but a collateral vein was acting as a pop off so no stent was needed. He was initially treated like a cystic fibrosis child with Pulmozyme nebulizer and a vest for 20 minutes twice a day.
He did well until July 2015 when he was readmitted to the hospital after coughing up another cast and desatting. He had another bronchoscopy and responded well to treatments. At this point we reached out to Children’s Hospital of Philadelphia who has a whole department that works with patients with lymphatic issues including Plastic Bronchitis. They recommended increasing Matthew’s Aldactone and Sildenafil and also advised us to add Q-Var, an inhaled steroid as well as discontinuing the Pulmozyme and adding Levalbuterol which is the cardiac version of Albuterol. Matthew coughed up one more cast on September 26th , he responded well to Prednisone and has been cast free since then.
He continues to receive 20 minute vest treatments twice a day with Q-Var and Levalbuterol. He also continues on Aldactone twice a day as well as Sildenafil three times a day. Coumadin, Amoxicillin and Lasix are also part of his medication regimen.
Matthew is currently in First Grade and doing well with Spelling Tests and learning to read. He is playing flag football and soccer and having fun with both! He continues to inspire us all to be happy and enjoy life!
Update on Matthew 2/17/2018…..
Matthew is currently in 3rd Grade taking nine medications a day! He struggles at times academically but gets a lot of help and works very hard, we are very proud of him! The last time he coughed up a cast was September 26th, 2015. We discontinued his vest treatments and nebulizer treatments in June 2016. In June 2017 we discontinued his Q-Var. He is currently on no respiratory treatments at all!
In November and December 2017 we noticed that his heart rate was high 148 to 156 at rest, also known as tachycardia. His blood work was normal, his ECHO was normal for him and he was well hydrated. We placed him on a Zio patch monitor for a week, which confirmed what we already knew, he was tachycardic at rest but we had no answers. Matthew grew one and a half inches in a two month time frame and we attributed the tachycardia to his heart simply trying to keep up with a rapidly growing boy.
As a family we have also made some memories and did some travelling. We celebrated Nana’s 80th birthday in Florida, visited The Bahamas and lastly visited Ireland for the first time in four and a half years in July 2017. Granny and Grandad Spaight were very happy to see Matthew and his brothers.
As everyone knows we take it one day at a time with Matthew! We are hopeful for the future and all the continued CHD Research. There are a lot of very smart people working very hard to make life better for single ventricle children everywhere. We greatly appreciate them all, as well as all your love, support and prayers! We could not do what we do without you all!
Tomorrow two very special birthdays will happen in Heaven for Faith Venezia and Eiren O’Hara. I realize every day how blessed we are to have Matthew here with us still. He has had some bumps in the road the last few years but continues to fight a courageous battle with his CHD.
On May 5th, the day Matthew received his First Holy Communion, we rushed him to MSCHONY with a very bad episode of Plastic Bronchitis. Within 24 hours he was on a ventilator with his oxygen levels in the 50’s, he was a very sick young man. We prayed and after an Emergency Bronchoscopy early in the morning on May 7th, he improved slightly. He remained on a ventilator for the next few days. We reached out to CHOP and they advised the ICU Doctors to use TPA to loosen up the Plastic Bronchitis Casts to improve his oxygen levels. Matthew was transferred via ambulance to CHOP within 2 weeks and Dr. Dori performed “The Dori Procedure” on Matthew a few days later. After a tough recovery, Matthew took a photo with his brothers at the Rocky Statue in Philadelphia, it was a very fitting tribute to our fighter!
In June 2021, Matthew had a Cardiac Cath and a stent was placed to open up his Pulmonary Vein.
Matthew fought Covid-19 twice ,the first time was in December 2020, he had no symptoms, the second time was in July 2021 and he had symptoms but with some meds, rest and hydration he recovered.
In the Summer of 2022, his energy level was quite diminished, and he was starting to look puffy around his eyes and lips in the mornings before taking his Lasix and his heart rate was high 120’s to 130’s. We did some bloodwork and found out that Matthew had low Albumin levels which was causing these symptoms. Through testing four weeks apart, it was confirmed that Matthew had Protein Losing Enteropathy (PLE). It is unfortunately a very debilitating diagnosis and Matthew had a difficult time with going to school. He was tired, had bad headaches and stomach pain and vomited on and off and his heart rate was high most of the time 130’s to 140’s. He ended up at New Milford ER receiving an Albumin infusion by the end of October 2022. We did start him on a steroid Budesonide, twice a day in the hopes that it would ease some of the symptoms. Most of these children end up getting listed for a Heart Transplant quickly since there is no good treatment or fix for PLE. We saw Dr. Zuckerman of the Heart Transplant and Heart Failure Team and he noticed a slight change in Matthew’s EKG. Matthew was later diagnosed with a Variable Ectopic Atrial Rhythm and he was started on Toprol XL once a day. He responded well to that treatment and his heart rate came down to the 80’s and his PLE symptoms eased.
In April 2023, we took a family vacation to Florida and then Dominican Republic and had a lovely time making memories! Unfortunately, when we got back Matthew suffered some very bad headaches and vomiting. We tried some IV Fluids and an Albumin Infusion at New Milford Hospital Emergency Room with no improvement. By Day #3, Matthew was vomiting blood. We ended up at MSCHONY and after a few days of IV Fluids, Matthew was feeling a lot better.
Matthew had another Cardiac Cath in June 2023 and another stent was placed. He struggled with dehydration while recovering and ended up staying in the hospital for two days.
Matthew graduated 8th grade on June 15th,2023 and has started High School at Immaculate High School in Danbury. We are a few weeks into the school year and he is enjoying it so far, making new friends and getting adjusted to waking up a lot earlier! He spent a week at Hope with Heart Camp in August and loved it. He has also been enrolled in a program called Team Impact where they take medically fragile children and pair them up with local college teams. Matthew hit the lottery and got paired up with Team Army Football out of Westpoint. He has been surrounded by a brotherhood like no other. He gets to have dinner with the team every other week and hangs out on the sidelines during the games. The guys have been so welcoming and even drove to Sherman to support Matthew at his 8th Grade Graduation. He will be part of this program for the next two years.
We are grateful for your continued love, support and prayers, it means the world to us! We take it one day at a time and know that we are blessed to have Matthew is our lives.
Thank you from the bottom of our hearts and Matthew’s half a heart for all your prayers and support and for joining on this journey through the complex world of Congenital Heart Defects. We are making a difference for sure!