Liam Hilferty
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Age 2 August 2, 2002
Dedication: Liam's Trach Page is dedicated to my Aunt Carol, who suddenly passed away, November 10, 2002 of Pancreatic Cancer. She loved Liam and was always there for me, and to make me smile. I miss you......and your laughter........
Liam is one of a twin born August 2, 2000 (twin B), following a 38 1/2 week pregnancy and scheduled C-section delivery weighing 5 lbs 7 oz 19 inches. Initially following the birth, Liam and his twin (Joseph) went to the regular nursery (apgar score of 8). Liam was transferred to the NICU on day #2 of life following an episode of coughing and tachypnea that was thought to be secondary to aspiration pneumonitis. Liam received a week of antibiotics and was seen by a number of specialists. He was noted of having a hearing deficit (failed two hearing tests), a ventriculoseptal defect, and undescended right testis, large anterior fontanelle, and dysmorphic features (micrognathia, long philtrum, posterior neck folds, hypoplastic toenails and hypotonia). After 3 weeks Liam was discharged from the hospital on August 21, 2000 on caffeine with an apnea monitor and referred to CHOP Genetics Clinic for evaluation regarding a potential genetic basis for his multiple medical issues. My husband and I were so confused with all the medical words and dystrophic features they said he had. To us he looked like this perfect beautiful boy with some breathing issues.
He was seen by genetics 1 week later, although the finding in Liam are not consistent with a specific genetic syndrome, some of his characteristics have been seen in other children with histidinuria and peroxisomal disorders such as zellweger syndrome and chrondrodysplasia punctuate. He was then tested for these syndrome but once again they came back fine. The waiting game is the hardest part. We were so upset when they had to take so much blood from him at 3 weeks old, I lost it for the first time in the middle of the hospital. We thought this was the end of our problems, this was just the beginning.
The next 6 weeks would change our lives forever. Six weeks later Liam started to have multiple blue spells (4 or 5 a day). We took him to the hospital where they told us Liam had contracted RSV. We never heard of this before, so we were educated fast, but I had no idea it would be so serious. He was in the hospital for 3 days in an oxygen tent, then released after 3 days. We took him home where he continued to have these blue spells. Back and forth to the ER several times. They told us we had a choice to ride it out, or he can be admitted to the hospital again where he can contact a more serious illness. We took him home....
November 15 Liam stopped breathing again, this time we took him to a Children's Hospital in Delaware. They did more tests and they informed us Liam had severe RSV (maybe he got it twice). They admitted him to a regular room for nasal congestion, strider, and frequent periods of desaturation and bradycardia where he stayed for 2 days. He was released again on oxygen. We were so overwhelmed with Liam coming home with this huge o2 tank and on a monitor 24 hours a day. Little did we know this was nothing compared to what we had to deal with later. People get so upset and annoyed when there children are sick. They don't understand that there are much more serious illness that children and parents are faced with every day. We got so upset about an oxygen tank and a apea monitor. Looking back I am ashamed that I was so upset. Now I understand how other parents feel when their babies have to be placed on a vent all day. (GOD BLESS ALL OF YOU).
Within four days Liam was readmitted with respiratory distress and a fever of 103.5 (we called 911). A CXR was done and showed bilateral infiltrates. Carbon dix. was up to 56. This time he was sent to the PICU at Children's Hospital in Del. He was then placed on nasal CPAP 5 and supported with increasing oxygen amounts. They also placed a feeding tube in his nose to give him a break. During this entire time, Liam never once stopped eating...he is such a fighter. My sons poor nose was so sore from all the suctioning they had to do. Eventually he was weaned to a nasal cannula at 41pm. Then on 12/12/00 he developed an increased work of breathing and began to desaturate with bradycardia. This time he was placed back on CPAP 6 and 40% through the VIP Bird. Another ridged bronchoscopy was performed and it showed severe Laryngotracheal Malacia. He was incubated and placed on the ventilator settings rate of 20 PEEP and 30 % o2 on 12/15/00. The Doctors and nurses started getting us ready for Liam to get a trach. We knew this because they started showing us babies with trachs and talking about it more. We were devastated at first, we had never even seen a child with a trach.
After 3 days an attempt to extubate was made. He was extubed and placed on a nasal cannula at 41pm. I still remember to this day, my cousin Carol was with me when Liam was extubed, he tried to cry and there was no voice at all, no cry, no sound. He was sore from the trauma of the vent.
Again after 3 days he developed an increase work of breathing, desaturations and bradycardia. Thought we had lost him a few times. He was reintubated and returned to the ventilator. The doctors and nurses pulled us into the conference room and let us know Liam's only hope was for a trach. It took all my strength not to break down. On 12/22/00 Liam was taken to the OR where a tracheotomy was performed and a 3.5 Cuffless Bivona was inserted. That was the last time I heard his voice for a long time. He remained on the vent support for the next 3 days and then easily weaned to CPAP 10 oxygen at 21%. By 12/29/00 short trials of HME were initiated for 1 to 2 hours as tolerated. The nebs were continued every 4 hours. He remained stable the rest of his stay. Some friends and family told us they were sorry that this had to happen so close to the boys first Xmas. " We weren't because it was the first time in a long time we saw Liam not fighting for every breath.
On 02/20/2001 Liam was discharged home (YA, YA) on CPAP 10 with no need for oxygen.
Update - Liam was sent to the ER around 4 times in the last year and a half for either a mucus plug or pulling out his trach, which caused him to stop breathing. We were trained and to perform CPR on Liam before ENT got to our home. He was sent to the hospital just to be check out. Only one time did he have to spend the night because he stopped breathing for about 1 1/2 minutes and it was hard to get the trach back in. Once he woke up, Liam had a small seizure in the Ambulance (very scary). The hospital wanted to keep him for observation. We are so lucky Liam never contacted any infections with his trach.
Liam had many follow-up appointments the next few months. Genetics: Still never found any reason for all of Liam's medical issues. Calling it LIAM SYNDROME. He was also seen by Endocrinology for skeleton dyplasia (because Liam was not growing the way he should) he is 2 and only 28 inches. His twin brother Joseph is 34 inches. They ruled out Liam being a short person, after receiving all of the X-ray's back. They think his short statue is either caused by Liam being on steroids the first year of his life or because he was on his back in the hospital for total of 6 months when he was born. Not putting any weight on his legs. They also ruled out hip dyplasia.
Liam also went for a routine follow-up appointment with Cardiologist for his VSD which was opened at birth. The good news was that the VSD had closed but the bad news was they found a small hole called PDA. This had to be surgically closed. This was done in January 25, 2002. Liam's recovery was outstanding he only spent 1 day in CICU, his respiratory status improved after the PDA was closed. After doing a routine X-Ray on his heart to make sure the heart looked ok, they found 2 enlarged kidneys. Thank God it was just reflex of the kidneys and he was placed on an antibiotic for a year or two. We are thankful they found this because Liam could have had kidney failure later on in life. No matter how much pain and sadness we have seen our little angel go thru, we always get a wake-up call..... Seeing Liam in the CICU he was the healthiest baby in there, so many other babies have it so much worst. So if you're ever down about your baby or child, think of the other babies out there that have so many more problems, and may not have a chance to survive. That is how we get thorough each day.
Update April 2002 - Liam has had a total of 7 bronchs. One bronch showed a lot of scar tissue which had to be removed and Liam still had some form of Laryngotracheal Malacia. The most recent bronchosopy showed no more signs of Laryngotracheal Malacia BUT now showed a Grade III Cotton-Myer Subglottic Stenosis in addition to stomal collapse. We were in shock to here of a new and more serious problem.
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Last Update - Liam still continues to have ot, pt, speech, hearing, feeding and speech teacher's every week. He still has an issue with gas. reflux and is still on medication to control the reflux. He is a year behind on physical development, we hope to see him walk soon.
We are driving out to Cinn, Ohio on Sept 17 for an evaluation by Dr. Cotton. We have seen 3 other Doctors and all agreed that this reconstruction surgery needs to be done. Dr. Cotton, MD, at Children's Hospital of Pediatric Otolaryngology, Cincinnati Children's Hospital, we feel has the most experience with correcting this particular anatomical defect. Wish us luck.
Liam is due for reconstruction on September 26 , 2002, this will include, Microlaryngosscopy, Bronchoscopy and Larynogotracheal Resection with Dr. Cotton at 11:00 a.m.
Thank you for this web site it has help me understand more about trachs and Liam's upcoming surgery. Without it, I think I would have been lost. The support from the other parents has been very helpful. Good luck and best wishes to all of the families.
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Liam & twin brother Joseph
2nd Birthday, August 2002
Update October 2002 - The trip to Cinn, Ohio was not what we expected. We had the Bronchoscopy done on Friday (Sept 19) by Dr. Cotton, GI and Pulmonologist. Liam also had a PH probe study for 24 hours to check for reflux.
The results were as followed: They found Liam to still have 8% of acid reflux even on medication (zantac and reglan). He had severe inflammation of the esophagus. Which the doctors called Esophologitis. The biopsy came back that this was cause by reflux and an allergic reaction to some kind of food. So, after 78 needles they found Liam to be allergic to SOY, PORK and LAMB. Well we found out that soy is in a lot of items. The solution for the reflux is to treat with a new kind of medication and stop the zantac. For the allergy problem we have to take Liam off all SOY products to see if the inflammation goes away.
Liam was found to have inflammation in the trachea. The biopsy came back to have no bacteria. The Doctors think this may be caused from the Esophologitis but not 100% sure.
The next problem was that they found Liam's stomach to have severe inflammation (they told us this is not caused by the Esophologist). After the biopsy came back they told us it is Helicobacter. Helicobacter is a form of bacteria, a bug or germ, that lives in the sticky fluid (mucus) which coats the lining of the human stomach and duodenum. It is usually a life-long infection and may cause no problems. However, it is often associated with peptic ulcer. It is hard to cure because it is in the second layer of the stomach, but we hope by taking two different kinds of antibiotics the infections will be cured.
Liam also is being tested for Mucopolysaccharides which are long molecular chains of sugar. They are used by the body in the building of connective tissues. There can be 7 different syndromes, some have a short life span. He is getting a blood test and urine sample for this. PRAY
We are upset in what they have found and that the surgery is delayed for now. But on the positive side of this I think maybe there is a reason they found all of this, maybe Liam was not ready for the surgery and he needs to get stronger, and this is God way of telling us. We also got to spend time as a family, we had some fun going to the zoo and the aquarium. Joe and I got to know each other again, and actually got to talk a lot, so there is always a positive side to everything. I wanted to thank Joe, my husband for all the support he has given me and the boys, without him this would have been a very difficult journey. We love you...
We are scheduled to go back to Cinn, Ohio on December 9 for another Bronchoscopy. Reconstruction surgery is scheduled, hopefully on December 12, 2002.
November, 2002 - After 6 weeks of waiting, the test results came back negative for the MPS. This is the best news. Thanks for all your prays.
Update December 2002 - The trip to Ohio was short and no reconstruction this time around, strike number two. Liam still had some inflammation of the trachea and the esophagus. The good news is that the biopsy's came back negative for Esophagitis and any infection. The reason for the inflammation in the airway and esophagus, it is still healing from the allergy's. Taking Liam off of all soy and pork products brought down the inflammation. Liam's stomach from the Helicobactor showed no more signs of any infection. The doctors were impressed with how fast Liam's airway looked. They placed him on pulmocort nebs to bring down the inflammation.
Dr. Cotton wants to wait until Liam is a 100% before they do the LTP. Which I strongly agree and respect. I was disappointed but not upset. Because, hopefully Liam will be decannulated in 2003 after his operation. We got to spend the holidays at home and as a family and Liam did not have to spend another Christmas in the ICU.
Dr. Cotton feels Liam need's the stage one reconstruction, and hopefully can be decannulated during his next stay. This can change once Dr. Cotton and his team examine Liam during the surgery and know for sure.
We return to Cinn, Ohio on March 5, 2003 for another Bronchoscopy/laryngoscopy. Laryngotracheoplasty (LTP) Reconstruction is scheduled for March 10, 2003. Three strikes and your out. Not for us, who is keeping score anyway, Liam can have as many strikes as he wants. We are not out until he is trach free.
Liam physical development is still delayed. He is now pushing up to sit on his own, and pulling up on furniture to stand. He has a special walker to get around that is on wheels. His twin, Joseph is showing him around. He is now into everything, and fighting and biting his brother, Joseph. Genetics are still looking into a syndrome. We are now on the "S's"...ha, ha.... (Stingler Syndrome). We have a follow-up appointment in March in Ohio.
He still continues PT, OT, Speech, feeding cognitive teacher, and hearing therapy weekly.
Our lives have not been easy and I do have my bad days, and wish life could be different. But, as I hate to say, God doesn't give you anything you can't handled. Boy is that bull.....(just kidding).
Liam and Joseph are such a joy. I did take things for granted, but now watching Liam fight and work so hard to do the little things, I cherish every little moment. I have learned a great lesson in life these last few years. Help a friend, family or stranger if they are having a hard time. Takes a minute to make a phone call or just give back to someone. We all need a helping hand.....
Update March, April, May 2003: Liam did go as planned with the reconstruction on March 10. Once Dr. Cotton and team stated the operation they noticed that he didn't need the posterior and anterior LTP as planned. His anterior airway looked normal. He received the Posterior LTP with the cartilage graft from his rib, they also placed a stent in his airway for a few weeks. Dr. Cotton mentioned that the subglottic stenosis was caused by intubation when he was 6 months old. The trach remained in placed for safety reason until Dr. Cotton feels he is ready for decannulation. We were in Ohio for about 2 weeks. Liam remained in the hospital for about 6 days. It was very hard to deal with seeing him so sad and not being able to play or even give Mom a smile. However, he did want to eat post-op day 2. Nothing stops him from a good meal.....Follow up in 2 weeks.
We returned to Ohio 2 weeks later for a follow up visit and stent removal. Per the Doctors, Liam's airway still looked of normal size. However there was
a lot of inflammation from the LTP and stent removal. We are to return again in a month.
It is now April and we are back in Ohio again for evaluation of Liam's airway. We hate to see Liam be placed under sedation like this each month, but we hope to see an end to this soon. The Doctors let us know
Liam's airway looked good, but there was a large granulation tissue (scar tissue) that formed where
Liam's stent was placed. They removed that with no problems. Liam had to be watched for any bleeding from the procedure. Again we are to return in a month.
One Month later...
Yes, we are back in Ohio for another Broch. My Mom came with me this time. Liam had the
bronch and again the waiting game for the Doctors to call us in that small room and tell us the good, the bad, or the ugly news.
This time the Doctors had some good news.
Liam did have some new stenosis, but it was soft and Dr. Cotton was able to push it back. He did have some inflammation still from the LTP, but everything else looked good. Dr. Cotton downsized his trach to a 3.0 trach. Liam is able to push more air. He already started making some noise. His sats are at 100% right now. We are back at the Ronald
McDonald House. We have to return to the Hospital to be admitted tomorrow for capping at 9:00
a.m. Either for 24 or 48 hours. So, tomorrow is an important day and we hope Liam is able to tolerate the capping. If he does, he will be sent home with the capping in place, a month to 3 months depends on how the Doctors feel tomorrow. One step at a time.
Well, today is Mother's Day and Liam is still breathing on his own with the cap.......yeah
Capping went well, tried three times and after the third try he was able to breath on his own.
I can't tell you the feeling I had when I placed the cap on his trach hole. I wanted to do it,
because I am his mommy. And when he took his first breath on his own for the first time in two 1/2 years. I felt like I just gave birth all over again. His big blue eyes looked at me and said wow Mom this is cool, I can pass air thru my nose and mouth. The joy I felt and the last two years of taking care of him passed by. My Mom was down getting something to drink and she walked in and I said
"say 'HAPPY BIRTHDAY' to LIAM", she started crying.
He is talking up a storm and can't stop saying DA, DA, ...and he is actually talking back to us. He seems so happy...
However, he did not tolerate it when he is sleeping or taking naps. Can't figure out how to get comfortable. His SATs stay at 94% but he can't relax. He hyperventilated once. The Doctors don't seem too concerned, they say time will correct this, because he still has inflammation from the
Bronch on Thus., and the LTP in March. The nurses say Liam did great with the capping. He sounds loud at times like froggie from the Little rascals...So, his new name is FROGGIE. They say it is strider, and time will correct this.
I am happy with Liam being capped during the day for now, that means no more suctioning and he gets to use his voice. I also got a wake up call when both boys were actually crying at the same time. That has not happened in over 2 years. Gosh. The cap is to be worn all day...he is to cough up his secretions on his own. Only take
it off if he is in distress.
May 20
Liam still cannot tolerate the capping when sleeping. SO the next visit the Doctors need to find out why....Everyday he is pulling out his trach 2 to 10 times a day. So scary. I get so mad at him, but then I want to cheer him on because maybe he is trying to tell me something. "Hey Mom I want this
out, enough is enough". I hope so. I am not going to say this has been easy . I
can explain it as a one way roller coaster of a ride.
Back to Ohio in June.
Thanks everyone.
I know my Aunt Carol and my Dad that passed away had something to do with this. Thanks up there you angels...
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Joseph kissing Liam April 2003
Update, August 2003 - Well today for me was a BIG day. Our equipment company is picking up all of Liam's equipment that he has had here for years. Including 4..... O2 machine pulse ox machine and sooooooo on (ok I did keep one o2 tank). What a feeling it is walking into his room and see a regular 3 year-old bedroom, with no signs of him having a trach. I also moved Joseph into his room because when I was cleaning Liam's room Joseph asked if his bed could be moved in with Liam. They took their first nap together on Sunday, was mixed with so many emotions. I walked into them talking to each other and laughing. For the first time it felt like they were actually twin brothers. I have waited three years for them to be together in the same room. I feel it is the best thing for them because Liam hates to be by himself. Since Sunday Liam has not woke up only once. They both sleep thru the night.
Liam had his bronch on Friday in Ohio. The doctors said his airway is wide open with no signs of scar tissue growing back. He called it a "HAPPY PLACE." The airway is still over-sized, to BIG. They thought he would have something grow back. So, we still have to look for signs of aspiration and in time his airway should go back to regular size. He still sounds like a frog and his voice has not come back yet but it could take up to 6 months. The doc said he will be no opera singer.
He has had one illness with a fevers of 101.9 for a few days and he worked his way thru it with nebs and O2 for one night. I, on the other hand, am still trying to get used to him not being hooked up to equipment. In time I will feel comfortable. It is like he is a new born and I have to listen to every breath he takes.
Liam and Joseph turned 3 on Aug 2, 2003 and we had the biggest party here at our house; trach free party and B-day party. We had a toast with champagne ,(apple cider for the kids) to Liam and all the people that supported us during Liam's 3 years of life. What a roller coaster of a ride, but the ride ended with a miracle and many new friends we picked up on the way. Friends from the Cincinnati, Ohio Hospital and Liams therapist, teacher's from the IU and my trach support group and Liams two nurses that were here since the beginning, Maryanne and Roseanne: WE LOVE YOU. I hope to stay in touch with all of you.
With that I thank each and every one of you and I hope life with us stays on a straight coarse with only small bumps here and there. I wish everyone a miracle someday.
Enjoy the Pictures of Liam. This day was the first day he smiled after the
surgery and he was looking for his trach.......Let the picture do the
talking....CLICK HERE!
June 17, 2003 Update: Liam was off the vent on June 17, 2003 and breathing on his own for the first time in a long time, since Dec 24, 2000 to be exact. The LTP reconstruction surgury went well with a few turns on the way there. He did have the surgery twice, first time was posterior reconstruction, Liam still had his trach, the trach at the time was being used as a stent. It failed after a 5 weeks. We were home in PA, at the time it was 5:45 a.m., when the nurse came knocking at the door I knew something was wrong. So I ran into the room and Liam was lying there, blue and no signs of life. I grabbed him from his bed and started the ABC, placed the trach back in and started CPR immediately. Within a minute or so he started getting his color back, 911 was called. He was sent to A.I. Dupont for an emergency bronch to determined what went wrong. The doctors informed us that he had pulled the trach out and the posterior walls of his airway had collapsed. I was so glad I was there to bring him back into this world. The nurse at the time panic and thought he had a heart attack. The Doctors decided to send him via airplane (Life Flight) back to Cincinnati Children Hospital. That is where we stayed for about 6 weeks.
There it was determined that he was to get the one stage LTP surgery, both anterior and posterior walls needed repair. Again this is where they insert rib cartilage from the rib area and insert in the airway to repair the damage caused by scar tissue. The trach would be removed this time around during the LTP.
It is hard to write about because it was such a difficult time for us. But I want parents that have to go thru this to be prepared for what lies in front of you.
I want to make it clear to all who are getting the LTP it is a hard time for both you and your child. But at the end of it all it was well worth it. The worst part for me and Liam was the withdraw from the drugs he was placed on during the healing process. He was on the vent for 7 days and placed in a drug induced coma, he had to have many sedation drugs to keep him comfortable and quiet. After the 7 days he went through drug withdraw like adults go thru in rehab.
He was hard to control and he was very upset, would not sleep and keep grabbing things that were not there. Was very scary, I can only explain it this way "he looked and acted like a animal with
rabies". So all parents that are getting the LTP be prepared for the drug withdraw. Liam also had to be placed back on the vent few days later for
pulmonary edema, fluids in the lungs. He was back in ICU for a few days, very scary time. But like I said this was the best decision we could of had made to get Liam where he needed to be and we felt he was ready at that time to have this LTP done. For us Liam made the decision for us because he was trying to kill himself by pulling out the trach 20 times a day and he would stop breathing because the scar tissue was collapsing around his airway. We thank Dr. Cotton and team so much, he saved our son and gave Liam a chance to breath on his own. Gave Liam a chance to be able to smell food and taste food, the simple things in life many take for granted.
October 2004: Liam is doing well with some concerns at this time.
He has had recurring respiratory distress problems due to aspiration pneumonia and sinus and ear infection. He was placed on a vent 2 times this year for respiratory concerns. His airway looks good and no concerns right now with scar tissue. The concerns now are his lungs and infection. All his food and drinks have to be thicken, so he will not aspirate. He keeps getting recurring ear infection.
They are retesting him for MPS. They say he wasn't tested for all of them before. I dropped Liam's Urine sample to be tested for MPS on October 13, 2004. He is being tested again because the doctors feel he has most of the characterized for this syndrome. The test before could test negative which really is positive....very confusing.... His test is being sent to a Specialist in Arizona. Mucopolysaccharidosis has 7 types, is an inherited disorder involved in the breakdown of complex carbohydrates called mucopolysaccharides. It accumulate in the organs and tissues of the body and become toxic. It is a rare disease and the lift span rages from 5 years to 20's depending on which of the 7 MPS's he may have.....which we hope is none.
Please say a prayer and keep him in your thoughts. Hopefully this test will stop the
doctor's from looking at this particular syndrome, and we can move on to finding a syndrome that Liam has.
Liam is 4 years old now, he still does not walk. Liam height is still 4 1/2 inches shorter then his twin brother, Joseph. He is using sign language to communicate. His voice had not fully come back yet. The
doctors say give it time, one of the vocal cords is higher then the other so his vocal cords aren't working at a 100%.
But I pray daily that he will be able to speak one day. I would love to hear the words from his voice telling me he loves me. But for now the hugs and kisses he gives me is enough. He is a very affection 4 year old. He loves to smile and give hugs by pulling you in close and lying his head on your shoulder. I won't lie to you it is hard dealing with the everyday concerns any special needs child may have, and I worry about his future. But for now I try and stay focus on his everyday process and giving Liam what he needs, and that is "LOVE".
He goes to school 4 day a week now and gets all his therapy's from ARC school in West Chester. It is a great program and they give Liam the education and special care he needs. He still wears glasses and his hearing aids and is using a walker to get around at times.
Thanks again for this website, a few families have contacted me on the LTP procedure and in reference to Dr. Cotton. I feel good about helping other families in the same situation. Will update this page in time.
Kathleen Hilferty @ twins82000@aol.com
