When Trek was first diagnosed with Niemann Pick Type A, we spent hours online trying to find out about the disease. We had never heard of it before. The doctors knew very little, and there was precious little online. Most of what we did learn about NPA was from other parents’ online blogs of their babies.
I tried to reach out a little to other NPA families, and the response was overwhelming, they all were there to help and for a shoulder to cry on. But I remember not being able to write them back for a long time. I stopped reading all the blogs, I had all the information I could handle.
I could not read anymore, or think about NPA for many weeks in the beginning of our travels with Trek. I just focused on him, his life, his death, but not on his illness. In a way I still do that. I feel very angry towards NPA, and we cuss it out often in our home. I rarely say the words in relation to Trek, it hurts too much, or I feel like it gives it power or something. I am not sure, I just hate it.
I remember one day Shannon (Wylder’s mom-another NPA baby) sent me a picture of a butterfly that her and Wylder grew from a caterpillar, and then released. They had named him Trek. It touched me so deeply, and I was able to read more about NPA babies again.
The first blog I read when we first found out about Trek’s diagnosis was Mia. Her parents wrote only one blog post about her, but it was the most powerful and helpful post I have ever read. Even though all of the other websites, youtube videos, and facebook pages had all the same information in the archives somewhere. Mia’s blog only has one page. That was all I could handle reading at the time, and it answered so many of my questions.
When I found out about Quinn the other day it was just awful. Just awful for us, and even more awful for them. I was crushed with a new weight-there were going to be more Niemann Pick Type A babies. I could not stop thinking about her and talking about her. I talk to Jarrett about a lot of things, and a lot of people, and he rarely knows who I am talking about (ha!). I have 3 close friends named Shannon, and he is always like-which Shannon are you talking about this time? I told Jarrett the first time I found out about Quinn. I need you to remember these 2 names-Eileen and Quinn, I am going to be talking about them a lot and please don’t ever ask me who I am talking about. He was so sweet and of course did not even need the push, he was affected a lot too when we found out about Quinn.
I should have known it was coming. I can see on the website what google searches lead people to my page. It is usually some version of “oursonnylife” or “Trek Ingram”, but for a week the top searches were “Niemann Pick Type A”. Someone else was diagnosed and was scouring the internet looking for the little information that is out there. That is what made me want to do this post, for that next person who is diagnosed with NPA, so they will have one spot to go to and get some info on this site. So they do not have to search thru my archives if they are not able too, and can just get the abridged version of the more medical side of Trek’s illness, which I do not usually like to write about. To add to Mia’s amazing story in one page.
I wrote this post 4 months ago. I have been scared to death to publish it, I did one time and then trashed it a few minutes later. The medical side of a disease is not easy to write about and not something I like to remember in relation to Trek. But it was such a big part of his life. I feel vulnerable, as I am sure every parent does when they have to make decisions for their children who have no say in them. But this is what we chose for Trek, we listened to him the best that we could, and loved him with our whole heart.
So here is Trek’s NPA timeline-
Trek Atlas Ingram-Niemann Pick Type A timeline
birth: April 11, 2011-death: June 21, 2012
Trek was diagnosed at 9 months old after 2 days of doctors visits, so before that time we had no clue he was sick. From birth to 9 months, I can see some things looking back that were symptoms of NPA, but at the time I attributed them to other factors or he was just a late bloomer.
April 11, 2011-born healthy at 8lbs 5 oz in the driveway of our Maui home:)
Birth-3 months old:
We lived in Maui and I stayed home with him and his big brothers. I stayed at the house a lot during those 3 months just snuggling him on the couch, and swinging with him on the porch swing. I thought he might be my last baby so I was really soaking him up.
Trek did everything normal and was growing and chubby. He was only breastfed, he never took a bottle, we tried once so I could go out for a girls night, but he would have nothing to do with it.
cuddled in his favorite sling getting a pedicure with mama and Nana
He slept in bed with us and nursed all night long and was a great sleeper. He would get startled very easily by sirens and cry and be very upset. We lived by the fire dept. and every time a fire truck would go by with it’s siren I would cover his ears. Even if he was asleep I would run back to the room and cover his ears while he slept. I now know that can be a symptom of NPA, sensitivity to sounds.
Trek started throwing up when he was 6 weeks old. It usually was once or twice a day, sometimes more. I took him to see our family doctor. My other 2 sons and I both have dairy allergies, so we thought that he might have a dairy allergy. I cut all dairy out of my diet so he would not get any through my breast milk. That seemed to help a little, but sometimes I would have some dairy, so if he threw up I attributed it to that. If it was a whole feeding, I would just nurse him again and he would always keep the second feeding down. Most of the time it was just a big spit up, it was just pretty often and more than most babies. This is one of the biggest symptoms of NPA, feeding problems.
3 months-6 months:
Trek hated to be on his tummy so I never made him. I think I have one picture of him on his tummy. I would always lay him on his back to play, he preferred that. I am guessing his liver was enlarged and it was uncomfortable.
I did not know that Trek’s liver was enlarged. Trek only went to his 2 week check up at the doctors for his PKU test. We never had Trek vaccinated or circumcised, and he was so happy so we never had a reason to go back. We also lived on the island of Maui, very disease free and fresh, so we did not go to the doctor a lot. I did take him to our family doctor twice for his throwing up, but we just discussed allergies since that is why I brought him in and we treated him for that. Looking back now, I found a photo of him from 6 months old in his diaper, I can see his liver protruding a bit from his little chubby tummy.
6 months old-
He still had a hard time holding his head up for long periods of time, but I carried him constantly in a baby carrier, so we assumed he was just super chill and had not been given the chance to work all of his muscles. We started putting him down more, but he HATED it. He would cry within 1 minute of being put in a bouncer or on a play mat, so I would just scoop him back up and snuggle him. He was so happy and smiley in my arms I found no reason to make him be any other way. It never occurred to me that he was sick.
When we would try to put him down to sit up he would topple right over, he could not even balance himself for a second. We thought that he was just a late bloomer, and was not ready to sit up yet since he was held all of the time.
He also stopped sitting in his car seat most of the time around this age of 6 months. We lived on Maui and we did not drive very much, but when we did I would sit in the back and hold Trek. He would sob when he was put in his car seat in a way that was different from when Peyton and Conner just did not like their seats. I was obviously very worried about getting in a wreck or being pulled over, but he was so upset and I just couldn’t do it. Now I know that he was very uncomfortable in any sort of sitting position because of his liver being smooshed, and he was very sensitive, so any time out of my arms was devastating and frightening for him. I also always felt like it hurt him to sit on his butt for some reason, maybe because it did not have a lot of cushion on it.
not his favorite place:)
One day Jarrett came home and asked me what I did all day, and I said, I held Trek. He said, did you ever put him down? I said, No, I can’t explain it, but when I do it feels like he thinks he is going to die. It was different with him than with the other boys. They loved for me to hold them, but I could distract them for a minute or give them one more toy to hold them off while I went to go pee or finished dinner, but with Trek I couldn’t do that. It was like he couldn’t bear to be away and he was devastated when he was not in my arms. I just took him with me. I just thought he was my little lover. Actually he was, even though NPA caused so many of his symptoms I went 9 months falling in love with those symptoms thinking they were just who my baby was, never thinking about it negatively for one moment. It was who Trek was no matter what the cause.
He was still nursing all day and all night about every 30 minutes. I did not think much about it. His brothers loved to nurse too, but I was also busy with homeschooling Peyton and Conner and playing with them I did not think too hard about how much Trek was eating. I thought if he was hungry then I would feed him.
Trek was still throwing up about 3-4 times a day, so we took him back to the doctor. If I was really good about my dairy intake it would help a little bit, so I continued to believe it was that and so did the doctor. The throwing up never bothered him, it was just like a big spit-up and he would not cry, just spit up some milk and then want to nurse again. We just got used to it and hoped he would grow out of it like his brothers did with their dairy allergy.
Trek had no teeth and never got any the entire 14 months he was alive.
Trek was so smiley and would laugh so hard any time his brothers were around. He was so fun to play with and so sweet, but he still only liked it when I held him. I just thought I was spoiling him rotten and I did not care one bit. Jarrett and my sister Amanda could hold him, but for the most part he did not really like it when other people did.He started to look a little smaller so we decided to try him on solid foods, even though he showed no interest. I had waited until he made any sign he was ready or had a tooth, but neither of those ever came so we decided to try it to help him gain weight.
He was 8 months old, and I bought a jar of organic apple baby food. I put some on my finger and put it in his mouth. He took a few bites, but that was all. He turned his face away after that. That was the only bites of food he ever had. He only breastfed the entire 14 months of his life. He was still throwing up, somedays he would only throw up 1 time, and sometimes it could be as many as 4 times a day, some big, some more of a spit up.
his first and only food
We had 2 friends who had babies born a couple months after Trek, and I started to notice that they could do things that Trek could not, and he was older than them. He then all of a sudden seemed a little small for his age, it seemed to happen overnight, like maybe 2 weeks he lost weight (he had been average size and chubby for the first 7 months of his life). I remember that day. Our friends had just come to play, and their chubby baby made Trek not seem so chubby anymore. After they left I sat on the bed looking at Trek and crying, I think I knew then, at least something.
It was Christmas 2011, and we were busy selling all of our belongings and planning our big move to Thailand and having fun with holiday festivities. Even though our lives were full with planning and fun, Jarrett and I started to notice more and more how Trek still seemed like a newborn and was getting smaller. We scheduled an appointment with our family doctor on Dec. 28. He looked at Trek and told us he thought it was a genetic disease. He had just seen him a few weeks before, and we were talking about allergies. But Trek had changed in such a way that he knew when he saw him too-just as we had feared.
Even as we drove away to the hospital though, I was certain he had some sort of severe acid reflux, I clung to that. I still did not think too much about him wanting to be held all of the time, I just thought he was a major snuggler (well, he totally was:)
We went from Maui to Oahu where they also thought he had some sort of metabolic or genetic disorder. We were crushed. We believed them, even after just a few hours, all of the symptoms fit. But we needed more information before we went down that road. They wanted to start on MRI’s and other major tests, but they could not give us much information. There are so many metabolic and genetic disorders, and they are all so rare.
Trek was so weak and despondent already from his fluid IV. I knew he could not take being away from me or any sort of pain. We ended up leaving Oahu after they told us it would take weeks of tests to discover his disorder because they did not have all the latest technology, being a hospital in the middle of the ocean.
We left the next day to take Trek to Denver Children’s hospital. We had Oahu call ahead and transfer all his paperwork.
happy and perfect at Christmas time
We got to Denver Children’s Hospital and they admitted him into the hospital for tests to diagnose him. They also believed he had a metabolic or genetic disorder. Before we got our room, and just a couple hours of meeting with doctors, they told us it was serious. That the best case scenario was that he would need lifelong treatment. They also told me that me nursing him every 30 minutes his entire life was what kept him alive and chubby for so long. It made me happy that I had listened to him, but sad that his life balance was that sensitive.
They scheduled him an MRI appointment for that afternoon. I was devastated. He would have to stop nursing for hours before the test and I knew that would be the hardest thing he had ever had to do. Right before his MRI an eye doctor came in to see if he had a cherry red spot in his eye. We asked if he could not use the speculum for the test, and he was able to do the exam by me holding Trek’s eyelids open. A cherry red spot is a red spot in the back of the eye that is surrounded by fatty tissue accumulated from a metabolic disorder. It is very rare, the doctor had never seen one in person, but he saw it in Trek’s big blue eyes.
The doctor lowered his eye scope and told us and we all knew what it meant-Trek was going to die. The eye doctor left really sad, Jarrett went into the bathroom and sobbed, and I just stood there clinging to beautiful Trek. That moment is frozen in time for me, I didn’t cry, I just sat there and felt my entire life disappear. I knew my life was over.
We cancelled his MRI. Four amazing genetic and metabolic specialists came in and told us it was Niemann Pick Type A. Trek only had months left to live, there was no cure or treatment. Children with NPA lived from 18 months-3 years old. We all decided that we should go home and come back in a couple of days to start working on some ways for Trek to gain weight.
He had only had 3 blood draws and 2 fluid IVs in his 2 days in the hospital, and that almost took everything out of him. He would not let Jarrett or anyone else hold him for weeks after his hospital stay and he lost much of his vigor and never got it back. I knew he was sensitive, but this really drove it home for me. I knew we would have to seriously consider any treatments options for him before doing them. Jarrett needed to be able to hold his baby, and Trek needed to be happy and not regress so quickly.happy to be out of the hospital and playing with his toys!
9 months old-trying to decide the best treatment plan
We spent the next 3 weeks reading, researching, going to every kind of doctor imaginable, and soul-searching. The plan from the metabolic clinic was to have a surgery to get Trek a feeding tube (g-tube) to help with is vomiting, and to prevent future feeding issues. But they also were open to anything we wanted to do, they were so sensitive. One doctor suggested us to get all of Trek’s vaccines, and to keep him indoors in a sterile environment so he would not get sick. They also strongly pushed pain medicine. We met with a palliative care team for hospice for Trek called the Butterfly Clinic. Every baby is different and has different needs. We just had to listen to Trek to see what he needed. It was hard, such a hard time for any parent of children with special needs.
The medical side of a child disease is not pretty. I hate writing about it, but it is the truth. No matter what a parent decides, to use medical interventions or none at all, there are rewards and risks and it is an impossible choice.
They were so amazing and we got a nurse for Trek that would be on call 24 hours a day for anything we would need. Our nurse, Terri, was so kind and very supportive of our decisions. She and another doctor in the genetic clinic were a big reason we felt so comfortable not getting Trek a g-tube. We all thought that Trek had a very severe case of NPA, and that he may not survive the surgery. They also thought that we did not have very long with him, that he would live on the shorter end of the NPA timeline. During this time we signed a DNR for Trek. It was surreal, just days before I was imagining how cute Trek would look walking, and now I was signing a paper to let him die.We tried to get Trek to take high calorie formula to gain weight. We tried a bottle, but he was not interested at all. It was not so much the nipple but the taste of the formula, even when I tried to sneak it in by dropper while he was breastfeeding he would gag and throw up. We even bought one of these supplemental nursing systems, but he would not nurse at all when I used it. After a few days of trying different ways and formulas we decided to not force it. He was throwing up more formula than he was keeping down, so we just let him be happy and breastfeed.
After a few days of meeting with doctors, Jarrett and I felt ourselves moving more and more towards pain management. The hospital had already given us some liquid morphine and we saw an immediate improvement in Trek. He was smiling more and so much more comfortable. And he was starting to let Jarrett hold him again when he would take it!
We even went through the process to get Trek his medical marijuana card in Colorado to help with pain management. They made Trek a very special medicine used for Cancer patients, and formulated just for a baby with his symptoms. In the end it did not help, it was not strong enough and he had to take too much volume and would throw up.
look at this happy boy picking up his medical marijuana;)
Even though Trek was only 9 months old, Niemann Pick type A was already causing him a lot of discomfort, so we started giving him 3-4 drops of morphine by mouth a few times a day. He was also given a perscription of Oxycodone, but he had to take too much volume, and he would gag and throw it up. Morphine was a small amount of liquid, so he would not throw it up, most would absorb under his tongue. He was so happy and content when he was on pain medicine. If I even got behind for an hour he would be fussy and uncomfortable, so it became our priority.
Just seeing how much was taken from Trek from his few pokes in the hospital we knew he would not be able to handle a g-tube surgery. We were scared what that might take from him and if it would present other issues for him. We also talked to Dr. Wasserstein who is an expert on Niemann Pick Type A. She assured us that we were not the first parents to decline medical intervention. It was one of the most powerful moments of my life when I read those words. It gave me the peace I needed to move forward in confidence.playing with a little bird toy and under a sign that friends made him
We also had already named our sweet boy Trek Atlas because we wanted to travel the world with him and Peyton and Conner, so we were starting to think about ways we could still do that.
We decided that even if he got sick and died earlier than planned, we would rather him be out exploring the world instead of at home in a sterile environment. It was a very personal choice that took a lot of soul-searching. Many tears and sleepless nights led us to that choice.
9 months-12 months:
We spent the next 3 months traveling in Central America and keeping a close eye on Trek in case we needed to get him back to the hospital. The doctors were available by phone 24 hours a day and shipped us pain medicine perscriptions. He was still throwing up, but nursing like a champ every 20 minutes.in Nicaragua
I held him non-stop even when he was sleeping at night, he slept in my arms. He was frantic if I was away or not holding him, so I happily snuggled him. I was not able to give Peyton and Conner very much it was so hard not being able to be there for Peyton and Conner like I used to. Thankfully Jarrett had already quit his job for us to travel, before Trek was diagnosed, so that helped so much.his favorite thing to do, breastfeed:)
Trek needed a few drops of morphine on his tongue about every 2 hours. He was still smiling and laughing and could hold a toy, the only thing that was noticeable about his disease was his size and frailty.
12 months-14 months:
Trek still had not hit the downward spiral that the doctors had warned us about, even though we felt like it was getting close. They had said that one day he would start pulling away from his environment and start to have more medical problems. He was still smiley and happy and coherent, even though he did not laugh anymore. He was just more subdued. He was only happy being snuggled on my shoulder, so he lived there, sweetest snuggle bug ever!
His stomach area was very sensitive because of his enlarged liver and spleen so we held him very carefully. His feet were also very sensitive and ticklish or maybe it was painful, so we never touched them. He also became more sensitive to sounds and people being in his face. He used to love it when his brother Conner would get in his face and make him smile or laugh. But now he would cry or turn his face away if anyone got too close or loud by him.
in Hawaii, even though he was still losing weight he was so happy and smiley
We decided to take the plunge and take Trek to Thailand. We knew it was a 1-way trip. We had been traveling close to America in case he needed medical care, but we saw that he did not have much longer and we wanted him to go to Thailand. We took a break from travels to visit friends and family in America. Jarrett and I noticed a small change in Trek when we were not exploring. He wasn’t quite as full of life. He seemed to really enjoy the energy of travel, so we decided to keep going. All he did was lay on my shoulder and watch the amazing things pass by us, he was so happy and never had a reason to cry. We hoped that he would pass peacefully before he hit his downward spiral or any major medical conditions.Trek’s happy place-his favorite way to be held all day long
We got a 3-year supply of morphine for Trek and got the blessing from all of his doctors to do his medical care over the phone. We bought one-way tickets to Thailand.
Right before we left for Thailand, I noticed purple pin spots on Trek’s legs. I looked it up and Idiopathic thrombocytopenic purpura (ITP) came up. I called his hospice nurse and his metabolic doctor, they agreed that it sounded like that too. His doctor said it was because of his enlarged spleen over working and his blood was not clotting properly. Trek was bleeding inside of his body. All I worried about was if it was hurting him, she said that it does not hurt.
I was on the phone with Terri, our hospice nurse, and I sobbed. It was the first time I really cried in front of someone about Trek besides Jarrett. She told me that Trek did not have as long to live as we had hoped, and to start preparing for his death. I did not tell anyone besides Jarrett and my sister, I was devastated. Even though I knew Trek was going to die, we had hoped he would live until he was 16-18 months old, I needed those extra months. It also was so different to know it was coming, and then for it to actually be there.
We decided to still leave for Thailand knowing that he would die there. We had thought long and hard about it, and we wanted it to just be us 5 when he passed away, and we didn’t want to have a funeral.
June 21, 2012
the day before Trek died at the beach in Thailand
Trek had been doing great since his ITP had come up 2 weeks before, he was smiling, holding a toy, snuggling, letting Jarrett hold him and being himself.
The purple spots did not go away on his legs (the doctor said they might) and his liver seemed to be more sensitive. He was still throwing up and very skinny, since he had continued to lose weight since his diagnosis 6 months before. He was still getting morphine every 2 hours.
On the night before he died, his throw up had blood spots in it, and throwing up seemed to hurt him for the first time. We stayed up all night with him giving him morphine and holding him.
On the morning of the day he died, he was back to his old self, smiling and comfortable. I think all of the morphine caught up to him after he threw so much of it up. Mid-morning he had a seizure, the first one he ever had. It was scary, but I knew it was a part of NPA, so we just held him and kissed him. It lasted 5 minutes. He came out of it and was happy again and nursed. Then another one started , this time I was worried he was going to die. We all came in and told him goodbye. We were all crying, then it stopped and we all laughed as he looked up at us and smiled.
3 hours later he had another one. He came out of it and breastfed for a minute and smiled. Then he started to fade in and out. We all told him our goodbyes and sang him his favorite song-Trek wins the honey pot from Winnie the Pooh. Right before he left his body he smiled, he just left. No pain, no body reactions, he just closed his eyes and his body went limp and I could tell he was not there anymore. It was the worst moment of my life and I would trade anything to have my baby back. No one should have to hold their dead child in their arms.
I wish that no one ever had to read this because their baby is about to go through the same thing. If you are reading this because your baby was just diagnosed with NPA, I am so sorry. I am so very, very sorry and my heart is broken in a million pieces for you. I am here for you if you ever need anything.
Love the mama of the sweetest little boy-Trek Atlas