Dear Doc

Katie Pasque, MD
Clinical Instructor
Department of Obstetrics and Gynecology
University of Michigan
Briarwood Center for Women, Children, and Young Adults
Building 2, Suite B
400 East Eisenhower Parkway
Ann Arbor, MI 48108

Dr. Neurodevelopmental Specialist
Helen DeVos Children’s Hospital
Neurodevelopmental Pediatrics
426 Michigan Ave NE Ste 102
Grand Rapids, MI 49503

Dear Neurodevelopmental Pediatrician at the only Intensive Feeding Program in Michigan:

I am writing to you directly because I am getting nowhere by trying to go through the suggested channels. I have been trying to get my son Lively placed on the waiting list for evaluation at your program for one year now. I have spoken to your coordinating nurse at least 4 times. I have left many voicemail messages that were not returned. Last week, the nurse from your program promised that she was faxing a referral form to my pediatrician, Dr. XXXXXXX. Dr. XXXXXXX has diligently followed through on multiple referrals to every specialist imaginable for Lively, including calling your program to try to make a referral himself. Still, we have not been successful in scheduling an evaluation. I am contacting you to ask for your help in having Lively seen by your group.

My son just turned 2 on July 15. At 6 months he started having seizures and was diagnosed with infantile spasms. An MRI showed a right parieto-occipital stroke. He was treated with ACTH for 3 months. He breastfed exclusively for 6 months without problems, and at 6 months when we introduced solids, and also started ACTH, he was ravenously hungry and ate big bowls of mashed potatoes with broccoli and peas. He ate until he was so puffy that I was afraid his skin was going to burst. At 9 months he stopped eating and drinking one day, for no reason anyone can understand. He was admitted to the hospital for dehydration, and developed diarrhea and vomiting. During one vomiting episode, he was on his back and obviously aspirated. He had breathing distress immediately and within a few hours was intubated. ENT scoped him and diagnosed tracheomalacia. After 24 hours, he was extubated, and immediately failed and had to be re-intubated for a week. When he finally came off the ventilator, he refused to breastfeed for days, and when he finally tried, he obviously aspirated. A swallow study confirmed this. An NG tube was placed, but he vomited with every feed, so the tube was advanced to an NJ. He tolerated continuous breastmilk feeds. At 11 months, his NG tube was misplaced (at home, by me) into his trachea. He had a pneumohemothorax that required surgery. Again he was intubated and failed extubation after 24 hours. His airway swelled, and it took the doctors more than 30 minutes to replace his ET tube. During that time he was difficult to ventilate, and he became very hypotensive. He required the ventilator again for a week. Later during that long hospitalization, he got a GJ tube.

After Lively came home again with us, my husband and I were able to transition him from continuous GJ feeds to bolus G-tube feeds over the course of many months. We slowly introduced pureed foods and thickened liquids. Today, a year later, he eats stage 2 purees, and occasionally some soft stage 3 foods. He is able to drink water from a sippy cup. He does not know how to chew, and when we offer solids, he holds them in his mouth and eventually spits them out. His OTs believe that he is not able to coordinate chewing to push foods between his teeth or to form a bolus that he can swallow. He receives around half his calories overnight through the G-tube.

My son’s other medical problems include global delays, likely due to his stroke. His seizures are under control with Keppra, and his EEG no longer shows hypsarrythmia, and continues to look more normal each time. He started walking at 19 months and is steadily gaining strength in his left side. He has fine motor delays, but they are improving with therapy. His cognition appears to be improving as well, as he is increasingly imitating, referencing, and trying to sign and speak. Nevertheless, he meets criteria for autism, and received this diagnosis in April, at age 21 months. He is a very happy and engaged little boy, and although my husband and I understand that he qualifies for the autism diagnosis, we have hope that he will continue to improve so that he no longer carries this diagnosis.

With regard to his feeding, we have hit a wall. We cannot get more than 50% of his calories into him by mouth. He is showing no improvement with his ability to tolerate solids.

I know that my son would not have received half of the care that has helped him if I had not been proactive in getting him the appointments that he needs. I have tried to go through the “regular” channels, but almost always, that has failed me, both at U of M, and now with your center. So I am asking you to please help my son be seen by someone who might help him. We have exhausted our resources here, and need your help.

Please contact me with any questions or advice. I look forward to hearing from you.


Sincerely,
Katie Pasque

To my most special boy, on his birthday

Dear Lively,

You are two!

You started your second year in the hospital, just 2 floors above the place you were born. You were hospitalized 5 times in your first year, but happily only once during your second, and you have managed to stay healthy enough that we have stayed out for 11 months now.

This is a picture of you on your first birthday. We spent it in the hospital. You had presents and cakes, and I gave you a little bit of icing on your lips because you weren't eating at all at that time. I think you liked it though!

Seeing these photos of you makes it feel even more surprising to me that a whole year has passed. You were so different then, in so many ways. One year ago you could not sit up. You basically did not eat at all. You had a G-J tube, which meant either I had to dress you in button down shirts, or I had to cut holes in your shirts so the tube could come through. We fed your intestine directly, and you weren't able to tolerate much more than a dribble, so we pumped feeds into you continuously.

This is a photo from your last readmission to the hospital, when you were 12 1/2 months old. Even though you had the G-J tube, your doctors placed an N-G tube as well to drain your stomach because you were vomiting so frequently. I fought this because your last N-G, placed by me, led to hydropneumothorax, surgery, intubation, failed extubation, and a very scary week in the PICU. In this picture, you are in the hospital, but not in the ICU. We waited, you received hydration through your tube, you got better, and we went home after about a week.

When you recovered enough to go home, you looked like this:

You were still so chubby due to all those months of ACTH. You were pretty uncomfortable, because of the new tube sticking out of your tummy, and also, I think, because you had so many rolls on you that it was hard for you to move around. You just didn't feel well at all. You were taking about a million medications, and you vomited several times each night.

Daddy and I slowly transitioned your feeds from your intestine to your stomach as your digestion improved. We weren't able to find a doctor or therapist who understood what was happening to you, or who could give us suggestions to help you improve, so I got most advice from other parents on the internet whose children were going through similar situations. In early September, when we had completely transitioned to stomach feeds, the big tube was replaced with a much more discrete G-button.

Despite being told that you were aspirating on a swallow study and that we should limit feeds to your G-tube, Pete and I felt you were making no progress in overcoming your oral aversion or in learning to eat, and so we started to feed you by mouth. We gave you thickened, pureed foods, and you spit out 100% for months. We would sit with you for hours at a time each meal, trying to convince you to swallow. You didn't for several months. And you looked like this when  you tried:

Beautiful!

By fall 2010 you were able to sit. You were even able to slide (with help)!

In this picture, you were actually well on your way to slimming down, although it's a little hard to tell. Look at the rolls behind your knees!

During all of this time, you were as weak as a kitten. You tired easily. We went to physical therapy 2-3 days a week. You learned to sit. And kneel. This is a post-sit, pre-crawl photo of you from October 2010.

By mid-November, right around 16 months, you were finally strong enough to crawl. As you developed, we also noticed more obvious weakness on your left side when compared with your right. None of these things are apparent in the following photo, which was taken in November 2010. You look upright and steady.

Your progress through the winter months was slow, but we felt we were heading in the right direction. You strengthened the muscles in your arms and legs. And you learned how to be a snow-dino.

Although you still struggled with food and drink, you learned pretty quickly how to eat snow.

In March, when you were 19 1/2 months, we finally celebrated your first steps. You worked so hard for them! We spent countless hours of physical therapy (not to mention 4 or 5 other kinds of therapy) trying to get to this milestone. When I watched you walk for the first time, I felt a weight lift that I hadn't even known I'd been carrying.

As soon as you started walking, you immediately started to lose your baby weight (and your ACTH weight). By Easter, you looked like this:

You started to eat more and more, but not enough. You were able to take in about half of your calories by mouth, and for the other half, we ran formula through your tube overnight. We realized that you didn't know how to chew, and so we added occupational therapy sessions to try to help. You don't seem to understand how to coordinate using your teeth and moving food around your mouth in order to swallow. Today, you continue only to be able to take in half your nutritional needs by mouth, and you are able to eat only soft or pureed foods.

In April, at 21 months old, you were diagnosed with autism. The worry I had lost when you walked resettled and brought friends. We don't know what to think about this diagnosis. We aren't sure what it means for you now, or what it will mean for you as you grow. The uncertainty is hard on me. I try to stay positive for you, and your happy disposition and smiling face make it easy to do when I am with you. You learn something new every day. This is a picture I took in May of you practicing your dancing:



I look at these photos of you, and still wonder if the autism diagnosis is correct.

Today was a bittersweet day for me, and for Daddy too. You are two! You walk! You eat (some)! You drink (a little)! You are snuggly and loving and happy. But today, when I went to the grocery to try to buy a gluten-free casein-free cake for you (I was sure Whole Foods would have something, but they didn't), I couldn't help but think, the life I planned for you included cake and ice cream. It didn't include g-tubes, intensive early intervention, feeding programs, autism studies, hospitalizations, and seizure medication. Tonight, we ate ice cream bars for dessert (you had a chocolate-covered coconut milk bar) instead of cake, and no one seemed to mind.

My wish for your second birthday is that when we have to make compromises in the future, it be no harder than replacing cake with coconut milk bars.

Happy birthday, my love. May you continue to be as happy as you are today, despite the challenges you face, and those to come. You are my sunshine.

Love,

Mama 

Vacation

I was off work last week (Pete is off for the summer) and since we just moved, we spent the week unpacking, at least a little, and generally trying to get settled. Lively had several appointments, including his new Early On Young Twos group, which is now twice a week, and I spent hours on the phone trying to coordinate speech therapy (I've been fighting with insurance and for new approval for this since March) and looking into intensive feeding programs. More on this another day, but suffice it to say, I'm beyond frustrated. However, Pete and I both got to spend a lot more time with Lively this week, and we really noticed what we are hoping is developmental progress. Here are the things I can remember right now:

1. Lots of uh ohs, used appropriately, or, perhaps, a bit sarcastically. (Ex: Lively throws spoon full of oatmeal on the floor. "Uh oh!")
2. More repetition. This morning as I was telling Pete that I put applesauce in Lively's cereal, he said, "a-poo" several times.
3. He has been signing "eat" appropriately consistently! If he's hungry, he'll walk to us and sign "eat" by holding the tips of his fingers to his mouth. This is a huge step in his language development: he wants something, he formulates a word in sign language (or sometimes verbally), and then uses it correctly. For his other words, check out my list on the right side of the page.
4. Lively has learned to flip the light switch on and off with one finger. Pointing is a skill that has not come naturally, and we have worked on it endlessly. The work continues. But he loves to flip the switch, and he does it with one finger.
5. When he wants food that's out of his reach, or a toy, he reaches for it instead of throwing himself on the floor and having a tantrum. We still have plenty of tantrums, but he's figured out that he can reach toward something to show us, "I want that." Most 9 months old do this, but Lively just learned it.
6. We've taken him out in public, at times to very crowded places, and he's done generally well. Pete took him to the last night of Top of the Park (part of Ann Arbor's summer festival) tonight, and it sounds like he only had the normal 2-year-old kind of tantrum (when he wanted to go in one direction and Pete needed him to go in another). Yesterday we took both kids to the Ann Arbor Farmers' Market, and he did great! It was so crowded we could barely make our way through, but Lively rode happily in the stroller with Annie and calmly took in all of it. A woman even walked up to him and baby-talked to him about how cute he is (she is so right!) and he gave her a winning smile.
7. We've also had friends over several times in the last week or so, and Lively has given lots of high fives, hugs, and has even allowed himself to be held for around 5 seconds by someone other than Pete or me. When kid-friends come over, he is delighted and follows/chases them. He wants to play!
8. Lively is babbling a lot! Sometimes he sounds like he is speaking another language that I don't understand fluently. He may be. He's speaking Lively.
9. A couple days ago when I was giving Lively a bath, I said, "Let me put bubbles on your foot," and he held his foot out of the water for me! He knows where his foot is! He knows what his foot is!
10. He let me give him a haircut. Sort of. This involved me sneaking up on him while he was playing in the sink and getting approximately 3 snips, following him around the house for about an hour (4 more snips), and then Pete came home and held him and distracted him so I could complete the "cut."
11. He's been singing! Well, humming. While I was driving him home from Early On, I heard humming coming from the back seat. I hummed with him, 2 or 3 notes at a time, and he imitated the tune.
12. He has been finding my shoes and stepping into them and walking around the house, saying and signing "shoes."
13. Yesterday Lively picked up my water bottle, pretended to drink out of it, and then pretended to feed me with it. This is called associative play, and we haven't seen a lot of it. It's a developmental milestone, so hopefully we'll see more.

It's been a great week.

Be vewy vewy quiet, I'm hunting wabbits.

We moved into our new house last month, and a family of bunnies moved in under our deck. They're very cute. There are little baby bunnies everywhere. And Annie loves them. She sees them and gets very excited, and wants to hold them so badly that she gets a little upset when they hop away.

Tonight Annie and her friend Alicia were playing in our back yard, and Annie decided they should go find the bunnies. The girls (4 years old and almost 4) had been running wildly around the yard, and so I suggested that they move quietly so that they might not startle and scare away any nearby bunnies. Annie went around to all of the adults with her finger to her lips, "Shhhhh. We'w going to look fow bunnies." Lively wanted to play with the girls so much, and was following them around, and after Annie starting shushing all of us, he turned to me with a big smile on his face, and put his index finger next to his mouth. "Shhhhh."

Here's a picture of Pete and Lively from this weekend. I like dressing them alike!

Socks

This story is per Pete's account:

This morning, Pete went to get Lively out of bed, and as soon as he lifted Lively out of his crib, Lively looked over to his dresser, pointed, and said, "Ssssaah." Pete put Lively down on the floor, and Lively walked over to the dresser, picked up the socks on top, then walked back to Pete, handed him the socks, and sat in Pete's lap. He then signed socks, as he said "Ssssah." Pete held the sock open, and Lively happily put his foot into it, signing "socks" over and over.

We've practiced the sign for socks before, awhile ago, but not lately, and I haven't ever seen Lively do the sign before today, especially unprompted.

When it was time for nap, I attempted to remove Lively's socks (it was 90 degrees outside, and not a lot cooler in his bedroom), and Lively expressed his dissent, and repeated the socks sign. I left them on. And gave him about a million kisses.

Today's other fun tidbit:

We sang, "If you're happy and you know it" with Lively today out on our porch, and Lively danced around delightedly for the whole song. He has learned to clap his hands, stomp his feet, or say "hurray!" (he just lifts his arms over his head with a happy smile) at the appropriate time in the song, instead of throughout the whole song. (If you're happy and you know it stomp your feet. *STOMP *STOMP.)

We were all happy. And we knew it.

23 1/2 months, some of today's accomplishments

My brother (aka Uncle Robbob) and his girlfriend Taylor are visiting for a long weekend. Taylor hasn't seen Lively in over a year, so he doesn't know her, but he has really warmed up to her quickly. He approached her several times today (the first day of their visit) and gave her hugs, and tried to climb up on the couch with her. He won't let her hold him - he really only lets Pete, our babysitter, and me hold him - but I've encouraged Taylor to keep trying, so maybe by the end of the visit he will tolerate it more.

My brother has a beautiful dog, Bo, who came along for the visit. Annie and Lively haven't spent much time around dogs, but they are crazy for Bo. Lively walked up to Bo several times this evening and touched him quickly, with one finger, and then ran away squealing delightedly and very proud of himself. Notice, I said one finger. We've been working hard to get Lively to gesture toward anything, and pointing with one finger is a new and advanced skill for him. I've been working on pointing with him multiple, multiple times a day. I make his finger point while holding back his others when I known he wants something, when we're reading a book, whenever I'm trying to show him something, and he seems to be starting to get it.

And, Lively also said "Bo." I'm trying not to be offended that he still doesn't really say "Mama" (just kidding, I'm thrilled for every new word.) There's something about the way he vocalizes that is seeming stranger to me. He seems to know that he sees Bo, he likes Bo, he wants to pet Bo, and that Bo is an all-around good idea, and he seems to understand that Bo is Bo's name. But Lively has a hard time getting it out. He tries to say Bo, but it just doesn't always come. Another example: he's obsessed with ceiling fans (not in an autistic repetitive motion spinny way, ok, well maybe just like that), and he often wants me to walk into a room and turn on the fan. So I've been working to teach him to go into the room, point to the fan and sort of trace the movement of the fan in the air (we're making up our own signs now), and say "fan". Instead, he says "ffffffff..." He just can't get the word out. He knows the word and what he wants to say, but he's not able to make his mouth say that word.

Just some of today's observations. Hopefully I'll have more (positive ones) to share tomorrow.