Little T's platelets came back today at 87, the lowest in a while. His hemo doctor talked about putting on Vincristine again, aka chemo if his numbers don't improve. Little T has been on high doses of steroids for over two months. His hemo doctor's not comfortable having on him high doses for too long due to steroids' long term effects. He's reaching the time limit where he needs to be weaned off, or put on Vincristine. Damn. His platelets were 112 last week, the highest in a while, so things were looking like we could wean him off steroids, and he was going to make it just on steroids. We're hoping that today's number was just an anomaly. We'll find out more next week. I can't help worrying. I need a hug. I need good vibes.

Through it all, Little T laughs and smiles, except when he flies into 'roid rages. He wants to play with other kids. He doesn't understand why he can't. The social isolation of the steroids is hard to take, but it will break my heart if Vincristine takes away his voice again.

I guessed Little T's platelets would be 95. They were 97. That's not normal, but also not dangerous. If his platelets continue to remain at this level or above, he's going to continue on the same high dose of steroids for another month, then gradually wean down.

He's finally started gaining weight again up to 9256g, though the steroids are definitely stunting his growth, because he hasn't grown at all since he last measured on 11/16 when he measured 69.7 cm.

We had to wait over half an hour for his blood draw, but we had Sean again, so once again he got it smoothly on one try.

We're going down to once a week appointments now. Phew!

Despite the high dose of steroids, Little T is still laughing, scooting, and babbling most of the time. He only flies in rages about twice a day. His arm continues to shrink and his elbow now bends ever so slightly as the tumor slowly shrinks milimeter by milimeter. Though I am concerned that with the steroids, he seems to have lost his desire to walk or cruise. When I try to walk him, he won't hold his legs straight. However he pulls up on C and me all the time. Go figure.

Unfortunately his platelets have dropped to 67. Below 50 is when we need to worry about him pulling up and cruising, but it's a downward trend. Also the clinic is closed on Friday, so instead of going in then as we would normally with a number like that, we have to wait until Tuesday.

The doctors have increased his steroids to 2.7mg/kg, so I expect him to get angrier. Right he flies into 'roid rages 2-3 a day. He already has a "moon face" from the steroids.

Poor guy had 3 sticks today to get an IV in him, because he had to go under general anesthesia for an MRA(an MRI with contrasting dye) to look at the tumor on his left arm. The MRA showed that his tumor is smaller than his last MRA in April. Not too surprising, and that it's not growing into anything important.

He came through much better than we expected. For the first time in numerous times under anesthesia, he woke up before he got to the recovery room. By the time I got there he appeared alert and pointed at me to try and get me to pick him up. Within five minutes he was drinking and then proceeded to drink 6oz over the course of an hour. That's a new drinking record for him. Poor guy he'd been off food since midnight the night before. And usually we give him a 600ml feed overnight through his g-tube.

Little T's platelets today were 87, so really about the same. That's what I thought going in. But I didn't hear what I was dreading: he would have to go back on Vincristine. I'm very grateful for that.

C came with me and really it's far easier to ask hard questions when he's there. So I asked what would put Little T back on Vincristine. The short answer is his platelets would have to drop below 20. His doctor actually mentioned Vincristine when his platelets were 211, but she tends to be a pessimist. The time before, his platelets were down to 40 and she was envisioning them dropping down below 20. Of course the long answer is his platelets do have to improve over time, but he has some time.

In addition he could get a higher dose of steroids. He's on 2mg/kg and he could go up to 3mg/kg for a short time.

Sadly he is definitely immunocompromised on this dose, so he can no longer be in occupational/physical therapy in a group setting.

He lost about 50g. I think it's the effect of the steroids. He eats more and loses weight. What a great diet except that you

My writing continues. I caught up on the days I missed and my non-fiction book is at almost 24k now. I also finished an article on my breastfeeding experiences with both kids, which I plan to submit once I get critiques. Please let me know if you want to read it.

I'm having a really hard time not worrying about Little T going back on Vincristine. I know we can get through it. We went through it before. But I feel sad when I think about him getting another Broviac, losing his voice, getting weakness in his legs. I don't know why I can't stop thinking about it. It does no good, since there's nothing I can do. And I'll feel silly if he does okay with the steroids.

Yesterday Little T said "Uh" Pause. "oh" when I dropped his bottle. He also said "Bye" to Roberta when she left. For the first time he said two words in one day. He hands out his words like precious gifts. Is that because he spent months unable to use his voice due to Vincristine? Prolly not because he sure says "Gah" all the time. He also says "Ahh!" when he's particularly pleased about something. Like when he got hold of the Tivo remote. He also gave me a big grin.

And I just went and read someone's blog whose baby is in the NICU teetering between CPAP and canula. Little T was on the canula for a day or so. To the doctors' amazement, his cardiovascular system held up even with the strain of supplying blood to a vascular tumor that was literally bigger than his head. I had forgotten about how worried they were in the first days with lots of tests and close monitoring. I guess I wasn't looking back far enough. In some ways, my boy is medically fragile, but in other ways he's incredibly strong.

I should add that Special K seems cheerful again. She's been dry for days. She's rushing about. She's bouncing again.

Her preschool schedules a phone call for every parent at this time. During our phone call, her teacher said that Special K was "smart, sweet, talkative. She finishes her work. She looks out for other kids. She wants to be a leader." After we talked more about that, we learned she's bossy. She also sometimes talks too much, but after she was removed from circle time once, she stopped talking when asked. That's my girl. Though I wouldn't know where she gets it from...

The best thing about the whole call was when I asked "How's Special K doing with her brother being sick off and on?" and her teacher said "I had no idea about her brother." We had written a note about Little T's tumor in the little form about your child you fill out at the beginning of the year, but evidently the teacher didn't read it. I hardly ever see her teachers because Special K likes to stay for lunch and different teachers look after the kids for lunch. Special K is certainly capable of saying her brother is sick, but obviously that's not on her mind when she goes to preschool.

Mostly Special K interacts with her brother by making him laugh, holding his hand, and telling him or us not to pull her hair or not to eat things (yes being bossy).

I'm so grateful Special K is doing well. I feel a bit better now. Thanks for reading.

Today Little T's platelets fell back down to 80, so Little T is back on massive amounts of steroids. We're not in the "fall down brain bleed" danger zone, but in the "fall off changing table" danger zone. Like most parents, we're already cautious about that type of falling anyway. He's already immuno-compromised. I'm bracing myself for more 'roid rages and days of holding Little T. Right now he's still scooting about on the floor, but we haven't given him the big dose of steroids yet. I'm enjoying the scooting while I can.

I had a bad feeling before I went in. His tumor seems to be growing at the back of his shoulder. He has two weird unexplained bruises on his thigh.

It's all very strange. His tumor is the smallest it's ever been in his life by several cm. And of course Torin's also the largest he's ever been. As the tumor shrinks or involutes, it also consumes platelets and fibronogen, because it's a vascular tumor, so it has to clot itself in order to shrink. Somehow he's managed to keep up with his clotting needs for months. Before this latest bout, he was KMS free since February. His bouts of Kasabach Merritt Syndrome seem to be related to the tumor's growth. But the growth itself seems to be small related to the amount that's shrinking. Overall the tumor itself is still smaller. Yet now he has KMS again.

My theory based, but on my reading of various KMS papers and my understanding of biology and no scientific testing whatsoever. But hey it's my blog. I can type whatever I want. I like the idea that hemangiomas/vascular tumors are placental tissue that wound up in the wrong place. It explains why they're relatively common and why most grows rapidly for a time and then eventually shrink. The tumors that cause KMS have a higher percentage of placental stem cells and that causes problems. Stem cells can morph into other cells. They can also call other cells to help them. The placental stem cells calls what it thinks are maternal blood cells, but are actually the kid's own blood cells. The poor blood cell platelets get trapped and so you have KMS. You get KMS when it grows, because of the influx of blood cells. It would also explain why some KMS tumors don't go away. The stem cells got morphed into kid cells aka tumor. Anyway, that's just my way of making sense of something that upsets me and doesn't have a well-understood mechanism right now.

This morning we had amazing news. Little T's platelets suddenly bounced back to normal (211) We're totally stunned. His tumor has never responded so quickly. We were expecting weeks of treatment.

This is our third round with it. The prior two times, it took weeks of steroids and Vincristine (chemo) before his platelet count returned to normal. Change was so slow. This time you can see drastic change. His arm circumference suddenly shrunk from 25cm to 20cm.

A slight worry is that his fibrinogen is now down to 156, which is below normal. It's probably because it's being used to clot off the tumor. At least we all hope so!

We'll give him two more doses of massive amounts of steroids. Then we'll cut back to half doses of massive amounts of steroids. We go back next Tuesday to see how things are going.

Steroids are awful. Not as bad as chemo, but steroid change his personality for the worse. I think of athletes on steroids all the time. No wonder they get into fights all the time and beat their wives. His doc says they're different 'roids. Whatever. They suck.

Yesterday I looked at the four syringes of medicine that C was going to give him and my heart broke just a little. In his early days, we gave him as many as 8 drugs at one time. Gods knows how all these drugs interact and they don't run drugs trials on babies. But my little observations are these. He used to smile at least once an hour. Now he smiles once a day and cries at least once an hour. He wants to be held most of the time if he's awake. He used to like to scoot about on the floor most of the time.

He flies into 'roid rages a lot. Little things just tick him off. Like if I hover over him too much. If I take away a toy. If someone is too noisy. His face contorts into this furious mask like the Incredible Hulk. He starts screaming very loudly. He behaved this way as a baby and I always said he was really a sweet guy. Sometimes the nurses would just look at me like "yeah right." Now I know he is normally a sweet guy.

At least I've removed one source of his rage. Normal platelets means no more hovering over him whenever he scoots about or pulls up. And I can count the doses until we cut back to half doses. Maybe that will take away the 'roid rage. Or at least bring back the scooting.

Anne Lammot's book Bird By Bird is one of several that changed my life. People ask "how do I get through this?" One way is dose by dose, day by day, hour by hour. Sometimes minute by minute.

I will tell you we also learned today that this will probably not be his last relapse. That some kids with his type of tumor are on Vincristine aka chemo for 3-5 years. So in some twisted sense, Little T's "lucky". Or maybe we're just parents and a doctor who won't take that course of treatment. Who knows? There's no standard course of treatment for Kasabach Merritt Syndrome, because it's so rare.

I also learned that the relapse may have been triggered by his getting the stomach flu. Or maybe not. So no more playdates. And we have to treat him as immuno-compromised for the rest of the winter. Though he is in fact immuno-compromised right now, because of the steroids.

But honestly right now that's more than I want to deal with right now. I'm mostly telling you, so you won't be surprised if you read that he had another relapse. And if you live locally, why you won't be seeing Little T anytime soon.

But I guess if they keep resolving as quickly without the complications that have landed him in the hospital five times, then it seems somehow manageable. I dunno. I can't really think about it too much. So I just think about today and how his platelets are 211 and just two more doses.

We had a bit of a scare last night, because we saw petechiace on his left forearm. We were worried that his platelet count might be dropping to the dangerous internal bleeding level. So this morning C took him into for another blood test. His platelet count was 97! Not normal, but definitely out of the worry if he bumps his head stage.

The platelet numbers are points in time. We have two numbers. So far, they indicate an upward trend. We hope it stays that way. We hope it means the tumor has started shrinking again. But we need more data and more time to know anything. We just have to watch and wait.

We also had another small victory. Armed with my list of best blood drawers, the blood draw took only one try. Previously it took at least three tries with lots of painful digging around the vein. Last time in desperation I asked what I should do and I got the list.

Little T's tumor is regrowing at the ends, though it continues to shrink around the middle. It would all be fascinating if it weren't my son. He's back on Amicar and Prednisolone (steroids), which means that he's immuno-compromised again. His tumor consumes platelets again. Kasabach Merritt Syndrome returns again.

Unfortunately his low platelet count (40) means that if he bumps his head, he can have a brain bleed. He's starting to cruise and he's wobbly. Normally he falls on his head every day. Not a hard fall, mind you. Just a slight bonk on the head. Not even enough to cause crying sometimes; certainly not enough to cause a bruise. He only has one functional arm, so he can't brace himself. So no more cruising or pulling up for him until his platelets come back up on their own. And we have to watch him very carefully. His platelets get measured again on Tuesday. It'll be a long few days.

We thought very carefully about getting him a platelet transfusion, but we decided that we don't want to risk platelet rejection, and the IV would be very tough on him. We'd still have to watch him just as carefully since we don't know what his platelets will do between now and Tuesday. And Wednesday was probably his lowest platelet point anyway when he had the bruising and petechiace. Each platelet transfusion increases the risk that he'll reject them. He's had so many platelet transfusions.

Yesterday Little T seemed quite recovered from his illness. He said his first word. He said "bye" clearly and distinctly as his sister left the car for preschool. He has uttered no other intelligible words since unless you count 'gah' and 'yeah yeah yeah yeah'. At this age, Special K loved to repeat words over and over again. She made sure everyone knew she was talking.

I think Little T might have said 'mama' and 'dada' on several different occasions, but I'm not really sure. He combines it with other noises and says it once. He has a speech evaluation on Saturday. I'm not sure what the speech therapist will make of him.

Today we woke up to find that Little T's left upper shoulder was covered in huge bruises and petechiace. We tried to remain calm, but this has heralded trips to the ER leading to one diagnosis of a relapse of his tumor and several false alarms. I lean towards the latter. We call the Hematology nurse practioner. She's very nice to me. She said "We're keen observers." We decide that the stomach flu combined with the antibiotics has probably lowered his platelets sufficiently to cause the symptoms. We watch and wait. We'll see if the bruising gets worse. So far so good. We'll take him in on Friday. He was scheduled to go see Hematology on Tuesday anyway.

I go to my previously scheduled neurologist appointment. I get the flu shot. I call Special K's doctor to arrange for her to get the flu shot. I tell C to get the flu shot. Little T can't get the flu shot until December. He can't get any shots until six months after his last chemo. Until then, his immune system won't produce enough antibodies to make the shot worthwhile.

I thought Little T was at the point where he could just get sick and we wouldn't have to call doctors, but clearly I was just wrong. And I really should stop saying things like 'it's nice to have a normal illness." It just tempts fate. And he's just not normal. I'm not normal either, though my disability is far less serious. And the expectation of normality hurts.

So please send Little T lots of platelets and tumor shrinking vibes his way.

The Water owl is a rare strange bird, so I provide you with this field guide to the medical issues that form a background to her daily life. I hope this background makes things less confusing, but maybe not. Learning the habits of a new animal tends to be confusing for anyone. I chose thirteen questions, because it worked out that way and thirteen is my lucky number.

1. Why does Little T have a feeding tube? He doesn't eat enough on his own. The treatments from his vascular tumour messed up his eating. The tube allows us to pour liquid food aka formula down the tube into his stomach. At first he had an NG tube The tube went through the back of his throat down his esophagus and into his stomach. Now he has a Mic-key gastronomy tube that's a tube directly into his stomach. Click here for more details about his Mic-key
2. What is a vascular tumour? A vascular tumour is a proliferation of nonfunctional blood vessels. Picture if your blood vessels grew uncontrollably and formed tangled balls. Hemangiomas and vascular tumours are relatively common, but fortunately most are pretty benign medically. Unfortunately Little T's tumor got so large he developed Kasabach Merritt Syndrome(KMS) It's very rare like .1%. For most, KMS is the bogey man that doctors state is a risk like how they state there's a risk you could die during routine surgery.
3. What is Kasabach-Merritt Syndrome? Kasabach-Merritt Syndrome (KMS) is a very rare condition in which a hemangioma or vascular tumour gets so large and invasive that blood flows into the tumour and gets trapped there. Like cars stuck in a traffic jam, enormous amounts of platelets and fibrogen (blood clotting factors) are consumed. If untreated with blood transfusions, KMS kills. Sometimes patients don't make it anyway. C and I have read mortality rates anywhere from 10% to 37%.
4. What do you mean by giant? How big is his arm now? When Little T was born, the circumference of his left arm was 24 cm, or about the size of my arm and looked like a balloon. My brother said he looked like Popeye. Here's a photo. Now his arm has shrunk a lot and at its widest point is about 15cm. It's also much more differentiated like a proper arm.
5. What treatments made his arm shrink? In the NICU, he was on Interferon. It made him lethargic, and possibly depressed. It killed his appetite. After his relapse of KMS in January, he was put on Vincristine, a type of chemo instead. It caused neuropathy (temporary partial paralysis) of his legs and vocal cords. Vincristine made him feel nauseated and gave him reflux. It also made him lose his hair. Both times he also took massive amounts of Prednisolone, a type of steroid. When he was on Prednisolone, he ate about 3/4 of his recommended daily calories, according to his nutritionist but the steroids retarded his growth. When he was taken off the steroids, he started eating even less, because the steroid stimulated his appetite. I wrote a lot about his eating in my personal essay "His Tube Ate My Boob and other Feeding Fables" that I've submitted to Brain, Child.
6. Why was Little T on chemo? Did he have cancer? Well, that depends on how you classify cancer. By the strict medical definition, no, he doesn't have cancer. However if you consider cancer to be an invasive tumour that is lethal if untreated and therefore you must attack the tumour with harmful methods like chemo and massive amounts of steroids, then yes he does. And we're actually part of an Children's Oncology support group. We went there at first, because there was nowhere else to go. We wondered if we belonged there, but as we told our story, all the other parents there had had similar experiences. It was incredibly sad and comforting at the same time.
7. Can his tumour regrow? Unfortunately it can and has. His hematologists thought that it wouldn't and he had his Broviac (central IV line) removed in December. Unfortunately it regrew in January and he had a reoccurrence of KMS and was hospitalised.
8. Does it hurt? Well, the doctors tell us that the tumour itself doesn't hurt. However the tumour compresses the surrounding tissue and nerves and causes Compartment Syndrome. The massive swelling from his tumour cuts off sensation and ironically blood flow to his lower arm and fingers. At first he didn't move his arm or fingers at all. Now he moves his fingers somewhat and shrugs his shoulders. So the process of growing doesn't appear to hurt, but the process of shrinking does, perhaps because circulation and sensation return to his arm.
9. What's the long-term future for his arm? No-one really knows. We're hoping he'll be able to use his arm. Right now he's rather lopsided, and he just learned to roll over at 10 months, because his left arm is so heavy and non-functional. We're hoping that through physical and occupational therapy, he'll be able to regain strength enough to crawl and eventually walk.
10. How many times has he been hospitalized?
    1. NICU - Birth to 6 weeks old for KMS
    2. December 2004 (5 days) for a urinary tract infection caused by being without liquid because he had an operation to get his Broviac removed. He had another Broviac installed after his relapse in January.
    3. January 2005 (10 days) for a reoccurence of KMS
    4. February 2005 (7 days) for RSV a few days after he was released from the hospital, so he probably caught it from the hospital.
    5. June 2005 (7 days) for line sepsis. His Broviac got infected.
    6. July 2005 (48 hours) recovery from G-tube surgery
11. You mention you have a movement disorder. What is it? I have Myoclonic dystonia a very peculiar rare neurological disorder that affects fine movement. Neurologists get excited when they see me, because it's so rare. Basically my head or arms or hands will randomly jerk when I perform small activity. It appears to be nonprogressive and in my case, it also only affects my upper body. Myoclonic dystonia is thought to be caused by a defect in the basal ganglia. The analogy that works best for computer geeks is that I have line noise in my basal ganglia that causes me to jerk. I agree with this theory, because it does seem to take me longer to learn fine motor activities. But once I've learned them, I do just fine, except for the random jerking.
12. What's the basal ganglia and what does it do? The basal ganglia help the brain perform more complex tasks. For example, after you've learned how to write or type, you don't consciously think about how your hand moves, you just think I want to write or type a particular word. Your basal ganglia helps you coordinate all the complex movements needed to perform these actions. Remember how when you first learned how to write or type, you had to think about everything and it was very slow at first. Well that was because you didn't have the proper neural pathways in your basal ganglia yet.
13. Is your medical condition related to your son's? I'm told they're not related as they affect two entirely different systems. However I think the answer is that no-one knows the true cause of either condition, so it's impossible to know with current medical information and technology.