Medical field guide

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.
   
   
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.