Look ma, no vent!

Hello all,

The blog has been getting quieter over the past few months, because we are finding that Peter's life is becoming almost too ordinary to justify his own blog. Nevertheless, we are rejoicing in his milestones. Many of these milestones are, again, quite ordinary but any of these are still miraculous in light of his long journey to get there. To be reaching ordinary infant milestones is a delight we could not have imagined to rejoice in last summer.

Peter is now crawling like the best, much better, in fact, than his brother every did and much earlier. He is also learning to cruise along furniture and walls and we have little doubt that before long, he will take his first real steps. We have had to consider changes to the way our house is organized to keep it safe for him. Luckily, the presence of nurses means that most of the time an adult is there to keep him out of trouble.

The fact that he is now moving out of the living room does indeed signal that he is now mostly tubeless. In fact, the biggest milestone in his life was reached today. A very not-ordinary one: he is now completely off the breathing machine. For the first time in his life he is now no longer on any vent, even at night! We rather unceremoniously removed the vent from the room today. The only tubes he still has attached to him are those that provide heat and humidity to his tracheostomy, and his attached to those practically only when he is napping and sleeping.

Peter remains a very happy boy who loves to do as his brother Daniel does. Recently, they discovered a game they can both enjoy playing together. The video below illustrates a particularly enjoyable round.



There are many more milestones; practically every day there seems to be one. He is a good and adventurous eater. Thanks to the six teeth he has, he is quite willing and able to eat all sorts of things. We are looking forward to the next appointment with Peter's cardiologist and pulmonologist on April 23. If the X-rays and all other things look well, we may be looking at a swift appointment with the ENT (Ear, Nose, Throat) doctor to take out the tracheostomy and do the restorative surgery, possibly as soon as May.

What the world needs now: an institute for chylothorax research

I know I have mentioned this to various people, but I finally wanted to write down what was on my mind. Two weeks ago we attended the funeral of one of Peter's little intensive care buddies. Totally different medical situation, but chylothorax was one of the major factors in that baby's struggle.

Chylothorax is a leakage of chyle (fluid from the lymphatic system) into the thoracic cavity. It is often triggered by thoracic surgery, such as open-heart surgery, but can occur spontaneously. It happens in infants and adults, and even in cats and dogs--just google 'chylothorax' and you get a lot of veterinary links. The lymphatic system, in addition to its infection-fighting job, also processes fats in the bloodstream. With chylothorax, the fats get dumped along with chyle into the chest cavity, putting pressure on the heart and lungs.

When Peter's chylothorax started a few days after his heart surgery, the surgeon explained what it was and listed the treatment options. He said, "From the variety of treatments you may rightly conclude that this condition is not well understood." The treatment protocol included removing all fats from the diet, an IV medication called Octreotide, a surgical procedure called pleurodesis, and using the ventilator settings to hyperexpand the patient's lungs against the rib cage. After the effusions stop, the patient must continue on a fat-free diet for three months, then gradually reintroduce fats.

Peter underwent all these treatments. It still took almost two months for the effusions to subside. Children who have heart defects requiring multiple surgeries could get chylothorax, then recover, then get it again after the next surgery. In all that time, an infant with this condition cannot have the fats its body needs to grow and develop. Proteins are washed out along with the fats, and thyroid deficiencies can arise. Chest tubes must be inserted to drain the effusions, but this limits movement and motor development. The domino effect of chylothorax is considerable.

Even from the insurance company's perspective, the cost of chylothorax is high. Peter's hospital stay was originally estimated at four weeks, but ended up at four months--because of chylothorax. As long as the effusions continued, he was in intensive care. He required chest X-rays every day or two, and plasma transfusions on a regular basis to replace lost fluids. Octreotide is an expensive drug, I am told. Then there was the pleurodesis (another surgery), and the need for a tracheostomy (surgery again) because of the long-term ventilator dependency. His custom trach tubes cost more than...well, never mind. And when Peter was ready to come home, he needed 24-hour nursing care and the support of a medical supply and service company.

Surely this condition is worthy of concentrated study. If Michel and I had millions, this is what we would do with it: we would found an institute for chylothorax research. It would be associated with a large hospital that saw a fair number of chylothorax patients every year. The work of this institute would be to come to an understanding of the condition while also working on treatments/prevention. There might be a veterinary wing or branch, because surely there must be correspondences between chylothorax in humans and the same condition in animals. An annual conference would be held to bring together the best new thinking from all over the world. There would also be a nutrition division to come up with healthy, palatable foods for chylothorax patients, especially babies. Peter enjoyed specially-defatted donor breast milk from the Denver Breast Milk Bank; some similar processing center might be part of the institute. There would also be counselling and support for parents and family of chylothorax patients.

We praise God that Peter's chylothorax is a thing of the past. We wish it could be a thing of the past for everyone.

There will be an update on Peter in a day or two. He is doing very well, by the way!

Ocean waves

Hi everyone,


Does this title refer to a family trip to the beach? If only! It refers to the new background noise we get to hear at night ... while Peter sleeps with the vent off! Peter has worked his way up to 12 daytime hours off the vent, and the pulmonologist just said we could begin night weaning. This will proceed, Lord willing, in weekly two-hour increments. Peter goes to bed at 8 pm, so tonight we reconnected him to the vent at 10 pm. Next week we'll reconnect him at midnight, and so on. Because the house is so quiet at night with the vent off, we run a machine that makes relaxing background noise so that Peter doesn't wake up from every little sound.

More good news: Peter's one remaining regular medication, aspirin, has been discontinued.

No more Broviac

We have had a happy week. Best of all was that Grandma came to visit. She and Daniel had a whale of a time. Her visit coincided (not altogether coincidentally) with Peter's Broviac removal surgery on Wednesday. That went very smoothly and quickly. When we went on Monday for the pre-op checkup with the pediatrician, he said that Peter used to have "more plumbing than a three-story outhouse." Wasn't that the truth. Now no more! The trach is now the only artificial hole in Peter's body.

After the last sterile dressing change.

Two bottom teeth broke through the day after the surgery, bringing the total up to six. Peter now has a toothy grin and is getting adventurous with his eating. He is also eating us out of house and home--must be a huge growth spurt! By our next update we will probably be able to say that he's getting his wisdom teeth and wearing a size 13 shoe.

More good news

As of this afternoon, Peter can stop taking thyroid medicine! The pediatrician just gave the all clear. He will have a follow-up blood test in a few weeks just to make sure everything is going fine.

Mostly good news

Time for another update. Peter now has three teeth, all on top! Lately he has been working on crawling. This is much more fun without a G-tube sticking out of his stomach.

The only not so good news is that Peter is currently fighting a bronchial infection. At first this was diagnosed as a virus, but the trach culture from his last doctor's visit finally came back showing no less than five different bacteria growing in there. These are common but nasty trach bacteria, and the pulmonology clinic said that Peter had been doing well to avoid them for so long. He is now receiving an antibiotic (Tobramycin) via nebulizer twice a day and seems to be doing better. We have had to go gently with the time off the vent because it tires him out so. Before the infection hit he was at 6 hours a day total.

Back to good news now. Peter is now off all his diuretics, leaving aspirin and synthroid as his only regular daily meds. We are waiting for the results of his most recent blood test to see if he can come off the synthroid. Also, both the cardiologist and pulmonologist said they had no further use for his Broviac catheter (a permanent IV line in his chest), so it can come out as soon as Peter gets over his present illness. This is really wonderful news. Apart from the danger of a potential blood infection, the Broviac takes a lot of maintenance. Every day it has to be flushed with heparin to keep it from clotting up inside. Every three days the caps on the two lumens (ports) have to be changed. Every week I have to don the surgical mask and sterile gloves and change the sterile dressing covering the spot where it enters Peter's chest while an assistant holds Peter and keeps him from touching the site. While Peter was in the hospital and receiving so many IV meds, it was a practical and useful appendage. Now it is no longer needed, praise the Lord, and soon Peter will be able to say goodbye to yet another bit of tubing.

Santa's surprise

Hi everyone,

The removal of Peter's G-tube on Wednesday went very well and quite quickly. The gastroenterologist even took pictures of Peter's innards during the procedure so we could see. The inside is held together with tiny staples until it heals. Eventually the staples (they looked more like clips to me) will release and make their way into his diaper. Sorry if that's too much information.... On the outside of his tummy he just has a small wound the size of a mosquito bite. After the procedure Peter was bright-eyed and playful, and we noticed that he immediately wanted to play more with his feet. Apparently his abdominal muscles immediately felt more comfortable!

The same day the pediatrician called and said that based on Monday's blood tests, Peter could reduce his thyroid medicine again by half--from 25 mcg to 12.5 mcg. In another 3-4 weeks he can be tested again and probably discontinue the medicine entirely.

Christmas morning we noticed a little something new that had arrived for Peter--his first tooth! It's on the top, as Peter will readily point out to anyone who asks.