Conversation with the doctor.

Hi all,

We had a very helpful discussion with the doctor and his nurse. We have a good idea of the extent of Noah's condition, but how it will affect him long term is still unknown as each child is different and some of the things Noah has are unique. During his incidents a lot of different tests were run and as yet the full findings are not known.
Dr Joost, Noah's primary physician, is planning meetings with all the other doctors involved in Noah's care and together they hope to create an overview of his condition plus a broad plan of treatment.
Dr Joost went on to explain that they work in concepts. The first concept was to send Noah home without a cannule after they had operated and removed the obstruction in his throat. Due to the setbacks and the slow progress he's making, they are looking at a different concept. The new concept is to wean Noah from his hospital breathing machine on to a machine that can be used at home. This way Noah can come home and do the necessary growing required to overcome his problems. They believe that an extended stay in hospital is not the ideal place for a young child’s development, home is best!

OK, so to the areas that CHAOS has affected in Noah. First there is the blockage in his throat. The blockage covers his voice box, and they see that his vocal chords are fusing. They are still unsure as to how much vocalisation he will have in the future.

Next are his lungs. The tubes that make up his lungs are soft and prone to collapsing. There are no drugs or operations available to fix this, it takes time and growth. However, the length and weight of a child has no bearing on the strengthening of the lungs. They have seen children grow over this, some take months and others years, so this is one of the unknown factors. It is a crucial factor affecting when Noah can receive his operation. His lungs must be strong in order for him to be able to breath on his own before they can remove the obstruction.
During last week’s camera examination into his lungs, they saw that his major bronchial tube, where it splits into each lung, pulses. They think that there is an artery pressing against the tubes and, because it is soft, this is why they see a pulse. The pulsing causes the tube to narrow, and this in turn is limiting the amount of air and pressure into Noah's lungs which they believe led to his incidents. They have done a CCT scan, where a phosphorous fluid is introduced to the blood stream and then photographed, to see whether this artery is malformed or is just out of place. Analysing the results is a complicated task and so we're waiting for the results of this test. We hope to have the results explained to us sometime next week.

After the lungs are Noah’s distended belly. They believe that there is a leak in his blood vessels and that’s why there is fluid collecting there, but as yet they cannot find where the leak is coming from. His stomach muscles are stretched; this could have an effect on his development such as sitting, and walking. They hope that in time the leaky vessel will stop leaking and that his lymphatic system will kick in to cope with the excess fluid. At the moment they treat Noah with a diuretic (plas medicine) when the fluid makes him too uncomfortable. Again time is needed to see how Noah’s body will handle this.

Noah has a gap in one of the chambers of his heart. This would normally cause flooding of blood towards the lungs; however, he also has a narrow valve in the chamber to the lungs, so this cancels out the effect of the gap! A lot of healthy children are born with this gap, and it can be self healing and so this is another unknown. We just have to wait until he’s a bit older to find out if this is a problem.

Noah has a small liver and his intestines are not where they would usually be. It’s not known if this means anything, but it’s a genetic fault that could be used as a marker to identify CHAOS. Again, only in the future will we know if it affects Noah in any way.

Working in Noah’s favour is the fact that his growth and weight and development are on target for a normal and healthy child. Considering all of the above, this is a very strong plus, quite a miracle really! So it’s an optimistic outlook for his future.

So now we have a roughly drawn box around Noah’s version of CHAOS. What does this mean for us as a family in the future? We’re still trying to work that out. The family is giving us very strong support and this is helping us to keep our options open. Uncle Jan will be joining us next week to help us understand a little bit more about Noah’s condition and perhaps have a better understanding of what his long term care needs will be. It’s ok knowing what’s wrong, but what exactly this means in terms of everyday life is still a bit fuzzy to us. So I’m glad we’ve got someone that can help us here.

You’ll be glad to hear that Noah has had a really relaxed couple of days. We’ve seen that he’s mastered the laugh. It’s no longer quick smiles, it’s full on toothless grins! He’s also reaching for things a lot more and he’s starting to want to eat everything that goes near his mouth. Yum-Yum! He’s still the darling of the nurses, they argue over who gets to look after him, he’s very popular.

Tomorrow we’re back to Den Haag for a visit with Frank van Velsen who is having his zero birthday. Noah is sad not being able to go, but he is looking forward to his Jelly and Ice cream flavoured mummy’s milk later in the afternoon!

Thanks again to all of you that comment on the blog, please keep them coming as it’s good to know how many people are out there reading this. Hopefully if things stay stable for a while we’ll get a chance to catch up with sleep, outstanding e-mails and phone calls.

xxx The Drostens

p.s. New photo's will be uploaded soon!