September update

July 2010
Theo's decannulation in July was a brilliant experience. The actual process was straightforward and simple. Several nurses, students and doctors had crammed into the cubicle to watch this epic moment. There were the noises of inhalation and then breath being held as we untied his tapes and then with a brief interruption of his toy car game, I pulled the trach tube out; theo coughed briefly and returned immediately to his play!
It was a very unimpressive moment and quite hilarious to see so many people waiting to see such a significant event for us.

Theo's birthday was a pleasant low key day. we had some family round for a slice of cake but nothing as impressive as last years party. Whilst it would have been great fun to see everyone again, we are generally worn out and need gentle events!

The ensuing weeks were rather fabulous for us as a family. Theo started walking independently days after the decannulation. It is almost as though he was waiting for the freedom, knowing he was always tied to a suction machine so long as he breathed through the tube.
The stress levels lifted tremendously and relations in the household improved. We started having some fun and this coincided with some lovely weather and the school holidays, so it felt delightful. The joy of being able to let Theo join Sacha and Tabitha down the garden without the chore of having to collect catheters and suction machines and barrels was unparalleled.
The other wonder for us, was the sound of his voice. He began making use of this skill immediately, interestingly with baby babble noises but mainly with shrieks and screams and nonsense sounds.
Eating remains problematic. We are out of ideas for how to manage him. Sometimes he show interest in food and we make the most of it by offering him everything we can. He seems to have real phobias about wet or cold food, shuddering if he touches it. Very few items pass the test to be picked up and sampled and even when he gets this far it generally provokes intense gagging and vomiting. This difficult reaction provokes a very negative cycle. Food is essentially going to make him sick and he seems to know it. The vomiting itself is an issue as he suffers terrible mouth ulcers and frequent oral thrush - so his mouth hurts a lot too, poor baby.

Although many people seem opposed to the idea, confident that they may be able to persuade him to eat (it is very frustrating, the amount of comments we receive about offering him baby rice or just forcing food into him- as though we havent tried everything we can think of, plus all the reasonable ideas other people, including therapists, have suggested) we have asked for a referral to the gastro-surgeon for theo to have the long awaited gastrostomy.

For the squeamish amongst us, a gastrostomy is a simple balloon device which provides a port directly into the stomach so that there is no confusion about tube movement or displacement. It is more comfortable for the patient and much more user-friendly. As Theo shows no signs of losing his tube dependency no matter how much effort we put into persuading him otherwise, we hope that this will be a better solution for all of us.

August

The visit to Great Ormond St transplant/ heart failure clinic was a happy one, we had good news, Theo's heart function was still good enough to avoid him being added to the transplant list. He was also well enough to be considered as a patient in a stem cell therapy research project being conducted this year.
After some consideration of the details, we decided to agree to the project as there was a remote possibility it could improve his heart function, even as a side effect of the trial, rather than a completed treatment.
However, within a week of this decision, Theo developed a cough and experienced several nosebleeds. I took him to the GP who diagnosed ng tube trauma in the nasal passage and prescribed some antibacterial ointment. To our dismay his nose bled from the other nostril and continued to bleed over the next week or two. His cough also persisted and at times he began to really struggle with his breathing, showing classic signs of respiratory distress, his ribs recessing and the extra work making him vomit more frequently and sweat. He also started to sleep much more in the daytime.

We took him to the childrens admissions unit for an assessment. My concern was Theo's heart function, as he seemed puffier around the face and his cough was not producing sputum like infections do.
An X-ray revealed pulmonary oedema (fluid on the lung) which is a feature of congestive heart failure. His heart was in acute failure again despite medication. Dr Duke, his cardiologist increased his diuretics and Theo was reviewed again 2 days later. The oedema was still present, as was an unexpected surprise - a coin apparent in his gut, visible in another x-ray. This caused a consternation and a great deal of amusement. Eventually a 5p was passed with a yelp! We endured a painful hospital admission of 5 days, unnecessarily and most frustratingly.
The ward we stayed on this time was new to us and very disappointing. I was repeatedly told that his breathing issues were not heart related and after 4 days, I looked at his drug chart out of boredom and noticed that one of his cardiac medications had not been given properly. Nurses continued to play down the situation, telling me he wasnt in any breathing distress. I felt quite vindicated when we finally made a trip to Glenfield for a cardiac review, in spite of the bad news it held for us.

The echo later that week revealed deterioration in theo's overall heart function, especially concerning his mitral valve which is now leaking significantly compared to the echo at great ormond street only a fortnight previously.
This is very worrying for us, as it means that the pressures in the left atrium are excessively high, causing pulmonary hypertension - this is causing the pulmonary oedema. It also puts strain on the already damaged areas of his heart, primarily the left ventricle. There is a very complex set of chemical reactions that I can barely comprehend, never mind explain. In answer to the question I get quite freqently about how he has grown and his heart will have grown stronger? No, as the stress his healthy heart tissue is under far outweighs the small portion of growth it has experienced. To put it simply, Dr Fenton at GOS drew me a cross section of Theo's left ventricle (the pumping chamber that services the body with oxygenated blood) and crossed out half of the chamber. 'This bit doesnt work'.

The options at this point in Dr Duke's opinion were 1)increase diuretics, 2) retrial captopril and digoxin, 3) enlarge stent under catheterisation 4)watchful waiting.
We went away for a fortnight with more diuretics and came back for more echos and diagnosis.

September
The first appointment at Glenfield this month showed Theo had not improved although the added diuretics were helping shift the fluid off his lungs, so his breathing struggle had lessened.
Our options now are to commence captopril and digoxin again. Everyone is very anxious about these as Theo's kidney failure last year was directly associated with these renal-toxic medicines. Unfortunately, there are very few medication options left, and the captopril/digoxin combination are extremely effective in the treatment of congestive heart failure.
So on Monday and Thursday last week, Theo and I spent 8 hours each day on the ward having his blood pressure monitored while the captopril was introduced and increased. He also has had blood tests on Tuesday and Friday.
Monday evening I drove to London for a Tuesday appointment at GOS.

The Great Ormond St review
I was told almost immediately that Theo is no longer suitable for the stem cell trial, as he is too sick.
Dr Fenton told us that our options for treatment are now drawing to an end and unless Theo responds well to captopril without suffering kidney problems, there is virtually nothing left to treat him with medication-wise. There is a drug called Losartan that they could try but compared to captopril it is almost not worth it. It is time to consider the transplant list again.

Sitting in the office with Dr Fenton, I could almost hear doors slamming, as each one of my increasingly desperate enquries met with a no, or a not worth it..
'could we not try nasal cpap?'
'if we are looking towards mechanical ventilation, it's time to list him'

'what about the catheterisation and stent enlargement?'
'it would offload the pressure but his overall heart function is worsening and besides, i wouldnt put him under anaesthetic unless it was life or death'

The most difficult consequence of this conversation is the realisation that the 2 operations we were hoping could take place, the gastrostomy and the tracheostomy stoma repair cannot happen. The stoma remaining open is a real issue as he continues to suffer infections adn colonisations as though his trachy was in situ which poses more demands upon his heart. The gastrostomy is more a quality of life issue.

Today

Having had an exhausting week of hospital appointments and the worry of where Theo will take us all next, emotionally and physically as a family has meant lots of shouting, arguing and tears. It is so horrible having to contemplate what a transplant means, it isnt even a cure, its simply a palliative measure to improve quality of life for up to 10 years. There's no denying it's an incredible awe-inspiring treatment but the fact remains that Theo will still meet his end sooner than he should, as a result of the viral infection of 2 years ago.

We are currently relieved that the ongoing monitoring of his kidneys, the blood tests, are showing a slight elevation in figures, but we have been reassured that things are ok (cannot help breathing the words 'for now').

An infection that has been really bothering Theo in the last week brought disastrous news that they would need to admit him for IV antibiotics to treat it as it was a strain resistant to anything he could have orally. For the first time since he came home, we actually decided not to take him in for selfish reasons. Colin is away on Monday night so I would not be able to stay in hospital with Theo as I prefer to do. There is also the liklihood of him being admitted to the ward that cocked things up last time and I could not stand that. Also, I was utterly exhausted, having spent 10-6 in hospital every day this week except Friday, during which I think I wondered around like a zombie, shouting at Colin and feeling dreadful.

So we must see what tomorrow brings. We would like to hear that his kidneys are bearing up and the medication is helping to restore some greater efficiency in his ticker. We would like to hear that the stoma repair can go ahead at Glenfield, or GOS as they would prefer. We do not want to see urea and electrolyte levels rise, we do not want any more breathing difficulties or pulmonary oedema, we do not want any other surprise symptoms or infections.

I will arrange some photographs soon and will update more regularly. It has been very difficult to prioritise news this way.
Willow