The Taming of the Shrew

Despite tonsillitis, Penny excelled in her starring role as 'shrew' in her first school Christmas play.  I was so proud, it was indeed a theatrical masterpiece!

Art!

Ice Skating at the NHM

We went skating with Yaya at the Christmas rink by the Natural History Museum.  It was a lot of fun, you did so well, and we got Minnie on the ice for the last 5 mins.

Minnie at the Science Museum

Now Penny is at school all day, Minnie and I are freer to take excursions into the city ... and one of our first big adventures was to the Science Museum.

It was brilliant!

RHS Wisley Again!

This time we visited Wisley as a family, and it captured Daddy's imagination just like it did with us last time, especially being the height of Autumn.  And of course we popped in to see the Keats on the way home.

What Happens on Blood Transfusion Day

At the moment Minnie is having blood transfusions every 8 weeks.  She needs them because her Pyruvate Kinase Deficiency causes haemolytic anaemia, which at the moment can only be relieved in her by regular transfusions.  This is now a very normal process for us, but we know that blood transfusions are a serious procedure and can sound like a bit of a mystery to most people.

We arrive on Husky Ward at the Evelina Children's Hospital (behind St Thomas') at about 8:30, where we are greeted by lots of friendly familiar faces.  The nurses and doctors we see are all great, and they make the ward a much jollier place to spend time.

We then get settled into our bed (and we hope that we get a bed with one of the comfy recliner chairs).  Until recently Min had a cot, but the bed is much more comfortable for everybody.  There are 6 beds on the ward, most of which are used for day patients who need transfusions, usually for sickle cell or Thalassemia.

Then the nurses take their observations, blood pressure, pulse rate, blood oxygen saturation, to make sure Min is in good shape for having a transfusion, along with taking her height and weight so they can calculate how much blood she will need.

By then I have usually asked what Min's Hb levels are, as we will have been in for a blood test the previous day.  As she is usually somewhere between 7-8 at the moment, this is usually neither good nor bad news.  Though she did go down to 6.6 in the phase after her cochlear implant operation, and she was 6 at her recent transfusion at the end of Oct after an 8 week interval, very low ...

The doctor then comes along to listen to her chest, ask about her general health, and confirm that it is ok to transfuse.  It sometimes takes a little wait for the doctor to come round, and then the blood order can be confirmed.

Minnie has a portacath (or port) which means we avoid a painful cannula insertion on transfusion day.  Magic cream is put on the port so putting the needle in doesn't hurt, but Min still cries and yells as she doesn't like being held still.  But it is infinitely better than the endless failed cannula insertions we had to go through when Min was a baby, before the port was fitted.  Once it took 8 attempts by experts ... that was very hard.

So once the port has been accessed and the blood has arrived, the transfusion can start.  At the moment it takes around 5/6 hours for the pump to slowly get the blood in, at her recent Sept transfusion 325ml of blood took 5 hours and 5 minutes to transfuse.  They take observations again after 15 mins to make sure there is not an adverse reaction to the blood, and again if they switch to an additional bag of blood.

So it is a long day, and if Min wants to wander around, or do some colouring or sticking at the table in the middle, I have to be right with her with her blood on the wheely stand.  There is a play specialist who helps the kids to be entertained during a long day in hospital, and helps to distract them during procedures.  It is easy to put the tv on, but not great to have it on for most of the day.

Usually I have grabbed lunch and a drink from AMT on the way up to the ward, and Minnie will have a lunchbox 'snack pack'.  And Minnie usually has a nap.

Normally we are finished just before rush hour starts, so it is not too chaotic to get home.  The needle is removed and we are then free to go.  We also pick up her Exjade medication, which helps her to eliminate the excess iron she accumulates fro her transfusions.

So normal to us, and it is a great that it is only a day procedure.  Sure we would rather not do it, but whenever we are in hospital we see kids who have to manage much more serious medical interventions, and we are always grateful for the (relatively) normal lives we lead.

Happy Birthday Granny!

Granny celebrated a significant birthday in September, and the whole family got together in October to celebrate.

Medical Options - Part 2

So, around this time last year we wrote a blog update about Minnie's health.  We are always happy to talk about Minnie's condition if people have questions or are interested, but there is a lot of detail so it is helpful to have a way to let anybody who wants to know get up to date.

A lot happens in a year, so it seems a good time to update as we visited the haematologist yesterday.

Pyruvate Kinase Deficiency (Minnie's primary condition, AKA the blood stuff)
This year we tried to stretch Minnie out between blood transfusions, her last interval was 8 weeks.  She was grumpy, listless and struggling in the couple of weeks leading up to transfusion day, so it was no surprise that her Hb was 6 (normal is 11-13).  So, we are now going back to 6-weekly transfusions and we are comfortable with that.  Better to have a day in hospital every few weeks than for her to be short of energy and feeling rotten.  And this will likely be the state of things ongoing, maybe forever.  As we mentioned in the last update, there are no cures or other suitable treatments for her condition at the moment.

Iron Levels
Minnie accumulates iron from her transfusions which, if not managed, can settle in her organs and cause damage.  So she takes a daily medicine called Exjade, and we are upping her dose so she can get rid of her iron a bit more efficiently, especially as she will have a regular regime of transfusions again.  The medicine has some potentially nasty side effects (hence trying to get her off the transfusions to avoid the iron), but so far she has had no adverse reaction.  Long may that last ...

Ears
This summer Minnie got her cochlear implant in her right ear, and she is already feeling the benefit.  Especially when she is not anaemic, she is talking ever more clearly and accurately.  She is hearing 'sss' and 'shhh' for the first time, and although the gobbledygook is still there, it is slowly being replaced by more comprehensible words and sentences.  It is still a long road, but it is refreshing to be able to communicate ever more clearly with her, and she understands a lot.

Unfortunately Minnie also suffers from Glue Ear, and is having regular ear infections which we have to treat immediately with antibiotics due to the added risk of her implant becoming infected.  We are about to have a conversation with the surgeon about whether to not to give Minnie grommets which may alleviate the infections.

Above all, we are still so grateful that our lives are (relatively) normal, that Minnie is growing up to be a strong, bright and vivacious young lady who is certainly not held back by her ongoing care.

Halloween!!