Endocrinology – 23rd Feb

After attending our local hospital in the morning, Angel had an appointment with Dr Didi at Alder Hey in the afternoon.We have not yet received the letter for this visit but as Angel is presenting symptoms of vascular rash swollen lips ect, he wanted to have a blood test done to check that she has not got a very rare deficiency, C1 angioedema deficiency deficiency.

So what was first thought to be a quick visit of being at Alder Hey for 1 hour ended up us not leaving till 2½ hours later due us having to go have her bloods done and one sample to be picked up by emergency transport for it to be tested whilst warm at Liverpool Royal Hospital, after the bloods was done at the pathology laboratory we had to go back to see Dr Didi again for him to discuss what happens from here, which we are still waiting to hear about the C1 test and a letter from himself, was no point in discussing the test till the result is back all Bev knows that this is something to do with immunology and cant even make sense of it from researching it.

Yet again a update post will follow 

B-E-V

EEG – 23rd of Feb

Angel attended a new clinic visit at Blackpool victoria hospital to have an EEG.As many blog followers are aware, she has been having episode of microseizures and vascular flush on her face.

What is an EEG
An EEG (ElectroEncephaloGraph) records the electrical impulses that your brain produces naturally whilst sending and receiving messages from your body.

Electro – indicates the electrical impulses that are being measured.
Encephalo– the area from which we are measuring i.e. the head

Graph– this is the form in which the impulses are generated.

The test took 45mins and 23 small electrodes were placed on Angels scalp using a paste, 2 further electrodes were placed on either shoulders- to record heart rhythm.

Angel had to lay on a bed and was to follow instructions of opening and closing eyes, these were all videoed. 

Photic Stimulation next took place – Angel has to look at a bright light which flashed at various speeds with her eyes opened and then closed.

The results of the EEG are not given on the day of investigation, as the recordings have to be analysed,and referred to clinicians (in this case Audiology and Endocrinology and Oncology at Alder Hey and Dr Laycovk at Blackpool Victoria) 

An update of the findings will be posted when the results are received!! 

17th Feb – Audiology Clinic, Alderhey Children’s Hospital.

Todays was to peform futher neurotolohical examination with a video head impulse test, which indicated a borderline function of her right posterior semi-circular canal plane with reduced vestibular ocular reflex as well as saccades,she is having positive gaze evoked nystagmus, also seen was some malar flush which resembled a butterfly rash. Angel has been having microseizures which resemble otolith crisis and shortly she will be having an EEG in Blackpool, she is prone to malar flushes along with purpuric spots on her face which sometimes are quite refractory to spontaneously resolve, in 2015 she presented some dysarthria and wobbliness, she tires quite easily she gets intermittent episodes of swaying, cant tolerate bright lights and indeed challenging visual targets with features of visual vertigo.

Balance rehabilitation did not commence last visit, this time a sheet has been given for Angel to start this.

There could be a case of peripheral vestibular hypofunction or even vestibular processing dysfunction at the level of vestibular nuclei. She will require a neurological opinion and this can happen after results of EEG are known.

Yet again a new clinic to attend for tumour trooper  

    
    
   

Orthotic Clinic – Blackpool Vic 8th Feb

Angel was referred by her physiotherapist in Nov2015 to orthotics with issues relating to sore ankles, on 8th Feb she attended the orthotic clinic at her local hospital Blackpool victoria hospital.
As this is her first visit to the clinic Bev had to go through everything that has gone on with Angel since diagnosis of the tumour to the present date with all the issues of her MPHD and hyperacusis and vascular problems.

An examination was done of Angel – she had to stand on her tip toes, her arch, facing the consultant with her back to him and also her big toe was pulled upwards.

She has high arches 

So she stood with her back to him and had her foot placed normally on the floor, concerns were her kneecaps as they are rotating inwards which will have an affect on her hips and that is what is causing the pains in her ankles and feet!!

So, she has to remove all insoles in her shoes and to wear interpod flex 8arch insoles in her shoes hopefully these will help sort her kneecaps out.

They are superior shock absorbant and maintain support, as soon as they were put in her shoes she could actually tell a difference which is amazing.

She will go to review appointments at this clinic is everytime her feet grow as new sizes will be needed

Christmas Day at Church 

On Xmas day Angel was asked to do a speech to the congregation at Kings Church Blackpool, re her journey and a charity she supports, who have helped her so much through her journey, The Wills Family Foundation.So she didn’t open any of her presents she had received till after attending church.

Also Angel and Bev would like to thank Kings Church for the hamper donation we received off them – they feel truly blessed. 
   
   

Letter from Dr Didi..November 2015

Angel attended clinic with the company of her oncology nurse specialist and her clinical psychologist.

Dr Didi was taken aback by Angel being in her wheelchair, but could not stress enough how extremely well the progress she has made with the weight battle.Bev said weight was not an issue for Angel to lose,she did her research online joined a group and introduced herself, gave a brief description of Angels medical issues with her rare syndrome and all the replies came back LOW CARB DIET, so no more than 90 carbs a day and result 2 stone 6lbs was lost.

Number off issues were discussed,symptoms of tiredness,achy muscles and musculoskeletal symptoms in sort can be explained by low growth hormone,thyroxine and cortisol (stress hormone), we have worked together assiduously and made hormone replacements that have made her hormone levels appropriate for her age.It is highly likely that a number of symptoms Angel has are due to the hypothalamic syndrome.

HYPOTHALAMIC SYNDROME

Hypothalamus is an area in the central core part of the brain just above the pituitary gland, which has a very important bearing on a number of bodily functions, centres in this area control

1  Appetite

2 Handling of energy

3 Short term memory

4 General behaviour

5 Anger and tearful behaviour

6 General energy levels

7 Control of body temperature

8 Sweating

9 Circulation to the skin

Angel has bouts of tiredness,aching pains in her muscles, episodic stomach ache, problems with memory and unusual oubursts,it is highly likely these symptoms are due to he syndrome as we have good evidence her original brain tumour was prominently encroaching on the hypothalamus.

Gait,stiffness and difficulty walking upstairs

Angel is walking in an unusual way,struggles walking upstairs.She has no proximal muscle weakness and no joint swelling,has a stiff gait with very little flexion at the knee and very little spring in her walk, so will be a rheumatology assemement.

Yet again more visits and another new clinic to add to everything else

 

 

MRI UPDATE NOVEMBER 2015

We had to go to oncology for angel to have a line inserted, due to last MRI they had issues finding a vein (they are not very straight and curve) and had to send for 2 different staff to find one. So they decided next visit for her mri to have a line inserted on oncology ward in the morning ready for her MRI in the afternoon. Angel did amazing,they cant  use the numbing cream as she is allergic to plasters and they normally use freeze spray but this can make your veins go smaller, so this time they did it with nothing and she was amazing  they got the line in first attempt. We will be doing this way every time contrast is used with the MRI from now on.