Thankyou for all your support and replys to our e-mail that we
posted. We have learnt so much and a special thankyou to karen
durrant, Colin bailey, Karen maxwell, Dori, Mellhoney and families.
As we are now gathering our thoughts and taking action on all your
needed and knowledgable advice, and now we know we are not alone.
If it is not to much to ask, could you please if you find the time
read fynleys medical diary to see if any one of the symptons are
familiar as we have had the genetic results back which are negative
so we feel a little bit more confused. We would appreciate any feed
back. thankyou lisa and colin x


FYNLEY SMITH MEDICAL DIARY


Fynley smith is now 14 months old and from the 29/10/04 has been
having all these symptoms:
• Spiking temperatures (highest recorded 40.3) (lowest
recorded 34.8)
• constant Rigors and 1 fibril convulsion
• Meningitis
• Pneumonia
• Rota virus
• Odd rashes,
• Loss of appetite/ does not eat food at all
• Joint swelling, of knees and elbows
• high inflammatory markers the highest recorded WCC 30.1 and
CRP 186
• He also has obvious joint pain in neck right arm, wrist and
shoulder, head.
• More recently he has had a rash that has travelled around
his body, form his elbows to his knees, back of legs, back and face.
• Strep in his throat
• Oral thrush and ulcers
• Acute pain (from unknown internal source)
The episodes always seem to follow a pattern which is:
DAY ONE one episode of diaohrea, and then that night would start
running a high temperature that we would treat with paracetemol and
nurofen, at first the temperatures would make him vomit his milk He
would always get an isolated rash on he's right collar bone that
would go deep red and look like he had been scolded, this mark would
always go away as he got better and started becoming an indication
to us as parents that he was going down hill again, he would also
have rashes that appeared under his chin round his neck.
DAY TWO Spiking temperatures of (40) with rigors, he would be
whimpering and often seemed to have flu like symptoms with a clear
runny nose and hurt when moved a round. His temperatures/ rigors
and obvious discomfort would always end with us having to go into
hospital with him, where his bloods were taking and he always had
high inflammatory markers. At night he would seem to get worse and
would only sleep for 1/2 hours at a time.
DAY THREE His temperatures would still be high (39) rigors were less
but still in a lot of discomfort, with flu like symptoms that went
on through the night
DAY FOUR the spiking temperature would become less frequent and the
rashes would start to fade he would start to play a bit and look
better
DAY FIVE the temperatures would fade away and normally be stopped by
the evening.
He was unwell twice with similar symptoms in September and October
but was not as severe and he was first taken into hospital in
October.
On the 24/04/04 Fynley, was also taken into hospital when he was 2
and a 1/2 weeks old with a rash that was diagnosed as erthyma.
On the 14/09/04 he went to the doctors due to an acutely swollen
left leg and knee, which they diagnosed as a sting of some sort.
He had various cold and chest complaints, with high fevers in the
first six months of his life and rashes that started when he was
five months that were put down to excma, teething and dermatitis.
The rashes use to drive him mad and he would be rubbing his feet all
night or scratching at his chin, neck, arms all day. In January he
stopped using his right arm and use to tuck it away like a broken
wing he would use it only when really necessary, since he has been
on voltarol his rashes and arm have been minimal.
Fynleys many symptoms have varied but he has consistently from six
months had high fevers, rigors, high inflammatory markers and
obvious acute pain, which is now believed to be sterile meningitis
(inflammation of the meninges)
He has a number of blood tests, bone scan, bone marrow, kidney scan,
10 x-rays which have all been inconclusive at this point.
He seems to have a problem with his right upper side when having an
episode, he has abnormal arm and shoulder movements, he twists his
arm and pushes down on the back of his wrists, his posture changes
and slumps forward to the right side. And seems in acute pain. In
the last eight weeks Fynley has been having pain break through,
through the night which are getting more and more severe, he can
wake up to 10 times a night i.e. every half hour rigid and screaming
in pain, he has to be picked up and body rubbed and manoeuvred until
he seems to relax and then is able to go back to sleep.
Fynley has been seen by a number of doctors, paediatricians and now
is under a paediatric rheumatologist. Dr Hull, from the queen
Alexander hospital, Portsmouth.
Dr hull has studied fynleys symptoms and has diagnosed (CINCA)
Chronic, Infantile, Neurological, Coetaneous, and Articular
syndrome. Which is a progressive multisystem inflammatory
disease.
Fynley is acutely ill and when not in hospital still have a lot of
problems due to pain from the irritation on the meninges, especially
through the night, and very often has to have morphine to relive the
pain. CINCA is a progressive multi-inflammatory disease that is
very rare, Dr Hull is now liasing with Professor Woo in Great Ormond
Street about the management of Fynleys illness, and fynleys medical
team are doing their very best to try to control the inflammatory
and pain, as there is no cure, and it is progressive. with a
variable long term prognosis.
.
Hospital & home episodes listed below:
1. 29/10/ 04 where he was treated for meningitis had joint
swelling on day 3 of the episode, his WCC and CRP was high
2. 20/12/04 and was treated for meningitis again again his WCC
and CRP was very high
3. 02/01/05 same symptoms and high markers wasn't treated with
antibiotics and returned to normal after following the same pattern
4. 18/01/05 same pattern (pyrexia of unknown origin)
5. 25/01/05 rota virus
6. 04/02/05 observation due to pain high and low temperatures
(07/02/05 pneumonia and ear infection diagnosed)
7. 19/02/05 same pattern of pyrexia but had new symptoms which
are - obvious severe joint pain- rashes that moved around - very hot
swollen joints (picture/video evidence available) voltarol
prescribed to use on a daily basis
8. 04/03/05 same symptoms temps/rigors joint pain, flu
symptoms/ rashes but did not take into hospital kept home and gave
voltarol 1 mg three times a day and paracetemol
9. 31/03/05 rushed into hospital after rigors all day, that got
so bad, voltarol dose upped to 1.5 mg three times a day, oral
morphine given on the 1st of April for pain relief
10. 13/04/05 went down hill rapidly and was in hospital within
two hours of spiking temperature and rigors occurring, was given
oral morph for pain and 250ml of paracetemol, fynleys `s temp was
still 40 and heart rate 220 bpm. Doctors have now decided he has
cerible irritation, (meningism, sterile meningitis) which is causing
acute pain. Prescribed steroids on alternate day basis 10mls
11. 04/05/05 spiked at 4.15am gave paracetemol 240mls and
voltarol went into hospital and spiked again at 9.15am Temp 40 and
heart rate 244 bpm. Was given morphine and more paracetemol at
10.30. 12pm Fynley had settled and had a relatively good night,
normal drugs proceeded. Bloods taken for DNA cinca test and
inflammatory markers, CRP was 120 went home that day.
12. 18/05/05 woke at 12.30am hot, woke again vomiting at 2.30
spiked gave paracetemol 240ml and 10mg of prednisolone, as advised
by doctor hull. Heart rate up vomited again at 4.00 am, woke at 6am
screaming gave morphine. 7.30 gave voltarol spoke to Simon Birch
(paediatrician) for advice and at 9.30 gave routine 10mg
prednisolone, He slept for 4 hours and then seemed more settled with
his temp going to 34.5. Did not take into hospital. Please go to
updated episodes.

Fynleys Medication
• Voltarol 1.5ml three times a day
• Prednisolone (steroid) 10mls every other day
• Prednisolone (steroid) an extra 10mgs when episodes arise
• Paracetemol 250mls three times a day for pain and temp (when
needed)
• Oral morph 2mls six times a day for head pain (when needed)
• Ranitidine 2mls (30mg) twice a day
• Daktarin oral gel for ulcerated mouth every day
Fynley was on polycal in formula milk and now has 600ml of fortini
multi fibre milk a day, as he does not tolerate food and is only
just holding on to his weight.
Fynleys medical team
G.P Dr Rogers, Baffin's surgery, Portsmouth
Health visitor Marion Owen, Lake rd health centre
Paediatrician Simon Birch, St Mary Hospital, Portsmouth
Rheumatologist Dr Hull, Queen Alexander Hospital, Portsmouth
Paediatric Dietician Angela Johnson, St Mary Hospital, Portsmouth
Eye specialist Dr Evans, Queen Alexander Hospital
Social worker Lyn Callaway,
Consultant community paediatrician Jo Lewis, Northern parade
clinic Portsmouth
Community Nurse Susan, Admiral house Cosham
Ear specialist Mr Thomas, Queen Alexander Hospital
Physiotherapist to be arranged
Speech therapist to be arranged
Occupational therapist to be arranged
Psychologist to be arranged

UPDATED EPISODES, MEDICATION AND PROGRESS OF FYNLEY
19/05/05 fynleys rygals started again and spiked another temp was in
obvious pain was given the an extra dose of steroids, 250ml of
paracetemol, usual voltarol and due to pain morphine.
27/05/05 Fynley has been in pain every day at some point since his
last episode which started on the 18th and has had to have a dose of
morphine to help with the pain at various times through the day and
night for the past 9 days. It has also been evident that his right
hand has now took a different shape and seems to curl inward most of
the time like he has some form of cramp, but it does not seem to
bother him. He is also twisting when he walks. We see Dr hull and
Dr barker who suggested that the next time an episode start to give
him steroids for five days continuous to see if this helps. We also
see Jo lewis the paediatric community doctor who is going to arrange
for a MRI scan, as she seems to think he may be having spasms in the
brain due to the inflammation.
04/06/05 Fynley has had another episode that started at 2.30pm with
rigors as usual, we immediately started him on steroids as suggested
by Dr Hull and will keep him on it for five days which is usually
the length of his episodes before going back to his alternate day
maintenance episode of 10 mgs of prednisolone every other day.
11/06/05 Fynley has had flu like symptoms, and had diahorea for five
days his temperature has been very low usually under 35.
20/06/05 Fynley started whimpering through the night on the 16th, on
the next night the 17th he was crying in pain at varies times
through the night, On the 18th he was irritable temp up to 37.1 and
was twisting and pulling arms up through the day also pulling at his
wrists, He started screaming with head locked back in usual position
about 11 pm and that carried on through the nights at staggered
intervals. The 19th Fynley was much the same all day and through
the night he again awoke in extreme pain with head locked back could
not be pacified and we had to administer morphine which quietened
him down, but we could not lay him back in a sleeping position as he
would just start screaming again, after two hours we decided to
sleep him in an upright position in his buggy and he did manage a
few hours of sleep. But awoke grunting and temp was up to 38.8 his
heart rate was again extremely high, we gave him an extra dose of 10
mg prednisolone and will now keep him on it for five days. He also
had his usual voltarol, which is every 8 hours and 250mls of
suppository paracetemol for his pyrexia. He has been still pulling
up with his arms, and whimpering through the day, especially when
his voltarol is wearing off.