Hi Ali,

I hope the physical therapy works too. I hope you hear from that hospital soon. How are you doing today? Jesse had a tough morning - he was basically exhausted this weekend after his busy week and then this morning really had a rough time going to school. He was mad at me because I made him go, but I'm afraid if I let him stay home, he won't go back. He thinks I am really mean right now - it's hard for him to understand that being active is the best medicine for him - I feel bad.

Did you have a nice weekend? I did some shopping - that was fun!

I wish you a pain free day and night.

love.
terry

Hi Terry,
I am going to Great Ormand Street Hospital on the 25th March, I am so excited, I know it will be hard work and very painful but I am hoping that I will make some progress there. A tutor is coming to see me tomorrow about home schooling.
The pain in my leg is about an 8 today
How is Jesse? I hope he is feeling a bit better. I totally understand how you are feeling about making Jesse go to school, it must be awful for you having to watch your son in so much pain. Is Jesse allowed home from school if he is in too much pain?
Take care and send Jesse my love

Hi Ali,

I'm sorry for the pain you are feeling - I wish I could say something to make you feel better, just know that I care about you and wish you pain free days and nights. That's great news about the tutor for homeschooling - it will be good to preoccupy your mind with something - even if it's school work!!

Jesse had his first session with a counselor yesterday to discuss the sadness he is feeling due to his illness. I think it went well - he will go again next week. He was better this morning and went to school without complaining. He can go to the nurse and take a rest if needed, but the doctor really wants him to stay in school unless he has a fever - he wants him to work through the pain - I don't know if i could do it.

Thank you for your good wishes for Jesse - say hi to your mum and have a good evening.

Love,
terry

Hi Terry,

Has Jesse been diagnosed with RSD?
There is a women whom attends my pain clinic with RSD/CRPS of her abdominal wall which she got following a C/section. She already has the disease in her wrist after a fracture.
She has sub cutaneous local anaesthetic run into her abdominal wall for about a week every few months and this is virtually making the pain a non event.
I wonder if this is something you could talk to the doctor about?
Good luck to your little man.
Love Tayla

Hi Tayla,

Thanks for responding to me - I really appreciate it. Jesse has been diagnosed with RND reflex neurovascular dystrophy which I think is very similar to RSD. We don't know how Jesse got this - but it is very painful - he is in constant pain. He is on a waiting list for an intense physical therapy program where he will be exercising 6 hours a day - their thought is that this will break the circuit - I hope it works. If the physical therapy doesn't work I will be sure to ask my doctor about the cutaneous local anaesthetic.

Thank you for your good wishes for Jesse - I wish them to you as well.

terry

Hi Terry,
Many thanks for your reply. It is really good that the counselor is helping Jesse (even if it is just a bit)
I heard from Great Ormond Street Hospital today, I am going to see a doctor there on the 25th March. The doctor will look at me and hopefully he will try his best to treat me. I saw the tutor today, he is really nice and helpful. I am starting home schooling on Tuesday.
Take care and tell Jesse to stay strong
Thanks

i am a new memebr and just wrote a bit on my extended biography which i thought kind of makde a link with your son's case. i too must enter into the diagnosis debate in terms of viscera and crps - thing is rsd/crps is still so unknown about and knew things and connections will be found and many of the processes may be the closest way to deacribe pain labelled under a variety of syndromes - there may be variants of complex regional pain syndrome.

i have continually had the problem of being hard to classify because my pain began with a UTI, but seemed to evolve into a kind of centralised neuropathic pain with autonomic disturbance. i have been diagnosed in america with a kind of crps but have never met anyone with the same kind of past story to me. I now have no bladder and a bag but have phantom bladder urgency continually still as well as deep searing burning pain where my bladder was, in my lower back, across my buttocks and pelvis.

other areas of my body also burn but I my lower legs and feet are like they are in ice all the time and thermography showed they were very cold. i have burning, mottled palms, poor temperature control and sweating in general, pain and soreness of my face and scalp and am exhausted from being too uncomfortable to sleep! i get a lot of headaches and tingling and have sensory changes which feel both numb and hypersensitive in my lower legs. i have tried almost every drug i think and have had endless nerve blocks to no avail and a failed attempt to relieve pain with a spinal cord stimulator.

have sought help from doctors here and in florida in the past. have tried endless alternative therapies and as a herbalist use herbs to support but they don't relieve the level of pain. am desperate and seeking info also on the ketamine comas trials and trying to seek advice from experts in america. Nearly 12 years on and with all the determination to get a first class degree and MPhil studentship afer years house and 'bathroom' bound (pre-bladder removal) and I feel I am worse if anything and don't know how to bear each minute and continue a life with the pain and lack of ever having respite. i feel really isolated in terms of my symptom profile and my symptoms may be seen as a kind of interstitial cystitis by some except there is no organ and the centralised changes are obvious. I know there are some suggestions that some kinds of interstitial cystitis are a form of CRPS. to an extent i don't care but it just makes me more lonely and would also love to here if anyone has this kind of burning in their trunk, back, viscera or similarities with my story to share.

i have been so positive and independnet the last few years trying to ignore and distract from the pain but it just isn't sustainable for me anymore and i don't know how to go on making so much mental effort etc to simply endure more pain despite all i cherish and love. i am terrified what is going to happen to me as each day feels so hard and i can cope with and do less and less as the pain and various symptoms all over seem to worsen.

i hope that something works for your son and you get some compassionate and openminded support from doctors.
bestest wishes,
SarahB

Hi Sarah,
Thank you so much for contacting me and wishing my son well. I am sorry for all that you have gone through and are still going through. Have you contacted Dr. Schwartzman in Philadelphia? I think he is doing a ketamine study - I think he is with Drexel University - I know he also sends people to Germany to get the Ketamine Coma - this might be something you'll want to look into. I saw information somewhere on Neurotalk - I'm just not sure where - you might want to start a thread asking about it - I'm sure somebody will have more information.

My son has recently been diagnosed with Reflex Neurovascular Dystophy. We have stopped all medication and are on the waiting list for an intense physical therapy program at Children's Hospital of Philadelphia. I finally got him back to school - but it is a struggle on Monday's after having the weekend to sleep in. I hate forcing him to go, but the doctors feel it is a good distraction. I am just happy to have him be with his friends again.

I wish you well.

Terry

Thank

Terry,

Has your son been tested for AIP (Acute Intermittent Porphyria)???

Here is some info on this,

Acute Intermittent Porphyria (AIP)

Urine can have this appearance during
an attack or upon standing in the light.
This is one of the hereditary hepatic porphyrias. Its inheritance is autosomal dominant. The deficient enzyme is porphobilinogen deaminase (PBGD), also known as hydroxymethylbilane synthase. This enzyme was formerly known as uroporphyrinogen I-synthase, and this term is still used by some clinical laboratories. A deficiency of PBGD is not sufficient by itself to produce AIP, and other activating factors must also be present. These include hormones, drugs and dietary changes. Sometimes, activating factors cannot be identified.

Symptoms
Most people who inherit the gene for AIP never develop symptoms. AIP manifests after puberty, especially in women (due to hormonal influences). Symptoms usually occur as attacks that develop over several hours or days. Abdominal pain, which can be severe, is the most common symptom. Others may include:

nausea

vomiting

constipation

pain in the back, arms and legs

muscle weakness (due to effects on nerves supplying the muscles)

urinary retention

palpitation (due to a rapid heart rate and often accompanied by increased blood pressure)

confusion, hallucinations and seizures

Sometimes the level of salt (sodium and chloride) in the blood decreases markedly and contributes to some of these symptoms. The skin is not affected.

Diagnosis
Because this disease is rare and can mimic a host of other more common conditions, its presence is often not suspected. On the other hand, the diagnosis of AIP and other types of porphyria is sometimes made incorrectly in patients who do not have porphyria at all, particularly if laboratory tests are improperly done or misinterpreted. The finding of increased levels of delta-aminolevulinic acid (ALA) and porphobilinogen (PBG) in urine establishes that one of the acute porphyrias is present. If PBGD is deficient in normal red blood cells then the diagnosis of AIP is established. However, measuring PBGD in red blood cells should not be relied upon by itself to exclude AIP in a sick patient, because the enzyme is not deficient in red blood cells of all AIP patients.

If it is known that someone in a family has AIP and their enzyme value is low in red blood cells, other family Sponsors who have inherited a deficiency of PBGD can be identified by measuring the enzyme in their red blood cells. Latent cases so identified can avoid agents known to cause attacks. However, in some AIP families, PBGD is normal in red blood cells and is deficient only in the liver and other tissues. Falsely low values sometimes occur due to problems with collecting and transporting the sample.

DNA is the material in cells that encodes all the genetic information of an individual. Many different mutations have been identified in the portion of DNA that comprises the gene for PBGD. Almost every family with AIP has a different mutation in this gene. Within one family, however, everyone who inherits a deficiency of PBGD has the same mutation. It is advantageous to know the precise mutation in a family, because that knowledge enables the identification of AIP gene carriers by DNA testing. This approach is much more precise than measuring PBGD enzyme activity in red blood cells. At present, DNA testing for AIP and other porphyrias is available only through a few research laboratories.

Treatment and Prognosis
Hospitalization is often necessary for acute attacks. Medications for pain, nausea and vomiting, and close observation are generally required.

A high intake of glucose or other carbohydrates can help suppress disease activity and can be given by vein or by mouth. Intravenous heme therapy is more potent in suppressing disease activity. It can be started after a trial of glucose therapy. However, the response to heme therapy is best if started early in an attack. Therefore, delaying heme therapy until glucose alone has not been effective may not be warranted unless an attack is mild.

Heme must be administered by vein. Panhematin®, from Ovation Pharmaceuticals, Inc., is the only commercially available heme therapy for treatment and prevention of acute porphyric attacks in the United States. Heme arginate, which is marketed in some other countries, is another preparation of heme for intravenous administration. It is however, not presently available in the United States. Panhematin® is less likely to produce phlebitis if it is mixed with human albumin before it is given. (Directions for preparing Panhematin® in this manner can be obtained from porphyria specialists.) Heme therapy is seldom indicated unless the diagnosis of acute porphyria is proven by a marked increase in urine PBG. How heme therapy should be used to prevent attacks is not well established.

During treatment of an attack, attention should be given to salt and water balance. Harmful drugs should be stopped. These include barbiturates, sulfonamides, and many others. Attacks are often precipitated by low intake of carbohydrates and calories in an attempt to lose weight. Thus dietary counseling is very important (see below). Premenstrual attacks often resolve quickly with the onset of menses; hormone manipulations may prevent such attacks.

AIP is particularly dangerous if the diagnosis has not been made and if harmful drugs are administered. The prognosis is usually good if the disease is recognized and if treatment and preventive measures are begun before severe nerve damage has occurred. Although symptoms usually resolve after an attack, some patients develop chronic pain. Nerve damage and associated muscle weakness can improve over a period of months or longer after a severe attack. Mental symptoms may occur during attacks, but are usually not chronic.

Wearing a Medic Alert bracelet is advisable for patients who have had attacks but is probably not warranted in most latent cases. It should be remembered that AIP patients can develop other diseases, and symptoms may not always be due to porphyria.

Diet
AIP patients prone to attacks should eat a normal or high carbohydrate diet and should not greatly restrict their intakes of carbohydrate and calories, even for short periods of time. If weight loss is desired, it is advisable to consult a physician who may then request that a dietitian estimate an individual's normal caloric intake, which varies greatly from one person to another. Then it may be appropriate to prescribe a diet that is approximately 10% below the normal level of calories for the patient. This should result in a gradual weight loss and usually will not cause an attack of porphyria.




I was diagnosed 15 years ago with a mild form of this and flares up with certian foods and just when ever i feel good it seems to pop up. Mind you i have a very mild form and still was in horrible pain there is light at the end of the tunnel.. I tested postive to one of the 2 tests that detects AIP. Any questions ask away!!

Amber

Hi Amber,
Thank you so much for the information - I had never heard of that. Now it is time for me to research that as well. I tell you, this site has been a blessing - thank you so much!

I wish you pain free days and nights.

Terry