Krsto and Urticaria Pigmentosa

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Index 1998 - 2000

First Spots
CT Scan
Head Surgery

Our son Krsto was born on December 5th 1997, having 3520 g / 50 cm, as a healthy child.

December 1997 – July 1998
First spots

Krsto got his first spot on forehead when he was 2 months old and we thought it was a mark as we all have on our skin. In a few next months more of them appeared all over his body. There are not so many of them - let's say 30-40. Our pediatrician didn't know what it may be so we went to a dermatologist who suspected (99%, she said) it was UP. My wife Brigitte is a doctor (works as an anesthesiologist.) but she had no idea what it would be as it is a rare illness mentioned in a few lines only in her books; what we have read wasn't good at all as it said the prognosis are poor. We were shocked at the moment wondering what have we done wrong to be punished in such a way.

Afterwards I found The Mastocytosis Society on the Internet and there I have learned everything I know about the illness. It’s address is:

A few days later Brigitte made an appointment with her professor, Dr. Dobric, from the University in Zagreb who is dermatologist specialised in histology. That is, if we did the biopsy in our hospital, they would send the skin to Dr. Dobric who would analyse it and send the results back here. That's why we wished him to see Krsto first, what he actually did. He also confirmed it may be UP but did not suggest the biopsy as Krsto feels OK and is a happy little boy, and knowing 100% he has UP would change nothing.

August 1998

At the beginning of August we went to the island of Krk, the greatest of all Adriatic islands. Water temperatures were so high that I haven't heard of before - up to 28 degrees Celsius and it was impossible for one to cool himself in such a warm water. Unfortunately, the day before we came a wind called 'bura' started to blow and cooled the sea; we didn't get into the water the first few days, but after the sun did its job well and it was again pleasant to swim.

Krsto overslept the trip which took 5,5 hours (from 2 in the night till 7:30 in the morning). Although it was hot in the sun, we spent most of the time in a shade where we found a nice breeze all day long. The worst was from noon till 4 PM when it was awfully hot. Krsto got a swimming pool into which we put the seawater and he seemed to like it. Twice we noticed some reddish skin on his head (scalp) and upper arm, but Brigitte said it must be some kind of an allergy to the sun and not connected with UP.

It was OK until our last 2-3 days (out of 7, as we stayed there) when he couldn't eat properly; was it because of the heat or something else, I can't tell. It's not that he didn't eat at all, no - he just didn't eat his regular amounts of food. Eating normalised a few days after we returned home.

It was our unlucky guess when to go there because it was a Krk fair which lasted for 3 days. It included huge amounts of food (whatever you like - from pork to lamb to fish to ... you name it, they have it), toys, clothes, shoes, paintings, sculptures, jewellery, etc. etc. The worst were the nights - there was music all night long, and our autocamp was just opposite the loudspeakers. It was really hard to fall asleep these days. The only important thing for Krsto was just to have his mother next to him and he slept well.

We walked through the pine forest which covers, let's say, 50 percent of autocamp. Krsto mostly liked it because it was never boring there as somebody always does something interesting.

As far as we could notice, no new spots appeared while we were there and we didn't see Krsto having any kind of health trouble, itching or something. Luckily, there are no mosquitoes in Krk; we left those monsters back home. Wasps flied around looking for a juice or a fruit, were boring but nothing we couldn't manage. Krsto wasn't exposed to direct sun; however, we used sunprotective cream with factor 15 and he got a nice tan during the vacation.

Autumn 1998

During the Autumn (beginning of October) Krsto learned how to walk. He had already overgrown two pairs of shoes and soon he'll need another one. We didn't know what will happen when he started to walk as it was inevitable for him to fall down - will he suffer of some kind of injuries, how will they reflect to UP, ...? Luckily, we didn't notice anything strange with his skin.

Winter 1998/99

During the Winter temperatures fell down to -15 degrees Celsius; we tried not to take him out when it was so cold, but once a day he takes a 10-minutes walk just to catch some fresh air. We didn't notice any consequences due to the cold weather either.

Food (generally) 1999

He eats all kinds of solid food - meat, fruit and vegetables, soups, cakes, milk, juices, ... we give him all food we eat.

March 1999
CT Scan

Krsto has a growth between left eye and nose. Our pediatrician couldn't tell what it was, so we went to Zagreb. Bridget's aunt is a psychiatrist (she's retired now) and she called her friend, pediatrician, to arrange an appointment in a hospital for children diseases. She didn't know what it might be either, but she sent us to another doctor - otorinolaringologist, who said it isn't his zone of interest because there are muscles that are used by an eye and he couldn't ever do a surgery because he might hurt them and damage an eye. He sent us to another hospital, a Clinic, and another doctor - ophthalmologist. It was an old doctor who admitted he had no idea what it might be and sent us to his college, a young woman doctor, dr. Ivekovic who said it might be a teratom or osteom (don't know if you know them by those names); teratom is a kind of a tumor that is developed in a human body from embryo cells; osteom is a growth from one's bone and is much worse than teratom as it might have a malign form. However, she couldn't tell it for sure before she did some tests.

So, we went home and did blood tests (which were normal) and EKG (normal as well). Those tests were necessary because Krsto had to go to CT which is done under the general anesthesia (if it is done to a child). As you probably know, it might be hazardous because drugs used during anesthesia can evoke potentially fatal reactions in a masto patient (Krsto has UP).

A week later we returned to a Clinic. We brought an excerpt (regarding anesthesia and masto patients) from the material Joe Palk sent hoping it might help doctors during CT. Although it was all arranged, the next morning we found out an anesthesiologist who was supposed to be there went home (?!?) and some other doctor came and he had no idea how to deal with a masto patient. It was quite unpleasant for Krsto as he wasn't allowed to eat or drink anything before a CT was done; and they didn't want to do a scan! We were told we should return some other day as if it was simple as that to travel more than 250 km with a small child, to make him starve for hours, to make him quiet and be still in a hospital … I don't know about other children, but it is impossible for me to explain such things to a 15 months old child.

It took them more than an hour to think over and take Krsto in. We, of course, had to stay in a waiting room thinking what was going on behind the closed door and praying everything will turn out good. Can't tell what worried me more - how will he survive anesthesia or what will they find out of that growth. Only Brigitte knows how she felt as she is an anesthesiologist and she knew what they might be doing to our son; she said "they must be choking him" (by the means of a halothan, a gas they use to put a patient asleep as they were afraid to use another drugs). Some 20-25 minutes later they brought him out and he was awake, dizzy a bit but alive! Thanks God! Krsto didn't suffer from any adverse reaction at the moment of anesthesia or ever after; I guess it's because they didn't apply any drugs but a gas.

A radiologist was so kind and he came out to tell us what he saw - he saw scan pictures at the screen but it was too fast to diagnose anything so he asked us to return later that day.

We did return - after he saw pictures he made, he told us it is a growth that doesn't have any connection with a bone or an eye - it "floats" in between and he thinks it might be a dermoid cyst. It is "something" Krsto was born with and it started growing; by now, it is some 4-5 mm in diameter. It can't have a malign form, which is the most important to me!

Having such news we went to dr. Ivekovic. She first asked who told us such things, and when we told her, she replied: "Oh, OK, if it was him, I believe that". However, she thinks it might grow further and push an eye to a side which might be harmful for Krsto's sight. Therefore a surgery is essential to make sure it won't happen. As anesthesiologists told her there must be a time space of 3 months before a child is taken into a general anesthesia for the next time, we have to call her at the beginning of a June to make another appointment. Until then, we'll watch the growth carefully to react sooner if necessary.

Another excerpt from Joe Palk's material says:

"(…) During our study of pediatric mastocytosis, the synthetic narcotic meperidine was a constituent of a sedative used prior to diagnostic and investigational procedures. This synthetic narcotic has been reported to be safely used in patients with mastocytosis. One child with urticaria pigmentosa who received the medication developed extensive bullae without other symptoms of mast cell degranulation 2h after receiving the meperidine-containing sedative. As we could not rule out an adverse reaction to the synthetic narcotic, we no longer routinely use it but now use intravenously administered lorazepam, a short-acting benzodiazepam. This sedative has not resulted in any adverse reactions to date in our patient population. We believe that routine use of narcotics should be avoided if possible in this patient population. If they must be used, caution and close observation are essential. (…)"

Anesthesiologist who put Krsto asleep told us they don't have either meperidine or lorazepam, so we will have to order it (lorazepam, that is) from abroad. I guess it won't be a problem, no matter what it costs.

November 1999
Head surgery

Keeping in mind it was Summer since we last visited a doctor and relatively high temperatures that can raise up to 37 degrees Celsius, we decided not to do the surgery during the Summer months of the year, but in Autumn.

It was a Monday in mid-October when we first returned to Zagreb. Krsto felt quite well during the weekend, but he caught some cold and started sneezing on Sunday evening. Anesthesiologist refused to take him to the surgery so we were forced to return home and wait for him to be healthy again.

The surgery was done four weeks later – it was Friday, November 12th. The weather was awful – snow which started falling during the night paralysed the traffic so, instead about 8 o’clock AM, medical stuff appeared almost an hour later. We brought Krsto downstairs and left him with a nurse who prepared him for the surgery. Almost hour and a half later, anesthesiologist brought Krsto back – he was still dizzy, but when he recognised us, never wanted to sleep again. Instead, he was rather sitting and watching another kid playing around.

What could we notice? He had a tiny bandage over his left eye, but he still could see under it. In a vein on an ankle was a needle that was used for infusion during the surgery. They said the wound was bleeding much so he got a lot of blood. Furthermore, we were told everything was OK (he was again put asleep by halothan) and all the time oxygen level was normal, just like his heart beat. Dr. Ivekovic thought the cyst was removed completely and the patohistological examination should show nothing strange.

We stayed in the hospital for another 4 hours waiting for Krsto to completely wake up. We were not forced to stay there as Bridget is an anesthesiologist and she know how to deal with such patients in a case of an urgency.

In spite of bad weather, we returned home – it took us more than four hours to pass 120 km; it was still snowing so we reached our hometown at 7 PM. Krsto was much too exhausted and overslept the complete journey.

A month later we finally got the results – it really was a dermoid cyst and it was completely removed, including its envelope (if some of it stayed inside the body, it might develop into something “strange” again).

In February 2000 Krsto feels OK – he learned how to speak and makes us laugh every now and then. A scarf is still visible if you look closely; otherwise, it seems to be hidden just under his eye bow.

January 2000

It seems Krsto has an allergy reaction to some kinds of food, especially chocolate (Nutella, Milky Way, etc.). We first noticed some tiny red spots on his right arm which didn’t disappear but spread all over it; next it showed on his left chick, the other arm and both legs.

We used two types of a cream – first (mild) one didn’t help much, so we started using the one containing kortikosteroids (sp?). It was successful and the allergy disappeared.

As a mean of anti-scratching drug, we used liquid powder which we had to spread over his skin.