Rare Disease Day 2012

Elliot on the top of a snow hill

After a long waiting the winter finally arrived to Stockholm for a couple of weeks, but now it seems like we're heading for springtime.

At our last visit to the hospital Elliot met a pshysioterapeut and last week we met her again and this time Elliot did some exercises that he enjoyed. He also got some exercises to do at home.

From now on it is the pshysioterapeut that will handle the 6-minutes walking test and she pushed Elliot (in a positive sense) to preform really well and he walked 455 meters! Unfortunately the saturation and pulse meter was broken so the pulse had to be taken manually. We have also met Elliot’s doctor for the usual examination (EKG and cardiac ultrasound) and the doctor couldn't see any changes, which we consider positive.

Elliot with physioterapeut

The day after tomorrow it is Rare Disease Day, but the Swedish patient association for PAH, which I now am chairman of, will not arrange any event. There has been a lot of administration with the association and not enough time to prepare. But I will attend a conference arranged by the association Rare Diseases Sweden on February 29.

A busy fall

The past weekend I was in Vienna for a meeting with PH Europe. We were discussing the PH White Paper that PHA Europe has taken initiative to. We were also planning for the forthcoming Rare Disease Day events on February 29, 2012. Another issue on the agenda was the web site Time Matters, a campaign with the purpose to inform about and to create awareness about PH: http://phtimematters.org. Please visit the site and share your story!

Last week we went to the hospital for the 6-MWT and for the third time in a row he walked 411 meters. But compared to his last visit he wasn't that tired after the walk. For the first time he meet a pshysioterapeut, this first meeting the terapeut was asking questions but next time Elliot will do different kinds of tests. Elliots headache that I mentioned in the last blog message is better now since we reduced his Revatio dose from 3 pills a day to 2 pills a day.

This fall I have been working about one day a week with the Swedish PH association. This has been very fruitful and I have meet PH specialists, people from the pharmacy industry and I have got in contacts with patients. During these months I have learnt a lot about PH and about managing an association. This work gives me energy!

The kids are asking where the winter is, in Stockholm, Sweden, we haven't seen a snow flake yet and that is unusual.

I wish you all a Happy Christmas!

Past weeks have been terrible and we need to find a solution

Elliot loses his temper far too many times a day. The most critical point is during the afternoon and almost every day he gets a big outburst when we get him from school. I don’t know how much of it that is connected to Elliot’s disease but I suppose the disease is a key factor.

Facts

• Elliot is tired after a day in school
• It’s getting colder and darker. In Sweden the temperature right now is about 3-5 degrees Celsius and it is dark when we wake up and it is getting darker every day.
• Elliot’s dose of Revatio has been doubled since our last visit at the PH-doctor.

If you have experiences that you would like to share or suggestions of how we can improve our situation please comment.  


Update: Elliot has been complaining about headace the past week and one of many potential side effects of Revatio is headace. Elliot today said that the headace easily gets him in a bad mood. 

Hospital visit

Elliot has been going to school for about 1 month now and he really likes it. Compared to pre school the school days are more intensive and when it is time to go home he often is tired. The school have bought a wagon (the type you have behind a bicycle) that Elliot can use if he gets tired when they are going for longer excursions.

Today we have been at the hospital for a routine check. The 6-minutes walking test was okey and Elliot did walk 411 metres but he was tired afterwards. The examination by the doctor did not show any changes compared to the recent visits. It was decided that the Revatio dose should be increased.

I told the doctor and the PH-nurse about my involvment in the Swedish patient association and did handover information material. I also mentioned the result presented of Novartis about the cancer drug Imatinib at ERS in Amsterdam 26th of September. The drug have proven positive on PH patients regarding the 6 minutes walking test.

Thank you!

Last week I spent some fantastic days in Castelldelfels, 25 km, south of Barcelona, Spain. The reason why I was there was the General Annual Meeting of PHA Europe. I was invited as a member of PHA Sweden together with about 50 other members representing 20 countries in Europe (including Russia and Israel).

The meeting was very well arranged with presentations from leading PH-specialists, PH-nurses and the pharmacy industry. But the most important was the meetings with all the fantastic people that participated and who told me their stories about PAH. Many were living with the disease and quite a few had undergone double lung transplant and others had, like me, family members and friends with the disease.

After my return to Sweden I have been filled with lust and energy to work with PHA Sweden and I have decided that I will work about one day a week with the association from now on.

School start

All summer Elliot has been talking about the school start. "I'm very excited about it, but I think it is also a bit scarry", he has been saying. Now he has been at school for two weeks and made new friends and he is very positive about the school in general. Unfortunately he fell from a jungle gym (klätterställning) in the playground at school last week and got a fracture in his collar bone. He is in pain but he adopted the new situation quit well and now he uses his left arm insteed.

Together with the school staff we have been discussing how they can support Elliot when they are going on excursions and they proposed that the school could buy a cart where he can sit when his legs get tired. 

The first week Marie and Elliot told about Elliots disease for the other children in his group (about 50) and that Elliot probably will have special treatment when they go on excursions and that me must stop playing to catch his breath more often than other children. 

My big day

Together with 35 other families with children with life-threatening diseases we were invited to a motor racing event arranged by the foundation Min stora dag (My big day). Sunday the 21st of August was the day for the event and Elliot had been longing for this day all summer and I must say it was an amazing arrangement. About 60 racing cars, police cars and fire trucks came to the drag racing arena in Tierp.

The cars did race in pairs and in every racing car a child was in the passenger seat. Elliot tried Lambogini, Ferrari, Porsche, BMW, Mini and Morgan. There was also a racing show and different competitions. This is a day that Elliot will remember for the rest of his life.

411 metres

Two weeks ago I and Elliot went to the hospital for the 6-minutes walking test. We had been talking to Elliot about the test and that it is important that he walkes as many metres as he can. The thing is that Elliot likes to compete and this time really tried. He walked longer than he ever done before and afterwards he was tired. His saturation was as low as 84, but after two minutes it was up at 97. I think it is strange that the pulse was low after immediately after the test, I suspect that these figures aren't correct.

Click at the picture to enlarge it.

At the playground

I'm with Elliot and two of his friends at an enormous indoor playground. The kids have a tempo and Elliot tries to keep up but it is difficult for him.




This week we have made a visit to the school Elliot will begin after summer. Elliot says it feels both a bit creepy and exciting. I'm sure that he will do just fine. Monday I and Marie will meet the teachers and staff and inform them about Elliots disease. You who have kids at school with PAH, what are your experiences?

Wow, we have a car

Finally we have bought a car! The first car trip went to Lasse Åbergs museum north of Stockholm and in the picture you can see Elliot in front of his favourite super hero Spider man. Elliot has been sick in chicken pox for the past week so he was very happy to get out of the apartment.

Today was the first day at nursery school for Ida our baby girl. She will go at the same school as her brothers.

Public performance

March 16th I am invited to speak about beeing a parent to a child with PAH on a event arranged by the Swedish PAH association. I'm looking forward to the event and the possibility to meet new connections who are engaged in the disease.

You will find more information about the event at: www.pah-sverige.se (in Swedish)

Chest pain and PAH?

Elliot is more aware of his disease and for the first time he doesn't want to take his medicines without us arguing with him. He often wants us to read a book about a girl who goes through the same kind of heart surgery that he did. In the book the surgery is performed by a doctor named Torsten and every time we read for him he says: “the doctor that mended my heart, his name is also Torsten”. And that is correct, it is the same doctor. Carolinas hjärta (Carolina’s heart) is the title of the book and I can recommend it if you know Swedish.
Quit often he says: "can you feel how much my heart is pounding". During our Christmas holiday Elliot said that it is aching in his chest, when we asked him if he had felt pain in the chest before he said yes but he also said that he deliberately had not told us so that we would not worry. When we asked Elliott’s doctor about this he told us that PAH patients can feel chest pains but that it is not common. Does anybody have similar experiences?
 Otherwise it is status quo, which is positive. Mentally he is preparing for school that starts this fall. He is curious and a bit nervous as most kids are.

Tired and angry



Spider-man

 Right now we're quit tired as a family. Elliot has been tired lately and when he is tired he can't control his temper and he explodes several times a day. This is frustrating for me and Marie, we try not to be dragged along, not to lose our temper, but it is difficult.
Last week we were at the hospital for Elliot routine control and the condition test result was similar to the two latest tests. This was positive because I feared that the test would show that he had become worse. We talked to Elliots doctor about two medicins that one of my readers had mentioned: Letairis (ambrisenten) and Tyvaso (treprostinil). The doctor was uncertain if they were available on the Swedish marked but he said that he would check it out.

Back in town

Back in town after two months in our cottage. It is always kind of strange to come back to Stockholm after being in the countryside. While we are in the cottage life is simple and without routines, we wake up and if it is nice weather, perhaps we go to the lake or into the forest. Days passes by. This summer was the first with three children and that was kind of interesting; there is always someone who is tired, hungry, in a bad mood, or pulling your leg. Now we're back in our daily life routines; work, child care, dinner, sleep. We are very satisfied with our summer holiday but now it feels good to have these routines.

Marie feels that Elliot have been more tired this summer, but I haven't noticed any differences. But I trust Marie's senses more than mine in this case. This week at the child care the staff have mentioned to us that Elliot has been more tired than usual. Next walking test will be in September.
Next week Elliot will be 5 years old. Every morning he wonders how many days it is left until birthday. Right now he is into Spider man and Spider man things tops his wish list.
This Sunday I look forward to an appointment with the chairman of PAH Europe who will come to Stockholm to attend a Cardiology conference. I will met her as a representative for the Swedish network for PAH. I haven't been working with the Swedish web page (http://www.pah-sverige.se/) during summer but before we went to our summer holiday I did start a Facebook group and now the group have 23 members.

Take care

Web site released!

Before I have written about the Swedish network for PAH and my ambition to get engaged in the network. Finally I decided to take the responsibility to create a web site for the network. This was the first time since 1997 that I have created a web site and I must say that a lot have happened during this time. When you created a web site in 1997 you had to write the HTML code and create the graphics by you own. Now days creating a web site can be done just by drag and drop. You simply choose what theme you wish to use and what features you want. I used the very popular blog/cms tool Wordpress and the result of my drags and drops you can see at: http://www.pah-sverige.se/. The information is in Swedish only. I have also started a facebook group where the network members can get together virtually and exchange experiences. A question to you who reads this, are you member of a facebook group dedicated for PAH? I have not found an international facebook group for PAH, have you? Or, should I start such a group?

Yesterday Elliot did the 6 minutes walking test again but this time my wife, Marie, went with Elliot and I have not the numbers but Marie told me that he didn't walk as long as the last time but in the other hand his saturation and pulse was better this time. Last week Ida, our daughter, was examined by a doctor to make sure she doesn't have PAH. The result was positive and now both Olle and Ida have been examined.

Last week’s we have had real summer weather in Stockholm and now it's only 4 weeks until my summer vacation starts.

Diagnosis: life crises

At the latest walking test Elliot managed to walk longer than before, but his saturation and pulse was higher than before. There were no indications from the heart and lung check with ultra sound that his condition had changed. Now days, that feels like positive news. Life really is strange.

Last week we had a meeting with the psychologist that we have met a couple of times since last spring when Elliot condition was worse. Elliot was with us and this time he wasn't really interested in talking about his disease, but still it was great to hear him discuss his life from his point of view with the psychologist. Yesterday me and Marie had a follow up meeting and we were told she couldn't see that Elliot was in a need to meet her right now. But, she said, she had written a diagnosis, and it said: life crises and there she has a point. We (me and Marie) are in a constant mode of life crises, sometimes it's better, sometimes it's worse.

Today I got a letter from the Swedish PAH group, they are asking for help to put up a web site and to contact medical companies for financial support. I will engage, but I haven't decided how yet.
Right now, we are so much looking forward to meet the spring. Yesterday, we had snow again, bah!


Finally, I really apreciate all the comments I get;)

Water animal

Heading home after this weeks bathing at the therapy pool at the hospital. The water is 34 degrees celsius so it is real nice and Elliot is a real water animal, he spends more time under the surface than above. This week it is time to take the walking test. The past month Elliot has been in a pretty good shape, hopefully the test will confirm that. Today ends winter in Stockholm if the weather forecasts are correct.

Job start

Ending my first week at work after I have been home about one year. Well, how does it feel? Fine, I must say but on the other hand it feels like I have abandoned Marie and the kids.
"Feel how my heart is pounding", Elliot has been saying lately. This does not have to do with pah, but never the less, I am worrying. Winter time is hard on people with pah and yes, Elliot is more tired. Sometimes it feels like being tired is a constant condition for him and maybe it is. Olle is now in the same kindergarden as Elliot and it feels fine that they have each other during day time.
I have not heard from the swedish pah association but hopefully there will be a start up meeting before springtime.

Our 2009

Tonight I have been on a lecture about sourdough (surdeg in Swedish) and it was very interesting and inspiring. My interest in baking has grown this fall and as I have become better I find it more and more difficult to buy bread at the grocery (it's a difference between bread and bread and my bread is 10 times better compared with the bread you will find in an ordinary store, I promise;)).

Time to summarize the year. 2009 have been a year with many ups and downs. The first months was probably the worst in my/our life, we were still in shock after the meeting with the doctors and everything was like a black hole (and I lost most of my hearing during 4 months). In the middle of this we found out that Marie was pregnant again. From Mars, April things have become much better. The fact that both I and Marie have been able to stay home together since then is one of the reasons, another is of course that Elliot's condition have been stable. And then of course we got our baby Ida a sunny day in September.

Since mid November Marie have noticed that Elliot have been more tired (maybe me to, but I'm not convinced) and last week the staff from preschool also said that Elliot have been more tired lately. Bang, and it feels like you are balancing on the edge again. A couple of days ago we went to the hospital to do the last routine check for this year. Elliot did not walk as long as he have walked before, but the other tests did not indicate that his condition have been worse. The Swedish winter that is dark, cold, and long is hard on people with PAH.

Soon it's Christmas and I do hope for a long weekend with no surprises. Last Christmas eve we had to drive to the local hospital near our cottage with Elliot and this I don not want to do this year.

Notch-3

Scientist in the US have discovered that PAH is characterized by overexpression of the protein Notch-3. The discovery, presented 25th of October, is described as ground breaking for future medicins for PAH. It will be interesting to see how long it will take from discovery to a medicine. 5 years, 10 years?

http://health.ucsd.edu/news/2009/10-26-pulmonary-arterial-hypertension.htm