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

PAH meeting in Stockholm

Two weeks ago I and Marie went to a meeting that was arranged by the Swedish network for people with PAH. The information about the meeting reached me from a contact in the Norwegian PAH-forum just a few days before the meeting (thanks Kristen). The meeting was financed by Actelion, the company behind the medicine Tracleer (bosentan). The person representing the company said that only 5-7 children use Tracleer in Sweden. Both Olle (16 months) and Ida (4 weeks) did come along at the meeting, so it was hard to stay focus all the time;)
For me and my wife this was the first time we met other persons with the diseases in real life. About 20 persons with PAH came to the meeting and it was interesting to hear their stories. If I remember correctly none of the participants was diagnosed with PAH before 2005. What I have read about the disease, woman between 20 and 40 is an over represented group but most of the participants was woman +60. I wonder why?
The main purpose was to get people with the disease together, but the network also had invited a PAH specialist and his team from the PAH center at Karolinska University Hospital in Stockholm. For me it was news that it did exist a PAH center in Stockholm. This center is for adults only and not for children, but still I think it is strange that they have not told us about this center at the Astrid Lindgrens Child Hospital where Elliot is a patient.
Today the people behind the network consist only of three amazing persons and this, I think, was the 3rd or 4th network meeting they arranged. We were the only ones with a child with PAH at the meeting and after the meeting a volunteered to become a member in the network and a contact to other parents with children with PAH.





The photo shows Elliots drawing of our family. This was the first time ever that he was drawing humans at home.

Condition test

Last week Elliot did the condition test again and his condition has not been worse since he did the test in May. Hopefully Elliot will get the flu vaccine this month. So far the weather has been fine in Stockholm, which means the children at kinder garden have been outside most of the time and Elliot has been free from diseases.

The chart shows the result after Elliots condition test from the start in December 2008 until the last one in October 2009. The condition test is simple, he walks for 6 minutes in normal pace. The vertical axis shows Meters/Sat before and after/Pulse before/after and the Horizonalt axis shows when he did the test.

Elliot got a little sister

28th of September Elliot got a little sister with the name Ida. He likes to hold Ida in his lap and so far he has been incredible with her. It’s incredible that we now have three fantastic children.

Back to reality

For 2,5 months we have been living in a happy bubble. Our bubble is a cottage on the Swedish west coast. There, life is simple, you wake up, eat breakfast, plan the day, which means either go to the lake for a bath or out in the forest to pick berries or mushrooms. This summer Elliot has developed incredibly. His new outside favorite is to climb; he climbs trees, stones and anything that comes in his way.
Since 2 weeks we are back in Stockholm and the ordinary life and today it was time to go for a routine check at the hospital. The nurse who usually takes care of the walking test was ill and it had to be cancelled. The heart and lung check with ultra sound gave (as usually) no hints of Elliot’s condition regarding the lung pressure, but the doctor’s short summary of the ultra sound was ok: nothing had become worse during summer.
There is a big playing room whith lot of toys and games at the hospital. The image shows Elliot and Marie playing table hockey which is a popular game in Sweden (we usually stay a while at the hospital to play after his doctor visit, this strategy has been good, now Elliot feels it is ok to go to the hospital and he looks forward to go to the playing room).
Right now I’m a bit worried about the new flu. I’m not alone in my worries, media have gone mad and it is happy days for a few medical companies. I'm worried about his lungs. I would like to go back to the cottage for another month. Our doctor hopes that Elliot can have his first vaccine injection in late September or early October. So it is at least 3 long weeks…
It’s only 5 weeks left to our third baby if everything goes according to the plan. Marie is now very pregnant;) Both me and Marie believes it is a boy.

Lazy summer days

Today we have been in our summer house on the Swedish west coast for three weeks. The past two weeks the summer weather have been incredible, we have been bathing in our lake and in the ocean every day. Elliot is a real water animal, he loves to play in the water and the past week he have made some brave attempts to swim. Look dad, he says and then he disappears under water a couple of seconds while his arms and legs are fencing. Did you see me swimming he says with big eyes while he is catching his breath. My beautiful Marie’s stomach is growing with our third baby, if everything follows the plan the baby will come in mid October.

Amazing response

A couple a days ago I posted a message to the PH Association message board. The response was quick and wonderful. It is great to the engagement and how they support each other. Several people suggested Flolan a medicine that I don’t recall we have discussed with Elliot’s doctor.

“Flolan or epoprostenol is a man-made form of a naturally occurring molecule in the human body called prostaglandin which helps the body open blood vessels. This synthetic substance contained in Flolan is called Prostacyclin.

Doctors use Flolan (Prostacyclin) to treat patients with Primary Pulmonary Hypertension (PPH). Flolan is helping many severely ill patients who do not respond to treatment with calcium channel blockers such as Nifedipine. Flolan imitates the natural prostaglandin produced by the body to keep blood vessels healthy by removing the build up of lipids, lowering blood pressure. People respond to increased doses of Flolan which dilate, or open up, blood vessels in the lungs and throughout the body. This Flolan-Prostacyclin also appears to prevent blood clots from forming. Flolan has been studied in clinical trials, and is an FDA approved drug treatment for PPH.

Flolan is administered intravenously directly into the bloodstream through a surgically implanted catheter by a portable, battery-operated pump. The pump is worn attached to a belt around the waist or carried in a small shoulder pack. Since the drug lasts only 3-5 minutes it must constantly be infused: it is slowly and continuously pumped into the body through the permanent catheter placed in a vein in the neck or chest. The pump is filled daily with the mixed Flolan solution.” From www.flolan-center.com.

Carpe diem

This weekend my wife Marie was away to meet other mothers who have heart related diseases. The weekend that was arranged by The Swedish Heart-Children's Association and Marie was a positive but tired when she returned. At home, grandma came and stayed with us while Marie was gone and we have had some nice days with great weather. Yesterday Elliot told us he had so much run in his legs and to hear him say something like that makes me feel very happy. I and Marie talks a lot about to live here and now and not to focus on what might happen with Elliot. Sometimes this is impossible but we try to do our best.

Today has been a good day.

A day at the hospital

Today we took both Elliot and Olle to the hospital. First Elliot did the condition test and this time it was positive, his sat before the test was 96 and the pulse 86 and after a 6 minutes’ walk the sat still was 96 and the pulse not higher than 90. This was the third time he did this test and this time he walked 328 meters compared to 250 meters the other times. The heart and lung check with ultra sound did not say anything new; the doctor only mentioned that the right heart chamber not had changed which is positive. The doctor also looked at Olle with ultra sound, this just to make sure that he not has the same symptoms as Elliot. Olle was okay so this was probably the last time Olle had to do this test.

Right know I’m tired but happy, these days at the hospital takes all my energy. Hopefully we don’t have to return to the hospital before August/September. In mid June we will go to our cottage in the Swedish west coast and we will stay there for more than 2 month. It will be luxury to be all together for such a long time.

We are not alone

Right now a lot happen, I heard from another parent that there is a big international study going about children and PH. I think there is a new medicine that is being tested. I wrote a mail to a professor in Sweden who is a specialist in PH and who is involved in this study. In the letter I described Elliot’s history with PH and I got a quick answer. The professor’s opinion was that we have the best treatment there is to have.

The day before yesterday I made my first entry to the Nordic message board for PH. I have already made contacts via this forum and it feels great to know that you are not alone in this mess. I have also found the message board that PH Association has and it was a positive discovery to find that they have a forum for parents with children that have PH. You will find the links to these two boards in my link list.

Yesterday I paid a visit to my ear doctor and this was not a positive meeting. I now have Ménière’s disease (tinnitus, loss of hearing, dizziness) on both my ears. I got diagnosed for Ménière on my left ear in 1996 and now it’s on both my ears. My belief is that it is Elliot’s disease has to do with this negative progress, I worry a lot and this affects my hearing.

Springtime in Stockholm

It's springtime in Stockholm and all over its green. It's really beautiful know. Since our last visit with Elliot to the hospital in February we have had a really good time. Both I and Marie have been home full time with our baby boy Olle and Elliot have been okay. When Elliot is playing and running he has to do frequent stops because his "legs are tired" as he explains it. But beside of these symptoms and that he has to take medicines three times a day we don't notice his disease. But still we worry a lot...

Our next visit to the hospital will be in late May and the routine is that he first do a condition test. He walks for 6 minutes and before and after the walk a nurse checks his pulse and saturation. Then the doctor checks his heart and lungs with ultrasound. But without catheterization it's difficult to say something about the lung pressure. The latest cat was made in December 2008 and the outcome was negative, Elliot has not responded to the medicines, but we still hope for a positive change.

During this spring I have got in contact with some other parents who have children with PAH in Sweden. But since the disease is very rare my hope with this blogg is to get in contact with parents outside Sweden.