The Story of Primary Immunodeficiencies
"Despite the challenges, the story is not one of despair"

Sweden 1995

"It's alright for you to be ill - you are used to it"

This story was told the first time at the Nordic meeting in Gothenburg, Sweden in March 1995. It was t hen published in the PIO newsletter, PIO-bladet. You may recognise yourself in the well-informed, s ometimes sad - but at the same time happy and humorous - picture that Vivan Storm gives us here from her life as a patient with congenital immunodeficiency.

My name is Vivan Storm. I come from Huskvarna - that is about 10 km north of Jönköping. I am the contact person for PIO in the province of Jönköping.

In the beginning of the 80's I was in Gothenburg for an investigation when I there found out that there was an organisation for us patients with primary immunodeficiencies, and since then I have been a member.

Now I go to the infections clinic in Jönköping and get 50 ml subcutaneous gammaglobulin every week.

I have combined IgA deficiency and IgG-subclass deficiency.

The tiredness

We get extraordinarily well taken care of by both Dr. Olinder-Nielsen and the nurses here in Jönköping. For me it is important socially to go there once a week. We only talk about the disease if necessary. There are so many other things that have happened during each week that has passed which we discuss.

We lie, two people in the same room, and through this we can exchange opinions on a little bit of everything. It is nice and interesting to meet fellow-patients and listen to their descriptions of how they experience their disease, how they were before they were diagnosed etc. We recognise so many of the common symptoms, i.e. the tiredness, which is indescribable and difficult to understand for those who haven't experienced anything like it. For myself the tiredness is what sometimes makes it so hard to live. The lack of understanding from healthy people can sometimes be difficult. But we have to understand that they can say: 'You look like health itself, how can you be ill!'

Many years ago I met a man on the stairs - a neighbour who knew me well. He was usually a healthy man, an athlete. He told me that he had been lying ill with flu and had felt so bad. The last thing he said was: 'It's all right for you to be ill, you're used to it!'

Polio

My history of disease started when I was 10 years old - that was in 1938. Then I came to the hospital in Nässjö with double pneumonia. The ear-drums of both ears were broken after many otitises that hadn't been attended to. In the countryside there was no doctor.

When I was 13 years old, we moved to Huskvarna and that's when the real misery began. I immediately caught Scarlet Fever, and lay in the isolation hospital for seven weeks with a number of complications. At the age of 15, I got polio. I became completely paralysed from the waist down. We were 17 patients in the same room. I don't remember how long I was hospitalised, but I remember being very ill. I'll never forget the ache I had in my head and in the neck.

My father carried me like a rag-doll when I eventually went home. I was put to bed at home where I lay for several months. I got help from someone in the family when I needed to change position. We were four children in the family, but I was the only one to catch polio. There was never any question of rehabilitation. I didn't get any help from the medical profession.

The paralysis gradually let go and with help from the family I was able to start to learn everything from the beginning, like a child does. I still have some sequelae in my right leg but despite all I got off lightly. There were some deaths while I was hospitalised and several others were badly disabled for the rest of their lives.

I didn't want to live any longer

My pneumonias occurred at regular intervals. Water in the pleural sack, jaundice and everything imaginable in infections. Sinusitis repeatedly. An operation of the maxillary sinuses etc. had good effect. In 1956 I had had about 30 pneumonias. My family doctor in Huskvarna really felt sorry for me. He said: 'If you hadn't got your good humour, you would be dead by now!'

So many times I have wished to know what the matter was with me. At that time I reached a 41°C fever within an hour, when I became sick. But I didn't have any sedimentation rate! Never over 5. He found this so surprising.

In 1954 I had a son. I worked until a month before the delivery and as far as I can recall I felt good.In 1956 I was pregnant again and this year was a black spot in my life. I was so ill for the entire year and I was on so many medicines that I can't now understand how my new daughter didn't get injured for life. I was later told by a doctor that I had received 'pure dope'. I didn't want to live any longer, I prayed many times that I would be allowed to die.

One of the ten first in Sweden

But fate ruled otherwise. After every pneumonia I got my lungs x-rayed. Each time I met a kind and pleasant doctor named Jerlock. Dr. Jerlock had attended a lecture by a Dr. Carl Cöster who, it turned out, was researching immunodeficiency diseases. My symptoms indicated that I could also have an immunodeficiency.

It was decided that when I had had my second child, which happened on January 31st 1957, I was to be hospitalised in the medical ward as soon as I could. In May 1957 the time came. The doctor was very interested, and lots of tests were taken. Answers arrived. The diagnosis I then received was Hypogammaglobulinaemia. According to Dr. Cöster I was one of the first ten in Sweden to be recognised with this disease.

Gammaglobulin was then, just as now, very expensive. I had to be hospitalised for 24 hours every two weeks to get it for free. After a few months the county council was contacted so that I could get it free of charge. In 1958 I think gammaglobulin became a free medicine. For 38 years I have received gammaglobulin. At first in the buttocks, with long needles, as it had to go in deep. My muscles eventually became as tough as chewing-gum. The needles sometimes bounced out.

In the 70's I was hospitalised for severe dizziness. Dr. Molender somehow connected the dizziness with the gammaglobulin. He then changed the prescription so that I should have the needles in the back in four places. Three times a week in my lunch-break I went to the medical reception. There I stood in the middle of the floor in the nurse's room as she stuck the needles into me, 5 ml in each place, at a frantic pace.

It wasn't as funny when she wasn't there and someone else had to do it. Sweat has flowed on many a forehead before they have dared to stick needles in me. In Strömstad I spent a whole day in a waiting-room, since no-one dared to do it. Finally an 'angel' came to my rescue. She had a daughter who needed gammaglobulin.

Gammaglobulin in the Canary Islands

In the Canary Islands it wasn't easy either to get a doctor who would stick needles in four places in my back. After a lot of shilly-shallying he stuck them in me in two places. I was there for 17 days and certainly I was afraid of the consequences. There were more visits during this time. He came in his jeep covered in smoke and dust. His hands weren't the cleanest I have seen and he didn't wash them either. He held a cigarette in one hand and stuck the needles in with the other. But everything turned out fine.

One should live in the Canary Islands during the winter. That would be great. After one day there my daughter said: 'But mum, you are so different here than at home! You're like a totally different person.' I have felt great the times I have been there.

Got work after being home for 15 years

I must tell you something positive about my life as well. When you had a baby in the 50's, you quit your job and stayed at home with the children, if you didn't hold a high office or education, any way.

In my case I think that was my saviour, and I got good use and help from the gammaglobulin. I was at home for 15 years, and then something happened which I could never have dreamt of after my long life of illness: In 1970 I started working full time at the tax administration office in Jönköping, and stayed there for 20 years. I'm happy for these years of my life.

Something else that I'm happy for is that in all these years I have had the great luck of being treated by the same doctors. For 38 years there have been three doctors taking care of my gammaglobulin treatment: Dr. Cöster, who gave the lecture in 1956. He was to write a treatise and said I would get a copy, he promised. He moved to Ystad and died quite soon of cancer. Dr. Molander succeeded him and he took care of me in the best possible way until I came to the infections clinic at my own request in the beginning of the 80s. Since then I have received, and still receive, the best possible help from Dr. Olinder-Nielsen.

My ear specialist has for many years been Dr. Nielsen; he is thus married to my infections specialist. I couldn't have had it better. He was the one who in the beginning of the 80's told me about Dr. Björkander at the Sahlgrenska hospital, and that he also knew that there was an immunodeficiency patients' organisation.

I took matters into my own hands and tried to persuade Dr. Molander on the medical ward, as my health was starting to decline again and it sounded very interesting to go to Gothenburg for further investigation. I am very grateful that Dr. Björkander took so many tests then. I met Alice Heestrand, who at that time was president of the Gothenburg section of IBF, which was the name of the organisation back then. She gave me good advice that I have thought about many times and often shared: For example; when it is about the tiredness: Rest as much as you can, when you can; don't use your remaining strength unnecessarily.

Last week I read in a paper: 'Happy types live longer'. Now it is once more confirmed: 'Mirth prolongeth life and causeth health'.

Scientists in California have found that laughter effects the human immune system. The ten healthy men who were part of the investigation all got to watch funny movies for 60 minutes. Afterwards all test subjects showed increased values of the important defence substance gamma-interferon. The values remained also during the following day. Earlier research has proved that laughter can effect a number of factors, for example stress-related hormones and several other immune defence substances.

Vivan Storm (First published in PIO-bladet 1995)