0255-In Memoriam

In memoriam Eric Baas

My dearest, our dear Dad, our father-in-law, my grandfather, my son isn’t there any more

Eric Baas

b. Amersfoort, March 16, 1956                                       d. Ede, October 1, 2017


Eric came in our life during the Walk of the World marches edition 2006, when he arrived at our door via the organisation for walkers who are in search for a bed and breakfast for the four marching days (starting the 3rd Tuesday of July every year). He continued to stay with us including the marches of the WOTW edition 101 last July. He already knew at the time of the marches that he was very ill: June 4th he sent me a WhatsApp, telling that he suffered from pancreatic cancer, and that his liver was already also infected. The prognosis is: not curable, and a time to live for about 3 – 6 months. However he insisted on marching, but was wise enough to switch from 40K a day to 30K a day. And July 21 he proudly collected his medal. IMG-20170723-WA0001

Eric in the center, with red cap

When Eric arrived on Monday June 17, he went off to get his registration, and hang out in the town. He told me about his visit to the St Stephen Church, and that he had written something in the Guestbook of the Remembrance Chapel. As I am a volunteer at the church, I checked the book during my next duty, and found the following entry: 

201707221538Stevenskerk

God. This could be my last WOTW. Because little Baas is doomed! But today I fight, tomorrow I shall fight and also the day after tomorrow. I can not win this soccer match, but I certainly go for the game extension. And perhaps I shall loose shooting the penalties.

Help me with this fight against that f..king cancer.

Little Baas

Eric never came alone during all these years. As he was caregiver for several soccer teams, he had lots of friends to accompany him walking the marches. Also his son Rutger Baas stayed with us. And -brought by Rutger- even his father in a wheelchair and later the handicapped grandson Twan were there to cheer his grandfather at the 3rd day of the marches, the so called Day of Groesbeek, after the Canadian War Cimetary at Groesbeek.

201507231858Vierdaagse

201507231859Vierdaagse

And when you are injured, it is always handy that you are staying in the house of a former G.P., such as here on July 23, 2015 🙂

0238-Walk of the World

Walk of the World edition #101

One guest stays always for five nights, and that is Eric Baas. He came to our “Marches Shelter” first in July 2006, when the WOTW #090 was cancelled after the first day: extreme heat and 2 deaths. Here an impression of his last breakfast with us, before picking up his wife Sandra @ Geldermalsen and going home to Ede.

ERIC’S ENTRY IN THE REMEMBRANCE BOOK IN THE CHAPEL OF SILENCE

(english translation under the picture)

July 17, 2017
God, this may be my last Four Days Marches. Because Boss is doomed to die. But today I fight, and tomorrow I shall fight, and the day after tomorrow I shall fight too. I can not win the match, but I’m sure to extend the playing time. And maybe lose, lose with the shooting of penalties. Help me with this fight against that horrible pancreas cancer.

BOSS.

THE GUESTS ALWAYS BRING A GIFT FOR THE HOSTS. ERIC: FROM T-SHIRTS TO HAND ENGRAVED WATER GLASSES; DICK: CHEESE SPECIALITIES FROM THE BEEMSTER AND ED WHINE.

AT THE MOMENT AN ART EXHIBITION IN THE SAINT STEPHEN CHURCH:

Mondriaan impression with the light of the church window covering the painting.

Mondriaan impression in its original form and colours.

0237-Walk of the World

Walk of the World edition #101

47,000 registrations

04,964 no show

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42,036 start permits day 1

00,792 no show/not finished

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41,244 start permits day 2

01,525 no show/not finished 

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39,719 start permits day 3

01,002 no show/not finished

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38,717 start permits day 4

00,308 no show/not finished

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38,409 COMPLETED MARCHES

TRYING TO GET TO THE FINISH… A REAL HELL! WE HAVE SEEN ALL PARTS OF NIJMEGEN, EXCEPT THE FINISH @ THE WEDREN 😦 😦 😦

A SURPRISE FOR ERIC WHEN HE PASSES (NEARLY) THE FINISH ON THE 4TH DAY: A LOT OF (EX) FOOTBALL MATES AND FRIENDS HAD GATHERED, WEARING BLACK T-SHIRTS WITH “BAAS” (“BOSS”) ON IT, TO GIVE HIM A WARM WELCOME ON HIS WOTW #101. ERIC IS WEARING THE WHITE T-SHIRT AND A RED CAP.

Under Bridges That You Built for Me Artwork

RUPERT BLACKMAN IS AN ENGLISH SINGER, SONGWRITER AND PRODUCER BASED IN AMSTERDAM. ONCE A SOLO ARTIST, HE NOW FRONTS THE BAND CAUSES WHOSE DEBUT SINGLE TEACH ME HOW TO DANCE WITH YOU WAS RELEASED IN 2015. HE ALSO WRITES SONGS FOR A VARIETY OF ARTISTS WORLDWIDE. YOU CAN FIND HIM ONLINE: 

@RUPERTBLACKMAN AND @CAUSESMUSIC.

0228-Twan Foundation

2017/07/06

  • Eric Baas (b. March 16, 1956) -Twan’s grandfather-  comes to Nijmegen for the Walk of the World marches (Vierdaagse) since 2006 and seeks shelter at our home. Eric is a fervent caregiver associated with several soccer teams as VV. Bennekom. As the WotW edition 2006 ended after the first day (temperatures >30C, 2 deaths), the counts for Eric (marching 50K per day, 4 days in a row) started in 2007. Last year he walked for the 10th time the WotW, in the 100th edition of the WotW. Nowadays Eric walks the 40K per day, preferably with an early start (distances per day for men, compulsary: 12y-15y: 30K, 16y-18y: 40K, 19y-49y: 50K, 50y-59y: 40K, 60y and older: 30K). Eric is a former marine. From his first marriage Eric has a son and a daughter.
  • Rutger Baas (b. October 4th, 1982) -the father of Twan- made also his career in the marine corps. He is a very sportive man, who rowed e.g. last year accross the North Sea to England, to raise funds for the Twan Foundtion. He joined his father several times in the WotW, and as a marine the compulsary 50K per day was easy for him 🙂 . Since the difficulties with Twan, Rutger does not walk anymore the WotW. But, he sometimes shows up. along the parcours… At the moment he works for the Defense department, and is specialised in escorting severe criminals, but is also charged escorting politicians and diplomats. Rutger is a member of the board of Twan Foundation; his wife Rianne is the president.
  • Foto-TwanTwan Baas (b. Sep-tember 2010) is the third generation in line. He suffers from the rare disease A-T (Ataxia Telangiectasia) -in the Netherlands about only 15 cases-. But he is a very lively, cheerful boy although he is very limited in action, because he gets tired very fast. You can read more about Twan and the A-T disease here. He is treated by Professor Doctor Michèl Willemsen, Child neuro-logist.
  • Prof. Dr. Michèl Willemsen, Child Neuro-logist, Management Expertise Center REEB – Children @ UMCN Radboud, Nijmegen, The Netherlands. Michèl Willemsen specializes in the treatment and guidance of children with neurometabolic and neurodegenerative diseases and of hq720children with movement disorders. He has specific expertise in ataxia teleangiectasia, GLUT1 deficiency syndrome, Sjögren-Larsson syndro-me and neurotransmitter diseases. Michèl Willem-sen studied medicine at the Radboud University Nijmegen from 1986 to 1990; In 1993 he obtained an art exam at UMC St Radboud. He then worked for a year as AGNIO for pediatrics, and from 1994 to 1999 he studied the pediatric and pediatric neurologist. In early 2000, Willemsen was registered as a pediatrician and a child neurologist. In 2001, Willemsen at Radboud University (cum laude) researched the Sjögren-Larsson Syndrome, a hereditary metabolic disease involving the brain, eyes and skin. His research activities have always focused on hereditary brain diseases (especially metabolic diseases), and movement disorders in that context. In 2006, Dr. Willemsen was appointed Head of Department of Child Neurology. Since 2008, Willemsen is a staff member of the Department of Neurology in UMC St Radboud and Head of Child Neurology. He is a member of the daily board of the Dutch Nursing for Child Neurology (NVKN) and the European Pediatric Neurology Society (EPNS).

201407181400Certificaat

YOUR GIFT IS IMPORTANT FOR ALL THE CHILDREN WORLD WIDE, WHO SUFFER FROM A-T. THE TWAN FOUNDATION HELPS INTERNATIONAL RESEARCH.

0224-Twan Foundation

TWAN BAAS FOUNDATION

Who is Twan?

Twan is the reason for the creation of the Twan Foundation. Twan is the son of Rutger Baas and Rianne Kranendonk (founders Twan Foundation). Via the link  you will get a picture of Twan and his parents. Twan was born in September 2010 and is a happy male. Until recently, Twan went 3 days a week for therapy to Rehabilitation Center Groot Klimmendaal in Arnhem. Twan, however, goes to school from August 2014; This is a school in Arnhem for children with physical disabilities. At this school, Twan also receives therapy. Therapy is aimed at maintaining the functions that are now there. Under the supervision of a rehabilitation physician, Twan receives physical therapy, speech therapy and ergotherapy. Twan currently has a child rollator with wich he walks along and has a wheelchair for longer distances. He can walk himself, but he has a lot of effort to stay alive and to walk straight, it seems like he’s drunk. Sometimes it may happen that he is sinking through his legs. Walking is often too tiring. Furthermore, Twan receives a low dose of antibiotics daily. This is to prevent him from becoming ill. Twan’s immune system operates less well than normal. As a result, he is more vulnerable than other children. Before the diagnosis was made, Twan was very ill at times. Because of the antibiotics he is almost no longer sick. Twan can therefore enjoy the “child” much more and everything that belongs to. Since September 2016 Twan gets an infusion of ‘gammaglobulin’ every three weeks, taking off his white blood cells, making him less effective against infections, etc. This infusion was given to the hospital for the first time, but nowadays Sanquin’s nurse will be at Twan home Infusion so that he can stay in his trusted and safe environment. Twan is very tired tired. Activities must be divided throughout the day and alternated with rest periods. Fortunately, Twan is starting to learn this now, which means he can get everything out of his way! Twan goes to a special ‘poli day’ at the St. Radboud Hospital in Nijmegen twice a year. There is a professor (for Twan doctor Michél) who specializes in, among other things, this rare disease.


Ataxia-Telangiectasia

Background

T3WAtaxia-telangiectasia (A-T) is an autosomal recessive, complex, multisystem disorder characterized by progressive neurologic impairment, cerebellar ataxia, variable immunodeficiency with susceptibility to sinopulmonary infections, impaired organ maturation, x-ray hypersensitivity, ocular and cutaneous telangiectasia (see image below), and a predisposition to malignancy. The disease is hetero-geneous, both clinically and genetically, as shown by the existence of 4 complementation groups (A, C, D, E). The responsible gene (ATM gene) has been mapped to band 11q22-23.  The clinical and immunological presentation of ataxia-telangiectasia may differ even within the same family, as described by Soresina et al.  Syllaba and Henner first published descriptions of patients with ataxia-telangiectasia in 1926.  They observed progressive choreo-athetosis and ocular telangiectasia in 3 members of a single family. A gap of some 15 years occurred before the next report in 1941 by Louis-Bar, who described progressive cerebellar ataxia and cutaneous telangiectasia in a Belgian child.  The syndrome subsequently received the name of Louis-Bar. Ataxia-telangiectasia was not described as a distinct clinical entity for another 16 years until Boder and Sedgwick[in 1957 and Biemond  in 1957, with the aid of autopsies, reported organ developmental abnormalities; neurologic manifestations; and a third major feature of the disease, recurrent sinopulmonary infection. Ataxia-telangiectasia can best be classified, according to its major clinical and pathologic features, as a predominantly cerebellar form of spinocerebellar degeneration, which is transmitted as an autosomal recessive trait and evolves ultimately to include motor neuron disease, with spinal muscular atrophy and peripheral neuropathy. Ataxia-telangiectasia can also be classified among the neurocutaneous syndromes, although not among the phakomatoses as originally proposed, because the vascular and cutaneous lesions of ataxia-telangiectasia are not congenital nevi but develop in the course of the disease as a progeric manifestation. Ataxia-telangiectasia should be considered among the immunodeficiency diseases, cancer-prone genetic disorders, chromosomal instability syndromes, disorders with abnormal radiosensitivity, syndromes with possible DNA-repair/processing defects, and (as is now evident) the progeroid syndromes. Elevated immunoglobulin M (IgM) occurs in only 60% of patients, challenging this finding as a probable diagnosis criterion. 

Pathophysiology

T2WThe ATM gene encodes the protein kinase ATM, which is the key regulator of cellular response to double-strand breaks (DSB) in DNA. Therefore, ataxia-telangiectasia symptoms include all the possible consequences of the perturbations in DNA damage response (DDR). One basic defect associated with the malady is the abnormal sensitivity of ataxia-telangiectasia cells to x-rays and certain radiomimetic chemicals but not to ultraviolet irradiation, which leads to chromosome and chromatid breaks. Breakpoints are randomly distributed, but nonrandom chromosome rearran-gements selectively affect chromo-somes 7 and 14 at sites that are concerned with T-cell receptors and heavy-chain immunoglobulin coding and with the development of hematologic malignancies. Such disturbances could account for the frequency of infections and neoplasias. As has been shown by Guerra-Maranhao et al, ataxia-telangiectasia patients are at high risk of having impaired responses to infection with pneumococci, which may be one of the causes of recurrent sinopulmonary infections in these patients.  The authors analyzed the production of antibodies to polysaccharide antigens in patients with ataxia-telangiectasia and found that the levels of immunoglobulin G (IgG) antibodies to serotypes 1, 3, 5, 6B, 9V, and 14 of Streptococcus pneumoniaebefore and after immunization with 23-valent polysaccharide vaccine were significantly lower than in a healthy population. ATM gene targets include well-known tumor suppressor genes such as TP53 and BRCA1, both of which play an important role in the predisposition to breast cancer. Studies of ataxia-telangiectasia families have consistently reported an increased risk of breast cancer in women with one mutated ATM gene,  but, to date, an increased frequency of ATM mutations has not been found in women with breast cancer. ATM mutations are poor prognostic factor in patients with lung cancer.  The mechanisms responsible for neurologic disease, thymus aplasia, telangiectasias, growth retardation, and impaired organ mutation have not been elucidated, but most likely, they are linked to accelerated telomere loss.  ATMhas been shown to be pivotal for neurodevelopment, especially for stem cell differentiation, as well as for elimination of damaged postmitotic cells.  Frappart and McKinnon showed that the ATM protein has a proapoptotic function in the developing mouse CNS, acting in cooperation with another key proapoptotic factor—Bax protein.  ATM-dependent apoptosis occurred only in postmitotic populations of neurons after irradiation. These results suggest that ATM may serve to eliminate neurons with excessive DNA damage during CNS development. A general disturbance in tissue differentiation accounts for the almost constant elevation of alpha-fetoprotein (AFP), a fetal serum protein of hepatic origin that indicates dedifferentiation of liver cells. Research suggests that ataxia-telangiectasia may be associated with dysregulation of the immunoglobulin gene superfamily, which includes genes for T-cell receptors. The normal switch from the production of immunoglobulin M (IgM) to IgG, immunoglobulin A (IgA), and immunoglobulin E (IgE) is defec-tive, and the same may apply to the switch from immature T cells that express the gamma/delta rather than the alpha/beta receptors. Conceivably, an absence or a mutation of a single protein coded for by chromosome 11 could explain the immunologic and perhaps even the neurologic features of the disease. The ATM protein apparently controls the cell cycle and plays a major role in the protection of the genome. The ATM gene product has been shown to be required for cell survival and genomic stability maintenance following exposure to low labile iron concentrations. Because iron chelation agents increase ataxia-telangiectasia cell genomic stability and viability and activate ATM-dependent cellular events in normal cells, Edwin Shackelford et al suggested that pharmacological manipulation of ATM activity via iron chelation might have clinical efficacy in Parkinson disease treat-ment.  Targeted next-generation sequencing is a rapid cost-effective method that identified five disease-causing variants in three Chinese probands in one study.

Epidemiology

Frequency

Ataxia-telangiectasia is reported in all regions of the world. The probable incidence of ataxia-telangiectasia is about 1 case in 100,000 births.  The frequency of ataxia-telangiectasia mutant allele heterozygosity was reported to be 1.4-2% of the general population.  The incidence of ataxia-telangiectasia was significantly higher in the highly consanguineous Bedouin population than in the relatively nonconsanguineous Jewish population of southern Israel.

Race

Ataxia-telangiectasia is reported in all races, although the mortality ratios differ between the ethnic groups.

Sex

Ataxia-telangiectasia occurs equally among males and females.

Age

No characteristic features are detectable during very early childhood. Ataxia is usually a first diagnostic hallmark, having its onset in the first years of life. Beyond the age of 5 years, the progression of the ataxia becomes increasingly apparent and the child requires a wheelchair by age 10 or 11 years. Trimis et al reported a 6-year-old girl without any neurological symptoms. Oculocutaneous telangiectasia, the second diagnostic hallmark of ataxia-telangiectasia, usually has a later onset than the ataxia, typically at age 3-6 years. The progression of the disease is apparent in subsequent years.

Prognosis

The neurologic features of ataxia-telangiectasia are relentlessly progressive. In addition to the classic early features, older patients tend to develop other signs of spinocerebellar degeneration (eg, posterior cord involvement with loss of the deep tendon reflexes, spinal muscular atrophy). Most patients are wheelchair dependent by age 10-15 years, but mild forms are not rare. Gene therapy holds promise for the future.  A 20-month-old girl with T-cell acute lymphoblastic leukemia and ataxia-telangiectasia was apparently cured after only 7 weeks of antileukemic therapy, as she was reported in remission for 8 years.  Death typically occurs in early or middle adolescence, usually from bronchopulmonary infection, less frequently from malignancy, or from a combination of both. The median age at death is reported to be approximately 20 years.  To date, the longest reported survival is 34 years.  In a retrospective study in the United States, mortality from all causes in ataxia-telangiectasia was 50- and 147-fold higher for white and black patients with ataxia-telangiectasia, respectively, than expected based on overall US mortality rates.  Boder reviewed 58 complete autopsy cases; 27 (46%) deaths were caused by pulmonary infection alone, 12 (21%) by malignancy alone, 16 (28%) by a combination of both, and 3 (5%) by other reasons.  The lifetime risk of cancer among patients with ataxia-telangiectasia has been estimated to be 10-38%, which is about 100-fold more than the population rate; however, in the absence of chronic bronchopulmonary disease and lymphoreticular malignancy, ataxia-telangiectasia is consistent with survival into the fifth or sixth decade. Ataxia-telangiectasia heterozygotes present an excess risk of death (they die 7-8 y earlier than the normal population), mostly from ischemic heart disease (ataxia-telangiectasia carriers die 11 y younger than noncarriers) or cancer (ataxia-telangiectasia carriers die 4 y younger than noncarriers). 

Patient Education

Good patient and parental education should include tactful genetic counseling and an explanation of the multisystem nature of the disease. Pay special attention to the susceptibility of adult members of families with ataxia-telangiectasia to malignant neoplasms and to the importance of regular examinations for early cancer detection.


We can not do without your support!

 

We can use your support very well … With your support, we mean: your ideas and initiatives, your time, but also your financial contribution. Do you have an idea or an initiative? And are the goals similar to those of us: namely to raise awareness of Ataxia Telangiectasia and / or raise money to support A-T research? Do you have a special gift, a special quality? Then we are very curious !! And we would appreciate it if you contact us …

Even if you can do something for us with your company or shop, we are happy to talk to you. Think of a piggy bank on the counter or at the corporate office, or maybe you would like to contribute to the Twan Foundation through your Christmas package … There are plenty of possibilities and we would like to think or arrange a brief presentation about our work during a meeting. . For example, the staff of ‘Stadscafé Mister Owl’ have made their money available for the Twan Foundation for two months. And the flower shop ‘De Veender’ from Ede has put a piggy bank for the Twan Foundation for a number of months.

Of course, it is also possible to make a contribution to our account number: BIC RABONL2U, IBAN NL63RABO0153.1109.96 in the name of Twan Foundation in Veenendaal

 Thank you very much!

0015-Walk of the World

As a gesture of gratitude for 11 (eleven 🙂 ) years of hospitality, Eric Baas honored us as “Friend of the Walk of the World Marches”. Eric started his career with us in 2006, when -because of the extreme temperatures and 2 deaths- the marches were cancelled after the first day. All following years Eric was a faithful guest, and he brought his son and friends with him. Our “normal” guest room has a double bed, and a third bed can be added easily. But 1 year we had 5 guests, also on our “own” sleeping floor. The last 5 years we have a steady group. We have the traditional welcome dinner on Monday evening, and the farewell dinner on Thursday evening, as only Eric stays over on the 5th (Friday) night.

201607241548Nijmegen

50,000 start permits Walk of the World edition #100

 -2,834 no show

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47,166 start permits day 1 Walk of the World edition #100

  -1,206 not started / not finished day 1

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45,960 start permits day 2 Walk of the World edition #100

 -2,050 not started / not finished day 2

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43,910 start permits day 3 Walk of the World edition #100

    -914 not started / not finished day 3

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42,996 start permits day 4 Walk of the World edition #100

  – 439 not started / not finished day 4

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42,557 finishers Walk of the World edition #100Walk_of_the_World_logo